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Sample records for hypocomplementemic urticarial vasculitis

  1. Hypocomplementemic Urticarial Vasculitis Syndrome

    PubMed Central

    Christensen, Jim; McCarty, Morgan

    2012-01-01

    Hypocomplementemic urticarial vasculitis syndrome, as opposed to urticarial vasculitis or urticarial vasculitis syndrome, is a rare disease process where the exact pathophysiology remains unknown. This article discusses the case of a 34-year-old Hispanic man with an ongoing history of chronic urticaria comprising episodes induced by low ambient temperatures, emotional stress, and spontaneous occurrences. This article serves as a consolidated reference for specialists to comprehensively review the plethora of systemic manifestations that may accompany urticarial vasculitis and highlights new systemic complications reported in association with this disease which are also observed in this case. PMID:22328958

  2. Urticarial Vasculitis

    MedlinePlus

    ... Store Home / Resources / Forms of Vasculitis / Urticarial Vasculitis Urticarial Vasculitis Urticarial vasculitis is a form of vasculitis that ... of complements; it’s called hypocomplementemic vasculitis. What causes Urticarial Vasculitis? The cause of most cases of urticarial vasculitis ...

  3. Hypocomplementemic urticarial vasculitis or systemic lupus erythematosus?

    PubMed

    Trendelenburg, M; Courvoisier, S; Späth, P J; Moll, S; Mihatsch, M; Itin, P; Schifferli, J A

    1999-10-01

    The 2 patients presented here showed the typical signs of hypocomplementemic urticarial vasculitis syndrome (HUVS). During follow-up, there was an inverse correlation between anti-C1q autoantibody titer and C1q antigen concentration in serum in both patients over a period of 2 years. The first patient had nephritis characterized by immune deposits in glomeruli and around the tubules. The histological findings, C1q deposits, and presence of tubuloreticular inclusions in capillary endothelial cells suggested a disease process identical to systemic lupus erythematosus (SLE). The second patient, after a lag phase of 2 years, fulfilled a fourth American College of Rheumatology criteria for SLE when she developed anti-double-stranded DNA antibodies. HUVS and SLE overlap, and the criteria for identifying HUVS as an entity distinct from SLE are lacking. PMID:10516358

  4. [Hypocomplementemic urticarial vasculitis syndrome. Successful therapy with intravenous immunoglobulins].

    PubMed

    Staubach-Renz, P; von Stebut, E; Bräuninger, W; Maurer, M; Steinbrink, K

    2007-08-01

    Autoimmune diseases can initially present as chronic urticaria. We describe the course of a patient with hypocomplementemic urticarial vasculitis syndrome (HUVS) as well as his successful treatment with high-dose intravenous immunoglobulins (IVIG). HUVS was diagnosed clinically and confirmed by histology and laboratory studies. After only one cycle with IVIG (2 g/kg) all HUVS symptoms were significantly decreased. PMID:17453168

  5. Cutaneous lesions and finger clubbing uncovering hypocomplementemic urticarial vasculitis and hepatitis C with mixed cryoglobulinemia.

    PubMed

    Pinto-Almeida, Teresa; Caetano, Mónica; Alves, Rosário; Selores, Manuela

    2013-01-01

    Urticarial vasculitis is a rare clinicopathologic entity characterized by urticarial lesions that persist for more than 24 hours and histologic features of leukocytoclastic vasculitis. Patients can be divided into normocomplementemic or hypocomplementemic. The authors report the case of a healthy 49-year-old woman with a 1-year history of highly pruritic generalized cutaneous lesions and finger clubbing. Laboratory tests together with histopathologic examination allowed the diagnosis of hypocomplementemic urticarial vasculitis, chronic hepatitis C and type II mixed cryoglobulinemia. The patient started symptomatic treatment and was referred to a gastroenterologist for management of the hepatitis C, with progressive improvement of the skin condition. The development of hypocomplementemic urticarial vasculitis in the context of chronic hepatitis C is exceedingly rare and possible pathogenic mechanisms are discussed. PMID:24474109

  6. Hypocomplementemic Urticarial Vasculitis Syndrome in an 8-year-old Boy: A Case Report and Review of Literature.

    PubMed

    Al Mosawi, Zakiya Saleh Adnan; Al Hermi, Badriya Ebrahim Ahmed

    2013-07-01

    Hypocomplementemic urticarial vasculitis syndrome is an immune complex-mediated disease of unknown etiology. The clinical course is characterized by urticaria, conjunctivitis, joint pain, and hypocomplementemia. We here report a case of a child with hypocomplementemic urticarial vasculitis syndrome that progressed to nephritis. Renal biopsy was consistent with diffuse proliferative glomerulonephritis with diffuse subendothelial immune deposits. He responded well to a combination of steroid and mofetil micofenolate. PMID:23904922

  7. Inflammatory myositis complicating hypocomplementemic urticarial vasculitis despite on-going immunosuppression.

    PubMed

    Chew, Gary Y J; Gatenby, Paul A

    2007-08-01

    We present a patient with previously diagnosed hypocomplementemic urticarial vasculitis syndrome, with skin, lung, and renal involvement, who presented with congestive cardiac failure. During the course of her hospitalization, she was also found to have profound proximal muscle weakness in both upper and lower limbs associated with raised creatinine kinase levels. A muscle biopsy was performed, which demonstrated evidence of an inflammatory myositis with vasculitis, which had returned despite on-going immunosuppression. This occurrence of a new autoimmune disease may well be an example of the "waste disposal" hypothesis. PMID:16944070

  8. A case of precocious emphysema and lung cancer in a woman with a history of hypocomplementemic urticarial vasculitis.

    PubMed

    Jamison, Suzanna C; Brierre, Stephen; Sweet, Jon; de Boisblanc, Ben

    2008-03-01

    Severe emphysema developed in a white woman with a 26-pack-year history of tobacco use. Serum alpha(1)-antitrypsin levels were normal. A history of autoimmune hemolytic anemia, angioedema, low complement, and recurrent urticaria prompted an immunologic workup that ultimately led to a diagnosis of hypocomplementemic urticarial vasculitis syndrome. Treatment with oral prednisone and inhaled bronchodilators improved symptoms, but 4 months after diagnosis non-small cell lung cancer was discovered and she ultimately died. Hypocomplementemic urticarial vasculitis is an uncommon cause of precocious emphysema and has not previously been reported in a patient with bronchogenic carcinoma. PMID:18321906

  9. Urticarial vasculitis.

    PubMed

    Venzor, Joe; Lee, Wai L; Huston, David P

    2002-10-01

    Urticarial vasculitis is a clinico-pathologic entity typified by recurrent episodes of urticaria that have the histopathologic features of leukocytoclastic vasculitis. The cutaneous features may include painful, burning or pruritic skin lesions, the persistence of individual lesions greater than 24 hours, palpable purpura, pronounced central clearing of lesions, and residual hyperpigmentation following resolution. However, because clinical characteristics of urticarial vasculitis may overlap with those of allergic urticaria, confirmation of the diagnosis requires a lesional skin biopsy. This condition is idiopathic in many patients but can also occur in the context of autoimmune disorders, infections, drug reactions, or as a paraneoplastic syndrome. In idiopathic urticarial vasculitis common laboratory findings are an elevation of erythrocyte sedimentation rate and reduction of serum complement. An association between urticarial vasculitis and systemic lupus erythematosus has been hypothesized as some clinical manifestations of disease overlap and C1q autoantibodies may be present in both diseases. Normo-complementemic patients usually have minimal or no systemic involvement and often have a better prognosis. On-the-other-hand, hypocomplementemic patients have the propensity to have more severe multi-organ involvement. Response to treatment is variable and a wide variety of therapeutic agents may be efficacious. Initial recommendations for treatment of urticarial vasculitis manifest only as non-necrotizing skin lesions include antihistamines, dapsone, colchicine, hydroxychloroquine or indomethacin, but corticosteroids are often required. With necrotizing skin lesions or visceral involvement, corticosteroids are regularly indicated. Cases of severe corticosteroid resistant urticarial vasculitis or where corticosteroid morbidity is evident [table: see text] may require treatment with other immunosuppressive agents such as azathioprine, cyclophosphamide, or

  10. Angioedema in a 47-year-old woman with hypocomplementemic urticarial vasculitis syndrome.

    PubMed

    Jones, Julie M; Reich, Keith A; Raval, Deena G

    2012-02-01

    Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a type III hypersensitivity reaction characterized by urticaria with persistent acquired hypocomplementemia. Although HUVS is uncommon, it is important for physicians to be familiar with this disease, as the initial presentation is often life-threatening angioedema. The author reports the case of a 47-year-old white woman with a history of HUVS. She presented to an outpatient clinic complaining of a rash and difficulty swallowing. Urticaria and angioedema were diagnosed. The patient was given epinephrine in the clinic and then transferred to the hospital. Laboratory testing confirmed urticaria, and the patient was given intravenous methylprednisolone sodium succinate and an additional dose of epinephrine. After 1 week, the patient's angioedema improved. PMID:22331803

  11. EBNA1 expression in a lung transplant recipient with hypocomplementemic urticarial vasculitis syndrome.

    PubMed

    Berggren, Malin A M; Heinlen, Latisha; Isaksson, Asa; Nyström, Ulla; Ricksten, Anne

    2007-07-01

    This article describes a transplant recipient with underlying hypocomplementemic urticarial vasculitis syndrome who expressed persistently Epstein-Barr virus nuclear antigen 1 (EBNA1) in peripheral blood. The patient received a bilateral lung transplant and was subsequently followed with monitoring of EBV expression in peripheral blood. Evaluation of viral expression in peripheral blood, serum, and graft tissue was performed with RT-PCR, Q-PCR, indirect immunofluorescence, anti-peptide assays, and in situ hybridization; samples were collected at various time-points up to 91 days post-transplantation. The patient expressed EBNA1 in 8/10 (80%) of the peripheral blood samples tested during the post-transplantation period, and interestingly, even including the day of transplantation. After analyses of indicative EBV mRNA, EBNA1 expression was found mainly to be Qp-initiated EBNA1, known to be important for EBV maintenance. Anti-EBNA1 epitope mapping showed significantly higher and broader antibody responses to EBNA1 epitopes pre-transplantation when compared to normal controls and a matched lung transplant control. Post-transplantation this response was largely diminished but there were still epitopes significantly higher than controls. Our results show the presence of EBV-positive proliferating cells before onset of intensive immunosuppressive treatment. Although no previous connection between EBV and hypocomplementemic urticarial vasculitis syndrome has been reported, it is tempting to speculate that the continuous EBNA1 expression is not caused by immunosuppression or post-transplant lymphoproliferative disease, but may be a factor involved in the etiology of the autoimmune disease. PMID:17516536

  12. Serum IgG antibodies to C1q in hypocomplementemic urticarial vasculitis syndrome.

    PubMed

    Wisnieski, J J; Naff, G B

    1989-09-01

    Urticaria, angioedema, and arthritis are cardinal features of hypocomplementemic urticarial vasculitis syndrome (HUVS). Considered to be an immune complex-mediated disorder, HUVS has been differentiated from systemic lupus erythematosus (SLE), based on its clinical manifestations and the C1q precipitin (C1q-p) reaction, which is manifested as gel precipitation of C1q by a small percentage of HUVS IgG molecules. This phenomenon has been attributed to an Fc region abnormality, and the responsible IgG molecules are said to possess C1q-p activity. We purified IgG from 4 HUVS patients and confirmed that HUVS IgG contains C1q binding activity. F(ab')2 fragments from these patients also bound to C1q, as measured by 2 different C1q binding methods at physiologic ionic strength; HUVS IgG Fc fragments did not bind to C1q. Preincubation of HUVS F(ab')2 fragments with antibody to human F(ab')2 prevented subsequent binding to C1q. We conclude that IgG antibodies to C1q are present in HUVS serum, and it is likely that these antibodies are C1q-p. Because the clinical manifestations of HUVS and the presence of anti-C1q antibodies have been described in patients with SLE, our findings support the concept that HUVS is an autoimmune syndrome related to SLE. PMID:2528353

  13. Comparison of autoantibodies to the collagen-like region of C1q in hypocomplementemic urticarial vasculitis syndrome and systemic lupus erythematosus.

    PubMed

    Wisnieski, J J; Jones, S M

    1992-03-01

    Hypocomplementemic urticarial vasculitis syndrome (HUVS) is an apparent autoimmune disorder that resembles SLE. We previously showed that C1q precipitins in HUVS sera are IgG autoantibody to human C1q. We have compared HUVS anti-C1q autoantibody to a similar autoantibody in the serum of some patients with SLE. As with anti-C1q autoantibody in SLE sera, the HUVS autoantibody binds only to the collagen-like region (CLR) of C1q. In both HUVS and SLE, IgG2 is the predominant subclass of IgG autoantibody and IgM autoantibody to C1q is uncommon. In both diseases, anti-C1q autoantibodies bind preferentially to surface-adsorbed C1q or CLR fragments compared to these antigens in solution. Finally, when HUVS or SLE autoantibodies were added to CLR-coated wells already bound, respectively, by SLE or HUVS autoantibodies, no increases in CLR binding were observed, suggesting that HUVS and SLE autoantibodies to C1q bind to the same CLR epitope(s). PMID:1538123

  14. Telmisartan induced urticarial vasculitis.

    PubMed

    Mahajan, Vikram K; Singh, Ravinder; Gupta, Mrinal; Raina, Rashmi

    2015-01-01

    A 53-year-old man developed urticarial vasculitis following ingestion of telmisartan and hydrochlorothiazide combination for hypertension. Treatment with prednisolone and cetirizine was curative, but his lesions recurred when he continued telmisartan and hydrochlorothiazide against medical advice. Re-challenge with the same doses of telmisartan precipitated similar lesions with telmisartan and not with hydrochlorothiazide. This uncommon cutaneous adverse reaction of angiotensin II receptor blockers has implication for the clinicians as more such cases may become apparent with their wider use than in premarketing studies. PMID:26600649

  15. Urticarial vasculitis and Schnitzler syndrome.

    PubMed

    Zuberbier, Torsten; Zuberbier, H C Torsten; Maurer, Marcus

    2014-02-01

    Both urticarial vasculitis and Schnitzler syndrome are serious diseases but a recently gained better understanding of their underlying pathomechanism allows better treatment options than only a decade ago. PMID:24262694

  16. Urticarial vasculitis and urticarial autoinflammatory syndromes.

    PubMed

    Marzano, A V; Tavecchio, S; Venturini, M; Sala, R; Calzavara-Pinton, P; Gattorno, M

    2015-02-01

    Urticaria is a frequent disorder classified as acute and chronic forms, which presents with wheals that can be associated with angioedema. Several entities may manifest with urticarial skin lesions, encompassing a heterogeneous group of conditions that have to be differentiated from ordinary urticaria. This review is focused on two of these urticarial syndromes: urticarial vasculitis (UV), which represents the most important differential diagnosis with common urticaria, and autoinflammatory diseases such as cryopyrin-associated periodic syndromes (CAPS) and Schnitzler's Syndrome, both rare multisystem forms that may masquerade as common urticaria. UV is a small-vessel vasculitis with predominant skin involvement, characterized by wheals persisting for more than 24 hours, burning rather than itching and resolving with hyperpigmentation as well as by other cutaneous manifestations including purpura, papules, vesicles, bullae and necrotic-ulcerative lesions. Histology shows a classic pattern of leukocytoclastic vasculitis, with possible presence of upper dermal edema. CAPS are classified as three distinct entities: familial cold autoinflammatory syndrome, Muckle-Wells Syndrome and chronic infantile neurological cutaneous and articular syndrome, which represent a spectrum of disorders caused by different mutations in a single gene, NLRP3 (NOD-like receptor 3). This gene encodes for cryopyrin, an inflammasome protein that activates interleukin-1β, leading to an overproduction of this pivotal proinflammatory cytokine. Histologically, urticarial lesions are generally characterized by a perivascular neutrophilic infiltrate. Unlike urticaria, neither UV nor urticarial autoinflammatory syndromes do respond to antihistamines: thus, it is important not to misdiagnose such conditions in order to give the patients specific treatments, potentially preventing serious systemic complications. PMID:25586657

  17. Urticarial vasculitis reveals unsuspected thyroiditis.

    PubMed

    Ferreira, Olga; Mota, Alberto; Baudrier, Teresa; Azevedo, Filomena

    2012-01-01

    A 38-year-old woman presented with erythematous, violaceous plaques with a serpiginous and unusual appearance located on the left shoulder, left thigh, and right buttock, evolving for 5 days, which eventually became generalized. A skin biopsy revealed leukocytoclastic vasculitis and a diagnosis of urticarial vasculitis was made. The complete blood count, biochemistry, complement levels, and other immunological test results were unremarkable. However, antithyroid antibody titers were increased. Despite having normal thyroid function tests and an absence of specific symptoms, the patient underwent a thyroid ultrasound, which revealed features of thyroiditis, and was subsequently referred to an endocrinologist. Several diseases can be associated with urticarial vasculitis, namely infections and autoimmune connective-tissue disorders such as systemic lupus erythematosus and Sjögren syndrome. Thyroiditis is an uncommon association. PMID:23000939

  18. Urticarial Vasculitis-Associated Intestinal Ischemia

    PubMed Central

    Wong, Uni; Yfantis, Harris; Xie, Guofeng

    2016-01-01

    Urticarial vasculitis (UV) is a rare small vessel vasculitis. UV is often idiopathic but can also present in the context of autoimmune disorders such as systemic lupus erythematosus, drug reactions, infections, or a paraneoplastic syndrome. Extracutaneous complications include intestinal ischemic injuries, in UV patients with nonspecific gastrointestinal symptoms such as abdominal pain and nausea. Prompt recognition and treatment can minimize morbidity and mortality. This paper describes a case of urticarial vasculitis-associated intestinal ischemia. PMID:27190661

  19. Urticarial Vasculitis-Associated Intestinal Ischemia.

    PubMed

    Wong, Uni; Yfantis, Harris; Xie, Guofeng

    2016-01-01

    Urticarial vasculitis (UV) is a rare small vessel vasculitis. UV is often idiopathic but can also present in the context of autoimmune disorders such as systemic lupus erythematosus, drug reactions, infections, or a paraneoplastic syndrome. Extracutaneous complications include intestinal ischemic injuries, in UV patients with nonspecific gastrointestinal symptoms such as abdominal pain and nausea. Prompt recognition and treatment can minimize morbidity and mortality. This paper describes a case of urticarial vasculitis-associated intestinal ischemia. PMID:27190661

  20. Autoimmunity in chronic urticaria and urticarial vasculitis.

    PubMed

    Napoli, D C; Freeman, T M

    2001-07-01

    In contrast to acute urticaria, etiology cannot be identified in most cases of chronic urticaria. Recent evidence suggests that a subset of patients with chronic urticaria may have an autoimmune basis for their condition. The demonstration of antithyroid autoantibodies in some patients with chronic idiopathic urticaria (CIU) provides support for an association. However, the discovery of a positive skin test response to intradermal injection of autologous serum in as many as 60% of patients with CIU led to the identification of autoantibodies to IgE and the alpha-chain of the high-affinity IgE receptor, Fc epsilon RI alpha. Additional studies have demonstrated that some of these autoantibodies are capable of releasing histamine from donor basophils and mast cells. This article reviews the literature that addresses a possible autoimmune etiology in a subset of patients with CIU. Urticarial vasculitis is differentiated from chronic urticaria based on clinical features and biopsy findings of leukocytoclastic vasculitis. Most cases of urticarial vasculitis are secondary to an underlying systemic disease. The presence of autoantibodies has also been demonstrated in a subset of patients with primary urticarial vasculitis. This article briefly reviews some of this data. PMID:11892055

  1. Hypocomplementaemic urticarial vasculitis syndrome: a mimicker of systemic lupus erythematosus.

    PubMed

    Roy, Krishnendu; Talukdar, Arunansu; Kumar, Bappaditya; Sarkar, Sumanta

    2013-01-01

    A middle aged female patient presented with generalised palpable purpura associated with intense pruritus along with subconjunctival haemorrhage and orbital inflammation. There was extensive dermographism. Other systemic examinations were within normal limits. Haematological profile was normal except raised D-dimer. Skin biopsy revealed the presence of leucocytoclastic vasculitis. Antinuclear antibody was positive in a titre of 1 : 160, but antidouble-stranded DNA was negative. Urine examination revealed haematuria and proteinuria. Complement C3, C4 and C1q levels were decreased with the presence of anti-C1q antibody. There was a diagnostic dilemma between systemic lupus erythematosus and hypocomplementaemic urticarial vasculitis syndrome. However, as the patient did not fulfil the American College of Rheumatology criteria for systemic lupus erythematosus, but fulfilled all the criteria for hypocomplementaemic urticarial vasculitis syndrome, the case was finally diagnosed as hypocomplementaemic urticarial vasculitis syndrome and treated accordingly with favourable outcome. PMID:23704433

  2. Urticarial Reactions: Vascular Erythema, Urticaria, Vasculitis

    PubMed Central

    Sibbald, R. Gary

    1987-01-01

    Vascular erythemas, including urticaria and vasculitis, represent diagnostic and therapeutic challenges. A careful systemic approach to history and physical examination should be followed by appropriate investigations to rule out systemic disease. Chronic urticaria patients should be physically tested to identify cholinergic, dermagraphic, and cold-induced responses. Food diaries and careful drug history may be important to identify exacerbating factors in chronic urticaria. A skin biopsy is necessary to diagnose vasculitis. Therapy of any underlying cause is supplemented with H1 antihistamines in urticaria patients, while vasculitis requires a different anti-inflammatory approach. PMID:21263956

  3. Urticarial vasculitis in northern Spain: clinical study of 21 cases.

    PubMed

    Loricera, Javier; Calvo-Río, Vanesa; Mata, Cristina; Ortiz-Sanjuán, Francisco; González-López, Marcos A; Alvarez, Lino; González-Vela, M Carmen; Armesto, Susana; Fernández-Llaca, Héctor; Rueda-Gotor, Javier; González-Gay, Miguel A; Blanco, Ricardo

    2014-01-01

    Urticarial vasculitis (UV) is a subset of cutaneous vasculitis (CV), characterized clinically by urticarial skin lesions of more than 24 hours' duration and histologically by leukocytoclastic vasculitis. We assessed the frequency, clinical features, treatment, and outcome of a series of patients with UV. We conducted a retrospective study of patients with UV included in a large series of unselected patients with CV from a university hospital. Of 766 patients with CV, UV was diagnosed in 21 (2.7%; 9 male and 12 female patients; median age, 35 yr; range, 1-78 yr; interquartile range, 5-54 yr). Eight of the 21 cases were aged younger than 20 years old. Potential precipitating factors were upper respiratory tract infections and drugs (penicillin) (n = 4; in all cases in patients aged <20 yr), human immunodeficiency virus (HIV) infection (n = 1), and malignancy (n = 1). Besides urticarial lesions, other features such as palpable purpura (n = 7), arthralgia and/or arthritis (n = 13), abdominal pain (n = 2), nephropathy (n = 2), and peripheral neuropathy (n = 1) were observed. Hypocomplementemia (low C4) with low C1q was disclosed in 2 patients. Other abnormal laboratory findings were leukocytosis (n = 7), increased erythrocyte sedimentation rate (n = 6), anemia (n = 4), and antinuclear antibody positivity (n = 2). Treatment included corticosteroids (n = 12), antihistaminic drugs (n = 6), chloroquine (n = 4), nonsteroidal antiinflammatory drugs (n = 3), colchicine (n = 2), and azathioprine (n = 1). After a median follow-up of 10 months (interquartile range, 2-38 mo) recurrences were observed in 4 patients. Apart from 1 patient who died because of an underlying malignancy, the outcome was good with full recovery in the remaining patients. In conclusion, our results indicate that UV is rare but not exceptional. In children UV is often preceded by an upper respiratory tract infection. Urticarial lesions and joint manifestations are the most frequent clinical manifestation

  4. A case of hypocomplementaemic urticarial vasculitis with a high serum level of rheumatoid factor.

    PubMed

    Ashida, Atsuko; Murata, Hiroshi; Ohashi, Atsuko; Ogawa, Eisaku; Uhara, Hisashi; Okuyama, Ryuhei

    2013-08-01

    We report a case of hypocomplementaemic urticarial vasculitis with an elevated serum rheumatoid factor level. Hypocomplementaemic urticarial vasculitis is an immune complex-mediated disease characterised by urticarial eruptions. High levels of rheumatoid factor may be associated with hypocomplementaemia due to the consumption of complement, because the rheumatoid factor can form immune complexes with immunoglobulin. It is necessary to pay attention to the amounts of complement in cases of urticarial eruptions with elevated rheumatoid factor level. The eruptions were relieved with a combination of prednisolone and colchicine. PMID:23905981

  5. Omalizumab for Urticarial Vasculitis: Case Report and Review of the Literature.

    PubMed

    Ghazanfar, Misbah Nasheela; Thomsen, Simon Francis

    2015-01-01

    Urticarial vasculitis is characterised by inflamed itching or burning red patches or wheals that resemble urticaria but persist for greater than 24 hours. It is often idiopathic but is sometimes associated with collagen-vascular disease, particularly systemic lupus erythematosus. Treatment options include oral antihistamines, oral corticosteroids, dapsone, colchicine or hydroxychloroquine. We describe a male patient with urticarial vasculitis who was treated with omalizumab (anti-IgE) with convincing results and provide a review of previous reports of patients with urticarial vasculitis treated with omalizumab. PMID:26435858

  6. Urticarial vasculitis: an autoimmune disorder following therapy for Hodgkin's disease.

    PubMed

    Strickland, D K; Ware, R E

    1995-09-01

    Immunological abnormalities have been described in patients with Hodgkin's disease, both associated with the malignancy itself and occurring secondary to therapy. These abnormalities often manifest as an immunodeficiency state, but can also present as immune dysregulation and autoimmune disease. We report two young patients with Hodgkin's disease who, following successful therapy, developed urticarial vasculitis (UV), a form of cutaneous autoimmune vasculitis. Both patients also had systemic symptoms including fever, an elevated erythrocyte sedimentation rate and serum copper, and abnormal in vitro studies of lymphocyte enumeration and proliferation. Distinguishing UV from recurrent Hodgkin's disease was especially difficult in one patient, and was possible only by lymph node biopsy. One patient has responded well to immunosuppressive therapy, while the other, who has more profound immune dysfunction, has developed a chronic autoimmune disorder. UV may thus occur in patients after therapy for Hodgkin's disease; we hypothesize that immune dysregulation, either associated with the malignancy or resulting from therapy, is important in the pathogenesis of this autoimmune process. PMID:7623731

  7. Florid urticarial vasculitis heralding a flare up of ulcerative colitis.

    PubMed

    Boules, Evon; Lyon, Calum

    2014-01-01

    A 75-year-old man with ulcerative colitis (UC) and diet controlled diabetes mellitus presented with a 3-week history of slightly itchy, red plaques on both lower limbs ascending gradually to cover the trunk and arms. One week later, he developed a flare up of his UC. Routine blood tests showed modest drop in haemoglobin (122 g/L) and C reactive protein (85 mg/L). Serology was remarkable for high antiproteinase 3 (c-ANCA). Serum electrophoresis showed a mildly positive paraprotein band (γ region). Stool culture was negative. Urine analysis showed proteinuria. Skin biopsy showed features of urticarial vasculitis (UV). He underwent a flexible sigmoidoscopy after the flare up showed mildly active UC. The patient was given hydrocortisone for 7 days and then prednisolone. Both rash and UC subsided. Electrophoresis was repeated 4 weeks later showing normal pattern. Prednisolone has been gradually reduced. Although rare, UV can be considered as one of the skin manifestations of UC. PMID:25535230

  8. Impact of Vasculitis on Employment and Income

    ClinicalTrials.gov

    2016-01-26

    Vasculitis; Systemic Vasculitis; Behcet's Disease; CNS Vasculitis; Cryoglobulinemic Vasculitis; Eosinophilic Granulomatosis; Temporal Arteritis; Wegener Granulomatosis; Henoch-Schoenlein Purpura; Microscopic Polyangiitis; Polyarteritis Nodosa (PAN); Takayasu's Arteritis; Urticarial Vasculitis

  9. A 14-year paraneoplastic rash: urticarial vasculitis and dermal binding bullous pemphigoid secondary to chronic lymphocytic leukaemia.

    PubMed

    Kassim, J M; Igali, L; Levell, N J

    2015-06-01

    A 72-year-old woman with a 14-year history of urticarial vasculitis (UV) and a 13-year history of bullous pemphigoid (BP) presented with associated progressive chronic lymphocytic leukaemia (CLL). Both skin conditions responded poorly to treatment, until chemotherapy for CLL was commenced. The skin features showed a clear paraneoplastic course, resolving with chemotherapy and recurring when the CLL relapsed and the lymphocyte count rose above 5 × 10(9)/L. No case of UV secondary to CLL, and very few cases of BP related to CLL have been reported, and no paraneoplastic rash of any type lasting 14 years has been reported previously. PMID:25524180

  10. Allergic vasculitis

    MedlinePlus

    ... Cutaneous vasculitis Images Vasculitis on the palm Vasculitis Vasculitis, urticarial on the hand References Stone JH. The systemic vasculitides. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine . 25th ed. ... small vessel vasculitis. In: Firestein GS, Budd RC, Gabriel SE, McInnes ...

  11. Cutaneous adverse effects of hormonal adjuvant therapy for breast cancer: a case of localised urticarial vasculitis following anastrozole therapy and a review of the literature.

    PubMed

    Bock, Vanessa L; Friedlander, Michael; Waring, Dale; Kossard, Steven; Wood, Glenda K

    2014-11-01

    Hormonal therapy with either tamoxifen or aromatase inhibitors is commonly used to treat women with breast cancer in both the adjuvant and recurrent disease setting. Cutaneous adverse reactions to these drugs have been rarely reported in the literature. We report an unusual case of urticarial vasculitis following the aromatase inhibitor anastrozole that localised to the unilateral trunk and mastectomy scar, and review the literature on the cutaneous adverse effects of hormonal therapy for breast cancer. PMID:24575835

  12. Successful use of interleukin 6 antagonist tocilizumab in a patient with refractory cutaneous lupus and urticarial vasculitis.

    PubMed

    Makol, Ashima; Gibson, Lawrence E; Michet, Clement J

    2012-03-01

    Tocilizumab (Actemra; Genentech, Inc) is the first biologic therapy targeting the cytokine interleukin 6 (IL-6). It is a humanized monoclonal immunoglobulin G1 antibody against the α-chain of the IL-6 receptor that prevents the binding of IL-6 to membrane-bound and -soluble IL-6 receptor. It was approved by the US Food and Drug Administration in January 2010 for rheumatoid arthritis refractory to other approved therapies and in April 2011 for systemic juvenile idiopathic arthritis. It has been used as an off-label treatment in many autoimmune diseases, where IL-6 plays a major role in pathogenesis. We report a case of refractory systemic lupus erythematosus in a 22-year-old woman with recurrent high-grade fever, polyarthritis, diffuse rash with urticarial vasculitis, and tumid lupus who did not respond to topical corticosteroids, photoprotection, antimalarials, methotrexate, anakinra, mycophenolate mofetil, etanercept, and intravenous immunoglobulin therapy. Symptoms recurred after corticosteroid tapers below 10 mg. She was noted to have an elevated IL-6 level, and tocilizumab was started. She responded favorably with remission of fever, arthritis, and skin manifestations and was able to taper corticosteroid therapy successfully. PMID:22334272

  13. Vasculitis Pregnancy Registry

    ClinicalTrials.gov

    2016-09-08

    Vasculitis; Behcet's Disease; CNS Vasculitis; Cryoglobulinemic Vasculitis; Eosinophilic Granulomatosis With Polyangiitis (EGPA); Churg-Strauss Syndrome (CSS); Granulomatosis With Polyangiitis (GPA); Wegener's Granulomatosis; IgA Vasculitis; Henoch-Schoenlein Purpura (HSP); Microscopic Polyangiitis (MPA); Polyarteritis Nodosa (PAN); Takayasu Arteritis (TAK); Urticarial Vasculitis; Systemic Vasculitis

  14. Tumor necrosis factor-like weak inducer of apoptosis and its receptor fibroblast growth factor-inducible 14 are expressed in urticarial vasculitis.

    PubMed

    Li, Mengmeng; Chen, Tao; Guo, Zaipei; Li, Jingyi; Cao, Na

    2013-11-01

    Tumor necrosis factor (TNF)-like weak inducer of apoptosis (TWEAK), a member of the TNF family, has been implicated as a pro-inflammatory cytokine in many types of autoimmune and infectious diseases. However, information about TWEAK in dermatological diseases is limited. To date, no studies have investigated the roles of TWEAK in patients with urticarial vasculitis (UV). This study aimed to assess serum TWEAK levels, together with TWEAK and fibroblast growth factor-inducible 14 (Fn14) expressions of skin lesions in patients with UV. Serum TWEAK levels in patients with UV, together with patients with cutaneous leukocytoclastic angiitis (CLA) and healthy controls were detected by enzyme-linked immunosorbent assay; TWEAK and Fn14 expressions of skin lesions were analyzed by immunohistochemistry. Results showed that TWEAK and Fn14 were abundantly expressed in the dermal vessel wall of lesional skin in patients with UV but not healthy controls. Serum TWEAK levels in the acute stage in patients with UV were significantly higher than those in the convalescent stage and healthy controls. Serum TWEAK levels were elevated significantly in patients with CLA compared with those in healthy controls. Our previous study indicated that TWEAK may be an important mediator for the development of vascular inflammation in skin. In addition, we also found that TWEAK blockade substantially reduced vascular damage and perivascular leukocyte infiltrates in lipopolysaccharide-induced cutaneous vasculitis. Our study shows that TWEAK may be associated with the pathogenesis of UV; it is therefore suggested that TWEAK may be a potential therapeutic target for UV and other types of cutaneous vasculitis. PMID:23968277

  15. [Neurophilic urticarial dermatosis].

    PubMed

    Ehrchen, J

    2016-05-01

    Neurophilic urticarial dermatosis (NUD) is a new, important differential diagnosis of urticarial rashes. It is a clinical and histological response pattern that strongly correlates with systemic disease. Both autoinflammatory and autoimmune conditions can be present in patients with NUD. In this article the clinical and histological criteria of NUD, diagnostic considerations and the diseases most frequently associated with NUD are discussed. PMID:27119369

  16. Vasculitis Foundation

    MedlinePlus

    ... to view full calendar Vasculitis Patient-Powered Research Network The V-PPRN seeks to transform how clinical ... patients with various forms of vasculitis. Join The Network! Latest News Brandon Qualifies for the 1500m Semi- ...

  17. Rheumatoid Vasculitis

    MedlinePlus

    ... RV) is an unusual complication of longstanding, severe rheumatoid arthritis. The active vasculitis associated with rheumatoid disease occurs ... a manifestation of “extra-articular” (beyond the joint)rheumatoid arthritis and involves the small and medium-sized arteries ...

  18. Necrotizing vasculitis

    MedlinePlus

    ... ncbi.nlm.nih.gov/pubmed/23045170 . Stone JH. Classification and epidemiology of systemic vasculitis. In: Firestein GS, ... MedlinePlus Connect for EHRs For Developers U.S. National Library of Medicine 8600 Rockville Pike, Bethesda, MD 20894 ...

  19. [Cryoglobulinemic vasculitis].

    PubMed

    Blank, N; Lorenz, H-M

    2016-04-01

    Cryoglobulinemic vasculitis is a disease of the small arteries and affects the skin, joints, peripheral nerves, kidneys and other organs. Even small amounts of cryoglobulins can lead to severe impairment for the patient but the detection of such low amounts of cryoglobulins can be difficult. The causes of cryoglobulinemic vasculitis include monoclonal hematological diseases, autoimmune diseases and chronic infections. Therapy involves treatment of the underlying disease and glucocorticoids, rituximab and plasmapheresis depending on the severity of the vasculitis. Recognition of typical clinical symptoms is essential in order to initiate appropriate laboratory diagnostic procedures. Sometimes many investigations are necessary. In the absence of clinical trials treatment recommendations can often only be derived from registry data. PMID:27034078

  20. Pediatric Vasculitis.

    PubMed

    Singh-Grewal, Davinder; Durkan, Anne Maria

    2016-02-01

    Vasculitis is defined as inflammation of the blood vessels and can result in stenosis or aneurysm, which may in turn lead to occlusion or rupture of the vessel compromising tissue perfusion. The manifestations of these diseases depend on the size and site of the vessels effected. Vasculitis can be secondary to numerous inflammatory and infectious diseases but this review will concentrate on the systemic primary vasculitides and aims to discuss the presentations and approaches to management of a number of these conditions. PMID:26365154

  1. Leukocytoclastic vasculitis in urticaria induced by ultraviolet irradiation.

    PubMed

    Armstrong, R B; Horan, D B; Silvers, D N

    1985-09-01

    A 35-year-old woman would develop urticarial lesions over one to four hours after irradiation with sunlight or artificial ultraviolet radiation. Lesions could persist for days before resolving. Although lesions were morphologically typical of solar urticaria, their evolution and resolution were both much slower than expected. Histologically, the lesions showed leukocytoclastic vasculitis. This unexpected finding suggests the possibility that there may be novel mechanisms involved in the pathogenesis of some cases of solar urticaria. PMID:4037839

  2. Burning vasculitis.

    PubMed

    Chadha, Priyanka; Hobday, Dorian; Fitzgerald O'Connor, Edmund; D'Cruz, David

    2016-01-01

    We present the case of a 69-year-old man who was found collapsed close to a heat source and admitted to hospital for severe sepsis. He was also found to have widespread blistering and ulceration of his right leg; however, a history was unobtainable due to reduced consciousness levels. The leg lesions had the initial appearance of mixed depth burns and a management plan was made to transfer the patient to a burns unit for debridement. It was subsequently noted that the patient had a previous diagnosis of seropositive erosive rheumatoid arthritis. A biopsy of the leg lesion was performed and a diagnosis of rheumatoid vasculitis confirmed. Treatment with systemic steroids, intravenous antibiotics and intravenous immunoglobulin therapy for severe hypogammaglobulinaemia was started, and the patient was not transferred for surgical debridement. Rheumatoid vasculitis is a rare and extremely serious complication of rheumatoid arthritis that can manifest in a number of ways, occasionally mimicking other conditions. This case is essential to raise awareness of rare, severe rheumatoid vasculitis and of the potential for its misdiagnosis as a mixed depth burn. PMID:27118745

  3. Pediatric Vasculitis Initiative

    ClinicalTrials.gov

    2016-08-19

    Wegeners Granulomatosis (Granulomatosis With Polyangiitis); Microscopic Polyangiitis; Churg Strauss Syndrome (Eosinophilic Granulomatosis With Polyangiitis); Polyarteritis Nodosa; Takayasu Arteritis; Primary CNS Vasculitis; Unclassified Vasculitis

  4. Cutaneous vasculitis in primary Sjögren syndrome: classification and clinical significance of 52 patients.

    PubMed

    Ramos-Casals, Manuel; Anaya, Juan-Manuel; García-Carrasco, Mario; Rosas, José; Bové, Albert; Claver, Gisela; Diaz, Luis-Aurelio; Herrero, Carmen; Font, Josep

    2004-03-01

    To analyze the different clinical and histologic types of cutaneous vasculitis in patients with primary Sjögren syndrome (SS), we investigated the clinical and immunologic characteristics of 558 consecutive patients with primary SS from our units and selected those with clinical evidence of cutaneous lesions, excluding drug reactions and xeroderma. All patients fulfilled 4 or more of the diagnostic criteria for SS proposed by the European Community Study Group in 1993. A total of 89 (16%) patients presented with cutaneous involvement (88 female patients and 1 male; mean age, 51.8 yr). The main cutaneous involvement was cutaneous vasculitis, present in 52 (58%) patients. There were 51 (98%) female patients and 1 (2%) male, with a mean age at diagnosis of cutaneous vasculitis of 51 years (range, 20-80 yr). Fourteen presented with cryoglobulinemic vasculitis, 11 with urticarial vasculitis, and the remaining 26, with cutaneous purpura not associated with cryoglobulins. A skin biopsy specimen was obtained in 38 patients (73%). Involvement of small-sized vessels was observed in 36 (95%) patients (leukocytoclastic vasculitis), while the remaining 2 (5%) presented with medium-sized vessel vasculitis (necrotizing vasculitis). Patients with cutaneous vasculitis had a higher prevalence of articular involvement (50% vs 29%, p = 0.044), peripheral neuropathy (31% vs 4%, p < 0.001), Raynaud phenomenon (40% vs 15%, p = 0.008), renal involvement (10% vs 0%, p = 0.028), antinuclear antibodies (88% vs 60%, p = 0.002), rheumatoid factor (78% vs 48%, p = 0.004), anti-Ro/SS-A antibodies (70% vs 43%, p = 0.011), and hospitalization (25% vs 4%, p = 0.005) compared with SS patients without vasculitis. Six (12%) patients died, all of whom had multisystemic cryoglobulinemia.In conclusion, cutaneous involvement was detected in 16% of patients with primary SS, with cutaneous vasculitis being the most frequent process. The main characteristics of SS-associated cutaneous vasculitis were the

  5. [Vasculitis in rheumatoid arthritis].

    PubMed

    Bély, M; Apáthy, A

    1996-07-21

    The frequency and histopathological characteristics of systemic vasculitis were studied in the autopsy material of 161 patients with rheumatoid arthritis. Systemic vasculitis was observed in 36 case (22.4%). In percentage of all cases with systemic vasculitis, the most frequently involved organs were the heart (66.7%), skeletal muscles (54.8%), and peripheral nerves (52%). The skin was involved only in about one third of 36 cases (36%). In most cases the arterioles and the small arteries were affected by vasculitis. Three types of vasculitis (non specific, fibrinoid necrotic, granulomatous) could be observed simultaneously in different vessels or combined in the same vessel. Different stages of inflammation could be found simultaneously, reflecting the relapsing nature of vasculitis. The frequency, the severity, and the recurrence of vasculitis are different aspects of the same phenomenon running usually parallel to each other in different organs and on different vessels. Vasculitis lead to local ischaemia and regressive changes depend on the number and size of the involved vessels. Systemic vasculitis led to death in 19 of 36 cases. Vasculitis was detected clinically in 7 of 36 cases. Exitus lethalis depend on the localization of the involved vessels. Vasculitis in the heart and brain are more life threatening, than that of localized to the skin. PMID:8757068

  6. [ANCA associated vasculitis].

    PubMed

    Ryba, Monika; Hruby, Zbigniew; Witkiewicz, Wojciech

    2013-01-01

    Vasculitis is a process caused by inflammation and necrosis of blood vessel walls and results in a variety of disorders. An accepted classification system for vasculitis is categorized by the size or type of the involved blood vessel as large-, medium-, or small-vessel vasculitis. Small-vessel vasculitis is defined as vasculitis that affects vessels smaller than arteries (i.e., arterioles, venules, and capillaries); however, small-vessel vasculitis can also involve medium-sized arteries. Granulomatosis with polyangiitis, Microscopoc polyangiitis, Churg Strauss syndrome and Renal Limited Vasculitis where the kidney is the only organ involved are clasified as a small vesselvasculitis. These disorders are described to be commonly associated with antineutrophil cytoplasm antibodies (ANCA). The etiology is not known and the incidence of vasculitis is incresasing occuring more often in elderly population. These diseases can cause the focal necrotizing lesions witch affect vessels and organs. In the lung it may cause alveolar hemorrhage, in the kidneys crescentic glomerulonephritis with acute renal failure, in the skin purpuric rash and ulcerations. Treatment usually includes corticosteroids, immunosupresive therapy and in some cases plasmapheresis. Advances in clinical management have been achieved during the past few years. PMID:24003670

  7. How Is Vasculitis Treated?

    MedlinePlus

    ... Clinical Trials Links Related Topics Aneurysm Blood Tests Kawasaki Disease Raynaud's Smoking and Your Heart Send a ... of vasculitis. For example, the standard treatment for Kawasaki disease is high-dose aspirin and immune globulin. ...

  8. Systemic necrotizing vasculitis.

    PubMed

    Gross, W L

    1997-05-01

    The revival of interest in systemic necrotizing vasculitis was initiated by the discovery of its association with anti-neutrophil cytoplasmic antibodies (ANCA). The close association of certain ANCA subspecificities, for example, proteinase 3 (Pr3) and myeloperxoidase ANCA, with Wegener's granulomatosis, microscopic polyangiitis and Churg-Strauss syndrome has led to their designation as 'ANCA-associated vasculitides'. This article describes the common and divergent clinical and immunological features of the members of this 'new' family of systemic necrotizing vasculitis, which continues to grow with the widespread use of ANCA testing. In addition, the 'standard' treatment for systemic necrotizing vasculitis (daily 'low dose' cyclophosphamide plus glucocorticosteroids or 'Fauci's scheme') is compared with new stage and activity adapted therapeutic regimens. PMID:9220078

  9. Rheumatoid vasculitis - Case report.

    PubMed

    Pecly, Inah Maria Drummond; Ocampo, Juan Felipe; Ramirez, Guillermo Pandales; de Oliveira, Hedi Marinho de Melo Guedes; Saud, Claudia Guerra Murad; Arantes, Milton Dos Reis

    2015-01-01

    Rheumatoid arthritis (RA) is a chronic systemic inflammatory autoimmune disease and its main manifestation is persistent synovitis affecting peripheral joints symmetrically, In spite of its destructive potential, the evolution of RA is highly variable. Some patients may have only a short-term process oligoarticular with minimum lesion, while others suffers a polyarthritis evolving with progressive and continuous involvement of other organ systems such as skin, heart, lungs, muscles and blood vessels rarely leading to rheumatoid vasculitis. The aim of this study was to describe a case of rheumatoid vasculitis a rare and severe condition. PMID:25772654

  10. How Is Vasculitis Diagnosed?

    MedlinePlus

    ... whether vasculitis is affecting your abdominal organs. Computed Tomography Scan A computed tomography (to-MOG-rah-fee) scan, or CT scan, ... that vein or artery. 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) identifies areas that show higher glucose ...

  11. Recurrent angioedema and urticaria.

    PubMed

    Bishop, P C; Wisnieski, J J; Christensen, J

    1993-11-01

    The case reported here illustrates the life-threatening aspects of angioedema and the need to thoroughly investigate the possible causes of this clinical finding. As discussed, the causes of angioedema are numerous. Commonly implicated in drug-induced angioedema are antihypertensive ACE inhibitor drugs, as was originally thought with this patient. Because of her skin lesions and macrocytic anemia, further studies were done. These studies led to a diagnosis of hypocomplementemic urticarial vasculitis syndrome, an uncommon to rare form of acquired angioedema, urticarial vasculitis, arthritis, and obstructive airway disease associated with the production of autoantibodies to C1q. It is an autoimmune disorder related to but separate from SLE. PMID:8279170

  12. Vasculitis in systemic lupus erythematosus.

    PubMed

    Drenkard, C; Villa, A R; Reyes, E; Abello, M; Alarcón-Segovia, D

    1997-01-01

    We studied the frequency, location, clinical and histopathological features, associated manifestations, and prognosis of vasculitides in a cohort of 667 SLE patients. Exclusion of patients with previous vasculitis or insufficient information left 540 patients, 194 of whom has vasculitis (incidence density: 0.053 new cases/person/year, cumulative incidence of 0.051 at one year, 0.232 at 5 years and 0.411 at 10 years). Vasculitis was confirmed by biopsy in 46 cases, by arteriography in five, and by both in three. A single episode of vasculitis occurred in 119 and two or more in 75 patients. Vasculitis was cutaneous in 160, visceral in 24, both in 10. In the first episode of cutaneous vasculitides, 111 had punctuate lesions, 32 palpable purpura, 6 urticaria, 6 ulcers, 8 papules, 5 erythematous plaques or macules confirmed with biopsy, 2 erythema with necrosis, and 1 panniculitis (plus small vessel vasculitis). Of 29 with visceral vasculitis in the first episode, 19 had mononeuritis multiplex, 5 digital necrosis, 3 large artery vasculitis of limbs, one mesenteric, and one coronary, more than one type could appear simultaneously or in subsequent episodes. Patients with vasculitis had longer disease duration and followup, younger age of onset of SLE, and were more frequently males than those without. Lupus manifestations associated with vasculitis in univariate logistic regression included myocarditis, psychosis, Raynaud's phenomenon, serositis, leukopenia, lymphopenia and pleuritis. Vasculitis also associated with the antiphospholipid syndrome. The strength of this association increased when patients with vasculitis confirmed by biopsy and/or arteriography were considered separately. Visceral vasculitis associated with increased mortality when controlled for age of onset and nephropathy. PMID:9104729

  13. [Systemic urticarias].

    PubMed

    Hachulla, E

    2003-05-01

    Systemic urticaria remains a challenge in terms of etiology, investigation and management. Most of cases are urticarial vasculitis consequence of inflammatory injury of capillaries and postcapillary venules in the skin. If hypocomplementemic urticarial vasculitis syndrome is a classical cause, the majority of patients have an underlying systemic disease like systemic lupus erythematosus, Sjögren's syndrome, mixed cryoglobulinemia, Still disease or cancer. Others systemic urticaria have been reported without clearly evidence of vasculitis like in primary or acquired angioedema, hereditary periodic fever syndromes and in some thyroiditis. Diagnosis needs a step to step procedure. Treatment depends the underlying disease. Some patients respond to nonsteroidal antiinflammatory drugs, some other need corticosteroids or immunosuppression. If urticarial vasculitis seems isolated in the absence of chronic obstructive pulmonary disease, antihistamines, nonsteroidal antiinflammatory drugs, colchicine, dapsone or hydroxychloroquine must be first used. PMID:12843810

  14. Dermal and bronchial hyperreactivity in urticarial dermographism and urticaria factitia.

    PubMed

    Henz, B M; Jeep, S; Ziegert, F S; Niemann, J; Kunkel, G

    1996-03-01

    For investigation of a possible relationship between cutaneous and bronchial hyperreactivity, 74 subjects were grouped according to the presence (n = 33) or absence (n = 41) of urticarial dermographism after application of a standardized shearing pressure with a dermographometer (12.7 x 10(5) Pa). the two groups did not differ in age, sex, smoking habits, presence of urticaria and atopy, or serum IgE levels. Erythema of the dermographic test sites was always significantly greater (P < 0.001) in the group with urticarial dermographism at 2, 4, and 8 min, and cutaneous reactivity with titrated prick tests was significantly increased in this group with low concentrations of histamine, 0.01% and substance P (0.25 mM) (P < 0.05). After bronchial provocation with acetylcholine, 51 of the 74 subjects, 25 with and 26 without urticarial dermographism, exhibited bronchial hyperreactivity. However, significantly more subjects with urticarial dermographism had an increase in airway resistance and a decrease in specific airway conductance (P < 0.05). In the subgroup (n = 9) of subjects with symptomatic urticarial dermographism (urticaria factitia), these differences were even more significant (P < 0.001). These subjects also had larger skin test reactions and significantly higher IgE levels (P < 0.01). Thus, the present data show an association, which may be based on common mechanisms of allergic inflammation, between cutaneous and bronchial hyperreactivity. PMID:8781671

  15. Differential Diagnosis of Retinal Vasculitis

    PubMed Central

    Abu El-Asrar, Ahmed M.; Herbort, Carl P.; Tabbara, Khalid F.

    2009-01-01

    Retinal vaculitis is a sight-threatening inflammatory eye condition that involves the retinal vessels. Detection of retinal vasculitis is made clinically, and confirmed with the help of fundus fluorescein angiography. Active vascular disease is characterized by exudates around retinal vessels resulting in white sheathing or cuffing of the affected vessels. In this review, a practical approach to the diagnosis of retinal vasculitis is discussed based on ophthalmoscopic and fundus fluorescein angiographic findings. PMID:20404987

  16. Increased serum levels of soluble vascular endothelial-cadherin in patients with systemic vasculitis.

    PubMed

    Chen, Tao; Guo, Zai-Pei; Cao, Na; Qin, Sha; Li, Meng-Meng; Jia, Rui-Zhen

    2014-08-01

    Henoch-Schönlein purpura (HSP) is a commonest systemic vasculitis (SV) in childhood characterized by an inflammatory reaction directed at vessels. Endothelial damage and perivascular leukocyte infiltrates are vital in the development of HSP. Vascular endothelial (VE)-cadherin is an endothelial cell-specific adhesion molecule, which plays critical roles in angiogenesis and endothelial integrity. Herein, we investigated the serum levels of soluble VE-cadherin (sVE-cadherin) in patients with HSP and other forms of SV. The serum levels of sVE-cadherin in 30 patients with HSP, together with patients with urticarial vasculitis, allergic vasculitis, Behcet disease, psoriasis vulgaris (PV) and atopic dermatitis (AD) and 26 health controls were measured by enzyme-linked immunosorbent assay. Serum levels of sVE-cadherin were significantly increased in patients with HSP in acute stage and patients with other forms of SV but not in patients with PV or AD. Moreover, Serum sVE-cadherin levels in HSP patients were correlated with the severity of this disease and serum concentrations of IgA anticardiolipin antibodies and vascular endothelial growth factor. Taken together, we show firstly that serum sVE-cadherin is abnormally increased in HSP patients. Increased serum levels of sVE-cadherin might be a novel biomarker for evaluating the severity of HSP and useful for identifying the presence of SV in inflammatory skin conditions. PMID:24469639

  17. Neutrophilic urticarial dermatosis: a variant of neutrophilic urticaria strongly associated with systemic disease. Report of 9 new cases and review of the literature.

    PubMed

    Kieffer, Carine; Cribier, Bernard; Lipsker, Dan

    2009-01-01

    We conducted the current study to define within the spectrum of the neutrophilic dermatoses a group of patients with an urticarial rash clinically and a neutrophilic dermatosis histopathologically. We reviewed the literature on neutrophilic urticaria and we report here a series of patients with this unique presentation. We reviewed all cutaneous biopsies submitted to our department between 2000 and 2006 in which histopathologic evaluation was compatible with this entity. We then retrieved the patient medical records and obtained information about follow-up and associated diseases. This allowed us to identify 9 patients with an urticarial eruption that was characterized histopathologically by a perivascular and interstitial neutrophilic infiltrate with intense leukocytoclasia but without vasculitis and without dermal edema. Four patients also had small foci of necrobiotic collagen bundles. The eruption consisted of pale, flat or only slightly raised, nonpruritic macules, papules, or plaques. Elementary lesions resolved within 24 hours. Purpura, angioedema, and facial swelling were not seen, but dermographism was present in 1 patient. Six patients had fever, 7 had polyarthritis, and 6 had leukocytosis. Seven patients had associated systemic diseases: adult-onset Still disease (3 patients), systemic lupus erythematosus (3 patients), and Schnitzler syndrome (1 patient).A similar rash has been reported previously in the literature, mostly in patients with systemic inflammatory diseases, but the majority of patients reported under the undefined designation of "neutrophilic urticaria" did have a different clinicopathologic presentation. Thus, we suggest naming this eruption "neutrophilic urticarial dermatosis," to emphasize that this entity expands the broad group of cutaneous manifestations of neutrophilic aseptic disease. This entity bears important medical significance as it is strongly indicative of an associated systemic disease, mainly Schnitzler syndrome, adult

  18. Urticarial dermographism: clinical features and response to psychosocial stress.

    PubMed

    Wallengren, Joanna; Isaksson, Anders

    2007-01-01

    Studies report that urticarial dermographism is exacerbated by "life events" and emotions. The aim of this study was to determine what aspects of life quality are affected by symptomatic dermographism and whether acute stress is a potential triggering factor. A total of 21 adult patients with urticarial dermographism completed a questionnaire on symptoms and quality of life. Twelve patients agreed to enrol in the study, which involved provocation by prick test and dermographism before and after a standardized psychosocial stress test (Trier Social Stress Test). Seventeen age-matched controls underwent corresponding tests. Of the patients answering the questionnaire, 43% reported that their disease had an impact on their quality of life and 33% that psychosocial stress precipitated the symptoms. However, the dermographic reaction in patients with urticaria factitia was not significantly intensified after the stress test. We conclude that the acute psychosocial stress test does not alter the magnitude of the dermographic reactions. PMID:17989886

  19. The ANCA Vasculitis Questionnaire (AAV-PRO©)

    ClinicalTrials.gov

    2016-05-10

    Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss) (EGPA); Churg-Strauss Syndrome (CSS); Granulomatosis With Polyangiitis (Wegener's) (GPA); Wegener Granulomatosis (WG); Microscopic Polyangiitis (MPA); ANCA-Associated Vasculitis (AAV); Vasculitis

  20. Cerebral vasculitis associated with cocaine abuse

    SciTech Connect

    Kaye, B.R.; Fainstat, M.

    1987-10-16

    A case of cerebral vasculitis in a previously healthy 22-year-old man with a history of cocaine abuse is described. Cerebral angiograms showed evidence of vasculitis. A search for possible causes other than cocaine produced no results. The authors include cocaine with methamphetamines, heroin, and ephedrine as illicit drugs that can cause cerebral vasculitis.

  1. Cutaneous small-vessel vasculitis.

    PubMed

    Lotti, T; Ghersetich, I; Comacchi, C; Jorizzo, J L

    1998-11-01

    Cutaneous small-vessel vasculitis (CSVV) refers to a group of disorders usually characterized by palpable purpura; it is caused by leukocytoclastic vasculitis of postcapillary venules. CSVV can be idiopathic or can be associated with a drug, infection, or underlying systemic disease. Initially, the pathogenesis of CSVV is immune complex related, but in its later stages different pathogenetic mechanisms may intensify the reaction and lymphocytes may predominate in the infiltrate. Cure requires elimination of the cause (ie, drugs, chemicals, infections, food allergens) when possible, as well as therapy with nonsteroidal antiinflammatory agents, corticosteroids, dapsone, potassium iodide, fibrinolytic agents, aminocaproic acid, immunosuppressive agents (ie, cyclophosphamide, azathioprine, methotrexate, cyclosporine) or even monoclonal antibodies, depending on disease severity. PMID:9810883

  2. Silica exposure and systemic vasculitis.

    PubMed Central

    Mulloy, Karen B

    2003-01-01

    Work in Department of Energy (DOE) facilities has exposed workers to multiple toxic agents leading to acute and chronic diseases. Many exposures were common to numerous work sites. Exposure to crystalline silica was primarily restricted to a few facilities. I present the case of a 63-year-old male who worked in DOE facilities for 30 years as a weapons testing technician. In addition to silica, other workplace exposures included beryllium, various solvents and heavy metals, depleted uranium, and ionizing radiation. In 1989 a painful macular skin lesion was biopsied and diagnosed as leukocytoclastic vasculitis. By 1992 he developed gross hematuria and dyspnea. Blood laboratory results revealed a serum creatinine concentration of 2.1 mg/dL, ethrythrocyte sedimentation rate of 61 mm/hr, negative cANCA (antineutrophil cytoplasmic antibody cytoplasmic pattern), positive pANCA (ANCA perinuclear pattern), and antiglomerular basement membrane negative. Renal biopsy showed proliferative (crescentric) and necrotizing glomerulonephritis. The patient's diagnoses included microscopic polyangiitis, systemic necrotizing vasculitis, leukocytoclastic vasculitis, and glomerulonephritis. Environmental triggers are thought to play a role in the development of an idiopathic expression of systemic autoimmune disease. Crystalline silica exposure has been linked to rheumatoid arthritis, scleroderma, systemic lupus erythematosus, rapidly progressive glomerulonephritis and some of the small vessel vasculitides. DOE workers are currently able to apply for compensation under the federal Energy Employees Occupational Illness Compensation Program (EEOICP). However, the only diseases covered by EEOICP are cancers related to radiation exposure, chronic beryllium disease, and chronic silicosis. PMID:14644669

  3. Medium-size-vessel vasculitis

    PubMed Central

    Eleftheriou, Despina; Brogan, Paul A.

    2009-01-01

    Medium-size-artery vasculitides do occur in childhood and manifest, in the main, as polyarteritis nodosa (PAN), cutaneous PAN and Kawasaki disease. Of these, PAN is the most serious, with high morbidity and not inconsequential mortality rates. New classification criteria for PAN have been validated that will have value in epidemiological studies and clinical trials. Renal involvement is common and recent therapeutic advances may result in improved treatment options. Cutaneous PAN is a milder disease characterised by periodic exacerbations and often associated with streptococcal infection. There is controversy as to whether this is a separate entity or part of the systemic PAN spectrum. Kawasaki disease is an acute self-limiting systemic vasculitis, the second commonest vasculitis in childhood and the commonest cause of childhood-acquired heart disease. Renal manifestations occur and include tubulointerstitial nephritis and renal failure. An infectious trigger and a genetic predisposition seem likely. Intravenous immunoglobulin (IV-Ig) and aspirin are effective therapeutically, but in resistant cases, either steroid or infliximab have a role. Greater understanding of the pathogenetic mechanisms involved in these three types of vasculitis and better long-term follow-up data will lead to improved therapy and prediction of prognosis. PMID:19946711

  4. Vasculitides with cutaneous expression in children: clinico-pathological correlations.

    PubMed

    Borroni, G; Grassi, S; Carugno, A

    2015-02-01

    The most recent pediatric vasculitis classifications (EULAR/PRINTO/PRES) have proposed the use of an integration of clinical signs and symptoms, laboratory data, imaging and pathologic data. Pediatric vasculitis represent a peculiar clinical-diagnostic model, compared to the corresponding adult pathology chapter, and in particular, dermatopathologic aspects of these diseases identify more specific issues, made contingent by crucial variables such as duration of vasculitis lesion, site of the biopsy, proper biopsy depth, and possibility to correlate histopathological findings with immunopathological results. Possible additional diagnostic difficulties may arise from the fact that, in children, the same systemic disease, such as lupus erythematosus, may present with different clinical manifestations, with histopathological features of a precise type of vasculitis specific for that type of clinical manifestation. Examples are provided by hypocomplementemic urticarial vasculitis, cryoglobulinemic purpura, lymphocytic vasculitis of livedoid lesions. This paper describes the cutaneous histopathological findings of some vasculitis related pediatric diseases, be they pertaining to a systemic vasculitis with corresponding cutaneous vasculitis, to a systemic vasculitis with sporadic cutaneous vasculitic involvement, and to a systemic vasculitis without cutaneous vasculitic involvement. Type and level of histopathological vasculitic involvement, caliber of the vessel, type of vasculitis associated infiltrate, are likewise reliable integration in the complex diagnostic path of vasculitis in childhood. On the basis of these criteria dermatopathologists should be confident in identifying the type of the vasculitis and relate them to a specific pediatric disease. PMID:25516220

  5. Educational Needs of Patients With Systemic Vasculitis

    ClinicalTrials.gov

    2014-07-11

    Behcet's Disease; Churg-Strauss Syndrome; Vasculitis, Central Nervous System; Giant Cell Arteritis; Wegener Granulomatosis; Henoch-Schoenlein Purpura; Microscopic Polyangiitis; Polyarteritis Nodosa; Takayasu's Arteritis

  6. Damage assessment in ANCA-associated vasculitis.

    PubMed

    Bhamra, Kuljeet; Luqmani, Raashid

    2012-12-01

    Antineutrophil cytoplasm antibody associated vasculitis has been transformed from life-threatening conditions to chronic relapsing long-term diseases as a result of significant advances in immunosuppressive therapy. Although mortality still occurs, it is much less frequent, with an average 5-year survival of over 70 %. In the setting of chronic conditions, it becomes increasingly important to monitor the burden of disease in terms of both active inflammation requiring immunosuppression and chronic damage (scarring) from vasculitis and its treatment and associated comorbidity. The damage that accumulates in patients with vasculitis does not respond to immunosuppressive treatment. It is important to distinguish disease activity from disease damage to prevent unnecessary immunosuppression, but it is equally important to recognize damage for what it is, so that it can be addressed appropriately. Damage is an inevitable consequence of long-term vasculitis for over 80 % of patients, which should not surprise us given the severity of the original illness. There is potential value in measuring damage as a means of providing prognostic information. Using a quantified score such as the Vasculitis Damage Index (VDI) allows us to predict mortality. Patients with at least five items of damage on the VDI score have substantially worse mortality (7- to 11-fold worse risk), as compared with those with lesser amounts of damage. These findings should be taken into context when planning the management of patients with vasculitis, as well as in clinical trials of vasculitis. Disease damage is an important surrogate for long-term outcome in vasculitis, and studies should be designed to limit the amount of damage accumulating as a result of therapeutic intervention, rather than simply controlling disease activity, as is currently the aim in recent randomized controlled trials in vasculitis. Furthermore, careful cataloguing of damage, as well as disease activity items, provides much

  7. [Diagnostic imaging of central nervous system vasculitis].

    PubMed

    Yokota, Hajime; Yamada, Kei

    2015-03-01

    Vasculitis involving the central nervous system presents with infarction and hemorrhage, which are often nonspecific findings. Laboratory examinations are essential for diagnosis of vasculitis in addition to comprehensive and systematic review of the clinical course. Although most findings tend to be nonspecific, enhancement and thickening of the vascular wall indicate vasculitis. Visualization of the vascular wall requires selection of the appropriate imaging modality and mode of image acquisition. Contrast-enhanced CT, MRI, and FDG-PET are useful for visualizing large vessel vasculitis, while CT, MRI, and angiography are effective for medium vessel vasculitis. The use of ultrasound is limited to evaluating vessels on the body surface. Although relatively thick vessels can be demonstrated by angiography, complete survey of small vessels is difficult. Here, we summarize the characteristics of each imaging modality and imaging findings of typical vasculitides-Takayasu arteritis, giant cell arteritis, ANCA-associated vasculitis, Behçet's disease, primary angiitis of the CNS, and vasculitis associated with systemic disease. Differential diagnoses are also shown, including infective endocarditis, tuberculous meningitis, Ehlers-Danlos syndrome, and reversible cerebral vasoconstriction syndrome. PMID:25846439

  8. Vasculitis

    MedlinePlus

    ... American College of Rheumatology Committee on Communications and Marketing. This information is provided for general education only. ... Lists Supporters About Us Leadership Careers at ACR Social Media Newsroom Annual Reports & Financial Statements Policies & Guidelines ...

  9. Systemic paraneoplastic vasculitis secondary to papillary carcinoma of the thyroid.

    PubMed

    Guerouaz, N; Alaoui, M; Raiss, M; Hassam, B; Senouci, K

    2016-08-01

    Systemic vasculitis secondary to thyroid carcinomas is exceptional. We report the case of a 55-year-old woman who presented with systemic vasculitis involving leucocytoclastic cutaneous vasculitis and renal disorders secondary to papillary thyroid carcinoma (PTC). Her symptoms resolved completely after total thyroidectomy. Other causes of vasculitis were excluded. To our knowledge, this is only the second case reported of systemic vasculitis associated with PTC in a paraneoplastic manner. PMID:27416971

  10. [Primary central nervous system vasculitis].

    PubMed

    Schuster, S; Magnus, T

    2015-12-01

    Primary angiitis of the central nervous system (PACNS) is a rare disorder. However, it is often considered in the differential diagnosis of vascular or inflammatory CNS diseases. Diagnosis is challenging, as specific biomarkers are lacking and the clinical presentation can be variable. A definitive diagnosis can only be established by biopsy of the inflammatory changes in the vascular wall. Alternatively, the diagnosis of PACNS can also be based on the synopsis of clinical, radiological, and laboratory findings. Different subtypes of PACNS have been described in recent years, depending on the size of the affected vessels or histopathological patterns. Based on selective literature research in the database PubMed on the subject of CNS vasculitis, this article reviews the diagnostic characteristics and differential diagnosis of the condition. We suggest a diagnostic algorithm customized to the size of the affected vessels. Lastly, therapeutic options and the outcome of PACNS are briefly outlined. PMID:26589203

  11. Biotherapies in large vessel vasculitis.

    PubMed

    Ferfar, Y; Mirault, T; Desbois, A C; Comarmond, C; Messas, E; Savey, L; Domont, F; Cacoub, P; Saadoun, D

    2016-06-01

    Giant cell arteritis (GCA) and Takayasu's arteritis (TA) are large vessel vasculitis (LVV) and aortic involvement is not uncommon in Behcet's disease (BD) and relapsing polychondritis (RP). Glucocorticosteroids are the mainstay of therapy in LVV. However, a significant proportion of patients have glucocorticoid dependance, serious side effects or refractory disease to steroids and other immunosuppressive treatments such as cyclophosphamide, azathioprine, mycophenolate mofetil and methotrexate. Recent advances in the understanding of the pathogenesis have resulted in the use of biological agents in patients with LVV. Anti-tumor necrosis factor-α drugs seem effective in patients with refractory Takayasu arteritis and vascular BD but have failed to do so in giant cell arteritis. Preliminary reports on the use of the anti-IL6-receptor antibody (tocilizumab), in LVV have been encouraging. The development of new biologic targeted therapies will probably open a promising future for patients with LVV. PMID:26883459

  12. Vasculitis: determinants of disease patterns.

    PubMed

    Hoffman, Gary S; Calabrese, Leonard H

    2014-08-01

    The vasculitides are a large group of heterogeneous diseases for which it has been assumed that pathogenesis is largely autoimmune. As clinicians, we distinguish one form of vasculitis from another on the basis of observed patterns of organ injury, the size of the vessels affected and histopathological findings. The terms 'small-vessel', 'medium-vessel' and 'large-vessel' vasculitis are useful clinical descriptors, but fail to inform us about why vessels of a certain calibre are favoured by one disease and not another. Classification based on vessel size also fails to consider that vessels of a specific calibre are not equally prone to injury. Distinct vulnerabilities undoubtedly relate to the fact that same-size vessels in different tissues may not be identical conduits. In fact, vessels become specialized, from the earliest stages of embryonic development, to suit the needs of different anatomical locations. Vessels of the same calibre in different locations and organs are as different as the organ parenchymal cells through which they travel. The dialogue between developing vessels and the tissues they perfuse is designed to meet special local needs. Added to the story of vascular diversity and vulnerability are changes that occur during growth, development and ageing. An improved understanding of the unique territorial vulnerabilities of vessels could form the basis of new hypotheses for the aetiopathogenesis of the vasculitides. This Review considers how certain antigens, including infectious agents, might become disease-relevant and how vascular diversity could influence disease phenotypes and the spectrum of vascular inflammatory diseases. PMID:24934189

  13. [Vasculitis caused by Pseudomonas: a case report].

    PubMed

    Escamilla, Y; Gutiérrez, M; Martínez, T; Bodoque, M; Gómez, J M; Moreno, A

    1996-01-01

    Pseudomona vasculitis is an exceptional disease. Only a few cases have been reported, non with oropharyngeal involvement. The case of a 30-year-old, HIV-positive man who suddenly developed septicemia and necrotizing lesions with tissue destruction of the oropharynx is reported. Histological study confirmed vasculitis. Pseudomona aeruginosa was isolated in peripheral blood and in the biopsy of the palatal lesion. Antibiotic treatment produced satisfactory results. PMID:8991411

  14. Immunohistological comparison of granulated cell proteins in induced immediate urticarial dermographism and delayed pressure urticaria lesions.

    PubMed

    McEvoy, M T; Peterson, E A; Kobza-Black, A; English, J S; Dover, J S; Murphy, G M; Bhogal, B; Greaves, M W; Winkelmann, R K; Leiferman, K M

    1995-12-01

    Urticarial dermographism and delayed pressure urticaria are two forms of physical urticaria which are well defined clinically and histologically. Previous studies have shown eosinophil granule protein deposition in urticarial reactions, including chronic urticaria, solar urticaria and delayed pressure urticaria. To evaluate and compare the involvement of granulated inflammatory cells in urticarial dermographism and delayed pressure urticaria, we studied sequential biopsies of induced lesions of urticarial dermographism and delayed pressure urticaria by indirect immunofluorescence, to detect eosinophil granule major basic protein (MBP) and neutrophil granule elastase. Biopsies from dermographic lesions at time 0, 5 min, 15 min, 2 h and 24 h, showed few infiltrating eosinophils, with minimal extracellular MBP deposition, and a few infiltrating neutrophils, with minimal neutrophil elastase deposition, throughout the evolution of the lesions. Sequential biopsies of delayed pressure urticaria at time 0, 20 min, 6, 12 and 24 h, showed eosinophil infiltration with extensive MBP deposition beginning at 20 min, and neutrophil infiltration with variable elastase deposition beginning at 20 min. Control tissue specimens from normal volunteers showed neutrophil infiltration and slight degranulation, but no eosinophil infiltration or degranulation. Comparison of urticarial dermographism with delayed pressure urticaria showed marked differences in the patterns of infiltration. Delayed pressure urticaria, with eosinophil and neutrophil degranulation, was strikingly similar to the IgE-mediated late phase reaction. In contrast, eosinophil and neutrophil involvement in urticarial dermographism was minimal. Considering the extent of eosinophil granule protein deposition and the biological activities of the eosinophil granule proteins, the findings in delayed pressure urticaria point to an important pathophysiological role of eosinophils in the disease. PMID:8547035

  15. Epigenetics and Vasculitis: a Comprehensive Review.

    PubMed

    Renauer, Paul; Coit, Patrick; Sawalha, Amr H

    2016-06-01

    Vasculitides represent a group of relatively rare systemic inflammatory diseases of the blood vessels. Despite recent progress in understanding the genetic basis and the underlying pathogenic mechanisms in vasculitis, the etiology and pathogenesis of vasculitis remain incompletely understood. Epigenetic dysregulation plays an important role in immune-mediated diseases, and the contribution of epigenetic aberrancies in vasculitis is increasingly being recognized. Histone modifications in the PR3 and MPO gene loci might be mechanistically involved in the pathogenesis of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Similarly, other studies revealed important epigenetic contribution to other vasculitides, including Kawasaki disease and IgA vasculitis. More recently, genome-wide epigenomic studies have been performed in several vasculitides. A recent genome-wide DNA methylation study uncovered an important role for epigenetic remodeling of cytoskeleton-related genes in the pathogenesis of Behçet's disease and suggested that reversal of some of these DNA methylation changes associates with disease remission. Genome-wide DNA methylation profiling characterized the inflammatory response in temporal artery tissue from patients with giant cell arteritis and showed increased activation of calcineurin/nuclear factor of activated T cells (NFAT) signaling, prompting the suggestion that a specific calcineurin/NFAT inhibitor that is well tolerated and with the added beneficial anti-platelet activity, such as dipyridamole, might be of therapeutic potential in giant cell arteritis. While epigenetic studies in systemic vasculitis are still in their infancy, currently available data clearly indicate that investigating the epigenetic mechanisms underlying these diseases will help to better understand the pathogenesis of vasculitis and provide novel targets for the development of disease biomarkers and new therapies. PMID:26093659

  16. [Neuromuscular biopsy and diagnosis of vasculitis].

    PubMed

    Vital, Anne; Vital, Claude

    2006-09-01

    One characteristic histological lesion on biopsy specimens is mandatory to establish the diagnosis of vasculitis. Combined nerve and muscle biopsies, by the same cutaneous incision, improve significantly the percentage of positive results. Nerve fragments should be taken in every patient presenting sensory manifestations. Such vasculitic lesions are present in medium-sized arterioles and/or small vessels, and correspond mainly to 4 necrotizing vasculitis: panarteritis nodosa (PAN), microscopic polyangiitis (MPA), Churg and Strauss syndrome and Wegener granulomatosis. Microvasculitis should be added to these classical entities, because it corresponds to small vessel wall infiltration by inflammatory cells, as observed in PAN and MPA, but without any necrosis. Microvasculitis has to be differentiated from the inflammatory cell infiltrates surrounding small vessels. However, such perivascular inflammatory cell infiltrates enable the diagnosis of probable vasculitis when associated with clusters of neo-vessels, hemosiderin deposits, or a focal damage of nerve fibers. Grossly, one third of vasculitis diagnosis is confirmed on muscle fragments, a second third on nerve fragments, and the last third on both nerve and muscle fragments. Moreover, in the search for vasculitis, an unpredicted diagnosis of lymphoma or amyloidosis is occasionally established on the neuro-muscular biopsy. PMID:17128151

  17. Reproductive Health in Men and Women With Vasculitis

    ClinicalTrials.gov

    2014-06-25

    Giant Cell Arteritis; Takayasu's Arteritis; Polyarteritis Nodosa; Wegener's Granulomatosis; Microscopic Polyangiitis; Churg-Strauss Syndrome; Behcet's Disease; Kawasaki Disease; Henoch-schoenlein Purpura; Vasculitis, Central Nervous System; Drug-induced Necrotizing Vasculitis

  18. HIV diagnosis in a patient presenting with vasculitis.

    PubMed

    Sharif, M; Hameed, S; Akin, I; Natarajan, U

    2016-02-01

    A patient with digital ischaemia and gangrene was treated with iloprost and antiplatelets for two weeks. His vasculitic screen was negative except for a positive HIV test. His vasculitis improved three weeks after treatment with antiretroviral medications. Though vasculitis is well known to be associated with HIV infection, very few cases of HIV present as vasculitis. PMID:25769887

  19. Connective Tissue Disorder-Associated Vasculitis.

    PubMed

    Sharma, Aman; Dhooria, Aadhaar; Aggarwal, Ashish; Rathi, Manish; Chandran, Vinod

    2016-06-01

    Vasculitides secondary to connective tissue diseases are classified under the category of 'vasculitis associated with systemic disease' in the revised International Chapel Hill Consensus Conference (CHCC) nomenclature. These secondary vasculitides may affect any of the small, medium or large vessels and usually portend a poor prognosis. Any organ system can be involved and the presentation would vary depending upon that involvement. Treatment depends upon the type and severity of presentation. In this review, we describe secondary vasculitis associated with rheumatoid arthritis, systemic lupus erythematosus, sarcoidosis, relapsing polychondritis, systemic sclerosis, Sjogren's syndrome and idiopathic inflammatory myositis, focusing mainly on recent advances in the past 3 years. PMID:27097818

  20. Imipenem-cilastatin-induced leukocytoclastic vasculitis.

    PubMed

    Reiner, M R; Brunetti, V A

    1997-05-01

    A maculopapular rash has been associated with the administration of imipenem-cilastatin, an antibiotic that was used for treatment of a postoperative infection. This is a first-time association of imipenem with a leukocytoclastic vasculitic reaction. Leukocytoclastic vasculitis has been previously documented with ciprofloxacin, zidovudine, piperazine, and lithium. PMID:9158321

  1. Primary CNS vasculitis presenting as intraventricular bleeding.

    PubMed

    Pillai, Sreeja Hareendranathan; Sreedharan, Sapna Erat; Menon, Girish; Kannoth, Santhosh; Pn, Sylaja

    2016-01-01

    Primary angiitis of the central nervous system (PACNS) is a rare disorder affecting both medium- and small-sized vessels. Intracranial haemorrhages though less reported are in the form of parenchymal haemorrhage and subarachnoid haemorrhage. We report a case of PACNS with intraventricular haemorrhage due to aneurysms secondary to progression of vasculitis. PMID:27570401

  2. Classification, pathogenesis, and treatment of systemic vasculitis.

    PubMed

    Griffith, M E; Gaskin, G; Pusey, C D

    1996-09-01

    Patients with systemic vasculitis (SV), especially Wegener's granulomatosis and microscopic polyangiitis, regularly present with renal involvement. Although considered a rare disease, either the incidence of SV is increasing or it is being increasingly recognized. Accurate classification systems are required to allow comparison of data from different groups investigating and treating these patients. Systemic vasculitis is known to be an autoimmune disease, but the mechanisms of pathogenesis have not been established, despite many studies on this topic in recent years. Most of this work has been done in vitro, although development of animal models is underway. Patient and renal survival have improved with aggressive immunosuppressive treatment, but morbidity is high and controversies remain in establishing the most effective regimens with minimum adverse effects. In this review we discuss the classification of SV, review the current knowledge of pathogenic mechanisms, and consider the relative merits of different treatment protocols. PMID:8903093

  3. Warfarin-induced leukocytoclastic vasculitis and proteinuria.

    PubMed

    Jumean, Khalid; Arqoub, Ahmad Abu; Hawatmeh, Amer; Qaqa, Firas; Bataineh, Ayham; Shaaban, Hamid

    2016-01-01

    Warfarin is typically prescribed for patients with thromboembolic diseases and atrial fibrillation. In addition to the complications of bleeding, allergic skin reaction is one of its rare adverse effects. We herein report a case of a 79 year old male patient with leukocytoclastic vasculitis and proteinuria secondary to warfarin. The warfarin was discontinued and oral prednisone therapy was initiated. The cutaneous lesions and the proteinuria resolved thereafter. PMID:27453863

  4. CHILDHOOD CUTANEOUS VASCULITIS: A COMPREHENSIVE APPRAISAL

    PubMed Central

    Palit, Aparna; Inamadar, Arun C

    2009-01-01

    Cutaneous vasculitides in childhood are rare and often present with clinical features distinct from adults. Diagnosis of cutaneous vasculitides in children was difficult because of lack of a satisfactory classification systems for this age group. A new international classification system for childhood vasculitis has been discussed in the following section along with important clinical features, diagnostic modalities, and recent therapeutic developments of important vasculitides in children. PMID:20101304

  5. Immunologic Cerebral Vasculitis and Extrapulmonary Tuberculosis: An Uncommon Association.

    PubMed

    Wang, Yiyi; Li, Qian; Zhen, Xiaohan; Liu, Yuan; Wu, Qi

    2015-09-01

    Infection can cause cerebral vasculitis not only by direct invasion of the vessel wall, but by immune complex deposition, or through secondary cryoglobulineamia. There are also two types of cerebral vasculitis associated with tuberculosis (TB). In TB treatment, cerebral vasculitis caused by immunologic injury received little attention than vasculitis due to direct invasion of TB infection. We report a case in a young woman who presented with fever, generalized lymphadenopathy, stroke-like events, movement disorder and coma, which was found to be active, lymph node TB with immunologic cerebral vacuities without tuberculosis meningitis. PMID:26500938

  6. Endoscopic Findings of Upper Gastrointestinal Involvement in Primary Vasculitis

    PubMed Central

    Gong, Eun Jeong; Kim, Do Hoon; Chun, Joo Hyun; Ahn, Ji Yong; Choi, Kwi-Sook; Jung, Kee Wook; Lee, Jeong Hoon; Choi, Kee Don; Song, Ho June; Lee, Gin Hyug; Jung, Hwoon-Yong; Kim, Jin Ho; Song, In Hye; Kim, Yong-Gil

    2016-01-01

    Background/Aims Gastrointestinal involvement in vasculitis may result in life-threatening complications. However, its variable clinical presentations and endoscopic features, and the rarity of the disease, often result in delayed diagnosis. Methods Clinical characteristics, endoscopic features, and histopathological findings were reviewed from medical records. Results Of 6,477 patients with vasculitis, 148 were diagnosed as primary vasculitis with upper gastrointestinal involvement. Of these, 21 cases (14.2%) were classified as large-vessel vasculitis, 17 cases (11.5%) as medium-vessel vasculitis, and 110 cases (74.3%) as small-vessel vasculitis. According to the specific diagnosis, IgA vasculitis (Henoch-Schönlein purpura) was the most common diagnosis (56.8%), followed by Takayasu arteritis (14.1%), microscopic polyangiitis (10.1%), and polyarteritis nodosa (6.8%). Gastrointestinal symptoms were present in 113 subjects (76.4%), with abdominal pain (78.8%) the most common symptom. Erosion and ulcers were striking endoscopic features, and the second portion of the duodenum was the most frequently involved site. Biopsy specimens were obtained from 124 patients, and only eight (5.4%) presented histopathological signs of vasculitis. Conclusions Diagnosis of vasculitis involving the upper gastrointestinal tract is difficult. Because of the widespread use of endoscopy, combining clinical features with endoscopic findings may facilitate making appropriate diagnoses; however, the diagnostic yield of endoscopic biopsy is low. PMID:27226428

  7. A Case of Leukocytoclastic Vasculitis Caused by Listeria monocytogenes Bacteremia.

    PubMed

    Bunker, Daniel R; Sullivan, Timothy

    2016-01-01

    Importance. Infections can cause leukocytoclastic vasculitis. Observations. We report the case of a patient with a left ventricular assist device who presented with acute kidney injury and biopsy proven leukocytoclastic vasculitis. Blood cultures grew Listeria monocytogenes. The patient's rash improved with treatment of the underlying Listeria infection. Conclusion. Clinicians should be aware that there are a number of broad categories of disease associated with the histologic finding of vasculitis, including infection. It is important to keep in mind the risk factors of a particular patient when formulating a differential diagnosis. This is the first reported case of Listeria bacteremia causing leukocytoclastic vasculitis. PMID:27313916

  8. Immunologic Cerebral Vasculitis and Extrapulmonary Tuberculosis: An Uncommon Association

    PubMed Central

    Wang, Yiyi; Li, Qian; Zhen, Xiaohan; Liu, Yuan

    2015-01-01

    Infection can cause cerebral vasculitis not only by direct invasion of the vessel wall, but by immune complex deposition, or through secondary cryoglobulineamia. There are also two types of cerebral vasculitis associated with tuberculosis (TB). In TB treatment, cerebral vasculitis caused by immunologic injury received little attention than vasculitis due to direct invasion of TB infection. We report a case in a young woman who presented with fever, generalized lymphadenopathy, stroke-like events, movement disorder and coma, which was found to be active, lymph node TB with immunologic cerebral vacuities without tuberculosis meningitis. PMID:26500938

  9. Systemic Lupus Erythematosus Vasculitis: A Current Therapeutic Overview.

    PubMed

    Toubi, Elias; Kessel, Aharon; Bamberger, Ellen; Golan, Theo Dov

    2004-04-01

    The development of systemic lupus erythematosus (SLE) vasculitis is of prognostic value. The earlier the vasculitis is treated, the better the prognosis for SLE. Cutaneous vasculitis is common in SLE, whereas visceral vasculitis is rare. Skin SLE vasculitis is successfully treated with antimalarials, but its discontinuation may result in an SLE flare even among patients in remission. When visceral SLE vasculitis is encountered, or when a disease state is perceived to be life-threatening, a more aggressive therapy is warranted. A combination of medications, plasmapheresis, and intravenous immunoglobulin treatment, along with high-dose steroids and cytotoxic drugs, are typically employed in the treatment of severe SLE vasculitis. Finally, patients with SLE vasculitis may benefit from a number of autoimmune disease therapies currently under investigation, such as switching cytokine responses from Th1 to Th2, and the manipulation of toll-like receptors, chemokines, and FcR receptors. Specific B-cell therapies (eg, anti-Blys, B-cell depletion) may also emerge as potential treatments for SLE vasculitis. PMID:15066237

  10. A Case of Leukocytoclastic Vasculitis Caused by Listeria monocytogenes Bacteremia

    PubMed Central

    2016-01-01

    Importance. Infections can cause leukocytoclastic vasculitis. Observations. We report the case of a patient with a left ventricular assist device who presented with acute kidney injury and biopsy proven leukocytoclastic vasculitis. Blood cultures grew Listeria monocytogenes. The patient's rash improved with treatment of the underlying Listeria infection. Conclusion. Clinicians should be aware that there are a number of broad categories of disease associated with the histologic finding of vasculitis, including infection. It is important to keep in mind the risk factors of a particular patient when formulating a differential diagnosis. This is the first reported case of Listeria bacteremia causing leukocytoclastic vasculitis. PMID:27313916

  11. Monoclonal antibody desensitization in a patient with a generalized urticarial reaction following denosumab administration.

    PubMed

    Gutiérrez-Fernández, D; Cruz, María-Jesús; Foncubierta-Fernández, A; Moreno-Ancillo, A; Fernández-Anguita, M J; Medina-Varo, F; Andres-García, J A

    2015-01-01

    Denosumab is a human monoclonal antibody indicated for the treatment of osteoporosis in postmenopausal women with a high risk of fractures. To our knowledge, no cases of desensitization to this drug have been described in the literature. We report the first case of generalized urticarial reaction and facial angioedema after therapy with denosumab. A subcutaneous desensitization protocol was successfully completed in this patient. Rapid desensitization is a promising method for the delivery of denosumab after a hypersensitivity reaction, and should be considered in osteoporosis treatment when no acceptable therapeutic alternatives are available. PMID:26504466

  12. Paraneoplastic vasculitis associated to pelvic chondrosarcoma: a case report

    PubMed Central

    Arvinius, Camilla; González-Pérez, Ana; García-Coiradas, Javier; García-Maroto, Roberto; Cebrián-Parra, Juan Luis

    2016-01-01

    Vasculopathic syndromes have been associated with hematological and solid organ malignancies. The pathogenesis of these syndromes remains largely unknown and there are no biologic markers identified. Whether it is or is not a paraneoplastic syndrome is under discussion, the close temporal relationship of cancer and vasculitis suggests that these vasculitides are a paraneoplastic condition. We report a case of a 45-year-old female patient with pelvic chondrosarcoma who underwent surgical treatment and started to present visual loss, systemic inflammatory response syndrome (SRIS), cardiac insufficiency, hepatosplenomegaly, cholestasis as well as pulmonary bleeding suggesting a sarcoma-associated vasculitis. All antibodies were negative as in secondary vasculitis. After corticoideal therapy the vasculitis resolved and at 3-year follow-up the patient had not showed any further medical complications or recurrences of the vasculitis. The parallel evolution of the vasculitis and the solid tumor combined with the resolution of the vasculitis after corticotherapy enhances the likelihood of a paraneoplastic vasculitis associated with a chondrosarcoma according to literature review. PMID:27163097

  13. Peroneal nerve involvement as initial manifestation of primary systemic vasculitis

    PubMed Central

    Ripellino, Paolo; Varrasi, Claudia; Maldi, Elena; Cantello, Roberto

    2014-01-01

    We report a case of two patients with foot drop due to peroneal nerve infarct as early sign of two different forms of primary systemic vasculitides: a predominantly small-vessel p-antineutrophil cytoplasmic antibody-positive vasculitis (microscopic polyangiitis) and a predominantly medium-vessel vasculitis (polyarteritis nodosa). PMID:24686810

  14. Cryoglobulinaemic vasculitis: classification and clinical and therapeutic aspects

    PubMed Central

    Braun, Gerald S; Horster, Sophia; Wagner, Katrin S; Ihrler, Stephan; Schmid, Holger

    2007-01-01

    Cryoglobulinaemia may cause cutaneous vasculitis and glomerulonephritis, potentially leading to end stage renal failure. An important proportion of cryoglobulinaemias are secondary to hepatitis C virus infection. Emerging antiviral treatment options offer a chance for causal therapy of these cases of cryoglobulinaemia. This review summarises the classification and clinical and therapeutic aspects of cryoglobulinaemic vasculitis and glomerulonephritis. PMID:17308210

  15. Time to focus on outcome assessment tools for childhood vasculitis

    PubMed Central

    2011-01-01

    Childhood systemic vasculitides are a group of rare diseases with multi-organ involvement and potentially devastating consequences. After establishment of new classification criteria (Ankara consensus conference in 2008), it is now time to establish measures for proper definition of activity and damage in childhood primary vasculitis. By comparison to adult vasculitis, there is no consensus for indices of activity and damage assessment in childhood vasculitis. Assessment of disease activity is likely to become a major area of interest in pediatric rheumatology in the near future. After defining the classification criteria for primary systemic childhood vasculitis, the next step was to perform a validation study using the original Birmingham vasculitis activity score as well as the disease extent index to measure disease activity in childhood vasculitis. Presently, there are efforts in place to develop a pediatric vasculitis activity score. This paper reviews the current understanding about the assessment tools (i.e., clinical features, laboratory tests, radiologic assessments, etc.) widely used for evaluation of the disease activity and damage status of the children with vasculitis. PMID:21943296

  16. Catastrophic primary central nervous system vasculitis.

    PubMed

    Salvarani, Carlo; Brown, Robert D; Morris, Jonathan M; Huston, John; Hunder, Gene G

    2014-01-01

    Primary central nervous system vasculitis (PCNSV) is an uncommon condition that affects the brain and the spinal cord. It is heterogeneous in presenting characteristics and outcomes. We report a patient with a catastrophic rapidly progressive course refractory to intensive treatment with pulses of methylprednisolone and iv cyclophosphamide. The condition rapidly deteriorated and the patient died 6 weeks after presentation. Rapidly progressive PCNSV represents the worst end of the clinical spectrum of PCNSV. These patients are characterised by bilateral, multiple, large cerebral vessel lesions on angiograms and multiple bilateral cerebral infarctions. PMID:24854370

  17. Phenytoin-Associated Granulomatous Pulmonary Vasculitis.

    PubMed

    Kheir, Fayez; Daroca, Philip; Lasky, Joseph

    2016-01-01

    Phenytoin was introduced in 1938 for the control of seizure disorders and remains widely used today. Since that time, many cases of phenytoin-induced allergic reactions and clinical pulmonary disease have been reported. However, pulmonary vascular pathology from phenytoin use has been only very rarely described. We report a case of phenytoin-associated vasculitis in a 39-year-old African American man presenting with progressive dyspnea and abnormal chest imaging. The importance of reviewing the medication history along with familiarity with the array of drug-associated lung diseases is crucial to recognize and treat pneumotoxicity. PMID:24247101

  18. Cerebral large vessel vasculitis in systemic lupus erythematosus.

    PubMed

    Böckle, B C; Jara, D; Aichhorn, K; Junker, D; Berger, T; Ratzinger, G; Sepp, N T

    2014-11-01

    Neuropsychiatric systemic lupus erythematosus (NPSLE) is defined by involvement of the central nervous system in systemic lupus erythematosus (SLE), with a wide range of both neurological and psychiatric manifestations. Although its aetiopathogenesis is not fully elucidated, NPSLE seems to be a consequence of cerebral vascular pathology including thromboembolism, small-vessel vasculopathy and, in rare cases, true vasculitis. Cerebral vasculitis is rare, and cerebral large-vessel vasculitis in SLE is even more unusual. We report the case of a female patient with the diagnosis of SLE. She presented with stroke-like symptoms, headache and vertigo, and palpable purpura on her legs. Further investigations revealed that she suffered from both vasculitis of the cerebral large vessels and coexisting cutaneous small-vessel vasculitis. PMID:24969082

  19. Enhancement of mite antigen-induced histamine release by deuterium oxide from leucocytes of chronic urticarial patients

    SciTech Connect

    Numata, T.; Yamamoto, S.; Yamura, T.

    1981-09-01

    The mite antigen-induced histamine release from leucocytes of chronic urticarial patients was enhanced in the presence of deuterium oxide, which stabilizes microtubules. This enhancing effect of deuterium oxide on the histamine release from leucocytes may provide a useful means for the detection of allergens in vitro in chronic urticaria.

  20. Lepra reaction with lucio phenomenon mimicking cutaneous vasculitis.

    PubMed

    Misra, Durga Prasanna; Parida, Jyoti Ranjan; Chowdhury, Abhra Chandra; Pani, Krushna Chandra; Kumari, Niraj; Krishnani, Narendra; Agarwal, Vikas

    2014-01-01

    Leprosy is a disease typically found in the tropics. Patients with leprosy can have varying presentation with constitutional symptoms, joint pains, skin nodules, and rarely a vasculitis-like picture with skin ulcers and neuropathy. We present a young lady who presented with the rare manifestation of skin infarcts mimicking cutaneous vasculitis, diagnosed on histopathology to have Lucio phenomenon on a background of lepromatous leprosy. With increasing migration and widespread use of biologic response modifiers, clinicians all over the world need to be aware of various presentations of leprosy as well as needing to keep an open mind while considering the differential diagnoses of vasculitis. PMID:25580317

  1. Lepra Reaction with Lucio Phenomenon Mimicking Cutaneous Vasculitis

    PubMed Central

    Misra, Durga Prasanna; Parida, Jyoti Ranjan; Chowdhury, Abhra Chandra; Pani, Krushna Chandra; Kumari, Niraj; Krishnani, Narendra

    2014-01-01

    Leprosy is a disease typically found in the tropics. Patients with leprosy can have varying presentation with constitutional symptoms, joint pains, skin nodules, and rarely a vasculitis-like picture with skin ulcers and neuropathy. We present a young lady who presented with the rare manifestation of skin infarcts mimicking cutaneous vasculitis, diagnosed on histopathology to have Lucio phenomenon on a background of lepromatous leprosy. With increasing migration and widespread use of biologic response modifiers, clinicians all over the world need to be aware of various presentations of leprosy as well as needing to keep an open mind while considering the differential diagnoses of vasculitis. PMID:25580317

  2. The Spectrum of Paraneoplastic Cutaneous Vasculitis in a Defined Population

    PubMed Central

    Loricera, Javier; Calvo-Río, Vanesa; Ortiz-Sanjuán, Francisco; González-López, Marcos A.; Fernández-Llaca, Hector; Rueda-Gotor, Javier; Gonzalez-Vela, Maria C.; Alvarez, Lino; Mata, Cristina; González-Lamuño, Domingo; Martínez-Taboada, Victor M.; González-Gay, Miguel A.; Blanco, Ricardo

    2013-01-01

    Abstract Cutaneous vasculitis may be associated with malignancies, and may behave as a paraneoplastic syndrome. This association has been reported in a variable proportion of patients depending on population selection. We conducted the current study to assess the frequency, clinical features, treatment, and outcome of paraneoplastic vasculitis in a large unselected series of 766 patients with cutaneous vasculitis diagnosed at a single university hospital. Sixteen patients (10 men and 6 women; mean age ± standard deviation, 67.94 ± 14.20 yr; range, 40–85 yr) presenting with cutaneous vasculitis were ultimately diagnosed as having an underlying malignancy. They constituted 3.80% of the 421 adult patients. There were 9 hematologic and 7 solid underlying malignancies. Skin lesions were the initial clinical presentation in all of them, and the median interval from the onset of cutaneous vasculitis to the diagnosis of the malignancy was 17 days (range, 8–50 d). The most frequent skin lesions were palpable purpura (15 patients). Other clinical manifestations included constitutional syndrome (10 patients) and arthralgia and/or arthritis (4 cases). Hematologic cytopenias (11 cases) as well as immature peripheral blood cells (6 cases) were frequently observed in the full blood cell count, especially in those with vasculitis associated with hematologic malignancies. Specific treatment for vasculitis was prescribed in 10 patients; nonsteroidal antiinflammatory drugs (4 patients), corticosteroids (3 patients), chloroquine (1 patient), antihistamines (1 patient), and cyclophosphamide (1 patient). Ten patients died due to the malignancy and 6 patients recovered following malignancy therapy. Patients with paraneoplastic vasculitis were older, more frequently had constitutional syndrome, and less frequently had organ damage due to the vasculitis than the remaining patients with cutaneous vasculitis. In summary, cutaneous paraneoplastic vasculitis is an entity not uncommonly

  3. A 15-year exercise program for rheumatoid vasculitis.

    PubMed

    Marley, W P; Santilli, T F

    1998-01-01

    A rare case of rheumatoid vasculitis and responses to a 15-year supervised exercise program. This patient presented with significant impairment in mobility and physical work capacity. His exercise tolerance improved considerably and he benefited emotionally. PMID:9572643

  4. Innate immune cells in the pathogenesis of primary systemic vasculitis.

    PubMed

    Misra, Durga Prasanna; Agarwal, Vikas

    2016-02-01

    Innate immune system forms the first line of defense against foreign substances. Neutrophils, eosinophils, erythrocytes, platelets, monocytes, macrophages, dendritic cells, γδ T cells, natural killer and natural killer T cells comprise the innate immune system. Genetic polymorphisms influencing the activation of innate immune cells predispose to development of vasculitis and influence its severity. Abnormally activated innate immune cells cross-talk with other cells of the innate immune system, present antigens more efficiently and activate T and B lymphocytes and cause tissue destruction via cell-mediated cytotoxicity and release of pro-inflammatory cytokines. These secreted cytokines further recruit other cells to the sites of vascular injury. They are involved in both the initiation as well as the perpetuation of vasculitis. Evidences suggest reversal of aberrant activation of immune cells in response to therapy. Understanding the role of innate immune cells in vasculitis helps understand the potential of therapeutic modulation of their activation to treat vasculitis. PMID:26403285

  5. What Are the Signs and Symptoms of Vasculitis?

    MedlinePlus

    ... from the NHLBI on Twitter. What Are the Signs and Symptoms of Vasculitis? The signs and symptoms ... symptoms develop quickly, over days or weeks. Systemic Signs and Symptoms Systemic signs and symptoms are those ...

  6. The long-term outcomes of systemic vasculitis.

    PubMed

    Westman, Kerstin; Flossmann, Oliver; Gregorini, Gina

    2015-04-01

    Patients with generalized ANCA-associated small vessel vasculitis (AAV) have a very poor outcome if the ANCA-associated vasculitis is not diagnosed, evaluated and treated properly. The introduction of treatment with immunosuppressive therapy has improved patient survival dramatically but with considerable side effects. Besides, almost 50% of surviving patients experience a relapse of vasculitis. Since 1995, the European Vasculitis Society (EUVAS) has designed and conducted several clinical trials on patients with AAV independently of pharmaceutical companies. The studies included patients with newly diagnosed AAV and were stratified according to renal function and generalized versus more localized forms. As the immediate patient survival has improved, the longer term outcome has become more important. There are several reports on outcome of patients with ANCA-associated vasculitis, but the patient groups were heterogeneous regarding diagnosis as well as treatment and follow-up. Therefore, EUVAS decided to further evaluate the effect and possible adverse events of the original randomized trials. This review presents an overview on long-term follow-up of patients with ANCA-associated vasculitis, with focus on relapse rate, patient and renal survival and development of cardiovascular disease and malignancy. PMID:25601266

  7. [Vasculitis as a reason of chronic headache].

    PubMed

    Ataia, I; Casaulta, C; von Vigier, R O; Pfammatter, J P; Brekenfeld, C; Sauvain, M J; Steinlin, M

    2008-03-19

    A 13-year-old girl presented to our emergency with a one week history of fever and skin rash and new onset of chorea for the last three days. There was a long standing history of right predominant headache; followed by personality change, fatigue, arthralgia and weight loss over the last few months. Previous investigations by head CT and ophthalmological examination did not explain the symptoms. Further investigations revealed peri- and pancarditis with aortic insufficiency, a renal involvement with elevated creatinin, protein- and hematuria and a hemolytic anemia. Diagnosis of lupus eythematodes was confirmed by high ANA, anti-dsDNS and Anticardiolipin antibodies. Within the first 48 hours after admission there was significant deterioration with reduced vigilance and dysarthria. MRI of the brain and dopplersonography of cerebral vessels showed a complete thrombosis of the right medial cerebral artery with a small net of collaterals, irregularities of the left cerebral artery due to vasculitis and several subacute leftsided ischemias. Immunosuppressive therapy with high-dose corticosteroids and cyclophosphamid together with antithrombotic therapy induced an improvement of neurologic, renal and cardiac function. PMID:18548940

  8. Biological Therapy-Induced Systemic Vasculitis.

    PubMed

    Gutiérrez-González, Luis Arturo

    2016-07-01

    The use of biologics has been associated with the paradoxical development of biologics-induced autoimmune diseases. The purpose of this review was to describe the key immunopathogenic mechanisms involved in the development of these conditions, and to discuss the clinical and laboratory characteristics usually described in the medical literature, reviewing case reports as well as records on national biologic therapies (BIOGEAS, RABBIT, BSRBR-RA, BIOBADAVEN). More than 200 cases have so far been reported, all of them diagnosed on the basis of the histopathology or meeting the ACR/Chapel Hill criteria. Over 75 % of the cases were females with a mean age of 48 ± 5 years. More than 50 % had rheumatoid arthritis. Most of the biologics-associated vasculitis developed in 90 ± 31 days. Complete resolution in almost 75 % of the cases was observed upon treatment discontinuation; however, steroid therapy was indicated for all patients and one death was recorded. The use of cyclophosphamide, rituximab or plasma exchange was reserved for the most severe cases. PMID:27165496

  9. [Usefulness of imaging in large vessel vasculitis].

    PubMed

    Mekinian, A; Djelbani, S; Viry, F; Fain, O; Soussan, M

    2016-04-01

    The imaging is essential for the diagnosis of large vessels arteritis, in order to assess the persistent inflammation of arterial lesions, to evaluate the treatment response and search the vascular complications. In patients with giant cell arteritis (GCA), the aortitis could be suspected in 2 situations: in the presence of general constitutional symptoms or systematic screening of aortitis in patient with confirmed GCA. The frequency of aortitis varies according to the imaging method and could be detected in 40 % of patients with computed tomography and MRI, and approximately in 60 % with FDG-PET/CT. The clinical and prognostic value of systematic detection of aortitis during the GCA remains to be determined. In Takayasu arteritis, imaging is performed to diagnose the large vessels vasculitis, to determine the arterial lesions extension to assess the persistent inflammation of arterial lesions. The persistent vascular inflammation should be suspected in the presence of arterial thickness, of arterial enhancement, a parietal edema and increased arterial FDG uptake (>liver). However, the value of these parameters and the threshold remain to be determined. Thus, the value of FDG-PET/CT and MRI and of parameters used to characterize the persistent arterial inflammation should be further studied. PMID:26880245

  10. Urinary neopterin in idiopathic retinal vasculitis

    PubMed Central

    Palmer, H; Giovannoni, G; Stanford, M; Wallace, G; Graham, E

    2001-01-01

    AIMS—To determine whether urinary neopterin:creatinine (UNC) ratios relate to disease activity in idiopathic retinal vasculitis (RV).
METHODS—18 patients with RV were prospectively recruited into a year long longitudinal study. Patients collected first morning urine samples on a weekly basis and on the same day completed a diary which documented their subjective view of RV activity and any concurrent infection. They were examined in clinic on a 6-8 weekly basis and an objective assessment was made of RV disease activity. 14 healthy controls collected urine samples in the same way.
RESULTS—UNC ratios were significantly higher in patients than in controls (p=0.004, Mann-Whitney U test). UNC ratios were significantly higher when, according to their diaries, the patients had a subjective flare up of RV (p=0.001, Mann-Whitney U test). Subjective increased RV activity occurred more often when the patients had a concurrent infection (p<0.0001, χ2 test). There was no significant difference in the UNC ratio between objective clinical relapse and non-relapse of RV. There was moderate agreement between the clinical assessment and patients' subjective impression of RV activity (κ=0.48).
CONCLUSIONS—Higher neopterin levels reflect cell mediated disease that occurs in RV, but UNC ratios are not recommended as a means of monitoring clinical disease activity in RV.

 PMID:11133708

  11. Lung vasculitis and alveolar hemorrhage: pathology.

    PubMed

    Fishbein, Gregory A; Fishbein, Michael C

    2011-06-01

    Pulmonary vasculitides are a diverse group of limited and systemic disorders associated with inflammation of pulmonary vessels and parenchyma. These diseases often have distinctive clinical, serological, and histopathological features-extrapulmonary sites of involvement, circulating autoantibodies, predispositions for small or large vessels, and others. Some have characteristic inflammatory lesions; others are characterized by the absence of such lesions. Frequently pathological findings overlap, rendering classification, and diagnosis a challenge. The anti-neutrophil cytoplasmic antibody (ANCA)-associated small-vessel diseases constitute the major pulmonary vasculitides. These include Wegener granulomatosis (WG), Churg Strauss syndrome (CSS), and microscopic polyangiitis (MPA). Less frequently, diseases such as polyarteritis nodosa, Takayasu arteritis, Behçet syndrome, and connective tissue diseases may involve pulmonary vessels, but these entities are better associated with extrapulmonary disease. Diffuse alveolar hemorrhage (DAH) is a severe manifestation of pulmonary vasculitis. DAH is most commonly seen in small-vessel vasculitides, specifically MPA and WG. Other syndromes associated with DAH include Goodpasture syndrome, Henoch-Schönlein purpura, and systemic lupus erythematosus. Less commonly, DAH may be secondary to infection or drugs/toxins. Furthermore, in the absence of discernable systemic disease, DAH may be idiopathic-referred to as isolated pulmonary capillaritis (IPC) or idiopathic pulmonary hemosiderosis (IPH), depending on the presence of capillaritis. PMID:21674412

  12. Pediatric vasculitis: recognizing multisystemic manifestations at body imaging.

    PubMed

    Khanna, Geetika; Sargar, Kiran; Baszis, Kevin W

    2015-01-01

    Pediatric vasculitides are multisystem diseases that can be diagnostic challenges because of variable clinical manifestations. The clinical manifestation is determined by the size of the affected vessels, organs involved, extent of vascular injury, and underlying pathologic characteristics. Henoch-Schönlein purpura and Kawasaki disease are the two most common subtypes of pediatric vasculitis. Diagnosis of pediatric vasculitis can be difficult, and the outcome can be serious or fatal in the absence of timely intervention. Imaging plays a central role in establishing the diagnosis of vasculitis involving large- and medium-sized vessels, visualizing its vascular and extravascular manifestations, and monitoring the disease course and response to treatment. Although imaging cannot depict the vessel changes of small-vessel vasculitis directly, it can be used to detect tissue damage resulting from vessel inflammation. This article discusses the classification and clinical features of the major pediatric vasculitides. The imaging approach to and nonneurologic findings of major pediatric vasculitis subtypes are reviewed for the pediatric body imager. PMID:25969938

  13. Vessel wall enhancement in herpes simplex virus central nervous system vasculitis.

    PubMed

    Guerrero, Waldo R; Dababneh, Haitham; Hedna, Shushrutha; Johnson, James A; Peters, Keith; Waters, Michael F

    2013-09-01

    Infection is a well-known cause of cerebral vasculopathy and vasculitis. We report a 36-year-old woman with cerebral vasculitis and ischemic stroke secondary to herpes simplex virus (HSV). MRI studies revealed a pontine stroke with basilar artery stenosis and vessel wall gadolinium enhancement. This case demonstrates the ability of HSV to cause a focal brainstem vasculitis and the utility of enhanced MRI in the diagnosis of stroke related to HSV central nervous system vasculitis. PMID:23517674

  14. Leukocytoclastic vasculitis in a patient with type 1 cryoglobulinemia.

    PubMed

    Liu, Paul Y; Prete, Pamela E; Kukes, Gary

    2011-01-01

    Cutaneous manifestations of type 1 cryoglobulinemia are usually related to vascular occlusion by noninflammatory thrombosis; rarely is leukocytoclastic vasculitis seen in type 1 cryoglobulinemia. We report the case of a 64-year-old male who presented with isolated cutaneous leukocytoclastic vasculitis that was initially attributed to essential mixed cryoglobulinemia after thorough diagnostic evaluation. A lack of adequate clinical response to therapy prompted further investigation, including cryoprecipitate electrophoresis and immunofixation, which revealed an IgM kappa monoclonal gammopathy consistent with type 1 cryoglobulinemia. A renewed search for an underlying malignancy led to the discovery of early Waldenstrom's macroglobulinemia. Although leukocytoclastic vasculitis is more characteristic of mixed cryoglobulinemia, it can be a presenting manifestation of type 1 cryoglobulinemia. PMID:22937435

  15. A Fatal Case of "Bullous Erysipelas-like" Pseudomonas Vasculitis.

    PubMed

    Yang, Sam Shiyao; Chandran, Nisha Suyien; Huang, Jing Xiang; Tan, Kong-Bing; Aw, Derrick Chen-Wee

    2016-01-01

    Erysipelas is a generally benign superficial bacterial skin infection, and its bullous form constitutes a rare and more severe variant. We describe the first and fatal case of "bullous erysipelas-like" septic vasculitis due to Pseudomonas bacteremi. A 69-year-old Chinese man presenting with diarrhea and septic shock initially began to rapidly develop sharply defined erythematous plaques with non-hemorrhagic bullae over his lower limbs. Culture of the aspirate from the bullae was positive for Pseudomonas aeruginosa. This was also consistent with his blood cultures showing Pseudomonas bacteremia. Histology of the skin lesion showed microthrombi and neutrophilic infiltrates in blood vessels with Gram-negative bacilli extruding from the vessel walls, characteristic of septic vasculitis. The bullous erysipelas-like lesions seen in this patient represents a rare manifestation of both septic vasculitis and Pseudomonas infection. PMID:26955132

  16. Isolated Central Nervous System Vasculitis as a Manifestation of Neurosarcoidosis.

    PubMed

    Macêdo, Philippe Joaquim Oliveira Menezes; da Silveira, Victor Calil; Ramos, Luíza Tavares; Nóbrega, Felipe Resende; Vasconcellos, Luiz Felipe Rocha

    2016-06-01

    A 62-year-old male presented to our clinic with recurrent fever, skin lesions (petechiae), scleral wounds, and hilar adenomegaly. A diagnosis of sarcoidosis was established, which resolved with corticosteroid treatment. After a few months, the patient developed confusion and behavioral changes, with few objective neurological deficits. Brain magnetic resonance imaging showed slight focal meningeal enhancement (prepontine region). The level of angiotensin-converting enzymes was normal in the serum and increased in the cerebrospinal fluid. The patient was diagnosed with neurosarcoidosis, and corticosteroid treatment was prescribed, yielding good clinical response. Nine months later, the patient presented with multiple ischemic strokes, and arteriography demonstrated multiple distal irregularities in all arterial territories, suggesting cerebral vasculitis. Even with corticosteroids, cyclophosphamide, and intravenous immunoglobulin, the patient died. Vasculitis is rarely seen in association with sarcoidosis, and in this case, no systemic manifestation was observed at the time that the patient developed vasculitis. PMID:27083067

  17. An approach to the diagnosis and management of systemic vasculitis.

    PubMed

    Miller, A; Chan, M; Wiik, A; Misbah, S A; Luqmani, R A

    2010-05-01

    The systemic vasculitides are a complex and often serious group of disorders which, while uncommon, require careful management in order to ensure optimal outcome. In most cases there is no known cause. Multi-system disease is likely to be fatal without judicious use of immunosuppression. A prompt diagnosis is necessary to preserve organ function. Comprehensive and repeated disease assessment is a necessary basis for planning therapy and modification of treatment protocols according to response. Therapies typically include glucocorticoids and, especially for small and medium vessel vasculitis, an effective immunosuppressive agent. Cyclophosphamide is currently the standard therapy for small vessel multi-system vasculitis, but other agents are now being evaluated in large randomized trials. Comorbidity is common in patients with vasculitis, including the cumulative effects of potentially toxic therapy. Long-term evaluation of patients is important in order to detect and manage relapses. PMID:20070316

  18. Cutaneous Vasculitis in a Patient with Antiphospholipid Antibody Syndrome.

    PubMed

    Sheth, Khushboo; Parke, Ann

    2016-02-01

    Antiphospholipid antibody syndrome (APS) is an acquired thrombophilia, caused by autoantibodies to anticardiolipin (aCL), or antibeta 2 glycoprotein I, or the presence of lupus anticoagulant (LA) in plasma. It is characterized by recurrent venous and/or arterial thrombi and/or pregnancy related morbidities. We present the case of a 52-year-old female with long-standing APS, who developed cutaneous vasculitis following a common cold. Most of the cutaneous manifestations of APS have been found to be thrombotic on histopathology without evidence of perivascular inflammation. Vasculitis is usually seen in APS patients with coexistent Systemic Lupus Erythematosus (SLE). However, our patient had evidence of vasculitis on skin biopsy and did not have SLE. Though rare, this is a disease process which must be considered in patients with primary APS which must be closely monitored for other vasculitic complications of APS, particularly diffuse alveolar hemorrhage. PMID:27024977

  19. Interstitial Lung Disease with ANCA-associated Vasculitis

    PubMed Central

    Katsumata, Yasuhiro; Kawaguchi, Yasushi; Yamanaka, Hisashi

    2015-01-01

    The association between interstitial lung disease (ILD) and anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), particularly microscopic polyangiitis (MPA), has been described in a number of case reports and case series reports in the last 2 decades. In addition, patients with pulmonary fibrosis and ANCA positivity but without other manifestations of systemic vasculitis have also been reported. Pulmonary fibrosis was clinically manifested at the time of diagnosis in the majority of AAV patients that developed this condition. Moreover, ANCA-positive conversion occurs in patients initially diagnosed with idiopathic pulmonary fibrosis, and as a result, other manifestations of systemic vasculitis develop in some of these patients. There is significant predominance of myeloperoxidase (MPO)-ANCA and MPA in patients with AAV and ILD. Radiological and pathological findings generally demonstrate usual interstitial pneumonia (pattern) in the lungs of these patients. In most studies, AAV patients with ILD have a worse prognosis than those without it. PMID:26448696

  20. A Case of Brucellosis with Recurrent Attacks of Vasculitis

    PubMed Central

    Korkmaz, Pınar; Kıdır, Mehtap; Namdar, Nazlı Dizen; Özmen, Ahmet; Uyar, Cemile; Değer, Ayşe Nur

    2016-01-01

    Brucellosis is a zoonosis that affects several organs or systems. Skin involvement is nonspecific and it is reported to range between 0,4 and 17% of the patients with brucellosis. Here, we defined a 36-year-old female patient presented to our clinic with a clinical picture of recurrent attacks of vasculitis due to brucellosis for the first time. Skin involvement and vasculitic lesions as a finding of skin involvement are nonspecific in brucellosis. Therefore, in the regions like Turkey where brucellosis is endemic, brucellosis should be kept in mind necessarily in the differential diagnosis of vasculitis. PMID:27042369

  1. Cutaneous necrotizing vasculitis as a manifestation of familial Mediterranean fever.

    PubMed

    Komatsu, Shigetsuna; Honma, Masaru; Igawa, Satomi; Tsuji, Hitomi; Ishida-Yamamoto, Akemi; Migita, Kiyoshi; Ida, Hiroaki; Iizuka, Hajime

    2014-09-01

    Familial Mediterranean Fever (FMF) is a hereditary autoinflammatory disease, which is characterized by recurrent and paroxysmal fever, peritonitis, arthritis, myalgia, and skin rashes. Although various skin lesions such as "erysipelas-like erythema", urticaria, nonspecific purpura, and subcutaneous nodules have been described, cutaneous vasculitis is rare. We report a Japanese case of sporadic FMF accompanied by cutaneous arteritis at the time of febrile attacks of FMF. Gene analysis revealed M694I mutation in a single allele of the MEFV gene, and oral colchicine successfully controlled both periodic fever and subcutaneous nodules of arteritis. Cutaneous necrotizing vasculitis repeatedly emerging with febrile attacks should be included among the skin manifestations of FMF. PMID:25109905

  2. Measurement of damage in systemic vasculitis: a comparison of the Vasculitis Damage Index with the Combined Damage Assessment Index

    PubMed Central

    Suppiah, Ravi; Flossmann, Oliver; Mukhtyar, Chetan; Alberici, Federico; Baslund, Bo; Brown, Denise; Hasan, Nadeem; Holle, Julia; Hruskova, Zdenka; Jayne, David; Judge, Andrew; Little, Mark A; Merkel, Peter A; Palmisano, Alessandra; Seo, Philip; Stegeman, Coen; Tesar, Vladimir; Vaglio, Augusto; Westman, Kerstin; Luqmani, Raashid

    2016-01-01

    Objectives To compare the Vasculitis Damage Index (VDI) with the Combined Damage Assessment Index (CDA) as measures of damage from vasculitis. Methods A total of 283 patients with vasculitis from 11 European centres were evaluated in a cross-sectional study using the VDI and CDA. Results Wegener’s granulomatosis (58.4%) and microscopic polyangiitis (11.0%) were the most common diagnoses. Agreement between VDI and CDA scores (Spearman’s correlation) was 0.90 (95% CI 0.87 to 0.92). There was good correlation between individual comparably evaluated organ systems (Spearman’s correlation 0.70–0.94). Interobserver reliability (assessed by intraclass correlation coefficient (ICC)) was 0.94 (95% CI 0.89 to 0.98) for VDI and 0.78 (95% CI 0.63 to 0.93) for CDA. Intraobserver reliability was 0.92 (95% CI 0.83 to 1.00) for VDI and 0.87 (95% CI 0.75 to 1.00) for CDA. A total of 13 items were not used in the VDI compared to 23 in the CDA. Observers agreed that the CDA covered the full spectrum of damage attributable to vasculitis but was more time consuming and thus possibly less feasible for clinical and research purposes. Conclusions The VDI and CDA capture reliable data on damage among patients with vasculitis. The CDA captures more detail but is more complex and less practical than the VDI. Further evolution of damage assessment in vasculitis is likely to include key elements from both instruments. PMID:20736392

  3. Drug-Induced Vasculitis: New Insights and a Changing Lineup of Suspects.

    PubMed

    Grau, Rafael G

    2015-12-01

    An increasing number of therapeutic agents have been associated with a vasculitic syndrome. This usually involves small vessels, primarily capillaries, venules, and arterioles in leukocytoclastic vasculitis, small-vessel disease similar to an antineutrophil cytoplasmic antibody-related vasculitis, or mid-sized muscular arteries in a polyarteritis-like picture. Antineutrophil cytoplasmic antibodies are present in many cases of vasculitis regardless of the size of the vessel involved. Monoclonal antibodies used to treat many autoimmune disorders have become the most common agents associated with drug-induced vasculitis. Important advances in epigenetics, genetics, and neutrophil apoptosis are providing new insights into the pathogenesis of both drug-induced vasculitis and idiopathic vasculitis. Although management has not changed significantly in the past few years where withdrawal of the offending agent is the primary intervention, increasing awareness of drug-induced vasculitis can lead to earlier diagnosis and prevention of severe organ damage and fatalities. PMID:26503355

  4. Epigenomic functional characterization of genetic susceptibility variants in systemic vasculitis.

    PubMed

    Sawalha, Amr H; Dozmorov, Mikhail G

    2016-02-01

    Systemic vasculitides are poorly understood inflammatory diseases of the blood vessels that are frequently associated with significant organ damage. Genetic risk variants contribute to the susceptibility of vasculitis, but functional consequences of these genetic variants are largely unknown. Most genetic risk variants in immune-mediated diseases, including systemic vasculitis, are localized to non-coding genetic regions suggesting they might increase disease risk by influencing regulatory elements within the genome. Long range regulatory interactions pose an additional obstacle in localizing functional consequences associated with risk variants to specific genes or cell types. We used cell-type specific enrichment patterns of histone changes that mark poised, primed, and active enhancers, and DNase hypersensitivity to identify specific immune cells mediating genetic risk in vasculitis. Our data suggest that genetic risk variants in ANCA-associated vasculitis are significantly enriched in enhancer elements in Th17 cells, supporting a role for Th17 cells in this disease. Primed and active enhancer elements in B cells can be potentially affected by genetic risk variants associated with Kawasaki disease. Genetic risk in Behçet's disease and Takayasu arteritis might affect enhancer elements in multiple cell types, possibly explained by influencing enhancers in hematopoietic stem cells. Interestingly, our analyses indicate a role for B cells in Kawasaki disease, Behçet's disease, and Takayasu arteritis, and suggest that further work to characterize the involvement of B cells in these diseases is warranted. PMID:26492816

  5. Cogan syndrome with severe medium and large vessel vasculitis

    PubMed Central

    Sevgi, Duriye D.; Sobrin, Lucia; Papaliodis, George N.

    2016-01-01

    Summary Cogan syndrome is a rare disease characterized by coexisting audiovestibular and ocular symptoms. Almost half of patients develop systemic manifestations. We report the case of a 38-year-old woman who presented with severe medium and large vessel vasculitis as a systemic manifestation of Cogan syndrome. PMID:27330475

  6. Ten year clinical experience with stroke and cerebral vasculitis.

    PubMed

    Kempster, Peter A; McLean, Catriona A; Phan, Thanh G

    2016-05-01

    Angiitis of the central nervous system (CNS) is difficult to diagnose but potentially fatal. When stroke occurs in a younger individual or is associated with multiple infarcts on imaging, clinicians must decide how far to pursue a possible diagnosis of vasculitis. The aim of this study is to establish the prevalence of primary and secondary cerebral angiitis among patients presenting with stroke. Hospital attendances over a 10year period were surveyed by searching for diagnostic codes and key words specific for cerebral vasculitis/angiitis. Case notes were reviewed by the authors using two sets of criteria for angiitis of the CNS. Thirty-two patients were initially considered likely to have cerebral angiitis by treating physicians. Thirteen had been admitted to hospital with stroke. During this period, there were 7475 admissions for ischaemic and haemorrhagic stroke. Six patients had a final diagnosis of vasculitic stroke but only one had definite CNS angiitis with a first presentation as ischaemic stroke (0.02%). Most patients who did have cerebral vasculitis developed multifocal or subacute neurological deficits, or already had an immunological disorder known to be associated with secondary CNS angiitis. Of 19 patients given an alternative final diagnosis, the most common were atherosclerotic/embolic cerebrovascular disease (n=9) and reversible cerebral vasoconstriction syndrome (n=7). Stroke is rarely the first manifestation of cerebral vasculitis. Our findings suggest that routine screening for angiitis in stroke patients may not be warranted. PMID:26778046

  7. Diagnostic approach and current treatment options in childhood vasculitis

    PubMed Central

    Barut, Kenan; Şahin, Sezgin; Adroviç, Amra; Kasapçopur, Özgür

    2015-01-01

    All inflammatory changes in the vessel wall are defined as vasculitis. Pediatric vasculitis may present with different clinical findings. Although Henoch-Schönlein purpura which is the most common pediatric vasculitis generally recovers spontaneously, it should be monitorized closely because of the risk of renal failure. Although Kawasaki disease is easy to diagnose with its classical findings, the diagnosis may be delayed in case of incomplete Kawasaki disease. Kawasaki disease should be considered especially in infants in case of prolonged fever even if the criteria are not fully met and intravenous immunoglobulin treatment should be administered without delay in order to prevent development of coronary artery aneurism. Reaction at the site of administration of Bacillus Calmette-Guerin (BCG) vaccine may be observed as commonly as cervical lymphadenopathy in Kawasaki disease and may be used as a valuable finding in suspicious cases. Although anti-neutrophil cytoplasmic antibody-associated vasculitides are rare in children, renal involvement is more common and progression is more severe compared to adults. Hence, efficient and aggressive treatment is required. Takayasu’s arteritis is observed commonly in young adult women and rarely in adolescent girls. Therefore, a careful physical examination and blood pressure measurement should be performed in addition to a detailed history in daily practice. In children with unexplained neurological findings, cerebral vasculitis should be considered in the absence of other systemic vasculitides and necessary radiological investigations should be performed in this regard. This review will provide an insight into the understanding of pediatric vasculitis, current diagnostic approaches and prognosis by the aid of new studies. PMID:26884688

  8. Anti-C1q autoantibodies.

    PubMed

    Kallenberg, Cees G M

    2008-09-01

    Autoantibodies to complement components are associated with various diseases. Anti-C1q antibodies are present in all patients with hypocomplementemic urticarial vasculitis, but also, with varying prevalence, in other conditions. In SLE, these antibodies are neither sensitive nor specific for this condition. They occur, however, more frequently in (proliferative) lupus nephritis, particularly during active disease. Furthermore, levels of anti-C1q rise, in many cases, prior to a relapse of lupus nephritis, suggesting a pathogenic role for the autoantibodies. Indeed, experimental studies strongly support a pathogenic role for anti-C1q in immune complex-mediated renal disease. In addition, anti-C1q may interfere with the clearance of apoptotic cells, so influencing induction and expression of autoimmunity. PMID:18606253

  9. Retinal vasculitis: a novel paradoxical effect of anti-TNFα?

    PubMed

    Ben Abdelghani, Kaouther; Slouma, Maroua; Ben Jalel, Wady; Zakraoui, Leith

    2014-01-01

    Retinal vasculitis (RV) is extremely rare in spondyloarthritis associated with Crohn's disease. Infliximab, a chimeric monoclonal antibody to tumour necrosis factor (TNF) α, is efficient in spondyloarthritis, Crohn's disease and RV. We present the case of a 41-year-old man with a known history of spondyloarthritis associated with Crohn's disease. He was under treatment with infliximab. Four days after his 12th infusion of infliximab, he presented with sudden blurred vision. Although his disease was in remission, ophthalmological examination revealed bilateral peripheral retinal occlusive vasculitis. The patient responded positively to the treatment by laser photocoagulation and peribulbar corticosteroid injection. Infliximab was not stopped. There was improvement in his eye disease. To the best of our knowledge, this is the first case of new onset of RV occurring under infliximab in a patient with Crohn's related spondyloarthritis. This case illustrates the possibility of a paradoxical effect of this kind of therapy. PMID:25143312

  10. A Case of Cerebral Vasculitis Associated with Ulcerative Colitis

    PubMed Central

    Raj, Naveen; Arkebauer, Matthew; Waters, Barry; Dickinson, Brucha

    2015-01-01

    Ulcerative colitis (UC) is a chronic, debilitating condition characterized by inflammation of the colonic mucosa. It is regarded as a systemic inflammatory disorder that can affect a number of organ systems. Central nervous system disease associated with UC is a rare sequela of inflammatory bowel disease, occurring in less than 5% of cases. These manifestations include arterial and venous thrombosis, leukoencephalitis, seizures, and vasculitis. We present a case of a 61-year-old female with a two-year history of well-controlled ulcerative colitis, who developed altered mental status and weakness. On brain imaging, she was found to have cerebral lesions which were biopsied. Histopathology subsequently revealed coagulative necrosis and inflammation characteristic of vasculitis. Rheumatology serologies were negative, and the patient was started on steroids that dramatically improved her neurological function, with no residual deficits, and led to resolution of the brain lesions. PMID:26557402

  11. Primary Central Nervous System Vasculitis With Optic Nerve Involvement.

    PubMed

    Benson, Christy E; Knezevic, Alexander; Lynch, Shannon C

    2016-06-01

    A 20-year-old woman presented with headache, decreased vision, eye pain, and urinary retention. During her clinical course, visual acuity declined to 20/800, right eye, and 20/50, left eye, associated with bilateral optic disc edema. Brain magnetic resonance imaging revealed enhancement of the leptomeninges, right optic nerve, and right side of the optic chiasm. Extensive evaluation of the central nervous system (CNS) for an infectious cause was negative. Brain biopsy showed a pattern consistent with vasculitis. The patient was treated with prednisone and cyclophosphamide, resulting in improvement of her vision and systemic symptoms. Primary CNS vasculitis is a rare condition that may affect the anterior visual pathways. PMID:26693942

  12. Dividing the Janus vasculitis? Pathophysiology of eosinophilic granulomatosis with polyangitis.

    PubMed

    Chaigne, Benjamin; Terrier, Benjamin; Thieblemont, Nathalie; Witko-Sarsat, Véronique; Mouthon, Luc

    2016-02-01

    Eosinophilic granulomatosis with polyangitis (EGPA) is a rare small- and medium-sized vessel vasculitis belonging to the group of anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAV). It is commonly divided into two phenotypes depending on the presence of ANCAs targeting myeloperoxidase (MPO). MPO-ANCAs are present in 31% to 38% of patients and are associated with a vasculitis phenotype of the disease, whereas patients without MPO-ANCA are at risk of cardiac involvement. Despite significant advances in understanding the overall pathogenesis of the disease, the explanation for this dichotomy is still unclear. In this review, we synthesize our knowledge of the pathogenesis of EGPA and attempt to i) distinguish EGPA from other diseases including other AAVs, asthma, allergy and hypereosinophilic-associated conditions and ii) speculate about the preponderant mechanisms, which could explain the two disease phenotypes. PMID:26506114

  13. Current Status of Outcome Measure Development in Vasculitis

    PubMed Central

    Merkel, Peter A.; Aydin, Sibel Z.; Boers, Maarten; Cornell, Christina; Direskeneli, Haner; Gebhart, Don; Hatemi, Gulen; Luqmani, Raashid; Matteson, Eric L.; Milman, Nataliya; Robson, Joanna; Seo, Philip; Tomasson, Gunnar

    2015-01-01

    The conduct of randomized controlled trials for vasculitis, especially for the antineutrophil cytoplasmic antibody-associated vasculitides [AAV, granulomatosis with polyangiitis (Wegener’s) and microscopic polyangiitis], has been greatly advanced by the development, use, and acceptance of validated outcome measures. Trials have subsequently provided the opportunity to validate and refine reliable, valid outcome measures for these multisystemic and relapsing rare diseases. The Outcome Measures in Rheumatology (OMERACT) Vasculitis Working Group was formed in 2004 to foster development of validated and widely accepted outcomes in vasculitis using data-driven analyses, a dedication to building consensus, and adherence to, and guidance by, the principles of the OMERACT approach. This work led to the endorsement by OMERACT of the core set of domains and associated outcome measures for AAV. Next steps for the study of existing outcome tools in AAV include better definition of response criteria through development of more data-driven weighting of the elements of activity and damage assessment. The Working Group is now also embarking on a series of linked projects to develop validated patient-reported outcomes for use in clinical research in vasculitis. Additionally, the Working Group is studying how current methods of disease assessment and plans for new outcomes can be informed by the conceptual framework of the International Classification of Function of the World Health Organization. The success of the Group’s work in AAV has also led to a formal process for developing outcomes for the large vessel vasculitides (Takayasu arteritis and giant cell arteritis) and Behçet disease. PMID:24429177

  14. Cutaneous Small Vessel Vasculitis Accompanied by Pustulosis Palmaris et Plantaris

    PubMed Central

    Kosaka, Motoko; Kato, Tokue; Kawana, Seiji

    2012-01-01

    We present the case of a 64-year-old woman who has suffered from pustulosis palmaris et plantaris for 10 years. At the first examination, many erythematous lesions with purpura, blood crusts, and blisters were present in the lower legs and dorsum of the feet. Painful swelling in the sternal region and dorsal pain were also noted. Elevation of the CRP and myogenic enzyme levels, and liver and renal dysfunctions were noted on blood testing. Histopathologically, leukocytoclastic vasculitis was noted in small blood vessels in the whole dermal layers, and deposition of IgM and C3 in the vascular wall was detected by the direct immunofluorescence techniques. Based on these findings, cutaneous small vessel vasculitis was diagnosed. Because the patient complained of a toothache during the clinical course, an X-ray examination was performed. On pantomography, a radicular cyst and apical periodontitis were noted. The tooth symptoms changed with exacerbation and remission of the skin symptoms. These findings indicate that odontogenic infection is very likely to be a cause of cutaneous small vessel vasculitis in a manner similar to pustulosis palmaris et plantaris. PMID:22548039

  15. Monitoring Vasculitis with 18F-FDG PET.

    PubMed

    Bucerius, Jan

    2016-09-01

    Whereas in the past the term "vasculitis" was most frequently used in context with systemic vasculitides, such as the large vessel vasculitides (LVV) Takayasa arteritis and giant cell arteritis, characterized by inflammation of blood vessel walls, it nowadays comprises also inflammatory changes of the vessel wall as a substantial part of the atherosclerotic disease process. Implementing non-invasive imaging techniques, such as computed tomography angiography (CTA), magnetic resonance angiography (MRA) as well as positron emission tomography (PET) in the diagnostic algorithm of atherosclerosis and LVV, depicts a promising step towards an earlier detection with a, consecutively, improved therapeutic approach and potentially prognostic benefit in patients suffering from vasculitis. Mainly molecular imaging with 18F-fluorodeoxyglucose (FDG) PET seems to be promising in offering an early and sensitive identification of inflammatory changes in both, atherosclerosis and LVV. This review will therefore provide an overview on the diagnostic performance and clinical relevance of FDG-PET in monitoring vasculitis in atherosclerosis and LVV, with a focus on LVV. PMID:27280732

  16. Ocular disease in patients with ANCA-positive vasculitis

    PubMed Central

    Watkins, Angela S.; Kempen, John H.; Choi, Dongseok; Liesegang, Teresa L.; Pujari, S. S.; Newcomb, Craig; Nussenblatt, Robert B.; Rosenbaum, James T.; Thorne, Jennifer E.; Foster, C. Stephen; Jabs, Douglas A.; Levy-Clarke, Grace A.; Suhler, Eric B.

    2009-01-01

    Anti-neutrophil cytoplasmic antibody (ANCA)-positive vasculitis—the term recently applied to Wegener's granulomatosis—is a rare multi-system inflammation characterized by necrotizing granulomas and vasculitis. We investigated the ocular manifestations of this disease in a group of patients drawn from five inflammatory eye disease clinics across the United States. Of 8,562 persons with ocular inflammation, 59 individuals were diagnosed with ANCA-positive vasculitis; 35 males and 21 females, aged 16 to 96 years, were included in this study. Ocular diagnoses were scleritis (75.0%), uveitis (17.9%), and other ocular inflammatory conditions (33.9%) including peripheral ulcerative keratitis and orbital pseudotumor. Mean duration of ocular disease was 4.6 years. Oral corticosteroids and other systemic immunosuppressive agents were used by 85.7% and 78.5% of patients, respectively. Over time, patients with ANCA-positive vasculitis experienced 2.75-fold higher mortality than other patients with inflammatory eye disease. PMID:20835396

  17. Challenges in diagnosis of isolated central nervous system vasculitis

    PubMed Central

    Amara, Amy W; Bashir, Khurram; Palmer, Cheryl A; Walker, Harrison C

    2011-01-01

    Isolated central nervous system (CNS) vasculitis is a rare and complicated disorder. Patients typically present with nonspecific neurologic symptoms such as headache and encephalopathy, and have variable progression and severity of the disease. Challenges to definitive diagnosis include the limitations of currently available diagnostic modalities with high likelihood of false-positive or false-negative findings. Imaging, serologic, and cerebrospinal fluid (CSF) evaluation, and even angiography can fail to establish the diagnosis. Often, brain biopsy is required. In order to illustrate these challenges, we report the case of a patient who presented with subacute cognitive decline and was ultimately diagnosed with isolated CNS eosinophilic vasculitis. Initial work-up included CSF and serologic analyses, magnetic resonance imaging (MRI), and cerebral angiography, but definitive diagnosis required brain biopsy. Immunosuppressive therapy resulted in clinical improvement and stabilization. To our knowledge, only one other case of isolated CNS eosinophilic vasculitis has been reported in the literature. We discuss the importance of a high index of clinical suspicion in cases of progressive nonspecific neurologic symptoms. PMID:22398982

  18. A rheumatology perspective on cutaneous vasculitis: assessment and investigation for the non-rheumatologist.

    PubMed

    Rawlings, Charlotte R; Fremlin, Georgina A; Nash, Julian; Harding, Keith

    2016-02-01

    Vasculitis, by definition, is inflammation of the vasculature. This inflammation can result in either vessel wall destruction causing aneurysm or rupture, or stenosis causing ischaemia or necrosis. This autoimmune response does not always have a clear cause. Vasculitis is a heterogeneous group of disorders that has been categorised not only by primary and secondary causes, but also by the size of the affected vessel. The secondary causes that can trigger vasculitis include infection (particularly hepatitis B and C and haemorrhagic fever);cancer, autoimmune diseases such as rheumatoid arthritis (RA), systemic lupus erythematosus (SLE) and Sjogren's; drugs or allergic reactions. As vasculitis can affect any part of the vasculature, it can result in a wide range of signs and symptoms. However, one of the most common presentations of vasculitis is a rash, due to small vessel vasculitis, which is most common. A vasculitic rash has certain characteristics that are easily identifiable and differentiate it from other rashes. This is a review from a rheumatologist's perspective of how to identify vasculitis skin changes. If cutaneous vasculitis is suspected, this article identifies other areas of skin that can be affected that need identification, in addition to what to screen for in the history and other differential diagnoses to consider. Subsequently, the article addresses the key investigations to request and a brief overview of the treatment principles for primary vasculitis. PMID:25818263

  19. Vasculitis as a Presenting Manifestation of Chronic Hepatitis B Virus Infection: A Case Report

    PubMed Central

    Singh, Harpreet; Sukhija, Gagandeep; Kaur, Parminder; Govil, Nikhil

    2016-01-01

    Hepatitis B virus is responsible for causing hepatic complications like acute and chronic hepatitis, cirrhosis and hepatocellular carcinoma along with some uncommon immune mediated extrahepatic manifestations. Vasculitis remains an uncommon extrahepatic complication of hepatitis B virus infection. Herein we report a case of hepatitis B infection that presented with leucocytoclastic vasculitis as an initial manifestation and managed successfully with entacavir therapy. PMID:27042512

  20. Cutaneous leukocytoclastic vasculitis in a child with interleukin-12 receptor beta-1 deficiency.

    PubMed

    Kutukculer, Necil; Genel, Ferah; Aksu, Guzide; Karapinar, Bulent; Ozturk, Can; Cavusoglu, Cengiz; Casanova, Jean-Laurent; Fieschi, Claire

    2006-03-01

    We report a patient with complete interleukin-12 receptor beta-1 deficiency associated with cutaneous leukocytoclastic vasculitis. The patient experienced Bacille Calmette Guérin, Mycobacterium chelonae, and Salmonella enteritidis infection. Vasculitis affecting both small arteries and postcapillary venules due to deposition of immune complexes was probably caused by S. enteritidis and/or M. chelonae infection. PMID:16615980

  1. ANCA-associated vasculitis in Hispanic Americans: an unrecognized severity.

    PubMed

    Sreih, Antoine G; Mandhadi, Ranadeep; Aldaghlawi, Fadi; Khan, Asad; Irshad, Vajiha; Finn, Katherine; Block, Joel A

    2015-05-01

    This study aims to compare the severity and outcomes of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) between Hispanics and Caucasians living in the same geographical area. All patients diagnosed with AAV at two academic institutions in Chicago from January 2006 to December 2012 were retrospectively and prospectively identified. Disease activity was measured with the Birmingham Vasculitis Activity Score (BVAS), and disease damage was measured with the Vasculitis Damage Index (VDI). Student's t test and chi-square tests were employed; p ≤ 0.05 was considered significant. Seventy patients with AAV were identified; 15 patients were excluded. Fifty-five patients were included in the study: 23 Hispanics and 32 Caucasians, 35 patients with granulomatosis with polyangiitis (Wegener's), 12 with microscopic polyangiitis, 7 with eosinophilic granulomatosis with polyangiitis, and 1 with renal-limited vasculitis. Compared to Caucasians, Hispanics had a higher BVAS at presentation (16.3 ± 7.6 versus 10.7 ± 7.5, p = 0.006), a higher VDI at presentation (2.90 ± 1.50 versus 2.06 ± 1.30, p = 0.030), and a cumulative VDI (3.90 ± 1.70 versus 2.50 ± 1.90, p = 0.010). Renal involvement was more common among Hispanics (85 % of Hispanics versus 48 % of Caucasians, p = 0.01). Seventy percent of Hispanics had acute renal failure (mean creatinine = 3.37 ± 4.4 mg/dl) of whom seven (50 %) required dialysis, versus 25 % of Caucasians (mean creatinine = 1.78 ± 1.57 mg/dl, p = 0.03) and only two requiring dialysis. Compared to Caucasians, Hispanics with AAV present with more severe disease and higher damage indices. Larger studies are required to confirm these findings and delineate the respective roles of environment and genetics in the pathogenesis of the disease. PMID:24752347

  2. Etiologies and prognostic factors of leukocytoclastic vasculitis with skin involvement

    PubMed Central

    Bouiller, Kévin; Audia, Sylvain; Devilliers, Hervé; Collet, Evelyne; Aubriot, Marie Hélène; Leguy-Seguin, Vanessa; Berthier, Sabine; Bonniaud, Philippe; Chavanet, Pascal; Besancenot, Jean-François; Vabres, Pierre; Martin, Laurent; Samson, Maxime; Bonnotte, Bernard

    2016-01-01

    Abstract In this study, outcomes of patients with leukocytoclastic vasculitis (LCV) were analyzed focusing on clinical, histopathology and laboratory findings, relapses, and survival. Data from patients with cutaneous vasculitis diagnosed between January 1, 2000, and December 31, 2010, at Dijon University Hospital (France) were retrospectively reviewed. LCV was defined as perivascular neutrophilic infiltrate, endothelial cell nuclear swelling, extravasation of red blood cells, and/or fibrin deposition in vessels. Patients were classified according to the 2012 Chapel Hill Consensus Conference. Relapses were defined as the recurrence of vasculitis symptoms after a period of remission >1 month. Time to relapse and/or death was calculated from the date of diagnosis. Univariate and multivariate (Cox model) analyses were performed. A total of 112 patients (57 males and 55 females), with a mean age of 60 ± 19 (18–98) years, were analyzed. Overall follow-up was 61 ± 38 months. At diagnosis, all patients had skin lesions, purpura being the most common (n = 83). Lesions were associated with systemic involvement in 55 (51%) patients. Only 41 (36.6%) patients received specific treatment: glucocorticoids in 29 of 41 (70.7%) and immunosuppressants in 9 of 41 (22%). Sixty-two patients (55%) had LCV due to underlying causes, 29 (25.9%) had single-organ cutaneous small vessel vasculitis (SoCSVV), and 21 (18.8%) had unclassifiable LCV. Twenty patients of the cohort (18%) experienced relapse, 14 ± 13 (1–40) months after the diagnosis of LCV. None of the 29 patients with SoCSVV relapsed. Independent risk factors for relapse were vascular thrombosis in the biopsy [hazard ratio (HR) = 4.9; P = 0.017], peripheral neuropathy (HR = 9.8; P = 0.001), hepatitis (HR = 3.1; P = 0.004), and positive antineutrophil cytoplasm antibodies (ANCA, HR = 5.9 P = 0.005). In contrast, SoCSVV was a protective factor for relapse (HR = 0.12; P = 0.043). The 1-, 3-, and 6-year overall

  3. Use of positron emission tomography (PET) for the diagnosis of large-vessel vasculitis.

    PubMed

    Loricera, J; Blanco, R; Hernández, J L; Martínez-Rodríguez, I; Carril, J M; Lavado, C; Jiménez, M; González-Vela, C; González-Gay, M Á

    2015-01-01

    The term vasculitis encompasses a heterogeneous group of diseases that share the presence of inflammatory infiltrates in the vascular wall. The diagnosis of large-vessel vasculitis is often a challenge because the presenting clinical features are nonspecific in many cases and they are often shared by different types of autoimmune and inflammatory diseases including other systemic vasculitides. Moreover, the pathogenesis of large-vessel vasculitis is not fully understood. Nevertheless, the advent of new imaging techniques has constituted a major breakthrough to establish an early diagnosis and a promising tool to monitor the follow-up of patients with largevessel vasculitis. This is the case of the molecular imaging with the combination of positron emission tomography with computed tomography (PET/CT) using different radiotracers, especially the (18)F-fluordeoxyglucose ((18)F-FDG). In this review we have focused on the contribution of (18)F-FDG PET in the diagnosis of large-vessel vasculitis. PMID:26272121

  4. Nodular Vasculitis That Developed during Etanercept (Enbrel) Treatment in a Patient with Psoriasis

    PubMed Central

    Park, Seung-Bae; Chang, In-Kyu; Im, Myung; Lee, Young; Kim, Chang-Deok; Seo, Young-Joon

    2015-01-01

    Nodular vasculitis was introduced by Montgomery for cases of erythema induratum-like lesions that were not associated with tuberculosis. Nodular vasculitis has been associated with both nontuberculous infections and noninfectious conditions. However, there has been no report on the development of nodular vasculitis during tumor necrosis factor-α inhibitor treatment. A 28-year-old man visited our clinic for the treatment of severe psoriasis with a 20-year history. Subcutaneous injection of etanercept (25 mg, twice weekly) was started. One year later, erythematous nodules developed on his lower leg. A skin biopsy showed lobular panniculitis with extensive necrosis and vasculitis. To exclude latent tuberculosis, an assay specific for Mycobacterium tuberculosis antigens was performed, with a negative result. After stopping etanercept under the diagnosis of nodular vasculitis associated with etanercept, the lesions gradually disappeared, leaving depressed scars in 3 months. There has been no recurrence after 6 months of follow-up. PMID:26512176

  5. Pauci-Immune Crescentic Glomerulonephritis: An ANCA-Associated Vasculitis

    PubMed Central

    Syed, Rafeel; Rehman, Amina; Valecha, Gautam; El-Sayegh, Suzanne

    2015-01-01

    Rapidly progressive glomerulonephritis (RPGN) is a syndrome signified by a precipitous loss of renal function, with features of glomerulonephritis including dysmorphic erythrocyturia and glomerular proteinuria. RPGN is associated with extensive crescent formation, and, thus, the clinical term RPGN is often used interchangeably with the pathologic term crescentic glomerulonephritis (CGN). From an immunopathologic standpoint, primary RPGN is divided into pauci-immune GN (PICG), anti-GBM GN, and immune complex GN. PICG, the most common etiology of primary RPGN, refers to a necrotizing glomerulonephritis with few or no immune deposits by immunofluorescence (IF) or electron microscopy (EM). In most patients, pauci-immune CGN is a component of a systemic small vessel vasculitis such as granulomatosis with polyangiitis (GPA). Approximately 90% of patients with PICG have circulating ANCA antibodies, leading to the nomenclature ANCA-associated vasculitis (AAV). Recent research has identified several other antibodies associated with PICG, which is now understood to be a complex spectrum of disease with considerable overlap in terms of clinical phenotype and outcomes. In addition, several genetic and environmental factors have recently been implicated in the pathogenesis of this disorder. With new prognostic classifications, enhanced understanding of immunopathologic mechanisms, and novel treatment paradigms, clinical and experimental interest in PICG remains high. PMID:26688808

  6. Pathogenesis of ANCA-associated vasculitis: An update.

    PubMed

    Jarrot, Pierre-André; Kaplanski, Gilles

    2016-07-01

    Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) constitutes a group of rare diseases characterized by necrotizing inflammation of small blood vessels and the presence of ANCA. Although these autoantibodies were initially used to classify pauci-immune vasculitis, increasing clinical and experimental evidence now supports their pathogenic role, mainly through ANCA-induced activation of primed neutrophils and monocytes leading to destructive vascular necrosis. The mechanisms of ANCA generation remain however unclear. Neutrophils play a central role in the pathophysiological process of AAV since they are both effector cells responsible for endothelial damage and targets of autoimmunity. Another role of neutrophils is due to their ability to generate neutrophil extracellular traps, which support the presentation of ANCA autoantigens, could break immune tolerance and induce autoantibody generation. Alternatively, the ANCA autoimmune response is facilitated by insufficient T-cell and B-cell regulation, and the role of complement alternative pathway has recently been emphasized. This review summarizes the main pathogenesis concepts of AAV as well as the putative mechanisms for the origin of ANCA autoimmune response. PMID:26970490

  7. T Cell–Macrophage Interactions and Granuloma Formation in Vasculitis

    PubMed Central

    Hilhorst, Marc; Shirai, Tsuyoshi; Berry, Gerald; Goronzy, Jörg J.; Weyand, Cornelia M.

    2014-01-01

    Granuloma formation, bringing into close proximity highly activated macrophages and T cells, is a typical event in inflammatory blood vessel diseases, and is noted in the name of several of the vasculitides. It is not known whether specific properties of the microenvironment in the blood vessel wall or the immediate surroundings of blood vessels contribute to granuloma formation and, in some cases, generation of multinucleated giant cells. Granulomas provide a specialized niche to optimize macrophage–T cell interactions, strongly activating both cell types. This is mirrored by the intensity of the systemic inflammation encountered in patients with vasculitis, often presenting with malaise, weight loss, fever, and strongly upregulated acute phase responses. As a sophisticated and highly organized structure, granulomas can serve as an ideal site to induce differentiation and maturation of T cells. The granulomas possibly seed aberrant Th1 and Th17 cells into the circulation, which are known to be the main pathogenic cells in vasculitis. Through the induction of memory T cells, aberrant innate immune responses can imprint the host immune system for decades to come and promote chronicity of the disease process. Improved understanding of T cell–macrophage interactions will redefine pathogenic models in the vasculitides and provide new avenues for immunomodulatory therapy. PMID:25309534

  8. Posterior Spinal Artery Aneurysm Presenting with Leukocytoclastic Vasculitis

    PubMed Central

    Tanweer, Omar; Thomas, Cheddhi; Engler, John; Shapiro, Maksim; Becske, Tibor

    2016-01-01

    Rupture of isolated posterior spinal artery (PSA) aneurysms is a rare cause of subarachnoid hemorrhage (SAH) that presents unique diagnostic challenges owing to a nuanced clinical presentation. Here, we report on the diagnosis and management of the first known case of an isolated PSA aneurysm in the context of leukocytoclastic vasculitis. A 53-year-old male presented to an outside institution with acute bilateral lower extremity paralysis 9 days after admission for recurrent cellulitis. Early magnetic resonance imaging was read as negative and repeat imaging 15 days after presentation revealed SAH and a compressive spinal subdural hematoma. Angiography identified a PSA aneurysm at T9, as well as other areas suspicious for inflammatory or post-hemorrhagic reactive changes. The patient underwent a multilevel laminectomy for clot evacuation and aneurysm resection to prevent future hemorrhage and to establish a diagnosis. The postoperative course was complicated by medical issues and led to the diagnosis of leukocytoclastic vasculitis that may have predisposed the patient to aneurysm development. Literature review reveals greater mortality for cervical lesions than thoracolumbar lesions and that the presence of meningitic symptoms portents better functional outcome than symptoms of cord compression. The outcome obtained in this case is consistent with outcomes reported in the literature. PMID:27114966

  9. Rapid regression of exudative maculopathy in idiopathic retinitis, vasculitis, aneurysms and neuroretinitis syndrome after intravitreal ranibizumab.

    PubMed

    Marín-Lambíes, Cristina; Gallego-Pinazo, Roberto; Salom, David; Navarrete, Javier; Díaz-Llopis, Manuel

    2012-05-01

    The idiopathic retinitis, vasculitis, aneurysms and neuroretinitis syndrome is a rare retinal vascular disorder characterized by multiple leaking aneurysmal dilations, retinal vasculitis, neuroretinitis and peripheral vascular ischemia. Visual loss mainly occurs due to the development of retinal neovascularization and/or exudative maculopathy. Although the treatment of choice has not yet been established, retinal photocoagulation seems to be the best option to control the disease and to prevent its progression. Herein, we report a case of idiopathic retinitis, vasculitis, aneurysms and neuroretinitis syndrome with both retinal neovascularization and macular exudation successfully managed with intravitreal ranibizumab (Lucentis(®)) as adjunctive therapy to retinal photocoagulation. PMID:22949913

  10. Focal cerebral vasculitis associated with circulating immune complexes and brain irradiation

    SciTech Connect

    Groothuis, D.R.; Mikhael, M.A.

    1986-06-01

    In this report we describe a patient with a benign glioma treated with surgery and radiation. After a period of stability he developed subacute bacterial endocarditis, and deteriorated neurologically. Computed tomographic scans did not show recurrent tumor. An angiogram showed vasculitis restricted to the previously irradiated area. Secondary to subacute bacterial endocarditis was the presence of high levels of circulating immune complexes. His neurological status was unchanged after antibiotics, but improved after treatment with dexamethasone. We interpret the clinical course as an immune-complex-mediated vasculitis superimposed on a subclinical radiation vasculitis. This case supports the hypothesis that immune mechanisms may be involved in delayed radiation injury to the nervous system.

  11. Disseminated gonococcal infection presenting as vasculitis: a case report.

    PubMed

    Jain, Sangita; Win, Htet Nwe; Chalam, Venkat; Yee, Lian

    2007-01-01

    A 50-year-old man with alcoholic liver disease presented with fever, tenosynovitis, polyarthritis and a vasculitic rash on the hands and feet for 4 days. He had neutrophilia and raised inflammatory markers. He had no history of sore throat, urethral discharge or travel abroad. His initial blood cultures were negative, and he was treated for vasculitis with steroids. The rash and arthritis seemed to improve initially, but he had another episode of fever. Repeat blood cultures grew Neisseria gonorrhoeae,and he received intravenous ceftriaxone followed by oral ciprofloxacin. He had marked improvement in rash, tenosynovitis and arthritis, and the fever dropped. He also had chlamydial urethritis and received azithromycin. The presentation of disseminated gonococcal infection after a presumptive episode of asymptomatic urethral gonorrhoea is highlighted. PMID:17213353

  12. Neutrophilic Dermatoses in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis

    PubMed Central

    de Boysson, Hubert; Martin Silva, Nicolas; de Moreuil, Claire; Néel, Antoine; de Menthon, Mathilde; Meyer, Olivier; Launay, David; Pagnoux, Christian; Guillevin, Loïc; Puéchal, Xavier; Bienvenu, Boris; Aouba, Achille

    2016-01-01

    Abstract A few reports suggest combination of ANCA-associated vasculitis (AAV) and neutrophilic dermatoses (ND). We aimed to describe the main characteristics of patients presenting with both AAV and ND in a French cohort and through a systematic literature review, and to discuss the possible common pathogenic process involved. We conducted a retrospective study of patients with both conditions. Patients were selected via the French Internal Medicine Society (SNFMI) and the French Vasculitis Study Group (FVSG). A literature review focusing on a combination of both conditions, concentrated only on publications with well-established diagnoses and individual detailed data. Seventeen patients diagnosed with AAV and ND were identified in this cohort. Twelve patients had granulomatosis with polyangiitis (GPA), 4 had microscopic polyangiitis (MPA) and one had eosinophilic GPA (EGPA). Eight patients, all with GPA, displayed pyoderma gangrenosum (PG). Sweet's syndrome was observed in 6 patients (4 with MPA, one with GPA and one with EGPA) and erythema elevatum diutinum in the other three (2 with GPA and 1 with MPA). The literature review identified 33 additional patients with both conditions, including 26 with GPA. Altogether, of the 50 patients (17 from our study and 33 from the literature review), 33 (66%) patients presented with PG associated with GPA in 29 cases (89%). Corticosteroids were the first-line treatment in conjunction with an immunosuppressive agent in most cases. Outcomes were good and a total of 15 patients experienced a relapse. Patients who relapsed were more likely to have ear, nose and throat manifestation than patients who did not [12/15 (80%) relapsing patients vs. 15/35 (43%) non-relapsing patients; p = 0.03)]. In our stud, the most frequent association concerned GPA and PG. ND should be considered and specifically researched within the spectrum of cutaneous manifestations observed in AAV. PMID:26986103

  13. Rituximab versus Cyclophosphamide for ANCA-Associated Vasculitis

    PubMed Central

    Stone, John H.; Merkel, Peter A.; Spiera, Robert; Seo, Philip; Langford, Carol A.; Hoffman, Gary S.; Kallenberg, Cees G.M.; St. Clair, E. William; Turkiewicz, Anthony; Tchao, Nadia K.; Webber, Lisa; Ding, Linna; Sejismundo, Lourdes P.; Mieras, Kathleen; Weitzenkamp, David; Ikle, David; Seyfert-Margolis, Vicki; Mueller, Mark; Brunetta, Paul; Allen, Nancy B.; Fervenza, Fernando C.; Geetha, Duvuru; Keogh, Karina A.; Kissin, Eugene Y.; Monach, Paul A.; Peikert, Tobias; Stegeman, Coen; Ytterberg, Steven R.; Specks, Ulrich

    2011-01-01

    BACKGROUND Cyclophosphamide and glucocorticoids have been the cornerstone of remission-induction therapy for severe antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis for 40 years. Uncontrolled studies suggest that rituximab is effective and may be safer than a cyclophosphamide-based regimen. METHODS We conducted a multicenter, randomized, double-blind, double-dummy, noninferiority trial of rituximab (375 mg per square meter of body-surface area per week for 4 weeks) as compared with cyclophosphamide (2 mg per kilogram of body weight per day) for remission induction. Glucocorticoids were tapered off; the primary end point was remission of disease without the use of prednisone at 6 months. RESULTS Nine centers enrolled 197 ANCA-positive patients with either Wegener’s granulomatosis or microscopic polyangiitis. Baseline disease activity, organ involvement, and the proportion of patients with relapsing disease were similar in the two treatment groups. Sixty-three patients in the rituximab group (64%) reached the primary end point, as compared with 52 patients in the control group (53%), a result that met the criterion for noninferiority (P<0.001). The rituximab-based regimen was more efficacious than the cyclophosphamide-based regimen for inducing remission of relapsing disease; 34 of 51 patients in the rituximab group (67%) as compared with 21 of 50 patients in the control group (42%) reached the primary end point (P = 0.01). Rituximab was also as effective as cyclophosphamide in the treatment of patients with major renal disease or alveolar hemorrhage. There were no significant differences between the treatment groups with respect to rates of adverse events. CONCLUSIONS Rituximab therapy was not inferior to daily cyclophosphamide treatment for induction of remission in severe ANCA-associated vasculitis and may be superior in relapsing disease. (Funded by the National Institutes of Allergy and Infectious Diseases, Genentech, and Biogen; Clinical

  14. Vasculitis in patients with inflammatory bowel diseases: A study of 32 patients and systematic review of the literature

    PubMed Central

    Sy, Alice; Khalidi, Nader; Dehghan, Natasha; Barra, Lillian; Carette, Simon; Cuthbertson, David; Hoffman, Gary S.; Koening, Curry L.; Langford, Carol A.; McAlear, Carol; Moreland, Larry; Monach, Paul A.; Seo, Philip; Specks, Ulrich; Sreih, Antoine; Ytterberg, Steven R.; Van Assche, Gert; Merkel, Peter A.; Pagnoux, Christian

    2016-01-01

    Background Published small case series suggest that inflammatory bowel disease [IBD; Crohn’s disease (CD) or ulcerative colitis (UC)] and vasculitis co-occur more frequently than would be expected by chance. Objectives To describe this association by an analysis of a large cohort of carefully studied patients and through a systematic literature review. Methods Patients with both IBD and vasculitis enrolled in the Vasculitis Clinical Research Consortium (VCRC) Longitudinal Studies, followed in Canadian Vasculitis research network (CanVasc) centers and/or in the University of Toronto’s IBD clinic were included in this case series. A systematic literature review of patients with IBD and vasculitis involved a PubMed search through February 2014. The main characteristics of patients with Takayasu arteritis (TAK) and IBD were compared to those in patients with TAK without IBD followed in the VCRC. Results The study identified 32 patients with IBD and vasculitis: 13 with large-vessel vasculitis [LVV; 12 with TAK, 1 with giant cell arteritis (GCA); 8 with CD, 5 with UC]; 8 with ANCA-associated vasculitis [AAV; 6 granulomatosis with polyangiitis (GPA), 2 with eosinophilic granulomatosis with polyangiitis (EGPA)]; 5 with isolated cutaneous vasculitis; and 6 with other vasculitides. Patients with LVV and AAV were mostly female (18/21). The diagnosis of IBD preceded that of vasculitis in 12/13 patients with LVV and 8/8 patients with AAV. The review of the literature identified 306 patients with IBD and vasculitis: 144 with LVV (133 TAK; 87 with IBD preceding LVV), 19 with AAV [14 GPA, 1 EGPA, 4 microscopic polyangiitis (MPA)], 66 with isolated cutaneous vasculitis, and 77 with other vasculitides. Patients with IBD and TAK were younger and had more frequent headaches, constitutional symptoms, or gastrointestinal symptoms compared to those patients in the VCRC who had TAK without IBD. Conclusions These findings highlight the risk of vasculitis, especially TAK, in patients

  15. Cerebral angiography as a guide for therapy in isolated central nervous system vasculitis

    SciTech Connect

    Stein, R.L.; Martino, C.R.; Weinert, D.M.; Hueftle, M.; Kammer, G.M.

    1987-04-24

    The authors present a case of isolated central nervous system vasculitis documented by cerebral arteriography in which remission, using a treatment regimen of prednisone and cyclophosphamide, was guided by serial arteriography during a 15-month period.

  16. Vasculitis in systemic sclerosis: association with Sjögren's syndrome and the CREST syndrome variant.

    PubMed

    Oddis, C V; Eisenbeis, C H; Reidbord, H E; Steen, V D; Medsger, T A

    1987-10-01

    We describe 7 patients with established systemic sclerosis who developed clinical evidence of vasculitis 1 to 33 (mean 12.7) years after the first symptoms of scleroderma. Six had the CREST variant of systemic sclerosis and also had features of Sjögren's syndrome (SS). Five of 6 patients tested had serum anti-SSA (Ro) antibodies. Vasculitis presented primarily as cutaneous lesions with ulceration and/or mononeuritis multiplex, and 6 patients had severe systemic manifestations. Vasculitis was histopathologically documented in 6 cases in biopsies of skin (4 of 4), muscle (2 of 3) and sural nerve (3 of 3). Patients with systemic sclerosis with CREST syndrome and SS appear to be at increased risk to develop vasculitis. PMID:3430523

  17. IL-17 production by CSF lymphocytes as a biomarker for cerebral vasculitis

    PubMed Central

    Thom, Vivien; Schmid, Sabrina; Gelderblom, Mathias; Hackbusch, Romy; Kolster, Manuela; Schuster, Simon; Thomalla, Götz; Keminer, Oliver; Pleß, Ole; Bernreuther, Christian; Glatzel, Markus; Wegscheider, Karl; Gerloff, Christian

    2016-01-01

    Objective: To explore the possibility of using interleukin-17 (IL-17) production by CD4+ T cells in the CSF as a potential biomarker for cerebral vasculitis in stroke patients. Methods: In this consecutive case study, we performed prospective analysis of CSF and blood in patients admitted to a university medical center with symptoms of stroke and suspected cerebral vasculitis. Flow cytometry was performed for intracellular detection of inflammatory cytokines in peripheral blood lymphocytes and expanded T cells from CSF. Results: CSF CD4+ lymphocytes from patients with cerebral vasculitis showed significantly higher levels of the proinflammatory cytokine IL-17 compared to patients with stroke not due to vasculitis or with other, noninflammatory neurologic diseases. There was no difference in the production of interferon-γ in the CSF and no overall differences in the relative frequencies of peripheral immune cells. Conclusions: Intracellular IL-17 in CSF cells is potentially useful in discriminating cerebral vasculitis as a rare cause in patients presenting with ischemic stroke. Classification of evidence: This study provides Class II evidence that an increased proportion of IL-17-producing CD4+ cells in CSF of patients presenting with stroke symptoms is indicative of cerebral vasculitis (sensitivity 73%, 95% confidence interval [CI] 39–94%; specificity 100%, 95% CI 74%–100%). PMID:27144213

  18. Minocycline-induced polyarteritis nodosa-like vasculitis presenting as brainstem stroke.

    PubMed

    Klaas, James P; Matzke, Thomas; Makol, Ashima; Fulgham, Jimmy R

    2015-05-01

    Minocycline use has been associated with the development of autoimmune disorders, including drug-induced vasculitis. Previously published reports suggest that clinical manifestations are limited to cutaneous, constitutional, or musculoskeletal symptoms. To our knowledge there has been only one reported patient with ischemic stroke in the setting of minocycline-induced vasculitis. We describe a 26-year-old woman, with no vascular risk factors, who had an ischemic pontine stroke in the setting of biopsy-proven minocycline-induced polyarteritis nodosa-like vasculitis. Discontinuation of minocycline resulted in resolution of the vasculitis, and she has not had any recurrent ischemic events. This report shows that ischemic strokes may occur as a result of minocycline-induced vasculitis. While this is likely a rare association, recognition is important given the widespread use of minocycline and the potential for devastating consequences in a young population. Consequently, drug-induced vasculitis should be considered in patients with an ischemic stroke taking minocycline. PMID:25778384

  19. D-penicillamine-induced ANA (+) ANCA (+) vasculitis in pediatric patients with Wilson's disease.

    PubMed

    Lee, Yeonhee; Lee, Sang Taek; Cho, Heeyeon

    2016-05-01

    Anti-neutrophil cytoplasmic antibodies (ANCA) are associated with systemic vasculitis. The pathophysiology of ANCA-associated vasculitis (AAV) has not been clearly proven, and drug-induced ANCA-associated vasculitis has been reported. Wilson's disease is an inborn error of copper metabolism caused by a mutation in the copper transporting gene ATP7B, and traditional treatment is based on copper chelation with agents such as D-penicillamine. There have been rare reports that prolonged D-penicillamine therapy might cause adverse renal events such as membranous nephropathy and minimal change disease, but it is questionable if D-penicillamine induces ANCA-associated vasculitis. We describe 2 patients with Wilson's disease treated with D-penicillamine who presented with ANCA (+) vasculitis and renal involvement. The 2 patients also showed positive results for antinuclear antibody (ANA). Their kidney biopsy findings were compatible with crescentic/necrotizing glomerulonephritis, pauci-immune type. After diagnosis of AAV, D-penicillamine was stopped. Patients were then treated with plasmapheresis and immunosuppressants, including methylprednisolone pulse therapy and intravenous cyclophosphamide. One patient progressed to end-stage renal disease and the other showed persistent proteinuria. These cases suggest that D-penicillamine may induce ANA (+) ANCA (+) vasculitis with severe renal involvement in pediatric patients, and plasmapheresis combined with immunosuppressant should be considered. PMID:26784915

  20. [Pathophysiology and Laboratory Findings in Patients with ANCA-Associated Vasculitis].

    PubMed

    Wada, Takashi

    2015-10-01

    Pathophysiological features and laboratory findings in patients with anti-neutrophil cytoplasmic antibody (ANCA) -associated vasculitis are described. In contrast to Western countries, MPO-ANCA-positive microscopic polyangiitis and renal-limited vasculitis is the most common form of ANCA-associated vasculitis in Japanese patients. Oral prednisolone in combination with immunosuppressive agents has improved patient survival in older Japanese patients. However, the recurrence rate has significantly increased in recent years Accumulative evidence supports a direct pathogenic role of ANCA in glomerulonephritis and vasculitis, in which inflammatory processes with the up-regulation of cytokines/chemokines and possible involvement of a neutrophil extracellular trap may play a role. However, whether or not the measurement of ANCA titers reflects disease activity and predicts the onset and/or relapse of ANCA-associated vasculitis remains controversial. Further studies will be required to determine the clinical significance of ANCA in more detail. In addition to ANCA, measurements of novel clinical test items, including anti-erythropoietin receptor antibody, may reveal the possibility of their application as useful biomarkers of the pathophysiology and clinical manifestations in patients with ANCA-associated vasculitis. PMID:26897859

  1. Leukocytoclastic vasculitis in an adolescent with ulcerative colitis: Report of a case and review of the literature

    PubMed Central

    Butts, G Tyler; Bishop, Phyllis R; Wyatt, Julie P

    2014-01-01

    An adolescent female with long-standing, difficult-to-control ulcerative colitis developed leukocytoclastic vasculitis, a rare cutaneous extra-intestinal manifestation of the inflammatory bowel disease. The authors provide a literature review on leukocytoclastic vasculitis complicating ulcerative colitis. Furthermore, the clinical features of leukocytoclastic vasculitis are compared and contrasted with the more common cutaneous extra-intestinal manifestations of inflammatory bowel disease, erythema nodosum, and pyoderma gangrenosum.

  2. Pulmonary Fibrosis in Antineutrophil Cytoplasmic Antibodies (ANCA)-Associated Vasculitis

    PubMed Central

    Comarmond, Cloé; Crestani, Bruno; Tazi, Abdellatif; Hervier, Baptiste; Adam-Marchand, Sylvain; Nunes, Hilario; Cohen-Aubart, Fleur; Wislez, Marie; Cadranel, Jacques; Housset, Bruno; Lloret-Linares, Célia; Sève, Pascal; Pagnoux, Christian; Abad, Sébastien; Camuset, Juliette; Bienvenu, Boris; Duruisseaux, Michaël; Hachulla, Eric; Arlet, Jean-Benoît; Hamidou, Mohammed; Mahr, Alfred; Resche-Rigon, Matthieu; Brun, Anne-Laure; Grenier, Philippe; Cacoub, Patrice; Saadoun, David

    2014-01-01

    Abstract Pulmonary fibrosis (PF) is an uncommon manifestation observed in patients with antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV), particularly microscopic polyangiitis (MPA). While patients with PF associated with AAV seem to have a worse prognosis, these patients have been described only in case reports or small retrospective case series. In this retrospective multicenter study, we report the main features and long-term outcomes of patients with PF associated with AAV, fulfilling the American College of Rheumatology criteria and/or Chapel Hill definitions. Forty-nine patients (30 men [61%]; median age at diagnosis of AAV, 68 [interquartile range, 58–73] years) with PF associated with AAV were identified. Forty (81.6%) patients had MPA and 9 (18.4%) had granulomatosis with polyangiitis. The diagnosis of PF preceded the onset of vasculitis in 22 (45%) patients. Usual interstitial pneumonia was the main radiologic pattern (n = 18, 43%). ANCA were mostly of antimyeloperoxidase specificity (88%). All patients were treated with glucocorticoids as induction therapy, combined with cyclophosphamide (CYC) (n = 36, 73.5%) or rituximab (RTX) (n = 1, 2%). Factors associated with mortality included occurrence of chronic respiratory insufficiency (hazard ratio [HR], 7.44; 95% confidence interval [CI], 1.6–34.5; p = 0.003), induction therapy with glucocorticoids alone (HR, 2.94; CI, 1.05–8.33; p = 0.04), and initial weigh loss (HR, 2.83; CI, 1.05–7.65; p = 0.041). The 3-year survival rate in patients treated with glucocorticoids alone or combined with an immunosuppressant (CYC or RTX) as induction therapy was 64% (95% CI, 41–99) and 94% (95% CI, 86–100), respectively (p = 0.03). After a median follow-up of 48 months [interquartile range, 14–88 mo], 18 (37%) patients died, including 11 related to respiratory insufficiency. PF is a rare manifestation of AAV with a very poor prognosis. Induction therapy with

  3. Neutrophil Extracellular Traps in ANCA-Associated Vasculitis

    PubMed Central

    Söderberg, Daniel; Segelmark, Mårten

    2016-01-01

    A group of pauci-immune vasculitides, characterized by neutrophil-rich necrotizing inflammation of small vessels and the presence of antineutrophil cytoplasmic antibodies (ANCAs), is referred to as ANCA-associated vasculitis (AAV). ANCAs against proteinase 3 (PR3) (PR3-ANCA) or myeloperoxidase (MPO) (MPO-ANCA) are found in over 90% of patients with active disease, and these ANCAs are implicated in the pathogenesis of AAV. Dying neutrophils surrounding the walls of small vessels are a histological hallmark of AAV. Traditionally, it has been assumed that these neutrophils die by necrosis, but neutrophil extracellular traps (NETs) have recently been visualized at the sites of vasculitic lesions. AAV patients also possess elevated levels of NETs in the circulation. ANCAs are capable of inducing NETosis in neutrophils, and their potential to do so has been shown to be affinity dependent and to correlate with disease activity. Neutrophils from AAV patients are also more prone to release NETs spontaneously than neutrophils from healthy blood donors. NETs contain proinflammatory proteins and are thought to contribute to vessel inflammation directly by damaging endothelial cells and by activating the complement system and indirectly by acting as a link between the innate and adaptive immune system through the generation of PR3- and MPO-ANCA. Injection of NET-loaded myeloid dendritic cells into mice results in circulating PR3- and MPO-ANCA and the development of AAV-like disease. NETs have also been shown to be essential in a rodent model of drug-induced vasculitis. NETs induced by propylthiouracil could not be degraded by DNaseI, implying that disordered NETs might be important for the generation of ANCAs. NET degradation was also highlighted in another study showing that AAV patients have reduced DNaseI activity resulting in less NET degradation. With this in mind, it might be that prolonged exposure to proteins in the NETs due to the overproduction of NETs and/or reduced

  4. Neutrophil Extracellular Traps in ANCA-Associated Vasculitis.

    PubMed

    Söderberg, Daniel; Segelmark, Mårten

    2016-01-01

    A group of pauci-immune vasculitides, characterized by neutrophil-rich necrotizing inflammation of small vessels and the presence of antineutrophil cytoplasmic antibodies (ANCAs), is referred to as ANCA-associated vasculitis (AAV). ANCAs against proteinase 3 (PR3) (PR3-ANCA) or myeloperoxidase (MPO) (MPO-ANCA) are found in over 90% of patients with active disease, and these ANCAs are implicated in the pathogenesis of AAV. Dying neutrophils surrounding the walls of small vessels are a histological hallmark of AAV. Traditionally, it has been assumed that these neutrophils die by necrosis, but neutrophil extracellular traps (NETs) have recently been visualized at the sites of vasculitic lesions. AAV patients also possess elevated levels of NETs in the circulation. ANCAs are capable of inducing NETosis in neutrophils, and their potential to do so has been shown to be affinity dependent and to correlate with disease activity. Neutrophils from AAV patients are also more prone to release NETs spontaneously than neutrophils from healthy blood donors. NETs contain proinflammatory proteins and are thought to contribute to vessel inflammation directly by damaging endothelial cells and by activating the complement system and indirectly by acting as a link between the innate and adaptive immune system through the generation of PR3- and MPO-ANCA. Injection of NET-loaded myeloid dendritic cells into mice results in circulating PR3- and MPO-ANCA and the development of AAV-like disease. NETs have also been shown to be essential in a rodent model of drug-induced vasculitis. NETs induced by propylthiouracil could not be degraded by DNaseI, implying that disordered NETs might be important for the generation of ANCAs. NET degradation was also highlighted in another study showing that AAV patients have reduced DNaseI activity resulting in less NET degradation. With this in mind, it might be that prolonged exposure to proteins in the NETs due to the overproduction of NETs and/or reduced

  5. Antineutrophil Cytoplasmic Autoantibody Associated Systemic Vasculitis Is Associated with Epstein - Barr virus in the Setting of HIV Infection

    PubMed Central

    Mirsaeidi, Mehdi; Syed, Fatima; Jaffe, Elaine S.

    2013-01-01

    Introduction EBV has been a leading candidate as a trigger for several autoimmune diseases. We describe an antineutrophil cytoplasmic autoantibody (ANCA) -associated systemic vasculitis as the initial presenting illness of AIDS. Case report and results The patient was diagnosed ANCA -associated systemic vasculitis in the setting of HIV infection because of a high level of ANCA level, crescent glomerulonephritis in pathology, and clinical signs and symptoms compatible with systemic vasculitis. He also had HIV associated lymphadenopathy with scattered. EBV-RNA positive cells and reactive germinal centers. Conclusion EBV genome was found in reactive lymph nodes and, therefore, may be associated with the immunopathogenesis of vasculitis. PMID:23483275

  6. Histologic and clinical features of primary and secondary vasculitis: a retrospective study of 42 dogs (2004-2011).

    PubMed

    Swann, James W; Priestnall, Simon L; Dawson, Charlotte; Chang, Yu-Mei; Garden, Oliver A

    2015-07-01

    Inflammation of the blood vessel wall has been reported infrequently in dogs, and it may occur without apparent cause (primary vasculitis) or as a pathologic reaction to a range of initiating insults (secondary vasculitis). The aims of our study were to report histologic, clinical, and survival data from a large series of cases with primary and secondary vasculitis, and to compare the clinical parameters and outcome data between groups. Clinical data was collected retrospectively from the medical records of 42 client-owned dogs with a histologic diagnosis of primary or secondary vasculitis, and follow-up information was obtained. Cases were grouped according to clinical and histologic descriptors, and biochemical, hematologic, and survival data was compared between groups. Several forms of primary vasculitis were observed, and vascular inflammation was observed in conjunction with numerous other diseases. Female dogs were more likely to develop primary vasculitis, and serum globulin concentration was greater in dogs with primary vasculitis compared to those with underlying disease. All dogs with primary vasculitis of the central nervous system died or were euthanized shortly after presentation, but other forms of primary vasculitis could be managed effectively. In conclusion, presentation of clinical cases in this series was variable, and there did not appear to be well-defined vasculitic syndromes as described in people. PMID:26077546

  7. Diffuse endothelial dysfunction is common to ANCA associated systemic vasculitis and polyarteritis nodosa

    PubMed Central

    Filer, A; Gardner-Medwin, J; Thambyrajah, J; Raza, K; Carruthers, D; Stevens, R; Liu, L; Lowe, S; Townend, J; Bacon, P

    2003-01-01

    Background: Excess cardiovascular mortality complicates systemic rheumatic disease, suggesting an accelerated atheromatous process, which it has been proposed relates to the vascular inflammation common in such diseases. Impaired endothelium dependent vasodilatation is an early marker of atheromatous disease. It has previously been shown that such endothelial cell dysfunction (ECD) occurring in the brachial artery can complicate primary systemic necrotising vasculitis (SNV). Objective: To determine if ECD occurs in a wider spectrum of primary SNV, if it is restricted to the major arteries, and whether vasculitis subgroup, ANCA status, or renal involvement influenced the endothelial responses. Methods: Fifty four patients attending the Birmingham vasculitis clinic, including patients with a range of ANCA and non-ANCA associated primary vasculitides, and a group of age matched controls were recruited. The length of patient follow up and disease activity was variable. Disease activity, damage scores, and cardiovascular risk factors were recorded before assessment of flow mediated brachial artery vasodilatation by high resolution ultrasound. Dermal microvascular responses to acetylcholine were also measured in 32 patients and 21 controls by laser Doppler flowmetry. Results: ECD was demonstrated in all primary SNV subgroups of patients with ANCA associated vasculitis and in polyarteritis nodosa, compared with controls. Significant impairment occurred in both vascular beds, regardless of vessel size targeted in the inflammatory vasculitis, ANCA association and titre, or renal involvement. Conclusions: Diffuse endothelial dysfunction, a predictor of atherosclerotic disease, is found extensively in primary systemic vasculitis. Involvement of different vascular beds is independent of target vessel size or ANCA association, and is unrelated to local disease expression. It is suggested that this results from a systemic response that may be a consequence of primary vasculitis

  8. Current and emerging techniques for ANCA detection in vasculitis.

    PubMed

    Csernok, Elena; Moosig, Frank

    2014-08-01

    Detection of antineutrophil cytoplasmic antibodies (ANCAs) is a well-established diagnostic test used to evaluate suspected necrotizing vasculitis of small blood vessels. Conditions associated with these antibodies, collectively referred to as ANCA-associated vasculitides, include granulomatosis with polyangiitis (formerly known as Wegener granulomatosis), microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (formerly known as Churg-Strauss syndrome). The diagnostic utility of ANCA testing depends on the type of assay performed and on the clinical setting. Most laboratories worldwide use standard indirect immunofluorescence tests (IFT) to screen for ANCA and then confirm positive IFT results with antigen-specific tests for proteinase 3 (PR3) and myeloperoxidase (MPO). Developments such as automated image analysis of immunofluorescence patterns, so-called third-generation PR3-ANCA and MPO-ANCA ELISA, and multiplex technology have improved the detection of ANCAs. However, challenges in routine clinical practice remain, including methodological aspects of IFT performance, the diverse antigen-specific assays available, the diagnostic value of testing in clinical settings and the prognostic value of serial ANCA monitoring in the prediction of disease relapse. This Review summarizes the available data on ANCA testing, discusses the usefulness of the various ANCA assays and advises on the clinical indications for the use of ANCA testing. PMID:24890776

  9. [Rare forms of hypertension : From pheochromocytoma to vasculitis].

    PubMed

    Haller, H; Limbourg, F; Schmidt, B M; Menne, J

    2015-03-01

    Secondary hypertension affects only 5-10 % of hypertensive patients. Screening is expensive and time-consuming and should be performed only in patients for whom there is a high clinical suspicion of secondary hypertension. Clinical signs of secondary forms of hypertension are new-onset hypertension in patients without other risk factors (i.e., family history, obesity, etc.), sudden increase of blood pressure (BP) in a previously stable patient, increased BP in prepubertal children, resistant hypertension, and severe hypertension or hypertensive emergencies. In adults, renal parenchymal and vascular diseases as well as obstructive sleep apnea are the most common causes of secondary hypertension. Medication-induced hypertension and non-adherence to medication have to be ruled out. Of the endocrine causes associated with hypertension, primary aldosteronism is the most common. Other endocrine causes of hypertension such as thyroid disease (hypo- or hyperthyroidism), hypercortisolism (Cushing's syndrome), hyperparathyroidism, and pheochromocytoma are rare. Monogenetic forms of hypertension are mostly of tubular origin and associated with alterations in mineralocorticoid handling or signaling. Rare causes of hypertension also include inflammatory vascular disease. Acute forms of vasculitis may present as "malignant" hypertension with associated thrombotic microangiopathy and organ damage/failure. It is important to diagnose these rare forms of hypertension in order to prevent acute organ damage in these patients or unnecessary invasive treatment strategies. PMID:25700646

  10. ANCA-Associated Systemic Vasculitis Presenting With Hypertrophic Spinal Pachymeningitis

    PubMed Central

    Li, Xia; Zhao, Jiuliang; Wang, Qian; Fei, Yunyun; Zhao, Yan

    2015-01-01

    Abstract Reports of hypertrophic pachymeningitis associated with myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) localized exclusively in the spine were quite rare. Two cases of ANCA-associated systemic vasculitis (AASV) presenting with hypertrophic spinal pachymeningitis (HSP) causing low back pain and numbness are described. Two patients showed prominent systemic and local inflammatory reactions manifested as fever, elevated levels of erythrocyte sedimentation rate and C-reactive protein, and markedly increased levels of total protein of cerebrospinal fluid. The gadolinium (Gd)-enhanced T1-weighted magnetic resonance imaging scan of spinal cord demonstrated diffuse spinal dura matter thickening. Additionally, simple microscopic hematuria was found in 1 case suggestive of renal involvement and the other 1 complicated with interstitial lung disease. Then, a diagnosis of HSP secondary to AASV was made. Combination therapy of corticosteroids and cyclophosphamide produced a rapid improvement in the clinical symptoms and laboratory parameters. Followed up for 6 months, 1 case relapsed when the dosage of prednisone was tapered to 10 mg daily. Since the patient refused rituximab-based regimen, an immunosuppressive triple-therapy (corticosteroid, cyclophosphamide, and azathioprine) was initiated and brought control of the disease during the subsequent 6 months of follow-up. HSP is a relatively rare form of central nervous system involvement of AASV. Early recognition and intervention are of great significance since the pathogenesis of HSP starts with an inflammatory and fibrosing process. PMID:26579814

  11. Bartonella henselae aortic valve endocarditis mimicking systemic vasculitis

    PubMed Central

    Teoh, Laurence S G; Hart, Hamish H; Soh, May Ching; Christiansen, Jonathan P; Bhally, Hasan; Philips, Martin S; Rai-Chaudhuri, Dominic S

    2010-01-01

    A 28-year-old man with a bicuspid aortic valve presented with facial droop and slurred speech with several months of constitutional symptoms of night sweats, weight loss and productive cough. Examination confirmed aortic regurgitation, palpable spleen and left facial droop. Multiple peripheral blood cultures were negative. Inflammatory markers, cytoplasmic staining antineutrophil cytoplasmic antibodies (cANCA) and anti-PR3 antibody were all elevated. MRI of the brain and CT of the chest and abdomen confirmed embolic infarcts to brain, kidney and spleen. Transoesophageal echocardiogram (ECG) showed valve vegetations and severe aortic regurgitation. Endocardial Wegener's granulomatosis was considered. Aortic valve replacement was performed. Grindings from aortic valve leaflets were analysed for rpoB gene, which confirmed the presence of Bartonella henselae. Serological assays demonstrated B henselae IgM 20 (normal <20) and IgG >2048 (normal < 64). The patient completely recovered after prolonged antibiotic treatment. Culture-negative infective endocarditis may mimic vasculitis and be associated with positive cANCA. Serology and molecular techniques may aid diagnosis. PMID:22791485

  12. Circovirus in Tissues of Dogs with Vasculitis and Hemorrhage

    PubMed Central

    Li, Linlin; McGraw, Sabrina; Zhu, Kevin; Leutenegger, Christian M.; Marks, Stanley L.; Kubiski, Steven; Gaffney, Patricia; Dela Cruz Jr, Florante N.; Wang, Chunlin; Delwart, Eric

    2013-01-01

    We characterized the complete genome of a novel dog circovirus (DogCV) from the liver of a dog with severe hemorrhagic gastroenteritis, vasculitis, and granulomatous lymphadenitis. DogCV was detected by PCR in fecal samples from 19/168 (11.3%) dogs with diarrhea and 14/204 (6.9%) healthy dogs and in blood from 19/409 (3.3%) of dogs with thrombocytopenia and neutropenia, fever of unknown origin, or past tick bite. Co-infection with other canine pathogens was detected for 13/19 (68%) DogCV-positive dogs with diarrhea. DogCV capsid proteins from different dogs varied by up to 8%. In situ hybridization and transmission electron microscopy detected DogCV in the lymph nodes and spleens of 4 dogs with vascular compromise and histiocytic inflammation. The detection of a circovirus in tissues of dogs expands the known tropism of these viruses to a second mammalian host. Our results indicate that circovirus, alone or in co-infection with other pathogens, might contribute to illness and death in dogs. PMID:23628223

  13. Mast cells contribute to peripheral tolerance and attenuate autoimmune vasculitis.

    PubMed

    Gan, Poh-Yi; Summers, Shaun A; Ooi, Joshua D; O'Sullivan, Kim M; Tan, Diana S Y; Muljadi, Ruth C M; Odobasic, Dragana; Kitching, A Richard; Holdsworth, Stephen R

    2012-12-01

    Mast cells contribute to the modulation of the immune response, but their role in autoimmune renal disease is not well understood. Here, we induced autoimmunity resulting in focal necrotizing GN by immunizing wild-type or mast cell-deficient (Kit(W-sh/W-sh)) mice with myeloperoxidase. Mast cell-deficient mice exhibited more antimyeloperoxidase CD4+ T cells, enhanced dermal delayed-type hypersensitivity responses to myeloperoxidase, and more severe focal necrotizing GN. Furthermore, the lymph nodes draining the sites of immunization had fewer Tregs and reduced production of IL-10 in mice lacking mast cells. Reconstituting these mice with mast cells significantly increased the numbers of Tregs in the lymph nodes and attenuated both autoimmunity and severity of disease. After immunization with myeloperoxidase, mast cells migrated from the skin to the lymph nodes to contact Tregs. In an ex vivo assay, mast cells enhanced Treg suppression through IL-10. Reconstitution of mast cell-deficient mice with IL-10-deficient mast cells led to enhanced autoimmunity to myeloperoxidase and greater disease severity compared with reconstitution with IL-10-intact mast cells. Taken together, these studies establish a role for mast cells in mediating peripheral tolerance to myeloperoxidase, protecting them from the development of focal necrotizing GN in ANCA-associated vasculitis. PMID:23138486

  14. CNS vasculitis and stroke in neonatal lupus erythematosus: a case report and review of literature.

    PubMed

    Saini, Arushi G; Sankhyan, Naveen; Bhattad, Sagar; Vyas, Sameer; Saikia, Biman; Singhi, Pratibha

    2014-05-01

    Neonatal lupus erythematosus refers to the clinical spectrum of cardiac, cutaneous and other systemic abnormalities in neonates born to mothers with autoantibodies against Ro/SSA and La/SSB antigens. Isolated central nervous system involvement is very rare and has been described as transient vasculopathy only. We describe a 2-months-old girl who presented with acute ischemic stroke secondary to central nervous system vasculitis without any cardiac, cutaneous or hematological manifestations. The mother was pauci-symptomatic with raised anti-Ro autoantibody titers; the baby was positive for autoantibodies against Ro-antigen. Angiography confirmed vasculitis in cerebral vasculature. Our case highlights that neonatal lupus erythematosus can present with isolated nervous system manifestations and the vascular damage can be permanent in the form of vasculitis. Early recognition will help pediatricians identify such possible permanent complications in newborns with neonatal lupus erythematosus. A review of previously reported central nervous system manifestations of neonatal lupus is also presented. PMID:24508360

  15. Central Retinal Vein Occlusion in a Patient with Retinal Vasculitis and Crohn's Disease

    PubMed Central

    Figueiredo, Lígia; Rothwell, Renata; Brandão, Arnaldo

    2014-01-01

    The authors report a rare case of a 47-year-old woman with Crohn's disease (CD) who presented with retinal vasculitis and central retinal vein occlusion (CRVO) during remission. The patient complained of sudden painless visual loss in her left eye (OS). Ophthalmologic evaluation revealed a best corrected visual acuity (BCVA) of 20/20 in the right eye and hand movements in OS. Ophthalmoscopy and fluorescein angiography of OS showed signs of nonischemic CRVO and extensive vasculitis. She was treated with oral prednisolone, mercaptopurine, and intravitreal bevacizumab in OS. After 1 month of treatment, VA of OS improved to 5/10 and after 1 year it was 10/10 with complete resolution of retinal vasculitis and nonischemic CRVO. PMID:25506451

  16. Delayed diagnosis of ocular syphilis that manifested as retinal vasculitis and acute posterior multifocal placoid epitheliopathy.

    PubMed

    Park, Jong Hoon; Joe, Soo Geun; Yoon, Young Hee

    2013-11-01

    A 55-year-old female presented with bilateral progressive retinal vasculitis. She was on systemic and intravitreal steroids on the basis of uveitis work-up result (negative result including rapid plasma reagin), but her visual acuity continued to deteriorate to light perception only. Ocular examination showed retinal vasculitis, multiple yellow placoid lesions and severe macula edema in both eyes. Repeated work-up revealed positivity of fluorescent treponemal antibody-absorption in serum and subsequently in cerebrospinal fluid. Ocular syphilis was diagnosed. And intravenous penicillin G resulted in rapid resolution of vasculitis and macular edema. To avoid delay in the diagnosis of ocular syphilis, high index of suspicion and repeating serological tests (including both treponemal and non-treponemal tests) are warranted. PMID:24145569

  17. A Fatal Case of “Bullous Erysipelas-like” Pseudomonas Vasculitis

    PubMed Central

    Yang, Sam Shiyao; Chandran, Nisha Suyien; Huang, Jing Xiang; Tan, Kong-Bing; Aw, Derrick Chen-Wee

    2016-01-01

    Erysipelas is a generally benign superficial bacterial skin infection, and its bullous form constitutes a rare and more severe variant. We describe the first and fatal case of “bullous erysipelas-like” septic vasculitis due to Pseudomonas bacteremi. A 69-year-old Chinese man presenting with diarrhea and septic shock initially began to rapidly develop sharply defined erythematous plaques with non-hemorrhagic bullae over his lower limbs. Culture of the aspirate from the bullae was positive for Pseudomonas aeruginosa. This was also consistent with his blood cultures showing Pseudomonas bacteremia. Histology of the skin lesion showed microthrombi and neutrophilic infiltrates in blood vessels with Gram-negative bacilli extruding from the vessel walls, characteristic of septic vasculitis. The bullous erysipelas-like lesions seen in this patient represents a rare manifestation of both septic vasculitis and Pseudomonas infection. PMID:26955132

  18. Systemic Levamisole-Induced Vasculitis in a Cocaine User without Cutaneous Findings: A Consideration in Diagnosis

    PubMed Central

    Baptiste, Gillian G.; Alexopoulos, Anastasia-Stefania; Masud, Tahsin; Bonsall, Joanna M.

    2015-01-01

    Levamisole is a known immunomodulating agent frequently used as a cutting agent in cocaine consumed in the United States today. Numerous cases of anti-neutrophil cytoplasmic antibody (ANCA) vasculitis connected with the use of levamisole-adulterated cocaine have previously been reported in the literature, classically characterized by a retiform purpuric rash. We report a case of a crack-cocaine user without cutaneous abnormalities who developed ANCA-associated glomerulonephritis that progressed to renal failure. This case demonstrates the difficulties in solidifying the diagnosis of levamisole-induced vasculitis in the absence of cutaneous findings and the need to pursue more testing to establish causality in ANCA-associated vasculitis that has potential for severe end-organ damage in patients who continue to use cocaine. PMID:26635879

  19. Acute ST elevation myocardial infarction in fulminant systemic p-ANCA vasculitis: a rare catastrophic complication.

    PubMed

    Mathur, Kanupriya; Saini, Aditya; Bah, Tonjeh; Katikaneni, Pavan

    2016-01-01

    A 45-year-old Caucasian man presented to the hospital with a 3-month history of fatigue, bilateral upper and lower limb paresthesias and gradually worsening ascending paralysis. A few weeks later, he developed acute renal failure requiring haemodialysis. Investigations revealed presence of myeloperoxidase (MPO) perinuclear antineutrophil cytoplasmic antibodies (ANCA). Renal biopsy was conclusive for rapidly progressive glomerulonephritis with crescents. Treatment for ANCA positive vasculitis was initiated with pulsed steroids, cyclophosphamide and plasmapheresis. The hospital course took an unexpected turn when the patient developed acute chest pain with an EKG consistent with inferior ST elevation myocardial infarction (STEMI). Urgent left heart catheterisation revealed distal occlusions in multivessel coronary distribution. Coronary involvement is rare in ANCA vasculitis and STEMI has not been reported in MPO-ANCA positive vasculitis, to the best of our knowledge. PMID:27358099

  20. Capecitabine-induced leukocytoclastic vasculitis under neoadjuvant chemotherapy for locally advanced colorectal cancer

    PubMed Central

    Kee, Bryan K.; Tetzlaff, Michael T.; Wolff, Robert A.

    2015-01-01

    We describe a case of capecitabine-induced leukocytoclastic vasculitis in a patient with locally advanced rectal cancer under curative neoadjuvant concurrent chemoradiation using capecitabine. After 5 days of the initiation of capecitabine the patient developed a pruritic maculopapular rash in her extremities consistent with vasculitis which was confirmed on skin biopsy without any signs of systemic involvement. Capecitabine was held and the rash was treated with topical steroids with complete resolution of both rash and pruritus. Due to a lack of other alternative chemotherapeutic options and the cutaneous-only involvement of vasculitis, the capecitabine was re-introduced. Two days later, the patient developed an identical maculopapular rash with a similar distribution. Prednisone was initiated while the capecitabine was continued with complete resolution of the rash. The patient successfully completed her curative neoadjuvant chemoradiation therapy treatment without the need to permanently discontinue the capecitabine. PMID:26029464

  1. Large Vessel Vasculitis Occurring in Rheumatoid Arthritis Patient under Anti-TNF Therapy

    PubMed Central

    Cestelli, Valentina; Spinella, Amelia; Campomori, Federica; Esposito, Carmela; Ciaffi, Sara; Sandri, Gilda; Ferri, Clodoveo

    2014-01-01

    Vasculitis is a heterogeneous group of disorders characterized by the presence of necrotic inflammatory phenomena and destruction of blood vessels. Vasculitis is classified as primary (idiopathic) or secondary to infections, connective tissue diseases and drugs but can also be considered as a paraneoplastic phenomenon. Evidence shows that the increasing use of biological agents results in a growing number of reports of autoimmune diseases induced by these therapies. An inflammatory articular chronic disease such as rheumatoid arthritis may be complicated by extra-articular manifestations, such as cutaneous or systemic vasculitis. Herewith, we describe the case of a great vessels arteritis in a patient affected by rheumatoid arthritis in therapy with an anti-TNF agent (etanercept). PMID:25544845

  2. An approach to the diagnosis and management of systemic vasculitis revised version with tracked changes removed

    PubMed Central

    Miller, A; Chan, M; Wiik, A; Misbah, S A; Luqmani, R A

    2010-01-01

    The systemic vasculitides are a complex and often serious group of disorders which, while uncommon, require careful management in order to ensure optimal outcome. In most cases there is no known cause. Multi-system disease is likely to be fatal without judicious use of immunosuppression. A prompt diagnosis is necessary to preserve organ function. Comprehensive and repeated disease assessment is a necessary basis for planning therapy and modification of treatment protocols according to response. Therapies typically include glucocorticoids and, especially for small and medium vessel vasculitis, an effective immunosuppressive agent. Cyclophosphamide is currently the standard therapy for small vessel multi-system vasculitis, but other agents are now being evaluated in large randomized trials. Comorbidity is common in patients with vasculitis, including the cumulative effects of potentially toxic therapy. Long-term evaluation of patients is important in order to detect and manage relapses. PMID:20070316

  3. Mouse models of anti-neutrophil cytoplasmic antibody-associated vasculitis.

    PubMed

    Gan, Poh-Yi; Ooi, Joshua D; Kitching, A Richard; Holdsworth, Stephen R

    2015-01-01

    Inflammation of blood vessels (vasculitis) results from many pathological processes and is found in many different diseases. However, in most situations, the pathological processes inducing vasculitis are unknown. The discovery of anti-neutrophil cytoplasmic autoantibodies (ANCAs) in the 1980s opened the door for studies that eventually led to the description of a new previously undescribed disease, ANCA-associated vasculitis (AAV). Unravelling the immunopathogenesis of this new disease resulted largely from the development of animal models. The major breakthroughs were the description of ANCA, its association with small vessel vasculitis and the discovery of its target autoantigens (myeloperoxidase and Proteinase 3). Three major disease syndromes comprise the AAVs, microscopic polyangiitis, granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis (EGPA). Recent human studies suggest that proteinase 3 and myeloperoxidase associated vasculitis are two separate but related diseases. The ability to induce murine autoimmunity to myeloperoxidase including ANCA (with the same immune staining patterns as human ANCA) and the capacity of this anti-myeloperoxidase autoimmunity to induce disease with many of the characteristic features of human AAV are well developed. However, the development of animal models of anti-proteinase 3 ANCA and EGPA is much less well developed. Animal models are important in understanding the human disease and in particular in defining potential therapeutic targets and in early stage therapeutic testing of potential drugs. Clearly the relevance of animal models depends on how closely they mimic human diseases. The current status of animal models of vasculitis will be described in detail with reference to these criteria. PMID:25777754

  4. Cerebral vasculitis in adults: what are the steps in order to establish the diagnosis? Red flags and pitfalls

    PubMed Central

    Berlit, P; Kraemer, M

    2014-01-01

    Cerebral vasculitis is a rare cause of juvenile stroke. It may occur as primary angiitis of the central nervous system (PACNS) or as CNS manifestation in the setting of systemic vasculitis. Clinical hints for vasculitis are headache, stroke, seizures, encephalopathy and signs of a systemic inflammatory disorder. Diagnostic work-up includes anamnesis, whole body examination, laboratory and cerebral spinal fluid (CSF) studies, magnetic resonance imaging (MRI), angiography and brain biopsy. Due to the rarity of the disease, exclusion of more frequent differential diagnoses is a key element of diagnostic work-up. This review summarizes the steps that lead to the diagnosis of cerebral vasculitis and describes the red flags and pitfalls. Despite considering the dilemma of angiography-negative vasculitis and false-negative brain biopsy in some cases, it is important to protect patients from ‘blind’ immunosuppressive therapy in unrecognized non-inflammatory differential diagnosis. PMID:24117125

  5. Management of Small Vessel Vasculitides.

    PubMed

    Lopalco, Giuseppe; Rigante, Donato; Venerito, Vincenzo; Emmi, Giacomo; Anelli, Maria Grazia; Lapadula, Giovanni; Iannone, Florenzo; Cantarini, Luca

    2016-06-01

    Inflammation mediated by cells of the immune system and necrosis are the most striking features observed at the histologic level in patients with vasculitides, clinical entities classified according to pathologic findings involving different organs, to etiology, or to size of vessels involved. Small vessel vasculitides (SVV) are a peculiar group of systemic disorders electively involving small intraparenchymal arteries, arterioles, capillaries, or venules and leading to different levels of vascular obstruction, tissue ischemia and risk of infarction; they can be divided into anti-neutrophil cytoplasmic antibody-associated vasculitides and immune complex vasculitides. Despite the significant advances in understanding the whole disease process and pathophysiology of SVV, strong efforts are still needed to draft, share and spread guidelines in the therapeutic management of these protean disorders. After an accurate evaluation of different open or double-blind trials and cohort studies in this review, we analyze the actual medical tools suggested for treating granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis, Henoch-Schönlein purpura, cryoglobulinemic vasculitis, anti-glomerular basement membrane disease and hypocomplementemic urticarial vasculitis. PMID:27118389

  6. Vasculitis complicating granulocyte colony stimulating factor treatment of leukopenia and infection in Felty's syndrome.

    PubMed

    Farhey, Y D; Herman, J H

    1995-06-01

    Recombinant myeloid growth factors have been increasingly used in recent years to combat induced and disease associated neutropenia. Their application in the management of Felty's syndrome with intercurrent infection has raised concern that resultant neutrophilia and activation of a diverse array of polymorphonuclear cell functions may have an adverse effect on the rheumatoid disease process. We describe a patient with Felty's syndrome receiving short term treatment with recombinant human granulocyte colony stimulating factor (GCSF), who then developed acute renal failure in conjunction with leukocytoclastic vasculitis and presumptive gout. We address the issue of "adding fuel to the fire" and review reported implications of GCSF in induction of vasculitis. PMID:7545756

  7. Influenza vaccination induced leukocytoclastic vasculitis and pauci-immune crescentic glomerulonephritis.

    PubMed

    Yanai-Berar, N; Ben-Itzhak, O; Gree, J; Nakhoul, F

    2002-09-01

    Influenza vaccination is a widely accepted practice, particularly among the elderly and high-risk individuals. Minor and transitory side effects following the vaccination are common, while systemic complications are infrequently reported. We describe here a case of a patient who presented to the emergency room with arthralgia, myalgias and purpura, following influenza vaccination. Necrotizing vasculitis associated with pauci-immune glomerulonephritis was observed on kidney biopsy. With increasing use of influenza vaccination, attention should be drawn to the possible expression of systemic adverse effects such as vasculitis and glomerulonephritis. PMID:12356192

  8. Connective tissue disorders associated with vasculitis and vaso-occlusive disease of the hand.

    PubMed

    Michelotti, Brett; Rizzo, Marco; Moran, Steven L

    2015-02-01

    Hand ischemia caused by vasculitis is a secondary finding in many autoimmune processes. Many of these autoimmune diseases are managed primarily with medications that can prevent the development of occlusive disease, tissue ischemia, and tissue loss. Unfortunately several disease conditions can be recalcitrant to medical management and can result in ischemic changes within the hand, which may require operative intervention. This article briefly reviews the major connective tissue disorders associated with vasculitis and vaso-occlusive disease of the hand, including scleroderma, lupus, and Buerger disease, and their surgical treatment. PMID:25455357

  9. Recurrent erythema nodosum: a red flag sign of hidden systemic vasculitis

    PubMed Central

    Gupta, Monica; Singh, Kamal; Lehl, SS; Bhalla, Mala

    2013-01-01

    Takayasu's arteritis is a rare, systemic vasculitis with varied presentations across multiple medical specialities. Here, we present a young woman who had recurrent episodes of erythema nodosum on the background of a low-grade fever and no vascular manifestations. The presence of a high erythrocyte sedimentation rate generated a high index of suspicion for underlying vasculitis, and radioimaging confirmed the suspicion of Takayasu's arteritis. The patient was found to have type III diseases in the vasculitic stage and was managed with systemic corticosteroids. PMID:23576669

  10. Value of anti-infective chemoprophylaxis in primary systemic vasculitis: what is the evidence?

    PubMed Central

    2009-01-01

    Although infections are a major concern in patients with primary systemic vasculitis, actual knowledge about risk factors and evidence concerning the use of anti-infective prophylaxis from clinical trials are scarce. The use of high dose glucocorticoids and cyclophosphamide pose a definite risk for infections. Bacterial infections are among the most frequent causes of death, with Staphylococcus aureus being the most common isolate. Concerning viral infections, cytomegalovirus and varicella-zoster virus reactivation represent the most frequent complications. The only prophylactic measure that is widely accepted is trimethoprim/sulfamethoxazole to avoid Pneumocystis jiroveci pneumonia in small vessel vasculitis patients with generalised disease receiving therapy for induction of remission. PMID:19886977

  11. Different pathways leading to cutaneous leukocytoclastic vasculitis in mice.

    PubMed

    Sunderkötter, C; Seeliger, S; Schönlau, F; Roth, J; Hallmann, R; Luger, T A; Sorg, C; Kolde, G

    2001-12-01

    To investigate the pathomechanisms of leukocytoclastic vasculitis (LcV) we compared mouse models of LcV with non-vasculitic irritant contact dermatitis (ICD). Criteria for LcV as met by the immune complex-mediated Arthus reaction (Art-r) were also fulfilled by the localized Shwartzman reaction (Shw-r) and by cutaneous Loxoscelism (Lox) (injection of venom from Loxosceles reclusa containing sphingomyelinase D). After depletion of PMN (by gamma-irradiation) vessel damage could not be elicited in these models, distinguishing them from models of direct endothelial insult (necrotizing ICD). Depletion of complement could only delay, but not inhibit the Art-r, and did not change ICD, Lox or the Shw-r. The Shw-r exclusively revealed a sustained local expression of vascular adhesion molecules for 24 h in the preparatory phase (LPS s.c.), not observed in the Art-r, in Lox or ICD. Subsequent challenge with LPS i.p. was associated with upregulation of Mac-1 and ICAM-1 on PMN, but not of VLA-4 or LFA-1 (FACS analysis). Cytokines which were able to replace LPS in priming for LcV in the Shw-r (TNF-alpha and IL-1beta) also induced sustained expression of adhesion molecules, whereas IL-12 and IFN-gamma did neither. Neutralizing IL-12 or IFN-gamma also inhibited neither LcV nor sustained expression of adhesion molecules, whereas anti-TNF-alpha inhibited both. Anti-TNF-alpha had no marked inhibitory effects in the Art-r, in Lox or ICD. Combined (but not separate) neutralization of both E-selectin and VCAM-1 by antibodies suppressed LcV independent from reducing influx of PMN, proving that their sustained expression is decisive for the Shw-r and interferes with normal diapedesis. Since Loxosceles venom is known to dysregulate diapedesis and degranulation of PMN in vitro, since adherent immune complexes activate PMN at the vessel wall, and since adhesion molecules are dysregulated in the Shw-r, we suggest that LcV develops when activation of PMN coincides with vascular alterations

  12. Hypernephroma Presenting with Cutaneous Leukocytoclastic Vasculitis and Lupus Anticoagulant: Resolution after Nephrectomy

    PubMed Central

    Murray, Nigel P.; Ruíz, Amparo; Reyes, Eduardo

    2012-01-01

    Hypernephroma can present as a variety of paraneoplastic, nonmetastatic conditions, including vasculitis, and rarely a lupus-type anticoagulant. Nephrectomy leads to the resolution of the systemic complaints. Malignancy, in this case hypernephroma, can present as an immune-mediated paraneoplastic syndrome which resolves after removal of the underlying tumor. PMID:22919534

  13. Recurrent stroke in a case of left atrial myxoma masquerading vasculitis.

    PubMed

    Singh, Prashant K; Sureka, R K; Sharma, Anjani K; Bhuyan, Sushant; Gupta, Vikas

    2013-12-01

    We report a case of left atrial myxoma presenting as recurrent neurological deficits with absent peripheral pulses mimicking systemic vasculitis. Due to absence of cardiac signs and symptoms, there was one year delay in diagnosis from initial symptom. Left atrial myxomas are rare but treatable cause of recurrent stroke. PMID:24968552

  14. Rapid Growth of Lung Nodules due to Combined Pulmonary Vasculitis, Silicoanthracosis, and Chondrocalcinosis

    PubMed Central

    Distler, Oliver; Kolios, Antonios G. A.; Weder, Walter; Franzen, Daniel

    2016-01-01

    Background. Silicoanthracosis is a pneumoconiosis due to occupational inhalation of silica and carbon dusts. Clinically, it can be associated with vasculitis or rheumatoid arthritis. In association with these diseases, silicoanthracosis can present within the lung with multiple pulmonary nodules which, as a differential diagnosis, can mimic metastatic disease or multiple abscesses. Case Presentation. We present the case of a 62-year old former pit worker with pulmonary nodules, chondrocalcinosis due to calcium pyrophosphate deposition (CPPD), and a history of renal cancer. Within a short period of time, pulmonary nodules grew rapidly. Thoracoscopically, the resected lung specimen revealed silicoanthracosis associated with small-to-medium-size vasculitis in the presence of antineutrophil cytoplasmatic autoantibodies (c-ANCA). Conclusion. Pulmonary silicoanthracotic lesions on the base of ANCA-associated vasculitis and CPPD arthritis can rapidly grow. A mutual correlation between silicoanthracosis, ANCA-associated vasculitis, and CPPD seems possible. Apart from this, consideration of metastatic disease should be obligatory in patients with a history of cancer at the same time being immunosuppressed. PMID:27478398

  15. Epitope specificity determines pathogenicity and detectability in ANCA-associated vasculitis

    EPA Science Inventory

    ABSTRACT BACKGROUND Anti-neutrophil cytoplasmic autoantibodies (ANCA) specific for myeloperoxidase (MPO) or proteinase 3 (PR3) are detectable in >90% of patients with ANCA-associated vasculitis (AAV). ANCA titers do not correlate well with disease activity. In vivo and in vi...

  16. The diagnostic value of perivascular infiltrates in muscle biopsy specimens for the assessment of rheumatoid vasculitis

    PubMed Central

    Voskuyl, A.; van Duinen, S. G; Zwinderman, A.; Breedveld, F.; Hazes, J.

    1998-01-01

    OBJECTIVE—To determine the diagnostic value of perivascular infiltrates (PVI) in randomly obtained muscle biopsy specimens for the assessment of rheumatoid vasculitis (RV).
METHODS—The number and size of PVIs, defined as the presence of mononuclear or polymorphonuclear cells around ⩾ 50% of the circumference of a vessel wall, as well as the presence of fibrinoid necrosis were determined in frozen sections of muscle samples of RV patients with histologically confirmed vasculitis in fixed muscle tissue (n=12). The findings were compared with those observed in frozen sections of muscle biopsy specimens of rheumatoid arthritis (RA) patients not suspected of vasculitis (n=14) and patients with osteoarthritis (OA) (n=11). The presence of PVIs and of fibrinoid necrosis were sought in four frozen sections of the muscle biopsy specimen.
RESULTS—PVIs were observed in 75% of the RV patients, which was significantly (p < 0.05) higher than the frequency found in RA (14%) or OA (18%) patients. PVIs with ⩾ three cell layers were found in 67% of the RV patients and in none of the RA and OA patients (p < 0.05). Fibrinoid necrosis was found in least one of four frozen section in 33% of the RV patients. There was a good intra-observer and inter-observer concordance on the presence of fibrinoid necrosis and of PVIs with ⩾ three cell layers.
CONCLUSIONS—The assessment of PVIs with ⩾ three cell layers in a muscle biopsy specimen is a specific and reliable test in discriminating RV from RA without vasculitis. The demonstration in muscle of PVIs with ⩾ three cell layers is more sensitive than that of fibrinoid necrosis in the diagnosis of RV.

 Keywords: rheumatoid arthritis; vasculitis; diagnosis; histology; perivascular infiltrates PMID:9613342

  17. The wound/burn guidelines - 4: Guidelines for the management of skin ulcers associated with connective tissue disease/vasculitis.

    PubMed

    Fujimoto, Manabu; Asano, Yoshihide; Ishii, Takayuki; Ogawa, Fumihide; Kawakami, Tamihiro; Kodera, Masanari; Abe, Masatoshi; Isei, Taiki; Ito, Takaaki; Inoue, Yuji; Imafuku, Shinichi; Irisawa, Ryokichi; Ohtsuka, Masaki; Ohtsuka, Mikio; Kadono, Takafumi; Kawaguchi, Masakazu; Kukino, Ryuichi; Kono, Takeshi; Sakai, Keisuke; Takahara, Masakazu; Tanioka, Miki; Nakanishi, Takeshi; Nakamura, Yasuhiro; Hashimoto, Akira; Hasegawa, Minoru; Hayashi, Masahiro; Fujiwara, Hiroshi; Maekawa, Takeo; Matsuo, Koma; Madokoro, Naoki; Yamasaki, Osamu; Yoshino, Yuichiro; Le Pavoux, Andres; Tachibana, Takao; Ihn, Hironobu

    2016-07-01

    The Japanese Dermatological Association prepared guidelines focused on the treatment of skin ulcers associated with connective tissue disease/vasculitis practical in clinical settings of dermatological care. Skin ulcers associated with connective tissue diseases or vasculitis occur on the background of a wide variety of diseases including, typically, systemic sclerosis but also systemic lupus erythematosus (SLE), dermatomyositis, rheumatoid arthritis (RA), various vasculitides and antiphospholipid antibody syndrome (APS). Therefore, in preparing the present guidelines, we considered diagnostic/therapeutic approaches appropriate for each of these disorders to be necessary and developed algorithms and clinical questions for systemic sclerosis, SLE, dermatomyositis, RA, vasculitis and APS. PMID:26972733

  18. A retrospective analysis of treatment outcomes in patients with hepatitis C related systemic vasculitis receiving intravenous methylprednisolone and cyclophosphamide.

    PubMed

    Shahin, Amira A; El Desouky, Soha M; Zayed, Hania S

    2011-05-01

    The aim of this work is to describe the outcome of a series of patients with hepatitis C virus (HCV)-related vasculitis who were treated with corticosteroids and I.V. cyclophosphamide without receiving any antiviral therapy. The data of 16 patients with HCV infection and vasculitis were retrospectively analyzed for the treatment outcome in the present study. Eleven patients were females (68.8%) with a mean age of 49.6 ± 10.0 years. Nine patients (56.2%) had medium-sized vessel vasculitis (group A) and seven patients (43.8%) had small vessel vasculitis (group B). Disease activity was assessed using the Birmingham Vasculitis Activity Score (BVAS 2003) and organ damage was assessed by the Vasculitis Damage Index (VDI). HCV infection was confirmed in all patients by the detection of antibodies to HCV in serum by ELISA and HCV RNA using qualitative PCR. Quantitative PCR was done using the branched DNA technique. None of our study patients had received antiviral therapy, but they all received I.V.-pulsed cyclophosphamide monthly for 6 months, then every 3 months for six times if needed, preceded by I.V. methylprednisolone. Twelve patients (75%) had undetectable viral load by the quantitative technique. The drop in mean BVAS recorded at different intervals was highly significant. Although there was a drop in the VDI mean between the first and second reading, it was not statistically significant. All patients responded to treatment. Seven patients (43.8%) had relapse. Two patients died (12.5%). One patient died from renal failure (group B) and another died from sepsis (group A). The treatment outcomes were not statistically significant between the two vasculitis groups. A subset of patients with HCV-related vasculitis and with low levels of viremia can be safely treated with corticosteroids and cyclophosphamide alone. Despite successful treatment, a significant proportion of patients relapse and some develop severe complications and death. PMID:20924628

  19. Stroke-like Phenomena Revealing Multifocal Cerebral Vasculitis in Pediatric Lyme Neuroborreliosis.

    PubMed

    Kurian, Mary; Pereira, Vitor Mendes; Vargas, Maria Isabel; Fluss, Joel

    2015-08-01

    Stroke-like presentation in Lyme neuroborreliosis is rare in the pediatric age group. We report a previously healthy 12-year-old boy who presented with acute left hemiparesis and meningeal signs. Neuroimaging failed to reveal any cerebral infarction but demonstrated a multifocal cerebral vasculitis involving small, medium and large-sized vessels affecting both the anterior and posterior circulation. Concentric contrast enhancement of the basilar artery was also observed. Further investigations and laboratory findings were consistent with Lyme neuroborreliosis. A rapidly favorable clinical outcome was obtained with appropriate antibiotic treatment along with antiaggregants and steroids. Lyme neuroborreliosis should be considered in the diagnostic differential, not only in adults but also among children, especially in the context of an unexplained cerebral vasculitis. PMID:25316727

  20. Membranous nephropathy with crescents associated with levamisole-induced MPO-ANCA vasculitis.

    PubMed

    Moinuddin, Irfan; Madhrira, Machaiah; Bracamonte, Erika; Thajudeen, Bijin; Sussman, Amy

    2016-07-01

    ANCA-associated vasculitis (AAV) is the most common cause of crescentic rapidly progressive glomerulonephritis (GN). Levamisole used as an adulterant in cocaine is increasingly recognized as a cause of AAV. We report the case of a 50 year old woman with atypical anti-MPO AAV associated with cocaine use and exposure to levamisole. In addition to the clinical and pathologic findings of crescentic GN, the patient also had biopsy evidence of secondary membranous nephropathy (MN). Although AAV and MN have been reported previously in the same patient and both have been induced by drug exposures, this is the first report of MN in a patient with AAV likely induced by levamisole. We suggest that MPO can cause both pauci-immune vasculitis and secondary membranous nephropathy in some cases, as in cases of levamisole-adulterated cocaine use. PMID:27067665

  1. A case of scrotal swelling mimicking testicular torsion preceding Henoch-Schönlein vasculitis.

    PubMed

    Akgun, C

    2012-01-01

    Henoch-Schönlein purpura, is one of the most common types of multisystemic vasculitis seen in childhood. The major clinical manifestations are cutaneous purpura, arthritis, abdominal pain, gastrointestinal bleeding, and nephritis. Isolated central nervous system vasculitis, seizures, coma and hemorrhage, Guillan--Barré syndrome, ataxia and central and peripheral neuropathy, ocular involvement, orchitis, epididymitis or testicular torsion are medical or surgical complications. In this study, we report a 7-year-old boy with scrotal swelling mimicking testicular torsion with ultrasonographic and clinical findings that the typical clinical features of Henoch-Schönlein purpura including rashes and arthritis were developed after one week of surgery (Ref. 15). PMID:22693978

  2. Acral keratoses and leucocytoclastic vasculitis occurring during treatment of essential thrombocythaemia with hydroxyurea.

    PubMed

    Worley, B; Glassman, S J

    2016-03-01

    Hydroxyurea is used in essential thrombocythaemia to lower thromboembolic risk. Cutaneous adverse effects from hydroxyurea are diverse. Small vessel vasculitis has been rarely reported, and the coexistence of several different morphologies has not been described. We report a case of acral keratoses, psoriasiform plaques and leucocytoclastic vasculitis (LCV) in a patient with essential thrombocythaemia. A 69-year-old woman developed a confusing array of skin lesions including keratotic papules, psoriasiform plaques and keratoderma 4 years after commencing hydroxyurea therapy. The initial diagnosis was hand and foot psoriasis, but lesions were resistant to therapy. With an increase in the dose of hydroxyurea, the lesions ulcerated. Skin biopsies taken from different sites indicated different diagnoses, including LCV. Discontinuation of hydroxyurea yielded rapid improvement. Although the most commonly reported cutaneous adverse effect from hydroxyurea is leg ulceration, this can be preceded or accompanied by less dramatic skin lesions. Unless recognized, delayed diagnosis and lesion progression can occur. PMID:26269121

  3. Cutaneous Necrotizing Vasculitis and Leukopenia in a Cocaine User: Is Levamisole the Culprit?

    PubMed Central

    Zeineddine, Nabil; Felix, Richard; Goldstein, Mark

    2016-01-01

    Levamisole is an antihelminthic drug banned by the US Food and Drug Administration (FDA) in 2000 because of its dangerous side effects. Over the past few years, it has been identified as an adulterant in cocaine and reported to cause cutaneous vasculitis in cocaine users. The health burden of levamisole is serious since it is estimated that over 5 million Americans use cocaine and that 70% of the cocaine used in the USA contains levamisole. In this paper we report the case of a 23-year-old female cocaine user that presented with purpuric rash and skin necrosis, found to have positive c-ANCA and anti-proteinase 3 antibodies. Her skin biopsy showed fibroconnective tissue with signs of necrosis, acute and chronic inflammation, and thrombus formation. She was diagnosed with levamisole-induced vasculitis and successfully treated with withdrawal of cocaine use and local wound care. PMID:27579207

  4. Juvenile gangrenous vasculitis of the scrotum: Is it a variant of pyoderma gangrenosum?

    PubMed

    Caputo, Ruggero; Marzano, Angelo V; Di Benedetto, Alessandra; Ramoni, Stefano; Cambiaghi, Stefano

    2006-08-01

    Juvenile gangrenous vasculitis of the scrotum was described by Piñol et al in 1974 as a unique variant of scrotal gangrene of unknown origin, occurring exclusively in young individuals. It was characterized by an acute onset of skin ulcers undergoing complete resolution after appropriate therapy, with no relapses. We present a typical case of this extremely rare disease affecting a 16-year-old boy in whom the scrotal ulcerations were preceded by an episode of pharyngitis with fever. The condition promptly regressed after administration of intramuscular betamethasone in combination with oral ciprofloxacin. According to Piñol et al, juvenile gangrenous vasculitis of the scrotum, although poorly known to dermatologists, should be regarded as a distinctive entity within the wide group of scrotal gangrenes. On the other hand, the hypothesis that this condition may well represent a variant of pyoderma gangrenosum is discussed. PMID:16843125

  5. Scabies presenting as a necrotizing vasculitis in the presence of lupus anticoagulant.

    PubMed

    Jarrett, P; Snow, J

    1998-10-01

    A 75-year-old man presented with a progressive eruption of 6 weeks' duration. Purpura and focal areas of cutaneous infarction were present on the legs. There were scabies burrows on the wrists and hands, and a mite was extracted. Histology of a skin biopsy showed a necrotizing leucocytoclastic vasculitis. Circulating lupus anticoagulant was detected. Treatment consisted of topical 5% permethrin cream and systemic steroids. PMID:9892918

  6. Letrozole-induced necrotising leukocytoclastic small vessel vasculitis: First report of a case in the UK

    PubMed Central

    Pathmarajah, Pirunthan; Shah, Karishma; Taghipour, Kathy; Ramachandra, Su; Thorat, Mangesh A; Chaudhry, Ziaullah; Patkar, Vivek; Peters, Francesca; Connor, Thomas; Spurrell, Emma; Tobias, Jeffrey S; Vaidya, Jayant S

    2015-01-01

    Introduction Letrozole, an aromatase inhibitor, is a commonly used neo-adjuvant drug to treat hormone-sensitive breast cancer. There have been a few cases of aromatase inhibitor induced vasculitis but the first case of letrozole-induced vasculitis was reported from Switzerland in 2014 (Digklia et al.) [1]. Presentation of case We report the case of a 72-year-old woman with a small breast cancer. She was started on pre-operative letrozole (2.5 mg/d) whilst awaiting surgery. Ten days later she presented with burning pain and purpuric skin lesions which progressed to extensive ischaemic superficial necrosis of the lower limb skin, resolving over 3–4 months after local and systemic steroids. Histologically, it showed leucocytoclasis with evidence of eosinophilia consistent with a diagnosis of cutaneous leukocytoclastic small vessel vasculitis. Discussion The initial clinical presentation was severe burning pain around the ankles and a spreading violaceous rash. Letrozole was stopped. Wide local excision (lumpectomy) and sentinel node biopsy were postponed because of the accompanying pneumonitis and gastrointestinal upset, and were carried out 3.5 months later. Fortunately, the tumour size did not increase, but appeared to reduce, and axillary lymph nodes remained negative, i.e., this patient’s cancer outcome does not seem to have been jeopardized. Conclusion Leukocytoclastic vasculitis is a hypersensitivity reaction that is usually self-resolving, though our case needed systemic steroid treatment. Letrozole is a commonly used drug in clinical practice and prescribers should be aware of this rare side effect, which in our case delayed treatment without any apparent harm and possibly reduced tumour size. PMID:26432999

  7. Pulmonary fibrosis—an uncommon manifestation of anti-myeloperoxidase-positive systemic vasculitis?

    PubMed Central

    Bhanji, Amir; Karim, Mahzuz

    2010-01-01

    Small vessel vasculitides such as microscopic polyangiitis and Wegener’s granulomatosis commonly involve the kidney and lung, with alveolar haemorrhage being the commonest manifestation of pulmonary involvement. Here we describe a patient who developed acute renal failure and pulmonary haemorrhage with positive autoantibodies against myeloperoxidase 1 year after a diagnosis of usual interstitial pneumonia had been made and we discuss the uncommon association of pulmonary fibrosis and anti-myeloperoxidase positive vasculitis. PMID:20640181

  8. Bioinspired engineering study of Plantae vascules for self-healing composite structures.

    PubMed

    Trask, R S; Bond, I P

    2010-06-01

    This paper presents the first conceptual study into creating a Plantae-inspired vascular network within a fibre-reinforced polymer composite laminate, which provides an ongoing self-healing functionality without incurring a mass penalty. Through the application of a 'lost-wax' technique, orthogonal hollow vascules, inspired by the 'ray cell' structures found in ring porous hardwoods, were successfully introduced within a carbon fibre-reinforced epoxy polymer composite laminate. The influence on fibre architecture and mechanical behaviour of single vascules (located on the laminate centreline) when aligned parallel and transverse to the local host ply was characterized experimentally using a compression-after-impact test methodology. Ultrasonic C-scanning and high-resolution micro-CT X-ray was undertaken to identify the influence of and interaction between the internal vasculature and impact damage. The results clearly show that damage morphology is influenced by vascule orientation and that a 10 J low-velocity impact damage event is sufficient to breach the vasculature; a prerequisite for any subsequent self-healing function. The residual compressive strength after a 10 J impact was found to be dependent upon vascule orientation. In general, residual compressive strength decreased to 70 per cent of undamaged strength when vasculature was aligned parallel to the local host ply and a value of 63 per cent when aligned transverse. This bioinspired engineering study has illustrated the potential that a vasculature concept has to offer in terms of providing a self-healing function with minimum mass penalty, without initiating premature failure within a composite structure. PMID:19955122

  9. Bioinspired engineering study of Plantae vascules for self-healing composite structures

    PubMed Central

    Trask, R. S.; Bond, I. P.

    2010-01-01

    This paper presents the first conceptual study into creating a Plantae-inspired vascular network within a fibre-reinforced polymer composite laminate, which provides an ongoing self-healing functionality without incurring a mass penalty. Through the application of a ‘lost-wax’ technique, orthogonal hollow vascules, inspired by the ‘ray cell’ structures found in ring porous hardwoods, were successfully introduced within a carbon fibre-reinforced epoxy polymer composite laminate. The influence on fibre architecture and mechanical behaviour of single vascules (located on the laminate centreline) when aligned parallel and transverse to the local host ply was characterized experimentally using a compression-after-impact test methodology. Ultrasonic C-scanning and high-resolution micro-CT X-ray was undertaken to identify the influence of and interaction between the internal vasculature and impact damage. The results clearly show that damage morphology is influenced by vascule orientation and that a 10 J low-velocity impact damage event is sufficient to breach the vasculature; a prerequisite for any subsequent self-healing function. The residual compressive strength after a 10 J impact was found to be dependent upon vascule orientation. In general, residual compressive strength decreased to 70 per cent of undamaged strength when vasculature was aligned parallel to the local host ply and a value of 63 per cent when aligned transverse. This bioinspired engineering study has illustrated the potential that a vasculature concept has to offer in terms of providing a self-healing function with minimum mass penalty, without initiating premature failure within a composite structure. PMID:19955122

  10. Update on Outcome Measure Development for Large Vessel Vasculitis: Report from OMERACT 12

    PubMed Central

    Aydin, Sibel Zehra; Direskeneli, Haner; Sreih, Antoine; Alibaz-Oner, Fatma; Gul, Ahmet; Kamali, Sevil; Hatemi, Gulen; Kermani, Tanaz; Mackie, Sarah L.; Mahr, Alfred; Meara, Alexa; Milman, Nataliya; Nugent, Heidi; Robson, Joanna; Tomasson, Gunnar; Merkel, Peter A.

    2015-01-01

    Objective The rarity of large vessel vasculitis (LVV) is a major factor limiting randomized controlled trials in LVV, resulting in treatment choices in these diseases that are guided mainly by observational studies and expert opinion. Further complicating trials in LVV is the absence of validated and meaningful outcome measures. The Outcome Measures in Rheumatology (OMERACT) vasculitis working group initiated the Large Vessel Vasculitis task force in 2009 to develop data-driven, validated outcome tools for clinical investigation in LVV. This report summarizes the progress that has been made on a disease activity assessment tool and patient-reported outcomes in LVV as well as the group’s research agenda. Methods The OMERACT LVV task force brought an international group of investigators and patient research partners together to work collaboratively on developing outcome tools. The group initially focused on disease activity assessment tools in LVV. Following a systematic literature review, an international Delphi exercise was conducted to obtain expert opinion on principles and domains for disease assessment. The OMERACT vasculitis working group’s LVV task force is also conducting qualitative research with patients, including interviews, focus groups, and engaging patients as research partners, all to ensure that the approach to disease assessment includes measures of patients’ perspectives and that patients have input into the research agenda and process. Results The preliminary results of both the Delphi exercise and the qualitative interviews were discussed at the OMERACT 12 (2014) meeting and the completion of the analyses will produce an initial set of domains and instruments to form the basis of next steps in the research agenda. Conclusion The research agenda continues to evolve, with the ultimate goal of developing an OMERACT-endorsed core set of outcome measures for use in clinical trials of LVV. PMID:26077399

  11. Kidney transplantation for a patient with refractory childhood-onset ANCA-associated vasculitis.

    PubMed

    Kaseda, Koji; Marui, Yuji; Suwabe, Tatsuya; Hoshino, Junichi; Sumida, Keiichi; Hayami, Noriko; Mise, Koki; Tanaka, Kiho; Takaichi, Kenmei; Tomikawa, Shinji; Fujii, Takeshi; Ohashi, Kenichi; Ubara, Yoshifumi

    2016-03-01

    A 14-year-old Japanese girl was admitted to our institution for the evaluation of renal dysfunction. Her serum creatinine was 1.1 mg/dL, proteinuria was 1.5 g/day, the urine sediment contained numerous erythrocytes per high-power field, and she was positive for myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA). Proteinuria was first noted at the age of 12 years. Renal biopsy showed crescentic glomerulonephritis with slight immunoglobulin A (IgA) deposition. A diagnosis of ANCA-associated vasculitis was made. Immunosuppressive therapy was initiated, including steroid pulse therapy and intravenous cyclophosphamide pulse therapy, but hemodialysis was required after 6 years. Eight months after the patient became anuric and her MPO-ANCA titer became negative, living-related donor kidney transplantation was done from her mother. ANCA became slightly positive 2 years later, but the patient remains stable without proteinuria or hematuria at 4 years after surgery. This case suggests that kidney transplantation can be performed successfully for a patient with refractory childhood-onset ANCA-associated vasculitis, and that remission of vasculitis associated with ANCA negativity at transplantation may contribute to a better renal prognosis in this patient. PMID:24645722

  12. Histopathological Characteristics of Post-inflamed Coronary Arteries in Kawasaki Disease-like Vasculitis of Rabbits.

    PubMed

    Fujii, Maiko; Tanaka, Hideo; Nakamura, Akihiro; Suzuki, Chinatsu; Harada, Yoshinori; Takamatsu, Tetsuro; Hamaoka, Kenji

    2016-02-27

    Kawasaki disease (KD) is a systemic vasculitis in infants that develops predominantly in the coronary arteries. Despite the clinically transient nature of active inflammation in childhood albeit rare complications (e.g., coronary artery aneurysm), KD has recently been suggested to increase the incidence of ischemic heart diseases in young adulthood. However, little is known about the histopathology of the coronary artery long after development of the acute KD vasculitis. To address this, we conducted histological studies of rabbit coronary arteries in adolescent phase after induction of the KD-like vasculitis induced by horse serum administration. After a transmural infiltration of inflammatory cells in acute phase at day 7, the artery exhibited a gradual decrease in the number of inflammatory cells and thickening of the intima during the chronic phase up to day 90, where proteoglycans were distinctly accumulated in the intima with abundant involvement of α-smooth muscle actin (α-SMA)-positive cells, most of which accompanied expression of VCAM-1 and NF-κB. Distinct from classical atherosclerosis, inflammatory cells, e.g., macrophages, were barely detected during the chronic phase. These observations indicate that the KD-like coronary arteritis is followed by intimal thickening via accumulation of proteoglycans and proliferation of α-SMA-positive cells, reflecting aberrant coronary artery remodeling. PMID:27006519

  13. [A case of elderly onset loss of consciousness due to bacterial meningitis and subsequent vasculitis].

    PubMed

    Kameno, Mami; Takata, Toshihiro; Yasuda, Hisahumi; Hara, Kenta; Okano, Hiroyuki; Sakurai, Takashi; Nagata, Masao; Yokono, Koichi

    2008-07-01

    We reported an 83-year-old woman, who suffered from bacterial meningitis and subsequent vasculitis. She experienced episodes of loss of consciousness several times in July, 2006. She also had recurrent fever and was admitted to a local hospital. Routine examinations, including brain MRI and electroencephalogram, were negative and urinary tract infection was diagnosed. After successful antibiotic therapy, she was transferred to a rehabilitation hospital. After transfer, she had no headache, but presented fever again, and a reduced level of consciousness. Cerebrospinal fluid test showed that cell counts were high with a predominance of neutrophils, and her glucose level was low. She received antibiotic therapy on her suspicion of bacterial meningitis. Bacterial cultures of CSF and blood were negative, probably due to the previous antibiotic therapy. Repeated CSF analysis showed a decrease in cell counts, but her lower consciousness did not improve. Moreover, neurological symptoms such as left pyramidal tract sign appeared. She was transferred to our hospital on the suspicion of vasculitis. Diffusion MRI showed high intensity in the right middle cerebral artery (MCA) area and CT angiography showed the stenosis of the right MCA at the M3 portion. Two courses of steroid pulse therapy were performed. Her consciousness gradually improved and eventually could talk although cognitive decline remained as a residual deficit. Our patient failed to be diagnosed early because of atypical symptoms of meningitis. Caution seems necessary for elderly cases presenting with atypical initial symptoms of meningitis. Steroid pulse therapy was effective for the subsequent vasculitis, as reported previously. PMID:18753720

  14. A pain in the neck: carotid artery dissection presenting as vasculitis.

    PubMed

    Collamer, Angelique N; Battafarano, Daniel

    2013-07-01

    Spontaneous cervical artery dissection is increasingly recognized as a common cause of ischemic stroke in the young and middle-aged. Noninvasive imaging techniques such as magnetic resonance imaging and magnetic resonance angiography have widely replaced conventional angiography as the initial diagnostic study of extracranial dissections, allowing greater numbers of patients to be screened and thus leading to increased frequency of the diagnosis. We present a case of spontaneous carotid artery dissection in a previously healthy 48-year-old woman who presented with neck pain and elevated inflammatory markers. Marked gadolinium enhancement of the right extracranial internal carotid artery on magnetic resonance imaging led to an initial diagnosis of vasculitis. This case shows that the vessel injury associated with spontaneous carotid artery dissection is associated with an inflammatory response that can mimic vasculitis on highly sensitive imaging techniques, a phenomenon not well described previously. In this report, we review the nonvasculitic conditions that can mimic vasculitis and present clinicians complex diagnostic challenges. Recognition of these pseudovasculitic syndromes is important to avoid overdiagnosis resulting in unnecessary and potentially harmful immunosuppressive and cytotoxic treatments. PMID:23820364

  15. Successful treatment of cerebral large vessel vasculitis in systemic lupus erythematosus with intravenous pulse cyclophosphamide.

    PubMed

    Kato, R; Sumitomo, S; Kawahata, K; Fujio, K; Yamamoto, K

    2015-07-01

    A 39-year-old woman with a six-year history of systemic lupus erythematosus (SLE) was admitted because of a prolonged high fever, discoid rash, and multiple lymphadenopathies. She also developed pericarditis, and was treated with intravenous methylprednisolone pulse therapy followed by prednisolone 50 mg daily and cyclosporine 100 mg daily. Meanwhile, she had a progressive headache, and a brain MRI revealed right pons infarction, although she did not have any abnormal neurological findings. An MRA revealed obvious irregular narrowing in the basilar, right vertebral and right posterior cerebral artery. There was no evidence of antiphospholipid syndrome. We concluded that the cause of the asymptomatic brain infarction was cerebral large vessel vasculitis associated with neuropsychiatric SLE. Intravenous cyclophosphamide pulse therapy was started, and two months later, we confirmed that the irregular arterial narrowing had markedly ameliorated.Cerebral large vessel vasculitis in neuropsychiatric SLE is very rare, and a marked amelioration has not been reported to date. Here, we present a rare case of cerebral large vessel vasculitis treated successfully with a clear visual presentation. PMID:25661835

  16. Valvular involvement in ANCA-associated systemic vasculitis: a case report and literature review

    PubMed Central

    2011-01-01

    Background Antineutrophil cytoplasmic antibodies (ANCA)-associated systemic vasculitides have a variety of presentations, but cardiac valvular involvement is rarely diagnosed and its management is not established. Case presentation We report the case of a 44 year old man who presented with an ANCA-associated systemic vasculitis and aortic regurgitation of unusual mechanism. Transthoracic and transesophageal echocardiography disclosed septal hypertrophy preventing a complete closure of the aortic valve and thus responsible for a massive aortic regurgitation. After 4 months of immunosuppressive therapy, the valve lesion did not subside and the patient had to undergo aortic valve replacement. This report also reviews the 20 cases of systemic ANCA-associated vasculitis with endocardial valvular involvement previously reported in the English language medical literature. Conclusions Valvular involvement in ANCA-associated systemic vasculitides is rarely reported. Most of these lesions are due to Wegener's granulomatosis and half are present when the diagnosis of vasculitis is made. The valvular lesion is usually isolated, aortic regurgitation being the most frequent type, and often requires valve replacement in the months that follow it's discovery. PMID:21345235

  17. Acute vasculitis resulting in free flap failure: the importance of early recognition and options for management.

    PubMed

    Brennan, P A; Colbert, S; Spedding, A V; Herd, M K; Mellor, T K; Anand, R; McCrae, F

    2012-11-01

    Unusual or unexpected medical causes for free flap failure do occur but are uncommon. We present a rare case of a fibula free flap failure due to an acute vasculitis which was undiagnosed until after the flap had failed. In addition to two successful flap salvages and intravenous heparin, an epoprostenol infusion was commenced but a third salvage was not successful. The vasculitis resulted in marked blood vessel wall thickening, and cutaneous manifestations which presented as late signs. High peri-nuclear anti nuclear cytoplasmic antibody (pANCA) and myeloperoxidase (MOP) titres were subsequently found and histology from several blood vessels showed marked inflammation throughout the wall. A diagnosis of microscopic polyangiitis was made and high dose steroids were subsequently commenced. Interestingly, he had vasculitis several years previously treated with oral steroids but had been discharged from the rheumatology clinic. This rare case illustrates the potential hazards of free flap surgery in the vasculitides and discusses the warning signs and various management options to reduce the likelihood of flap failure in these patients. PMID:22534125

  18. Immunomodulation with eicosapentaenoic acid supports the treatment of autoimmune small-vessel vasculitis

    PubMed Central

    Hirahashi, Junichi; Kawahata, Kimito; Arita, Makoto; Iwamoto, Ryo; Hishikawa, Keiichi; Honda, Mie; Hamasaki, Yoshifumi; Tanaka, Mototsugu; Okubo, Koshu; Kurosawa, Miho; Takase, Osamu; Nakakuki, Masanori; Saiga, Kan; Suzuki, Kazuo; Kawachi, Shoji; Tojo, Akihiro; Seki, George; Marumo, Takeshi; Hayashi, Matsuhiko; Fujita, Toshiro

    2014-01-01

    Small-vessel vasculitis is a life-threatening autoimmune disease that is frequently associated with anti-neutrophil cytoplasmic antibodies (ANCAs). Conventional immunotherapy including steroids and cyclophosphamide can cause serious adverse events, limiting the efficacy and safety of treatment. Eicosapentaenoic acid (EPA), a key component of fish oil, is an omega-3 polyunsaturated fatty acid widely known to be cardioprotective and beneficial for vascular function. We report two elderly patients with systemic ANCA-associated vasculitis (AAV) in whom the administration of EPA in concert with steroids safely induced and maintained remission, without the use of additioal immunosuppressants. To explore the mechanisms by which EPA enhances the treatment of AAV, we employed SCG/Kj mice as a spontaneous murine model of AAV. Dietary enrichment with EPA significantly delayed the onset of crescentic glomerulonephritis and prolonged the overall survival. EPA-derived anti-inflammatory lipid mediators and their precursors were present in the kidney, plasma, spleen, and lungs in the EPA-treated mice. Furthermore, a decrease in ANCA production and CD4/CD8-double negative T cells, and an increase in Foxp3+ regulatory T cells in the lymph nodes of the kidney were observed in the EPA-treated mice. These clinical and experimental observations suggest that EPA can safely support and augment conventional therapy for treating autoimmune small-vessel vasculitis. PMID:25230773

  19. Cutaneous lymphocytic vasculitis: a definition, a review, and a proposed classification.

    PubMed

    Carlson, J A; Mihm, M C; LeBoit, P E

    1996-02-01

    Lymphocytic vasculitis is not widely accepted as a pathologic mechanism by dermatopathologists, and a comprehensive list of its causes cannot be found in the literature. This state of affairs stems largely from the lack of a rigorous definition. In this report, the authors review past efforts at coming to terms with lymphocytic vasculitis and why those efforts have fallen short. The authors propose that lymphocytic vasculitis can be separated from the ubiquitous perivascular dermatitides in routinely processed specimens by requiring the presence of either acute or chronic damage to the walls of small vessels (eg, fibrin deposition, lamination by pericytes). In the case of muscular vessels, the presence of lymphocytes within the vessel walls is sufficient, because diapedesis of lymphocytes does not occur in arteries or veins. Although lymphocytic infiltrates meeting this definition are uncommon, there are a number of conditions, with both typical and atypical lymphocytes, in which damage to vessels occurs. The authors review these conditions, outline possible pathogenetic mechanisms, ranging from delayed hypersensitivity reactions directed against endothelial cells to direct infection of these cells, and present classifications based on morphological changes and pathogenesis respectively. PMID:8834516

  20. Morphologic features and development of granulomatous vasculitis in feline infectious peritonitis.

    PubMed

    Kipar, A; May, H; Menger, S; Weber, M; Leukert, W; Reinacher, M

    2005-05-01

    Feline infectious peritonitis (FIP) is a fatal, coronavirus (CoV)-induced systemic disease in cats, characterized by granulomas in organs and granulomatous vasculitis. This study describes the morphologic features of granulomatous vasculitis in FIP as well as its development in the course of monocyte-associated feline CoV (FCoV) viremia in five naturally infected Domestic Shorthair cats with FIP. Monocyte-associated FCoV viremia was demonstrated by immunohistology, RNA in situ hybridization, and electron micropscopy. Granulomatous phlebitis at different stages of development was observed. Vasculitic processes ranged from attachment and emigration of FCoV-infected monocytes to vascular/perivascular granulomatous infiltrates with destruction of the vascular basal lamina. Monocytes as well as perivascular macrophages were activated because they were strongly positive for CD18 and expressed cytokines (tumor necrosis factor-alpha and interleukin-1beta) and matrix metalloproteinase-9. In addition, general activation of endothelial cells, represented by major histocompatibility complex II upregulation, was observed in all cases. These results confirm FIP as a monocyte-triggered systemic disease and demonstrate the central role of activated monocytes in FIP vasculitis. PMID:15872378

  1. [Pulmonary Nocardiosis due to Nocardia asiatica in a Patient with ANCA-associated Vasculitis].

    PubMed

    Suemori, Koichiro; Miyamoto, Hitoshi; Murakami, Shinobu; Yamazaki, Hitoshi; Ishizaki, Jun; Matsumoto, Takuya; Murakami, Yuichi; Hasegawa, Hitoshi; Yasukawa, Masaki

    2015-07-01

    Nocardia asiatica is a rare causative organism responsible for opportunistic infection, and was first reported by Kageyama et al. in 2004. We report herein on a very rare case of N. asiatica infection in a 76-year old male patient with ANCA-associated vasculitis and a history of pulmonary tuberculosis. The patient developed pulmonary nocardiosis due to N. asiatica while receiving glucocorticoid therapy. Chest computed tomography demonstrated multiple granules and cavity formation mainly in the left lower lobe. From the images, we suspected opportunistic infection, possibly pulmonary tuberculosis or pulmonary damage due to ANCA-associated vasculitis. Nocardia sp. was detected from a bronchoalveolar lavage culture and N. asiatica was identified by 16S ribosomal DNA gene sequencing. Cranial magnetic resonance imaging revealed no abnormality. Administration of Doripenem (1.5g/day) and sulfamethoxazole-trimethoprim (4g/day) was started, and the patient's clinical and imaging findings promptly improved. Thereafter, he received sulfamethoxazole-trimethoprim (2g/day) and prednisolone (10 mg/day) as maintenance therapy for ANCA-associated vasculitis for more than one year, and there has since been no recurrence of the Nocardia infection. PMID:26554223

  2. Histopathological Characteristics of Post-inflamed Coronary Arteries in Kawasaki Disease-like Vasculitis of Rabbits

    PubMed Central

    Fujii, Maiko; Tanaka, Hideo; Nakamura, Akihiro; Suzuki, Chinatsu; Harada, Yoshinori; Takamatsu, Tetsuro; Hamaoka, Kenji

    2016-01-01

    Kawasaki disease (KD) is a systemic vasculitis in infants that develops predominantly in the coronary arteries. Despite the clinically transient nature of active inflammation in childhood albeit rare complications (e.g., coronary artery aneurysm), KD has recently been suggested to increase the incidence of ischemic heart diseases in young adulthood. However, little is known about the histopathology of the coronary artery long after development of the acute KD vasculitis. To address this, we conducted histological studies of rabbit coronary arteries in adolescent phase after induction of the KD-like vasculitis induced by horse serum administration. After a transmural infiltration of inflammatory cells in acute phase at day 7, the artery exhibited a gradual decrease in the number of inflammatory cells and thickening of the intima during the chronic phase up to day 90, where proteoglycans were distinctly accumulated in the intima with abundant involvement of α-smooth muscle actin (α-SMA)-positive cells, most of which accompanied expression of VCAM-1 and NF-κB. Distinct from classical atherosclerosis, inflammatory cells, e.g., macrophages, were barely detected during the chronic phase. These observations indicate that the KD-like coronary arteritis is followed by intimal thickening via accumulation of proteoglycans and proliferation of α-SMA-positive cells, reflecting aberrant coronary artery remodeling. PMID:27006519

  3. Goodpasture's Syndrome and p-ANCA Associated Vasculitis in a Patient of Silicosiderosis: An Unusual Association

    PubMed Central

    Bal, Amanjit; Das, Ashim; Gupta, Dheeraj; Garg, Mandeep

    2014-01-01

    Introduction. Goodpasture's syndrome is a rare clinical entity and is characterized by circulating autoantibodies which are principally directed against the glomerular/alveolar basement membrane. The etiology of Goodpasture's syndrome is still unknown. Lung involvement occurs as a result of lung injury and the exposure of new epitopes to the immune system. Recently, several studies have suggested the role of silica as one of etiological factors in ANCA associated vasculitis and glomerulonephritis. Materials and Methods. We present a case of a 40-year-old welder with silicosiderosis, who developed anti-GBM disease with p-ANCA positivity. Case Report. Patient presented to an emergency with gradually increasing breathlessness along with renal failure and died after short hospital stay. Autopsy pathology findings revealed crescentic glomerulonephritis with linear glomerular basement membrane antibody deposition, splenic vasculitis, pulmonary haemorrhage, and pulmonary silicosiderosis. Conclusion. This case reinforces the role of environmental triggers like exposure to silica, metal dust, and tobacco in pathogenesis of Goodpasture's syndrome and p-ANCA associated vasculitis. PMID:25349763

  4. Antigen inhalation as a triggering factor in systemic small-sized-vessel vasculitis. Four cases.

    PubMed

    Mouthon, L; Khaled, M; Cohen, P; Subra, J F; Guillevin, L

    2001-04-01

    In order to identify small-sized-vessel vasculitis occurring immediately after massive inhalation of particles which could be considered antigenic, we performed a retrospective review of patients who consulted our Department of Internal Medicine between 1980 and 1998 and were diagnosed as having small-vessel vasculitis that developed immediately after massive exposure to inhaled particles. Four patients, 27 to 55 years old, presented with small-sized-vessel vasculitis (one with Wegener's granulomatosis and three with Churg-Strauss syndrome) which developed after massive inhalation of particles. Clinical manifestations occurred from a few hours to 10 days after inhalation of diesel fumes, cereal dust, flour or cereal dust and/or pigeon droppings. Three patients remain disease-free 8, 9 and 18 years after discontinuing treatment. One patient remains well after 3 years on low-dose corticosteroids and cyclophosphamide therapy. Our data suggest that primary antigens can cause small-sized-vessel vasculitides. Identifying such etiological factors could improve our understanding of the pathogenesis of systemic vasculitides and prevent relapses due to similar antigenic exposure. PMID:11431572

  5. A role for mast cells in the development of adjuvant-induced vasculitis and arthritis.

    PubMed Central

    Johnston, B.; Burns, A. R.; Kubes, P.

    1998-01-01

    The objective of this study was to characterize the role of mast cells in the development of vasculitis and joint swelling in adjuvant-immunized rats. Leukocyte trafficking within mesenteric venules (rolling and adhesion) and mast cell activation (ruthenium red uptake) were examined in vivo. Elevated leukocyte trafficking was observed by 4 days after immunization, whereas joint swelling developed between days 10 and 12. Perivascular mast cells took up ruthenium red and appeared activated by electron microscopy at 4 but not 12 days after immunization. Treatment with the mast cell stabilizer cromolyn on days 1 to 4 after immunization blocked ruthenium red uptake at day 4 and reduced leukocyte rolling and adhesion by approximately 50%. This treatment also reduced rolling, adhesion, and joint swelling at day 12 by approximately 50%. Cromolyn treatment over days 9 to 12 reduced joint swelling but increased leukocyte emigration into the mesentery. Peritoneal mast cells isolated 4 days after immunization elicited significant neutrophil chemotaxis in vitro, whereas day 12 mast cells did not. Mast cell activation and vasculitis were absent in adjuvant-resistant Fisher/344 rats. These data suggest that mast cells play an early role in the initiation of vasculitis and may function by day 12 to limit infiltration of leukocytes from the vasculature. In the joint, however, mast cells appear to contribute to inflammation at early as well as later time points. Images Figure 2 PMID:9466582

  6. EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis.

    PubMed

    Yates, M; Watts, R A; Bajema, I M; Cid, M C; Crestani, B; Hauser, T; Hellmich, B; Holle, J U; Laudien, M; Little, M A; Luqmani, R A; Mahr, A; Merkel, P A; Mills, J; Mooney, J; Segelmark, M; Tesar, V; Westman, K; Vaglio, A; Yalçındağ, N; Jayne, D R; Mukhtyar, C

    2016-09-01

    In this article, the 2009 European League Against Rheumatism (EULAR) recommendations for the management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) have been updated. The 2009 recommendations were on the management of primary small and medium vessel vasculitis. The 2015 update has been developed by an international task force representing EULAR, the European Renal Association and the European Vasculitis Society (EUVAS). The recommendations are based upon evidence from systematic literature reviews, as well as expert opinion where appropriate. The evidence presented was discussed and summarised by the experts in the course of a consensus-finding and voting process. Levels of evidence and grades of recommendations were derived and levels of agreement (strengths of recommendations) determined. In addition to the voting by the task force members, the relevance of the recommendations was assessed by an online voting survey among members of EUVAS. Fifteen recommendations were developed, covering general aspects, such as attaining remission and the need for shared decision making between clinicians and patients. More specific items relate to starting immunosuppressive therapy in combination with glucocorticoids to induce remission, followed by a period of remission maintenance; for remission induction in life-threatening or organ-threatening AAV, cyclophosphamide and rituximab are considered to have similar efficacy; plasma exchange which is recommended, where licensed, in the setting of rapidly progressive renal failure or severe diffuse pulmonary haemorrhage. These recommendations are intended for use by healthcare professionals, doctors in specialist training, medical students, pharmaceutical industries and drug regulatory organisations. PMID:27338776

  7. [Immunocorrection in combined prevention of early postoperative complications in patients with systemic vasculitis].

    PubMed

    Andrievskikh, I A; Omel'ianiuk, M Iu

    2015-01-01

    The authors studied peculiarities of the immune status in angiosurgical patients with systemic vasculitis, as well as possibilities of immunodiagnosis and immunocorrection in prevention of early coagulopathic and reparative complications after angiosurgical interventions in this cohort of patients. A total of 172 angiosurgical patients presenting with systemic vasculitis were subdivided into two groups depending on the preoperative preparation methods used. In Group One (Study Group) comprising 81 patients preoperative preparation was carried out using immunosuppressive therapy with hormones and cytostatics according to the rheumatologist's indications. In Group Two (n=91) hormones and/or cytostatics were replaced by the proposed four-component immunocorrection including various combinations of correcting the lifestyle, use of antioxidative-activity immunomodulators, plasmapheresis and intravenous administration of immunoglobulins. It was determined that using this method of correction made it possible to achieve a good anti-inflammatory effect in angiosurgical patients with systemic vasculitis, thus avoiding negative aftermaths of immunosuppression. The developed method of immunocorrection makes it possible to decrease the rate of early postoperative coagulopathic and reparative complications in angiosurgical patients as compared to therapy with hormones and/or cytostatics. PMID:26673291

  8. A novel immunopathological association of IgG4-RD and vasculitis with Hashimoto's thyroiditis

    PubMed Central

    Minamino, Hiroto; Ariyasu, Hiroyuki; Furuta, Hiroto; Nishi, Masahiro; Yoshimasu, Takashi; Nishikawa, Akinori; Nakanishi, Masanori; Tsuchihashi, Shigeki; Kojima, Fumiyoshi; Murata, Shin-ichi; Inoue, Gen; Akamizu, Takashi

    2016-01-01

    Summary A 73-year-old man with Hashimoto's thyroiditis (HT) suffered from purpura on the lower legs. He was diagnosed with IgG4-related disease (IgG4-RD) with serum IgG4 elevation and dacryo-sialadenitis confirmed histologically. Serum Th2 and Treg cytokines, interleukin 7 (IL7), IL8 and Th2 chemokine levels were elevated, while skewed Th1 balance was seen in fluorescence-activated cell sorting (FACS). Therefore, preferential Th1 balance in HT appeared to be followed by IgG4-RD characterized with Th2 and Treg polarization. The commencement of steroid therapy dramatically exacerbated clinical manifestations including IgG4-RD-associated HT. The measurement of cytokine and chemokine levels as well as FACS analysis in the development of IgG4-RD seemed to be beneficial. In conclusion, an innovative association of HT, IgG4-RD and vasculitis was observed. This report also offers novel diagnostic and therapeutic approaches for IgG4-RD. Learning points Recently, a subtype of HT has been considered to be a thyroid manifestation of IgG4-RD, although the etiology of IgG4-RD is not established yet. Immunologically a close association between HT and vasculitis was reported. Leukocytoclastic vasculitis is a rare skin presentation of IgG4-RD. In the current case, during the course of HT, IgG4-RD and leukocytoclastic vasculitis occurred; thus, innate immunity and acquired immunity seem to be involved in the development of IgG4-RD. The measurement of cytokine and chemokines appeared to be beneficial in the development of IgG4-RD. Remarkably, effectiveness of steroid therapy for HT suggested presence of IgG4-RD-associated HT. Therefore, this report highlights the pathogenesis of IgG4-RD and proposes novel therapeutic mechanisms. Clinicians should pay attention to the development of IgG4-RD and vasculitis during long course of HT. PMID:26966543

  9. Testicular vasculitis: findings differentiating isolated disease from systemic disease in 72 patients.

    PubMed

    Hernández-Rodríguez, José; Tan, Carmela D; Koening, Curry L; Khasnis, Atul; Rodríguez, E René; Hoffman, Gary S

    2012-03-01

    Testicular vasculitis (TV) may be part of systemic (testicular) vasculitis (STV) or may exist as single-organ/isolated (testicular) vasculitis (ITV). In the current study we sought to identify clinical and histologic features that distinguish STV from ITV. The distinction was deemed important because it is already well established that in other forms of single organ vasculitis, surgical therapy alone may be curative. We identified patients with biopsy-proven TV from pathology databases from our institution and from an English-language PubMed search. Patients were included if data were available to determine TV extent confidently. Data recorded included clinical, laboratory, and histologic features; treatment; and clinical follow-up. The study included 72 patients with TV (mean age, 42 yr; range, 4-78 yr) (7 from our institution). About 74% of patients presented with painful testicular swelling/mass, 10% with a painless testicular swelling/mass, and 4% with epididymal swelling/mass. Eleven percent had no testicular complaints and vasculitis was discovered at autopsy or in other surgical interventions. Vasculitis involved the testicle in 80.3% of cases, the epididymis in 44.6%, and the spermatic cord in 30.6%. Thirty-seven (51%) patients had ITV and 35 (49%) had STV. No differences between ITV and STV patients were found in regards to age, presenting testicular features, duration of testicular symptoms, and time of follow-up. Compared to ITV patients, STV patients presented more often with constitutional/musculoskeletal symptoms (74.3% vs. 8.3%, respectively; p = 0.0001), elevated erythrocyte sedimentation rate (94.7% vs. 16%; p = 0.0001), and anemia (50% vs. 0%; p = 0.0001). Neoplasm was more frequently suspected in ITV than in STV (74.2% vs. 31.6%; p = 0.001), but only occurred in 2 ITV patients. Long-term glucocorticoid therapy was given only to STV patients, and 59.1% of them also received cytotoxic agents. ITV was diagnosed more often by orchiectomy (81.1% vs

  10. Fiber optic LDF to monitor vascular dynamics of urticarial dermographism in pressure-tested patients before and after treatment with antihistamines

    NASA Astrophysics Data System (ADS)

    Eikje, Natalja Skrebova; Arase, Seiji

    2008-02-01

    The local microcirculatory dynamics underlying phenomenon of urticarial dermographism (UD) are not yet sufficiently elucidated in dermatological patients. A fiber optic laser Doppler flowmeter (LDF) was used to monitor skin blood flow (SBF) changes on the back of the patients with UD before and after application of the series of pressure stimuli (9.8×10 4, 14.7×10 4, 19.6×10 4 and 24.5×10 4 Pa). All patients acted as self-controls to assess their disease activity by means of SBF values based on response to pressure stimuli before and after treatment with antihistamines, when compared to baseline SBF. Throughout 30 minutes evaluation inter-subject SBF values at pressure-tested sites were noticeably distinguished as high, moderate and low. By LDF we could differentiate the highest development of vascular dynamics after 5 minutes, coming back to normal within about 30 minutes in one group of patients, and the vascular dynamics reaching its maximum in 15 minutes, but with no fade after 30 minutes, in another group of patients. All treatment regimens in both groups of patients by LDF produced a measurable reduction already during 1-2 days of therapy, accompanied by a reduction in SBF baseline values in patients with severe and moderate symptoms of UD.

  11. Histopathology of the Inner Ear in a Case With Recent Onset of Cogan's Syndrome: Evidence for Vasculitis.

    PubMed

    Jung, David H; Nadol, Joseph B; Folkerth, Rebecca D; Merola, Joseph F

    2016-01-01

    The association of sensorineural hearing loss and vertigo with inflammatory eye disease, usually interstitial keratitis, has been called Cogan's syndrome. The pathogenesis of Cogan's syndrome is unknown, but it has been assumed to be an immune mediated disorder with vasculitis. The histopathology of the inner ear in Cogan's syndrome has been described in 6 case reports. Although common pathologic findings in these reports include degeneration of the auditory and vestibular neuroepithelium, endolymphatic hydrops, fibrosis, and new bone formation, direct pathologic evidence of a vasculitis has not been published. A possible reason for this failure to identify vasculitis was a substantial delay (range, 4-40 years) between the onset of symptoms and examination of the otopathology. In the current case report, the patient had both auditory and vestibular symptoms and interstitial keratitis with a time delay of only 2 to 4 weeks between symptoms and death. Evidence of a vasculitis as a possible underlying etiology included H&E histopathology and anti-CD45 immunostaining of vessels both in the auditory and vestibular systems, supporting the hypothesis of a vasculitis as a mechanism in this disorder. PMID:26195577

  12. Cocaine/levamisole-induced systemic vasculitis with retiform purpura and pauci-immune glomerulonephritis

    PubMed Central

    Veronese, F.V.; Dode, R.S.O.; Friderichs, M.; Thomé, G.G.; da Silva, D.R.; Schaefer, P.G.; Sebben, V.C.; Nicolella, A.R.; Barros, E.J.G.

    2016-01-01

    Levamisole has been increasingly used as an adulterant of cocaine in recent years, emerging as a public health challenge worldwide. Levamisole-associated toxicity manifests clinically as a systemic vasculitis, consisting of cutaneous, hematological, and renal lesions, among others. Purpura retiform, cutaneous necrosis, intravascular thrombosis, neutropenia, and less commonly crescentic nephritis have been described in association with anti-neutrophil cytoplasmic antibodies (ANCAs) and other autoantibodies. Here we report the case of a 49-year-old male who was a chronic cocaine user, and who presented spontaneous weight loss, arthralgia, and 3 weeks before admission purpuric skin lesions in the earlobes and in the anterior thighs. His laboratory tests on admission showed serum creatinine of 4.56 mg/dL, white blood count 3,800/μL, hemoglobin 7.3 g/dL, urinalysis with 51 white blood cells/μL and 960 red blood cells/μL, and urine protein-to-creatinine ratio 1.20. Serum ANCA testing was positive (>1:320), as well as serum anti-myeloperoxidase and anti-proteinase 3 antibodies. Urine toxicology screen was positive for cocaine and levamisole, with 62.8% of cocaine, 32.2% of levamisole, and 5% of an unidentified substance. Skin and renal biopsies were diagnostic for leukocytoclastic vasculitis and pauci-immune crescentic glomerulonephritis, respectively. The patient showed a good clinical response to cocaine abstinence, and use of corticosteroids and intravenous cyclophosphamide. Last serum creatinine was 1.97 mg/dL, white blood cell count 7,420/μL, and hemoglobin level 10.8 g/dL. In levamisole-induced systemic vasculitis, the early institution of cocaine abstinence, concomitant with the use of immunosuppressive drugs in severe cases, may prevent permanent end organ damage and associate with better clinical outcomes. PMID:27119429

  13. The predictive value of kidney biopsy in renal vasculitis: a multicenter cohort study.

    PubMed

    Diaz-Crespo, Francisco; Villacorta, Javier; Acevedo, Mercedes; Cavero, Teresa; Guerrero, Carmen; García Díaz, Eugenio; Orradre, Juan Luis; Martinez, Miguel Angel; Praga, Manuel; Fernandez-Juarez, Gema

    2016-06-01

    The histopathologic classification of antineutrophil cytoplasmic antibody-associated vasculitis has been demonstrated to have prognostic value in small cohorts of patients with pauci-immune extracapillary glomerulonephritis. We aimed to validate this histologic subgrouping system in a large cohort of patients with renal vasculitis from 3 Spanish centers. The additional value of several histologic parameters for predicting renal outcome was investigated. A total of 151 biopsies of patients with renal vasculitis were reviewed and classified as follows: 41% crescentic, 24% mixed, 21% focal, and 14% sclerotic. The cumulative proportions of renal survival at 5 years were 83.2%, 81.2%, 60.5%, and 50.7% for the focal, mixed, crescentic, and sclerotic categories, respectively (P < .05). In the crescentic category, patients with less than 75% of glomeruli showing crescents had better survival at 1 and 5 years compared with those having greater than or equal to 75% of crescents (77.9% and 70.6% versus 51.3% and 45.6%; P = .02). When adjusted by renal function and other histologic parameters, the percentage of extracapillary proliferation and glomerulosclerosis remained as significant predictors for renal survival (hazard ratio, 1.03; 95% confidence interval, 1.01-1.05; P = .001, and hazard ratio, 1.03; 95% confidence interval, 1.01-1.05; P = .002, respectively). In conclusion, patients with pauci-immune crescentic glomerulonephritis experienced different outcomes depending on the percentage of crescents observed, so that extensive extracapillary proliferation was associated with the poorest renal survival. These findings validate the prognostic utility of the histologic classification scheme in antineutrophil cytoplasmic antibody positive and negative patients and suggest a subdivision of crescentic category (<75% and ≥75% of crescents) based on the different survival rates observed among these subgroups. PMID:26980047

  14. Dysregulation of Autoantigen Genes in ANCA-Associated Vasculitis Involves Alternative Transcripts and New Protein Synthesis

    PubMed Central

    McInnis, Elizabeth A.; Badhwar, Anshul K.; Muthigi, Akhil; Lardinois, Olivier M.; Allred, S. Colby; Yang, Jiajin; Free, Meghan E.; Jennette, J. Charles; Preston, Gloria A.; Falk, Ronald J.

    2015-01-01

    Proteinase 3 (PR3) and myeloperoxidase (MPO) are two major autoantigens in patients with vasculitis with ANCA. The genes encoding these autoantigens are abnormally expressed in peripheral granulocytes of patients with active ANCA-associated vasculitis. This study provides evidence that this transcriptional dysregulation results in a variety of mRNA processing events from the PRTN3 gene locus. In addition to elevated levels of PR3 message, leukocyte RNA from patients contained PR3 transcripts with an alternative 3′ untranslated region. Furthermore, we detected usage of an alternative transcription start site within intron 1 of the PRTN3 gene locus that coincided with active disease (odds ratio, 3.3; 95% confidence interval, 1.3 to 8.4; P=0.01). This promoter may be developmentally regulated, because it was active in normal human bone marrow, multiple leukemia cell lines, MCF-7 cells, and subjects after GM-CSF treatment but not subjects with a neutrophil left shift. This transcript, which lacks exon 1 of PRTN3, encodes a 24-kD protein (p24PR3/MBN) with a sequence similar to that previously described for myeloblastin. Notably, PR3, p24PR3/MBN, and MPO were synthesized in cultured neutrophils from patients with active ANCA-associated vasculitis, indicating that increased transcription results in newly synthesized autoantigens in peripheral neutrophils of patients. The synthesis of p24PR3/MBN seems to expand the autoantigen repertoire, because immunoblots showed that sera from patients recognized p24PR3/MBN. These findings emphasize the importance of transcriptional dysregulation of the autoantigen in autoimmune disease. PMID:25060059

  15. Circulating Markers of Vascular Injury and Angiogenesis in ANCA-Associated Vasculitis

    PubMed Central

    Monach, Paul A; Tomasson, Gunnar; Specks, Ulrich; Stone, John H; Cuthbertson, David; Krischer, Jeffrey; Ding, Linna; Fervenza, Fernando C; Fessler, Barri J; Hoffman, Gary S; Ikle, David; Kallenberg, Cees GM; Langford, Carol A; Mueller, Mark; Seo, Philip; St.Clair, E William; Spiera, Robert; Tchao, Nadia; Ytterberg, Steven R; Gu, Yi-Zhong; Snyder, Ronald D; Merkel, Peter A

    2011-01-01

    Objective To identify biomarkers that distinguish between active ANCA-associated vasculitis (AAV) and remission in a manner superior or complementary to established markers of systemic inflammation. Methods Markers of vascular injury and angiogenesis were measured before and after treatment in a large clinical trial in AAV. 163 subjects enrolled in the Rituximab in ANCA-Associated Vasculitis (RAVE) trial were studied. Serum levels of E-selectin, ICAM-3, MMP1, MMP3, MMP9, P-selectin, thrombomodulin, and VEGF were measured at study screening (time of active disease) and at month 6. ESR and CRP levels had been measured at the time of the clinical visit. The primary outcome was the difference in marker level between screening and month 6 among patients in remission (BVAS/WG score of 0) at month 6. Results All subjects had severe active vasculitis (mean BVAS/WG score 8.6 +/− 3.2 SD) at screening. Among the 123 subjects clinically in remission at month 6, levels of all markers except E-selectin showed significant declines. MMP3 levels were also higher among the 23 subjects with active disease at month 6 than among the 123 subjects in remission. MMP3 levels correlated weakly with ESR and CRP. Conclusion Many markers of vascular injury and angiogenesis are elevated in severe active AAV and decline with treatment, but MMP3 appears to distinguish active AAV from remission better than the other markers studied. Further study of MMP3 is warranted to determine its clinical utility in combination with conventional markers of inflammation and ANCA titers. PMID:21953143

  16. Traumatic endophthalmitis presenting as isolated retinal vasculitis and white-centered hemorrhages: Case report and review of literature

    PubMed Central

    Relhan, Nidhi; Jalali, Subhadra; Nalamada, Suma; Dave, Vivek; Mathai, Annie

    2012-01-01

    The article reports a case and review of the literature of endophthalmitis presenting as isolated retinal vasculitis. A 26-year-old male was observed to have white-centered retinal hemorrhages and retinal vasculitis following an occult scleral perforation. At presentation, the visual acuity was 20/60. With clinical suspicion of early endophthalmitis, he underwent wound exploration, scleral tear repair, vitreous biopsy and administration of intravitreal antibiotics. Microbiology evaluation revealed significant presence of methicillin-resistant coagulase-negative Staphylococcus epidermidis. Final visual acuity improved to 20/20 at 6 weeks postoperatively. Literature search revealed eight similar cases, all of them due to Staphylococcus species. Retinal vasculitis and white-centered retinal hemorrhages can be a presenting sign of early endophthalmitis, especially with non-fulminant pathogens like S. epidermidis. PMID:22824604

  17. Studies of epitope restriction on myeloperoxidase (MPO), an important antigen in systemic vasculitis

    PubMed Central

    SHORT, A K; LOCKWOOD, C M

    1997-01-01

    Anti-neutrophil cytoplasmic antibodies are important components of the inflammatory response in patients with systemic vasculitis. Their role in the pathogenesis of these conditions remains incompletely defined. Several antigens have been identified, and MPO is one of the most important. To gain more understanding of the immune mechanisms involved, we were keen to see if the antibody response to MPO was restricted, or whether there was a general loss of tolerance to the whole surface of the molecule. To study the epitopes we employed both ELISA and biosensor technology, and were able to demonstrate restriction both in the number and localization of the epitopes being recognized. PMID:9367412

  18. Hemorrhagic Occlusive Retinal Vasculitis After First Eye Cataract Surgery Without Subsequent Second Eye Involvement.

    PubMed

    Balducci, Nicole; Savini, Giacomo; Barboni, Piero; Ducoli, Pietro; Ciardella, Antonio

    2016-08-01

    A case of monocular postoperative hemorrhagic occlusive retinal vasculitis (HORV) after uncomplicated bilateral cataract surgery is described. HORV is a severe syndrome that leads to painless visual loss after uncomplicated cataract surgery. The same surgical procedure was adopted in both eyes except for the use of intracameral vancomycin, which was injected only in the eye that developed HORV. Diffuse retinal ischemia with vascular sheathing and intraretinal hemorrhages were detected during the fourth postoperative day. Despite treatment, the patient developed severe neovascular glaucoma. This case supports the causative role of vancomycin in the pathogenesis of HORV and suggests avoiding it for chemoprophylaxis. [Ophthalmic Surg Lasers Imaging Retina. 2016;47:764-766.]. PMID:27548454

  19. Erdheim-Chester Disease: An Unusual Cause of Intracranial Vasculitis and Progressive Leukoencephalopathy.

    PubMed

    Sagnier, Sharmila; Debruxelles, Sabrina; Lepreux, Sébastien; Sibon, Igor

    2016-05-01

    Erdheim-Chester disease (ECD) is a non-Langerhans histiocytosis affecting multiple organs. Stroke as symptom onset of ECD with intracranial vasculitis is unusual. We report the case of a 64-year-old man who presented with an acute ischemic stroke associated with a moderate leukoencephalopathy and intracranial arteries stenosis. Four years later, he developed movement disorders with dysarthria and cognitive impairment. Neuroradiological findings demonstrated a rapidly progressive and diffuse leukoencephalopathy associated with brain atrophy and infiltration of the intracranial vertebral artery wall. Brain postmortem evaluation confirmed the diagnosis of ECD. This diagnosis should be evoked in patients with cryptogenic stroke, progressive leukoencephalopathy, and infiltration of the arterial wall. PMID:26996751

  20. A case report and DSA findings of cerebral hemorrhage caused by syphilitic vasculitis.

    PubMed

    Zhang, Xia; Xiao, Guo-Dong; Xu, Xing-Shun; Zhang, Chun-Yuan; Liu, Chun-Feng; Cao, Yong-Jun

    2012-12-01

    Syphilis is now rare and easily misdiagnosed because of the wide use of antibiotics in the clinical. We report a case of cerebral hemorrhage in a patient with hypertension who was first diagnosed as hypertensive cerebral hemorrhage. However, treponema pallidum particle agglutination and rapid plasma regain tests of cerebrospinal fluid revealed the existence of neurosyphilis. Interestingly, digital subtraction angiography (DSA) showed severe stenosis in both middle cerebral arteries and right anterior cerebral artery. The case reminded us to pay attention to syphilitic vasculitis in patients with cryptogenic stroke. DSA sometimes may play a critical role in differential diagnosis of neurosyphilis. PMID:22198645

  1. Collapsing glomerulopathy in a case of anti-neutrophil cytoplasmic antibody associated vasculitis

    PubMed Central

    Singh, N.; Rathi, M.; Nada, R.; Sharma, A.; Goyal, A.; Ramachandran, R.; Kumar, V.; Kohli, H. S.; Gupta, K. L.

    2016-01-01

    Collapsing glomerulopathy (CG) is a pathological entity characterized by collapse and wrinkling of glomerular tuft, podocyte dedifferentiation and hyperplasia. CG may be idiopathic or secondary to other diseases. CG has been described with IgA nephropathy, membranous glomerulopathy, diabetic nephropathy, and lupus nephritis. However, till date there is no report of CG in association with the anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV). Here, we present a case of CG that developed during follow-up in a case of AAV with biopsy proven pauci-immune glomerulonephritis. PMID:27051140

  2. Severe meningeal fibrinoid vasculitis associated with Theileria taurotragi infection in two short-horned Zebu cattle.

    PubMed

    Biasibetti, Elena; Sferra, Chiara; Lynen, Godelieve; Di Giulio, Giuseppe; De Meneghi, Daniele; Tomassone, Laura; Valenza, Federico; Capucchio, Maria Teresa

    2016-08-01

    The Authors describe a severe vasculitis with fibrinoid necrosis of the meningeal arteries observed in two brains of indigenous short-horn zebu (Bos indicus) cattle, with bovine cerebral theileriosis (BCT) caused by a tick-transmitted hemoprotozoan, Theileria taurotragi, from Northern Tanzania. In the Author's opinion, the role of T. taurotragi infection in the angiocentric and angiodestructive detected features remains to be evaluated. A possible immunopathologic cancerous mechanism, secondary to the lymphoid deregulation, could be involved. This report suggests further studies to better characterize the lymphoid cell involvement in the pathogenesis of the meningeal vascular lesions by T. taurotragi. PMID:27145963

  3. Sarcoid Pericarditis and Large Vessel Vasculitis Detected on FDG PET/CT.

    PubMed

    Patel, Darshan; Xie, Karen; Sweiss, Nadera J; Lu, Yang

    2016-08-01

    A 65-year-old woman with history of sarcoidosis, hypertension, and peripheral vascular disease presented with lower extremity claudication and low left ventricular ejection fraction of 45% on echocardiogram. Further FDG PET/CT revealed hypermetabolic bilateral chest lymphadenopathy and pulmonary nodules in a butterfly-shaped distribution pattern, which was typically seen in patients with sarcoidosis. In addition, abnormal increased radiotracer uptake was present in pericardium, and along the walls of inferior vena cava, aorta, and pulmonary artery, which correlated with delayed enhancement on further cardiac MRI. These findings were suggestive of sarcoid-related pericarditis and great vessel vasculitis. PMID:27280907

  4. Levamisole adulterated cocaine and pulmonary vasculitis: Presentation of two lethal cases and brief literature review.

    PubMed

    Karch, Steven B; Busardò, Francesco Paolo; Vaiano, Fabio; Portelli, Francesca; Zaami, Simona; Bertol, Elisabetta

    2016-08-01

    The first case reports of levamisole-related disease in cocaine users were published in 2010, although levamisole adulteration of cocaine was first recognized several years earlier. Currently, more than 70% of street cocaine seizures, in the US and the EU, contain levamisole, which could potentially be converted to aminorex, though the reasons for this practice still remain obscure. Here we report two fatal cases of isolated pulmonary vasculitis in abusers of levamisole-adulterated cocaine, where a complete autopsy, full toxicological analysis by gas chromatography-mass spectrometry (GC-MS) using a previously published method of Karch et al. and histological examination were performed. A control group composed of 11 cases of cocaine related deaths, where the presence of levamisole was excluded in blood, urine and hair, was used. Recent literature on the human pharmacokinetics of levamisole and aminorex is also reviewed. The toxicological analysis revealed positive qualitative and quantitative results for cocaine, benzoylecgonine and levamisole in both cases. In case 1 levamisole was found at the concentration of 13.5 and 61.3mg/L in blood and urine respectively, whereas in case 2 at 17.9 and 70.2mg/L. The histological examination highlighted in case 1 in heart samples microscopic evidence of the typical remodeling changes associated with chronic stimulant abuse, whereas lungs showed numerous lymphocytes surrounding and infiltrating the wall of small pulmonary vessels and a perivascular fibrosis with transforming fibroblasts. In case 2, the myocardial samples showed wide fields of myocardial necrosis characterized by hypercontraction of the myocytes with thickened Z-lines and short sarcomeres, whereas lung samples showed a significant intimal thickening of arteriole walls and lymphocytic infiltration of the wall and edema. Moreover, there were also numerous perivascular lymphocytic infiltrates. Although the pathological cardiac findings have allowed us to establish

  5. Large vessel vasculitis in a patient with acute Q-fever: A case report

    PubMed Central

    Baziaka, Fotini; Karaiskos, Ilias; Galani, Lamprini; Barmpouti, Eleftheria; Konstantinidis, Stilianos; Kitas, George; Giamarellou, Helen

    2014-01-01

    Q fever is a zoonosis caused by the rickettsial organism Coxiella burnetii. Infection has an acute course, usually with a self-limited febrile illness and the possibility of the evaluation to a chronic course with endocardial involvement. The presence of autoantibodies and various autoimmune disorders have also been associated with C. burnetii infection. We report a case of acute Q fever in which the patient developed large vessel vasculitis. The FDG-PET/CT scan detected inflammation of the thoracic aortic wall, suggesting an unusual immunologic host response to acute Q fever infection. PMID:26952153

  6. Angiooedema due to acquired deficiency of C1-esterase inhibitor associated with leucocytoclastic vasculitis.

    PubMed

    Farkas, H; Szongoth, M; Bély, M; Varga, L; Fekete, B; Karádi, I; Füst, G

    2001-01-01

    A hereditary and an acquired type of C1-esterase inhibitor deficiency have been described. Manifestations characteristic of both forms include recurrent subcutaneous and submucosal angiooedema. Acquired C1-esterase inhibitor deficiency has been observed in association with lymphoproliferative disorders, malignancy, autoimmune diseases and infections. We report on a case with the acquired form of the disease accompanied by leucocytoclastic vasculitis. Treatment with antimalarial agents resulted in complete resolution of symptoms and signs. Furthermore, C1-esterase inhibitor concentration and activity, as well as C1 levels, all returned to normal. PMID:11720182

  7. Possible implication of disordered neutrophil extracellular traps in the pathogenesis of MPO-ANCA-associated vasculitis.

    PubMed

    Nakazawa, Daigo; Tomaru, Utano; Ishizu, Akihiro

    2013-10-01

    Neutrophil extracellular traps (NETs) are characterized by the presence of extracellular DNA fibers studded with antimicrobial proteins, including myeloperoxidase (MPO). Although NETs play an important role in the innate immune system, the scattered extracellular enzymes, such as MPO, pose risks to the host. Therefore, NETs are strictly regulated by DNase I in the serum, which prevents them from persisting. Recent studies have demonstrated that dysregulation of NETs could be involved in the pathogenesis of autoimmune diseases, including systemic lupus erythematosus. In this review, we interpret the association of disordered NETs with autoimmune diseases, especially propylthiouracil-induced MPO-ANCA-associated vasculitis. PMID:23224024

  8. Complement C3 is expressed by mast cells in cutaneous vasculitis and is degraded by chymase.

    PubMed

    Lipitsä, Tiina; Naukkarinen, Anita; Laitala, Joel; Harvima, Ilkka T

    2016-10-01

    The complement factor C3 and chymase released from tryptase(+), chymase(+) mast cells may be involved in the pathogenesis of cutaneous leukocytoclastic vasculitis. To study whether mast cells contain C3 in vasculitis and whether chymase interacts with C3, cryosections from vasculitis biopsies were double-stained histochemically for C3c in tryptase(+) mast cells, as well as for chymase and vessel wall C3c, or they were treated with 5 µg/ml rh-chymase for 24 h followed by immunofluorescence (IF) analysis of C3c, IgG, IgM and IgA. The effect of rh-chymase on purified human C3, C3a and IgG was studied using SDS-PAGE electrophoresis and LAD2 mast cell cultures. The results show that 34.2 ± 17.9, 37.4 ± 15.5 and 43.4 ± 18.6 % (mean ± SD) of the mast cells express C3c immunoreactivity in the healthy skin, initial petechial (IP) and palpable purpura (PP) lesions, respectively. About 9.4-12.1 % of the chymase(+) mast cells were in apparent contact with C3c(+) vessels in IP and PP. The treatment of cryosections with rh-chymase decreased the IF staining of C3c, but not that of immunoglobulins. In SDS-PAGE, 1-10 µg/ml rh-chymase degraded the alpha- and beta-chains of C3, but did not degrade IgG. Unexpectedly, the rh-chymase treatment of C3 produced fragments that resulted in the release of tryptase and histamine from LAD2 cells. However, rh-chymase degraded C3a and consequently inhibited C3a activity on LAD2. In conclusion, mast cells can be one source for C3 in the early and late phases of vasculitis pathogenesis. However, rh-chymase degraded native C3, vessel wall C3c, and biologically active C3a. Therefore, chymase may control C3-related pathology. PMID:27465068

  9. [VASCULITIDES IN CHILDHOOD: A RETROSPECTIVE STUDY IN A PERIOD FROM 2002 TO 2012 AT THE DEPARTMENT OF PAEDIATRICS, UNIVERSITY HOSPITAL CENTRE ZAGREB].

    PubMed

    Jelusić, Marija; Kostić, Lucija; Frković, Marijan; Davidović, Masa; Malcić, Ivan

    2015-01-01

    The aim of our study was to analyze clinical features, laboratory findings, treatment, course and outcome of different types of vasculitis in children. All children aged up to 18 years that have been diagnosed with a vasculitis disorder from 2002. to 2012. at the Department of Paediatric, University Hospital Centre Zagreb according to EULAR/PRES/PRINTO criteria were included in the study. Vasculitis was diagnosed in 180 children, 101 girls and 79 boys, mean age 7.19 ± 3.7 years, with an average follow-up of 5.58 ± 3.28 years. Most of the children (155 or 86%) were diagnosed with Henoch-Shönlein purpura (HSP), polyarteritis nodosa (PAN) was diagnosed in 6 children (3.3%), isolated cutaneous leukocytoclastic vasculitis in 5 (2.8%), Takayasu arteritis (TA) and Kawasaki disease in 2 (1.1%) respectively, hypocomplementemic urticarial vasculitis in one patient (0.5%) and other types of vasculitis in 10 (5.5%) patients (vasculitides in systemic connective tissue disorders in 7 and unclassified vasculitides in 3 patients). All patients had elevated inflammatory markers (C-reactive protein and erythrocyte sedimentation rate). Anti-neutrophil cytoplasmatic antibodies (ANCA) were positive only in one patient, suffering from microscopic polyangiitis. Treatment modality in most patients were NSAIDs, while children with kidney or gastrointestinal system affection were treated with glucocorticoids and/or immunosuppresive drugs. Biological therapy (anti-CD20, rituximab) was used in patients with most severe symptoms. One child (0.56%), suffering from microscopic polyangiitis, died due to kidney failure during the follow-up. Forty patients (22.6%) had one disease relapse, while 6 (3.4%) had two relapses. In conclusion, we found some differences in laboratory parameters (e.g. lower incidence of elevated antistreptolysin O titer in HSP) and epidemiological data (e.g. higher prevalence of PAN in female children) in comparison to data from available studies, while other clinical

  10. The relation of autologous serum and plasma skin test results with urticarial activity score, sex and age in patients with chronic urticaria

    PubMed Central

    Aktar, Sirac; Akdeniz, Necmettin; Calka, Omer; Karadag, Ayse Serap

    2015-01-01

    Introduction Some previous studies reported autoimmunity as an etiologic factor in chronic urticaria (CU), but the results of some autoimmunity tests in these studies are conflicting. Aim To concretize whether there was any relation of autologous serum skin test (ASST) and autologous plasma skin test (APST) results with sex, age and urticarial activity score (UAS) in patients with CU. Material and methods Fifty patients with CU and twenty healthy subjects admitted to our dermatology clinic were included in the present study. The ASST and APST were applied to all individuals. Results The positiveness rates of ASST and APST were significantly higher in the patient group than controls (p = 0.027, p = 0.001, respectively). Among patients, the APST positiveness rate (72%) was significantly (p < 0.05) higher than ASST (46%). It was seen that 48% of patients with negative ASST results had positive APST. However, no patient with negative APST results had positive ASST. There were significant (p < 0.05) relations of the tests’ positiveness rates with sex and old age but with UAS. The diameter of the erythematous papule was remarkably (p < 0.05) larger in APST than ASST and also significantly (p < 0.05) larger in females compared to males in both tests (p < 0.05). It was positively increased with old age (p < 0.05). Conclusions We can suggest that APST is more sensitive than ASST in the assessment of autoimmunity in CU. A high positiveness rate of APST results may be attributed to high numbers of autoantibodies and coagulation factors present in plasma that might probably play a role in etiopathogenesis of CU. PMID:26161057

  11. Pathogenesis and diagnosis of otitis media with ANCA-associated vasculitis.

    PubMed

    Yoshida, Naohiro; Iino, Yukiko

    2014-12-01

    Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is histologically characterized by systemic necrotizing vasculitis and is clinically classified into two phases, systemic or localized. Recently, otological symptoms such as otitis media and hearing loss, not previously often associated with AAV, have been reported in AAV cases. In these cases we propose a diagnosis of otitis media with AAV (OMAAV). The ANCA titer is important for the diagnosis of OMAAV, and in most cases rapid progressive hearing loss is observed as localized AAV. Peripheral facial nerve palsy or hypertrophic pachymeningitis are coupled with 25% of cases and 18% of cases respectively. Proteinase 3-ANCA (PR3-ANCA) positive otitis media causes granulomatous formation or middle ear effusion in the middle ear, on the other hand myeloperoxidase-ANCA (MPO-ANCA) positive otitis media predominantly presents as otitis media with effusion. The early diagnosed case and the sensorineural hearing loss not progressed deaf could be recovered by the immunosuppressive therapy. Delayed diagnosis of AAV occasionally leads to progression to the irreversible phase; therefore, diagnosis at the early-localized stage is important for treating AAV. In this review, we discuss the current understanding of this newly proposed concept of OMAAV. PMID:25339433

  12. Use of antineutrophil cytoplasmic autoantibodies in diagnosing vasculitis in a Chinese patient population.

    PubMed

    Li, P K; Leung, J C; Lai, F M; Wang, A; Lui, S F; Leung, C B; Lai, K N

    1994-01-01

    Antineutrophil cytoplasmic autoantibodies (ANCA) have been used as markers of systemic vasculitides, including microscopic polyarteritis (MPA) and Wegener's granulomatosis. The diagnostic potential of ANCA assays together with antibodies against the neutrophil enzymes myeloperoxidase (MPO) and proteinase 3 for detecting a systemic vasculitis was tested in a Chinese patient population. 672 sera were received for ANCA assay, and ANCA detected by indirect immunofluorescence was positive in 73 sera from 42 patients. Of the 42 patients, 3 had cytoplasmic ANCA, while 39 had a perinuclear pattern. There was no patient with Wegener's granulomatosis. Two cytoplasmic ANCA positive patients suffered from ulcerative colitis. Another cytoplasmic ANCA positive patient was a carrier of human immunodeficiency virus. Of the 39 perinuclear ANCA positive patients, 10 had MPA. Eight of them were tested for anti-MPO antibody, and all were positive. Other immune disorders that were perinuclear ANCA positive included: 13 patients with systemic lupus erythematosus, 3 with mixed connective tissue disease, 1 with Goodpasture's syndrome, 2 with inflammatory bowel disease, and 2 patients with IgA nephropathy. Anti-MPO antibody was not specific for MPA, and 7 out of the 13 patients with systemic lupus erythematosus were anti-MPO antibody positive. Our study suggests that ANCA and anti-MPO antibody are not specific for MPA in a Chinese population. They would alert the clinician of the possibility of vasculitis, but a clinicopathological correlation is essential in making the diagnosis. PMID:7915885

  13. Platelet satellitism, spurious neutropenia, and cutaneous vasculitis: casual or causal association?

    PubMed

    Lazo-Langner, Alejandro; Piedras, Josefa; Romero-Lagarza, Penélope; Lome-Maldonado, Carmen; Sánchez-Guerrero, Jorge; López-Karpovitch, Xavier

    2002-07-01

    About 90 cases of platelet satellitism (PS) have been published, most of them involving neutrophils, a few comprising monocytes, and one case involving basophils. The case of a 30-year-old female patient with cutaneous vasculitis who developed asymptomatic severe neutropenia is reported. All blood smears (BS) prepared from peripheral blood samples collected with either ethylenediaminetetraacetic acid, trisodium citrate, and heparin showed PS involving neutrophils, eosinophils, and monocytes. Immunohistochemistry analysis of her skin biopsy and BS, employing peroxidase stain for the detection of antibodies directed against IgG, IgA, IgM, and C3, revealed an intense positive reaction only for IgG in the endothelium and leukocyte clumps within the microvasculature, as well as in peripheral blood neutrophils displaying the PS phenomenon. Transfer of the PS phenomenon was demonstrated by incubating the patient's plasma with leukocytes from an ABO-compatible healthy donor. In our patient, PS did not disappear after incubation at 37 degrees C, suggesting that this might be a different or "atypical" PS phenomenon probably mediated by an autoimmune process involving an IgG-class antibody responsible for both conditions, PS, and cutaneous vasculitis. PMID:12111772

  14. Vasculitis-like hemorrhagic retinal angiopathy in Wegener’s granulomatosis

    PubMed Central

    2013-01-01

    Background Granulomatosis with polyangiitis, also known as Wegener’s granulomatosis, is a chronic systemic inflammatory disease that can also involve the eyes. We report a case of massive retinal and preretinal hemorrhages with perivascular changes as the initial signs in granulomatosis with polyangiitis (Wegener’s granulomatosis). Case presentation A 39-year-old Caucasian male presented with blurred vision in his right eye, myalgia and arthralgia, recurrent nose bleeds and anosmia. Fundus image of his right eye showed massive retinal hemorrhages and vasculitis-like angiopathy, although no fluorescein extravasation was present in fluorescein angiography. Laboratory investigations revealed an inflammation with increased C-reactive protein, elevated erythrocyte sedimentation rate and neutrophil count. Tests for antineutrophil cytoplasmic antibodies (ANCA) were positive for c-ANCA (cytoplasmatic ANCA) and PR3-ANCA (proteinase 3-ANCA). Renal biopsy demonstrated a focal segmental necrotizing glomerulonephritis. Granulomatosis with polyangiitis (Wegener’s granulomatosis) was diagnosed and a combined systemic therapy of cyclophosphamide and corticosteroids was initiated. During 3 months of follow-up, complete resorption of retinal hemorrhages was seen and general complaints as well as visual acuity improved during therapy. Conclusion Vasculitis-like retinal changes can occur in Wegener’s granulomatosis. Despite massive retinal and preretinal hemorrhages that cause visual impairment, immunosuppressive therapy can improve ocular symptoms. PMID:24021028

  15. Drug-induced lupus with leukocytoclastic vasculitis: a rare expression associated with adalimumab*

    PubMed Central

    Amarante, Carolina Forte; Acedo, Livia Mendes Sabia; Rabay, Fátima Maria de Oliveira; Campos, Benedito do Espírito Santo; Lira, Márcia Lanzoni de Alvarenga; Mandelbaum, Samuel Henrique

    2015-01-01

    TNF alpha antagonist-induced lupus-like syndrome is a rare condition which predominantly affects women (4:1). The average age of onset is 46-51 years. It occurs after exposure to TNF alpha antagonist and disappears after discontinuation of such agents. The pathogenic mechanism for development of the TNF alpha antagonist-induced lupus-like syndrome is not fully defined. It is believed that the medication induces apoptosis, leading to an accumulation of nucleosomal antigens of apoptotic cells. This would cause autoantibodies to be produced by susceptible individuals. The most common cutaneous manifestations include maculopapular exanthem, malar rash, alopecia, photosensitivity and, more rarely, vasculitis. Extracutaneous manifestations include: fever, weight loss, arthritis or arthralgia, myositis and hematological abnormalities. Antinuclear antibody may be positive in 80% of cases and anti-histone antibody is considered a disease marker for TNF alpha antagonist-induced lupus-like syndrome. Treatment corresponds to drug discontinuation. We report a rare case of sub-acute cutaneous lupus erythematosus with leukocytoclastic vasculitis induced by adalimumab in a 42-year-old patient. PMID:26312693

  16. Long-term efficacy and safety of tocilizumab in giant cell arteritis and large vessel vasculitis

    PubMed Central

    Evans, Jobie; Steel, Lauren; Borg, Frances; Dasgupta, Bhaskar

    2016-01-01

    Giant cell arteritis (GCA) is a chronic systemic vasculitis affecting large-sized and medium-sized vessels. Glucocorticoids are currently the mainstay of treatment for GCA and associated large vessel vasculitis (LVV) but are associated with frequent adverse events. Methotrexate has only demonstrated a modest benefit while anti-TNF biological agents (infliximab and etanercept) have been inefficacious. Elevated levels of interleukin-6 (IL-6), a proinflammatory cytokine, has been associated with GCA. Tocilizumab (TCZ), a humanised antihuman IL-6 receptor antibody, has been used successfully in several reports as a treatment for GCA and LVV. We report the potentially long-term successful use of TCZ in 8 cases of refractory LVV. All of our patients achieved a good clinical response to TCZ and C reactive protein reduced from an average of 70.3 to 2.5. In all cases, the glucocorticoid dose was reduced, from an average of 24.6 mg prednisolone prior to TCZ treatment to 4.7 mg, indicating that TCZ may enable a reduction in glucocorticoid-associated adverse events. However, regular TCZ administration was needed for disease control in most cases. TCZ was discontinued in one case due to the development of an empyema indicating the need for careful monitoring of infection when using this treatment. PMID:26819753

  17. A Case of Polyarteritis Nodosa Associated with Vertebral Artery Vasculitis Treated Successfully with Tocilizumab and Cyclophosphamide.

    PubMed

    Watanabe, Kae; Rajderkar, Dhanashree A; Modica, Renee F

    2016-01-01

    Pediatric polyarteritis nodosa is rare systemic necrotizing arteritis involving small- and medium-sized muscular arteries characterized by aneurysmal dilatations involving the vessel wall. Aneurysms associated with polyarteritis nodosa are common in visceral arteries; however intracranial aneurysms have also been reported and can be associated with central nervous system symptoms, significant morbidity, and mortality. To our knowledge extracranial involvement of the vertebral arteries has not been reported but has the potential to be deleterious due to fact that they supply the central nervous system vasculature. We present a case of a 3-year-old Haitian boy with polyarteritis nodosa that presented with extracranial vessel involvement of his vertebral arteries. After thorough diagnostic imaging, including a bone scan, ultrasound, Magnetic Resonance Imaging/Angiography, and Computed Tomography Angiography, he was noted to have vertebral artery vasculitis, periostitis, subacute epididymoorchitis, arthritis, and myositis. He met diagnostic criteria for polyarteritis nodosa and was treated with cyclophosphamide, methylprednisolone, and tocilizumab, which resulted in improvement of his inflammatory markers, radiographic findings, and physical symptoms after treatment. To the authors' knowledge, this is the first report of vertebral artery vasculitis in polyarteritis nodosa as well as successful treatment of the condition using the combination cyclophosphamide and tocilizumab for this condition. PMID:27018080

  18. A Case of Polyarteritis Nodosa Associated with Vertebral Artery Vasculitis Treated Successfully with Tocilizumab and Cyclophosphamide

    PubMed Central

    Watanabe, Kae; Rajderkar, Dhanashree A.; Modica, Renee F.

    2016-01-01

    Pediatric polyarteritis nodosa is rare systemic necrotizing arteritis involving small- and medium-sized muscular arteries characterized by aneurysmal dilatations involving the vessel wall. Aneurysms associated with polyarteritis nodosa are common in visceral arteries; however intracranial aneurysms have also been reported and can be associated with central nervous system symptoms, significant morbidity, and mortality. To our knowledge extracranial involvement of the vertebral arteries has not been reported but has the potential to be deleterious due to fact that they supply the central nervous system vasculature. We present a case of a 3-year-old Haitian boy with polyarteritis nodosa that presented with extracranial vessel involvement of his vertebral arteries. After thorough diagnostic imaging, including a bone scan, ultrasound, Magnetic Resonance Imaging/Angiography, and Computed Tomography Angiography, he was noted to have vertebral artery vasculitis, periostitis, subacute epididymoorchitis, arthritis, and myositis. He met diagnostic criteria for polyarteritis nodosa and was treated with cyclophosphamide, methylprednisolone, and tocilizumab, which resulted in improvement of his inflammatory markers, radiographic findings, and physical symptoms after treatment. To the authors' knowledge, this is the first report of vertebral artery vasculitis in polyarteritis nodosa as well as successful treatment of the condition using the combination cyclophosphamide and tocilizumab for this condition. PMID:27018080

  19. An unexpected cause of acute kidney injury in a patient with ANCA associated vasculitis.

    PubMed

    Choudhry, Wajid M; Nori, Uday S; Nadasdy, Tibor; Satoskar, Anjali A

    2016-05-01

    Diagnostic kidney biopsies sometimes yield clinically unsuspected diagnoses. We present a case of a 69-year-old woman with established ANCA-associated vasculitis (AAV) of 4 years duration who was in clinical remission following cytotoxic therapy and was on maintenance immunosuppression. She presented to the hospital with acute kidney injury (AKI), symptoms suggestive of a systemic vasculitis, and in addition had hypercalcemia, metabolic alkalosis. A relapse in the AAV was suspected but a diagnostic kidney biopsy showed acute tubular necrosis, patchy interstitial inflammation, and calcium phosphate deposits. It was found that the patient recently started consuming large doses of over-the-counter calcium-containing antacids and vitamin Dcontaining multivitamin supplements. Cessation of these drugs led to improvement of renal function to baseline. This case highlights several teaching points: (1) the kidney biopsy can prove to be critically important even in cases where there appears to be a more obvious clinical diagnosis, (2) AK due to calcium-alkali syndrome has characteristic histopathological changes, and (3) that the triad of hypercalcemia, metabolic alkalosis, and AKI is exclusively associated with the ingestion of excessive quantities of calcium-containing antacids. The physician should keep this in mind, and pro-actively seek pertinent medication history from the patient. A brief review of calcium-alkali syndrome is given. PMID:26932179

  20. The role of IgM rheumatoid factor in experimental immune vasculitis.

    PubMed Central

    Floyd, M; Tesar, J T

    1979-01-01

    The effect of IgM rhematoid factor (RF) on reversepassive cutaneous Arthus reaction in rats was studied. The RF was obtained from the serum cryoglobulin of a patient with symptoms of purpura, arthralgia and digital gangrene. The cryoglobulins was of IgG-IgM type and when given i.v it induced a prompt hypocomplementaemia in experimental animals. The purified RF also induced low serum complement levels when injected i.v. along with complexes of non-complement-fixing, aggregated IgG. A reverse passive Arthus reaction was induced by intradermal injection of IgG anti-bovine serum albumin (BSA), followed by an i.v. dose of antigen (Ag). The cutaneous inflammatory reaction was aggravated by simultaneous administration of IgM RF intradermally, but not by IgM without antibody (Ab) properties. Intradermal injection of low concentrations of non-complement-fixing IgG anti-BSA, along with normal human IgM, followed by i.v. injection of BSA, resulted in a complete lack of cutaneous inflammation. At higher Ab concentrations there was only a mild inflammation. However, when IgM RF was substituted for normal IgM and injected with non-complement-fixing anti-BSA, an effective reverse passive cutaneous Arthus reaction and vasculitis was induced. The inflammatory response was greatly suppressed by decomplementation of animals by cobra venom factor. This study provides evidence favouring an inflammatory, complement-dependent role for RF in vasculitis. PMID:157238

  1. Rare type of pancreatitis as the first presentation of anti-neutrophil cytoplasmic antibody-related vasculitis

    PubMed Central

    Iida, Tomoya; Adachi, Takeya; Tabeya, Tetsuya; Nakagaki, Suguru; Yabana, Takashi; Goto, Akira; Kondo, Yoshihiro; Kasai, Kiyoshi

    2016-01-01

    A pancreatic tumor was suspected on the abdominal ultrasound of a 72-year-old man. Abdominal computed tomography showed pancreatic enlargement as well as a diffuse, poorly enhanced area in the pancreas; endoscopic ultrasound-guided fine needle aspiration biopsy and endoscopic retrograde cholangiopancreatography failed to provide a definitive diagnosis. Based on the trend of improvement of the pancreatic enlargement, the treatment plan involved follow-up examinations. Later, he was hospitalized with an alveolar hemorrhage and rapidly progressive glomerulonephritis; he tested positive for myeloperoxidase-anti-neutrophil cytoplasmic antibody (ANCA) and was diagnosed with ANCA-related vasculitis, specifically microscopic polyangiitis. It appears that factors such as thrombus formation caused by the vasculitis in the early stages of ANCA-related vasculitis cause abnormal distribution of the pancreatic blood flow, resulting in non-uniform pancreatitis. Pancreatic lesions in ANCA-related vasculitis are very rare. Only a few cases have been reported previously. Therefore, we report our case and a review of the literature. PMID:26900301

  2. Rare type of pancreatitis as the first presentation of anti-neutrophil cytoplasmic antibody-related vasculitis.

    PubMed

    Iida, Tomoya; Adachi, Takeya; Tabeya, Tetsuya; Nakagaki, Suguru; Yabana, Takashi; Goto, Akira; Kondo, Yoshihiro; Kasai, Kiyoshi

    2016-02-21

    A pancreatic tumor was suspected on the abdominal ultrasound of a 72-year-old man. Abdominal computed tomography showed pancreatic enlargement as well as a diffuse, poorly enhanced area in the pancreas; endoscopic ultrasound-guided fine needle aspiration biopsy and endoscopic retrograde cholangiopancreatography failed to provide a definitive diagnosis. Based on the trend of improvement of the pancreatic enlargement, the treatment plan involved follow-up examinations. Later, he was hospitalized with an alveolar hemorrhage and rapidly progressive glomerulonephritis; he tested positive for myeloperoxidase-anti-neutrophil cytoplasmic antibody (ANCA) and was diagnosed with ANCA-related vasculitis, specifically microscopic polyangiitis. It appears that factors such as thrombus formation caused by the vasculitis in the early stages of ANCA-related vasculitis cause abnormal distribution of the pancreatic blood flow, resulting in non-uniform pancreatitis. Pancreatic lesions in ANCA-related vasculitis are very rare. Only a few cases have been reported previously. Therefore, we report our case and a review of the literature. PMID:26900301

  3. [Disease-modifying factors in chronic polyarthritis. Correlations amongsystemic vasculitis, secondary amyloidosis, septic infections and occurrence of miliary epitheloid-cell granuloma. A review of autopsies].

    PubMed

    Bély, M; Apáthy, A

    1993-03-01

    The following findings were obtained from autopsies performed on 169 deceased with chronic polyarthritis (CP): systemic vasculitis in 26 cases (15.4%), systemic secondary amyloidosis in 32 cases (18.9%), sepsis in 13 cases (7.7%) and miliary epithelioid-cell granulomatosis (probably tuberculosis) in six cases (3.6%). Vasculitis was combined with amyloidosis in five patients, with sepsis in two and with miliary epithelioid-cell granulomatosis in four. Critical random check, using the Mann-Whitney test, did not support significance of relationship between vasculitis and amyloidosis or fatal sepsis, whereas significant correlations were found to exist in CP cases between vasculitis and miliary epithelioid-cell granulomatosis (P < 0.005). The latter had no effect on the severity of vasculitis, but the incidence of the granulomatous type of vasculitis was higher with significance (P < 0.02). The conclusion is that biopsy evidence of granulomatous vasculitis in CP patients should be followed by systematic clinical search for miliary tuberculosis because of above-average incidence of that combination. PMID:8499420

  4. Vasculitis (image)

    MedlinePlus

    ... These purplish spots can be felt in the skin. They do not turn white (blanch) when pressed. As the condition progresses, they may become larger and more bruise-like (ecchymotic), and some may develop central ulceration or necrosis (tissue death).

  5. Hypersensitivity Vasculitis

    MedlinePlus

    ... you think. More serious than you know. Support. Awareness. Education. Become a member today! Our Mission The ... Foundation supports and empowers our community through education, awareness and research. Contact Us Click here to send ...

  6. Immunoglobulin A Vasculitis Complicated with Clostridium difficile Infection: a Rare Case Report and Brief Review of the Literature.

    PubMed

    Cojocariu, Camelia; Stanciu, Carol; Ancuta, Codrina; Danciu, Mihai; Chiriac, Stefan; Trifan, Anca

    2016-06-01

    Immunoglobulin A (IgA) vasculitis, formerly called Henoch-Schönlein purpura, is a leukocytoclastic type of vasculitis affecting small vessels with a deposition of immune IgA complexes, clinically characterized by the classic tetrad of nonthrombocytopenic palpable purpura, arthralgia (or arthritis), and gastrointestinal and renal involvement. Although the cause of the disease remains unknown, immune complexes of IgA and unidentified antigens seem to play a central pathogenic role. The diagnosis is easily established in the presence of purpura, but may be challenging in its absence, especially when colicky abdominal pain precedes the cutaneous lesions. IgA vasculitis is usually a self-limited disease with a benign course and symptomatic treatment is sufficient for most; in severe cases, however, corticosteroids are necessary. We describe the case of a young adult male presenting with severe abdominal pain, vomiting and fever (38.4ºC). Clinical examination, abdominal ultrasound and plain abdominal radiography excluded an acute abdomen. The occurrence of arthralgia involving both knees and erosive duodenitis at endoscopy, 48 hours upon admission, suggested the diagnosis of IgA vasculitis, confirmed on the following day by the presence of typical purpuric rash on the lower extremities. Corticosteroid therapy led to the resolution of all gastrointestinal and joint manifestations as well as to a significant improvement of cutaneous purpura. However, during the 3rd week of corticosteroid treatment, the patient developed watery diarrhea and the clinical suspicion of Clostridium difficile infection (CDI) was confirmed. The treatment with metronidazole led to the resolution of diarrhea.The peculiarity of this case resides in several aspects: the gastrointestinal and joint manifestations preceded purpura, making diagnosis more difficult; CDI is an extremely rare complication of IgA vasculitis, being, in fact, the second case reported in adults in the literature. PMID

  7. Pyothorax caused by Nocardia otitidiscaviarum in a patient with rheumatoid vasculitis.

    PubMed

    Yoshida, Kazuya; Bandoh, Shuji; Fujita, Jiro; Tokuda, Michiaki; Negayama, Kiyoshi; Ishida, Toshihiko

    2004-07-01

    We report a case of pyothorax caused by Nocardia (N.) otitidiscaviarum infection in a 69-year-old man with rheumatoid vasculitis, who was regularly treated with prednisolone in our hospital. Initially, the patient responded poorly to intravenous imipenem/cilastatin (IPM/CS), minocyclin (MINO), and oral trimethoprim-sulfamethoxazole (TMP-SMX), but later improved after treatment with levofloxacin (LVFX) and gentamicin sulfate (GM) according to in vitro susceptibility tests. To our knowledge, this is the first description of pyothorax caused by N. otitidiscaviarum infection. It is a rare disease, but recognition of the disease in immunocompromised patients and the prompt initiation of appropriate treatments based on isolation of the pathogen and susceptibility testing can lead to a successful outcome. PMID:15335193

  8. Severe gastrointestinal cytomegalovirus disease in two patients with renal vasculitis after immunosuppression.

    PubMed

    Lee, Kian-Guan; Teo, Su-Hooi; Lim, Cynthia; Loh, Alwin; Chidambaram, Viswanath; Choo, Jason

    2016-09-01

    Although the use of current immunosuppressive regimens has significantly improved the outcomes of autoimmune renal diseases, infectious complications remain an important clinical concern. Cytomegalovirus (CMV) infection has been shown to be one of the major causes of mortality in this group of patients. We report two cases of renal vasculitis (Granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA)) that developed into severe gastrointestinal CMV disease and manifested with massive small bowel bleeding, resulting in an eventual fatal outcome for one of the patients. Risk factors, pathogenesis, role of immunosuppression in the development of CMV infection, and antiviral treatment are discussed in this review. These cases highlight the need for further research to evaluate the complex mechanisms between immunosuppression and CMV occurrence as well as the role of antiviral prophylaxis in high-risk patients undergoing immunosuppressive therapies.
. PMID:27443566

  9. [Digoxin-related leukocytoclastic vasculitis in a very elderly woman: A case report].

    PubMed

    Ludwig-Béal, S; Vernier, N; Popitean, L; Levêque, L; Combret, S; Manckoundia, P

    2016-05-01

    Even though digoxin causes many side effects, few cases of skin involvement are recorded in the French Pharmacovigilance Database. We report a case of leukocytoclastic vasculitis (LV) very probably due to digoxin. A 91-year-old woman, hospitalized following a fall, presented cardiac decompensation in a context of rapid atrial fibrillation requiring treatment with digoxin. Eight days later, a rash appeared on her back and trunk. It was neither itchy, nor painful and persisted despite local treatment. There were no other clinical anomalies. After a few days, the rash spread with appearance of bullous lesions, ulcerations and a necrosis on lymphedema of the two legs. Among the complementary examinations, skin biopsy revealed LV with necrosis and subepidermal detachment suggested toxic dermal necrolysis, while direct immunofluorescence was negative. The rash resolved progressively once the digoxin was stopped. The pharmacovigilance department recorded that digoxin was the probable cause. The evidence allowed us to conclude that digoxin was the cause. PMID:26970812

  10. Varicella-Zoster Virus Vasculopathy: A Case Report Demonstrating Vasculitis using Black-Blood MRI

    PubMed Central

    Shah, Jay; Poonawala, Husain; Keay, Susan K; Serulle, Yafell; Steven, Andrew; Gandhi, Dheeraj; Cole, John W

    2016-01-01

    Infections are rare but important causes of stroke. Among these, varicella zoster virus has been known to cause ischemic stroke. During an attack of herpes zoster ophthalmicus, it has been hypothesized that the virus replicates in the trigeminal ganglion and travels via the trigeminal nerve centrally to cause cerebral vasculopathy. Here we present a case of a 69 year-old Caucasian immunocompromised woman who suffered recurrent ischemic infarcts within the same vascular distribution following an episode of zoster ophthalmicus three months prior. An imaging technique termed black-blood magnetic resonance imaging was utilized to aid in the diagnosis of cerebral vasculitis. The case is used to provide a literature review of the pathogenesis, diagnosis, and treatment of cerebral varicella zoster vasculopathy. In situations where an isolated unilateral cerebral vasculopathy is identified, neurologists are urged to consider varicella zoster as a treatable etiologic agent, as untreated vasculopathy can lead to further strokes. PMID:27065314

  11. Childhood-onset eosinophilic granulomatosis with polyangiitis: a rare childhood vasculitis mimicking anthrax and eosinophilic leukaemia.

    PubMed

    Sahin, Sezgin; Adrovic, Amra; Barut, Kenan; Kasapcopur, Ozgur

    2016-01-01

    A 14-year-old boy previously misdiagnosed as having cutaneous anthrax was referred with a 2-month history of multiple wide and deep ulceronecrotic lesions in the lower extremities, which occurred after contact with animals. Skin biopsy was compatible with vasculitis. Further examination at our hospital elicited eosinophilia and a history of asthma. On the second day of hospitalisation, he developed deep vein thrombosis. A diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA) was established and intravenous methylprednisolone was administered. The patient showed remarkable improvement of the cutaneous lesions. Diagnosis of EGPA is challenging in the vasculitic phase and necessitates a detailed history that specifically questions the patient for an asthma background. This case illustrates a severe cutaneous presentation of EGPA and emphasises the difficulty of diagnosis as a result of overlapped signs and symptoms with cutaneous anthrax and leukaemia. EGPA should be kept in mind in the differential diagnosis of cutaneous lesions associated with eosinophilia. PMID:26887883

  12. Combination anti-VEGF and corticosteroid therapy for idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome.

    PubMed

    Sawhney, Gagan K; Payne, John F; Ray, Robin; Mehta, Sonia; Bergstrom, Chris S; Yeh, Steven

    2013-11-01

    Vision loss associated with the idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome most commonly occurs from macular edema or complications related to neovascularization. The authors present a case of advanced IRVAN associated with a massive exudative response characterized by peripheral retinal telangiectasias, exudative retinal detachment, and macular edema with lipid maculopathy. The patient was managed successfully with visual acuity from hand motion to 20/150 using a combination of local corticosteroids, intravitreal bevacizumab, panretinal photocoagulation, and eventually pars plana vitrectomy for progressive vitreomacular traction. VEGF- and non-VEGF-mediated mechanisms appear to be involved in the pathogenesis of IRVAN given the efficacy of combination therapy. [ophthalmic surg lasers imaging retina. 2013;44:599-602.]. PMID:24221466

  13. Unusual cause of flare in antineutrophil cytoplasmic antibody-associated vasculitis.

    PubMed

    Glanville, James Robert William; Penn, Henry

    2016-01-01

    A 53-year-old woman attended for a routine outpatient appointment for follow-up of antineutrophil cytoplasmic antibody-positive vasculitis. Her disease had relapsed despite appropriate medical management with mycophenolate mofetil (MMF), as evidenced by rising acute phase response and antimyeloperoxidase titre with ongoing symptoms. On further questioning, she had been taking oral charcoal as part of a detoxification diet, which we postulate was causing significantly impaired MMF absorption. This case report summarises the presentation and highlights the importance of a thorough drug history, and should prompt the reader to keep an open mind with regard to drug interactions and treatment regimen adherence when treatment is, unexpectedly, seemingly failing. PMID:26746838

  14. Recent advances in anti-neutrophil cytoplasmic antibody-associated vasculitis

    PubMed Central

    Lazarus, B.; John, G. T.; O’Callaghan, C.; Ranganathan, D.

    2016-01-01

    Anti-neutrophil cytoplasmic antibody-associated vasculitis is an uncommon inflammatory disease of small to medium-sized vessels that frequently presents with rapidly progressive glomerulonephritis and renal failure though it can affect any organ system. If untreated, the vast majority of patients will die within a year. Current treatments improve prognosis but affected patients remain at a substantially higher risk of death and adverse outcomes. We review the classification of the disease, our understanding of the pathogenesis and epidemiology, and propose future directions for research. We also evaluate the evidence supporting established treatment regimens and the progress of clinical trials for newer treatments to inform the design of future studies. PMID:27051131

  15. The expanding spectrum of HCV-related cryoglobulinemic vasculitis: a narrative review.

    PubMed

    Dammacco, Franco; Racanelli, Vito; Russi, Sabino; Sansonno, Domenico

    2016-08-01

    Cryoglobulinemic vasculitis (CV) is a small-to-medium-vessel vasculitis that appears in 10-15 % of patients chronically infected with hepatitis C virus (HCV). The classic symptom triad of CV, purpura/asthenia/arthralgia, is accompanied by clinical features that include glomerulonephritis, neuropathy, interstitial pneumonitis, and cardiomyopathy, ranging in their severity from mild to life threatening. The risk of developing non-Hodgkin lymphoma is also higher. The cumulative 10-year survival rate of CV patients is significantly lower than in the age- and sex-matched general population, with death typically caused by nephropathy, malignancies, liver involvement, and severe infections. Unfailing serological stigmata include both a cryoglobulin IgM fraction with rheumatoid factor activity and decreased complement C4 levels. On peripheral B cells, the expression of the CD81 B cell receptor is reduced while that of the CD19 receptor is increased. A monoclonal B cell lymphocytosis develops in almost one-third of patients. HCV-related proteins (but not HCV-RNA genomic sequences) can be detected on biopsy samples by immunofluorescence and immunohistochemistry and involve the vessel lumen, vessel walls, and the perivascular spaces of the skin, kidney, and peripheral nerves, supporting the pathogenetic role of HCV in the onset of a widespread microvasculitis. Based on the demonstration of HCV infection in the large majority of CV patients, a therapeutic regimen consisting of once-weekly pegylated interferon-α and the daily administration of ribavirin results in a sustained virologic response in ~50 % of patients. In those with refractory and relapsing disease, addition of the anti-CD20 monoclonal antibody rituximab has significantly increased the overall response rates. The extension to CV of latest-generation direct-acting antivirals, strikingly successful in non-CV HCV-positive patients, has yielded high complete response rates according to the few studies published

  16. Farm Exposure as a Differential Risk Factor in ANCA-Associated Vasculitis

    PubMed Central

    Willeke, P.; Schlüter, B.; Sauerland, C.; Becker, H.; Reuter, S.; Jacobi, A.; Schotte, H.

    2015-01-01

    Objective To investigate the association of farm exposure and the development of ANCA-associated vasculitis (AAV). Methods One hundred eighty-nine well defined patients with AAV (n = 119 with granulomatosis with polyangiitis [GPA], n = 48 with microscopic polyangiitis [MPA], n = 22 patients with eosinophilic granulomatosis with polyangiitis [EGPA]) and 190 controls (n = 119 patients with rheumatoid arthritis, n = 71 with large vessel vasculitis) were interrogated using a structured questionnaire. Factors investigated were occupation, farm exposure, contact to different livestock, participation in harvesting, residence next to a farm, MRSA status, and contact to domestic pets at disease onset or ever before. The odds ratio (OR) and 95% confidence interval [95%CI] were calculated for each item. Results Univariate analysis revealed a strong association of AAV with regular farm exposure; OR 3.44 [95%CI 1.43–8.27]. AAV was also associated with regular contact to cattle 4.30 (1.43–8.27), pigs 2.75 (1.12–6.75) and MRSA carriage 3.38 (1.11–10.3). This association was stronger in the subgroup of GPA patients. OR in this group for farm exposure was 4.97; [2.02–12.2], for cattle 6.71 [95% CI 2.19–20.7], for pigs 4.34 [1.75–10.9], and MRSA carriage 5.06 [1.62–15.8]). There was no significant association of MPA or EGPA with these parameters. Conclusion A significant association between farm exposure or farm animal exposure and AAV especially in the subgroup of patients with GPA has been identified. This suggests that these entities are distinct and have different triggers for the immune process. PMID:26339905

  17. Polyarteritis nodosa. Diagnostic challenges in a patient with cutaneous vasculitis, psoriasis, psoriatic arthritis and pancytopenia: fatal progression after treatment with G-CSF

    PubMed Central

    Jobanputra, Paresh

    2016-01-01

    A 60-year-old man presented with cutaneous vasculitis, leucopenia and psoriasis. He was treated initially with ciclosporin A. On withdrawal of ciclosporin, due to inadequate improvement of cutaneous vasculitis, he developed psoriatic arthritis. Worsening neutropenia and pancytopenia, believed to be immune mediated, developed. He was treated with prednisolone, methotrexate and adalimumab but developed pneumocystis pneumonia. Leucocyte levels improved markedly with granulocyte colony-stimulating factor (G-CSF). However, whilst being treated with G-CSF his condition deteriorated. He developed gastrointestinal and neurological symptoms and progressive weight loss. Diagnosis was delayed, but eventually polyarteritis nodosa was diagnosed and he was treated with cyclophosphamide. The patient improved initially but died from small bowel perforation due to vasculitis. Evidence showing a temporal association of his deterioration with use of G-CSF is shown. The use of G-CSF in patients with autoimmune conditions including vasculitis should be undertaken with great caution. PMID:27123310

  18. Increased cellularity and expression of adhesion molecules in muscle biopsy specimens from patients with rheumatoid arthritis with clinical suspicion of vasculitis, but negative routine histology

    PubMed Central

    Verschueren, P.; Voskuyl, A.; Smeets, T; Zwinderman, K.; Breedveld, F.; Tak, P.

    2000-01-01

    OBJECTIVE—Histological analysis of random quadriceps muscle biopsy specimens can be used to detect vasculitis in patients with rheumatoid arthritis (RA). This study aimed at determining the immunohistological features in patients with clinical suspicion of rheumatoid vasculitis, but without a transmural infiltrate or fibrinoid necrosis of the vessel wall on routine histology.
METHODS—Three groups of patients with RA were studied: (a) without clinical signs of vasculitis (n=6); (b) with recent onset of extra-articular features and a clinical suspicion of vasculitis but normal routine histology (n=11); and (c) with recent onset of extra-articular features and vasculitis, histologically proved either in muscle or other biopsy specimens (n=14). A control group of patients with osteoarthritis was also included (n=5). Frozen sections from quadriceps muscle biopsy specimens were analysed with monoclonal antibodies to detect CD3, CD4, CD8, CD68, ICAM-1, VCAM-1, and HLA-DR. The slides were evaluated using a semiquantitative scoring system (0-4).
RESULTS—The mean scores gradually increased from group 1 to 3, leading to significant differences between groups 1 and 2, but not between groups 2 and 3 for most markers (p< 0.05). Thus the pathological changes were similar for the two groups with clinical signs of vasculitis, even when the conventional histological evaluation was negative. Higher immunohistological scores were associated with perivascular infiltrates on routine histology.
CONCLUSION—The pathophysiological events leading to vasculitis are reflected by the changes in the quadriceps muscle biopsy specimens. The data indicate that the sensitivity of examination of muscle biopsy specimens for the diagnosis of rheumatoid vasculitis can be increased by the use of new criteria.

 PMID:10913056

  19. Safety and efficacy of rituximab treatment for vasculitis in hepatitis B virus-associated type II cryoglobulinemia: a case report

    PubMed Central

    2012-01-01

    Introduction Systemic B-cell depletion and clinical remission of the systemic effects of cryoglobulins have already been achieved using rituximab in hepatitis C virus-positive immunocompetent patients. Conversely, to the best of our knowledge there are no reports in the literature regarding the use of rituximab in hepatitis B virus-associated cryoglobulinemia. Case presentation We report here the case of a 60-year-old Caucasian man who presented with hepatitis B virus-associated type II cryoglobulinemia with severe multisystem disease, including membranoproliferative glomerulonephritis with acute renal failure. The vasculitis was refractory to conventional and antiviral therapy but rituximab use led to a fall in cryoglobulin levels and disease control. The B-cell depletion was safe and efficient to induce a complete remission of the disease. Conclusion Our case highlights the benefit and the efficacy of rituximab in association with antiviral therapy in small vessel vasculitis related to hepatitis B virus-associated mixed cryoglobulinemia. PMID:22284897

  20. Crohns disease with central nervous system vasculitis causing subarachnoid hemorrhage due to aneurysm and cerebral ischemic stroke.

    PubMed

    Garge, Shaileshkumar S; Vyas, Pooja D; Modi, Pranav D; Ghatge, Sharad

    2014-10-01

    Cerebral vasculitis secondary to Crohn's disease (CD) seems to be a very rare phenomenon. We report a 39-year-old male who presented with headache, vomiting, and left-sided weakness in the known case of CD. Cross-sectional imaging (computed tomography and magnetic resonance imaging,) showed right gangliocapsular acute infarct with supraclinoid cistern subarachnoid hemorrhage (SAH). Cerebral digital substraction angiography (DSA) showed dilatation and narrowing of right distal internal carotid artery (ICA). Left ICA was chronically occluded. His inflammatory markers were significantly raised. Imaging features are suggestive of cerebral vasculitis. Arterial and venous infarcts due to thrombosis are known in CD. Our case presented with acute subarachnoid hemorrhage in supraclinoid cistern due to rupture of tiny aneurysm of perforator arteries causing SAH and infarction in right basal ganglia. Patient was treated conservatively with immunosuppression along with medical management of SAH. PMID:25506170

  1. Exploration, Development, and Validation of Patient-reported Outcomes in Antineutrophil Cytoplasmic Antibody–associated Vasculitis Using the OMERACT Process

    PubMed Central

    Robson, Joanna C.; Milman, Nataliya; Tomasson, Gunnar; Dawson, Jill; Cronholm, Peter F.; Kellom, Katherine; Shea, Judy; Ashdown, Susan; Boers, Maarten; Boonen, Annelies; Casey, George C.; Farrar, John T.; Gebhart, Don; Krischer, Jeffrey; Lanier, Georgia; McAlear, Carol A.; Peck, Jacqueline; Sreih, Antoine G.; Tugwell, Peter; Luqmani, Raashid A.; Merkel, Peter A.

    2016-01-01

    Objective Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of linked multisystem life- and organ-threatening diseases. The Outcome Measures in Rheumatology (OMERACT) vasculitis working group has been at the forefront of outcome development in the field and has achieved OMERACT endorsement of a core set of outcomes for AAV. Patients with AAV report as important some manifestations of disease not routinely collected through physician-completed outcome tools; and they rate common manifestations differently from investigators. The core set includes the domain of patient-reported outcomes (PRO). However, PRO currently used in clinical trials of AAV do not fully characterize patients’ perspectives on their burden of disease. The OMERACT vasculitis working group is addressing the unmet needs for PRO in AAV. Methods Current activities of the working group include (1) evaluating the feasibility and construct validity of instruments within the PROMIS (Patient-Reported Outcome Measurement Information System) to record components of the disease experience among patients with AAV; (2) creating a disease-specific PRO measure for AAV; and (3) applying The International Classification of Functioning, Disability and Health to examine the scope of outcome measures used in AAV. Results The working group has developed a comprehensive research strategy, organized an investigative team, included patient research partners, obtained peer-reviewed funding, and is using a considerable research infrastructure to complete these interrelated projects to develop evidence-based validated outcome instruments that meet the OMERACT filter of truth, discrimination, and feasibility. Conclusion The OMERACT vasculitis working group is on schedule to achieve its goals of developing validated PRO for use in clinical trials of AAV. (First Release September 1 2015; J Rheumatol 2015;42:2204–9; doi:10.3899/jrheum.141143) PMID:26329344

  2. Acute dilated cardiomyopathy in a patient with beriberi and cryoglobulinaemic vasculitis: an unusual potential complication of two rare disorders.

    PubMed

    Tejedor, Ana; Solé, Manel; Prieto-González, Sergio; Alba, Marco Antonio; Grau, Josep Maria; Cid, Maria Cinta; Hernández-Rodríguez, José

    2014-01-01

    We report the case of a 45-year-old patient who presented with acute dilated cardiomyopathy. During admission the patient was consecutively diagnosed with cryoglobulinaemic vasculitis and beriberi. In both diseases, cardiac involvement may occur as dilated cardiomyopathy. Thiamin deficiency was the final cause for the severe cardiac manifestations (cardiac acute beriberi or Shoshin syndrome), which returned to normal after thiamin supplementation. PMID:24429381

  3. Cerebral vascular findings in PAPA syndrome: cerebral arterial vasculopathy or vasculitis and a posterior cerebral artery dissecting aneurysm.

    PubMed

    Khatibi, Kasra; Heit, Jeremy J; Telischak, Nicholas A; Elbers, Jorina M; Do, Huy M

    2016-08-01

    A young patient with PAPA (pyogenic arthritis, pyoderma gangrenosum, and acne) syndrome developed an unusual cerebral arterial vasculopathy/vasculitis (CAV) that resulted in subarachnoid hemorrhage from a ruptured dissecting posterior cerebral artery (PCA) aneurysm. This aneurysm was successfully treated by endovascular coil sacrifice of the affected segment of the PCA. The patient made an excellent recovery with no significant residual neurologic deficit. PMID:26122324

  4. Long-Term Maintenance Therapy Using Rituximab-Induced Continuous B-Cell Depletion in Patients with ANCA Vasculitis

    PubMed Central

    Pendergraft, William F.; Cortazar, Frank B.; Wenger, Julia; Murphy, Andrew P.; Rhee, Eugene P.; Laliberte, Karen A.; Niles, John L.

    2014-01-01

    Background and objectives Remission in the majority of ANCA vasculitis patients is not sustained after a single course of rituximab, and risk of relapse warrants development of a successful strategy to ensure durable remission. Design, setting, participants, & measurements A retrospective analysis of ANCA vasculitis patients who underwent maintenance therapy using rituximab-induced continuous B-cell depletion for up to 7 years was performed. Maintenance therapy with rituximab was initiated after achieving remission or converting from other prior maintenance therapy. Continuous B-cell depletion was achieved in all patients by scheduled rituximab administration every 4 months. Disease activity, serologic parameters, adverse events, and survival were examined. Results In the study, 172 patients (mean age=60 years, 55% women, 57% myeloperoxidase–ANCA) treated from April of 2006 to March of 2013 underwent continuous B-cell depletion with rituximab. Median remission maintenance follow-up time was 2.1 years. Complete remission (Birmingham Vasculitis Activity Score [BVAS]=0) was achieved in all patients. Major relapse (BVAS≥3) occurred in 5% of patients and was associated with weaning of other immunosuppression drugs. Remission was reinduced in all patients. Survival mirrored survival of a general age-, sex-, and ethnicity-matched United States population. Conclusion This analysis provides evidence for long-term disease control using continuous B-cell depletion. This treatment strategy in ANCA vasculitis patients also seems to result in survival rates comparable with rates in a matched reference population. These findings suggest that prospective remission maintenance treatment trials using continuous B-cell depletion are warranted. PMID:24626432

  5. Serum proteins reflecting inflammation, injury and repair as biomarkers of disease activity in ANCA-associated vasculitis

    PubMed Central

    Monach, Paul A; Warner, Roscoe L; Tomasson, Gunnar; Specks, Ulrich; Stone, John H; Ding, Linna; Fervenza, Fernando C; Fessler, Barri J; Hoffman, Gary S; Iklé, David; Kallenberg, Cees GM; Krischer, Jeffrey; Langford, Carol A; Mueller, Mark; Seo, Philip; St. Clair, E William; Spiera, Robert; Tchao, Nadia; Ytterberg, Steven R; Johnson, Kent J; Merkel, Peter A

    2016-01-01

    Objective To identify circulating proteins that distinguish between active anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and remission in a manner complementary to markers of systemic inflammation. Methods Twenty-eight serum proteins representing diverse aspects of the biology of AAV were measured before and 6 months after treatment in a large clinical trial of AAV. Subjects (n=186) enrolled in the Rituximab in ANCA-Associated Vasculitis (RAVE) trial were studied. Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels were available for comparison. The primary outcome was the ability of markers to distinguish severe AAV (Birmingham Vasculitis Activity Score for Wegener’s granulomatosis (BVAS/WG)≥3 at screening) from remission (BVAS/WG=0 at month 6), using areas under receiver operating characteristic (ROC) curve (AUC). Results All subjects had severe active vasculitis (median BVAS/WG=8) at screening. In the 137 subjects in remission at month 6, 24 of the 28 markers showed significant declines. ROC analysis indicated that levels of CXCL13 (BCA-1), matrix metalloproteinase-3 (MMP-3) and tissue inhibitor of metalloproteinases-1 (TIMP-1) best discriminated active AAV from remission (AUC>0.8) and from healthy controls (AUC>0.9). Correlations among these markers and with ESR or CRP were low. Conclusions Many markers are elevated in severe active AAV and decline with treatment, but CXCL13, MMP-3 and TIMP-1 distinguish active AAV from remission better than the other markers studied, including ESR and CRP. These proteins are particularly promising candidates for future studies to address unmet needs in the assessment of patients with AAV. PMID:22975753

  6. Long-term Prognosis of Anti-Neutrophil Cytoplasmic Antibody-Negative Renal Vasculitis: Cohort Study in Korea

    PubMed Central

    Yu, Mi-Yeon; Baek, Seon Ha; Ahn, Shin-Young; Kim, Sejoong; Na, Ki Young; Chae, Dong-Wan

    2016-01-01

    Few studies have reported on the long-term prognosis of anti-neutrophil cytoplasmic antibody (ANCA)-negative renal vasculitis. Between April 2003 and December 2013, 48 patients were diagnosed with renal vasculitis. Their ANCA status was tested using indirect immunofluorescence and enzyme-linked immunosorbent assays. During a median (interquartile range) follow-up duration of 933.5 (257.5–2,079.0) days, 41.7% of patients progressed to end stage renal disease (ESRD) and 43.8% died from any cause. Of 48 patients, 6 and 42 were ANCA-negative and positive, respectively. The rate of ESRD within 3 months was higher in ANCA-negative patients than in ANCA-positive patients (P = 0.038). In Kaplan-Meier survival analysis, ANCA-negative patients showed shorter renal survival than did ANCA-positive patients (log-rank P = 0.033). In univariate Cox-proportional hazard regression analysis, ANCA-negative patients showed increased risk of ESRD, with a hazard ratio 3.190 (95% confidence interval, 1.028–9.895, P = 0.045). However, the effect of ANCA status on renal survival was not statistically significant in multivariate analysis. Finally, ANCA status did not significantly affect patient survival. In conclusion, long-term patient and renal survival of ANCA-negative renal vasculitis patients did not differ from those of ANCA-positive renal vasculitis patients. Therefore, different treatment strategy depending on ANCA status might be unnecessary. PMID:27051237

  7. Primary central nervous system vasculitis preceded by granulomatous hypophysitis: Case report with a review of the literature

    PubMed Central

    Huang, Meng; Steele, William J.; Baskin, David S.

    2015-01-01

    Background: Primary central nervous system (CNS) vasculitis is an idiopathic inflammatory process that selectively affects CNS vasculature without a systemic inflammatory response, and causes luminal obstruction with resultant ischemia of recipient tissue. Its varying clinical symptoms and signs depend on the caliber of vessels involved and distribution and location of the affected structures. Granulomatous hypophysitis (GH) is an autoimmune inflammatory process typically affecting women, and usually presents with hypopituitarism, and at times, diabetes insipidus, and/or visual loss. Both entities are rare CNS diseases, which, to our knowledge, have never been previously reported in the same patient. Case Description: We present a unique case of chronic progressive primary CNS vasculitis causing limbic encephalopathy in a 30-year-old male with only a history of medication-controlled hypertension. He initially presented 4 months prior with nonspecific neurological complaints and was found to have a homogenously enhancing and enlarged pituitary, which was biopsy proven to be GH. Conclusion: This rather unique presentation highlights the need to maintain a high index of suspicion for underlying PCNS vasculitis in a patient who does not fit the typical demographic for isolated GH. PMID:26539311

  8. Static and fatigue tensile properties of cross-ply laminates containing vascules for self-healing applications

    NASA Astrophysics Data System (ADS)

    Luterbacher, R.; Trask, R. S.; Bond, I. P.

    2016-01-01

    The effect of including hollow channels (vascules) within cross-ply laminates on static tensile properties and fatigue performance is investigated. No change in mechanical properties or damage formation is observed when a single vascule is included in the 0/90 interface, representing 0.5% of the cross sectional area within the specimen. During tensile loading, matrix cracks develop in the 90° layers leading to a reduction of stiffness and strength (defined as the loss of linearity) and a healing agent is injected through the vascules in order to heal them and mitigate the caused degradation. Two different healing agents, a commercial low viscosity epoxy resin (RT151, Resintech) and a toughened epoxy blend (bespoke, in-house formulation) have been used to successfully recover stiffness under static loading conditions. The RT151 system recovered 75% of the initial failure strength, whereas the toughened epoxy blend achieved a recovery of 67%. Under fatigue conditions, post healing, a rapid decay of stiffness was observed as the healed damage re-opened within the first 2500 cycles. This was caused by the high fatigue loading intensity, which was near the static failure strength of the healing resin. However, the potential for ameliorating (via self-healing or autonomous repair) more diffuse transverse matrix damage via a vascular network has been shown.

  9. A Randomized Controlled Trial of Rituximab Following Failure of Antiviral Therapy for Hepatitis C-Associated Cryoglobulinemic Vasculitis

    PubMed Central

    Sneller, Michael C.; Hu, Zonghui; Langford, Carol A.

    2011-01-01

    OBJECTIVES To report on the results of a randomized controlled trial of rituximab in hepatitis C virus (HCV)-associated mixed cryoglobulinemic vasculitis. METHODS We conducted an open-label single center randomized controlled trial of rituximab (375 mg/m2 per week for 4 weeks) compared to best available therapy for treatment of patients with HCV-associated cryoglobulinemic vasculitis in whom antiviral therapy failed to induce remission. The primary endpoint was remission at 6 months from study entry. RESULTS A total of 24 patients were enrolled. Baseline disease activity and organ involvement were similar in the two groups. Ten patients in the rituximab group (83%) were in remission at study month 6, compared with 1 patient in the control group (8%), a result that met criterion for stopping the study (P<0.001). The median duration of remission for rituximab-treated patients reaching the primary endpoint was 7 months. No adverse effect of rituximab on HCV plasma viremia or hepatic transaminase levels was observed. CONCLUSIONS Therapy with rituximab was well tolerated and effective treatment for patients with HCV-associated cryoglobulinemic vasculitis in whom antiviral therapy fails to induce remission. PMID:22147444

  10. Hepatotropic viral infection associated systemic vasculitides-hepatitis B virus associated polyarteritis nodosa and hepatitis C virus associated cryoglobulinemic vasculitis.

    PubMed

    Sharma, Aman; Sharma, Kusum

    2013-09-01

    Two hepatotropic viruses have been shown to have causal relationship with systemic vasculitis-hepatitis B with classical polyarteritis nodosa and hepatitis C with cryoglobulinemic vasculitis. The present paper provides an updated overview on the clinical presentations and management of these vasculitides. HBV associated PAN patients have higher weight loss, peripheral neuropathy, mononeuritis multiplex, abdominal pain, gastrointestinal manifestations requiring surgery, cardiomyopathy, orchitis, hypertension, and/or elevated transaminase levels. Microaneurysms are also more common in mesenteric artery. Skin manifestations, however are less common. These patients also have a severe disease as suggested by higher five factor score and higher BVAS. Though relapses are less common, mortality is higher in patients with HBV PAN as compared to non HBV PAN. Plasmapheresis has a role in treatment in clearing off immune complexes. The common clinical manifestations of HCV associated cryoglobulinemic vasculitis are skin lesions, peripheral neuropathy, glomerulonephritis, arthritis, and sicca symptoms. Though combination therapy comprising of pegylated interferon α and ribavirin is the first line of management, immunotherapy is needed for severe or life threatening manifestations. Recent randomized trials have shown the efficacy of rituximab in such situations. PMID:25755502

  11. Clinical Outcomes of Remission Induction Therapy for Severe Antineutrophil Cytoplasmic Antibody–Associated Vasculitis

    PubMed Central

    Miloslavsky, E. M.; Specks, U.; Merkel, P. A.; Seo, P.; Spiera, R.; Langford, C. A.; Hoffman, G. S.; Kallenberg, C. G. M.; St.Clair, E. W.; Tchao, N. K.; Viviano, L.; Ding, L.; Sejismundo, L. P.; Mieras, K.; Iklé, D.; Jepson, B.; Mueller, M.; Brunetta, P.; Allen, N. B.; Fervenza, F. C.; Geetha, D.; Keogh, K.; Kissin, E. Y.; Monach, P. A.; Peikert, T.; Stegeman, C.; Ytterberg, S. R.; Stone, J. H.

    2016-01-01

    Objective To evaluate the reasons that complete remission is not achieved or maintained with original treatment in some patients with antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV) treated with rituximab (RTX) or with cyclophosphamide/azathioprine (CYC/AZA). Methods The Rituximab in AAV trial was a randomized, double-blind, placebo-controlled trial comparing the rate of remission induction among patients treated with RTX (n = 99) and patients treated with CYC followed by AZA (n = 98). Glucocorticoids were tapered over a period of 5 months. The primary outcome measure was lack of disease activity without glucocorticoid treatment at 6 months. To determine the most important reason for failure to achieve the primary outcome, 7 hierarchical categories of reasons were defined retrospectively (uncontrolled disease, adverse event leading to therapy discontinuation, severe flare, limited flare, Birmingham Vasculitis Activity Score for Wegener’s Granulomatosis >0, prednisone treatment at any dosage, and other). Results Although remission (lack of disease activity) was achieved in 170 of the 197 patients (86%) in the first 6 months, the primary outcome measure was not achieved in 42%. There were 3 deaths. Twenty-four percent of the patients failed to achieve the primary end point due to active disease: 10 (5%) experienced uncontrolled disease in the first month and 37 (19%) experienced flares after initial improvement. In the majority of such patients, treatment with blinded crossover or according to best medical judgment led to disease control. Ninety-one percent of patients who had uncontrolled disease or experienced a severe flare had proteinase 3 (PR3)–ANCA. When patients with uncontrolled disease were excluded from analysis, those who were PR3-ANCA positive were found to experience fewer flares when treated with RTX compared to CYC/AZA (8 of 59 [14%] versus 20 of 62 [32%]; P = 0.02). Neither ANCA titers nor B cell counts predicted disease

  12. Anti-oxidized low-density lipoprotein antibodies in myeloperoxidase–positive vasculitis patients preferentially recognize hypochlorite-modified low density lipoproteins

    PubMed Central

    Slot, M C; Theunissen, R; van Paassen, P; Damoiseaux, J G M C; Cohen Tervaert, J W

    2007-01-01

    Many patients surviving vasculitis are prone to accelerated atherosclerosis and often have enhanced levels of antibodies to oxidized low-density lipoprotein (oxLDL). To measure anti-oxLDL antibodies, oxidation of LDL is achieved with copper (Cu) or malondialdehyde (MDA). Because, in vivo, LDL may be oxidized with myeloperoxidase (MPO) or its product hypochlorite, we measured anti-hypochlorite LDL antibodies in patients with vasculitis, haemodialysis patients and healthy controls. A newly developed enzyme-linked immunosorbent assay (ELISA) was used to detect antibodies to oxLDL as modified by hypochlorite. Results are compared with data obtained by standard LDL oxidation using MDA–LDL or Cu–LDL as substrate. Results were compared between anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) patients (n = 93), haemodialysis (HD) patients (n = 59) and healthy controls (HC; n = 43). Furthermore, patients with MPO–ANCA-associated vasculitis (n = 47) were compared with patients with proteinase 3 (PR3)–ANCA associated vasculitis (n = 46). Optimal cut-off points were determined by receiver operator characteristic (ROC) curve analysis. Anti-oxLDL antibodies are enhanced in AAV patients (MDA–LDL and hypochlorite–LDL) and in HD patients (hypochlorite–LDL), when compared to HC. Furthermore, patients with MPO–ANCA-associated vasculitis had higher levels of antibodies to hypochlorite–LDL than patients with PR3–ANCA-associated vasculitis. Our newly developed assay, in which hypochlorite–LDL is used as substrate, seems a more sensitive assay than traditional assays to measure oxLDL antibodies. Furthermore, our results suggest that enhanced MPO-mediated LDL oxidation occurs in patients with MPO–ANCA. PMID:17521320

  13. Relapsing polychondritis with p-ANCA associated vasculitis: Which triggers the other?

    PubMed Central

    File, Ibolya; Trinn, Csilla; Mátyus, Zsolt; Ujhelyi, László; Balla, József; Mátyus, János

    2014-01-01

    Relapsing polychondritis (RP) is a rare autoimmune disease with chronic inflammatory/destructive lesions of the cartilaginous tissues. In one third of the cases it is associated with other autoimmune disorders, mostly with anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV). We report three cases of RP with p-ANCA positive AAV. In the first patient RP developed 1.5 years after the onset of AAV. In the others the signs of RP were present before the onset of severe crescent glomerulonephritis. Patients responded well on steroid and cyclophosphamide. In dialysis dependent cases plasmapheresis was also used successfully. During the 2 and 1.5 years of follow up, they were symptom-free, and had stable glomerular filtration rate. The first patient died after four years of follow-up due to the complications of sudden unset pancytopenia, which raises the possibility of associated hemophagocytic syndrome. In the setting of RP or AAV physicians should always be aware of the possibility of sudden or insidious appearance of the other disease. PMID:25516870

  14. Possible intrinsic association of anti-neutrophil cytoplasmic antibody-associated vasculitis coexisting with multiple myeloma

    PubMed Central

    Liu, Huifang; Xiong, Jiachuan; Zhang, Jun; Zhang, Ying; Nie, Ling; Wang, Yiqin; Zhao, Jinghong

    2016-01-01

    Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a life-threatening condition that causes renal failure. Multiple myeloma (MM) is a malignant proliferation of monoclonal plasma cells in the blood that can also cause renal failure. The two diseases have high morbidity and mortality rates in the elderly, with a poor prognosis. A 64-year-old female presented to Xinqiao Hospital (Chonqing, China) with fatigue and a poor appetite that had been apparent for 6 weeks. Laboratory tests revealed a serum creatinine level of 10.31 mg/dl, a cytoplasmic ANCA titer of 1:10, a positive result for myeloperoxidase and a serum globulin level of 3.96 g/dl. A renal biopsy revealed crescent glomerulonephritis, combined with the rapid progression of renal function. Based on these observations (ANCA titer, crescent glomerulonephritis and rapid progression of renal function) a diagnosis of AAV was established. MM was confirmed by serum immunofixation electrophoresis combined with bone marrow aspiration. The present study discusses what is, to the best of our knowledge, the first case of AAV coexisting with MM in order to highlight it as a clinical concern. PMID:27602144

  15. Systemic lupus erythematosus presenting with leukocytoclastic vasculitis and seizure during pregnancy.

    PubMed

    Borahay, Mostafa A; Kelly, Brent C; Harirah, Hassan M

    2009-06-01

    Systemic lupus erythematosus (SLE) is a rare multisystem disease with a wide array of presentation and is a diagnostic challenge during pregnancy. A 20-year-old gravida 1 at 39 weeks' gestation was referred to our hospital for elevated blood pressure, headache, and history of seizure. She was admitted with the impression of severe preeclampsia. Intravenous magnesium sulfate for seizure prophylaxis and oxytocin for induction of labor were started. Primary lower-segment cesarean section was performed for nonreassuring fetal heart tracing. The postoperative course was complicated with fever requiring prolonged intravenous antibiotic therapy, appearance of violaceous skin lesions on the periungual areas of fingers and toes, recurrent seizures, and altered sensorium. Biopsy of the lesions revealed leukocytoclastic vasculitis (LCV) with thrombi. Laboratory workup confirmed SLE with a dramatic improvement of the patient's condition upon initiating intravenous steroid therapy. LCV and neuropsychiatric SLE are rare presentations of SLE during pregnancy, and obstetricians should be aware of them. Workup for SLE is warranted in cases with atypical presentation of preeclampsia that does not resolve with delivery. PMID:19326323

  16. Current and novel biomarkers in anti-neutrophil cytoplasm-associated vasculitis

    PubMed Central

    Draibe, Juliana Bordignon; Fulladosa, Xavier; Cruzado, Josep Maria; Torras, Joan; Salama, Alan David

    2016-01-01

    Anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) is characterized by a variable disease course, with up to 50% of patients having one relapse within 5 years and many progressing to end-stage organ damage despite modern treatment strategies. Moreover, complications arising from treatment dominate the causes of mortality and morbidity both early and late during disease, especially in the elderly and those with severe renal involvement, and there is additional uncertainty as to how long treatment should be continued. There is, therefore, an urgent clinical need to identify robust biomarkers to better predict treatment responses, risk of disease relapse and eventual complete clinical and immunological quiescence. To date, no such biomarkers exist, but better understanding of disease pathogenesis and the underlying immune dysfunction has provided some potential candidates linked to the discovery of new antibodies, different leukocyte activation states, the role of the alternative complement pathway and markers of vascular activation. With all promising new biomarkers, there is the need to rapidly replicate and validate early findings using large biobanks of samples that could be brought together by leaders in the field. PMID:27478594

  17. A case of primary central nervous system vasculitis diagnosed by second brain biopsy and treated successfully.

    PubMed

    Mizuno, Yuri; Shigeto, Hiroshi; Yamada, Takeshi; Maeda, Norihisa; Suzuki, Satoshi O; Kira, Jun-Ichi

    2016-03-30

    We report a case of primary central nervous system vasculitis (PCNSV) diagnosed by second brain biopsy. A 53-year-old man initially presented with left lateral gaze diplopia. Brain MRI revealed multiple enhanced lesions in the bilateral frontal lobe, bilateral basal ganglia, left cerebellum and brainstem. An initial brain biopsy of the right frontal lobe suggested immune-related encephalitis with angiocentric accumulation of chronic inflammatory cells, while malignant lymphoma could not be completely ruled out. The patient deteriorated despite being treated with repeated methylprednisolone pulse therapy, cyclophosphamide, and plasmapheresis. A second brain biopsy of the right temporal lobe was then performed. The biopsied specimens showed vascular wall disruption and fibrinoid necrosis with perivascular inflammatory infiltrates, mainly composed of CD8-positive T cells, and PCNSV was diagnosed. He was treated with high dose corticosteroids, in combination with methotrexate (8 mg/week), which reduced the brain lesions. As brain biopsy is an essential investigation for the histological diagnosis of PCNSV; subsequent biopsies may be required when a histopathological diagnosis has not been obtained by the first biopsy, and further aggressive therapy is being considered. PMID:26960271

  18. [Clinical practice guideline for ANCA-associated vasculitis with renal involvement].

    PubMed

    Vazquez, Vanina; Fayad, Alicia; González, Gabriela; Smuclir Quevedo, Alejandra; Robaina Sindín, Javier

    2015-01-01

    The ANCA-associated vasculitis (AAV) is a group of systemic conditions characterized by inflammation and necrosis in small and medium vessels. AAV with different levels of disease severity could respond to different therapeutics protocols. Early diagnosis and treatment could significantly improve the outcome of the disease. The objective was to investigate the quality evidence in different therapeutical protocols proposed to AAV with renal involvement in pediatrics and adults patients and evaluate the ANCA applicability in AAV diagnosis and outcome. Using methodological search filters, we identified literature in Medline, Embase, Lilacs and Cochrane Trials Register published between 1997 and July 2015. From 4236 articles, 59 were included. The quality of evidence was assessed using the check list designed by the Cochrane Renal Group. The strength of recommendation was determinated by Levels of Evidence (Oxford Centre for Evidence-based Medicine). On the basis of current evidence, 20 recommendations were elaborated for the treatment and monitoring of patients with AAV with renal involvement in several clinical scenarios, in order to provide physicians a rational approach in daily clinical practice. PMID:26738202

  19. Anti-endothelial cell antibodies in patients with rheumatoid arthritis complicated by vasculitis.

    PubMed Central

    Heurkens, A H; Hiemstra, P S; Lafeber, G J; Daha, M R; Breedveld, F C

    1989-01-01

    IgG antibodies reactive with human umbilical vein endothelial cells were found in 19 out of 28 patients with rheumatoid vasculitis (RV), in four out of 24 patients with rheumatoid arthritis (RA), in seven out of 10 patients with systemic lupus erythematosus (SLE), but not in healthy donors. In four patients with RV who were followed longitudinally, regression of vasculitic episodes coincided with decreasing titres of anti-endothelial antibodies (AEA). Binding activity to endothelial cells was observed in intact IgG and F(ab')2 fragments of IgG. AEA activity was unrelated to antibodies against nuclear, blood group or major histocompatibility complex antigens and did not involve immune complexes. AEA activity was not specific for endothelial cells since the AEA-positive sera and the IgG fractions prepared from these sera also reacted with fibroblasts. Adsorption of positive sera and corresponding IgG fractions with endothelial cells decreased the IgG binding reactivity on both fibroblasts and endothelial cells. These findings show that RV patients have IgG-AEA, and suggest that these antibodies may play a role in the pathogenesis of the disease. PMID:2805426

  20. [Primary Sjögren's syndrome with cutaneous vasculitis manifested as leg ulcerations].

    PubMed

    Souza, Sonia Cristina de Magalhães; Kuruma, Katia Akemi Miyazato; Andrade, Danieli Castro Oliveira de; Azevedo, Pedro Ming; Figueiredo, Camille Pinto; Borba, Eduardo Ferreira; Gonçalves, Célio Rodrigues; Borges, Cláudia Teresa Lobato

    2004-04-01

    Primary Sjögren's Syndrome (pSS) is an autoimmune disease with a large spectrum of clinical manifestations extending from an organ-specific involvement to a systemic process. The skin is affected quite commonly and the estimated frequency of inflammatory vascular lesions is from 20% to 30%. Two specific, clinically recognizable forms of cutaneous vasculitis predominate, palpable purpura and chronic urticaria, but erythema multiforme, erythema perstans, erythema nodosum, erithematous macules and subcutaneous nodules have also been described. The authors report the case of a 46-year-old female patient, diagnosed as primary SS, who presented ocular and oral symptoms, poliarthritis and laboratory alterations (with a positive ANA, anti-SSA, rheumatoid factor, and hypergammaglobulinemia). Ten years after the diagnosis, she presented leg ulcers. The biopsy confirmed the presence of vasculitic process, and the ulcers improved rapidly after the treatment with endovenous cyclophosphamide. There are only two reports of chronic ulceration of the legs as cutaneous manifestation of SS. The authors stress the importance of considering ulcers in the differential diagnosis of cutaneous involvement of primary SS. PMID:21503546

  1. Circulating ADAM17 Level Reflects Disease Activity in Proteinase-3 ANCA-Associated Vasculitis.

    PubMed

    Bertram, Anna; Lovric, Svjetlana; Engel, Alissa; Beese, Michaela; Wyss, Kristin; Hertel, Barbara; Park, Joon-Keun; Becker, Jan U; Kegel, Johanna; Haller, Hermann; Haubitz, Marion; Kirsch, Torsten

    2015-11-01

    ANCA-associated vasculitides are characterized by inflammatory destruction of small vessels accompanied by enhanced cleavage of membrane-bound proteins. One of the main proteases responsible for ectodomain shedding is disintegrin and metalloproteinase domain-containing protein 17 (ADAM17). Given its potential role in aggravating vascular dysfunction, we examined the role of ADAM17 in active proteinase-3 (PR3)-positive ANCA-associated vasculitis (AAV). ADAM17 concentration was significantly increased in plasma samples from patients with active PR3-AAV compared with samples from patients in remission or from other controls with renal nonvascular diseases. Comparably, plasma levels of the ADAM17 substrate syndecan-1 were significantly enhanced in active AAV. We also observed that plasma-derived ADAM17 retained its specific proteolytic activity and was partly located on extracellular microparticles. Transcript levels of ADAM17 were increased in blood samples of patients with active AAV, but those of ADAM10 or tissue inhibitor of metalloproteinases 3, which inhibits ADAMs, were not. We also performed a microRNA (miR) screen and identified miR-634 as significantly upregulated in blood samples from patients with active AAV. In vitro, miR-634 mimics induced a proinflammatory phenotype in monocyte-derived macrophages, with enhanced expression and release of ADAM17 and IL-6. These data suggest that ADAM17 has a prominent role in AAV and might account for the vascular complications associated with this disease. PMID:25788529

  2. Presumed reactive polyarthritis and granulomatous vasculitis in a Mississippi sandhill crane (Grus canadensis pulla).

    PubMed

    MacLean, Robert; Beaufrère, Hugues; Heggem-Perry, Brittany; Field, Cara; Garner, Michael

    2013-12-01

    A 1.5-year-old female Mississippi sandhill crane (Grus canadensis pulla) was presented and managed for a polyarthritis of the intertarsal and tarsophalangeal articulations. Results of aerobic bacterial cultures, Mycoplasma species culture, and polymerase chain reaction testing of articular fluid did not identify any causative organisms. Results of radiographs and cytologic examination of articular fluid were consistent with an inflammatory, nonerosive polyarthritis. The arthritis did not improve with systemic anti-inflammatory and antibiotic treatment and with joint lavage. A large necrotic granulomatous mass was detected on the right shoulder area from which Staphylococcus aureus and Enterococcus species were isolated as opportunistic pathogens. Two days after surgical resection of the mass, the distal polyarthritis resolved. Histopathologic examination of the mass was consistent with granulomatous vasculitis with abscess formation of unknown origin. In this crane, the unresponsiveness to standard therapy, the presence of an infected and inflammatory mass, and the resolution of the polyarthritis after the resection of the mass strongly supported a diagnosis of reactive immune-mediated nonerosive polyarthritis. Analysis of this case suggests that immune-mediated idiopathic arthritis should be a differential diagnosis of distal polyarthritis in cranes and that an inciting source remote from the joints should be investigated in case of lack of response to standard therapy. PMID:24640933

  3. Kaposi's sarcoma with HHV8 infection and ANCA-associated vasculitis in a hemodialysis patient.

    PubMed

    Fatma, Lilia Ben; Rais, Lamia; Mebazza, Amel; Azzouz, Haifa; Beji, Somaya; Krid, Madiha; Smaoui, Wided; Maiz, Hedi Ben; Zouaghi, Karim; Zitouna, Moncef; Osmane, Amel Ben; Moussa, Fatma Ben

    2013-11-01

    The association between Kaposi's sarcoma (KS) and human herpes virus eight (HHV-8) infection is rarely reported in hemodialysis (HD) patients. We report here the rare association of KS, HHV-8 and hepatitis C virus (HCV) infection as well as syphilis in a HD patient. We report the case of a 72-year-old woman who presented with microscopic polyangiitis with alveolar hemorrhage and pauci-immune necrosing and crescentic glomerulonephritis as well as renal failure requiring HD. Biological tests showed positive HCV and syphilis tests. The patient was treated by HD and intravenous pulse, followed by oral corticosteroids and six cyclophosphamide monthly pulses with remission of the alveolar hemorrhage, but without renal functional recovery as the patient remained HD dependent. Five months after the first treatment administration, she developed extensive purpuric lesions on her lower limbs, abdomen face and neck. A skin biopsy showed KS. The HHV-8 test was positive, with positive polymerase chain reaction-HHV8 in the serum and skin. After immunosuppression withdrawal, the KS skin lesions regressed rapidly without relapse after 12 months of follow-up, but alveolar hemorrhage relapsed after 16 months of follow-up. Our case showed that the immunosuppressed state related to multiple factors such as aging, vasculitis, HHV-8, HCV, syphilis, immunosuppressive therapy and HD may all have contributed to the development of KS in our patient. PMID:24231484

  4. Rituximab for the Treatment of Relapses in ANCA-associated Vasculitis

    PubMed Central

    Miloslavsky, EM; Specks, U; Merkel, PA; Seo, P; Spiera, R; Langford, CA; Hoffman, GS; Kallenberg, CGM; St. Clair, EW; Tchao, NK; Viviano, L; Ding, L; Ikle, D; Villarreal, M; Jepson, B; Brunetta, P; Allen, NB; Fervenza, FC; Geetha, D; Keogh, K; Kissin, EY; Monach, PA; Peikert, T; Stegeman, C; Ytterberg, SR; Stone, JH

    2014-01-01

    Introduction Disease relapses are frequent in antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV). We evaluated the outcomes of patients re-treated with rituximab (RTX) and prednisone for severe disease relapses. Methods The Rituximab in AAV trial was a randomized, double-blind, placebo-controlled trial comparing the rates of remission induction among patients treated with RTX (n = 99) and patients treated with cyclophosphamide (CYC) followed by azathioprine (AZA) (n = 98). Prednisone was tapered to discontinuation after 5.5 months. After achieving remission, patients who experienced a severe disease relapse between months 6 and 18 were eligible to receive RTX and prednisone on an open-label basis according to a pre-specified protocol. Investigators remained blinded to the original treatment assignment. Results Twenty-six patients received treatment with RTX for disease relapse after initially achieving remission on their originally assigned treatment. Fifteen patients were initially randomized to RTX and 11 to CYC/AZA. Thirteen (87%) of the patients originally assigned to RTX and 10 (91%) originally assigned to CYC/AZA achieved remission again with open-label RTX, an overall percentage of 88%. Half of the patients treated with open-label RTX were able to discontinue prednisone entirely. Patients in this cohort experienced fewer adverse events compared to the overall study population (4.7 adverse events/patient-year versus 11.8 adverse events/patient-year, respectively). Conclusion Re-treatment of AAV relapses with RTX and glucocorticoids appears to be a safe and effective strategy, regardless of previous treatment. PMID:25047592

  5. Classification, epidemiology and clinical subgrouping of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis.

    PubMed

    Watts, Richard A; Mahr, Alfred; Mohammad, Aladdin J; Gatenby, Paul; Basu, Neil; Flores-Suárez, Luis Felipe

    2015-04-01

    It is now 25 years since the first European studies on vasculitis--the anti-neutrophil cytoplasmic antibody (ANCA) standardization project. Over that period of time, there have been major developments in the classification of the vasculitides, which has permitted the conduct of high-quality epidemiology studies. Studying the epidemiology of rare diseases such as the ANCA-associated vasculitides (AAV) poses considerable challenges to epidemiologists. The first is the need for a clear definition of a case with good differentiation from similar disorders. The second is case capture. The vasculitides are rare, and therefore, a large population is required to determine the incidence and prevalence, and this poses questions of feasibility. A large population increases the risk of incomplete case detection but permits a reasonable number of cases to be collected in a practicable time frame, whereas a smaller population requires a much longer time frame to collect the necessary cases, which may also not be feasible. Statistical methods of capture-recapture analysis enable estimates to be made of the number of missing cases. The third is case ascertainment. The AAV are virtually always managed in secondary care, and therefore, hospital-based case ascertainment may be appropriate. Fourthly, the rarity of the conditions makes prospective case-control studies investigating risk factors difficult to conduct because the population size required to achieve statistical confidence is in excess of that which is readily available. Thus, much of the data on risk factors are derived from retrospective studies with inherent potential bias. PMID:25805746

  6. Proteinase 3 on apoptotic cells disrupts immune silencing in autoimmune vasculitis

    PubMed Central

    Millet, Arnaud; Martin, Katherine R.; Bonnefoy, Francis; Saas, Philippe; Mocek, Julie; Alkan, Manal; Terrier, Benjamin; Kerstein, Anja; Tamassia, Nicola; Satyanarayanan, Senthil Kumaran; Ariel, Amiram; Ribeil, Jean-Antoine; Guillevin, Loïc; Cassatella, Marco A.; Mueller, Antje; Thieblemont, Nathalie; Lamprecht, Peter; Mouthon, Luc; Perruche, Sylvain; Witko-Sarsat, Véronique

    2015-01-01

    Granulomatosis with polyangiitis (GPA) is a systemic necrotizing vasculitis that is associated with granulomatous inflammation and the presence of anti-neutrophil cytoplasmic antibodies (ANCAs) directed against proteinase 3 (PR3). We previously determined that PR3 on the surface of apoptotic neutrophils interferes with induction of antiinflammatory mechanisms following phagocytosis of these cells by macrophages. Here, we demonstrate that enzymatically active membrane-associated PR3 on apoptotic cells triggered secretion of inflammatory cytokines, including granulocyte CSF (G-CSF) and chemokines. This response required the IL-1R1/MyD88 signaling pathway and was dependent on the synthesis of NO, as macrophages from animals lacking these pathways did not exhibit a PR3-associated proinflammatory response. The PR3-induced microenvironment facilitated recruitment of inflammatory cells, such as macrophages, plasmacytoid DCs (pDCs), and neutrophils, which were observed in close proximity within granulomatous lesions in the lungs of GPA patients. In different murine models of apoptotic cell injection, the PR3-induced microenvironment instructed pDC-driven Th9/Th2 cell generation. Concomitant injection of anti-PR3 ANCAs with PR3-expressing apoptotic cells induced a Th17 response, revealing a GPA-specific mechanism of immune polarization. Accordingly, circulating CD4+ T cells from GPA patients had a skewed distribution of Th9/Th2/Th17. These results reveal that PR3 disrupts immune silencing associated with clearance of apoptotic neutrophils and provide insight into how PR3 and PR3-targeting ANCAs promote GPA pathophysiology. PMID:26436651

  7. Detection of Anti-Pentraxin-3 Autoantibodies in ANCA-Associated Vasculitis

    PubMed Central

    Guilpain, Philippe; Jeannin, Pascale; Pignon, Pascale; Blanchard, Simon; Garo, Erwan; Jaillon, Sébastien; Chevailler, Alain; Renier, Gilles; Puéchal, Xavier; Bottazzi, Barbara; Mantovani, Alberto; Delneste, Yves; Augusto, Jean-François

    2016-01-01

    Objectives Pentraxin 3 (PTX3), in common with myeloperoxidase and proteinase 3, is stored in human neutrophil granules and is expressed on apoptotic neutrophil surface. We therefore investigated the presence of anti-PTX3 autoantibodies (aAbs) in the sera of antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) patients. Methods Presence of anti-PTX3 autoantibodies was analysed by a specific enzyme-linked immunosorbent assay in sera from 150 patients with microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA), and eosinophilic granulomatosis with polyangiitis (EGPA), and in sera of 227 healthy subjects (HS), 40 systemic sclerosis (SSc) patients, and 25 giant cell arteritis patients (GCA). Using indirect immunofluorescence on fixed human neutrophils, we also analyzed the staining pattern associated with the presence of anti-PTX3 aAbs. Results Anti-PTX3 aAbs were detected in 56 of 150 (37.3%) of the AAV patients (versus 12 of 227 (5.3%) of HS, p<0.001) and, interestingly, in 7 of 14 MPO and PR3 ANCA negative AAV patients. Moreover, by indirect immunofluorescence on fixed neutrophils, anti-PTX3 aAbs gave rise to a specific cytoplasmic fluorescence pattern distinct from the classical cytoplasmic (c-ANCA), perinuclear (p-ANCA), and atypical (a-ANCA) pattern. Anti-PTX3 aAbs levels were higher in patients with active AAV as compared to patients with inactive disease. Conclusion Our work suggests that PTX3 is as a novel ANCA antigen. Anti-PTX3 aAbs appear thus as a promising novel biomarker in the diagnosis of AAV, including in patients without detectable MPO and PR3 ANCA. PMID:26797217

  8. Proteinase 3 on apoptotic cells disrupts immune silencing in autoimmune vasculitis.

    PubMed

    Millet, Arnaud; Martin, Katherine R; Bonnefoy, Francis; Saas, Philippe; Mocek, Julie; Alkan, Manal; Terrier, Benjamin; Kerstein, Anja; Tamassia, Nicola; Satyanarayanan, Senthil Kumaran; Ariel, Amiram; Ribeil, Jean-Antoine; Guillevin, Loïc; Cassatella, Marco A; Mueller, Antje; Thieblemont, Nathalie; Lamprecht, Peter; Mouthon, Luc; Perruche, Sylvain; Witko-Sarsat, Véronique

    2015-11-01

    Granulomatosis with polyangiitis (GPA) is a systemic necrotizing vasculitis that is associated with granulomatous inflammation and the presence of anti-neutrophil cytoplasmic antibodies (ANCAs) directed against proteinase 3 (PR3). We previously determined that PR3 on the surface of apoptotic neutrophils interferes with induction of antiinflammatory mechanisms following phagocytosis of these cells by macrophages. Here, we demonstrate that enzymatically active membrane-associated PR3 on apoptotic cells triggered secretion of inflammatory cytokines, including granulocyte CSF (G-CSF) and chemokines. This response required the IL-1R1/MyD88 signaling pathway and was dependent on the synthesis of NO, as macrophages from animals lacking these pathways did not exhibit a PR3-associated proinflammatory response. The PR3-induced microenvironment facilitated recruitment of inflammatory cells, such as macrophages, plasmacytoid DCs (pDCs), and neutrophils, which were observed in close proximity within granulomatous lesions in the lungs of GPA patients. In different murine models of apoptotic cell injection, the PR3-induced microenvironment instructed pDC-driven Th9/Th2 cell generation. Concomitant injection of anti-PR3 ANCAs with PR3-expressing apoptotic cells induced a Th17 response, revealing a GPA-specific mechanism of immune polarization. Accordingly, circulating CD4+ T cells from GPA patients had a skewed distribution of Th9/Th2/Th17. These results reveal that PR3 disrupts immune silencing associated with clearance of apoptotic neutrophils and provide insight into how PR3 and PR3-targeting ANCAs promote GPA pathophysiology. PMID:26436651

  9. Nomenclature and classification of vasculitis: lessons learned from granulomatosis with polyangiitis (Wegener's granulomatosis)

    PubMed Central

    Jennette, J C

    2011-01-01

    Names influence how something is perceived. Diagnostic terms (diagnoses) are the names of diseases that are usually derived either from some distinctive characteristic of the disease or include an eponym recognizing someone who elucidated the disease. No matter how logical and appropriate a name may be, if it is not usable and used it is of no lasting value. This brief commentary focuses on the nomenclature of systemic vasculitides, and uses as a prime example Wegener's granulomatosis, which has been renamed recently ‘granulomatosis with polyangiitis’, in part because of concerns about the suitability of Friedrich Wegener as the source of an eponym. The most distinctive pathological feature of Wegener's granulomatosis is multi-focal necrotizing inflammation that has long been called granulomatosis. The systemic variant of Wegener's granulomatosis also is characterized by inflammation in many different vessels or different types, i.e. polyangiitis. Thus, granulomatosis with polyangiitis is a very appropriate alternative term for Wegener's granulomatosis. This term also is in accord with the name for a closely related vasculitis, i.e. microscopic polyangiitis. Terms that indicate aetiology and pathogenesis, when known, are useful to include in names for diseases (diagnoses). Anti-neutrophil cytoplasmic autoantibodies specific for myeloperoxidase (MPO-ANCA) or proteinase 3 (PR3-ANCA) are implicated in the cause of granulomatosis with polyangiitis and thus also should be specified in the diagnosis (e.g. PR3-ANCA-positive granulomatosis with polyangiitis or MPO-ANCA-positive microscopic polyangiitis). As our understanding of the clinical manifestations, pathogenesis and aetiology of vasculitides change over time, the names and approaches for diagnosing these diseases will change accordingly. PMID:21447122

  10. Identification of Fetal Inflammatory Cells in Eosinophilic/T-cell Chorionic Vasculitis Using Fluorescent In Situ Hybridization.

    PubMed

    Katzman, Philip J; Li, LiQiong; Wang, Nancy

    2015-01-01

    Eosinophilic/T-cell chorionic vasculitis (ETCV) is an inflammatory lesion of placental fetal vessels. In contrast to acute chorionic vasculitis, inflammation in ETCV is seen in chorionic vessel walls opposite the amnionic surface. It is not known whether inflammation in ETCV consists of maternal cells from the intervillous space or fetal cells migrating from the vessel. We used fluorescent in situ hybridization (FISH) to differentiate fetal versus maternal cells in ETCV. Placentas with ETCV, previously identified for a published study, were used. Infant sex in each case was identified using the electronic medical record. For male infants, 3-μm sections were cut from archived tissue blocks from placentas involving ETCV and stained with fluorescent X- and Y-chromosome centromeric probes. A consecutive hematoxylin/eosin-stained section was used for correlation. FISH analysis was performed on 400 interphase nuclei at the site of ETCV to determine the proportion of XX, XY, X, and Y cells. Of 31 ETCV cases, 20 were female and 10 were male (1 sex not recorded). Six of 10 cases with male infants had recuts with visible ETCV. In these 6 cases the average percentages (ranges) of XY cells, X-only cells, and Y-only cells in the region of inflammation were 81 (70-90), 11 (6-17), and 8 (2-14), respectively. There was a 2:1 female:male infant ratio in ETCV. Similar to acute chorionic vasculitis, the inflammation in ETCV is of fetal origin. It is still unknown, however, whether the stimulus for ETCV is of fetal or maternal origin. PMID:25756311

  11. Fatal-Stroke Syndrome Revealing Fungal Cerebral Vasculitis Due to Arthrographis kalrae in an Immunocompetent Patient ▿

    PubMed Central

    Pichon, N.; Ajzenberg, D.; Desnos-Ollivier, M.; Clavel, M.; Gantier, J. C.; Labrousse, F.

    2008-01-01

    We report an uncommon clinical presentation of a unique case of fatal invasive fungal cerebral vasculitis due to Arthrographis kalrae in a nonimmunocompromised host. The identity of the fungus was determined by morphological characteristics and by analysis of internal transcribed spacer 1 sequences and was confirmed by postmortem examination of the brain tissues. Establishing rapidly the link between the clinical syndromes and the fungal infection of the central nervous system is essential to improve the outcome. As our case has shown, it is more challenging to make a diagnosis of fungal infection when there are no risk factors of immunodeficiency and when the clinical presentation seems uncommon. PMID:18650350

  12. Toxic epidermal necrolysis due to therapy with cyclophosphamide and mesna. A case report of a patient with seronegative rheumatoid arthritis and rheumatoid vasculitis.

    PubMed

    Chowdhury, A C; Misra, D P; Patro, P S; Agarwal, V

    2016-03-01

    Rheumatoid vasculitis usually occurs on the background of seropositive rheumatoid arthritis, although in rare cases the patients can be seronegative. We report a woman with seronegative rheumatoid arthritis with rheumatoid vasculitis who developed toxic epidermal necrolysis involving most of her body surface area, while on therapy with intravenous cyclophosphamide and mesna. After withdrawal of suspected offending agents, administration of intravenous immunoglobulin, and supportive therapy, she had a favorable outcome. Such an occurrence is rare and serves to educate about a potentially life-threatening adverse event associated with a commonly used immunosuppressive agent. PMID:26555550

  13. Cutaneous Vasculitis, Interstitial Pneumonia with Crazy-Paving Appearance, and Positive pANCA in a Patient with Severe Crohn's Disease

    PubMed Central

    Chen, Guang-liang; Wang, Juan; Li, Li-mei; Mo, Han-you; Ye, Shuang

    2014-01-01

    Cutaneous vasculitis, interstitial pneumonia with crazy-paving appearance on high-resolution computed tomography, and repeated positive perinuclear anti-neutrophil cytoplasmic antibodies (pANCA) are rarely found together in patients with inflammatory bowel disease in the existing literature. We report the case of a Chinese patient previously diagnosed with cutaneous vasculitis and interstitial pneumonia, who presented with acute pain and mass in his right lower quadrant a couple of years later. The terminal ileum biopsy and postoperative pathology confirmed Crohn's disease (CD). PMID:25371834

  14. Multidrug-related leukocytoclastic vasculitis raising suspicion of sexual homicide-things are not always what they seem.

    PubMed

    Tattoli, Lucia; Krocker, Klaus; Sautter, Julia; Tsokos, Michael

    2016-01-01

    Ambiguous findings during external examination of a deceased in combination with dubious autopsy findings can raise doubts concerning the manner and cause of death. We report the case of a 35-year-old female deceased who had suffered from a borderline personality and depressive disorder with suicidal ideation. At the death scene, the body showed massive facial swelling accompanied by complete reddening of the skin of the face, with patchy skin abrasions on the forehead and neck, and purple bruise-like discolorations distributed symmetrically over both shoulders, elbows, hands, hips, knees, lower legs, and feet, raising the suspicion of underlying massive external blunt force injury. Police investigators strongly suspected sexual homicide. At autopsy, dissection in layers revealed massive subcutaneous hemorrhages as the cause of the reddish skin discolorations. Toxicological analyses showed fatal levels of lamotrigine with additional proof of zopiclone, zolpidem, diphenhydramine, O-desmethylvenlafaxine, pregabalin, tramadol, and modafinil in venous blood. Histologically, both the macroscopically impressive purple skin changes with underlying bleeding into the subcutaneous tissue and the skin abrasions were due to leukocytoclastic vasculitis, a form of acute hypersensitivity vasculitis that was a reaction to the multiple therapeutic drugs that the woman had taken shortly before death. The manner of death was classified as suicide, and sexual homicide was ruled out. PMID:25957602

  15. Hereditary leukoencephalopathy with axonal spheroids: a spectrum of phenotypes from CNS vasculitis to parkinsonism in an adult onset leukodystrophy series

    PubMed Central

    Jaunmuktane, Zane; Sheerin, Una-Marie; Phadke, Rahul; Brandner, Sebastian; Milonas, Ionnis; Dean, Andrew; Bajaj, Nin; McNicholas, Nuala; Costello, Daniel; Cronin, Simon; McGuigan, Chris; Rossor, Martin; Fox, Nick; Murphy, Elaine; Chataway, Jeremy; Houlden, Henry

    2016-01-01

    Background Hereditary diffuse leukoencephalopathy with neuroaxonal spheroids (HDLS) is a hereditary, adult onset leukodystrophy which is characterised by the presence of axonal loss, axonal spheroids and variably present pigmented macrophages on pathological examination. It most frequently presents in adulthood with dementia and personality change. HDLS has recently been found to be caused by mutations in the colony stimulating factor-1 receptor (CSF1R) gene. Methods In this study, we sequenced the CSF1R gene in a cohort of 48 patients from the UK, Greece and Ireland with adult onset leukodystrophy of unknown cause. Results Five pathogenic mutations were found, including three novel mutations. The presentations ranged from suspected central nervous system (CNS) vasculitis to extrapyramidal to cognitive phenotypes. The case histories and imaging are presented here, in addition to neuropathological findings from two cases with novel mutations. Conclusion We estimate that CSF1R mutations account for 10% of idiopathic adult onset leukodystrophies and that genetic testing for CSF1R mutations is essential in adult patients presenting with undefined CNS vasculitis or a leukodystrophy with prominent neuropsychiatric signs or dementia. PMID:25935893

  16. East Coast Fever Caused by Theileria parva Is Characterized by Macrophage Activation Associated with Vasculitis and Respiratory Failure.

    PubMed

    Fry, Lindsay M; Schneider, David A; Frevert, Charles W; Nelson, Danielle D; Morrison, W Ivan; Knowles, Donald P

    2016-01-01

    Respiratory failure and death in East Coast Fever (ECF), a clinical syndrome of African cattle caused by the apicomplexan parasite Theileria parva, has historically been attributed to pulmonary infiltration by infected lymphocytes. However, immunohistochemical staining of tissue from T. parva infected cattle revealed large numbers of CD3- and CD20-negative intralesional mononuclear cells. Due to this finding, we hypothesized that macrophages play an important role in Theileria parva disease pathogenesis. Data presented here demonstrates that terminal ECF in both Holstein and Boran cattle is largely due to multisystemic histiocytic responses and resultant tissue damage. Furthermore, the combination of these histologic changes with the clinical findings, including lymphadenopathy, prolonged pyrexia, multi-lineage leukopenia, and thrombocytopenia is consistent with macrophage activation syndrome. All animals that succumbed to infection exhibited lymphohistiocytic vasculitis of small to medium caliber blood and lymphatic vessels. In pulmonary, lymphoid, splenic and hepatic tissues from Holstein cattle, the majority of intralesional macrophages were positive for CD163, and often expressed large amounts of IL-17. These data define a terminal ECF pathogenesis in which parasite-driven lymphoproliferation leads to secondary systemic macrophage activation syndrome, mononuclear vasculitis, pulmonary edema, respiratory failure and death. The accompanying macrophage phenotype defined by CD163 and IL-17 is presented in the context of this pathogenesis. PMID:27195791

  17. East Coast Fever Caused by Theileria parva Is Characterized by Macrophage Activation Associated with Vasculitis and Respiratory Failure

    PubMed Central

    Schneider, David A.; Frevert, Charles W.; Nelson, Danielle D.; Morrison, W. Ivan; Knowles, Donald P.

    2016-01-01

    Respiratory failure and death in East Coast Fever (ECF), a clinical syndrome of African cattle caused by the apicomplexan parasite Theileria parva, has historically been attributed to pulmonary infiltration by infected lymphocytes. However, immunohistochemical staining of tissue from T. parva infected cattle revealed large numbers of CD3- and CD20-negative intralesional mononuclear cells. Due to this finding, we hypothesized that macrophages play an important role in Theileria parva disease pathogenesis. Data presented here demonstrates that terminal ECF in both Holstein and Boran cattle is largely due to multisystemic histiocytic responses and resultant tissue damage. Furthermore, the combination of these histologic changes with the clinical findings, including lymphadenopathy, prolonged pyrexia, multi-lineage leukopenia, and thrombocytopenia is consistent with macrophage activation syndrome. All animals that succumbed to infection exhibited lymphohistiocytic vasculitis of small to medium caliber blood and lymphatic vessels. In pulmonary, lymphoid, splenic and hepatic tissues from Holstein cattle, the majority of intralesional macrophages were positive for CD163, and often expressed large amounts of IL-17. These data define a terminal ECF pathogenesis in which parasite-driven lymphoproliferation leads to secondary systemic macrophage activation syndrome, mononuclear vasculitis, pulmonary edema, respiratory failure and death. The accompanying macrophage phenotype defined by CD163 and IL-17 is presented in the context of this pathogenesis. PMID:27195791

  18. Trypanosoma cruzi Causes Paralyzing Systemic Necrotizing Vasculitis Driven by Pathogen-Specific Type I Immunity in Mice.

    PubMed

    Roffê, Ester; Marino, Ana Paula M P; Weaver, Joseph; Wan, Wuzhou; de Araújo, Fernanda F; Hoffman, Victoria; Santiago, Helton C; Murphy, Philip M

    2016-04-01

    Infectious agents are often considered potential triggers of chronic inflammatory disease, including autoimmunity; however, direct evidence is usually lacking. Here we show that following control of acute infection of mice with the myotropic Colombiana strain of Trypanosoma cruzi, parasites persisted in tissue at low levels associated with development of systemic necrotizing vasculitis. Lesions occurred in many but not all organs and tissues, with skeletal muscle arteries being the most severely affected, and were associated with myositis, atrophy, paresis/paralysis, and death. Histopathology showed fibrinoid vascular necrosis, rare amastigote nests within skeletal muscle myocytes, and massive leukocyte infiltrates composed mainly of inflammatory monocytes, F4/80(+)macrophages, and T. cruzi tetramer-specific CD8(+) T lymphocytes capable of producing gamma interferon (IFN-γ) and tumor necrosis factor alpha (TNF-α) but not interleukin-17 (IL-17). T. cruzi-specific IgG was detected in sera from infected mice, but antibody deposits and neutrophilic inflammation were not features of the lesions. Thus,T. cruzi infection of mice may be a specific infectious trigger of paralyzing systemic necrotizing vasculitis most severely affecting skeletal muscle, driven by pathogen-specific type I immune responses. PMID:26857570

  19. Henoch-Schönlein purpura from vasculitis to intestinal perforation: A case report and literature review

    PubMed Central

    Lerkvaleekul, Butsabong; Treepongkaruna, Suporn; Saisawat, Pawaree; Thanachatchairattana, Pornsri; Angkathunyakul, Napat; Ruangwattanapaisarn, Nichanan; Vilaiyuk, Soamarat

    2016-01-01

    Henoch-Schönlein purpura (HSP) is generally a self-limited vasculitis disease and has a good prognosis. We report a 4-year-old Thai boy who presented with palpable purpura, abdominal colicky pain, seizure, and eventually developed intestinal ischemia and perforation despite adequate treatment, including corticosteroid and intravenous immunoglobulin therapy. Imaging modalities, including ultrasonography and contrast-enhanced computed tomography, could not detect intestinal ischemia prior to perforation. In this patient, we also postulated that vasculitis-induced mucosal ischemia was a cause of the ulcer, leading to intestinal perforation, and high-dose corticosteroid could have been a contributing factor since the histopathology revealed depletion of lymphoid follicles. Intestinal perforation in HSP is rare, but life-threatening. Close monitoring and thorough clinical evaluation are essential to detect bowel ischemia before perforation, particularly in HSP patients who have hematochezia, persistent localized abdominal tenderness and guarding. In highly suspicious cases, exploratory laparotomy may be needed for the definite diagnosis and prevention of further complications. PMID:27468201

  20. A case of systemic lupus erythematosus associated with longitudinal extensive transverse myelitis, cerebral neutrophilic vasculitis, and cerebritis.

    PubMed

    Modjinou, Dodji; Gurin, Lindsey; Chhabra, Arpit; Mikolaenko, Irina; Lydon, Eileen; Smiles, Stephen

    2014-01-01

    Systemic Lupus erythematosus (SLE) is an autoimmune disease with multiple clinical presentations and manifestations. Here, we report an intriguing case of a 30-year-old female with full-blown SLE, associated with longitudinal extensive transverse myelitis (LETM) on Magnetic Resonance Imaging (MRI) manifested by lower extremity weakness, neurogenic bladder and bowel, and central nervous system (CNS) lupus clinically manifested by changes in mood and behavior as well as neutrophilic vasculitis and cerebritis on pathology. LETM is a rare complication of SLE; however, what makes this case even more intriguing is that it additionally had cerebral lesions consistent with neutrophilic vasculitis and cerebritis, and that it may all have started at least 10 years prior with nonspecific musculoskeletal manifestations subsequently followed by a rash as well as intractable fevers of unknown etiology—much later attributed to her lupus. Although she had a most concerning and dramatic presentation, she, so far, had responded very well to therapy including pulse dose steroids, plasmapheresis, intravenous immunoglobulins (IVIG), cyclophosphamide, and related medications. PMID:25986355

  1. Henoch-Schönlein purpura from vasculitis to intestinal perforation: A case report and literature review.

    PubMed

    Lerkvaleekul, Butsabong; Treepongkaruna, Suporn; Saisawat, Pawaree; Thanachatchairattana, Pornsri; Angkathunyakul, Napat; Ruangwattanapaisarn, Nichanan; Vilaiyuk, Soamarat

    2016-07-14

    Henoch-Schönlein purpura (HSP) is generally a self-limited vasculitis disease and has a good prognosis. We report a 4-year-old Thai boy who presented with palpable purpura, abdominal colicky pain, seizure, and eventually developed intestinal ischemia and perforation despite adequate treatment, including corticosteroid and intravenous immunoglobulin therapy. Imaging modalities, including ultrasonography and contrast-enhanced computed tomography, could not detect intestinal ischemia prior to perforation. In this patient, we also postulated that vasculitis-induced mucosal ischemia was a cause of the ulcer, leading to intestinal perforation, and high-dose corticosteroid could have been a contributing factor since the histopathology revealed depletion of lymphoid follicles. Intestinal perforation in HSP is rare, but life-threatening. Close monitoring and thorough clinical evaluation are essential to detect bowel ischemia before perforation, particularly in HSP patients who have hematochezia, persistent localized abdominal tenderness and guarding. In highly suspicious cases, exploratory laparotomy may be needed for the definite diagnosis and prevention of further complications. PMID:27468201

  2. An Update of the Mayo Clinic Cohort of Patients With Adult Primary Central Nervous System Vasculitis

    PubMed Central

    Salvarani, Carlo; Brown, Robert D.; Christianson, Teresa; Miller, Dylan V.; Giannini, Caterina; Huston, John; Hunder, Gene G.

    2015-01-01

    Abstract Primary central nervous system vasculitis (PCNSV) is an uncommon condition in which lesions are limited to vessels of the brain and spinal cord. Because the clinical manifestations are not specific, the diagnosis is often difficult, and permanent disability and death are frequent outcomes. This study is based on a cohort of 163 consecutive patients with PCNSV who were examined at the Mayo Clinic over a 29-year period from 1983 to 2011. The aim of the study was to define the characteristics of these patients, which represents the largest series in adults reported to date. A total of 105 patients were diagnosed by angiographic findings and 58 by biopsy results. The patients diagnosed by biopsy more frequently had at presentation cognitive dysfunction, greater cerebrospinal fluid total protein concentrations, less frequent cerebral infarcts, and more frequent leptomeningeal gadolinium-enhanced lesions on magnetic resonance imaging (MRI), along with less mortality and disability at last follow-up. The patients diagnosed by angiograms more frequently had at presentation hemiparesis or a persistent neurologic deficit or stroke, more frequent infarcts on MRI and an increased mortality. These differences were mainly related to the different size of the vessels involved in the 2 groups. Although most patients responded to therapy with glucocorticoids alone or in conjunction with cyclophosphamide and tended to improve during the follow-up period, an overall increased mortality rate was observed. Relapses occurred in one-quarter of the patients and were less frequent in patients treated with prednisone and cyclophosphamide compared with those treated with prednisone alone. The mortality rate and degree of disability at last follow-up were greater in those with increasing age, cerebral infarctions on MRI, angiographic large vessel involvement, and diagnosis made by angiography alone, but were lower in those with gadolinium-enhanced lesions on MRI and in those with

  3. Incidence of Cancer in ANCA-Associated Vasculitis: A Meta-Analysis of Observational Studies

    PubMed Central

    Shang, Weifeng; Ning, Yong; Xu, Xiu; Li, Menglan; Guo, Shuiming; Han, Min; Zeng, Rui; Ge, Shuwang; Xu, Gang

    2015-01-01

    Objective The purpose of this paper is to examine cancer incidence in patients with ANCA-associated vasculitis (AASV) derived from population-based cohort studies by means of meta-analysis. Methods Relevant electronic databases were searched for studies characterizing the associated risk of overall malignancy in patients with AASV. Standardized incidence rates (SIRs) with 95% confidence intervals (CIs) were used to evaluate the strength of association. We tested for publication bias and heterogeneity and stratified for site-specific cancers. Results Six studies (n = 2,578) were eventually identified, of which six provided the SIR for overall malignancy, five reported the SIR for non-melanoma skin cancer (NMSC), four for leukemia, five for bladder cancer, three for lymphoma, three for liver cancer, four for lung cancer, three for kidney cancer, four for prostate cancer, four for colon cancer and four for breast cancer. Overall, the pooled SIR of cancer in AASV patients was 1.74 (95%CI = 1.37–2.21), with moderate heterogeneity among these studies (I2 = 65.8%, P = 0.012). In sub-analyses for site-specific cancers, NMSC, leukemia and bladder cancer were more frequently observed in patients with AASV with SIR of 5.18 (95%CI = 3.47–7.73), 4.89 (95%CI = 2.93–8.16) and 3.84 (95%CI = 2.72–5.42) respectively. There was no significant increase in the risk of kidney cancer (SIR = 2.12, 95%CI = 0.66–6.85), prostate cancer (SIR = 1.45, 95%CI = 0.87–2.42), colon cancer (SIR = 1.26, 95%CI = 0.70–2.27), and breast cancer (SIR = 0.95, 95%CI = 0.50–1.79). Among these site-specific cancers, only NMSC showed moderate heterogeneity (I2 = 55.8%, P = 0.06). No publication bias was found by using the Begg’s test and Egger's test. Conclusions This meta-analysis shows that AASV patients treatment with cyclophosphamide (CYC) are at increased risk of late-occurring malignancies, particularly of the NMSC, leukemia and bladder cancer. However, there is no significant

  4. Neutrophil extracellular trap formation is associated with autophagy-related signalling in ANCA-associated vasculitis

    PubMed Central

    Tang, S; Zhang, Y; Yin, S-W; Gao, X-J; Shi, W-W; Wang, Y; Huang, X; Wang, L; Zou, L-Y; Zhao, J-H; Huang, Y-J; Shan, L-Y; Gounni, A S; Wu, Y-Z; Zhang, J-B

    2015-01-01

    Increasing evidence indicates that aberrant neutrophil extracellular trap (NET) formation could contribute to the pathogenesis of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Recent research has provided evidence that a novel type of ANCA autoantibody, anti-lysosomal membrane protein-2 (LAMP-2) antibody, may have a pathogenic role in AAV. We have shown previously that anti-LAMP-2 antibody-stimulated NET formation contains autoantigens and anti-microbial peptides. The current study sought to determine whether LAMP-2, as a novel antigen of ANCA, was present on NETs in AAV patients, the influence of the anti-LAMP-2 antibody on the neutrophil apoptosis rate and the role of autophagy in anti-LAMP-2 antibody-induced NET formation. NET formation was assessed using immunofluorescence microscopy, scanning electron microscopy or live cell imaging. The neutrophil apoptosis rate was analysed using fluorescence activated cell sorting (FACS). Autophagy was detected using LC3B accumulation and transmission electron microscopy. The results showed that enhanced NET formation, which contains LAMP-2, was observed in kidney biopsies and neutrophils from AAV patients. The apoptosis rate decreased significantly in human neutrophils stimulated with anti-LAMP-2 antibody, and this effect was attenuated by the inhibitors of autophagy 3-methyladenine (3MA) and 2-morpholin-4-yl-8-phenylchromen-4-one (LY294002). The anti-LAMP-2 antibody-stimulated NET formation was unaffected by benzyloxycarbonyl-Val- Ala-Asp (OMe)-fluoromethylketone (zVAD-fmk) and necrostatin-1 (Nec-1), which are inhibitors of apoptosis and necrosis, respectively, but was inhibited by 3MA and LY294002. Moreover, the proportion of LC3BI that was converted to LC3BII increased significantly (P = 0·0057), and massive vacuolizations that exhibited characteristics typical of autophagy were detected in neutrophils stimulated with anti-LAMP-2 antibody. Our results provide further evidence that

  5. Autoantibodies against complement C1q specifically target C1q bound on early apoptotic cells.

    PubMed

    Bigler, Cornelia; Schaller, Monica; Perahud, Iryna; Osthoff, Michael; Trendelenburg, Marten

    2009-09-01

    Autoantibodies against complement C1q (anti-C1q) are frequently found in patients with systemic lupus erythematosus (SLE). They strongly correlate with the occurrence of severe lupus nephritis, suggesting a pathogenic role in SLE. Because anti-C1q are known to recognize a neoepitope on bound C1q, but not on fluid-phase C1q, the aim of this study was to clarify the origin of anti-C1q by determining the mechanism that renders C1q antigenic. We investigated anti-C1q from serum and purified total IgG of patients with SLE and hypocomplementemic urticarial vasculitis as well as two monoclonal human anti-C1q Fab from a SLE patient generated by phage display. Binding characteristics, such as their ability to recognize C1q bound on different classes of Igs, on immune complexes, and on cells undergoing apoptosis, were analyzed. Interestingly, anti-C1q did not bind to C1q bound on Igs or immune complexes. Neither did we observe specific binding of anti-C1q to C1q bound on late apoptotic/necrotic cells when compared with binding in the absence of C1q. However, as shown by FACS analysis and confocal microscopy, anti-C1q specifically targeted C1q bound on early apoptotic cells. Anti-C1q were found to specifically target C1q bound on cells undergoing apoptosis. Our observations suggest that early apoptotic cells are a major target of the autoimmune response in SLE and provide a direct link between human SLE, apoptosis, and C1q. PMID:19648280

  6. Role of Brain Perfusion SPECT with 99mTc HMPAO in the Assessment of Response to Drug Therapy in Patients with Autoimmune Vasculitis: A Prospective Study

    PubMed Central

    Mauro, Liberatore; Manuela, Morreale; Valentina, Megna; Sara, Collorone; Chondrogiannis, Sotirios; Maria, Drudi Francesco; Christos, Anagnostou; Liana, Civitelli; Ada, Francia; Maffione, Anna Margherita; Marzola, Maria Cristina; Rubello, Domenico

    2015-01-01

    Background: The diagnosis of vasculitis in the brain remains a quite difficult achievement. To the best of our knowledge, there is no imaging method reported in literature which is capable of reaching to a diagnosis of vasculitis with very high sensitivity. Aim: The aim of this study was to determine whether perfusion brain single photon emission computed tomography (SPECT) can be usefully employed in monitoring the treatment of vasculitis, allowing treating only potentially responder patients and avoiding the side effects on patients who do not respond. Materials and Methods: Twenty patients (two males and 18 females) suffering from systemic lupus erythematosus (SLE; n = 5), Behcet's disease (BD; n = 5), undifferentiated vasculitis (UV; n = 5), and Sjogren's syndrome (SS; n = 5) were included in the study. All patients underwent a wide neurological anamnestic investigation, a complete objective neurological examination and SPECT of the brain with 99mTc-hexamethyl-propylene-aminoxime (HMPAO). The brain SPECT was then repeated after appropriate medical treatment. The neurological and neuropsychiatric follow-up was performed at 6 months after the start of the treatment. Results: Overall, the differences between the scintigraphic results obtained after and before the medical treatment indicated a statistically significant increase of the cerebral perfusion (CP). In 19 out of 200 regions of interest (ROI) studied, the difference between pre- and post treatment percentages had negative sign, indicating a worsening of CP. This latter event has occurred six times (five in the same patients) in the UV, 10 times (eight in the same patients) in the SLE, never in BD, and three times (two in the same patient) in the SS. Conclusion: The reported results seem to indicate the possibility of identifying, by the means of a brain SPECT, responder and nonresponder (unchanged or worsened CP) patients, affected by autoimmune vasculitis, to the therapy. PMID:25973400

  7. Antineutrophil Cytoplasmic Antibody-associated Vasculitis Superimposed on Infection-related Glomerulonephritis Secondary to Pulmonary Mycobacterium avium Complex Infection.

    PubMed

    Asano, Shuichi; Mizuno, Shige; Okachi, Shotaro; Aso, Hiromichi; Wakahara, Keiko; Hashimoto, Naozumi; Ito, Satoru; Kozaki, Yohei; Katsuno, Takayuki; Maruyama, Shoichi; Hasegawa, Yoshinori

    2016-01-01

    A 73-year-old woman was diagnosed with pulmonary Mycobacterium avium complex (MAC) infection and received no treatment. Disease progression was evident one year later with the development of myeloperoxidase-antineutrophil cytoplasmic antibody (ANCA) titers and systemic symptoms of a fever, polyarthritis, purpura, and rapidly progressive glomerulonephritis. Her symptoms did not improve with antibiotic treatment. A renal biopsy revealed crescentic glomerulonephritis with immunodeposition. According to these findings, she was diagnosed with ANCA-associated vasculitis (AAV) superimposed on infection-related glomerulonephritis (IRGN). Although there was a risk of aggravating an underlying infection, the combination therapy of corticosteroid and antibiotics improved AAV, IRGN, and even the lung radiological findings. To the best of our knowledge, this is the first case of AAV and IRGN secondary to pulmonary MAC infection. PMID:27580547

  8. Sequential occurrence of anti-glomerular basement membrane disease 9 years after anti-neutrophil cytoplasmic antibody-associated vasculitis

    PubMed Central

    Chan, Pui Shan Julia; Leung, Moon Ho

    2016-01-01

    We report a case of 63-year-old Chinese man, having a history of anti-myeloperoxidase (MPO) antibody anti-neutrophil cytoplasmic antibody (ANCA)-associated pulmonary-renal syndrome 9 years ago, presented with second episode of rapidly progressive glomerulonephritis (RPGN) and alveolar haemorrhage compatible with anti-glomerular basement membrane (GBM) disease. In first presentation, his anti-GBM antibody was negative. This time, anti-MPO antibody was negative, but anti-GBM antibody was positive. The long interval of sequential development of anti-GBM disease after ANCA-associated vasculitis in this patient may provide clues to the potential immunological links between these two distinct conditions. Clinicians should be aware of such double-positive association. PMID:27123311

  9. Immune complexes activate human endothelium involving the cell-signaling HMGB1-RAGE axis in the pathogenesis of lupus vasculitis.

    PubMed

    Sun, Wenping; Jiao, Yulian; Cui, Bin; Gao, Xuejun; Xia, Yu; Zhao, Yueran

    2013-06-01

    Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by the formation of immune complexes (ICs), which contain a complex mixture of autoantigens nucleic acids, nucleic acids-associated proteins and corresponding autoantibodies. In SLE, ICs are deposited in multiple organs. Vasculopathy and vasculitis in SLE are typical complications and are associated with deposition of ICs on endothelium, endothelial activation and inflammatory cell infiltration. However, the effects of ICs on endothelial cells and the mechanisms involved remain unclear. In this study, we have demonstrated for the first time that ICs upregulated cell surface expression of the receptor for advanced glycation end products (RAGE), the expression of intercellular adhesion molecule-1 (ICAM-1), vascular cell adhesion molecule-1 (VCAM-1), increased the secretion of the chemokines interleukin 8 (IL-8), monocyte chemoattractant protein-1 (MCP-1), the proinflammatoy cytokines interleukin 6 (IL-6), tumor necrosis factor-α (TNF-α) and promoted the activation of the transcription factor NF-κB p65 in human endothelial cells (P<0.05). ICs also increased transendothelial migration of monocytes (P<0.05). One of the mechanisms underlying these activating effects of ICs on human endothelial cells involves cell signaling by high-mobility group box 1 protein (HMGB1)-RAGE axis, as these effects can be partially blocked by HMGB1 A-box, soluble RAGE (sRAGE), SB203580, PD98059, Bay 117082 (P<0.05) and co-treatment with these agents (P<0.05). In conclusion, ICs elicit proinflammatory responses in human endothelial cells and alter their function involving cellular signaling via the HMGB1-RAGE axis in the pathogenesis of SLE vasculitis. PMID:23628898

  10. Higher Serum Angiotensinogen Is an Indicator of IgA Vasculitis with Nephritis Revealed by Comparative Proteomes Analysis

    PubMed Central

    Luan, Jiangwei; Zhao, Peiwei; Yue, Xin; Yu, Chunhua; Laing, Xiaohui; Zhao, YuLan

    2015-01-01

    IgA vasculitis (IgAV), previously named as Henoch–Schönlein purpura, is the most common systematic vasculitis with unknown etiology. Lack of appropriate study system and/or animal model limits the understanding of its molecular pathogenesis and hinders the identification of targets for rational therapy, especially for its long-term complication, IgAV nephritis (IgAVN). In this study, we applied comparative analysis of serum proteomes to obtain an insight about disease pathogenesis. This study has utilized high sensitivity nanoscale ultra performance liquid chromatography-mass spectrometry (nanoLC-MS/MS) to investigate the alterations in serum proteomic profiles in patients with IgAV (n=6), IgAVN (n=6) and healthy subjects (n=7). The differentially expressed proteins were subjected to functional pathway analysis by PANTHER and DAVID software. We identified 107 differentially expressed proteins among three different groups, and functional analysis suggested that, in addition to earlier reported pathways, such as acute phase response, immune response, complement and blood coagulation pathways, hemostasis and Wnt signaling pathway were probably involved in pathogenesis of IgAV. A few differentially abundant proteins identified, such as C4a, serum amyloid A, angiotensinogen, and kininogen 1, were further validated by ELISA. More importantly, we found that angiotensinogen concentration is correlated with IgAVN and could be used as a potential marker for the progression of IgAV. This is the first report of analyzing the proteomic alterations in IgAV patients and the differentially proteins identified in this study may enhance understanding of the pathology of IgAV and a few of them may be used to monitor disease progression. PMID:26098644

  11. Neutrophilic Dermatoses in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis: A French Multicenter Study of 17 Cases and Literature Review.

    PubMed

    de Boysson, Hubert; Martin Silva, Nicolas; de Moreuil, Claire; Néel, Antoine; de Menthon, Mathilde; Meyer, Olivier; Launay, David; Pagnoux, Christian; Guillevin, Loïc; Puéchal, Xavier; Bienvenu, Boris; Aouba, Achille

    2016-03-01

    A few reports suggest combination of ANCA-associated vasculitis (AAV) and neutrophilic dermatoses (ND). We aimed to describe the main characteristics of patients presenting with both AAV and ND in a French cohort and through a systematic literature review, and to discuss the possible common pathogenic process involved. We conducted a retrospective study of patients with both conditions. Patients were selected via the French Internal Medicine Society (SNFMI) and the French Vasculitis Study Group (FVSG). A literature review focusing on a combination of both conditions, concentrated only on publications with well-established diagnoses and individual detailed data. Seventeen patients diagnosed with AAV and ND were identified in this cohort. Twelve patients had granulomatosis with polyangiitis (GPA), 4 had microscopic polyangiitis (MPA) and one had eosinophilic GPA (EGPA). Eight patients, all with GPA, displayed pyoderma gangrenosum (PG). Sweet's syndrome was observed in 6 patients (4 with MPA, one with GPA and one with EGPA) and erythema elevatum diutinum in the other three (2 with GPA and 1 with MPA). The literature review identified 33 additional patients with both conditions, including 26 with GPA. Altogether, of the 50 patients (17 from our study and 33 from the literature review), 33 (66%) patients presented with PG associated with GPA in 29 cases (89%). Corticosteroids were the first-line treatment in conjunction with an immunosuppressive agent in most cases. Outcomes were good and a total of 15 patients experienced a relapse. Patients who relapsed were more likely to have ear, nose and throat manifestation than patients who did not [12/15 (80%) relapsing patients vs. 15/35 (43%) non-relapsing patients; p = 0.03)]. In our stud, the most frequent association concerned GPA and PG. ND should be considered and specifically researched within the spectrum of cutaneous manifestations observed in AAV. PMID:26986103

  12. Higher Serum Angiotensinogen Is an Indicator of IgA Vasculitis with Nephritis Revealed by Comparative Proteomes Analysis.

    PubMed

    He, Xuelian; Yin, Wei; Ding, Yan; Cui, Shu-jian; Luan, Jiangwei; Zhao, Peiwei; Yue, Xin; Yu, Chunhua; Laing, Xiaohui; Zhao, YuLan

    2015-01-01

    IgA vasculitis (IgAV), previously named as Henoch-Schönlein purpura, is the most common systematic vasculitis with unknown etiology. Lack of appropriate study system and/or animal model limits the understanding of its molecular pathogenesis and hinders the identification of targets for rational therapy, especially for its long-term complication, IgAV nephritis (IgAVN). In this study, we applied comparative analysis of serum proteomes to obtain an insight about disease pathogenesis. This study has utilized high sensitivity nanoscale ultra performance liquid chromatography-mass spectrometry (nanoLC-MS/MS) to investigate the alterations in serum proteomic profiles in patients with IgAV (n=6), IgAVN (n=6) and healthy subjects (n=7). The differentially expressed proteins were subjected to functional pathway analysis by PANTHER and DAVID software. We identified 107 differentially expressed proteins among three different groups, and functional analysis suggested that, in addition to earlier reported pathways, such as acute phase response, immune response, complement and blood coagulation pathways, hemostasis and Wnt signaling pathway were probably involved in pathogenesis of IgAV. A few differentially abundant proteins identified, such as C4a, serum amyloid A, angiotensinogen, and kininogen 1, were further validated by ELISA. More importantly, we found that angiotensinogen concentration is correlated with IgAVN and could be used as a potential marker for the progression of IgAV. This is the first report of analyzing the proteomic alterations in IgAV patients and the differentially proteins identified in this study may enhance understanding of the pathology of IgAV and a few of them may be used to monitor disease progression. PMID:26098644

  13. C-ANCA-positive IgG fraction from patients with Wegener's granulomatosis induces lung vasculitis in rats

    PubMed Central

    WEIDEBACH, W; VIANA, V S T; LEON, E P; BUENO, C; LEME, A S; ARANTES-COSTA, F M; MARTINS, M A; SALDIVA, P H N; BONFA, E

    2002-01-01

    The aim of the present study was to analyse in rats the ability of C-ANCA-positive IgG fraction in triggering inflammatory response on pulmonary tissue. Wistar rats (n = 18) were injected via the the internal jugular vein with 20 mg of total C-ANCA-positive IgG fraction isolated from serum of three different Wegener's granulomatosis patients obtained before therapy. Similarly, control rats were treated with IgG fraction from two rheumatoid arthritis patients (n = 7), IgG from six normal human sera (n = 15) or saline (n = 18), respectively. Animals were sacrificed after 24h of injection for histological analysis of the lungs. Vasculitis and inflammatory infiltrate were consistently absent in rats injected with rheumatoid arthritis IgG or saline and in 14/15 of normal IgG treated animals. In contrast, marked vasculitis was observed in all 18 animals injected with C-ANCA-positive IgG fraction. The histological features were characterized by the presence of a perivascular pleomorphic cellular sheath, particularly around small vessels, endothelial adherence and diapedesis of polymorphonuclear leucocytes and presence of granuloma-like lesions. A dose–response relationship was observed between protein concentration of C-ANCA IgG sample and the intensity of the inflammatory response in the animals. In addition, IgG fraction with undetectable C-ANCA, obtained from one patient in remission after treatment, was not able to reproduce the pulmonary tissue alterations induced by its paired IgG that was positive for C-ANCA taken before therapy. The experimental model described herein may be useful to characterize more effectively the pathogenic mechanism of C-ANCA in Wegener's disease. PMID:12100022

  14. Successful Treatment of Dual-Positive Anti-Myeloperoxidase and Anti-Glomerular Basement Membrane Antibody Vasculitis with Pulmonary-Renal Syndrome

    PubMed Central

    Huang, Jinxian; Wu, Ling; Huang, Xiaoyan; Xie, Yan; Yu, Jinquan; Yang, Jin; Fang, Huiqiong; Zhang, Lijun

    2016-01-01

    Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis and anti-glomerular basement membrane (GBM) disease are two separate diseases, while sometimes they can coexist together. The exact mechanisms are not clear, but due to the rapid progression and poor prognosis, prompt and aggressive treatment is usually required. We treated with steroids combined with cyclophosphamide and rituximab an 84-year-old man with ANCA-associated vasculitis and anti-GBM disease who had prior pulmonary fibrosis and a coexisting anterosuperior mediastinal mass. Conventional therapy including steroids, plasmapheresis and cyclophosphamide failed to attenuate the anti-GBM disease, yet he responded to an alternative treatment of rituximab. This case suggests the efficacy of steroids and immunosuppressant for the treatment of a dual-positive case with an anterosuperior mediastinal mass. PMID:26889474

  15. Leukocytoclastic vasculitis as early manifestation of Epstein-Barr virus-positive diffuse large B-cell lymphoma of the elderly.

    PubMed

    Zoroquiain, Pablo; González, Sergio; Molgó, Montserrat; Rodríguez, Alejandra; Valbuena, José R

    2012-05-01

    Extensive necrotizing vasculitis (ENV) is a rare paraneoplastic phenomenon, and the majority of cases reported are associated with hematolymphoid neoplasms. Histologically, most cases of ENV represent leukocytoclastic vasculitis (LCV). Here we report the clinicopahological features of a 68-year-old man with ENV associated to a Epstein Barr virus-positive diffuse large B-cell lymphoma (EBV+DLBCL) of the elderly, a newly recognized lymphoproliferative disorder, most likely representing a paraneoplastic manifestation. The patient was treated with standard chemotherapy regimen for malignant lymphoma. Due to the extensive involvement of the extremities by ENV, surgical debridement was not feasible and a novel therapy based on CHITOSAN apposits was initiated with overall good response and subsequent re-epithelization of the skin lesions. The patient died of sepsis secondary to a Pseudomona pneumonia 17 months after diagnosis. PMID:22197862

  16. Levamisole-induced leukocytoclastic vasculitis and neutropenia in a patient with cocaine use: an extensive case with necrosis of skin, soft tissue, and cartilage.

    PubMed

    Arora, Natasha Purai; Jain, Tania; Bhanot, Ravinder; Natesan, Suganthini Krishnan

    2012-01-01

    Levamisole-induced vasculitis is a relatively new entity in people who use cocaine. We describe a 44-year-old woman with a history of cocaine use who presented with a complaint of a painful rash of 2-3 month's duration on her extremities, cheeks, nose, and earlobes. She had not experienced fever, weight loss, alopecia, dry eyes, oral ulcers, photosensitivity, or arthralgia. Examination revealed tender purpuric eruptions with central necrosis on her nose, cheeks, earlobes, and extremities. Laboratory investigations revealed neutropenia, an elevated erythrocyte sedimentation rate (ESR), presence of lupus anticoagulant, low complement component 3 (C3), and presence of perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA). A urine toxicology screen was positive for cocaine, and gas chromatography-mass spectrometry was positive for levamisole. Skin biopsy showed leukocytoclastic vasculitis and small vessel thrombosis. Necrotic lesions of the nose led to its self-amputation. Large bullae on the lower extremities ruptured, leading to wound infection and extensive necrosis that required multiple surgical debridements. When necrosis progressed despite debridement, bilateral above-knee amputation of the legs was performed. Once new lesions stopped appearing, the patient was discharged home. Two months later, she had a recurrence related to cocaine use. To the best of our knowledge, this is only the second reported case of levamisole-induced vasculitis that required above-knee amputation. PMID:23186390

  17. Tissue microscopic changes and artifacts in multi-phase post-mortem computed tomography angiography in a hospital setting: a fatal case of systemic vasculitis.

    PubMed

    Capuani, Caroline; Guilbeau-Frugier, Céline; Mokrane, Fatima-Zohra; Delisle, Marie-Bernadette; Marcheix, Bertrand; Rousseau, Hervé; Telmon, Norbert; Rougé, Daniel; Dedouit, Fabrice

    2014-09-01

    A 27-year-old man suddenly died in hospital of acute respiratory distress syndrome secondary to severe systemic vasculitis. Multi-phase post-mortem computed tomography angiography followed by scientific autopsy of the thoracic and abdominal cavity and histology was performed, illustrating the advantages and drawbacks of such techniques. Imaging enabled us to examine the cranium, as the family refused cerebral dissection. MPMCTA revealed absence of opacification of the left middle cerebral artery. But parenchymal findings of thoracic and abdominal organs were still difficult to interpret after both imaging and macroscopic examination during the autopsy. Microscopic examination provided the definitive diagnosis of cause of death. Analysis revealed systemic vasculitis of the lung complicated by diffuse alveolar, mediastinal, splenic and retroperitoneal lesions. We were unable to determine the type of vasculitis, whether polyarteritis nodosa or microscopic polyangiitis, because of artifactual glomerular collapse. We observed some structural changes in tissue secondary to contrast agent injection, affecting the vascular system and renal parenchyma in particular. Such artifacts must be known in order to avoid misinterpreting them as pathological findings. MPMCTA and conventional autopsy are two complementary techniques showing both their specific advantages and limits which have to be known in order to choose the appropriate technique. One limit of both techniques is the detection of microscopic findings which can only be obtained by additional histological examination. This case report underlines this fact and demonstrates that caution is required in some cases if microscopic analyses are carried out after contrast agent injection. PMID:25085763

  18. Propylthiouracil-Induced Vasculitis With Alveolar Hemorrhage Confirmed by Clinical, Laboratory, Computed Tomography, and Bronchoscopy Findings: A Case Report and Literature Review

    PubMed Central

    Chen, Bo; Yang, Xiaoqing; Sun, Shihai; Guo, Weina; Li, Xiaosheng; Zhang, Lei; Guo, Zhongliang; Han, Jie; Li, Ning

    2016-01-01

    Introduction Propylthiouracil (PTU) is commonly used to treat hyperthyroidism and can induce antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Although this is a rare side effect, ANCA-associated vasculitis can progress to severe disease if its diagnosis and treatment are delayed, leading to a poor prognosis. Case Presentation A 43-year-old woman with Graves’ disease developed pulmonary vasculitis and diffuse alveolar hemorrhage (DAH) associated with ANCA against myeloperoxidase and proteinase-3 that was confirmed by computed tomography (CT) and bronchoscopy and treated with PTU. The symptoms and signs of alveolar hemorrhage were rapidly resolved after PTU withdrawal and treatment with corticosteroids. After 6 months of follow-up, the patient maintained complete ANCA-negative clinical remission status, as confirmed by normal CT and bronchoscopy findings. To our knowledge, this is the first documented case of bronchoscopic comparison of PTU-induced DAH before and after steroid treatment. Conclusions Patients treated with PTU should be closely monitored and followed up, even if the drug has been used for several years. When patients develop progressive dyspnea with alveolar opacities on chest imaging that cannot be explained otherwise, alveolar hemorrhage should be an important differential diagnosis while investigating the case. Early diagnosis and prompt discontinuation of the PTU treatment are essential for improving patient outcomes. PMID:27257510

  19. Levamisole-induced leukocytoclastic vasculitis and neutropenia in a patient with cocaine use: An extensive case with necrosis of skin, soft tissue, and cartilage

    PubMed Central

    2012-01-01

    Levamisole-induced vasculitis is a relatively new entity in people who use cocaine. We describe a 44-year-old woman with a history of cocaine use who presented with a complaint of a painful rash of 2-3 month’s duration on her extremities, cheeks, nose, and earlobes. She had not experienced fever, weight loss, alopecia, dry eyes, oral ulcers, photosensitivity, or arthralgia. Examination revealed tender purpuric eruptions with central necrosis on her nose, cheeks, earlobes, and extremities. Laboratory investigations revealed neutropenia, an elevated erythrocyte sedimentation rate (ESR), presence of lupus anticoagulant, low complement component 3 (C3), and presence of perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA). A urine toxicology screen was positive for cocaine, and gas chromatography–mass spectrometry was positive for levamisole. Skin biopsy showed leukocytoclastic vasculitis and small vessel thrombosis. Necrotic lesions of the nose led to its self-amputation. Large bullae on the lower extremities ruptured, leading to wound infection and extensive necrosis that required multiple surgical debridements. When necrosis progressed despite debridement, bilateral above-knee amputation of the legs was performed. Once new lesions stopped appearing, the patient was discharged home. Two months later, she had a recurrence related to cocaine use. To the best of our knowledge, this is only the second reported case of levamisole-induced vasculitis that required above-knee amputation. PMID:23186390

  20. Clinical presentation and outcome prediction of clinical, serological, and histopathological classification schemes in ANCA-associated vasculitis with renal involvement.

    PubMed

    Córdova-Sánchez, Bertha M; Mejía-Vilet, Juan M; Morales-Buenrostro, Luis E; Loyola-Rodríguez, Georgina; Uribe-Uribe, Norma O; Correa-Rotter, Ricardo

    2016-07-01

    Several classification schemes have been developed for anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), with actual debate focusing on their clinical and prognostic performance. Sixty-two patients with renal biopsy-proven AAV from a single center in Mexico City diagnosed between 2004 and 2013 were analyzed and classified under clinical (granulomatosis with polyangiitis [GPA], microscopic polyangiitis [MPA], renal limited vasculitis [RLV]), serological (proteinase 3 anti-neutrophil cytoplasmic antibodies [PR3-ANCA], myeloperoxidase anti-neutrophil cytoplasmic antibodies [MPO-ANCA], ANCA negative), and histopathological (focal, crescenteric, mixed-type, sclerosing) categories. Clinical presentation parameters were compared at baseline between classification groups, and the predictive value of different classification categories for disease and renal remission, relapse, renal, and patient survival was analyzed. Serological classification predicted relapse rate (PR3-ANCA hazard ratio for relapse 2.93, 1.20-7.17, p = 0.019). There were no differences in disease or renal remission, renal, or patient survival between clinical and serological categories. Histopathological classification predicted response to therapy, with a poorer renal remission rate for sclerosing group and those with less than 25 % normal glomeruli; in addition, it adequately delimited 24-month glomerular filtration rate (eGFR) evolution, but it did not predict renal nor patient survival. On multivariate models, renal replacement therapy (RRT) requirement (HR 8.07, CI 1.75-37.4, p = 0.008) and proteinuria (HR 1.49, CI 1.03-2.14, p = 0.034) at presentation predicted renal survival, while age (HR 1.10, CI 1.01-1.21, p = 0.041) and infective events during the induction phase (HR 4.72, 1.01-22.1, p = 0.049) negatively influenced patient survival. At present, ANCA-based serological classification may predict AAV relapses, but neither clinical nor serological

  1. ANCA-Associated Systemic Vasculitis Presenting With Hypertrophic Spinal Pachymeningitis: A Report of 2 Cases and Review of Literature.

    PubMed

    Li, Xia; Zhao, Jiuliang; Wang, Qian; Fei, Yunyun; Zhao, Yan

    2015-11-01

    Reports of hypertrophic pachymeningitis associated with myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) localized exclusively in the spine were quite rare. Two cases of ANCA-associated systemic vasculitis (AASV) presenting with hypertrophic spinal pachymeningitis (HSP) causing low back pain and numbness are described. Two patients showed prominent systemic and local inflammatory reactions manifested as fever, elevated levels of erythrocyte sedimentation rate and C-reactive protein, and markedly increased levels of total protein of cerebrospinal fluid. The gadolinium (Gd)-enhanced T1-weighted magnetic resonance imaging scan of spinal cord demonstrated diffuse spinal dura matter thickening. Additionally, simple microscopic hematuria was found in 1 case suggestive of renal involvement and the other 1 complicated with interstitial lung disease. Then, a diagnosis of HSP secondary to AASV was made. Combination therapy of corticosteroids and cyclophosphamide produced a rapid improvement in the clinical symptoms and laboratory parameters. Followed up for 6 months, 1 case relapsed when the dosage of prednisone was tapered to 10 mg daily. Since the patient refused rituximab-based regimen, an immunosuppressive triple-therapy (corticosteroid, cyclophosphamide, and azathioprine) was initiated and brought control of the disease during the subsequent 6 months of follow-up.HSP is a relatively rare form of central nervous system involvement of AASV. Early recognition and intervention are of great significance since the pathogenesis of HSP starts with an inflammatory and fibrosing process. PMID:26579814

  2. ANCA-positive vasculitis induced by levamisole-adulterated cocaine and nephrotic syndrome: The kidney as an unusual target

    PubMed Central

    Álvarez Díaz, Hortensia; Marińo Callejo, Ana Isabel; García Rodríguez, José Francisco; Rodríguez Pazos, Laura; Gómez Buela, Inmaculada; Bermejo Barrera, Ana María

    2013-01-01

    Patient: Male, 36 Final Diagnosis: Levamisole-induced vasculopathy Symptoms: Purpuric skin lesions Medication: Levamisole Clinical Procedure: — Specialty: Internal Medicine Objective: Unusual clinical course Background: Levamisole has been detected in seized cocaine samples and a levamisole-induced vasculopathy (LIV) has been described, mainly focused on skin. Case Report: A 36-year-old Caucasian man with history of antibodies to hepatitis C infection (negative hepatitis C virus RNA and negative HIV serology), smoking, and intravenous use of cocaine and brown heroin, presented to the hospital with purpuric skin lesions on extremities and earlobes. One month before the current presentation, a skin punch biopsy of one of these lesions was performed, showing histopathologic findings suggestive of mixed cryoglobulinemia. Laboratory testing revealed leukopenia, renal failure, and nephrotic syndrome. Antimyeloperoxidase antineutrophil cytoplasmic antibodies (MPO-ANCA) were positive. The previous skin punch biopsy was revised and demonstrated pathologic findings consistent with leukocytoclastic vasculitis. An analysis of a cocaine sample for personal use, provided by the patient, was performed using mass spectrometry-gas chromatography and levamisole was detected. Three boluses of intravenous methylprednisolone were administered, followed by oral prednisone 1 mg/Kg per day. Skin lesions and renal function improved. Conclusions: To our knowledge, this is the first report of nephrotic syndrome induced by levamisole-adulterated cocaine, proven by cocaine sample toxicology. Lack of renal biopsy is a limitation of this report. PMID:24478818

  3. A case report of Epstein–Barr virus-associated retinal vasculitis: successful treatment using only acyclovir therapy

    PubMed Central

    Keorochana, Narumon

    2016-01-01

    The purpose of this study was to describe a presumed case of Epstein–Barr virus (EBV)-associated retinal vasculitis in a 42-year-old female with sudden unilateral vision loss and successful treatment with acyclovir therapy. Diagnostic vitreous biopsy of the right eye was performed to test for EBV and other known infectious causes of retinitis and evaluate vitreous cells and serological testing. Vitreous polymerase chain reaction viral DNA testing result was positive for EBV but negative for herpes simplex virus, varicella-zoster virus, and cytomegalovirus. Serologic testing was negative for toxoplasma gondii, syphilis, tuberculosis, and HIV. Histopathologic analysis of vitreous cells revealed atypical lymphocytes. Fluorescein angiography showed disk leakage, occluded retinal artery, peripheral vascular leakage, and ischemic area of the right eye. Intravenous acyclovir, 10 mg/kg/d, was prescribed for 14 days followed by oral acyclovir for 3 months. All lesions have become quiet. EBV may be a cause of retinal disease, and intravenous acyclovir is a successful treatment choice. PMID:27524923

  4. Refractory polymyalgia rheumatica as presenting manifestation of large-vessel vasculitis associated to sarcoidosis. Successful response to adalimumab.

    PubMed

    Bejerano, Carmen; Blanco, Ricardo; González-Vela, Carmen; Agüero, Ramón; Carril, José M; González-Gay, Miguel A

    2012-01-01

    Sarcoidosis may present with musculoskeletal features or mimic rheumatic diseases. We report on a patient who had been initially diagnosed as having polymyalgia rheumatica. Because of refractory disease associated to atypical features such as severe inflammatory low back pain, dull and achy pain in the thighs, claudication of the lower limbs and bad response to corticosteroids and methotrexate (MTX), an 18F-fluorodeoxyglucosepositron emission tomography with CT (FDG PET/CT) was performed. This technique disclosed data suggestive of arteritis of large vessels involving the ascending, arch and descending aorta as well as high FDG uptake in the femoral and posterior tibial arteries of both lower extremities. Also, increased FDG uptake was observed in the right paratracheal, retrotracheal, subcarinal, gastrohepatic ligament, coeliac and right renal hilar lymph nodes. Four lymph nodes, taken during mediastinoscopy, confirmed a diagnosis of sarcoidosis. Treatment with adalimumab (40 mg every 2 weeks subcutaneously) along with prednisone and MTX was initiated yielding progressive improvement of symptoms and normalisation of laboratory abnormalities. Five months after the onset of adalimumab a new FDG PET/CT showed complete absence of uptake of lymph nodes as well as decrease of vascular FDG uptake. To our knowledge, this is the first patient treated with adalimumab because of a large-vessel vasculitis in the setting of sarcoidosis refractory to conventional therapy. This case reinforces the claim that sarcoidosis should be considered a diagnostic challenge in the assessment of patients presenting with inflammatory musculoskeletal symptoms. PMID:22410180

  5. A case report of Epstein-Barr virus-associated retinal vasculitis: successful treatment using only acyclovir therapy.

    PubMed

    Keorochana, Narumon

    2016-01-01

    The purpose of this study was to describe a presumed case of Epstein-Barr virus (EBV)-associated retinal vasculitis in a 42-year-old female with sudden unilateral vision loss and successful treatment with acyclovir therapy. Diagnostic vitreous biopsy of the right eye was performed to test for EBV and other known infectious causes of retinitis and evaluate vitreous cells and serological testing. Vitreous polymerase chain reaction viral DNA testing result was positive for EBV but negative for herpes simplex virus, varicella-zoster virus, and cytomegalovirus. Serologic testing was negative for toxoplasma gondii, syphilis, tuberculosis, and HIV. Histopathologic analysis of vitreous cells revealed atypical lymphocytes. Fluorescein angiography showed disk leakage, occluded retinal artery, peripheral vascular leakage, and ischemic area of the right eye. Intravenous acyclovir, 10 mg/kg/d, was prescribed for 14 days followed by oral acyclovir for 3 months. All lesions have become quiet. EBV may be a cause of retinal disease, and intravenous acyclovir is a successful treatment choice. PMID:27524923

  6. Decreased Numbers of Blood Dendritic Cells and Defective Function of Regulatory T Cells in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis

    PubMed Central

    Rimbert, Marie; Hamidou, Mohamed; Braudeau, Cécile; Puéchal, Xavier; Teixeira, Luis; Caillon, Hélène; Néel, Antoine; Audrain, Marie; Guillevin, Loic; Josien, Régis

    2011-01-01

    Background Dendritic cells (DC) and regulatory cells (Treg) play pivotal roles in controlling both normal and autoimmune adaptive immune responses. DC are the main antigen-presenting cells to T cells, and they also control Treg functions. In this study, we examined the frequency and phenotype of DC subsets, and the frequency and function of Treg from patients with ANCA-associated vasculitis (AAV). Methodology/Principal Findings Blood samples from 19 untreated patients with AAV during flares and before any immunosuppressive treatment were analyzed, along with 15 AAV patients in remission and 18 age-matched healthy controls. DC and Treg numbers, and phenotypes were assessed by flow cytometry, and in vitro suppressive function of Treg was determined by co-culture assay. When compared to healthy volunteers, absolute numbers of conventional and plasmacytoid DC were decreased in AAV patients. During the acute phase this decrease was significantly more pronounced and was associated with an increased DC expression of CD62L. Absolute numbers of Treg (CD4+CD25highCD127low/− Tcells) were moderately decreased in patients. FOXP3 and CD39 were expressed at similar levels on Treg from patients as compared to controls. The suppressive function of Treg from AAV patients was dramatically decreased as compared to controls, and this defect was more pronounced during flares than remission. This Treg functional deficiency occurred in the absence of obvious Th17 deviation. Conclusion In conclusion, these data show that AAV flares are associated with both a decrease number and altered phenotype of circulating DC and point to a role for Treg functional deficiency in the pathogenesis of AAV. PMID:21494636

  7. Systemic Lupus Erythematosus and Antineutrophil Cytoplasmic Antibody-Associated Vasculitis Overlap Syndrome in Patients With Biopsy-Proven Glomerulonephritis

    PubMed Central

    Jarrot, Pierre-Andre; Chiche, Laurent; Hervier, Baptiste; Daniel, Laurent; Vuiblet, Vincent; Bardin, Nathalie; Bertin, Daniel; Terrier, Benjamin; Amoura, Zahir; Andrés, Emmanuel; Rondeau, Eric; Hamidou, Mohamed; Pennaforte, Jean-Loup; Halfon, Philippe; Daugas, Eric; Dussol, Bertrand; Puéchal, Xavier; Kaplanski, Gilles; Jourde-Chiche, Noemie

    2016-01-01

    Abstract The aim of the study was to report the clinical, biological, and pathological characteristics of patients with glomerulonephritis (GN) secondary to systemic lupus erythematosus (SLE)/antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) overlap syndrome. A nationwide survey was conducted to identify cases of SLE/AAV overlap syndrome. Data were collected from SLE and AAV French research groups. Inclusion criteria were diagnosis of both SLE and AAV according to international classification criteria and biopsy-proven GN between 1995 and 2014. Additional cases were identified through a systematic literature review. A cohort of consecutive biopsy-proven GN was used to study the prevalence of overlapping antibodies and/or overlap syndrome. The national survey identified 8 cases of SLE/AAV overlap syndrome. All patients were female; median age was 40 years. AAV occurred before SLE (n = 3), after (n = 3), or concomitantly (n = 2). Six patients had rapidly progressive GN and 3/8 had alveolar hemorrhage. All patients had antinuclear antibodies (ANA); 7/8 had p-ANCA antimyeloperoxidase (MPO) antibodies. Renal biopsies showed lupus nephritis (LN) or pauci-immune GN. Remission was obtained in 4/8 patients. A literature review identified 31 additional cases with a similarly severe presentation. In the GN cohort, ANCA positivity was found in 30% of LN, ANA positivity in 52% of pauci-immune GN, with no correlation with pathological findings. The estimated prevalence for SLE/AAV overlap syndrome was 2/101 (2%). In patients with GN, SLE/AAV overlap syndrome may occur but with a low prevalence. Most patients have an aggressive renal presentation, with usually both ANA and anti-MPO antibodies. Further studies are needed to assess shared pathogenesis and therapeutic options. PMID:27258503

  8. Systemic Lupus Erythematosus and Antineutrophil Cytoplasmic Antibody-Associated Vasculitis Overlap Syndrome in Patients With Biopsy-Proven Glomerulonephritis.

    PubMed

    Jarrot, Pierre-Andre; Chiche, Laurent; Hervier, Baptiste; Daniel, Laurent; Vuiblet, Vincent; Bardin, Nathalie; Bertin, Daniel; Terrier, Benjamin; Amoura, Zahir; Andrés, Emmanuel; Rondeau, Eric; Hamidou, Mohamed; Pennaforte, Jean-Loup; Halfon, Philippe; Daugas, Eric; Dussol, Bertrand; Puéchal, Xavier; Kaplanski, Gilles; Jourde-Chiche, Noemie

    2016-05-01

    The aim of the study was to report the clinical, biological, and pathological characteristics of patients with glomerulonephritis (GN) secondary to systemic lupus erythematosus (SLE)/antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) overlap syndrome.A nationwide survey was conducted to identify cases of SLE/AAV overlap syndrome. Data were collected from SLE and AAV French research groups. Inclusion criteria were diagnosis of both SLE and AAV according to international classification criteria and biopsy-proven GN between 1995 and 2014. Additional cases were identified through a systematic literature review. A cohort of consecutive biopsy-proven GN was used to study the prevalence of overlapping antibodies and/or overlap syndrome.The national survey identified 8 cases of SLE/AAV overlap syndrome. All patients were female; median age was 40 years. AAV occurred before SLE (n = 3), after (n = 3), or concomitantly (n = 2). Six patients had rapidly progressive GN and 3/8 had alveolar hemorrhage. All patients had antinuclear antibodies (ANA); 7/8 had p-ANCA antimyeloperoxidase (MPO) antibodies. Renal biopsies showed lupus nephritis (LN) or pauci-immune GN. Remission was obtained in 4/8 patients. A literature review identified 31 additional cases with a similarly severe presentation. In the GN cohort, ANCA positivity was found in 30% of LN, ANA positivity in 52% of pauci-immune GN, with no correlation with pathological findings. The estimated prevalence for SLE/AAV overlap syndrome was 2/101 (2%).In patients with GN, SLE/AAV overlap syndrome may occur but with a low prevalence. Most patients have an aggressive renal presentation, with usually both ANA and anti-MPO antibodies. Further studies are needed to assess shared pathogenesis and therapeutic options. PMID:27258503

  9. Mapping of the Outcome Measures in Rheumatology Core Set for Antineutrophil Cytoplasmic Antibody–Associated Vasculitis to the International Classification of Function, Disability and Health

    PubMed Central

    MILMAN, NATALIYA; BOONEN, ANNELIES; MERKEL, PETER A.; TUGWELL, PETER

    2015-01-01

    Objective The International Classification of Functioning, Disability and Health (ICF) is a framework and classification of health that describes health along 4 components: body functions, body structures, activities and participation, and contextual factors. This study examined the content of instruments that constitute the Outcome Measures in Rheumatology (OMERACT) core set of outcome measures for antineutrophil cytoplasmic antibody–associated vasculitis (AAV) by “mapping” them to the ICF. Methods The content of the instruments included in the AAV core set were linked to the ICF by 2 independent investigators according to previously established ICF linkage rules. Results The AAV core set includes 3 measures of disease activity (3 versions of the Birmingham Vasculitis Activity Score), 1 damage measure (Vasculitis Damage Index), 1 patient-reported outcome (Short Form 36 health survey), and death. Linking these instruments to the ICF revealed comprehensive coverage of the ICF components body functions and body structures, limited coverage of the ICF component activities and participation, and complete absence of coverage of contextual factors. Conclusion ICF was found to be useful for thematic characterization of a heterogeneous group of outcome measures for AAV, i.e., a group of complex medical conditions. Linking of the instruments selected for the OMERACT AAV core set of outcome measures to the ICF classification revealed limitations in the representation of constructs related to life impact of AAV, represented by the ICF components activities and participation and contextual factors. Further research and methods development are needed to better incorporate important aspects of functioning and health relevant to patients into clinical trials of AAV. PMID:25048363

  10. PUPPP (Pruritic Urticarial Papules Plaques of Pregnancy)

    MedlinePlus

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  11. Clozapine-Induced Late Agranulocytosis and Severe Neutropenia Complicated with Streptococcus pneumonia, Venous Thromboembolism, and Allergic Vasculitis in Treatment-Resistant Female Psychosis

    PubMed Central

    Voulgari, Christina; Giannas, Raphael; Paterakis, Georgios; Kanellou, Anna; Anagnostopoulos, Nikolaos; Pagoni, Stamata

    2015-01-01

    Clozapine is a second-generation antipsychotic agent from the benzodiazepine group indicated for treatment-resistant schizophrenia and other psychotic conditions. Using clozapine earlier on once a case appears to be refractory limits both social and personal morbidity of chronic psychosis. However treatment with second-generation antipsychotics is often complicated by adverse effects. We present a case of a 33-year-old Caucasian woman with a 25-year history of refractory psychotic mania after switching to a 2-year clozapine therapy. She presented clozapine-induced absolute neutropenia, agranulocytosis, which were complicated by Streptococcus pneumonia and sepsis. Clozapine-induced thromboembolism of the common femoral and right proximal iliac vein, as well as allergic vasculitis, was diagnosed. She achieved full remission on granulocyte-colony stimulating factor and specific antibiotic treatment. Early detection of severe clozapine-induced absolute neutropenia and agranulocytosis enabled the effective treatment of two among its most severe complications. Additional evidence to the previously reported possible causal relation between clozapine and venous thromboembolism is offered. Finally, clozapine-induced allergic vasculitis is confirmed as a late adverse effect of clozapine therapy. PMID:25755670

  12. A rare association of localized scleroderma type morphea, vitiligo, autoimmune hypothyroidism, pneumonitis, autoimmune thrombocytopenic purpura and central nervous system vasculitis. Case report

    PubMed Central

    2012-01-01

    Background The localized scleroderma (LS) known as morphea, presents a variety of clinical manifestations that can include systemic involvement. Current classification schemes divide morphea into categories based solely on cutaneous morphology, without reference to systemic disease or autoimmune phenomena. This classification is likely incomplete. Autoimmune phenomena such as vitiligo and Hashimoto thyroiditis associated with LS have been reported in some cases suggesting an autoimmune basis. To our knowledge this is the first case of a morphea forming part of a multiple autoimmune syndrome (MAS) and presenting simultaneously with autoimmune thrombocytopenic purpura and central nervous system vasculitis. Case presentation We report an uncommon case of a white 53 year old female patient with LS as part of a multiple autoimmune syndrome associated with pneumonitis, autoimmune thrombocytopenic purpura and central nervous system vasculitis presenting a favorable response with thrombopoietin receptor agonists, pulses of methylprednisolone and cyclophosphamide. Conclusion Is likely that LS have an autoimmune origin and in this case becomes part of MAS, which consist on the presence of three or more well-defined autoimmune diseases in a single patient. PMID:23256875

  13. T lymphocyte responses to anti-neutrophil cytoplasmic autoantibody (ANCA) antigens are present in patients with ANCA-associated systemic vasculitis and persist during disease remission

    PubMed Central

    King, W J; Brooks, C J; Holder, R; Hughes, P; Adu, D; Savage, C O S

    1998-01-01

    ANCA with specificity for myeloperoxidase (MPO) and proteinase 3 (PR3) are present in patients with systemic vasculitis. The aim of this work was to determine whether such patients have T cell responses to these antigens and whether these responses are related to disease activity. Peripheral blood lymphocytes from 45 patients and 19 controls were cultured with ANCA antigens and proliferation measured. The antigens used were heat-inactivated (HI) MPO, HI PR3, native (non-HI) PR3, HI whole α-granules, and 25 overlapping peptides covering the entire PR3 sequence. Significant responses to both whole PR3 preparations were seen from patient and control groups, and to the α-granules from the patient group. Patients responded at all stages of disease: active, remitting, treated or untreated. Only two patients responded significantly to MPO. Responses were significantly higher with the patient group than the control group to all four whole ANCA antigens. Responses to those PR3 peptides containing epitopes known to be recognized by ANCA were detected from one patient. Thus, these studies demonstrate that T cells from vasculitis patients can proliferate to PR3 and occasionally to associated ANCA antigens. Further, responses may persist even after disease remission has been achieved. PMID:9649227

  14. Hepatic and Mesenteric Vasculitis as Presenting Manifestation of Mixed Cryoglobulinemia Related to Chronic Hepatitis C Virus Infection in a Female Patient.

    PubMed

    Calle Toro, Juan S; Davalos, Diana M; Charry, Jose D; Arrunategi, Ana M; Tobon, Gabriel

    2016-06-01

    Approximately 80% of patients with hepatitis C virus infection develop chronic liver disease as cirrhosis, and 40% develop autoimmune complications as mixed cryoglobulinemia (MC). Gastrointestinal involvement in MC is rare, and even more so is hepatic involvement. We report a case of an 87-year-old woman with a 10-year history of blood transfusion-acquired hepatitis C virus infection, without treatment. She consulted the emergency department for diffuse abdominal pain, associated with vomiting. After 2 weeks of hospitalization in the intensive care unit, a diagnosis of MC was made; cirrhosis and secondary mesenteric and hepatic vasculitis were confirmed by a diagnostic laparoscopy. Unfortunately the condition of the patient worsened with sepsis and resulted in death in the fourth week from admission. This case highlights the importance of having in mind gastrointestinal tract vasculitis as a medical cause of abdominal pain in patients with chronic hepatitis C virus infection and using data laboratory tests, images, and histopathologic studies to aid with the diagnosis. PMID:27219310

  15. Low Serum Complement C3 Levels at Diagnosis of Renal ANCA-Associated Vasculitis Is Associated with Poor Prognosis

    PubMed Central

    Augusto, Jean-François; Langs, Virginie; Demiselle, Julien; Lavigne, Christian; Brilland, Benoit; Duveau, Agnès; Poli, Caroline; Chevailler, Alain; Croue, Anne; Tollis, Frederic; Sayegh, Johnny; Subra, Jean-François

    2016-01-01

    Background Recent studies have demonstrated the key role of the complement alternative pathway (cAP) in the pathophysiology of experimental ANCA-associated vasculitis (AAV). However, in human AAV the role of cAP has not been extensively explored. In the present work, we analysed circulating serum C3 levels measured at AAV onset and their relation to outcomes. Methods We conducted a retrospective observational cohort study including 45 consecutive patients with AAV diagnosed between 2000 and 2014 with serum C3 measurement at diagnosis, before immunosuppressive treatment initiation. Two groups were defined according to the median serum C3 level value: the low C3 group (C3<120 mg/dL) and the high C3 level group (C3≥120 mg/dL). Patient and renal survivals, association between C3 level and renal pathology were analysed. Results Serum complement C3 concentration remained in the normal range [78–184 mg/dL]. Compared with the high C3 level, the patients in the low C3 level group had lower complement C4 concentrations (P = 0.008) and lower eGFR (P = 0.002) at diagnosis. The low C3 level group had poorer patient and death-censored renal survivals, compared with the high C3 level group (P = 0.047 and P = 0.001, respectively). We observed a significant negative correlation between C3 levels and the percentage of glomeruli affected by cellular crescent (P = 0.017, r = -0.407). According to the Berden et al renal histologic classification, patients in the crescentic/mixed category had low C3 levels more frequently (P<0.01). Interestingly, we observed that when patients with the crescentic/mixed histologic form were analysed according to C3 level, long term renal survival was significantly greater in the high C3 level group than in the low C3 level group (100% vs 40.7% at 6 years, p = 0.046). No relationship between serum C4 and renal outcome was observed. Conclusion A Low C3 serum level in AAV patients at diagnosis is associated with worse long-term patient and renal

  16. CD8+/perforin+/WC1- gammadelta T cells, CD8+ alphabeta T cells, infiltrate vasculitis lesions of American bison (Bison bison) with experimental sheep-associated malignant catarrhal fever

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Sheep-associated malignant catarrhal fever (SA-MCF) caused by ovine herpesvirus-2 (OvHV-2) is a fatal disease associated with lymphoproliferation, lymphocytic vasculitis, and mucosal ulceration in clinically susceptible species. SA-MCF is an important threat to American bison (Bison bison) due to th...

  17. Central Nervous System Vasculitis

    MedlinePlus

    ... you think. More serious than you know. Support. Awareness. Education. Become a member today! Our Mission The ... Foundation supports and empowers our community through education, awareness and research. Contact Us Click here to send ...

  18. Types of Vasculitis

    MedlinePlus

    ... Digg. Share this page from the NHLBI on Facebook. Add this link to the NHLBI to my ... This Content: NEXT >> Updated: September 23, 2014 Twitter Facebook YouTube Google+ SITE INDEX ACCESSIBILITY PRIVACY STATEMENT FOIA ...

  19. Acute and chronic urticaria. Challenges and considerations for primary care physicians.

    PubMed

    Krishnaswamy, G; Youngberg, G

    2001-02-01

    Urticaria and angioedema are common dermatologic problems seen by primary care physicians. A carefully taken history, physical examination, specific tests, and skin biopsy often provide useful diagnostic information. In patients with chronic urticaria, urticarial vasculitis and diseases that mimic urticaria need to be ruled out. A variety of treatment options are available for patients with urticaria and urticarial vasculitis. Pharmacologic therapy is useful when the specific cause is undetermined. When a trigger has been identified, the patient must avoid exposure to it. Patient education is an important component of management and should include instructions on crisis management, particularly for patients who have angioedema or a tendency for anaphylaxis. PMID:11272687

  20. Observational Study of the Genetic Architecture of Neutrophil-Mediated Inflammatory Skin Diseases

    ClinicalTrials.gov

    2014-06-11

    Other Specified Inflammatory Disorders of Skin or Subcutaneous Tissue; Pyoderma Gangrenosum; Erosive Pustular Dermatosis of the Scalp; Sweet's Syndrome; Behcet's Disease; Bowel-associated Dermatosis-arthritis Syndrome; Pustular Psoriasis; Acute Generalized Exanthematous Pustulosis; Keratoderma Blenorrhagicum; Sneddon-Wilkinson Disease; IgA Pemphigus; Amicrobial Pustulosis of the Folds; Infantile Acropustulosis; Transient Neonatal Pustulosis; Neutrophilic Eccrine Hidradenitis; Rheumatoid Neutrophilic Dermatitis; Neutrophilic Urticaria; Still's Disease; Erythema Marginatum; Unclassified Periodic Fever Syndromes / Autoinflammatory Syndromes; Dermatitis Herpetiformis; Linear IgA Bullous Dermatosis; Bullous Systemic Lupus Erythematosus; Inflammatory Epidermolysis Bullosa Aquisita; Neutrophilic Dermatosis of the Dorsal Hands (Pustular Vasculitis); Small Vessel Vasculitis Including Urticarial Vasculitis; Erythema Elevatum Diutinum; Medium Vessel Vasculitis

  1. Bronchial lesions of mouse model of asthma are preceded by immune complex vasculitis and induced bronchial associated lymphoid tissue (iBALT).

    PubMed

    Guest, Ian C; Sell, Stewart

    2015-08-01

    We systematically examined by immune histology the lungs of some widely used mouse models of asthma. These models include sensitization by multiple intraperitoneal injections of soluble ovalbumin (OVA) or of OVA with alum, followed by three intranasal or aerosol challenges 3 days apart. Within 24 h after a single challenge there is fibrinoid necrosis of arterial walls with deposition of immunoglobulin (Ig) and OVA and infiltration of eosinophilic polymorphonuclear cells that lasts for about 3 days followed by peribronchial B-cell infiltration and slight reversible goblet cell hypertrophy (GCHT). After two challenges, severe eosinophilic vasculitis is present at 6 h, increases by 72 h, and then declines; B-cell proliferation and significant GCHT and hyperplasia (GCHTH) and bronchial smooth muscle hypertrophy recur more prominently. After three challenges, there is significantly increased induced bronchus-associated lymphoid tissue (iBALT) formation, GCHTH, and smooth muscle hypertrophy. Elevated levels of Th2 cytokines, IL-4, IL-5, and IL-13, are present in bronchial lavage fluids. Sensitized mice have precipitating antibody and positive Arthus skin reactions but also develop significant levels of IgE antibody to OVA but only 1 week after challenge. We conclude that the asthma like lung lesions induced in these models is preceded by immune complex-mediated eosinophilic vasculitis and iBALT formation. There are elevations of Th2 cytokines that most likely produce bronchial lesions that resemble human asthma. However, it is unlikely that mast cell-activated atopic mechanisms are responsible as we found only a few presumed mast cells by toluidine blue and metachromatic staining limited to the most proximal part of the main stem bronchus, and none in the remaining main stem bronchus or in the lung periphery. PMID:26006019

  2. Non HCV-related infectious cryoglobulinemia vasculitis: Results from the French nationwide CryoVas survey and systematic review of the literature.

    PubMed

    Terrier, Benjamin; Marie, Isabelle; Lacraz, Adeline; Belenotti, Pauline; Bonnet, Fabrice; Chiche, Laurent; Graffin, Bruno; Hot, Arnaud; Kahn, Jean-Emmanuel; Michel, Catherine; Quemeneur, Thomas; de Saint-Martin, Luc; Hermine, Olivier; Léger, Jean-Marc; Mariette, Xavier; Senet, Patricia; Plaisier, Emmanuelle; Cacoub, Patrice

    2015-12-01

    In patients with infectious cryoglobulinemia vasculitis (CryoVas) in the absence of hepatitis C virus infection, data on presentation, therapeutic management and outcome are lacking. We conducted a nationwide survey that included patients with HCV-negative CryoVas. We describe here the presentation, therapeutic management and outcome of 18 patients with non-HCV infectious CryoVas and 27 additional patients identified form a systematic review of the literature. We included 18 patients, mean age 57.9±13.5 years. Infectious causes were viral infections in 8 patients [hepatitis B virus (HBV) in 4, and cytomegalovirus, Epstein Barr virus, parvovirus B19 and human immunodeficiency virus in one case each], pyogenic bacterial infection in 6 patients, parasitic infection in 2 patients, and leprosy and candidiasis in one case each. Baseline manifestations were purpura (78%), glomerulonephritis (28%), arthralgia (28%), peripheral neuropathy (22%), skin necrosis (22%), cutaneous ulcers (17%), and myalgia (11%). Cryoglobulinemia was type II in 2/3 of cases. Most cases received specific anti-infectious therapy as first-line therapy, sometimes associated with corticosteroids, achieving sustained remission in the majority of cases. Refractory or relapsing patients, frequently related to HBV infection, showed a complete remission after rituximab in addition to antiviral therapy. In contrast, corticosteroids and/or immunosuppressive agents used in the absence of anti-infectious agents were frequently associated with refractory CryoVas. Viral and pyogenic bacterial infections represent the main causes of non-HCV infectious CryoVas. Antimicrobial therapy is commonly associated with sustained remission. Immunosuppressive agents should be considered only as a second-line option in patients with refractory vasculitis. PMID:26320984

  3. Local and systemic activation of the whole complement cascade in human leukocytoclastic cutaneous vasculitis; C3d,g and terminal complement complex as sensitive markers.

    PubMed Central

    Dauchel, H; Joly, P; Delpech, A; Thomine, E; Sauger, F; Le Loet, X; Lauret, P; Tron, F; Fontaine, M; Ripoche, J

    1993-01-01

    We have studied complement activation both in plasma samples and in lesional skin from patients with leukocytoclastic cutaneous vasculitis (LCV). Enzyme immunoassay (EIA) quantification of the complement activation markers, C3d,g and the terminal complement complex (TCC) in plasma, showed that their levels were significantly increased in 66% and 55% of the patients, respectively (n = 29) compared with healthy controls, whereas the standard measurements of C3, factor B, C1q, C4 and C2 were generally within normal range. Elevations of C3d,g and TCC levels in plasma were significantly correlated. Importantly, a significant correlation was found between the severity of the vasculitis and both C3d,g and TCC plasma levels. Immunofluorescence studies of skin biopsy specimens demonstrated simultaneous presence of perivascular dermal deposits of C3d,g and TCC in lesional skin from 96% and 80% respectively of the patients (n = 25). There was a significant correlation between the intensity of the deposits of both markers. Clusterin, a TCC inhibitory protein, was always found at the same sites of perivascular TCC deposits. Immunofluorescence studies at the epidermal basement membrane zone (BMZ) revealed in each case deposits of C3d,g which were accompanied by TCC deposits in 52% of the biopsy specimens. These data demonstrate that there is a local and systemic activation of the whole complement cascade in human LCV. The presence of both C3d,g and clusterin-associated TCC perivascular deposits suggests an intervention of a regulatory mechanism of local complement activation in LCV. Finally, measurement of plasma C3d,g and TCC appears to be a sensitive indicator of systemic complement activation and disease severity in LCV. Images Fig. 1 Fig. 2 PMID:8485913

  4. Hepatitis C virus (HCV) in cryoglobulinaemic leukocytoclastic vasculitis (LCV): could the presence of HCV in skin lesions be related to T CD8+ lymphocytes, HLA-DR and ICAM-1 expression?

    PubMed

    Bernacchi, E; Civita, L L; Caproni, M; Zignego, A L; Bianchi, B; Monti, M; Fabbri, P; Pasero, G; Ferri, C

    1999-12-01

    An association between mixed cryoglobulinaemia (MC) and hepatotropic viruses, chiefly hepatitis C virus (HCV), has been widely reported. The presence of HCV genomic sequences or HCV-related viral proteins in the serum, purified cryoglobulins, peripheral blood mononuclear cells and into several tissues has suggested an important triggering role for HCV in MC patients. However, only few reports investigated the presence of HCV in cutaneous vasculitis and its potential pathogenetic role. Biopsies of cutaneous purpuric lesions from 5 MC female patients (aged from 40 to 80 years) were carried out for virological and histopathological evaluation. A leukocytoclastic vasculitis pattern was found in 4/5 subjects, while the presence of HCV RNA was detected in 3/5. In only 3 cases biopsy specimens were sufficient for immunohistochemical and direct immunofluorescence (DIF) studies. Immunohistochemical evaluation was performed by means of alkaline phosphatase and monoclonal anti-alkaline phosphatase (APAAP) immune-complexes. In the same skin specimen APAAP and DIF findings were compared with the presence/absence of HCV genomic sequences (PCR technique). In 1 MC patient, the detection of HCV-RNA was associated to a prevalent CD8+ T suppressor pattern with a perivascular and subjunctional distribution as well as an intense expression of second class (HLA-DR) and intercellular adhesion (ICAM-1) molecules on basal keratinocytes, endothelial cells and perivascular infiltrate. These findings suggest a marked inflammatory activation that spreads from endothelial cells to keratinocytes and Langerhans cells. In the 2 HCV-RNA negative specimens the scanty immunopathological staining could indicate a residual activity due to the previous inflammatory event triggered by cryoglobulins. The deposition of circulating HCV-containing immune complexes (CIC) in the skin could be the initial pathogenic event for cryoglobulinemic vasculitis; subsequently CIC could spread from the vascular bed to

  5. Unusual urticarias.

    PubMed

    Black, A K

    2001-11-01

    Typical urticarial lesions are transient cutaneous swellings of sudden onset, often itchy, persisting for less than 24 hours and resolving to leave normal appearing skin. Angioedema lesions are similar subcutaneous lesions. Atypical urticarias persist for longer than 24 hours, may be painful and bruised in appearance and accompanied with severe systemic symptoms. Conditions where prolonged weals are present include delayed pressure urticaria and urticarial vasculitis. These conditions do not respond well to antihistamine therapy. In delayed pressure urticaria, weals appear after a delay of hours at sites of sustained pressure on the skin and occur in association with ordinary chronic 'idiopathic' urticaria. Weals of urticarial vasculitis show histological features of venulitis, and can be accompanied by arthralgia and abdominal pain. Rarely, the condition is due to infective or autoimmune disease. Urticarial diseases, sometimes with features of urticarial vasculitis, and with associated systemic features include Schnitzler's Syndrome, Still's disease and Muckle-Wells syndrome. The latter syndrome is linked with chromosome 1q44, as is autosomal dominant cold urticaria, an unusual physical urticaria. Persistent cholinergic erythema, a variant of cholinergic urticaria, has been mistaken for a drug eruption or cutaneous mastocytosis. Rarely, food and exercise induced urticaria and anaphylaxis occur when exercise follows a specific food or any meal within a few hours. The early stages of inflammatory disease may be mistaken for urticaria and angioedema, but lesions usually persist for longer than 48 hours and are accompanied by epidermal changes. PMID:11770721

  6. Emergency testing for antineutrophil cytoplasmic antibodies combined with a dialog-based policy between clinician and biologist: effectiveness for the diagnosis of ANCA-associated vasculitis.

    PubMed

    Sayegh, Johnny; Poli, Caroline; Chevailler, Alain; Subra, Jean-François; Beloncle, François; Deguigne, Pierre Antoine; Beauvillain, Céline; Augusto, Jean-François

    2015-04-01

    A prompt immunosuppressive treatment initiation is crucial in ANCA-associated vasculitis (AAV) to minimize organ injury. The aim of the present work was to analyze the accuracy of emergency ANCA screening to identify rapidly patients with AAV. In our Institution, emergency ANCA screening is based on a telephone call between a Clinician and a Biologist. Indirect immunofluorescence (IIF) for ANCA detection was performed using a commercial kit (Euroimmun(®) Granulocyte Mosaic 12). Positive serums for c- or p-ANCA at IIF are subsequently screened for antigenic specificity (MPO or PR3) by an immunodot technique (immunodot, D-Tek(®).) Positive samples with atypical c- or p-ANCA pattern at IIF are subsequently screened for antigenic specificity by ELISA. Data were retrieved from patients' medical records and confronted to emergency ANCA screening results. Between 2005 and 2012, 114 patients were screened. IIF was positive in 27.2% of patients, but c-/p-ANCA anti-MPO/-PR3 was detected in 13.2% of patients. The sensibility and specificity of IIF combined with immunodot for newly diagnosed AAV were 83.3 and 100%, respectively. Ten patients were newly diagnosed with AAV. In these patients, a specific AAV treatment was initiated less than 24 h following ANCA screening. Emergency ANCA screening based on a clinical gating policy was relevant to identify patients with AAV diagnosis, and was associated with a rapid treatment initiation. PMID:25343851

  7. Granulomatous slack skin - seven years follow-up of a case with features of "parapsoriasis en plaques", muscle involvement, granulomatous vasculitis, and necrobiotic changes.

    PubMed

    ChiriŢă, Aurel Doru; Mărgăritescu, Irina

    2016-01-01

    Granulomatous slack skin (GSS) represents an extremely rare variant of mycosis fungoides with only 70 cases reported in the literature to date. It is characterized clinically by the occurrence of bulky, pendulous skinfolds, usually located in flexural areas and histologically by an infiltrate composed of small neoplastic T-lymphocytes joined by granulomatous inflammation with scattered multinucleated giant cells containing nuclei arranged in a wreath-like fashion. Since its first description, very rare cases of GSS with muscle involvement, large vessels involvement, or necrobiotic changes have been reported. We present an extraordinary case of GSS with all these unusual features developing in the lesions of the same patient. The long follow-up of seven years allowed us to document the evolution of each lesion. Some lesions appeared and evolved in a manner very reminiscent of those of "parapsoriasis en plaques", others were classical GSS lesions, and still others developed large ulcerated lesions. These ulcerated lesions consistently failed to respond to conventional wound therapy, skin directed therapy [retinoids + psoralen combined with ultraviolet A (PUVA)-therapy], and interferon-alpha therapy. Remarkably, the ulcers completely healed when systemic corticosteroids were added. We hence postulate that the ulcers appeared because of large vessel vasculitis rather than tumoral direct destruction. PMID:27516031

  8. [MPO-ANCA related vasculitis complicating mucinous cystadenoma of the pancreas and severe acute pancreatitis after steroid pulse therapy: a case report].

    PubMed

    Tsuruoka, Kayori; Sekiya, Syuusuke; Yokoyama, Takeshi; Koitabashi, Kenichirou; Shimazaki, Minako; Sakurada, Tsutomu; Shirai, Sayuri; Yasuda, Takashi; Kimura, Kenjirou

    2008-01-01

    A 72-year-old woman was admitted to our hospital because of massive proteinuria of 2.2 g/day. She had seen a general practitioner for management of Basedow disease, diabetes mellitus and hypertension for 24 years. On admission, she complained of anorexia and nausea. Laboratory data showed serum creatinine of 3.62 mg/dL and MPO-ANCA of 68 EU. Renal biopsy revealed crescentic glomerulonephritis complicated with membranous nephropathy. Thiamazole (MMI), which was being given for Basedow disease for years, was withdrawn on the suspicion as a cause of MPO-ANCA. Three years after the withdrawal of MMI, renal failure slowly progressed to the end-stage, while MPO-ANCA was negative. She was introduced onto hemodialysis. At that time, MPO-ANCA became positive again, the titer being 12.9 EU. Therefore, we suspected a relapse of ANCA-related vasculitis and performed steroid pulse therapy on the patient with methylprednisolone at 0.5 g/day for 3 days. On hospital day 14, MPO-ANCA became negative. On day 25, however, severe acute pancreatitis developed and a pancreatic tumor lesion was found on CT. In spite of amelioration of the pancreatitis by medical treatment, the pancreatic tumor lesion did not show any significant change. On day 48, she died of multiple organ failure. Autopsy showed a mucinous cyst adenoma of pancreas and necrotizing pancreatitis. We suspected steroid therapy as the cause of the pancreatic lesions. PMID:19069154

  9. Systemic lupus erythematosus in association with ulcerative colitis: related autoimmune diseases.

    PubMed

    Stevens, H P; Ostlere, L S; Rustin, M H

    1994-03-01

    We report a patient who developed urticaria, angio-oedema and polyarthropathy secondary to the hypocomplementaemic urticarial vasculitis syndrome, a year prior to the onset of ulcerative colitis. Ten years later, primary sclerosing cholangitis and the antiphospholipid syndrome developed concomitantly. We believe this patient represents only the second reported case of idiopathic systemic lupus erythematosus (SLE) occurring in association with ulcerative colitis. PMID:8148283

  10. Restricted Diffusion of Pus in the Subarachnoid Space: MRSA Meningo-Vasculitis and Progressive Brainstem Ischemic Strokes – A Case Report

    PubMed Central

    Rose, David Z.; Parra-Herran, Carlos; Petito, Carol K.; Post, M. Judith D.

    2010-01-01

    Extra-axial restriction on diffusion weighted imaging (DWI) is an unusual finding on brain magnetic resonance imaging (MRI). Intra-axial restriction on DWI, however, is common, and can represent brain parenchymal infarction, tumor, abscess, or toxic-metabolic process. The infrequency of extra-axial DWI restriction and the paucity of clinico-pathological correlation in the literature limit its differential diagnosis. Scant case reports suggest that extra-axial DWI restriction could be a lymphoma, neurenteric cyst, or, in one patient, subdural empyema [1,2,3]. We postulate that pus formation must be excluded first, because it can provoke an aggressive meningo-vasculitis with rapidly fatal, intra-axial infarctions. Our patient was a 45-year-old man, presenting to our hospital with left facial droop and right (contralateral) arm and leg weakness. Initial MRI revealed DWI restriction in the left lateral pons, consistent with a classic Millard-Gubler stroke. Also noted was a subtle, extra-axial area of curvilinear diffusion restriction in the left cerebellar-pontine angle's subarachnoid space. Days later, the patient had a headache, and repeat MRI revealed extension of the two DWI lesions – both the intra-axial pontine infarction and the extra-axial area of restricted diffusion in the subarachnoid space. The patient became comatose, a third MRI revealed more extensive DWI restrictions, and he expired despite aggressive care. Autopsy revealed massive brainstem infarcts, a thick lymphoplasmacytic infiltrate, copious Gram-Positive cocci (likely MRSA) and arteries partially occluded with fibrointimal proliferation. This emphasizes the concept that extra-axial DWI restriction can represent pus development in the subarachnoid space – a radiographic marker to identify a patient at risk for demise due to septic, meningo-vasculitic infarctions. PMID:21045937

  11. Plasma exchange and glucocorticoid dosing in the treatment of anti-neutrophil cytoplasm antibody associated vasculitis (PEXIVAS): protocol for a randomized controlled trial

    PubMed Central

    2013-01-01

    Background Granulomatosis with polyangiitis (GPA, Wegener’s) and microscopic polyangiitis (MPA) are small vessel vasculitides collectively referred to as anti-neutrophil cytoplasm antibody-associated vasculitis (AAV). AAV is associated with high rates of morbidity and mortality due to uncontrolled disease and treatment toxicity. Small randomized trials suggest adjunctive plasma exchange may improve disease control, while observational evidence suggests that current oral glucocorticoid doses are associated with severe infections in patients with AAV. A randomized study of both plasma exchange and glucocorticoids is required to evaluate plasma exchange and oral glucocorticoid dosing in patients with AAV. Methods/design PEXIVAS is a two-by-two factorial randomized trial evaluating adjunctive plasma exchange and two oral glucocorticoid regimens in severe AAV. Five hundred patients are being randomized at centers across Europe, North America, Asia, and Australasia to receive plasma exchange or no plasma exchange, and to receive standard or reduced oral glucocorticoid dosing. All patients receive immunosuppression with either cyclophosphamide or rituximab. The primary outcome is the time to the composite of all-cause mortality and end-stage renal disease. PEXIVAS is funded by the National Institute of Health Research (UK), the Food and Drug Administration (USA), the National Institutes of Health (USA), the Canadian Institute of Health Research (Canada), the National Health and Medical Research Council (Australia), and Assistance Publique (France). Additional in-kind supplies for plasma exchange are provided by industry partners (TerumoBCT, Gambro Australia, and Fresenius Australia). Discussion This is the largest trial in AAV undertaken to date. PEXIVAS will inform the future standard of care for patients with severe AAV. The cooperation between investigators, funding agencies, and industry provides a model for conducting studies in rare diseases. Trial registration

  12. Adverse events and infectious burden, microbes and temporal outline from immunosuppressive therapy in antineutrophil cytoplasmic antibody-associated vasculitis with native renal function

    PubMed Central

    McGregor, JulieAnne G.; Negrete-Lopez, Roberto; Poulton, Caroline J.; Kidd, Jason M.; Katsanos, Suzanne L.; Goetz, Lindsey; Hu, Yichun; Nachman, Patrick H.; Falk, Ronald J.; Hogan, Susan L.

    2015-01-01

    Background Disease control in anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) with immunosuppression is effective but burdened by adverse events, especially infections. The study goal was to evaluate risks and types of infections in patients with AAV. Methods Biopsy-proven AAV patients (diagnosed 1/1991–6/2011) followed in an inception cohort were evaluated for adverse events. Severe infections (requiring intravenous antibiotics, intensive care unit, or causing death) were recorded. Infection number was grouped as none, 1–2 or ≥3. Cox regression was used to estimate hazard ratios with 95% confidence intervals. Results A total of 489 patients (median age 59; 47% female, 55% myeloperoxidase-ANCA) were followed for 2.8 years (median). At 1, 2 and 5 years cumulative incidence of infection was 51, 58 and 65% and severe infection was 22, 23 and 26%. Pulmonary and upper respiratory infections were most common (42 and 30% ever experienced each, respectively), highest in the first 3 months. Staphylococcus aureus was most frequently seen among positive cultures (41%, 78 S. aureus/192 total positive cultures), and only one Pneumocystis jiroveci pneumonia (6 weeks into treatment). All-cause death in 12 months was associated with infections (% deaths: 0 infections 3%; 1–2 infections 10%, ≥3 infections 13%, P = 0.002). Controlling for age, sex and kidney function, patients with severe infections were 4.2 times more likely to die within 12 months (95% CI 2.0–8.7; P = 0.001). Conclusions More infections increase the risk of a severe infection which increases risk of all-cause mortality. Respiratory and S. aureus infections are dominant. Targeted prophylactic therapy could decrease morbidity. PMID:25805747

  13. Double Filtration Plasmapheresis in the Treatment of Antineutrophil Cytoplasmic Autoantibody Associated Vasculitis With Severe Renal Failure: A Preliminary Study of 15 Patients.

    PubMed

    Chen, Yinghua; Yang, Liu; Li, Kang; Liu, Zhengzhao; Gong, Dehua; Zhang, Haitao; Liu, Zhihong; Hu, Weixin

    2016-04-01

    Our aim was to investigate the clinical efficacy of double filtration plasmapheresis (DFPP) in the treatment of antineutrophil cytoplasmic autoantibody-(ANCA) associated vasculitis (AAV) with severe renal involvement. Fifteen AAV patients who had severe renal failure (median SCr 5.6(IQR 5.2-9.0) mg/dL) and needed initial renal replacement therapy (RRT) were treated with DFPP and immunosuppressive therapy. Two plasma volumes were processed during each DFPP session. The changes of serum ANCA and renal function were investigated. After the DFPP treatment for three to five sessions, serum MPO-ANCA level decreased from 250.0 ± 86.9 RU/mL to 70.5 ± 64.7RU/mL (P = 0.00), with a median reduction rate of 67.6%. Eleven patients (73.3%) no longer needed from RRT 3 months after DFPP treatment, while another four patients remained on dialysis. During the follow up for median 10 (IQR 6-24) months, SCr level decreased to normal in one patient, one patient progressed into ESRD. The 1 year renal survival rate was 62.9%. Five (33.3%) patients were complicated with pulmonary infection. DFPP combined with immunosuppressive therapy could increase the renal recovery rate through rapidly decreasing serum ANCA levels for AAV patients with severe renal failure, but its clinical efficacy and impact on long-term renal survival require further studies. PMID:26948291

  14. Neutrophil-Related Gene Expression And Low-Density Granulocytes Associated with Disease Activity and Response to Treatment in ANCA-Associated Vasculitis

    PubMed Central

    Grayson, Peter C.; Carmona-Rivera, Carmelo; Xu, Lijing; Lim, Noha; Gao, Zhong; Asare, Adam L.; Specks, Ulrich; Stone, John H.; Seo, Philip; Spiera, Robert F.; Langford, Carol A.; Hoffman, Gary S.; Kallenberg, Cees G.M.; St Clair, E. William; Tchao, Nadia K.; Ytterberg, Steven R.; Phippard, Deborah J.; Merkel, Peter A.; Kaplan, Mariana J.; Monach, Paul A.

    2015-01-01

    Objectives To discover biomarkers involved in the pathophysiology of ANCA-associated vasculitis (AAV) and determine if low-density granulocytes (LDGs) contribute to gene expression signatures in AAV. Methods The source of clinical data and linked biospecimens was a randomized controlled treatment trial in AAV. RNA-sequencing of whole blood from patients with AAV was performed during active disease at the baseline visit (BL) and during remission 6 months later (6M). Gene expression was compared between patients who met versus did not meet the primary trial outcome of clinical remission at 6M (responders vs. nonresponders). Measurement of neutrophil-related gene expression was confirmed in PBMCs to validate findings in whole blood. A negative selection strategy isolated LDGs from PBMC fractions. Results Differential expression between responders (n=77) and nonresponders (n=35) was detected in 2,346 transcripts at BL visit (p<0.05). Unsupervised hierarchical clustering demonstrated a cluster of granulocyte-related genes, including myeloperoxidase (MPO) and proteinase 3 (PR3). A granulocyte multi-gene composite score was significantly higher in nonresponders than responders (p<0.01) and during active disease compared to remission (p<0.01). This signature strongly overlapped an LDG signature identified previously in lupus (FDRGSEA<0.01). Transcription of PR3 measured in PBMCs was associated with active disease and treatment response (p<0.01). LDGs isolated from patients with AAV spontaneously formed neutrophil extracellular traps containing PR3 and MPO. Conclusions In AAV an increased expression of a granulocyte gene signature is associated with disease activity and decreased response to treatment. The source of this signature is likely LDGs, a potentially pathogenic cell type in AAV. PMID:25891759

  15. Reduced CD5(+) CD24(hi) CD38(hi) and interleukin-10(+) regulatory B cells in active anti-neutrophil cytoplasmic autoantibody-associated vasculitis permit increased circulating autoantibodies.

    PubMed

    Aybar, L T; McGregor, J G; Hogan, S L; Hu, Y; Mendoza, C E; Brant, E J; Poulton, C J; Henderson, C D; Falk, R J; Bunch, D O

    2015-05-01

    Pathogenesis of anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis is B cell-dependent, although how particular B cell subsets modulate immunopathogenesis remains unknown. Although their phenotype remains controversial, regulatory B cells (Bregs ), play a role in immunological tolerance via interleukin (IL)-10. Putative CD19(+) CD24(hi) CD38(hi) and CD19(+) CD24(hi) CD27(+) Bregs were evaluated in addition to their CD5(+) subsets in 69 patients with ANCA-associated vasculitis (AAV). B cell IL-10 was verified by flow cytometry following culture with CD40 ligand and cytosine-phosphate-guanosine (CpG) DNA. Patients with active disease had decreased levels of CD5(+) CD24(hi) CD38(hi) B cells and IL-10(+) B cells compared to patients in remission and healthy controls (HCs). As IL-10(+) and CD5(+) CD24(hi) CD38(hi) B cells normalized in remission within an individual, ANCA titres decreased. The CD5(+) subset of CD24(hi) CD38(hi) B cells decreases in active disease and rebounds during remission similarly to IL-10-producing B cells. Moreover, CD5(+) B cells are enriched in the ability to produce IL-10 compared to CD5(neg) B cells. Together these results suggest that CD5 may identify functional IL-10-producing Bregs . The malfunction of Bregs during active disease due to reduced IL-10 expression may thus permit ANCA production. PMID:25376552

  16. [Systemic urticaria in 2014].

    PubMed

    Doutre, M-S

    2014-11-01

    Systemic urticaria are defined as urticaria, most often chronic, associated with systemic diseases. At present time, urticarial vasculitis and neutrophilic urticarial dermatosis associated to autoinflammatory syndromes are not considered to be subtypes of chronic spontaneous urticaria due to their distinctly clinical and histological characteristics as well different pathomechanisms. Sometimes, chronic urticaria is associated to thyroid autoimmunity. However, the majority of cases of chronic spontaneous urticaria have no discernible cause and further investigations are not necessary, as already suggested by some authors and French consensus conference more than 10 years ago. PMID:25539679

  17. How not to miss autoinflammatory diseases masquerading as urticaria.

    PubMed

    Krause, K; Grattan, C E; Bindslev-Jensen, C; Gattorno, M; Kallinich, T; de Koning, H D; Lachmann, H J; Lipsker, D; Navarini, A A; Simon, A; Traidl-Hoffmann, C; Maurer, M

    2012-12-01

    Urticarial skin reactions are one of the most frequent problems seen by allergists and clinical immunologists in daily practice. The most common reason for recurrent wheals is spontaneous urticaria. There are, however, several less common diseases that present with urticarial rash, such as urticarial vasculitis and autoinflammatory disorders. The latter include cryopyrin-associated periodic syndrome and Schnitzler's syndrome, both rare and disabling conditions mediated by increased interleukin-1 secretion. Apart from the urticarial rash, patients are suffering from a variety of systemic symptoms including recurrent fever attacks, arthralgia or arthritis and fatigue. Autoinflammatory diseases are often associated with a diagnostic delay of many years and do not respond to antihistamines and other treatments of urticaria. Also, the chronic inflammation may lead to long-term complications such as amyloidosis. It is therefore important not to miss these diseases when diagnosing and treating patients with chronic recurrent urticarial rash. Here, we present clinical clues and tips that can help to identify autoinflammatory disorders in patients presenting with chronic urticarial rash and discuss their clinical picture and management. PMID:22978406

  18. Urticaria mimickers in children.

    PubMed

    Mathur, Anubhav N; Mathes, Erin F

    2013-01-01

    Acute urticaria is a self-limited cutaneous condition marked by transient, erythematous, and pruritic wheals. It is a hypersensitivity response that is often secondary to infection, medications, or food allergies in children. In contrast, the urticarial "mimickers" described in this review article are often seen in the context of fever and extracutaneous manifestations in pediatric patients. The differential diagnosis ranges from benign and self-limited hypersensitivity responses to multisystem inflammatory diseases. Establishing the correct diagnosis of an urticarial rash in a pediatric patient is necessary to both prevent an unnecessary work up for self-limited conditions and to appropriately recognize and evaluate multisystem inflammatory disorders. Herein, we describe two cases to illustrate the clinical manifestations, laboratory findings, histopathology and differential diagnoses for several mimickers of acute urticaria including: urticaria multiforme, serum sickness like reaction, Henoch-Schönlein purpura, acute hemorrhagic edema of infancy, systemic onset juvenile idiopathic arthritis, cryopyrin associated periodic syndromes, and urticarial vasculitis. PMID:24552410

  19. [Cutaneous vasculitides. A diagnostic approach].

    PubMed

    Schäkel, K; Meurer, M

    2008-05-01

    Vasculitis is characterized by an inflammatory reaction of vessel walls with damage to the dependent tissues. Forms of vasculitis which frequently have skin changes include leukocytoclastic angiitis (LcV), Henoch-Schönlein purpura (HSP), cutaneous polyarteriitis nodosum (cPAN), erythema elevatum et diutinum (EED) and urticarial vasculitis (UV). In other forms of vasculitis, systemic manifestations predominate but there are a variety of skin changes. Kawasaki disease (MK), cryoglobulinemic vasculitis (kV), Wegener granulomatosis (WG), Churg-Strauss syndrome (CSS) and microscopic polyangitis (MPA) belong to this group. The causes of vasculitis are heterogeneous. Triggers include infections, drugs, collagen vascular diseases, autoimmune diseases and lymphoproliferative disorders. Idiopathic vasculitis, particularly LcV and EED, occur only once and have a self-limited course. The diagnostic work up depends on the clinical picture and includes inflammatory markers, circulating immune complexes, different types of cryoglobulins and anti-neutrophilic cytoplasmic antibodies, collagen vascular disease specific autoantibodies and additional hematological studies. Vasculitis can manifest in many organs and requires a thorough work up specifically in cases where WG, MPA, CSS and PAN are under consideration. PMID:18415062

  20. Recent advances in understanding and treating vasculitis

    PubMed Central

    Koster, Matthew J.; Warrington, Kenneth J.

    2016-01-01

    Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs) are near universally fatal conditions if untreated. Although effective therapeutic options are available for these diseases, treatment regimens are associated with both short- and long-term adverse effects. The recent identification of effective B-cell-targeted therapy with an anti-CD20 monoclonal antibody has transformed the treatment landscape of AAV. Questions, nevertheless, remain regarding the appropriate timing, dose, frequency, duration, and long-term effects of treatment. The aim of this article is to provide an overview of the current information, recent advances, ongoing clinical trials, and future treatment possibilities in AAV. PMID:27347395

  1. Schnitzler's syndrome with monoclonal IgG kappa gammopathy: good response to cyclosporin.

    PubMed

    Pascual-López, M; Hernández-Núñez, A; Sánchez-Pérez, J; Fernández-Herrera, J; García-Díez, A

    2002-05-01

    Schnitzler's syndrome (SS) is a rare entity characterized by the association of chronic urticaria and monoclonal IgM gammopathy. Usually, intermittent fever, arthralgia and elevation of erythrocyte sedimentation rate also occur. We report a patient with the same symptoms, but with monoclonal IgG instead of IgM paraproteinaemia. Histological examination of the urticarial lesions showed signs of leucocytoclastic vasculitis. After 20 years of therapeutic failure, cyclosporin has achieved a total clearance of urticarial lesions in our patient. Two previous similar cases with clinical features of SS and monoclonal IgG immunoglobulin have been described. We suggest our case also represents a variant of SS with IgG gammopathy. PMID:12195569

  2. Chronic urticaria: recent advances.

    PubMed

    Greaves, Malcolm W; Tan, Kian Teo

    2007-10-01

    Chronic urticaria is an umbrella term, which encompasses physical urticarias, chronic "idiopathic" urticaria and urticarial vasculitis. It is important to recognize patients with physical urticarias as the investigation and treatment differs in important ways from patients with idiopathic chronic urticaria or urticarial vasculitis. Although relatively uncommon, urticarial vasculitis is an important diagnosis to make and requires histological confirmation by biopsy. Underlying systemic disease and systemic involvement, especially of the kidneys, should be sought. It is now recognized that chronic "idiopathic" urticaria includes a subset with an autoimmune basis caused by circulating autoantibodies against the high affinity IgE receptor (FceR1) and less commonly against IgE. Although the autologous serum skin test has been proven useful in prompting search for and characterization of circulating wheal-producing factors in chronic urticaria, its specificity as a screening test for presence of functional anti-FceR1 is low, and confirmation by demonstration of histamine-releasing activity in the patient's serum must be the benchmark test in establishing this diagnosis. Improved screening tests are being sought; for example, ability of the chronic urticaria patient's serum to evoke expression of CD 203c on donor human basophils is showing some promise. The strong association between autoimmune thyroid disease and autoimmune urticaria is also an area of ongoing research. Drug treatment continues to be centered on the H1 antihistamines, and the newer second-generation compounds appear to be safe and effective even in off-label dosage. Use of systemic steroids should be confined to special circumstances such as tapering regimens for acute flare-ups. Use of leukotriene antagonists is becoming popular, but the evidence for efficacy is conflicting. Cyclosporin is also effective and can be used in selected cases of autoimmune urticaria, and it is also effective in non

  3. Loa loa infection as a cause of migratory angioedema: report of three cases from the Texas Medical Center.

    PubMed

    Rakita, R M; White, A C; Kielhofner, M A

    1993-10-01

    Loa loa is a filarial parasite that is endemic in West and Central Africa. Expatriates infected with L. loa commonly develop characteristic migratory angioedema called Calabar swellings. We describe three patients who presented with classical Calabar swellings for whom the diagnosis of loiasis was delayed. Unusual features that were present included a leukocytoclastic vasculitis seen on skin biopsy (case 1), a relatively short exposure to an area of endemicity (cases 1 and 3), and a normal eosinophil count on initial evaluation (cases 2 and 3). We discuss the characteristic clinical manifestations of, pathological findings of, and therapy for loiasis. With the increased frequency of international travel, L. loa infection must be considered in the differential diagnosis for patients with migratory angioedema, urticarial vasculitis, or eosinophilia. PMID:8268351

  4. Overexpression of p16(INK4a) in Mastocytosis (Urticarial Pigmentosa).

    PubMed

    Tsujita, Jun; Doi, Kazuko; Nakahara, Makiko; Nakahara, Takeshi; Kaku, Yumiko; Nishio, Kiichiro; Kan, Nagisa; Sato, Yuki; Nagata, Shoko; Nakao, Asako; Yoshida, Maiko; Uchi, Hiroshi; Furue, Masutaka

    2016-01-01

    The expression of p16(INK4a) has been reported to induce cell-cycle arrest and cellular senescence. The p16(INK4a) expression has never been examined in human mast cells and mastocytosis. We immunohistologically examined the expression of p16(INK4a) and tryptase in 5 normal human skin and 4 mastocytosis. In normal mast cells, only 5.9 ± 3.4 (mean ± standard deviation) % of tryptase-positive mast cells coexpressed p16(INK4a). However, significantly higher percentage (86.0 ± 14.1%) of tryptase-positive tumor cells was immunoreactive to p16(INK4a) in all of 4 mastocytosis. The p16(INK4a) overexpression may induce the senescence of neoplastic mast cells to undergo spontaneous regression of mastocytosis. PMID:27333655

  5. Neutrophilic Dermatosis Limited to Lipo-Lymphedematous Skin in a Morbidly Obese Woman on Dasatinib Therapy.

    PubMed

    Ainechi, Sanaz; Carlson, John Andrew

    2016-02-01

    Neutrophilic dermatosis (ND) confined to postmastectomy lymphedema, localized Sweet syndrome, is a newly recognized disease. In this study, the authors describe a 44-year-old obese woman with chronic myelogenous leukemia in molecular remission on dasatinib therapy, who presented with a painful urticarial eruption limited to lipo-lymphedematous skin and accompanied by malaise, episodic fever, diarrhea, neutrophilia, and leukocytosis. Initially transient and migratory, the rash became fixed, papular, and vesicular and showed minimal response to corticosteroids. Biopsy demonstrated sparse perivascular and interstitial dermal neutrophilic infiltrates, without vasculitis or significant dermal edema. Aggregates of neutrophils were found within and surrounding lymphangiectases. Biopsy of a new onset papule 3 weeks later demonstrated papillary dermal edema, denser neutrophilic infiltrate, and vasculitis-like changes. These 2 histopathologic patterns of ND, early and late, resemble neutrophilic urticarial dermatitis (also known as neutrophilic dermatitis with systemic inflammation) and Sweet syndrome, respectively. Extensive workup did not reveal evidence of relapsed chronic myelogenous leukemia, infection, or a coexisting systemic inflammatory disease. Dasatinib was discontinued and the eruption gradually resolved over 2.5 months. Still in molecular remission (no detectable BCR-ABL gene fusion), dasatinib therapy was recommenced at 3-month follow-up. After 10 months, she complains of malaise and arthralgia, but no cutaneous symptoms. The evolution and slow resolution of this ND in lipo-lymphedematous skin implicate poor lymphatic clearance of factors, antigenic and/or toxic, involved in the pathogenesis of ND. PMID:26825166

  6. Skin rash and arthritis a simplified appraisal of less common associations.

    PubMed

    Cozzi, A; Doria, A; Gisondi, P; Girolomoni, G

    2014-06-01

    Skin and joint manifestations are part of the clinical spectrum of many disorders. Well-known associations include psoriatic arthritis and arthritis associated with autoimmune connective tissue diseases. This review focuses on less common associations where skin lesions can provide easily accessible and valuable diagnostic clues, and directly lead to the specific diagnosis or limit the list of possibilities. This may also affect health care resources as diagnostic tests are often low-specific, highly expensive and poorly available. This group of diseases can be divided into two subsets, based on the presence/absence of fever, and then further classified according to elementary skin lesions (macular, urticarial, maculo-papular, vesico-bullous, pustular, petechial and nodular). In most instances joint involvement occurs as peripheral migrating polyarthritis. Erythematosus macular or urticarial rashes occur in most febrile disorders such as monogenic autoinflammatory syndromes, Schnitzler's syndrome, Still's disease and rheumatic fever and afebrile diseases as urticarial vasculitis. Pustular rash may be observed in chronic recurrent multifocal osteomyelitis (CRMO) and pyogenic arthritis with pyoderma gangrenosum and acne (PAPA) syndrome (both febrile) as well as in Behcet's disease and Synovitis, acne, pustulosis, hyperostosis and osteitis syndrome (both non-febrile). Papular lesions are typical of secondary syphilis, sarcoidosis, interstitial granulomatous dermatitis, papular petechial of cutaneous small-vessel vasculitis and nodular lesions of polyarteritis nodosa and multicentric reticulohistiocytosis all of which are afebrile. Differential diagnosis includes infections and drug reactions which may mimic several of these conditions. To biopsy the right skin lesion at the right time it is essential to obtain relevant histological information. PMID:23980929

  7. Bullous reactions to bed bug bites reflect cutaneous vasculitis

    Technology Transfer Automated Retrieval System (TEKTRAN)

    This study evaluates bullous cutaneous reactions and sequential histopathology in an individual sensitized to bed bug bites in an effort to better understand the allergic response and histology associated with these bites. There was a progression of the inflammatory response across time ranging from...

  8. Central nervous system vasculitis in adults and children.

    PubMed

    Twilt, Marinka; Benseler, Susanne M

    2016-01-01

    Primary angiitis of the central nervous system (PACNS) is an inflammatory brain disease targeting the cerebral blood vessels, leading to a wide spectrum of signs and symptoms, including neurologic deficits, cognitive dysfunction, and psychiatric symptoms. The inflammation could be reversible if diagnosed and treated early. The diagnosis requires the careful consideration and rapid evaluation of systemic underlying conditions and disease mimics. The differential diagnosis is distinctly different for angiography-positive and -negative PACNS subtypes and differs depending on age, so there is childhood PACNS or adult PACNS. Distinct disease subtypes have been described, with characteristic disease course, neuroimaging findings, and histopathologic features. Novel and traditional biomarkers, including von Willebrand factor antigen and cytokine levels, can help diagnose, and define subtype and disease activity. Treatment of PACNS should be tailored to the disease subtypes and clinical symptoms. Beyond immunosuppression it should include medications to control symptoms in order to support and enhance the child's or adult's ability to actively participate in rehabilitation. The mortality of PACNS has decreased; studies determining the morbidity and its determinants are urgently needed. PMID:27112683

  9. The lung in systemic vasculitis: radiological patterns and differential diagnosis.

    PubMed

    Feragalli, Beatrice; Mantini, Cesare; Sperandeo, Marco; Galluzzo, Michele; Belcaro, Giovanni; Tartaro, Armando; Cotroneo, Antonio R

    2016-05-01

    The respiratory system may be involved in all systemic vasculitides, although with a variable frequency. The aim of our review is to describe radiographic and high-resolution CT (HRCT) findings of pulmonary vasculitides and to correlate radiological findings with pathological results. Lung disease is a common feature of antineutrophil cytoplasmic autoantibody-associated small-vessel vasculitides, including granulomatosis with polyangiitis (Wegener's), eosinophilic granulomatosis with polyangiitis (Churg-Strauss) and microscopic polyangiitis. Pulmonary involvement is less frequent in immune-complex-mediated small-vessel vasculitides, such as Behçet's disease and Goodpasture's syndrome. Pulmonary involvement associated to large-vessel (gigantocellular arteritis and Takayasu's disease) or medium-vessel (nodose polyarteritis and Kawasaki's disease) vasculitides is extremely rare. The present review describes the main clinical and radiological features of pulmonary vasculitides with major purpose to correlate HRCT findings (solitary or multiple nodules, cavitary lesions, micronodules with centrilobular or peribronchial distribution, airspace consolidations, "crazy paving", tracheobronchial involvement, interstitial disease) with pathological results paying particular attention to the description of acute life-threatening manifestations. A thorough medical history, careful clinical examination and the knowledge of radiological patterns are mandatory for a correct and early diagnosis. PMID:26876879

  10. Undifferentiated vasculitis or an evolving systemic autoimmune rheumatic disease?

    PubMed

    Fatimah, Nafeesah; Ussaid, Ahmad; Rasheed, Aflak

    2016-08-01

    Undifferentiated connective tissue diseases usually present with arthralgias, sicca symptoms, Raynaud's phenomenon and leucopenia. This case presents the atypical presentation of an undifferentiated connective tissue disease with extensive cutaneous involvement of fingers and toes leading to gangrene with absence of typical rheumatological symptoms. The autoimmune profile showed positive ANA and anti-Ro/SS-A. Thromboembolism was ruled out on the basis of transthoracic and transesophageal echo. She was treated with I/V corticosteroids and cyclophosphamide that halted the disease progression. PMID:27574560

  11. Undifferentiated vasculitis or an evolving systemic autoimmune rheumatic disease?

    PubMed Central

    Fatimah, Nafeesah; Ussaid, Ahmad; Rasheed, Aflak

    2016-01-01

    Undifferentiated connective tissue diseases usually present with arthralgias, sicca symptoms, Raynaud's phenomenon and leucopenia. This case presents the atypical presentation of an undifferentiated connective tissue disease with extensive cutaneous involvement of fingers and toes leading to gangrene with absence of typical rheumatological symptoms. The autoimmune profile showed positive ANA and anti-Ro/SS-A. Thromboembolism was ruled out on the basis of transthoracic and transesophageal echo. She was treated with I/V corticosteroids and cyclophosphamide that halted the disease progression. PMID:27574560

  12. The role of myeloperoxidase in the pathogenesis of systemic vasculitis.

    PubMed

    Rutgers, A; Heeringa, P; Tervaert, J W

    2003-01-01

    Wegener's granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome and idiopathic pauci-immune necrotizing crescentic glomerulonephritis are strongly associated with the presence of anti-neutrophil cytoplasmic antibodies (ANCA). These ANCA-associated vasculitides can serologically be separated into myeloperoxidase (MPO)-ANCA and proteinase 3 (PR3)-ANCA positive patients. The unique properties of the antigen targeted by the anti-MPO antibodies could help to explain the specific characteristics of MPO-ANCA associated disease. Recently, an animal model has been developed that proves that anti-mouse MPO immunoglobulins alone are capable of causing disease similar to that in humans. Also, the in vitro pathologic effects of binding of MPO-ANCA to MPO are better understood. MPO-ANCA can activate (primed) neutrophils directly causing extensive reactive oxygen species formation and degranulation of neutrophil constituents, including MPO, resulting in a destructive inflammatory response towards the vessel wall. MPO-ANCA can prevent the clearing and inactivation of MPO by ceruloplasmin as well, resulting in increased myeloperoxidase activity. Myeloperoxidase produces not only the strong oxidant bleach (hypochlorous acid) out of hydrogen peroxide and chloride ions but also oxidizes LDL into a macrophage high-uptake form, inactivates protease inhibitors, and consumes nitric oxide. These may contribute to endothelial dysfunction and add to the chronic renal lesions observed in patients with MPO-ANCA. MPO levels are influenced by genetic factors including two, MPO463 and MPO129, single nucleotide polymorphisms. The MPO 463 polymorphism has been associated with an increased risk of development of MPO-ANCA associated disease. PMID:14740428

  13. Vasculitis Syndromes of the Central and Peripheral Nervous Systems

    MedlinePlus

    ... disturbances, such as double vision, blurred vision, or blindness seizures, convulsions stroke or transient ischemic attack (TIA, ... years and is rarely fatal. Abrupt but reversible blindness is the most dramatic complication of temporal arteritis. ...

  14. Macrophages in Vascular Inflammation – From Atherosclerosis to Vasculitis

    PubMed Central

    Shirai, Tsuyoshi; Hilhorst, Marc; Harrison, David G.; Goronzy, Jörg J.; Weyand, Cornelia M.

    2015-01-01

    The spectrum of vascular inflammatory disease ranges from atherosclerosis and hypertension, widespread conditions affecting large proportions of the population, to the vasculitides, rare syndromes leading to fast and irreversible organ failure. Atherosclerosis progresses over decades, inevitably proceeding through multiple phases of disease and causes its major complications when the vessel wall lesion ruptures, giving rise to lumen-occlusive atherothrombosis. Vasculitides of medium and large arteries progress rapidly, causing tissue ischemia through lumen-occlusive intimal hyperplasia. In both disease entities, macrophages play a decisive role in pathogenesis, but function in the context of other immune cells that direct their differentiation and their functional commitments. In atherosclerosis, macrophages are involved in the removal of lipids and tissue debris and make a critical contribution to tissue damage and wall remodeling. In several of the vasculitides, macrophages contribute to granuloma formation, a microstructural platform optimizing macrophage-T cell interactions, antigen containment and inflammatory amplification. By virtue of their versatility and plasticity, macrophages are able to promote a series of pathogenic functions, ranging from the release of cytokines and enzymes, the production of reactive oxygen species, presentation of antigen and secretion of tissue remodeling factors. However, as short-lived cells that lack memory, macrophages are also amendable to reprogramming, making them promising targets for anti-inflammatory interventions. PMID:25811915

  15. [If you go looking for trouble you'll find it. Urticarial reactions to insects of our local areas].

    PubMed

    Maitre, Sylvie; Kaeser, Amelie; Di Lucca, Julie; Spertini, François; Ribi, Camillo

    2016-04-01

    Itchy skin rashes are a frequent reason to seek medical advice. The symptoms may be caused by hypersensitivity reactions to arthropod bites, waterborne parasites or setae from moth caterpillars and are sometimes mistaken for spontaneous urticaria or eczema. Some of these pests are resurging in Switzerland and elsewhere and increasingly responsible for emergency consultation. In this article we review itchy skin rashes caused by bed bugs, scabies, lice, cercariae, Pyemotes spp, caterpillars and harvest mites, which may be confounded with urticaria and allergic contact dermatitis. We detail here clinical manifestations, topographical distribution of skin lesions, epidemiology, treatment and preventive measures. PMID:27197324

  16. Exercise-induced purpura.

    PubMed

    Ramelet, Albert-Adrien

    2004-01-01

    Exercise-induced purpura (EIP) occurs on the lower legs after unusual or major muscular activity, as in marathon runners or as after long walks, especially in the mountains in hot weather. In leisure walkers, patients are otherwise healthy females. There is no relation with chronic venous disorder. Erythematous, urticarial or purpuric plaques arise on the lower leg, usually sparing the skin compressed by socks. Symptoms include itch, pain and a burning sensation. Histopathology demonstrates leukocytoclastic vasculitis. The lesions fade after some days, with frequent relapses at further muscular exercises and may be prevented in some cases by compression, intake of venoactive drugs and local application of steroids. EIP is not uncommon, even if very few descriptions have yet been published. It appears to be consecutive to venous stasis induced by an acute failure of the muscle pump of the calf and thermoregulation decompensation, after a prolonged and unusual exercise, such as running or walking in hot weather. PMID:15178910

  17. Lovenox Induced Tissue Necrosis, a Case Report and Literature Review

    PubMed Central

    Issa, Abdelfatah Abou; Simman, Richard

    2015-01-01

    Lovenox is a trade name for Enoxaparin. It is a low molecular weight heparin (LMWH) and has other trade names like Clexane and Xaparin. It is an anticoagulant used to prevent and treat venous thromboembolism events (VTE) like deep vein thrombosis or pulmonary embolism, and is given as a subcutaneous injection. General speaking, the most common skin reactions as a result of enoxaparin use are: urticarial, ecchymosis, and even skin necrosis due to vasculitis. These side effects are usually located at the injection site. New studies have pointed out the side effect that could occur a distance from the site of Lovenox injection. In our case extensive skin and subcutaneous tissue necrosis developed at the abdominal wall injection site. PMID:26199895

  18. Chronic urticaria.

    PubMed

    Burrall, B A; Halpern, G M; Huntley, A C

    1990-03-01

    Urticaria affects 15% to 20% of the population once or more during a lifetime. Chronic urticaria is a frequent recurrent eruption over a period greater than 6 weeks; the cause remains a mystery in more than 75% of cases. Urticaria and angioedema may be produced by immunologic or nonimmunologic means. Urticarial vasculitis, contact urticaria, mastocytosis, physical urticarias, dermatographism, cholinergic urticaria, localized heat urticaria, cold urticaria, aquagenic urticaria, and vibratory angioedema all require specific evaluation and treatment. Chronic idiopathic urticaria is usually controlled by antihistamines; depending on the circadian rhythm of the eruption, sedative or nonsedative antihistamines are prescribed. Some patients will require a combination of H1 and H2 antagonists, or even parenteral corticosteroids. PMID:1970697

  19. Chronic urticaria.

    PubMed Central

    Burrall, B. A.; Halpern, G. M.; Huntley, A. C.

    1990-01-01

    Urticaria affects 15% to 20% of the population once or more during a lifetime. Chronic urticaria is a frequent recurrent eruption over a period greater than 6 weeks; the cause remains a mystery in more than 75% of cases. Urticaria and angioedema may be produced by immunologic or nonimmunologic means. Urticarial vasculitis, contact urticaria, mastocytosis, physical urticarias, dermatographism, cholinergic urticaria, localized heat urticaria, cold urticaria, aquagenic urticaria, and vibratory angioedema all require specific evaluation and treatment. Chronic idiopathic urticaria is usually controlled by antihistamines; depending on the circadian rhythm of the eruption, sedative or nonsedative antihistamines are prescribed. Some patients will require a combination of H1 and H2 antagonists, or even parenteral corticosteroids. PMID:1970697

  20. Chronic urticaria: new management options.

    PubMed

    Greenberger, Paul A

    2014-01-01

    Chronic urticaria is defined as episodic or daily hives lasting for at least 6 weeks and impairs quality of life. Two main subtypes include chronic idiopathic (spontaneous) urticaria and inducible (physical) urticaria, but some patients have urticarial vasculitis. "Autoimmune chronic urticaria" implies the presence of histamine releasing or mast cell activating autoantibodies to IgE or FcϵRI, the high affinity receptor on mast cells and basophils. In patients not readily controlled with labeled dosages of second generation H1 receptor antagonists (antihistamines), there is evidence for reduction of urticaria using up to 4 fold increases in labeled dosages. The biologic modifier, omalizumab, helps to reduce lesions of chronic urticaria within 1-2 weeks. PMID:25383135

  1. Lovenox Induced Tissue Necrosis, a Case Report and Literature Review.

    PubMed

    Issa, Abdelfatah Abou; Simman, Richard

    2013-12-01

    Lovenox is a trade name for Enoxaparin. It is a low molecular weight heparin (LMWH) and has other trade names like Clexane and Xaparin. It is an anticoagulant used to prevent and treat venous thromboembolism events (VTE) like deep vein thrombosis or pulmonary embolism, and is given as a subcutaneous injection. General speaking, the most common skin reactions as a result of enoxaparin use are: urticarial, ecchymosis, and even skin necrosis due to vasculitis. These side effects are usually located at the injection site. New studies have pointed out the side effect that could occur a distance from the site of Lovenox injection. In our case extensive skin and subcutaneous tissue necrosis developed at the abdominal wall injection site. PMID:26199895

  2. The progressive systemic sclerosis/systemic lupus overlap: an unusual clinical progression.

    PubMed Central

    Asherson, R A; Angus, H; Mathews, J A; Meyers, O; Hughes, G R

    1991-01-01

    Three patients with the unusual combinations of discoid lupus, systemic lupus erythematosus (SLE), and progressive systemic sclerosis (PSS) are reported. The first patient developed PSS eight years after a diagnosis of discoid lupus had been made and this was complicated by myositis six years later. The second patient developed PSS more than 20 years after being diagnosed as having SLE. The third patient developed SLE with predominant features of urticarial vasculitis six years after PSS. Mild myositis also ensued. There were no antibodies to U1RNP demonstrable in any of these patients. The clinical progression of SLE to PSS or vice versa in the absence of features of mixed connective tissue disease is distinctly uncommon. Images PMID:2042989

  3. Comorbidity and pathogenic links of chronic spontaneous urticaria and systemic lupus erythematosus--a systematic review.

    PubMed

    Kolkhir, P; Pogorelov, D; Olisova, O; Maurer, M

    2016-02-01

    Chronic spontaneous urticaria (CSU) is a common mast cell-driven disease characterized by the development of wheals (hives), angioedema (AE), or both for > 6 weeks. It is thought that autoimmunity is a common cause of CSU, which is often associated with autoimmune thyroiditis, whereas the link to other autoimmune disorders such as systemic lupus erythematosus (SLE) has not been carefully explored. Here, we systematically reviewed the existing literature for information on the prevalence of CSU in SLE (and vice versa) and we examined the possible clinical and pathogenetic relationship between CSU and SLE. The prevalence of CSU and CSU-like rash in SLE was investigated by 42 independent studies and comorbidity in adult patients reportedly ranged from 0% to 21.9% and 0.4% to 27.5%, respectively (urticarial vasculitis: 0-20%). In children with SLE, CSU was reported in 0-1.2% and CSU-like rash in 4.5-12% (urticarial vasculitis: 0-2.2%). In contrast, little information is available on the prevalence of SLE in patients with CSU, and more studies are needed to determine the rate of comorbidity. Recent insights on IgG- and IgE-mediated autoreactivity suggest similarities in the pathogenesis of CSU and SLE linking inflammation and autoimmunity with the activation of the complement and coagulation system. Future studies of patients with either or both conditions could help to better define common pathomechanisms in CSU and SLE and to develop novel targeted treatment options for patients with CSU and SLE. PMID:26545308

  4. Identification of the etiologies of chronic urticaria in children: a prospective study of 94 patients.

    PubMed

    Jirapongsananuruk, Orathai; Pongpreuksa, Sureerat; Sangacharoenkit, Preeda; Visitsunthorn, Nualanong; Vichyanond, Pakit

    2010-05-01

    The etiologies of chronic urticaria (CU) in childhood remains incompletely understood because of limited data in children. The objective of this study was to examine some of the possible etiologies of CU in children by focusing on the functional autoantibody to FcepsilonRIalpha and IgE, thyroid autoimmunity, urticarial vasculitis, parasitic infestation and food allergy. Children 4-15 yr of age with CU were investigated for complete blood count, erythrocyte sedimentation rate (ESR), antinuclear antibody (ANA), CH(50), free-T4 (FT(4)), thyroid stimulating hormone (TSH), anti-thyroglobulin and anti-microsomal antibody, autologous serum skin test (ASST), skin prick tests (SPT) for foods, food challenges, and stool examination for parasites. Ninety-four children who met the criteria for CU were recruited. Patients with physical urticaria were excluded. Eosinophilia and elevated ESR were found in 23% and 13%, respectively. High ANA titers were found in 2%. None of these patients had clinical features of urticarial vasculitis, abnormal CH(50) level, abnormal TSH and FT(4). Anti-thyroglobulin and anti-microsomal antibodies were not detected. Positive ASST was found in 38%. There were no differences in medication requirement and CU remission between patients with positive and negative ASST. Parasites were found in 5% without clinical correlation. SPT to foods was positive in 35%. Positive food challenges were found in six/nine patients with positive history of food allergy and two/seven patients with negative history. Food avoidance was beneficial to the subgroup of patients with positive history of food allergy only. PMID:19555353

  5. Skin biopsies in the evaluation of atypical optic neuropathies.

    PubMed

    Bielory, L; Kupersmith, M; Warren, F; Bystryn, J; Frohman, L

    1993-01-01

    Patients with atypical clinical presentations of common optic neuropathies such as optic neuritis (ON), anterior ischemic optic neuropathy (AION), or optic neuropathy of unknown etiology (UON) are difficult to distinguish from inflammatory autoimmune optic neuropathy (AON) which is typically associated with a poor visual prognosis, unless treated with high doses of corticosteroids and/or immunosuppressive agents. The authors retrospectively evaluated 34 patients [AON (n = 12); AION (n = 5); ON (n = 9); UON (n = 8)] with visual loss which deteriorated over weeks to months or followed an atypical course, for the presence of immunological markers suggestive of AON. These markers included serological testing for antiphospholipid (APA) and antinuclear (ANA) antibodies, and evaluation of histopathologic and immunofluorescent staining of skin biopsies. All patients underwent a skin biopsy. Four of the 12 patients with AON had urticarial cutaneous lesions which revealed leukocytoclastic and/or lymphohistiocytic vasculitis. Seven of the remaining eight AON patients had skin biopsies of non-lesional skin which revealed immunoreactant deposition. Seven of the 21 skin biopsies obtained from the non-AON patients had findings of vacuolization or mild perivascular infiltration of lymphocytes (n = 5) and immunofluorescent deposits (n = 2). Abnormal skin biopsies (92%;p = 0.0009) and circulating APA (82%; p = 0.013) were common in AON patients while ANA was not statistically increased in AON patients (p = 0.06) when compared to the remaining patients as a whole. AON patients typically demonstrate evidence of systemic autoimmune involvement, as manifested by cutaneous abnormalities such as urticarial vasculitis and/or immunoreactant deposition and circulating APA. These may serve as markers for identifying AON patients who may be treated with immunomodulatory agents. PMID:22822778

  6. Anti-C1q in systemic lupus erythematosus.

    PubMed

    Stojan, G; Petri, M

    2016-07-01

    C1q is the first component of the classical complement pathway. Both clinically validated in-house ELISA assays as well as commercial ELISA kits are used for detection of anti-C1q antibodies. Anti-C1q autoantibodies can be detected in a wide range of autoimmune diseases and are highly sensitive for hypocomplementemic uticarial vasculitis. In SLE, anti-C1q are strongly associated with proliferative lupus nephritis, and their absence carries a negative predictive value for development of lupus nephritis of close to 100%. Anti-C1q in combination with anti-dsDNA and low complement has the strongest serological association with renal involvement. The anti-C1q titers correlate with global disease activity scores in patients with renal involvement, and higher titers seem to precede renal flares. After the successful treatment of a renal flare, anti-C1q has the tendency to decrease or even become undetectable. The main obstacle to the inclusion of anti-C1q in the classification criteria and clinical management of SLE is the lack of standardized laboratory assays. PMID:27252264

  7. Increased angiogenesis and enhanced bone formation in patients with IgM monoclonal gammopathy and urticarial skin rash: new insight into the biology of Schnitzler syndrome

    PubMed Central

    Terpos, Evangelos; Asli, Bouchra; Christoulas, Dimitrios; Brouet, Jean-Claude; Kastritis, Efstathios; Rybojad, Michel; Bengoufa, Djaouida; Dimopoulos, Meletios A.; Fermand, Jean Paul

    2012-01-01

    Schnitzler syndrome is a rare plasma cell disorder the pathogenesis of which is still not fully understood. We evaluated the circulating levels of four major angiogenic cytokines (VEGF, angiogenin, angiopoietin-1 and angiopoietin-2) and six bone remodeling markers (sRANKL, osteoprotegerin, dickkopf-1, CTX, osteocalcin and bone-specific alkaline phosphatase-bALP) in 13 patients with Schnitzler syndrome. At diagnosis, patients had elevated angiogenic cytokines. The mean VEGF levels were almost 3.5-fold higher in Schnitzler syndrome compared to controls, while 10 of 13 patients had higher VEGF than the upper control value. Successful treatment led to a significant reduction in VEGF. Patients with Schnitzler syndrome had increased bone formation (high bALP, osteocalcin and osteoprotegerin) which was not balanced by an increase in bone resorption (normal CTX and sRANKL). These data support a role for VEGF as a new minor criterion in the diagnosis and follow up of Schnitzler syndrome, while the uncoupling of bone remodeling in favor of bone formation justifies the presence of bone densification. PMID:22689688

  8. IgG4-related skin disease.

    PubMed

    Tokura, Y; Yagi, H; Yanaguchi, H; Majima, Y; Kasuya, A; Ito, T; Maekawa, M; Hashizume, H

    2014-11-01

    IgG4-related disease (IgG4-RD) is a recently established clinical entity characterized by high levels of circulating IgG4, and tissue infiltration of IgG4(+) plasma cells. IgG4-RD exhibits a distinctive fibroinflammatory change involving multiple organs, such as the pancreas and salivary and lacrimal glands. The skin lesions of IgG4-RD have been poorly characterized and may stem not only from direct infiltration of plasma cells but also from IgG4-mediated inflammation. Based on the documented cases together with ours, we categorized the skin lesions into seven subtypes: (1) cutaneous plasmacytosis (multiple papulonodules or indurations on the trunk and proximal part of the limbs), (2) pseudolymphoma and angiolymphoid hyperplasia with eosinophilia (plaques and papulonodules mainly on the periauricular, cheek and mandible regions), (3) Mikulicz disease (palpebral swelling, sicca syndrome and exophthalmos), (4) psoriasis-like eruption (strikingly mimicking psoriasis vulgaris), (5) unspecified maculopapular or erythematous eruptions, (6) hypergammaglobulinaemic purpura (bilateral asymmetrical palpable purpuric lesions on the lower extremities) and urticarial vasculitis (prolonged urticarial lesions occasionally with purpura) and (7) ischaemic digit (Raynaud phenomenon and digital gangrene). It is considered that subtypes 1-3 are induced by direct infiltration of IgG4(+) plasma cells, while the other types (4-7) are caused by secondary mechanisms. IgG4-related skin disease is defined as IgG4(+) plasma-cell-infiltrating skin lesions that form plaques, nodules or tumours (types 1-3), but may manifest secondary lesions caused by IgG4(+) plasma cells and/or IgG4 (types 4-7). PMID:25065694

  9. Rituximab as maintenance therapy for ANCA associated vasculitis: how, when and why?

    PubMed

    Alba, Marco A; Flores-Suárez, Luis Felipe

    2016-01-01

    ANCA-associated vasculitides (AAV) are chronic autoimmune diseases characterized by inflammation and destruction of small vessels. Rituximab is now licensed for use as a remission-induction agent in the treatment of these disorders. During recent years, several non-controlled studies have suggested that rituximab may be of value in maintaining disease remission in AAV. In these series, 3 techniques have been tried: "watch-and-wait", repeated cycles in fixed intervals, or administration based on proposed biomarkers. More importantly, the results of the MAINRITSAN trial showed that this anti-CD20 agent is superior to azathioprine for preventing major relapses in AAV. This review summarizes current information regarding the effectiveness, timing, dosing, duration and safety of rituximab as a valid option for remission maintenance. PMID:26255570

  10. Vasculitis resulting from a superficial femoral artery angioplasty with a paclitaxel-eluting balloon.

    PubMed

    Thomas, Shannon D; McDonald, Robert R A; Varcoe, Ramon L

    2014-02-01

    Drug-eluting balloons (DEBs) coated with the antiproliferative agent paclitaxel may improve primary patency by reducing recurrent luminal stenosis. A proportion of the active drug and excipient coating are known to embolize distally, but until now, there have been no reports of adverse events resulting from their use. We report an unusual case of a painful nodular, biopsy specimen-proven vasculitic rash that afflicted the ipsilateral lower limb of a patient after superficial femoral artery treatment with a DEB. This adverse event may have implications for the use of DEB in this and other vascular territories. PMID:23642919

  11. High-Resolution Vessel Wall Magnetic Resonance Imaging in Varicella-Zoster Virus Vasculitis.

    PubMed

    Tsivgoulis, Georgios; Lachanis, Stefanos; Magoufis, Georgios; Safouris, Apostolos; Kargiotis, Odysseas; Stamboulis, Elefterios

    2016-06-01

    Varicella-zoster virus vasculopathy is a rare but potentially treatable condition. Diagnosis has been based on angiography, brain magnetic resonance imaging (MRI), and cerebrospinal fluid analysis. High-resolution vessel wall MRI may aid to the diagnosis by differentiating inflammation from other vessel wall pathologies. We present the characteristic MRI findings of this condition in a young patient presenting with ischemic stroke. PMID:27067878

  12. Non-necrotizing Granulomatous Pulmonary Vasculitis Mimicking Lung Cancer on PET/CT.

    PubMed

    Dixon, Matthew; Shaw, Jason; Rankin, Linda; Lazzaro, Richard

    2013-12-01

    Fluorodeoxyglucose positron emission tomography ((18)FDG-PET) scan has become a valuable resource in the staging of lung cancer. Inflammation is known to cause false positives on (18)FDG-PET scan. In the absence of symptoms suggesting a diagnosis of an inflammatory condition, (18)FDG-avid lung masses on PET/CT scan is strongly suggestive of a diagnosis of lung cancer, rather than an inflammatory condition. We report the case of a 57-year-old man, with a history of heavy smoking and working in the sandblasting industry, with two suspicious (18)FDG-avid nodules in the left lung. Surgical specimens of these nodules revealed findings suspecting giant cell arteritis rather than malignancy. PMID:25360406

  13. In vivo approaches to investigate ANCA-associated vasculitis: lessons and limitations

    PubMed Central

    2011-01-01

    Anti-neutrophil cytoplasm autoantibody (ANCA)-associated diseases are autoimmune conditions characterized by necrotizing inflammation of small blood vessels. The immunogenesis and etiology of these conditions are unknown, but our knowledge of the immunopathogenesis has increased considerably in recent years. In this review, we discuss the animal models currently used to investigate the mechanisms of vascular injury and to test novel therapies. We outline their advantages and limitations and propose potential directions for future research. PMID:21371348

  14. [Report of a case of allergic granulomatous vasculitis (Churg-Strauss syndrome)].

    PubMed

    Albert, F; Bertani, E; Carabelli, A; Pavia, G

    1989-03-01

    A 35 year female with a previous history of asthma came to our observation about 10 months ago. She was dyspneic, showed small nodules (2 to 5 mm) in arms, hands and legs and had radiological evidence of pulmonary granulomatosis and pleural effusion. Diagnosis was made by means of cutaneous biopsy showing a necrotic granulomatous vasculitic lesions with eosinophilic infiltration and giant cells, consistent with allergic granulomatosis of Churg-Strauss syndrome. Steroid therapy alone (prednisone 1 mg/kg/die) led to a rapid and complete clinical and humoral remission. The patient is still doing an alternate day prednisone therapy (15 mg) and is well after a 10 months follow-up. PMID:2572542

  15. Paravascular pathways contribute to vasculitis and neuroinflammation after subarachnoid hemorrhage independently of glymphatic control

    PubMed Central

    Luo, C; Yao, X; Li, J; He, B; Liu, Q; Ren, H; Liang, F; Li, M; Lin, H; Peng, J; Yuan, T F; Pei, Z; Su, H

    2016-01-01

    Subarachnoid hemorrhage (SAH) is a devastating disease with high mortality. The mechanisms underlying its pathological complications have not been fully identified. Here, we investigate the potential involvement of the glymphatic system in the neuropathology of SAH. We demonstrate that blood components rapidly enter the paravascular space following SAH and penetrate into the perivascular parenchyma throughout the brain, causing disastrous events such as cerebral vasospasm, delayed cerebral ischemia, microcirculation dysfunction and widespread perivascular neuroinflammation. Clearance of the paravascular pathway with tissue-type plasminogen activator ameliorates the behavioral deficits and alleviates histological injury of SAH. Interestingly, AQP4−/− mice showed no improvements in neurological deficits and neuroinflammation at day 7 after SAH compared with WT control mice. In conclusion, our study proves that the paravascular pathway dynamically mediates the pathological complications following acute SAH independently of glymphatic control. PMID:27031957

  16. The kidneys and ANCA-associated vasculitis: from pathogenesis to diagnosis

    PubMed Central

    Rowaiye, Olumide Olatubosun; Kusztal, Mariusz; Klinger, Marian

    2015-01-01

    Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are a group of pauci-immune small vessel vasculitides that often affect the kidneys manifesting as rapidly progressive glomerulonephritis. Although the exact pathogenesis of AAV is not fully known, evidence from in vitro, in vivo and clinical studies all point to the involvement of ANCA in the pathogenesis of AAV. In this review, we highlight the contributory roles played by various factors (e.g. genetics, environment, B and T-regulatory cells, toll-like receptors, etc.) in the pathogenesis of AAV. Furthermore, we discuss renal involvement in AAV in terms of clinical features and the various histopathological classification patterns, which are also known to be of prognostic importance. We also present information on useful imaging techniques for localizing kidney and other organ system involvement in AAV, and also on novel laboratory methods and assays useful for rapid and more specific determination of patients' ANCA status. Finally, we demonstrate evidence on novel serum biomarkers that have been shown to correlate with disease activity in AAV. PMID:26034600

  17. Using Mass Spectrometry to Quantify Rituximab and Perform Individualized Immunoglobulin Phenotyping in ANCA-Associated Vasculitis.

    PubMed

    Mills, John R; Cornec, Divi; Dasari, Surendra; Ladwig, Paula M; Hummel, Amber M; Cheu, Melissa; Murray, David L; Willrich, Maria A; Snyder, Melissa R; Hoffman, Gary S; Kallenberg, Cees G M; Langford, Carol A; Merkel, Peter A; Monach, Paul A; Seo, Philip; Spiera, Robert F; St Clair, E William; Stone, John H; Specks, Ulrich; Barnidge, David R

    2016-06-21

    Therapeutic monoclonal immunoglobulins (mAbs) are used to treat patients with a wide range of disorders including autoimmune diseases. As pharmaceutical companies bring more fully humanized therapeutic mAb drugs to the healthcare market analytical platforms that perform therapeutic drug monitoring (TDM) without relying on mAb specific reagents will be needed. In this study we demonstrate that liquid-chromatography-mass spectrometry (LC-MS) can be used to perform TDM of mAbs in the same manner as smaller nonbiologic drugs. The assay uses commercially available reagents combined with heavy and light chain disulfide bond reduction followed by light chain analysis by microflow-LC-electrospray ionization-quadrupole-time-of-flight mass spectrometry (ESI-Q-TOF MS). Quantification is performed using the peak areas from multiply charged mAb light chain ions using an in-house developed software package developed for TDM of mAbs. The data presented here demonstrate the ability of an LC-MS assay to quantify a therapeutic mAb in a large cohort of patients in a clinical trial. The ability to quantify any mAb in serum via the reduced light chain without the need for reagents specific for each mAb demonstrates the unique capabilities of LC-MS. This fact, coupled with the ability to phenotype a patient's polyclonal repertoire in the same analysis further shows the potential of this approach to mAb analysis. PMID:27228216

  18. Circulating Angiopoietin-2 as a Biomarker in ANCA-Associated Vasculitis

    PubMed Central

    Monach, Paul A.; Kümpers, Philipp; Lukasz, Alexander; Tomasson, Gunnar; Specks, Ulrich; Stone, John H.; Cuthbertson, David; Krischer, Jeffrey; Carette, Simon; Ding, Linna; Hoffman, Gary S.; Iklé, David; Kallenberg, Cees G. M.; Khalidi, Nader A.; Langford, Carol A.; Seo, Philip; St. Clair, E. William; Spiera, Robert; Tchao, Nadia; Ytterberg, Steven R.; Haubitz, Marion; Merkel, Peter A.

    2012-01-01

    The endothelial-specific Angiopoietin-Tie2 ligand-receptor system is an important regulator of endothelial activation. Binding of angiopoietin-2 (Ang-2) to Tie2 receptor renders the endothelial barrier responsive to pro-inflammatory cytokines. We previously showed that circulating Ang-2 correlated with disease severity in a small cohort of critically ill patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis. The current study reassessed Ang-2 as a biomarker of disease activity and relapse in AAV. Circulating Ang-2 was measured in 162 patients with severe AAV (BVAS/WG≥3, with or without glomerulonephritis) in a clinical trial. Ang-2 levels during active AAV were compared to levels in the same patients during remission (BVAS/WG = 0). Levels in clinical subsets of AAV were compared, and association with future disease course was assessed. Ang-2 levels were elevated in severe disease (median 3.0 ng/ml, interquartile range 1.9–4.4) compared to healthy controls (1.2, 0.9–1.5). However, they did not reliably decline with successful treatment (median 2.6 ng/ml, interquartile range 1.9–3.8, median change −0.1). Ang-2 correlated weakly with BVAS/WG score (r = 0.17), moderately with markers of systemic inflammation (r = 0.25–0.41), and inversely with renal function (r = −0.36). Levels were higher in patients with glomerulonephritis, but levels adjusted for renal dysfunction were no different in patients with or without glomerulonephritis. Levels were higher in patients with newly diagnosed AAV and lower in patients in whom treatment had recently been started. Ang-2 levels during active disease did not predict response to treatment, and Ang-2 levels in remission did not predict time to flare. Thus, Ang-2 appears to have limited practical value in AAV as a biomarker of disease activity at time of measurement or for predicting future activity. PMID:22279570

  19. Skin Conditions during Pregnancy

    MedlinePlus

    ... during pregnancy? • What is pruritic urticarial papules and plaques of pregnancy (PUPPP)? • What is prurigo of pregnancy? • ... itchy skin. What is pruritic urticarial papules and plaques of pregnancy (PUPPP)? In this condition, small, red ...

  20. Increased serum HMGB1 levels in patients with Henoch-Schönlein purpura.

    PubMed

    Chen, Tao; Guo, Zai-Pei; Wang, Wen-Ju; Qin, Sha; Cao, Na; Li, Meng-Meng

    2014-06-01

    High-mobility group box-1 (HMGB1) has been implicated as a pro-inflammatory cytokine in the pathogenesis of various inflammatory and autoimmune diseases. However, information about HMGB1 in Henoch-Schönlein purpura (HSP) is still unclear. Herein, we investigated the role of HMGB1 in patients with HSP and the pro-inflammatory effects of HMGB1 on human dermal microvascular endothelial cell line (HMEC-1). Serum HMGB1 levels in patients with HSP together with patients with allergic vasculitis (AV) and urticarial vasculitis (UV) were detected by enzyme-linked immunosorbent assay (ELISA). HMEC-1 cells were treated with HMGB1 at concentrations ranging from 4 ng/ml to 100 ng/ml. Serum HMGB1 levels were significantly increased in patients with HSP, AV and UV, when compared with those in control group. Moreover, abundant cytoplasmic expression of HMGB1 was observed in endothelial cells in lesional skin of HSP patients. Using membrane cytokine antibody array, we indicate that HMGB1 markedly induced TNF-α and IL-6 release in cultured supernatant. Furthermore, by real-time quantitative PCR and ELISA, the effects of HMGB1 on these cytokines production in HMEC-1 cells were established. Finally, Western blot data revealed that HMGB1 can induce phosphorylation of inhibitor of κB-α (IκBα) and the nuclear translocation of nuclear factor-κB (NF-κB) p65 in HMEC-1 cells. In conclusion, this study provides first observations on the association of HMGB1 with HSP. We suggest that HMGB1 may be an important mediator of endothelial inflammation through the induction of TNF-α and IL-6 production and may play a crucial role in the pathogenesis of HSP. PMID:24758390

  1. Autoimmunity-related neutrophilic dermatosis: a newly described entity that is not exclusive of systemic lupus erythematosus.

    PubMed

    Saeb-Lima, Marcela; Charli-Joseph, Yann; Rodríguez-Acosta, Elva Dalia; Domínguez-Cherit, Judith

    2013-08-01

    Neutrophilic dermatoses have long been known to be associated with autoinmune systemic diseases. Recently, a small number of cases of a disorder distinct from Sweet syndrome or bullous lupus erythematosus (LE) have been described as specifically related to systemic LE under diverse terms, including nonbullous neutrophilic dermatosis, nonbullous neutrophilic LE, and Sweet-like neutrophilic dermatosis. We describe 7 patients that developed urticarial lesions in the context of a known or concurrently diagnosed autoimmune connective tissue disease. Of a total of 7 patients, 6 were afflicted by systemic LE and 1 by rheumatoid arthritis and secondary Sjögren syndrome. Histological findings in all patients included an interstitial and perivascular neutrophilic infiltrate with leukocytoclasia, vacuolar alteration along the dermal-edidermal junction, and no vasculitis. Most patients had active systemic disease at the time of the cutaneous eruption. Skin lesions resolved rapidly after the administration of immunomodulating agents. In conclusion, we provide additional evidence of the existence of a recently defined nonbullous neutrophilic dermatosis in the context of autoimmune connective tissue diseases and propose the term autoimmunity-related neutrophilic dermatosis as an appropriate designation. Furthermore, we believe that this entity should prompt physicians to screen the presence of an active systemic disorder in afflicted patients. PMID:23518639

  2. When your patients are itching to see you: not all hives are urticaria.

    PubMed

    Weldon, David

    2005-01-01

    When patients present with itching and the perception that they have hives, what other processes can mimic urticaria? With the exception of urticarial vasculitis, urticaria typically lasts less than 24 to 36 hours at one site. A rash that persists longer should raise the suspicion of another inflammatory process. When the hive-like rash does not respond to antihistamines, a biopsy may reveal an alternative diagnosis. All biopsies should also be submitted for immunofluorescence to exclude atypical presentations of inflammatory bullous disease presenting with urticaria. However, even biopsies can be subject to misinterpretation and if the clinical picture does not support the biopsy, an alternative consultation with a dermatopathologist may be required. The extent of the laboratory and radiologic evaluation should be dictated by the clinician's suspicion of alternative causes for the hive(s) because rarely malignancies may present with urticaria. Common things are indeed common with urticaria and the more urticaria does not appear to be typical, the more often the clinician should consider alternative diagnoses. PMID:15813281

  3. Skin involvement and outcome measures in systemic autoimmune diseases.

    PubMed

    Albrecht, J; Atzeni, F; Baldini, C; Bombardieri, S; Dalakas, M C; Demirkesen, C; Yazici, H; Mat, C; Werth, V P; Sarzi-Puttini, P

    2006-01-01

    This paper focuses on skin manifestations that can be observed in autoimmune diseases such as rheumatoid arthritis (RA), Sjögren syndrome (SS), dermatomyositis (DM) and Behçet syndrome (BS). In RA the most widely recognized skin lesion is the rheumatoid nodule. Other cutaneous manifestations can be observed either non-specific or related to the disease itself and/or to the commonly used drugs. Cutaneous manifestations are considered one of the most typical extraglandular features of primary SS, generally they are distinguished in vasculitic and non vasculitic lesions. Among non-vasculitc lesions, skin dryness (xerosis) has been shown to be very common in pSS while vasculitis lesions include typically flat and palpable purpura and urticarial vasculits. In DM the skin manifestations are also frequent and include a heliotrope rash (blue-purple discoloration) on the upper eyelids with edema, a flat red rash on the face and upper trunk, and erythema of the knuckles with a raised violaceous scaly eruption (Gottron rash). The most frequent mucocutaneous finding in BS is aphthous stomatitis which can not usually be differentiated from idiopatic reccurrent aphthous stomatitis on clinical grounds. The most typical skin manifestations are nodular lesions, which are commonly seen in BS and may be due to panniculitis [erythema nodosum (EN)-like lesions] or superficial thrombophlebitis. PMID:16466625

  4. Chronic urticaria: pathophysiology and etiology, or the what and why.

    PubMed

    Schocket, Alan L

    2006-01-01

    Chronic urticaria (CU) is a relatively common but vexing disease. The pathophysiology is based on the cutaneous mast cell release of mediators, predominantly histamine. Release can be induced via specific immunoglobulin E (IgE), components of complement activation and nonspecifically by various compounds including endogenous peptides, endorphins, and enkephalins. In >30% of CU patients, autoimmune phenomena have been found, characterized by positive autologous serum skin test, antibodies to the alpha-subunit of the basophil IgE receptor, to IgE itself, and, perhaps, the most clinically relevant, thyroid autoimmunity. Studies suggest that the products of the activated immune system can lower the cutaneous mast cell release threshold, possibly allowing activation by endogenous compounds. The resulting release of mediators produces the clinical picture of recurrent hives. Although the goal of management of CU is the identification of a treatable cause, in most CU patients, especially adults, a cause is not frequently found. Identified causes include drugs, foods, infections, immune complex production leading to urticarial vasculitis, autoantibody production, and underlying autoimmune disease, particularly autoimmune thyroiditis. The treatment of the thyroiditis with suppressive doses of thyroid hormone often results in the remission of the CU. Given the marginally effective and sometimes dangerous medical therapy available for CU, a systematic and thorough approach to identify a treatable cause in difficult CU patients is warranted. PMID:16724623

  5. East coast fever caused by Theileria parva is characterized by macrophage activation associated with vasculitis and respiratory failure

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Respiratory failure and death in East Coast Fever (ECF), a clinical syndrome of African cattle caused by the apicomplexan parasite Theileria parva, has historically been attributed to pulmonary infiltration by infected lymphocytes. However, immunohistochemical staining of tissue from T. parva infect...

  6. Intestinal Behçet's Disease: A True Inflammatory Bowel Disease or Merely an Intestinal Complication of Systemic Vasculitis?

    PubMed Central

    Kim, Duk Hwan

    2016-01-01

    Behçet's disease (BD) is a multi-systemic inflammatory disorder of an unknown etiology and shows a chronic recurrent clinical course. When the disease involves the alimentary tract, it is called intestinal BD because of its clinical importance. Intestinal BD is more frequently reported in East Asian countries than in Western or Middle Eastern countries. While any part of the gastrointestinal tract can be involved, the most common location of intestinal BD is the ileocecal area. A few, large, deep ulcerations with discrete border are characteristic endoscopic findings of intestinal BD. Currently, there is no single gold standard test or pathognomonic finding of intestinal BD. However, recently developed novel diagnostic criteria and a disease activity index have helped in assessing intestinal BD. As intestinal BD shares a lot of characteristics with inflammatory bowel disease, including genetic background, clinical manifestations, and therapeutic strategies, distinguishing between the two diseases in clinical practice is quite difficult. However, biologic agents such as anti-tumor necrosis factor α antibody shows a considerable efficacy similar to inflammatory bowel disease cases. It is important to distinguish and treat those two disease entities separately from the standpoint of precise medicine. Clinicians should require comprehensive knowledge regarding the similarities and differences between intestinal BD and inflammatory bowel disease for making an accurate clinical decision. PMID:26632379

  7. The practical usefulness of dermoscopy in general dermatology.

    PubMed

    Errichetti, E; Stinco, G

    2015-10-01

    Beside to traditional use, dermoscopy is more and more used in the assessment of other "general" dermatologic conditions, namely scalp and hair disorders (trichoscopy), nails abnormalities (onychoscopy), skin infections and infestations (entomodermoscopy), and cutaneous inflammatory diseases (inflammoscopy). Among the list of new applications of dermoscopy, the study of inflammatory dermatoses is probably the most promising topic in terms of development and usefulness, considering the large number of such disorders and the frequent problems in their differential diagnosis which the dermatologist encounters in own daily clinical practice. In this paper, we report selected relatively common clinical differential diagnosis issues concerning inflammatory dermatoses (and some clinically related noninflammatory conditions), analysing them by a dermoscopic point of view in order to assist their noninvasive resolution according to the available literature data and our personal experience, including papulosquamous dermatoses (guttate psoriasis, pityriasis lichenoides chronica, pityriasis rosea, lichen planus, lymphomatoid papulosis, classic pityriasis rubra pilaris, papulosquamous sarcoidosis, disseminated forms of porokeratosis and papulosquamous chronic GVHD), dermatoses presenting with erythematous-desquamative patches/plaques (plaque psoriasis, eczematous dermatitis, pityriasis rosea, mycosis fungoides, subacute cutaneous lupus erythematosus), palmar psoriasis vs. chronic hand eczema, scalp psoriasis vs. seborrheic dermatitis, erythematous-desquamative disorders typically involving the elbows (psoriasis vulgaris, circumscribed juvenile pityriasis rubra pilaris, dermatomyositis/Gottron's sign), itchy papulonodular dermatoses (hypertrophic lichen planus, prurigo nodularis, nodular scabies and acquired perforating dermatosis), common facial inflammatory skin diseases (rosacea, seborrheic dermatitis and demodicidosis), lichen sclerosus vs. morphea, urticaria vs. urticarial

  8. Vasculitides and the Complement System: a Comprehensive Review.

    PubMed

    Chimenti, Maria Sole; Ballanti, Eleonora; Triggianese, Paola; Perricone, Roberto

    2015-12-01

    Systemic vasculitides are a group of rare diseases characterized by inflammation of the arterial or venous vessel wall, causing stenosis or thrombosis. Clinical symptoms may be limited to skin or to other organs or may include multiple manifestations as systemic conditions. The pathogenesis is related to the presence of leukocytes in the vessels and to the IC deposition, which implies the activation of the complement system (CS) and then the swelling and damage of vessel mural structures. The complement system (CS) is involved in the pathogenesis of several autoimmune diseases, including systemic vasculitides. This enzymatic system is a part of the innate immune system, and its function is linked to the modulation of the adaptive immunity and in bridging innate and adaptive responses. Its activation is also critical for the development of natural antibodies and T cell response and for the regulation of autoreactive B cells. Complement triggering contributes to inflammation-driven tissue injury, which occurs during the ischemia/reperfusion processes, vasculitides, nephritis, arthritis, and many others diseases. In systemic vasculitides, a group of uncommon diseases characterized by blood vessel inflammation, the contribution of CS in the development of inflammatory damage has been demonstrated. Treatment is mainly based on clinical manifestations and severity of organ involvement. Evidences on the efficacy of traditional immunosuppressive therapies have been collected as well as data from clinical trials that involve the modulation of the CS. In particular in small-medium-vessel vasculitides, the CS represents an attractive target. Herein, we reviewed the pathogenetic role of CS in these systemic vasculitides as urticarial vasculitis, ANCA-associated vasculitides, anti-glomerular basement membrane disease, cryoglobulinaemic vasculitides, Henoch-Schönlein purpura/IgA nephropathy, and Kawasaki disease and therefore its potential therapeutic use in this context. PMID

  9. Methotrexate: a useful steroid-sparing agent in recalcitrant chronic urticaria.

    PubMed

    Perez, A; Woods, A; Grattan, C E H

    2010-01-01

    Background Reports of methotrexate for chronic urticaria are anecdotal. Objectives To assess the effectiveness of methotrexate in steroid-dependent chronic urticaria, its impact on steroid reduction and any differences in response between patients with and without functional autoantibodies. Methods A retrospective case-note review of 16 patients with steroid-dependent chronic urticaria treated with methotrexate was carried out. Ten patients had chronic ordinary/spontaneous urticaria (CU), including three with associated delayed-pressure urticaria; four patients had normocomplementaemic urticarial vasculitis (UV); and two patients had idiopathic angio-oedema without weals. Median disease duration before methotrexate was 48.5 months (range 12-164). All were unresponsive to antihistamines and second-line agents, except prednisolone. Eleven were assessed for autoimmune urticaria with the basophil histamine release assay (n = 5), autologous serum skin test (n = 5) or both (n = 1). Response to methotrexate was scored: no benefit; some benefit (fewer weals and symptomatic improvement but no steroid reduction); considerable benefit (improvement with steroid reduction); or clear (no symptoms, off steroids but on antihistamines). Results Twelve of 16 patients (eight CU, three UV, one idiopathic angio-oedema) responded. Three showed some benefit, seven considerable benefit and two cleared. Four of eight responders and three out of three nonresponders showed evidence of functional autoantibodies. The dose to achieve a steroid-sparing effect was 10-15 mg weekly (cumulative dose range 15-600 mg, median 135 mg). Methotrexate was well tolerated. Conclusions Methotrexate may be a useful treatment for steroid-dependent chronic urticaria. Functional autoantibodies do not correlate with response. The beneficial effects of methotrexate may be anti-inflammatory and immunosuppressive. It may therefore benefit chronic urticaria independently of the pathogenic mechanism, whether autoimmune

  10. Angioimmunoblastic T-cell lymphoma: clinical and laboratory features at diagnosis in 77 patients.

    PubMed

    Lachenal, Florence; Berger, Francoise; Ghesquières, Hervé; Biron, Pierre; Hot, Arnaud; Callet-Bauchu, Evelyne; Chassagne, Catherine; Coiffier, Bertrand; Durieu, Isabelle; Rousset, Hugues; Salles, Gilles

    2007-09-01

    We retrospectively analyzed 77 patients with pathologically diagnosed angioimmunoblastic T-cell lymphoma from a single city. There were 43 men and 34 women; the median age was 64.5 years (range, 30-91 yr). Average time between first symptoms of the disease and diagnosis was 3.6 months. At diagnosis, peripheral nodes were present in all but 1 patient, and were generalized in 90% of cases. Constitutional symptoms were reported in 77% of cases and spleen enlargement in 51%. A cutaneous eruption--morbilliform, urticarial, or more polymorphic--was present in 45% of patients; in one-third of them, the eruption occurred after drug administration. Other clinical manifestations included pleuritis (22%); arthralgia or arthritis (17%); ear, nose, and throat involvement (14%); central or peripheral neurologic manifestations (10%); and ascites (5%). Most patients presented with advanced disease at diagnosis (bone marrow involvement in 60% of cases). The main laboratory abnormalities were elevated lactate dehydrogenase levels (71%), inflammatory syndrome (67%), hypergammaglobulinemia (50%), anemia (51%), and lymphopenia (52%). Auto- or disimmune manifestations were reported in one-third of patients: autoimmune hemolytic anemia was present at diagnosis in 19% of patients and thrombocytopenic purpura in 7%. Documented vasculitis was described in 12% of cases. Clonality was analyzed in lymph nodes in 47 patients: T-cell and B-cell clones were found in 45 (96%) and 20 (45%) patients, respectively. Chromosomal abnormalities were identified in 62% of cases: trisomies 3, 5, 18, 19, additional X chromosome, and deletion of chromosome 7 were the most common abnormalities. The current study underlines the diversity of presenting manifestations of angioimmunoblastic T-cell lymphoma. PMID:17873758

  11. High Basal Activity of the PTPN22 Gain-of-Function Variant Blunts Leukocyte Responsiveness Negatively Affecting IL-10 Production in ANCA Vasculitis

    PubMed Central

    Cao, Yali; Yang, Jiajin; Colby, Kerry; Hogan, Susan L.; Hu, Yichun; Jennette, Caroline E.; Berg, Elisabeth A.; Zhang, Youkang; Jennette, J. Charles; Falk, Ronald J.; Preston, Gloria A.

    2012-01-01

    Consequences of expression of the protein tyrosine phosphatase nonreceptor 22 (PTPN22) gain-of-function variant were evaluated in leukocytes from patients with anti-neutrophil cytoplasmic autoantibody (ANCA) disease. The frequency of the gain-of-function allele within the Caucasian patient cohort was 22% (OR 1.45), compared to general American Caucasian population (16.5%, p = 0.03). Examination of the basal phosphatase activity of PTPN22 gain-of-function protein indicated persistently elevated activity in un-stimulated peripheral leukocytes, while basal activity was undetectable in leukocytes from patients without the gain-of-function variant. To examine consequences of persistently high PTPN22 activity, the activation status of ERK and p38 MAPK were analyzed. While moderate levels of activated ERK were observed in controls, it was undetectable in leukocytes expressing PTPN22 gain-of-function protein and instead p38MAPK was up-regulated. IL-10 transcription, reliant on the ERK pathway, was negatively affected. Over the course of disease, patients expressing variant PTPN22 did not show a spike in IL-10 transcription as they entered remission in contrast to controls, implying that environmentally triggered signals were blunted. Sustained activity of PTPN22, due to the gain-of-function mutation, acts as a dominant negative regulator of ERK activity leading to blunted cellular responsiveness to environmental stimuli and expression of protective cytokines. PMID:22880107

  12. Cutaneous lesions associated with coronavirus-induced vasculitis in a cat with feline infectious peritonitis and concurrent feline immunodeficiency virus infection.

    PubMed

    Cannon, Martha J; Silkstone, Malcolm A; Kipar, Anja M

    2005-08-01

    This report describes a clinical case of feline infectious peritonitis (FIP) with multisystemic involvement, including multiple nodular cutaneous lesions, in a cat that was co-infected with feline coronavirus and feline immunodeficiency virus. The skin lesions were caused by a pyogranulomatous-necrotising dermal phlebitis and periphlebitis. Immunohistology demonstrated the presence of coronavirus antigen in macrophages within these lesions. The pathogenesis of FIP involves a viral associated, disseminated phlebitis and periphlebitis which can arise at many sites. Target organs frequently include the eyes, abdominal organs, pleural and peritoneal membranes, and central nervous tissues, but cutaneous lesions have not previously been reported. PMID:16055009

  13. Sensitivity to non-acetylated salicylates in a patient with asthma, nasal polyps, and rheumatoid arthritis.

    PubMed

    Chudwin, D S; Strub, M; Golden, H E; Frey, C; Richmond, G W; Luskin, A T

    1986-08-01

    A woman experienced exacerbations of bronchial asthma after taking aspirin and other non-steroidal anti-inflammatory drugs (NSAIDs) for rheumatoid arthritis. On oral challenges, she developed an urticarial reaction after tartrazine; urticarial and bronchospastic reactions after salicylsalicylic acid; and urticarial and bronchospastic reactions after choline magnesium trisalicylate. Non-acetylated salicylates have been recommended for use in aspirin- and/or tartrazine-sensitive patients. The results of sensitivity studies of our patient indicates that such patients may also be sensitive to non-acetylated salicylates. PMID:3740556

  14. Low fasting serum triglyceride level as a precocious marker of autoimmune disorders.

    PubMed

    Iannello, Silvia; Cavaleri, Antonina; Milazzo, Paolina; Cantarella, Santi; Belfiore, Francesco

    2003-08-01

    The authors recently reported the occurrence of low fasting serum triglyceride (TG) and high free fatty acid (FFA) levels in idiopathic pulmonary fibrosis. TG estimation in diverse groups of patients with autoimmune disease or hyperactive immune response confirmed the occurrence of a similar decrease of TG. In some patients, serum FFA level was also evaluated. TG value in lean and obese patients was compared with that in lean (n = 108) and obese (n = 208) control subjects without autoimmune disease. In patients affected by autoimmune chronic thyroiditis with enhanced concentration of antithyroglobulin antibodies and without thyroidal failure (n = 24), lean and obese patients had reduced TG (-69/%, P < .01 and -52%, P < .0001, respectively). Both lean and obese patients affected by chronic active B or C hepatitis (n = 26), with autoantibodies and without signs of hepatic insufficiency or cirrhosis, presented reduced TG (-57%, P < .01 and -61%, P < .001, respectively). A marked TG decrease (-73%, P < .001) was observed in the lean patients affected by lupus-like syndrome (n = 7). The lean and obese patients with systemic lupus erythematosus or rheumatoid arthritis (n = 11) showed TG decrease (-66%, P < .01 and -55%, P < .05, respectively). In patients affected by anamnestic allergy or atopic dermatitis/asthma (n = 66), both lean and obese, TGs were reduced (-67%, P < .0001 and -62%, P < .001, respectively). In isolated cases of diverse autoimmune diseases (scleroderma, APECED [autoimmune polyendocrinopathy, candidiasis, and ectodermal dystrophy], urticaria or urticarial vasculitis, Reiter or Sjogren syndromes, ulcerative colitis or Crohn's disease, multiple sclerosis or Guillain-Barré syndrome) (n = 14), decreased TG was also observed both in the lean and obese subjects (-59%, P < .01 and -57%, P < .01, respectively). Concerning FFA (n = 69), value in lean patients (n = 22) vs that in lean controls (n = 18) was increased (520 +/- 31 vs 299 +/- 30 mcEq/L, +74%, P

  15. C1q and systemic lupus erythematosus.

    PubMed

    Walport, M J; Davies, K A; Botto, M

    1998-08-01

    In this chapter we review the association between SLE and C1q. In the first part of the chapter we discuss the clinical associations of C1q deficiency, and tabulate the available information in the literature relating to C1q deficiency and autoimmune disease. Other clinical associations of C1q deficiency are then considered, and we mention briefly the association between other genetically determined complement deficiencies and lupus. In the review we explore the relationship between C1q consumption and lupus and we discuss the occurrence of low molecular weight (7S) C1q in lupus, which raises the possibility that increased C1q turnover in the disease may result in unbalanced chain synthesis of the molecule. Anti-C1q antibodies are also strongly associated with severe SLE affecting the kidney, and with hypocomplementaemic urticarial vasculitis, and these associations are also examined. We address the question of how C1q deficiency may cause SLE, discussing the possibility that this may be due to abnormalities of immune complex processing, which have been well characterised in a umber of different human models. There is clear evidence that immune complex processing is abnormal in patients with hypocomplementaemia, and this is compatible with the hypothesis that ineffective immune complex clearance could cause tissue injury, and this may in turn stimulate an autoantibody response. We have also considered the possibility that C1q-C1q receptor interactions are critical in the regulation of apoptosis, and we explore the hypothesis that dysregulation of apoptosis could explain important features in the development of autoimmune disease associated with C1q deficiency. An abnormally high rate of apoptosis, or defective clearance of apoptotic cells, could promote the accumulation of abnormal cellular products that might drive an autoimmune response. Anti-C1q antibodies have been described in a number of murine models of lupus, and these are also briefly discussed. We focus

  16. Pathogenesis of chronic urticaria.

    PubMed

    Kaplan, A P; Greaves, M

    2009-06-01

    Chronic urticaria is defined as the presence of urticaria (hives) for at least 6 weeks with the assumption that it occurs daily or close to it. If we eliminate physical urticarias and urticarial vasculitis from consideration, the remainder can be divided into autoimmune chronic urticaria (45%) and idiopathic chronic urticaria (55%). The autoimmune subgroup is associated with the IgG anti-IgE receptor alpha subunit in 35-40% of patients and IgG anti-IgE in an additional 5-10%. These autoantibodies have been shown to activate blood basophils and cutaneous mast cells in vitro with augmentation of basophil activation by complement and release of C5a, in particular. Binding methods (immunoblot and ELISA) yield positives in many autoimmune diseases as well as occasional normal subjects or patients with other forms of urticaria but most such sera are non-functional. Activation of basophils or mast cells causing histamine release is quite specific for chronic urticaria and defines the autoimmune subgroup. Although pathogenicity is not formally proven, the antibodies cause wealing upon intradermal injection, and removal of the autoantibody leads to remission. A cellular infiltrate is seen to be characterized by mast cell degranulation and infiltration of CD4+ T lymphocytes, monocytes, neutrophils, eosinophils, and basophils. The intensity of the infiltrate and clinical severity of the disease (including accompanying angio-oedema) is more severe in the autoimmune subpopulation. This latter group also has a higher evidence of human leucocyte antigen DR alleles associated with autoimmunity and a 25% incidence of antithyroid antibodies with diagnosed hypothyroidism in some. Hypo-responsiveness of patients' basophils to anti-IgE and hyperresponsiveness to serum defines another subpopulation (at least 50%) that overlaps the idiopathic and autoimmune subgroups. Hypo-responsiveness to anti-IgE has been shown to be associated with elevated levels of cytoplasmic phosphatases that

  17. Localized heat induced urticaria: report of a case.

    PubMed

    Darling, Matthew; Lambiase, Matthew C; Hodson, Darryl S

    2004-01-01

    Localized heat induced urticaria is a rare clinical entity. Other physical urticarial subtypes include cholinergic, solar, cold, aquagenic, vibratory, and dermatographic. It is characterized by a well-demarcated urticarial lesion provoked by heat in direct contact with the skin. We describe a case of localized heat-induced urticaria in a 49-year-old woman after a heat-challenge test to her forearm. PMID:14964751

  18. IgA nephropathy

    MedlinePlus

    ... family history of IgA nephropathy or Henoch Schonlein purpura , a form of vasculitis that affects many parts ... End-stage kidney disease Hypersensitivity vasculitis Nephrotic syndrome Purpura Urine - bloody Update Date 9/22/2015 Updated ...

  19. ESR

    MedlinePlus

    ... due to: Anemia Cancers such as lymphoma or multiple myeloma Kidney disease Pregnancy Thyroid disease The immune system ... Heart failure - overview Hypersensitivity vasculitis Macroglobulinemia of Waldenstrom Multiple ... vasculitis Polycythemia - newborn Polycythemia vera Polymyalgia ...

  20. Granulomatosis with polyangiitis, a new nomenclature for Wegener's Granulomatosis - Case report*

    PubMed Central

    de Lima, Alexandre Moretti; Torraca, Pedro de Freitas Silva; da Rocha, Sheila Pereira; Santiago, Carmelia Matos Reis; Ferraz, Fabio Humberto Ribeiro Paes

    2015-01-01

    The granulomatosis with polyangiitis, initially known as Wegener's granulomatosis, is a small and medium vessels vasculitis. It's classic form presents a triad: necrotizing granuloma of respiratory tract, necrotizing cutaneous vasculitis and glomerulonephritis. This vasculitis has cytoplasmic antineutrophil antibodies as signal. This work illustrates a case, of multisystemic rare disease, in which the segment and treatment were considered satisfactory for symptoms remission. PMID:26312687

  1. [The pine processionary caterpillar Thaumetopoea pityocampa].

    PubMed

    Solt, Ido; Mendel, Zvi

    2002-09-01

    The pine processionary caterpillar Thaumetopoea pityocampa (Lepidoptera: Notodontidae) is considered to be a serious pest of medical importance. The hair on the dorsum of the last instar larvae of the moth may cause urticarial reactions (erucism) as well as eye problems and temporary blindness. In Israel, the pest occurs in all pine plantations as well as on ornamental pine trees in urban areas. The biology, ecology and management of the moth population are discussed as well as the mechanism of action of the urticarial hairs and their medical significance. Awareness of the life cycle and ecology of the pest may reduce the contact of the population with the urticarial hairs and prevent the morbidity caused by it. PMID:12362487

  2. Severe osteoporosis due to systemic mast cell disease: successful treatment with interferon alpha-2B.

    PubMed

    Lehmann, T; Beyeler, C; Lämmle, B; Hunziker, T; Vock, P; Olah, A J; Dahinden, C; Gerber, N J

    1996-09-01

    We describe a 33-year old man suffering from severe vertebral osteoporosis and urticaria pigmentosa due to systemic mast cell disease (SMCD). Because i.v. clodronate therapy could not prevent further vertebral fractures, an additional treatment with interferon alpha-2b was initiated. During 24 months of treatment, our patient had no further pain episodes, no new vertebral fractures were discovered, trabecular bone mineral density (BMD) increased significantly and urticarial symptoms improved. Nevertheless, the extent of skin lesions remained unchanged. On histological examination, a remarkable decrease of mast cells was observed in the bone marrow, but not in the skin. Five months after discontinuation of interferon alpha-2b, trabecular BMD decreased and urticarial symptoms deteriorated. These findings illustrate a beneficial effect of interferon alpha-2b on SMCD-induced osteoporosis as well as urticarial symptoms, and raise the question whether this treatment may have a diverse impact on mast cell population in different tissues. PMID:8810675

  3. [Cutaneous symptoms of various vasculitides].

    PubMed

    Sunderkötter, C; Pappelbaum, K I; Ehrchen, J

    2015-08-01

    The skin is one of the organs most frequently involved in vasculitides. Cutaneous vasculitis may present (1) part of a systemic vasculitis (e.g., IgA vasculitis), (2) a skin-restricted or skin-dominant variant of the corresponding systemic vasculitis without clinically apparent visceral involvement (e.g., cutaneous IgA vasculitis), or (3) a vasculitis occurring exclusively in the skin (e.g., erythema elevatun diutinum). The clinical symptoms of vasculitides are markedly determined by the size of the predominantly affected blood vessels. Systemic polyarteritis nodosa is regarded as a medium vessel vasculitis and is associated with multiple skin symptoms: (1) vasculitis of digital arteries with ensuing digital infarction, (2) livedo racemosa and subcutaneous nodules, and (3) in some patients even purpura and hemorrhagic macules due to additional small vessel vasculitis. In contrast, in its skin-restricted entity (i.e., cutaneous polyarteritis nodosa), the predominant symptoms are subcutaneous nodules surrounded by livedo racemosa, often on the lower legs. Among small-vessel vasculitides palpable purpura with predilection for the legs is a nearly pathognomonic feature of immune complex vasculitis. Variations in clinical symptoms indicate additional pathophysiological mechanisms or different vascultides: (1) ANCA-associated vasculitides often also entail nodules or sometimes livedo, (2) cryoglobulinemic vasculitis additionally may present with necrosis at cold exposed areas and involvement of vessels of various size, (3) small vessel vasculitis associated with systemic lupus erythematosus or rheumatoid arthritis shows predilection for additional sites (e.g., nailfolds) and also involvement of vessels beyond postcapillary venules, (4) recurrent macular vasculitis in hypergammaglobulinemia also occurs on dependent parts, but shows numerous small hemorrhagic macules instead of palpable purpura, (5) erythema elevatum diutinum begins with brightly red to violaceous plaques

  4. Pregnancy and Medically Assisted Conception in Rare Diseases

    ClinicalTrials.gov

    2016-06-23

    Rheumatoid Arthritis; Spondyloarthritis; Psoriatic Arthritis; Systemic Lupus Erythematosus; Antiphospholipid Syndrome; Sjogren Syndrome; Scleroderma; Myositis; Vasculitis; Mastocytosis; Various Autoimmune and/or Systemic and/or Rare Diseases

  5. Fingers that change color

    MedlinePlus

    ... conditions can cause fingers or toes to change color: Buerger disease Chilblains. Painful inflammation of small blood vessels. Cryoglobulinemia Frostbite Necrotizing vasculitis Peripheral artery disease ...

  6. Diagnosis and therapeutic options for peripheral vasculitic neuropathy.

    PubMed

    Blaes, Franz

    2015-04-01

    Vasculitis can affect the peripheral nervous system alone (nonsystemic vasculitic neuropathy) or can be a part of primary or secondary systemic vasculitis. In cases of pre-existing systemic vasculitis, the diagnosis can easily be made, whereas suspected vasculitic neuropathy as initial or only manifestation of vasculitis requires careful clinical, neurophysiological, laboratory and histopathological workout. The typical clinical syndrome is mononeuropathia multiplex or asymmetric neuropathy, but distal-symmetric neuropathy can frequently be seen. Standard treatments include steroids, azathioprine, methotrexate and cyclophosphamide. More recently the B-cell antibody rituximab and intravenous immunoglobulins have shown to be effective in some vasculitic neuropathy types. PMID:25829955

  7. Diagnosis and therapeutic options for peripheral vasculitic neuropathy

    PubMed Central

    2015-01-01

    Vasculitis can affect the peripheral nervous system alone (nonsystemic vasculitic neuropathy) or can be a part of primary or secondary systemic vasculitis. In cases of pre-existing systemic vasculitis, the diagnosis can easily be made, whereas suspected vasculitic neuropathy as initial or only manifestation of vasculitis requires careful clinical, neurophysiological, laboratory and histopathological workout. The typical clinical syndrome is mononeuropathia multiplex or asymmetric neuropathy, but distal-symmetric neuropathy can frequently be seen. Standard treatments include steroids, azathioprine, methotrexate and cyclophosphamide. More recently the B-cell antibody rituximab and intravenous immunoglobulins have shown to be effective in some vasculitic neuropathy types. PMID:25829955

  8. Generalized acquired cutis laxa type 1: a case report and brief review of literature.

    PubMed

    Kumar, Piyush; Savant, Sushil S; Das, Anupam

    2016-01-01

    Cutis laxa, clinically characterized by loose and pendulous skin related to loss of elastic tissue, is a rare heterogeneous condition. It is classified into congenital and acquired types. We report a case of generalized acquired cutis laxa type 1 in a young man following pruritic urticarial plaques. We have done a brief review of literature. PMID:27136630

  9. [Vasculitic urticaria: study of 12 cases].

    PubMed

    Hassan, M L; Perez Cejudo, J A; Yachi del Pino, E; Schroh, R G

    1990-01-01

    Twelve cases of chronic urticaria with histopathologic features of lecocitoclastic allergic angitis are studied. The type of cutaneous lesion, personal and familiar atopic history and the presence of autoimmune disease are described. Light microscopy, direct immunofluorescence, anti DNA, antinuclear, antithyroid, Ro, La, Rnp and Sm antibodies, total complement levels, C3 and C4, rheumatoid factor, latex, ASTO, cryoglobulines and complete workup were investigated, taking into account natural progression and response to therapy. Two different groups are defined: 1) normocomplementemic (5 patients) and 2) hypocomplementemic (7 patients). They were all women except one. The cutaneous lesions were indistinguishable in the two groups. Only in the second group there was an associated disease (systemic lupus erythematosus, Sjogren syndrome disease, lupus-Sjogren overlap, autoimmune thyroid disease). Urticaria had been present from the onset of the disease in 4 patients, and occurred later during its course in 8 others. Five patients had thyroid disease (Hashimoto thyroiditis or Graves disease), two of them being mother and daughter. Another patient had a family history of Grave's disease and urticaria. Anti DNA antibodies were found in 7 cases, and anti Ro + La + in 3 cases. Response to treatment was variable with spontaneous cycles of worsening and remissions. One of the patients found a relationship with certain foods. Histopathologic results are related in both clinical normocomplementemic and hypocomplementemic groups. No significant differences were found between the two groups, but Ro+ and La+ patients exhibit more intense cariorexis and neutrophilic infiltrates. PMID:2263094

  10. Cryoglobulinemia

    MedlinePlus

    ... the VF E-News HERE A A A Youtube LinkedIn Twitter Facebook Takayasu’s Arteritis Webinar + Vasculitis Behcet’s ... Book Vasculitis Terms A – Z VF Medical Consultants YouTube Education Videos YouTube Education Videos produced by Alliance ...

  11. Vasculitic neuropathy following exposure to minocycline

    PubMed Central

    Baratta, John M.; Dyck, P. James B.; Brand, Patricio; Thaisetthawatkul, Pariwat; Dyck, Peter J.; Engelstad, JaNean K.; Goodman, Brent

    2015-01-01

    Objective: To report 3 patients with minocycline-induced autoimmunity resulting in peripheral nerve vasculitis. Methods: We report 3 patients who, during minocycline treatment for acne vulgaris, developed subacute onset of pain and weakness caused by vasculitis in single and multiple mononeuropathy patterns. Results: Each patient underwent either a nerve or muscle biopsy that confirmed vasculitis. One patient additionally developed systemic symptoms (including fever, fatigue, and night sweats) and another had a posterior circulation stroke. Symptoms developed with either early or prolonged use of minocycline. Despite withdrawal of minocycline, patients needed long-term immunotherapy to gain neurologic improvement. Conclusions: Our findings suggest that the typical neuropathy associated with minocycline use is painful single or multiple mononeuropathy due to peripheral nerve vasculitis, which may also be accompanied by presumed CNS vasculitis (presenting as stroke). PMID:26601119

  12. Characterization and analysis of carbon fibre-reinforced polymer composite laminates with embedded circular vasculature

    PubMed Central

    Huang, C.-Y.; Trask, R. S.; Bond, I. P.

    2010-01-01

    A study of the influence of embedded circular hollow vascules on structural performance of a fibre-reinforced polymer (FRP) composite laminate is presented. Incorporating such vascules will lead to multi-functional composites by bestowing functions such as self-healing and active thermal management. However, the presence of off-axis vascules leads to localized disruption to the fibre architecture, i.e. resin-rich pockets, which are regarded as internal defects and may cause stress concentrations within the structure. Engineering approaches for creating these simple vascule geometries in conventional FRP laminates are proposed and demonstrated. This study includes development of a manufacturing method for forming vascules, microscopic characterization of their effect on the laminate, finite element (FE) analysis of crack initiation and failure under load, and validation of the FE results via mechanical testing observed using high-speed photography. The failure behaviour predicted by FE modelling is in good agreement with experimental results. The reduction in compressive strength owing to the embedding of circular vascules ranges from 13 to 70 per cent, which correlates with vascule dimension. PMID:20150337

  13. Hydralazine-induced pauci-immune glomerulonephritis: intriguing case series with misleading diagnoses

    PubMed Central

    Babar, Faizan; Posner, Jeffery N.; Obah, Eugene A.

    2016-01-01

    Hydralazine has been used since the 1950s for the management of hypertension. Evidence for hydralazine-associated vasculitis dates to pre-ANCA (antineutrophil cytoplasmic antibodies) era. This abstract describes two cases of ANCA-positive pauci-immune glomerulonephritis (GN) in challenging scenarios where diagnosis was misconstrued. A comprehensive literature review was done to understand the pathogenesis of drug-induced pauci-immune GN. We have described key diagnostic features that are helpful in distinguishing idiopathic ANCA vasculitis from drug-induced vasculitis. Additionally, we have also described different treatments meant to provide therapy options with the least side effects. PMID:27124161

  14. Erythema elevatum diutinum in association with IgA monoclonal gammopathy: A rare case report

    PubMed Central

    Patnala, Guru Prasad; Sunandini, Anila P.; Rayavarapu, Rama; Yandapalli, Padmasri Somala

    2016-01-01

    Erythema elevatum diutinum (EED) is a rare form of vasculitis characterized clinically by red-violet brown papules, plaques, and nodules mainly involving the extensor surfaces; histologically by leukocytoclastic vasculitis in early lesions, and fibrosis and cholesterolosis in late lesions. EED has been associated with many systemic disorders including infections, autoimmune disorders, and both benign and malignant hematological disorders. As it is a rare form of vasculitis and only 250 cases reported till date, we report a case of EED in association with IgA monoclonal gammopathy with partial response to dapsone treatment. PMID:27559509

  15. Severe dermographism after topical therapy with diphenylcyclopropenone for alopecia universalis.

    PubMed

    Skrebova, N; Nameda, Y; Takiwaki, H; Arase, S

    2000-04-01

    We describe here a 19-year-old Japanese man with an 11-year history of alopecia universalis, who, after the 1st application of a 0.003% diphenylcyclopropenone (DPCP) solution to the whole scalp, developed acute contact dermatitis at the test site, together with widespread severe dermographism. Every 3 weeks, persistence of the severe urticarial reaction and efficacy of treatment were monitored by constant pressure stimuli in a series of pressure tests, and subsequently evaluated by laser Doppler flowmetry (LDF). Although, on pressure tests, the urticarial response was found to significantly improve after starting treatment, erythematous responses continued to appear for nearly 3 months. The persistent course of these side-effects in our patient strongly suggests that precautions must currently be taken in the therapeutic use of potent sensitizers such as DPCP. PMID:10750852

  16. Contact urticaria from rice.

    PubMed

    Yamakawa, Y; Ohsuna, H; Aihara, M; Tsubaki, K; Ikezawa, Z

    2001-02-01

    A 30-year-old man with atopic dermatitis had had erythema and itching of the hands after washing rice in water, though he had always eaten cooked rice without problems. Handling test with water used to wash regular rice was performed on abraded hands, and produced urticarial erythema after several minutes. Applications of water used to wash allergen-reduced rice were negative for urticarial reaction. Prick test with water used to wash regular rice was +++. However prick test reaction with water used to wash allergen-reduced rice was +. Histamine-release test of regular rice-washing water was grade 3 and that of allergen-reduced rice grade 1. In immunoblotting analysis with regular rice washing water, there were no bands with this patient. These results suggest that the allergen responsible for contact urticaria in this patient might be water-soluble, heat-unstable, and not contained in allergen-reduced rice. PMID:11205411

  17. Caterpillar dermatitis.

    PubMed

    Dunlop, K; Freeman, S

    1997-11-01

    A 3-year-old girl presented with recurrent urticarial eruptions presumed due to infestation of her garden with Euproctis edwardsi, Euproctis edwardsi, the mistletoe browntail moth is a variety of hairy caterpillar widely distributed in south-eastern Australia. They are often called 'woolly bears' by children. These caterpillars possess barbed hairs that fragment readily and are difficult to extract from the skin in one piece. Itching urticarial wheals and papular eruptions can follow contact with the caterpillars or their detached hairs. The hairlets may be identified by microscopy from skin scrapings and can be removed by tape stripping or with the aid of fine forceps. The skin lesions are treated symptomatically with calamine lotion, sodium bicarbonate solution and antihistamines. Infestation with Euproctis edwardsi can be minimized by removal of mistletoe from eucalyptus trees and by spraying affected areas with white oil or carbaryl 0.1%. PMID:9431713

  18. Common skin conditions during pregnancy.

    PubMed

    Tunzi, Marc; Gray, Gary R

    2007-01-15

    Common skin conditions during pregnancy generally can be separated into three categories: hormone-related, preexisting, and pregnancy-specific. Normal hormone changes during pregnancy may cause benign skin conditions including striae gravidarum (stretch marks); hyperpigmentation (e.g., melasma); and hair, nail, and vascular changes. Preexisting skin conditions (e.g., atopic dermatitis, psoriasis, fungal infections, cutaneous tumors) may change during pregnancy. Pregnancy-specific skin conditions include pruritic urticarial papules and plaques of pregnancy, prurigo of pregnancy, intrahepatic cholestasis of pregnancy, pemphigoid gestationis, impetigo herpetiformis, and pruritic folliculitis of pregnancy. Pruritic urticarial papules and plaques of pregnancy are the most common of these disorders. Most skin conditions resolve postpartum and only require symptomatic treatment. However, there are specific treatments for some conditions (e.g., melasma, intrahepatic cholestasis of pregnancy, impetigo herpetiformis, pruritic folliculitis of pregnancy). Antepartum surveillance is recommended for patients with intrahepatic cholestasis of pregnancy, impetigo herpetiformis, and pemphigoid gestationis. PMID:17263216

  19. Solar urticaria.

    PubMed

    Goetze, Steven; Elsner, Peter

    2015-12-01

    Solar urticaria is a rare IgE-mediated and chromophore-dependent photodermatosis. In some cases, these chromophores, designated as "serum factor", may be detected in serum or plasma. To date, the exact pathogenesis of solar urticaria has, however, not been elucidated. Typical clinical features include the onset of urticarial lesions within a few minutes after light exposure, which already raises diagnostic suspicion. The most common triggers are UVA and visible light. Determination of the action spectrum as well as the minimal urticarial dose (MDU) is diagnostically crucial. Other photodermatoses such as polymorphic light eruption or porphyrias (especially erythropoietic protoporphyria) have to be ruled out. Apart from sunlight avoidance, which is always required, further therapeutic options used include nonsedating antihistamines as well as light hardening. Newer treatment modalities such as plasmapheresis or the anti-IgE antibody omalizumab are reserved for severe, recalcitrant forms of solar urticaria. PMID:26612794

  20. Urticaria and dermographism in patients with adult-onset Still's disease.

    PubMed

    Criado, Paulo Ricardo; de Carvalho, Jozélio Freire; Ayabe, Liliane Akemi; Brandt, Hebert Roberto Clivati; Romiti, Ricardo; Maruta, Celina W

    2012-08-01

    Adult-onset Still's disease (AOSD) patients typically present with arthralgia, fever, lymphadenopathy and a transient salmon maculopapular rash. Only approximately 25 cases of AOSD with urticaria were described in the literature. In this article, the authors report three additional cases of AOSD with urticarial and dermographic lesions who had a good clinical response to glucocorticoid and antihistamines. A review of the literature concerning this issue is also herein written. PMID:21785958

  1. [Association of chronic urticaria with Helicobacter pylori-induced antrum gastritis].

    PubMed

    Bohmeyer, J; Heller, A; Hartig, C; Wetenberger-Treumann, M; Huchzermeyer, H; Otte, H G; Stadler, R

    1996-02-01

    In spite of its high incidence, not much is known about the etiology of chronic urticaria. We performed gastroscopic evaluation of 10 patients in whom no cause for chronic urticaria had been found. In 8 of these 10 patients, Helicobacter pylori was identified in the gastric mucosa. The chronic urticarial lesions disappeared within a few days after starting therapy with amoxicillin and omeprazol. PMID:8868453

  2. Mast cells respond to urticating extract from lepidoptera larva Morpheis ehrenbergii in the rat.

    PubMed

    Galicia-Curiel, María Fernanda; Quintanar, J Luis; Jiménez, Mariela; Salinas, Eva

    2014-01-01

    Mast cells and histamine participate in toxic effects of hairs from some caterpillars. This study reports that a crude extract of Morpheis ehrenbergii caterpillar hairs induces in vitro mast cells activation, triggers the release of histamine and causes a rapid urticarial reaction in the rat skin. Heating of the extract abolishes the inflammatory reaction. These results suggest that the use of antihistamines may improve the adverse skin reactions caused by the Mexican caterpillar M. ehrenbergii. PMID:24269786

  3. The many faces of Merlin: IgG4-associated pulmonary-renal disease.

    PubMed

    Sprangers, Ben; Lioen, Pieter; Meijers, Björn; Lerut, Evelyne; Meersschaert, Joke; Blockmans, Daniel; Claes, Kathleen

    2011-09-01

    Pulmonary-renal syndrome is a common and serious disorder with a broad differential diagnosis. We describe a case of a middle-aged man presenting with interstitial pulmonary disease and severe renal impairment caused by a hypocomplementemic immune-complex-mediated interstitial nephritis. Serum levels of IgG4 were elevated, and renal biopsy specimens revealed the presence of interstitial IgG4(+) plasma cells. There was a rapid improvement of both pulmonary and renal abnormalities after the initiation of corticosteroids. To our knowledge, this report is the first to show interstitial pulmonary disease in association with interstitial kidney disease as the predominant and presenting symptoms of IgG4-related disease. PMID:21896524

  4. THE VASCULAR LESIONS OF A COW AND BISON WITH SHEEP-ASSOCIATED MALIGNANT CATARRHAL FEVER CONTAIN OVINE HERPESVIRUS 2-INFECTED CD8+ T LYMPHOCYTES

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Malignant catarrhal fever (MCF) is a serious herpesviral disease syndrome of certain ruminant species. The microscopic pathology of MCF is characterized by lymphoid proliferation and infiltration, necrotizing vasculitis, and necrosis of lymphoid and epithelial tissues. Previous attempts to detect vi...

  5. Spontaneous ureteral rupture in a patient with systemic lupus erythematosus

    SciTech Connect

    Benson, C.H.; Pennebaker, J.B.; Harisdangkul, V.; Songcharoen, S.

    1983-08-01

    A patient with known systemic lupus erythematosus had fever and symptoms of a lower urinary tract infection. Bone scintigraphy showed left ureteral perforation and necrosis with no demonstrable nephrolithiasis. It is speculated that this episode was due to lupus vasculitis.

  6. Granulomatosis with Polyangiitis

    MedlinePlus

    Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis, is a rare disease. It is a type of vasculitis, or inflammation of the ... trachea (windpipe), lungs, and kidneys. The cause of GPA is unknown. It can affect people at any ...

  7. The CARRA Registry

    ClinicalTrials.gov

    2015-11-16

    Juvenile Idiopathic Arthritis; Systemic Lupus Erythematosus; Mixed Connective Tissue Disease; Juvenile Ankylosing Spondylitis; Juvenile Dermatomyositis; Localized Scleroderma; Systemic Sclerosis; Vasculitis; Sarcoid; Fibromyalgia, Primary; Auto-inflammatory Disease; Idiopathic Uveitis Idiopathic

  8. Vertebrobasilar circulatory disorders

    MedlinePlus

    ... travel to the vertebrobasilar arteries and cause a stroke. Less common causes of vertebrobasilar vascular disorders include connective tissue diseases, blood vessel inflammation (vasculitis), and problems in the spinal bones of the ...

  9. Occipital Neuralgia

    MedlinePlus

    ... inflammation or infection, gout, diabetes, blood vessel inflammation (vasculitis), and frequent lengthy periods of keeping the head ... done? The National Institute of Neurological Disorders and Stroke (NINDS) and other institutes at the National Institutes ...

  10. Bronchoscopy

    MedlinePlus

    ... lungs Narrowing (stenosis) of the trachea or bronchi Sarcoidosis Vasculitis Other inflammatory lung disease Risks Main risks ... Lung Diseases Lung Cancer Lung Diseases Pulmonary Fibrosis Sarcoidosis Browse the Encyclopedia A.D.A.M., Inc. ...

  11. Nerve biopsy

    MedlinePlus

    ... Loss of axon tissue Metabolic neuropathies Necrotizing vasculitis Sarcoidosis Risks Allergic reaction to the local anesthetic Discomfort ... Neurosarcoidosis Peripheral neuropathy Primary amyloidosis Radial nerve dysfunction Sarcoidosis Tibial nerve dysfunction Update Date 6/1/2015 ...

  12. Granulomatosis with polyangiitis and facial palsy: Literature review and insight in the autoimmune pathogenesis.

    PubMed

    Iannella, Giannicola; Greco, Antonio; Granata, Guido; Manno, Alessandra; Pasquariello, Benedetta; Angeletti, Diletta; Didona, Dario; Magliulo, Giuseppe

    2016-07-01

    Granulomatosis with polyangiitis (GPA) is an autoimmune systemic necrotizing small-vessel vasculitis associated with the presence of anti-neutrophil cytoplasmic antibodies (ANCA). Oto-neurological manifestations of ANCA-associated vasculitis according to PR3-ANCA positivity and MPO-ANCA positivity are usually reported. Facial nerve palsy is usually reported during the clinical course of the disease but it might appear as the presenting sign of GPA. Necrotizing vasculitis of the facial nerve 'vasa nervorum' is nowadays the most widely accepted etiopathogenetic theory to explain facial damage in GPA patients. A central role for PR3-ANCA in the pathophysiology of vasculitis in GPA patients with oto-neurological manifestation is reported. GPA requires prompt, effective management of the acute and chronic manifestations. Once the diagnosis of GPA has been established, clinicians should devise an appropriate treatment strategy for each individual patient, based on current clinical evidence, treatment guidelines and recommendations. PMID:26851550

  13. Isolated Aortitis

    MedlinePlus

    ... relieve blocked arteries. Care of aortitis requires periodic surveillance of the aorta and large arteries. Vasculitis experts and expert vascular surgeons are important part of the multi-specialty care team. Frequent visits and follow imaging ...

  14. Behcet's Syndrome

    MedlinePlus

    Behcet's syndrome is a disease that involves vasculitis, which is inflammation of the blood vessels. It causes problems in many parts of the body. The ... National Institute of Arthritis and Musculoskeletal and Skin Diseases

  15. Safety and Efficacy of Anti-Pandemic H1N1 Vaccination in Rheumatic Diseases

    ClinicalTrials.gov

    2010-06-25

    Rheumatoid Arthritis; Spondyloarthritis; Systemic Lupus Erythematosus (SLE); Dermatomyositis (DM); DMixed Connective Tissue Disease; Systemic Vasculitis; Systemic Sclerosis (SSc); Sjögren's Syndrome; Antiphospholipid Syndrome; Juvenile Idiopathic Arthritis; Juvenile SLE; Juvenile DM

  16. ANCA / MPO / PR3 Antibodies Test

    MedlinePlus

    ... Vasculitis .) Help distinguish between ulcerative colitis (UC) and Crohn disease (CD), two common types of inflammatory bowel disease ( ... the health practitioner is attempting to distinguish between Crohn disease and ulcerative colitis. Symptoms of an IBD may ...

  17. A Real World, Observational Registry of Chronic Wounds and Ulcers

    ClinicalTrials.gov

    2016-05-18

    Diabetic Foot; Varicose Ulcer; Pressure Ulcer; Surgical Wound Dehiscence; Vasculitis; Skin Ulcer; Leg Ulcer; Wounds and Injuries; Pyoderma; Peripheral Arterial Disease; Diabetic Neuropathies; Lymphedema; Venous Insufficiency; Diabetes Complications; Amputation Stump

  18. Nerve biopsy

    MedlinePlus

    Nerve biopsy may be done to help diagnose: Axon degeneration (destruction of the axon portion of the nerve cell) Damage to the ... Demyelination Inflammation of the nerve Leprosy Loss of axon tissue Metabolic neuropathies Necrotizing vasculitis Sarcoidosis

  19. Microscopic Polyangiitis

    MedlinePlus

    ... include purplish bumps and spots pictured below (palpable purpura). These areas range in size from several millimeters ... Syndrome (EGPA) Cryoglobulinemia Giant Cell Arteritis Henoch-Schönlein Purpura Microscopic Polyangiitis Polyarteritis Nodosa Rheumatoid Vasculitis Takayasu’s Arteritis ...

  20. Polyarteritis Nodosa

    MedlinePlus

    ... are very common in PAN and may include purpura, livedo reticularis, ulcers, nodules or gangrene. Skin involvement ... the skin (where vasculitis may appear as palpable purpura, livedo reticularis, digital gangrene, or tender nodules), or ...

  1. Eosinophilic Granulomatosis with Polyangiitis, formerly Churg-Strauss Syndrome (EGPA)

    MedlinePlus

    ... Diffuse interstitial lung disease (rarely) Skin Rashes Palpable purpura Nodules (above or below the skin), often at ... Syndrome (EGPA) Cryoglobulinemia Giant Cell Arteritis Henoch-Schönlein Purpura Microscopic Polyangiitis Polyarteritis Nodosa Rheumatoid Vasculitis Takayasu’s Arteritis ...

  2. Protein urine test

    MedlinePlus

    ... problems, such as a bladder tumor or infection Multiple myeloma Risks There are no risks with this test. ... uremic syndrome Interstitial nephritis Membranoproliferative glomerulonephritis Membranous nephropathy Multiple myeloma Necrotizing vasculitis Polycystic kidney disease Renal vein thrombosis ...

  3. Multiple strokes associated with herpes simplex virus type-2 infection: case report.

    PubMed

    Joshi, Prajwol

    2016-04-01

    Herpes simplex virus (HSV) type-2 is known to cause meningitis and usually runs a benign course. Association of such infection with vasculitis of the central nervous system is not well known. Presented here is a case initially diagnosed as aseptic meningitis that subsequently evolved as stroke and exhibited angiographic evidence of widespread vasculitis of the intracranial vessels in association with a positive polymerase chain reaction (PCR) for HSV-2 in the cerebrospinal fluid (CSF). PMID:26443565

  4. Necrotizing ANCA-Positive Glomerulonephritis Secondary to Culture-Negative Endocarditis

    PubMed Central

    Van Haare Heijmeijer, Sophie; Wilmes, Dunja; Aydin, Selda; Clerckx, Caroline; Labriola, Laura

    2015-01-01

    Infective endocarditis (IE) and small-vessel vasculitis may have similar clinical features, including glomerulonephritis. Furthermore the association between IE and ANCA positivity is well documented, making differential diagnosis between IE- and ANCA-associated vasculitis particularly difficult, especially in case of culture-negative IE. We report on one patient with glomerulonephritis secondary to culture-negative IE caused by Bartonella henselae which illustrates this diagnostic difficulty. PMID:26819786

  5. Renal findings in rheumatoid arthritis: clinical aspects of 132 necropsies.

    PubMed

    Boers, M; Croonen, A M; Dijkmans, B A; Breedveld, F C; Eulderink, F; Cats, A; Weening, J J

    1987-09-01

    Renal abnormalities in 132 necropsied patients with rheumatoid arthritis were studied. Clinical findings before death included extra-articular manifestations of the disease (86% of patients), systemic vasculitis (6%), and uraemia (23%). Necropsy findings included nephrosclerosis (90%), systemic vasculitis (14%) with kidney involvement in 8%, amyloidosis (11%), membranous glomerulopathy (8%), and focal glomerular disease (8%). Association with clinical data suggests that both rheumatoid and non-rheumatoid disease may play a part in the cause of these abnormalities. PMID:3675007

  6. Livedoid Vasculopathy and Mononeuritis Multiplex, with a Fulminant Hepatic Failure which was caused by Herpes Simplex Hepatitis: A Case Report

    PubMed Central

    Pai B, Sathish; Pai, Kanthilatha

    2013-01-01

    Livedoid vasculopathy with mononeuritis multiplex is a rare association. We are presenting a case of an unusual association of livedoid vasculopathy with mononeuritis multiplex, who developed fulminant hepatic failure which was secondary to Herpes simplex virus (HSV) hepatitis, while she was on treatment with immunosuppressants. Her skin biopsy and immunofluorescence studies showed the features of vasculitis. A biopsy from the sural nerve showed the features of chronic vasculitis. PMID:23814745

  7. [Vasculitic Peripheral Neuropathies: Clinical Features and Diagnostic Laboratory Tests].

    PubMed

    Ogata, Katsuhisa

    2016-03-01

    Vasculitic peripheral neuropathy (VPN) occurs due to ischemic changes of peripheral nerves, resulting from a deficit of vascular blood supply due to damaged vasa nervorum leading to vasculitis. VPN usually manifests as sensorimotor or sensory disturbances accompanied by pain, presenting as a type of multiple mononeuropathy, with a scattered distribution in distal limbs. VPN may also present as a mononeuropathy, distal symmetric polyneuropathy, plexopathy, or radiculopathy. The rapidity of VPN is variable, ranging from days to months, with symptoms occasionally changing with the appearance of new lesions. Careful history taking and neurological examination provides an exact diagnosis. The most common cause of VPN is primary vasculitis predominantly affecting small vessels, including vasa nervorum, anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, and polyarteritis nodosa. Similar vasculitic processes can also result from a systemic collagen disorder or secondary vasculitis. Electrophysiological studies and pathological investigation of biopsied peripheral nerves and muscles are important for diagnosis of vasculitis. Serological tests, including ANCA, are useful for diagnosis of vasculitis. Accurate neurological examinations are essential for diagnosis and evaluation of clinical course. PMID:27001769

  8. Physical urticarias and cholinergic urticaria.

    PubMed

    Abajian, Marina; Schoepke, Nicole; Altrichter, Sabine; Zuberbier, Torsten; Zuberbier, H C Torsten; Maurer, Marcus

    2014-02-01

    Physical urticarias are a unique subgroup of chronic urticaria in which urticarial responses can be reproducibly induced by different specific physical stimuli acting on the skin. These conditions include urticaria factitia/symptomatic dermographism, delayed pressure urticaria, cold contact urticaria, heat contact urticaria, solar urticaria, and vibratory urticaria/angioedema. Physical urticarias and cholinergic urticarias are diagnosed based on the patients' history and provocation tests including trigger threshold testing where possible. Treatment is mainly symptomatic. Many patients benefit from avoiding eliciting triggers, and desensitization to these triggers can be helpful in some physical urticarias and in cholinergic urticaria. PMID:24262690

  9. Lichen planus pemphigoides associated with pregnancy mimicking pemphigoid gestationis.

    PubMed

    Rullán, Jennifer; Diaz, Nicole C; Vazquez-Botet, Miguel

    2016-06-01

    Lichen planus pemphigoides (LPP) is a rare condition characterized by tense blisters that arise on lesions of lichen planus (LP) and on unaffected skin. We present the case of a 25-year-old pregnant woman at 12 weeks' gestation who developed an acute bullous eruption after 5 months of worsening LP. Similarities to pemphigoid gestationis (PG) included lesions around the periumbilical area and multiple urticarial erythematous papules and plaques in addition to linear C3 and IgM deposition along the basement membrane zone (BMZ) on direct immunofluorescence (DIF). PMID:27416093

  10. A Review and Proposed Approach to the Neutrophilic Dermatoses of Childhood.

    PubMed

    Webb, Kate; Hlela, Carol; Jordaan, H Francois; Suliman, Sara; Scriba, Thomas; Lipsker, Dan; Scott, Chris

    2015-01-01

    Neutrophilic dermatoses (NDs) are inflammatory skin conditions that are not associated with infection. The classification and clinical approach to these conditions in children is poorly described. This review classifies these conditions into five nosological subtypes: Sweet's syndrome, pyoderma gangrenosum, aseptic pustules, neutrophilic urticarial dermatoses, and Marshall's syndrome. In addition, we review the various secondary diseases that need to be excluded in the clinical management of the NDs of childhood, with a focus on the autoinflammatory conditions that the reader may not be familiar with. We propose a practical clinical approach to these disorders. PMID:25727235

  11. Pruritus in pregnancy

    PubMed Central

    Bergman, Hagit; Melamed, Nir; Koren, Gideon

    2013-01-01

    Abstract Question Some of my pregnant patients complain about pruritus. Are there conditions in pregnancy that present with pruritus that might put the mother or fetus at risk? Answer Although most cases of pruritus can be attributed to itchy dry skin, there are conditions unique to pregnancy that involve pruritus as a leading symptom. These include pemphigoid gestationis, pruritic urticarial papules and plaques of pregnancy, intrahepatic cholestasis of pregnancy, and atopic eruption of pregnancy. These conditions are associated with severe pruritus and some might be associated with adverse fetal outcomes. Clinical history and physical examination are the most important diagnostic clues when evaluating pruritus in pregnancy. PMID:24336540

  12. Advances in understanding and managing bullous pemphigoid

    PubMed Central

    Zhao, Cathy Y.; Murrell, Dedee F.

    2015-01-01

    Bullous pemphigoid (BP) is the commonest subtype of autoimmune blistering disease in most countries of the world. It occurs most frequently in elderly patients and is characterised clinically by large, tense blisters in the skin preceded by urticarial plaques and pruritus. Immunopathologically, it is characterised by autoantibodies directed against the 180 kD antigen (BP180) and the 230 kD antigen (BP230). New knowledge regarding BP is being continually uncovered. This article reviews the recent advances in BP, including newer diagnostic tests, standardised outcome measures and emerging therapeutic options, as well as the evidence supporting their use. PMID:26918143

  13. Cutaneous Larva Migrans in Early Infancy.

    PubMed

    Siddalingappa, Karjigi; Murthy, Sambasiviah Chidambara; Herakal, Kallappa; Kusuma, Marganahalli Ramachandra

    2015-01-01

    Cutaneous larva migrans or creeping eruptions is a cutaneous dermatosis caused by hookworm larvae, Ancylostoma braziliense. A 2-month-old female child presented with a progressive rash over the left buttock of 4 days duration. Cutaneous examination showed an urticarial papule progressing to erythematous, tortuous, thread-like tract extending a few centimeters from papule over the left gluteal region. A clinical diagnosis of cutaneous larva migrans was considered. Treatment with albendazole led to complete resolution, confirming the diagnosis. This is to the best of our knowledge, the youngest age at which this condition is being reported. PMID:26538729

  14. Familial polymorphous light eruption with aquagenic urticaria: successful treatment with PUVA.

    PubMed

    Juhlin, L; Malmros-Enander, I

    1986-12-01

    A patient is described with both polymorphous light eruption (PLE) and aquagenic urticaria appearing at 29 yr of age. Her father had the same symptoms after exposure to solar irradiation or water. Four to 6 h after 5-15 min sun-exposure, both had symptoms of general malaise and swelling of various joints. The skin symptoms in our patient were initially urticarial and later mainly papular and vesicular. They were elicited by irradiation with low doses of 300-360 nm and also appeared after 400 and 500 nm. Window glass offered little protection. PUVA treatment improved both conditions remarkably. PMID:3588355

  15. ALLERGIC REACTIONS TO “KISSING BUG” BITES

    PubMed Central

    Nichols, Norman; Green, Thomas W.

    1963-01-01

    In addition to local allergic skin reactions to the bite of the kissing bug, Triatoma protracta, the systemic reactions can vary from a mild urticarial reaction to a severe anaphylactoid reaction consisting of shock, generalized angioneurotic edema and laryngeal edema. The diagnosis can be established by the clinical history, the finding of an engorged kissing bug, the presence of typical local and systemic signs, and finally, a positive reaction to a skin test with an extract of the bug. ImagesFigure 1. PMID:13938346

  16. [Eosinophilic cellulitis (Wells' syndrome)].

    PubMed

    Cerri, D; Carabelli, A; Bertani, E; Portaluppi, F; Novi, C; Gianotti, R; Gelmetti, C

    1990-09-01

    The Authors report a case of eosinophilic cellulitis (Wells' syndrome). The patient was a 61 year old woman, diabetic, with a cardio-respiratory insufficiency and a maniaco-depressive psycosis. She presented, on the upper arms and trunk, a cutaneous eruption of erythematous-urticarial plaques, that histopathologically were characterized by a dermic leukocyte population, with a prevalence of eosinophils, distributed in the perivascular site. Laboratory tests revealed eosinophilia and circulating immune complexes. The etiopathogenesis of the disease is discussed as is the possible role of immune complexes in eosinophilic cellulitis. PMID:2079351

  17. Cutaneous Larva Migrans in Early Infancy

    PubMed Central

    Siddalingappa, Karjigi; Murthy, Sambasiviah Chidambara; Herakal, Kallappa; Kusuma, Marganahalli Ramachandra

    2015-01-01

    Cutaneous larva migrans or creeping eruptions is a cutaneous dermatosis caused by hookworm larvae, Ancylostoma braziliense. A 2-month-old female child presented with a progressive rash over the left buttock of 4 days duration. Cutaneous examination showed an urticarial papule progressing to erythematous, tortuous, thread-like tract extending a few centimeters from papule over the left gluteal region. A clinical diagnosis of cutaneous larva migrans was considered. Treatment with albendazole led to complete resolution, confirming the diagnosis. This is to the best of our knowledge, the youngest age at which this condition is being reported. PMID:26538729

  18. Is There any Relationship Between Extra-Pulmonary Manifestations of Mycoplasma Pneumoniae Infection and Atopy/Respiratory Allergy in Children?

    PubMed Central

    Poddighe, Dimitri; Marseglia, Gian Luigi

    2016-01-01

    Mycoplasma pneumoniae is a common cause of respiratory infections in children, but sometimes extra-pulmonary diseases can be observed. The immunological mechanisms involved in these extra-respiratory complications are unknown. Here, we report a small case series of Mycoplasma-related diseases including 5 children who developed: i) aseptic meningitis; ii) urticarial rash and pericardial effusion; iii) pleural effusion with severe eosinophilia; iv) Stevens-Johnson syndrome; v) multiform erythema. Interestingly, all children were moderately to highly atopic, as a common immunologic feature. PMID:27114818

  19. Acebrophylline-induced angioedema

    PubMed Central

    Kuriachan, Sanitha; Amberkar, Mohan Babu V; Mohan, Manu K.; Shahul, Hameed Aboobackar; Kishore, Meenakumari Kamal

    2015-01-01

    A 53-year-old woman visited her physician complaining of acute breathlessness and productive cough. Her medications included budesonide and formoterol for asthma, fixed-dose combination aspirin 150 mg + clopidogrel 75 mg + atorvastatin 20 mg for ischemic heart disease. History revealed that she had allergic rhinitis and was hypersensitive to penicillins. The patient was prescribed acebrophylline (ABP). Six hours after ABP therapy she presented with generalized urticarial lesions, swelling of hands, feet, lips and face, suggestive of angioedema. ABP was stopped immediately, and the patient was treated symptomatically. This case was categorized as probable as per standard causality assessment scale. PMID:25878387

  20. Acebrophylline-induced angioedema.

    PubMed

    Kuriachan, Sanitha; Amberkar, Mohan Babu V; Mohan, Manu K; Shahul, Hameed Aboobackar; Kishore, Meenakumari Kamal

    2015-01-01

    A 53-year-old woman visited her physician complaining of acute breathlessness and productive cough. Her medications included budesonide and formoterol for asthma, fixed-dose combination aspirin 150 mg + clopidogrel 75 mg + atorvastatin 20 mg for ischemic heart disease. History revealed that she had allergic rhinitis and was hypersensitive to penicillins. The patient was prescribed acebrophylline (ABP). Six hours after ABP therapy she presented with generalized urticarial lesions, swelling of hands, feet, lips and face, suggestive of angioedema. ABP was stopped immediately, and the patient was treated symptomatically. This case was categorized as probable as per standard causality assessment scale. PMID:25878387