Science.gov

Sample records for vasculitis

  1. Vasculitis

    MedlinePLUS

    ... Symptoms of vasculitis can vary, but usually include fever, swelling and a general sense of feeling ill. The main goal of treatment is to stop the inflammation. Steroids and other medicines to stop inflammation are often ...

  2. Urticarial Vasculitis

    MedlinePLUS

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

  3. 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 UCLA Vasculitis Patient Conference – April 30, ...

  4. Necrotizing vasculitis

    MedlinePLUS

    Necrotizing vasculitis is a group of disorders that involve inflammation of the blood vessel walls. The size of the ... Necrotizing vasculitis is common with: Polyarteritis nodosa Rheumatoid ... Granulomatosis with polyangiitis (formerly called Wegener ...

  5. Hypersensitivity Vasculitis

    MedlinePLUS

    ... Health Educational Webinars Frequently Asked Questions VF Medical Consultants Patient Advocacy Groups Pediatrics/Young Adults Newly Diagnosed ... Information Book Vasculitis Terms A – Z VF Medical Consultants YouTube Education Videos More common than you think. ...

  6. Types of Vasculitis

    MedlinePLUS

    ... page from the NHLBI on Twitter. Types of Vasculitis There are many types of vasculitis. Each type ... the blood vessels they affect. Mostly Large Vessel Vasculitis These types of vasculitis usually, but not always, ...

  7. Central Nervous System Vasculitis

    MedlinePLUS

    ... Nervous System (CNS) Vasculitis Central Nervous System (CNS) Vasculitis Central nervous system (CNS) vasculitis is inflammation of ... CNS (PACNS). What is the cause of CNS Vasculitis? How the vessels in the brain become inflamed ...

  8. Vasculitis Pregnancy Registry

    ClinicalTrials.gov

    2016-01-14

    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

  9. Ocular vasculitis.

    PubMed

    Espinoza, Gabriela M; Desai, Ankit; Akduman, Levent

    2013-09-01

    Vasculitis is the inflammation of blood vessels that leads to loss of perfusion and ischemia with necrosis. When this occurs in the orbit, the consequences are typically very conspicuous and can be devastating with decreased quality of life and loss of vision. Systemic vasculitides are often related to ophthalmic disorders, which can serve as the first diagnostic manifestation of potentially life-threatening disease. Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (e.g., granulomatosis with polyangiitis), Behcet's disease, rheumatoid arthritis, sarcoidosis, and systemic lupus erythematosus are a few of the diseases commonly associated with ocular vasculitis. Collaboration between ophthalmologists and rheumatologists is important in the successful diagnosis and treatment of patients with vasculitis. PMID:23888364

  10. 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

  11. Golfer's vasculitis.

    PubMed

    Kelly, Robert I; Opie, Jacinta; Nixon, Rosemary

    2005-02-01

    A number of patients presented with an erythematous, purpuric rash occurring on the legs in association with playing golf and also after prolonged walks or hikes. Many patients believed that it was an allergic reaction to grasses or insecticides and had sometimes undergone extensive allergy testing. We collected reports of 17 such cases from dermatologists in the state of Victoria, Australia. Patients were interviewed by phone and asked to submit photographs of the rash if possible. Of these, the eruption developed in 15 after playing 18 holes of golf and in three following prolonged hikes. The rash would usually develop over the summer months under hot conditions. Most patients were over 50 years of age when the tendency to develop the eruption began. Biopsies of the rash in the active phase showed leukocytoclastic vasculitis. Patch testing and investigations for potential underlying causes for vasculitis were negative or unremarkable. It would seem that this is a common but poorly documented condition. The clinical presentation and histology would support the conclusion that it represents a leukocytoclastic vasculitis induced by prolonged exercise under hot conditions. The findings would suggest that it occurs in healthy people and extensive investigation with blood tests or allergy testing is inappropriate. We believe the condition should be termed 'golfer's vasculitis', as golf appears to be the most common precipitating event and such a term would enable the condition to become more widely recognized. PMID:15670170

  12. Cerebral vasculitis.

    PubMed

    Adams, Harold P

    2014-01-01

    The noninfectious, inflammatory vasculitides include giant cell arteritis, Takayasu disease, Churg-Strauss angiitis, Wegener disease, polyarteritis nodosa, microscopic polyangiitis, Buerger disease, amyloid-?-related angiitis, and isolated vasculitis of the central nervous system. While these disorders are relatively uncommon, they produce a variety of neurologic diseases including muscle disease, mononeuropathy multiplex, polyneuropathy, cranial nerve palsies, visual loss, seizures, an encephalopathy, venous thrombosis, ischemic stroke, and intracranial hemorrhage. The multisystem vasculitides often have stereotypical clinical findings that reflect disease of the kidney, sinuses, lungs, skin, joints, or cardiovascular system. These disorders also usually have abnormalities found on serologic testing. Isolated vasculitis of the central nervous system is more difficult to diagnose because the clinical and brain imaging findings are relatively nonspecific. Examination of the cerebrospinal fluid will demonstrate changes consistent with an inflammatory process. Arteriography often shows areas of segmental narrowing affecting multiple intracranial vessels and brain/meningeal biopsy may be required to establish the diagnosis. Management of patients with a multisystem vasculitis or isolated vasculitis of the central nervous system is centered on the administration of immunosuppressive agents. In many cases, corticosteroids remain the mainstay of medical treatment. PMID:24365314

  13. Pediatric Vasculitis Initiative

    ClinicalTrials.gov

    2015-07-16

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

  14. Cryoglobulinemia Vasculitis.

    PubMed

    Cacoub, Patrice; Comarmond, Cloe; Domont, Fanny; Savey, Léa; Saadoun, David

    2015-09-01

    Cryoglobulinemic vasculitis (CryoVas) is a small-vessel vasculitis involving mainly the skin, the joints, the peripheral nervous system, and the kidneys. Type I CryoVas is single monoclonal immunoglobulins related to an underlying B-cell lymphoproliferative disorder. Type II and III cryoglobulins, often referred to as mixed cryoglobulinemia, consist of polyclonal immunoglobulin (Ig)G with or without monoclonal IgM with rheumatoid factor activity. Hepatitis C virus (HCV) infection represents the main cause of mixed CryoVas. The 10-year survival rates are 63%, 65%, and 87% in HCV-positive mixed CryoVas, HCV-negative mixed CryoVas, and type I CryoVas patients, respectively. In HCV-positive patients, baseline poor prognostic factors include the presence of severe liver fibrosis, and central nervous system, kidney, and heart involvement. Treatment with antivirals is associated with a good prognosis, whereas use of immunosuppressants (including corticosteroids) is associated with a poor outcome. In HCV-negative patients, pulmonary and gastrointestinal involvement, renal insufficiency, and age > 65 years are independently associated with death. Increased risk of lymphoma also should be underlined. Treatment of type I CryoVas is that of the hemopathy; specific treatment also includes plasma exchange, corticosteroids, rituximab, and ilomedine. In HCV-CryoVas with mild-to-moderate disease, an optimal antiviral treatment should be given. For HCV-CryoVas with severe vasculitis (ie, worsening of renal function, mononeuritis multiplex, extensive skin disease, intestinal ischemia…) control of disease with rituximab, with or without plasmapheresis, is required before initiation of antiviral therapy. Other immunosuppressants should be given only in case of refractory forms of CryoVas, frequently associated with underlying B-cell lymphoma. PMID:25837517

  15. 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

  16. Vasculitis and infectious diseases.

    PubMed

    Satta, R; Biondi, G

    2015-04-01

    Vasculitis usually presents without a well-known underline cause (idiopathic vasculitis), nevertheless, it is sometimes possible to find out one or more causative agents (secondary vasculitis). Nowadays, thanks to the increasing amount of precise diagnostic tools, a piece of idiopathic vasculitis is reclassified as associated with probable etiology, which can be set off by several factors, such as infections. Infections are considered to be the most common cause of secondary vasculitis. Virtually, every infectious agent can trigger a vasculitis by different mechanisms which can be divided in two main categories: direct and indirect. In the former, infectious agents destroy directly the vascular wall leading, eventually, to a subsequent inflammatory response. In the latter, indirect form, they stimulate an immune response against blood vessels. Different infectious agents are able to directly damage the vascular wall. Among these, it is possible to recognize Staphylococcus spp, Streptococcus spp, Salmonella spp, Treponema spp, Rickettsia spp, Cytomegalovirus, Herpes Simplex Virus 1 and 2, and many others which have a peculiar tropism for endothelial cells. Conversely, another group of microbial agents, such as Mycobacterium tuberculosis, Mycobacterium leprae, Hepatits B Virus, Human Immunodeficiency Virus and others, trigger vasculitis in the indirect way. This is due to the fact that they can share epitopes with the host or modify self-antigens, thus leading to a cross-self reaction of the immune system. These mechanism, in turn, leads to immunological responses classified as type I-IV by Gell-Coombs. Nevertheless, it is difficult to strictly separate the direct and indirect forms, because most infectious agents can cause vasculitis in both ways (mixed forms). This paper will analyze the link between infectious agents and vasculitis, focusing on direct and indirect secondary vasculitis, and on a group of probable infection-related idiopathic vasculitis, and finally on a group of idiopathic vasculitis with microbiological triggers. Furthermore, a diagnostic and therapeutic approach to vasculitis when an underline infection has been suspected is suggested. PMID:25876145

  17. [Histopathology of systemic vasculitis].

    PubMed

    Holl-Ulrich, K

    2010-02-01

    Inflammatory diseases of blood vessels can occur in any part of the vascular system with a variety of clinical and histopathological manifestations. Possible etiologies include primary systemic vasculitis, secondary vasculitis or isolated single organ vasculitis. Key criteria of morphological vasculitis work-up are vessel size, type of inflammation (granulomatous, necrotizing and/or leukocytoclastic) as well as presence or absence of immune complexes and extravascular inflammatory changes. Together with the typical organ involvement and serological data, these criteria constitute the basis of vasculitis classification. A histopathologically confirmed biopsy is the gold standard for a diagnosis of vasculitis, although a definite diagnosis can frequently not be made solely on histopathological grounds. Differential diagnostic overlaps and possible ways of discrimination are presented. In many cases, however, histopathology can only provide a definite diagnosis in combination with clinical and serological data. A conclusive morphological diagnosis depends on the right time of biopsy and selection of appropriate biopsy material. PMID:19533137

  18. 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

  19. The neuropathies of vasculitis.

    PubMed

    Collins, Michael P; Arnold, William David; Kissel, John T

    2013-05-01

    Vasculitic neuropathy can occur as an isolated entity (nonsystemic vasculitic neuropathy) but more commonly evolves in the setting of primary systemic vasculitides or secondary vasculitides related to infections, drugs, or connective tissue disorders. Vasculitic neuropathies are usually but not always painful and tend to produce sensory motor or sensory symptoms. Patients with purely motor or small-fiber dysfunction are unlikely to have vasculitis. Deficits are typically multifocal or asymmetric, but distal symmetric polyneuropathy occurs uncommonly. Evaluation requires laboratory tests, electrodiagnostic studies, and nerve or nerve/muscle biopsy. This article reviews classification, clinical presentation, diagnostic evaluation, and management of peripheral nerve vasculitis. PMID:23642724

  20. Biomarkers in Vasculitis

    PubMed Central

    Monach, Paul A.

    2014-01-01

    Purpose of review Better biomarkers are needed for guiding management of patients with vasculitis. Large cohorts and technological advances had led to an increase in pre-clinical studies of potential biomarkers. Recent findings The most interesting markers described recently include a gene expression signature in CD8+ T cells that predicts tendency to relapse or remain relapse-free in ANCA-associated vasculitis, and a pair of urinary proteins that are elevated in Kawasaki disease but not other febrile illnesses. Both of these studies used “omics” technologies to generate and then test hypotheses. More conventional hypothesis-based studies have indicated that the following circulating proteins have potential to improve upon clinically available tests: pentraxin-3 in giant cell arteritis and Takayasu’s arteritis; von Willebrand factor antigen in childhood central nervous system vasculitis; eotaxin-3 and other markers related to eosinophils or Th2 immune responses in eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome); and MMP-3, TIMP-1, and CXCL13 in ANCA-associated vasculitis. Summary New markers testable in blood and urine have the potential to assist with diagnosis, staging, assessment of current disease activity, and prognosis. However, the standards for clinical usefulness, in particular the demonstration of either very high sensitivity or very high specificity, have yet to be met for clinically relevant outcomes. PMID:24257367

  1. Dabigatran Associated Leukocytoclastic Vasculitis

    PubMed Central

    Potolidis, Evangelos; Mandros, Charalampos; Kotsa, Kalliopi; Mitsiou, Evdoxia; Potolidis, Dimitris; Fanourgiakis, Panagiotis

    2015-01-01

    Common side effects of dabigatran are bleeding, bruising, nausea, diarrhea, and abdomen discomfort. Skin reactions were not often noted (<0.1%). We report a case of 70-year-old male who developed dabigatran related skin reaction resistant to usual therapy. Skin biopsy revealed leukocytoclastic vasculitis. PMID:26843867

  2. How Is Vasculitis Diagnosed?

    MedlinePLUS

    ... vasculitis. Duplex ultrasonography combines an image of the structure of the blood vessel with a color image of the blood flow through that vein or artery. 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) identifies areas that show higher glucose metabolism leading ...

  3. Telmisartan induced urticarial vasculitis

    PubMed Central

    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

  4. 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

  5. Vancomycin-induced leukocytoclastic vasculitis.

    PubMed

    Felix-Getzik, Erika; Sylvia, Lynne M

    2009-07-01

    Vancomycin is well recognized as causing the nonallergic skin reaction known as red man syndrome; however, it is rarely suspected as causative in the setting of an immune-mediated skin reaction. We describe a 76-year-old Caucasian woman with a history of penicillin and sulfa allergies who was transferred to our medical center while receiving vancomycin for treatment of persistent methicillin-sensitive Staphylococcus aureus (MSSA) bacteremia. After admission, the patient's pacemaker was explanted; cultures from the pacemaker grew MSSA. Based on the culture data and her allergy to penicillin, vancomycin was continued. On day 4 of therapy, the patient developed a papular rash with small blisters on her distal upper extremities. Furosemide, which she was receiving intermittently to maintain fluid balance, was initially suspected as the likely cause. Furosemide was withheld; however, the rash worsened and spread to her neck and torso. Results of skin biopsy confirmed a severe leukocytoclastic, necrotizing small-cell vasculitis that met the criteria for a hypersensitivity vasculitis associated with drug therapy. Five days after discontinuation of vancomycin, the vasculitis was resolving and continued to resolve throughout the remainder of her hospitalization. Furosemide was readministered without worsening of the vasculitis. Use of the Naranjo adverse drug reaction probability scale indicated that the likelihood of vancomycin being the cause of the vasculitis was probable (score of 5). Clinicians should be aware of vancomycin as a potential cause of small-vessel vasculitis. PMID:19558258

  6. Cutaneous vasculitis update: small vessel neutrophilic vasculitis syndromes.

    PubMed

    Carlson, J Andrew; Chen, Ko-Ron

    2006-12-01

    A broad and diverse spectrum of vasculitic syndromes exists. These syndromes affect the skin with varying levels of associated systemic manifestations, running the gamut from a self-limited, localized, cutaneous phenomenon to rapidly progressive, multiorgan disease. The majority of cases of cutaneous vasculitis will show a neutrophilic small vessel vasculitis that can be either a primary (idiopathic) disorder (eg, cutaneous leukocytoclastic angiitis) or a secondary disorder that is associated with drugs, infection (eg, streptococcal infection, viral hepatitis), or underlying disease (eg, connective tissue disease, malignancy). Biopsy is the gold standard for the diagnosis of cutaneous vasculitis and also necessary for the detection of cutaneous vascular immune complexes by direct immunofluorescence. Based on the type of vessel disrupted by inflammation (small and/or muscular), the distribution of vasculitis in the dermis and subcutis, and predominate inflammatory cell-type mediating vessel wall damage, a list of relevant differential diagnoses can be generated. This histologic information coupled with extravascular findings such as tissue eosinophilia, tissue neutrophilia, and/or granulomas, plus pathophysiologic markers such as direct immunofluorescent examination for immune complexes and serologic evaluation for antineutrophil cytoplasmic antibodies allows for more accurate diagnosis of specific vasculitic entities. Herein, we review both primary and secondary vasculitic syndromes that affect the skin and show a small vessel neutrophilic mediated vasculitis. PMID:17122493

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

    ClinicalTrials.gov

    2015-10-19

    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

  8. Gastroenterology Cases of Cutaneous Leukocytoclastic Vasculitis

    PubMed Central

    Arabaci, Elif; Yildiz, Kemal; Cakirca, Mustafa; Cikrikcioglu, Mehmet Ali; Ergun, Fatih; Danalioglu, Ahmet; Kocaman, Orhan; Senturk, Hakan

    2013-01-01

    Rarely, leukocytoclastic vasculitis can result from ischemic colitis, inflammatory bowel disease, and cryoglobulinemia. There is no established standard for the treatment of leukocytoclastic vasculitis associated with gastroenterologic diseases. This paper presents three cases of leukoytoclastic vasculitis, each of which is associated with a different gastroenterologic condition: ischemic colitis, Crohn's disease, and chronic hepatitis C. Each condition went into remission by treatment of leukocytoclastic vasculitis, regardless of the underlying disease. PMID:24250337

  9. 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.

  10. 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

  11. [Septic vasculitis caused by Listeria monocytogenes].

    PubMed

    Vera-Kellet, Cristin; Del Puerto, Constanza; Ruiz, Felipe; Gonzlez, Sergio; Manrquez, Jorge

    2014-12-01

    Septic vasculitis is a medium and small-vessel vasculitis caused by direct action of pathogens, associated with bacteremia. It is an uncommon condition; clinical manifestations include purpura, ulcers and vesicles-pustules. Most cases of septic vasculitis are related to meningococcemia. There are no cases reported in medical literature associated to Listeria spp. We report a case of a 71 year-old man who presented sepsis by Listeria monocytogenes, and then evolved with purpuric skin lesions. Skin biopsy revealed a septic vasculitis. PMID:25679934

  12. 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

  13. Cutaneous leukocytoclastic vasculitis associated with letrozole.

    PubMed

    Digklia, Antonia; Tzika, Evangelia; Voutsadakis, Ioannis A

    2014-04-01

    Aromatase inhibitors are increasingly used in the treatment of early and metastatic breast cancer. They can produce various skin adverse effects but are only rarely associated with cutaneous vasculitis. We report the first case of cutaneous vasculitis clearly associated with the use of aromatase inhibitor letrozole. PMID:23676512

  14. 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 greater detail in describing and observing the long-term natural history of primary systemic vasculitis in patients treated with immunosuppressive agents who survive their initial disease process. PMID:22983618

  15. Biologic agents in systemic vasculitis

    PubMed Central

    Henderson, Charles F; Seo, Philip

    2012-01-01

    The treatment of systemic necrotizing vasculitis has made great strides in both efficacy and outcomes. Standard therapies, however, are associated with numerous side effects, and not all patients will respond to conventional immunosuppression. These realities have prompted the search for safer and more efficacious treatments, most notably among biologic agents. For example, the role of TNF-α in the pathophysiology of several vasculitides has led to the investigation of targeted inhibitors of this cytokine, albeit with mixed results. There have been some disappointing results in the area of giant cell arteritis and Wegener’s granulomatosis (granulomatosis with polygiitis), but anti-TNF therapy has shown promise in the treatment of Takayasu’s arteritis, although additional trials to demonstrate its efficacy are required. Anti-B-cell therapy seems to be the most promising advance in the management of these diseases. Complete and partial responses have been seen in both primary and secondary mixed cryoglobulinemic vasculitis. Recent trials have demonstrated that rituximab is effective for the treatment of Wegener’s granulomatosis and microscopic polyangiitis. These trials have, however, raised concerns regarding the long-term safety of these agents. The future holds promise for additional targeted therapies with improved patient response and fewer side effects. PMID:23785387

  16. Disease assessment in systemic vasculitis.

    PubMed

    Luqmani, Raashid Ahmed

    2015-04-01

    The anti-neutrophil cytoplasm antibody-associated vasculitides are complex multi-system disorders with many overlapping clinical features. Their outcome has been transformed by effective immunosuppression, preventing death in over 70% of cases. The quality of survival is affected by the disease course, which is characterized by a significant likelihood of relapse in 38%, chronic effects from the disease and its treatment, as well as emerging or worsening comorbidity, all of which contribute to the patient's clinical condition and outcome. Whilst imaging and laboratory testing including histology are important aspects of diagnosis, they are of limited value in assessing response to therapy or subsequent disease course. We have developed standardized validated clinical methods to quantify disease activity and damage; we are developing effective measures of patient experience to complement these procedures. This approach provides a rational basis for clinical management as well as being essential in the conduct of clinical trials and studies in vasculitis, by providing reproducible definitions of relapse, remission and response to therapy for patients with systemic vasculitis. Clinical assessment remains the current gold standard for evaluating disease progress, but requires regular training to ensure standardization. The development of biomarkers in future may produce a more accurate description of disease and identify potential targets for therapy as well as predictors of response to drugs. PMID:25662796

  17. Vasculitis

    MedlinePLUS

    ... Researchers Six Minute Walk Test (SMWT) Arthritis Impact Measurement Scales (AIMS) Evidence Based Practice (EBP) Fibromyalgia Impact ... Outcomes Instrumentation Introduction (OII) Physical Therapy Evaluation Tools & Measurements (PTETM) quickDASH (QD) Timed Up & Go (TUG) Western ...

  18. Localized striated muscle vasculitis in rheumatoid arthritis.

    PubMed

    Ali, Zarmeena; Ranganathan, Prabha; Perry, Arie; Gelbart, Michael

    2007-02-01

    Rheumatoid arthritis (RA) is one of the most prevalent connective tissue diseases and can be complicated by vasculitis with systemic manifestations. Rheumatoid vasculitis can range in severity from a widespread, life-threatening disease refractory to treatment to a more benign, localized disease responsive to treatment. We describe here a patient with RA who presented with bilateral calf muscle pain secondary to rheumatoid vasculitis. The patient had intractable calf muscle pain which did not respond to nonsteroidal anti-inflammatory drugs and muscle relaxants. He did not have any other manifestations of rheumatoid vasculitis such as skin ulcers, peripheral neuropathy, or gastrointestinal involvement. A thorough diagnostic work-up concluding with a muscle biopsy revealed a non-necrotizing small vessel vasculitis. The patient's symptoms responded very well to a course of steroids and he required no additional cytotoxic agents. This case illustrates that rheumatoid vasculitis can present as an isolated striated muscle vasculitis characterized by a relatively benign course and prompt resolution with the initiation of steroids. Clinicians need to be aware that such a phenomenon can exist to facilitate early recognition and appropriate treatment. PMID:17278948

  19. Urticarial Vasculitis in Northern Spain

    PubMed Central

    Loricera, Javier; Calvo-Ro, Vanesa; Mata, Cristina; Ortiz-Sanjun, Francisco; Gonzlez-Lpez, Marcos A.; Alvarez, Lino; Gonzlez-Vela, M. Carmen; Armesto, Susana; Fernndez-Llaca, Hctor; Rueda-Gotor, Javier; Gonzlez-Gay, Miguel A.; Blanco, Ricardo

    2014-01-01

    Abstract 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, 178 yr; interquartile range, 554 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, 238 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. Low complement serum levels are observed in a minority of cases. The prognosis is generally good, but depends on the underlying disease. PMID:24378743

  20. [Allergic vasculitis following ingestion of celecoxib?].

    PubMed

    Gscheidel, D; Daspet, M K; Le Coz, C J; Lipsker, D

    2002-07-01

    A 76-year-old patient developed cutaneous vasculitis on the lower legs on the 8th day of treatment with the selective cox-2-inhibitor celecoxib (Celebrex((R))) and the proton-pump inhibitor omeprazole. The patient had no history of allergic reactions. The patient had already been treated previously with omeprazole without any side effects. A cutaneous biopsy confirmed the diagnosis of leucocytoclastic vasculitis. The purpuric skin lesions regressed within 3 weeks after withdrawing the two newly introduced drugs. We discuss the potential role of Celecoxib in triggering this vasculitis. PMID:12219273

  1. Vasculitis: mechanisms involved and clinical manifestations

    PubMed Central

    Guillevin, Loic; Drner, Thomas

    2007-01-01

    Systemic vasculitis, an inflammatory necrotizing disease of the blood vessel walls, can occur secondary to autoimmune diseases, including connective tissue diseases. Various pathogenic mechanisms have been implicated in the induction of vasculitis, including cell-mediated inflammation, immune complex-mediated inflammation and autoantibody-mediated inflammation. This inflammatory activity is believed to contribute to accelerated atherosclerosis, and also leads to increased risk for cardiovascular events in patients with rheumatoid arthritis and systemic lupus erythematosus. Endothelial cell activation is a common pathogenic pathway in the systemic vasculitis associated with rheumatoid arthritis and systemic lupus erythematosus, with elevated levels of endothelin-1 potentially inducing vascular dysregulation. PMID:17767747

  2. Levamisole-Induced Vasculitis with Renal Involvement.

    PubMed

    Chawdhary, Karan; Parke, Ann

    2015-01-01

    A significant amount of cocaine used in the United States today is adulterated with levamisole. In some instances, prolonged use of contaminated cocaine is associated with the development of levamisole-induced vasculitis (LIV) with features of cutaneous vasculitis and agranulocytosis along with other constitutional symptoms and arthritis. We describe a case of a crack cocaine user with LIV, who developed significant renal disease secondary to crescentic glomerulonephritis, confirmed on renal biopsy. Renal vasculitis is an uncommon feature of LIV and significantly affects clinical course and management. PMID:26263714

  3. [Primary central nervous system vasculitis].

    PubMed

    Schuster, S; Magnus, T

    2015-12-01

    Primary angiitis of the central nervous system (PACNS) is arare 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. Adefinitive 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 adiagnostic algorithm customized to the size of the affected vessels. Lastly, therapeutic options and the outcome of PACNS are briefly outlined. PMID:26589203

  4. What matters for patients with vasculitis?

    PubMed

    Novakovich, Elaine; Grayson, Peter C

    2015-06-01

    Advances in clinical care for patients with vasculitis have improved survival rates and created new challenges related to the ongoing management of chronic disease. Lack of curative therapies, burden of disease, treatment-related side effects, and fear of relapse contribute to patient-perceived reduction in quality of life. Patient-held beliefs about disease and priorities may differ substantially from the beliefs of their health care providers, and research paradigms are shifting to reflect more emphasis on understanding vasculitis from the patient's perspective. Efforts are ongoing to develop disease outcome measures in vasculitis that better represent the patient experience. Health care providers who care for patients with vasculitis should be sensitive to the substantial burdens of disease commonly experienced by patients living with the disease and should strive to provide comprehensive care directed towards the medical and biopsychological needs of these patients. PMID:25986940

  5. [Pathogenesis of large vessel vasculitis].

    PubMed

    Samson, M; Bonnotte, B

    2016-04-01

    Giant cell arteritis (GCA) and Takayasu's arteritis (TA) are two granulomatous vasculitis affecting large arteries that present specific epidemiological and clinical features. Their pathogenesis is not fully understood but major advances have been obtained during the last years, thus allowing the emergence of new therapeutic strategies. GCA and TA develop on a specific genetic background but share some similarities regarding the immunological pathways involved in their pathogenesis. The trigger of these diseases is not clearly identified but it is thought that an infectious agent could activate and lead to the maturation of dendritic cells that are localized in the adventitia of arteries. Then, the cells of the adaptative immune response are recruited and activated: CD4 T cells that polarize into Th1 and Th17 cells, cytotoxic CD8 T cells and Natural Killer cells. Furthermore, the T regulatory cells (Treg) are decreased both in GCA and TA. Humoral immune response seems also to be involved, especially in TA. Then, the cytokines produced by T lymphocytes (especially IL-17 and IFN-γ) trigger the recruitment and activation of monocytes and their differentiation into macrophages and multinuclear giant cells that produce IL-1β and IL-6 that are responsible for general symptoms of GCA and TA, and cytotoxic mediators and growth factors that trigger the remodeling of the arterial wall leading to aneurysms and ischemic manifestations of GCA an TA. PMID:26620872

  6. Biologic therapy in ANCA-negative vasculitis.

    PubMed

    Loricera, Javier; Blanco, Ricardo; Hernández, José L; Pina, Trinitario; González-Vela, M Carmen; González-Gay, Miguel A

    2015-08-01

    Standard therapeutic schemes for vasculitis are usually associated with numerous side effects and uneven clinical response. However, recent advances in understanding of the pathogenesis of these systemic diseases have resulted in the development of a group of biologic agents potentially useful in patients with vasculitis. Thus, anti-tumor necrosis factor-α drugs may be effective in patients with refractory Kawasaki disease but have failed to do so in giant cell arteritis, and their role in Takayasu arteritis is yet unclear. Preliminary reports on the use of the anti-IL6-receptor antibody, tocilizumab, in large-vessel vasculitis have been encouraging. Interferon alpha has showed positive results in hepatitis B virus-associated polyarteritis nodosa, and hepatitis C virus-induced cryoglobulinemia. Early experience with rituximab in several types of vasculitis has been quite promising, but must be confirmed in ongoing randomized clinical trials. The development of new biologic targeted therapies will probably open a hopeful future for patients with vasculitis. PMID:25828585

  7. Ischemic Retinal Vasculitis and Its Management

    PubMed Central

    Lightman, Sue

    2014-01-01

    Ischemic retinal vasculitis is an inflammation of retinal blood vessels associated with vascular occlusion and subsequent retinal hypoperfusion. It can cause visual loss secondary to macular ischemia, macular edema, and neovascularization leading to vitreous hemorrhage, fibrovascular proliferation, and tractional retinal detachment. Ischemic retinal vasculitis can be idiopathic or secondary to systemic disease such as in Behçet's disease, sarcoidosis, tuberculosis, multiple sclerosis, and systemic lupus erythematosus. Corticosteroids with or without immunosuppressive medication are the mainstay treatment in retinal vasculitis together with laser photocoagulation of retinal ischemic areas. Intravitreal injections of bevacizumab are used to treat neovascularization secondary to systemic lupus erythematosus but should be timed with retinal laser photocoagulation to prevent further progression of retinal ischemia. Antitumor necrosis factor agents have shown promising results in controlling refractory retinal vasculitis excluding multiple sclerosis. Interferon has been useful to control inflammation and induce neovascular regression in retinal vasculitis secondary to Behçet's disease and multiple sclerosis. The long term effect of these management strategies in preventing the progression of retinal ischemia and preserving vision is not well understood and needs to be further studied. PMID:24839552

  8. 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

  9. 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

  10. Clinical use of biologics in vasculitis syndromes

    PubMed Central

    Paroli, Marino

    2012-01-01

    Vasculitis syndromes are relative rare conditions but can cause significant mortality and morbidity if not treated adequately. Recent advances in immunosuppressant therapy have radically changed the course of these diseases. However, the standard therapy is not always well tolerated by patients, and some cases are refractory to treatment. New therapeutic possibilities have emerged with the use of so-called “biologics,” a new class of genetically engineered drugs used for inflammatory rheumatic diseases, including rheumatoid arthritis, ankylosing spondylitis, and psoriatic arthritis. In the present review, summarized are the most recent data on the efficacy and safety of biologics in the treatment of vasculitis syndromes that cannot be treated with standard therapy. PMID:23118526

  11. Leukocytoclastic vasculitis associated with perforated diverticular disease.

    PubMed

    Okpala, Amalachukwu Michael; Joshi, Meera; Andrews, Brian

    2016-01-01

    Leukocytoclastic vasculitis (LV) is a small vessel vasculitis associated with infections, connective tissue disease, malignancies and, rarely, gastrointestinal conditions. An association between LV and acute diverticulitis has not previously been reported. LV may be localised to the skin as a purpuric rash or might manifest with systemic involvement, such as of the joints, gastrointestinal tract or kidneys. Management of LV can be medical or surgical, based on the degree of systemic involvement. We present the case of a 56-year-old man with a 2-year history of a purpuric rash associated with diarrhoea, who presented acutely with abdominal pain. Imaging studies revealed sigmoid diverticulitis with a pericolic collection. Operative findings were purulent peritonitis secondary to perforated diverticular abscess, which was treated with a Hartmann's procedure. Postoperatively, the purpuric rash resolved rapidly on treatment with antibiotics and steroids. Histopathology of the resected bowel and skin punch biopsy confirmed sigmoid diverticulitis and LV, respectively. PMID:26791117

  12. Levamisole-Induced Vasculitis: A Characteristic Cutaneous Vasculitis Associated With Levamisole-Adulterated Cocaine.

    PubMed

    Roberts, Jordan A; Chvez-Barrios, Patricia

    2015-08-01

    Levamisole-induced vasculitis is a characteristic cutaneous vasculitis syndrome associated with the use of levamisole-adulterated cocaine. Patients will typically present with a painful, purpuric rash in a retiform or stellate pattern with or without central necrosis involving the extremities, trunk, nasal tip, digits, cheeks, and/or ears. A history of cocaine abuse can be elicited. Histologic features include microvascular thrombi and/or leukocytoclastic vasculitis involving small vessels of the superficial and deep dermis. Epidermal involvement is variably seen. Laboratory findings include leukopenia, neutropenia (including agranulocytosis), elevated erythrocyte sedimentation rate, normal coagulation studies, and positive autoantibodies including perinuclear and cytoplasmic antineutrophil cytoplasmic antibodies, antinuclear antibody, and lupus anticoagulant. Differential diagnosis includes other microscopic vasculitides, and clinical and laboratory correlation with histologic findings is essential. Lesions typically resolve with the cessation of cocaine use. Because of the treatment implications and rising incidence of this entity, rapid and accurate diagnosis is essential. PMID:26230600

  13. 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

  14. Rare Case of Vasculitis of the Hepatic Artery

    PubMed Central

    Mali, Padmavathi; Muduganti, Sudheer R.; Goldberg, Jerry

    2015-01-01

    Vasculitis is an accumulation of inflammatory leucocytes in the blood vessels with reactive damage to mural structures. Isolated vasculitis of the gastrointestinal tract without systemic involvement is rare. We report a unique case of a female patient who presented with abdominal pain, and was found, on serology, to have elevated inflammatory markers without autoantibodies. A computed tomography scan of the abdomen and pelvis was suggestive of vasculitis of the hepatic artery. To the best of our knowledge, this is the first case, to date, of vasculitis of hepatic artery. PMID:26387709

  15. Rare Case of Vasculitis of the Hepatic Artery.

    PubMed

    Mali, Padmavathi; Muduganti, Sudheer R; Goldberg, Jerry

    2015-12-01

    Vasculitis is an accumulation of inflammatory leucocytes in the blood vessels with reactive damage to mural structures. Isolated vasculitis of the gastrointestinal tract without systemic involvement is rare. We report a unique case of a female patient who presented with abdominal pain, and was found, on serology, to have elevated inflammatory markers without autoantibodies. A computed tomography scan of the abdomen and pelvis was suggestive of vasculitis of the hepatic artery. To the best of our knowledge, this is the first case, to date, of vasculitis of hepatic artery. PMID:26387709

  16. The impact of vasculitis on patients social participation and friendships

    PubMed Central

    Carpenter, Delesha M.; Meador, Amy E.; Elstad, Emily A.; Hogan, Susan L.; DeVellis, Robert F.

    2013-01-01

    Objectives Our objective is to explore how vasculitis, affects patients friendships and social participation. Methods Vasculitis patients (n=221) completed an online questionnaire that asked if, and how, relationships with friends have changed since receiving a vasculitis diagnosis. Participants written responses were imported into Atlas.ti, and two independent researchers used both structured and unstructured coding to identify themes. After reaching 100% consensus on the themes present in each participants responses, the coders determined how themes were interrelated across participants. Results Over half of patients (52%) expressed that vasculitis negatively impacted their friendships and 25% noted a negative impact on their social participation. At limes, this negative impact was related to structural changes in patients social networks due to loss of friendships. Reduced social participation was also associated with friends inability to understand vasculitis and its effects, vasculitis-related fatigue, and lifestyle changes such as not being able to drink alcohol and avoiding infection-prone events. Additionally, patients withdrew from social engagements due to fatigue or because of physical symptoms and side effects. Conclusion The unique circumstances associated with a rare chronic illness like vasculitis can create significant barriers to friendships, including loss of these relationships. Interventions designed to help patients cope with the social impact of vasculitis are implicated, especially if they increase patients ability to engage in dialogue about their illness with their friends. PMID:22325346

  17. 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

  18. Idiopathic granulomatous vasculitis: response to immunosuppressive therapy.

    PubMed Central

    Alguacil-Garcia, G F; Moreno-Requena, J; Martinez-Albadalejo, M; Hallal-Hachem, H; Gonzalez-Pina, B; de Paco-Moya, M

    1995-01-01

    A case of idiopathic granulomatous vasculitis (disseminated visceral giant cell arteritis) is described in an old woman, the seventh case of this rare disorder reported to date. The main organ affected was the liver and, to our knowledge, this is the first patient to be diagnosed while still alive and the only case to have received medical treatment. It is also the first time that muscular involvement has been documented in this condition. Cyclophosphamide treatment resulted in disappearance of symptoms and increase in weight. The patient died of an unrelated condition. Images PMID:7665707

  19. 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

  20. [Necrotizing cutaneous vasculitis with massive gastrointestinal bleeding following naproxen treatment].

    PubMed

    Horev, Amir; Etzion, Ohad; Halevy, Sima

    2010-03-01

    Cutaneous necrotizing vasculitis is usually induced by an acute infection or exposure to a drug. Cutaneous vasculitis may precede severe systemic involvement, and may end in death. Accordingly, diagnosis of cutaneous vasculitis, identification of etiological factors, follow-up for systemic involvement and treatment are important. The authors present a case study of a 58-year-old male with fever and extensive eruption involving the trunk and extremities which appeared two days after initiation of treatment with oral naproxen. The clinical and histological findings were consistent with cutaneous necrotizing leukocytoclastic vasculitis. Two events of massive upper gastrointestinal bleeding occurred during treatment with systemic corticosteroids. The possible etiological factors and the mechanisms involved in the induction of the vasculitis and the gastrointestinal bleeding are discussed. PMID:20684166

  1. Update in the diagnosis and management of pulmonary vasculitis.

    PubMed

    Frankel, Stephen K; Cosgrove, Gregory P; Fischer, Aryeh; Meehan, Richard T; Brown, Kevin K

    2006-02-01

    The term vasculitis encompasses a number of distinct clinicopathologic disease entities, each of which is characterized pathologically by cellular inflammation and destruction of the blood vessel wall, and clinically by the types and locations of the affected vessels. While multiple classification schemes have been proposed to categorize and simplify the approach to these diseases, ultimately their diagnosis rests on the identification of particular patterns of clinical, radiologic, laboratory, and pathologic features. While lung involvement is most commonly seen with the primary idiopathic, small-vessel or antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides of Wegener granulomatosis, microscopic polyangiitis, and Churg-Strauss syndrome, one should remember that medium-vessel vasculitis (ie, classic polyarteritis nodosa), large-vessel vasculitis (ie, Takayasu arteritis), primary immune complex-mediated vasculitis (ie, Goodpasture syndrome), and secondary vasculitis (ie, systemic lupus erythematosus) can all affect the lung. However, for the purpose of this review, we will focus on the ANCA-associated vasculitides. PMID:16478866

  2. Two rare cases of retinal vasculitis following vaccination.

    PubMed

    Kwok, T; Al-Bermani, A

    2013-05-01

    We write to report two rare cases of retinal vasculitis following administration of vaccinations. Both patients received recent vaccinations (within 4 weeks and 2 months respectively) and presented with unilateral visual loss due to retinal arteriolar vasculitis. Investigations did not reveal any other causes of vasculitis. The first patient's ocular inflammation settled following periocular steroid injection, whereas the second patient required the additional use of oral corticosteroid. Both patients had improved vision following treatment. Vaccinations can cause an autoimmune reaction. Systemic vasculitis has previously been described, whereas ocular vasculitic involvement is also possible but extremely rare. When seeing a patient with visual loss due to retinal vasculitis of unknown aetiology, a history of any recent vaccinations should be elicited. PMID:23728763

  3. 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

  4. 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

  5. Vasculitis and stroke due to Lyme neuroborreliosis - a review.

    PubMed

    Zajkowska, Joanna; Garkowski, Adam; Moniuszko, Anna; Czupryna, Piotr; Ptaszy?ska-Sarosiek, Iwona; Tarasw, Eugeniusz; Ustymowicz, Andrzej; ?ebkowski, Wojciech; Pancewicz, S?awomir

    2015-01-01

    Abstract Lyme neuroborreliosis (LNB) is a rare cause of vasculitis and stroke. It may manifest as subarachnoid hemorrhage, intracerebral hemorrhage, and most often ischemic stroke due to cerebral vasculitis. The vast majority of reported cases have been described by European authors. A high index of suspicion is required in patients who live or have traveled to areas with high prevalence of tick-borne diseases, and in the case of stroke-like symptoms of unknown cause in patients without cardiovascular risk factors. In this review, we also present four illustrative cases of vasculitis and stroke-like manifestations of LNB. PMID:25342573

  6. Cocaine Induced Vasculitis: Have We Found a Culprit?

    PubMed Central

    Snchez-Cruz, Alfredo; Marrero, Sylmarie; Betancourt, Jos; Andino, Myrna; Lopez, Adolfo; Gutierrez-Nuez, Jose

    2012-01-01

    Cocaine abuse is relatively common in our society. To enhance profitability and acceptability of the product, it is not uncommon for illicit drugs to undergo several processes. The Drug Enforcement Agency (DEA) has reported that seventy percent (70%) of cocaine seized at USA borders has been adulterated with levamisole, previously used as chemotherapeutic and immunomodulator for several conditions. Among the side effects of levamisole-adulterated cocaine, necrotizing vasculitis is the more dramatic. We report three cases of necrotizing vasculitis associated with antineutrophils cytoplasmic antibodies (ANCAs) positivity, linked to the use of cocaine. To our knowledge, these are the first cases of cocaine induced vasculitis reported in the Caribbean. PMID:23346447

  7. 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 encountered by clinicians. The most common underlying malignancy is generally hematologic. In these cases the presence of cytopenias and immature cells may be red flags for the diagnosis of cancer. In patients with paraneoplastic cutaneous vasculitis, the prognosis depends on the underlying neoplasia. PMID:24145696

  8. Vasculitis: an unusual manifestation in an HIV-infected patient.

    PubMed

    Manuel, Ana; Victrio, Tnia; Gomes, Constana; Martins, Telmo; Dias Neto, Antnio

    2015-01-01

    Human immunodeficiency virus (HIV) positive patients may develop vasculitis, either mediated by immunological factors or by direct vascular injury. We describe a patient who developed manifestations suggestive of extremities vasculitis with no identifiable risk factors other than HIV, Epstein Barr and Herpes Simplex Virus (HSV) type 1 co-infection. Physicians should be aware that vasculitis may have a heterogeneous presentation and occur associated with HIV infection. Although unusual, this association should be recognized for early proper treatment and prevention of ischemia. PMID:26070539

  9. Localized, single-organ vasculitis: clinical presentation and management.

    PubMed

    Atisha-Fregoso, Yemil; Hinojosa-Azaola, Andrea; Alcocer-Varela, Jorge

    2013-01-01

    The term vasculitis usually evokes a systemic disease with catastrophic outcomes; however, vasculitides may also present in a localized form, with a better prognosis when compared with their systemic counterpart. In order to avoid confusion and facilitate classification, the term single-organ vasculitis (SOV) has been proposed. Remarkably, current criteria for the classification of the vasculitis do not include the SOV term, due in part to the lack of appropriate definitions, since most data come from case series; moreover, the scarce information available is also extremely heterogeneous. This review focuses on the epidemiology, clinical course, prognosis, and suggested treatment of the SOV, with emphasis in the most recent information available. PMID:22918493

  10. Lymphocytic enteritis and systemic vasculitis in sheep.

    PubMed Central

    Rae, C A

    1994-01-01

    Lymphocytic enteritis and systemic lymphocytic vasculitis may be a new or previously unrecognized syndrome in sheep suffering from chronic wasting. Seven cases in three separate flocks were seen over an eight-year period at Veterinary Laboratory Services in Brighton, Ontario. Most of the animals were between five and twelve months of age and exhibited weight loss and inappetance, with or without diarrhea. All were Suffolks or crossbred Suffolks. In one of the flocks, there were also several sheep with lymphoma. Postmortem examination of carcasses and ancillary testing did not reveal an etiology; however, the intestinal and vascular lesions resembled those described in certain viral diseases, such as malignant catarrhal fever and Border disease, and immune-mediated diseases, such as polyarteritis nodosa. Submission for necropsy of sheep that exhibit signs of chronic wasting is encouraged, to help establish the etiology of the disease and its significance to the industry. Images Figure 1. PMID:7994703

  11. Anti-TNF-Alpha Therapy and Systemic Vasculitis

    PubMed Central

    Kaplanski, Gilles

    2014-01-01

    TNF-α is a pleiotropic cytokine, which plays a major role in the pathogenesis of numerous autoimmune and/or inflammatory systemic diseases. Systemic vasculitis constitutes a group of rare diseases, characterized by inflammation of the arterial or venous vessel wall, causing stenosis and thrombosis. Treatment of the different type of vasculitis mainly relies on steroids and immunosuppressive drugs. In case of refractory or relapsing diseases, however, a second line of treatment may be required. Anti-TNF-α drugs have been used in this setting during the last 15 years with inconsistent results. We reviewed herein the use of anti-TNF-α therapy in different kind of vasculitis and concluded that, except for Behcet's disease, this therapeutic option has not demonstrated significant improvement in the treatment of vasculitis. PMID:24719524

  12. 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, ?? 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

  13. Role of therapeutic plasmapheresis in ANCA-associated vasculitis.

    PubMed

    Walters, Giles

    2016-02-01

    Plasma exchange, or plasmapheresis, is a treatment method that developed over a period of two decades and involves the removal and replacement of a patient's circulating plasma. The aim of treatment is to remove disease-associated molecules and therefore interrupt disease progression. This article summarizes the developmental history of this treatment and then looks in more detail at data on the use of plasma exchange in treating antineutrophil antibody (ANCA)-associated vasculitis. The eight randomized trials and the Cochrane Systematic Review on treating renal vasculitis are summarized to show that plasma exchange may be effective in this disease, specifically in reducing the development of end-stage kidney disease (ESKD) by approximately 40%. The plasma exchange and glucocorticoid dosing in the treatment of anti-neutrophil cytoplasm antibody associated vasculitis (PEXIVAS) study is a currently enrolling study aiming to answer some of the outstanding questions relating to the use of this treatment in ANCA-associated vasculitis. PMID:25986911

  14. Infantile pulmonary hypertension associated with foreign body vasculitis.

    PubMed

    Bowen, J H; Woodard, B H; Barton, T K; Ingram, P; Shelburne, J D

    1981-04-01

    An infant dying with pulmonary hypertension had a pulmonary vessel foreign body vasculitis as identified by light microscopy and characterized ultrastructurally by scanning electron microscopy and energy-dispersive x-ray analysis. The inclusions were of two distinct types: those containing silicon and titanium, and others consisting of talc. The possible sources of these inclusions and the importance of considering foreign body vasculitis in the pathogenesis of clinically idiopathic pulmonary hypertension are discussed. PMID:7223722

  15. [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

  16. Genetically Distinct Subsets within ANCA-Associated Vasculitis

    PubMed Central

    Lyons, Paul A.; Rayner, Tim F.; Trivedi, Sapna; Holle, Julia U.; Watts, Richard A.; Jayne, David R.W.; Baslund, Bo; Brenchley, Paul; Bruchfeld, Annette; Chaudhry, Afzal N.; Tervaert, Jan Willem Cohen; Deloukas, Panos; Feighery, Conleth; Gross, Wolfgang L.; Guillevin, Loic; Gunnarsson, Iva; P, Lorraine Harper M.R.C; Hrušková, Zdenka; Little, Mark A.; Martorana, Davide; Neumann, Thomas; Ohlsson, Sophie; Padmanabhan, Sandosh; Pusey, Charles D.; Salama, Alan D.; Sanders, Jan-Stephan F.; Savage, Caroline O.; Segelmark, Mårten; Stegeman, Coen A.; Tesař, Vladimir; Vaglio, Augusto; Wieczorek, Stefan; Wilde, Benjamin; Zwerina, Jochen; Rees, Andrew J.; Clayton, David G.; Smith, Kenneth G.C.

    2013-01-01

    BACKGROUND Antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis is a severe condition encompassing two major syndromes: granulomatosis with polyangiitis (formerly known as Wegener’s granulomatosis) and microscopic polyangiitis. Its cause is unknown, and there is debate about whether it is a single disease entity and what role ANCA plays in its pathogenesis. We investigated its genetic basis. METHODS A genomewide association study was performed in a discovery cohort of 1233 U.K. patients with ANCA-associated vasculitis and 5884 controls and was replicated in 1454 Northern European case patients and 1666 controls. Quality control, population stratification, and statistical analyses were performed according to standard criteria. RESULTS We found both major-histocompatibility-complex (MHC) and non-MHC associations with ANCA-associated vasculitis and also that granulomatosis with polyangiitis and microscopic polyangiitis were genetically distinct. The strongest genetic associations were with the antigenic specificity of ANCA, not with the clinical syndrome. Anti–proteinase 3 ANCA was associated with HLA-DP and the genes encoding α1-antitrypsin (SERPINA1) and proteinase 3 (PRTN3) (P = 6.2×10−89, P = 5.6×10−12, and P = 2.6×10−7, respectively). Anti–myeloperoxidase ANCA was associated with HLA-DQ (P = 2.1×10−8). CONCLUSIONS This study confirms that the pathogenesis of ANCA-associated vasculitis has a genetic component, shows genetic distinctions between granulomatosis with polyangiitis and microscopic polyangiitis that are associated with ANCA specificity, and suggests that the response against the autoantigen proteinase 3 is a central pathogenic feature of proteinase 3 ANCA–associated vasculitis. These data provide preliminary support for the concept that proteinase 3 ANCA–associated vasculitis and myeloperoxidase ANCA–associated vasculitis are distinct autoimmune syndromes. (Funded by the British Heart Foundation and others.) PMID:22808956

  17. Cryoglobulinemic vasculitis with multiple digital necrosis in viral hepatitis.

    PubMed

    Mironiuc, A; Comes, Lavinia; Constantinescu, Ioana; Mironiuc, Clara; Bontea, Dana

    2008-01-01

    Cryoglobulinemic vasculitis (CV) associated with viral hepatitis C (VHC) is halfway between classical autoimmune disease and neoplasia. The extrahepatic manifestations of viral hepatitis C are various, their majority being due to mixed cryoglobulinemia. Patients diagnosed with viral hepatitis C often have mixed serum cryoglobulins, but only 5-10% of them will develop clinically manifest cryoglobulinemic vasculitis. CV symptomatology is most frequently obvious at skin level. The clinical manifestation characteristic of CV is purpura. Digital necrosis as the last stage of trophic disorders associated with CV has been rarely described in the literature. All CV types may be complicated by renal impairment. A 10-30% proportion of patients with VHC infection will develop glomerulonephritis. The treatment of cryobulinemic vasculitis associated with viral hepatitis C is focused on the reduction of viral load, the control of vasculitis symptoms using corticoids, plasmapheresis, immunosuppressive drugs, and the reduction of the amount of cryoglobulins acting on B lymphocytes. We present a rare case of cryoglobulinemic vasculitis with multiple digital necrosis associated with viral hepatitis C. PMID:19157277

  18. Preliminary classification criteria for the cryoglobulinaemic vasculitis

    PubMed Central

    De Vita, S; Soldano, F; Isola, M; Monti, G; Gabrielli, A; Tzioufas, A; Ferri, C; Ferraccioli, G F; Quartuccio, L; Corazza, L; De Marchi, G; Casals, M Ramos; Voulgarelis, M; Lenzi, M; Saccardo, F; Fraticelli, P; Mascia, M T; Sansonno, D; Cacoub, P; Tomsic, M; Tavoni, A; Pietrogrande, M; Zignego, A L; Scarpato, S; Mazzaro, C; Pioltelli, P; Steinfeld, S; Lamprecht, P; Bombardieri, S; Galli, M

    2011-01-01

    Background To develop preliminary classification criteria for the cryoglobulinaemic syndrome or cryoglobulinaemic vasculitis (CV). Methods Study part I developed a questionnaire for CV to be included in the formal, second part (study part II). Positivity of serum cryoglobulins was defined by experts as an essential condition for CV classification. In study part II, a core set of classification items (questionnaire, clinical and laboratory items, as agreed) was tested in three groups of patients and controlsthat is, group A (new patients with the CV), group B (controls with serum cryoglobulins but lacking CV) and group C (controls without serum cryoglobulins but with features which can be observed in CV). Results In study part I (188 cases, 284 controls), a positive response to at least two of three selected questions showed a sensitivity of 81.9% and a specificity of 83.5% for CV. This questionnaire was employed and validated in study part II, which included 272 patients in group A and 228 controls in group B. The final classification criteria for CV, by pooling data from group A and group B, required the positivity of questionnaire plus clinical, questionnaire plus laboratory, or clinical plus laboratory items, or all the three, providing a sensitivity of 88.5% and a specificity of 93.6% for CV. By comparing data in group A versus group C (425 controls), the same classification criteria showed a sensitivity 88.5% and a specificity 97.0% for CV. Conclusion Classification criteria for CV were developed, and now need validation. PMID:21571735

  19. Systemic vasculitis: an annual critical digest of the most recent literature.

    PubMed

    Della Rossa, Alessandra; Cioffi, Elisa; Elefante, Elena; Ferro, Francesco; Parma, Alice; Vagelli, Roberta; Talarico, Rosaria

    2014-01-01

    Herewith we provide our annual digest of the recent literature on systemic vasculitis in which we reviewed all the articles published during the last 12 months on large-, medium- and small-vessel vasculitis, and selected the most relevant studies regarding the epidemiology, pathogenesis and management of systemic vasculitis. In particular, we focused the attention on giant cell arteritis, ANCA-associated vasculitis and cryoglobulinaemia. PMID:24854379

  20. Genital vasculitis secondary to all-trans-retinoic-acid.

    PubMed

    Yanamandra, Uday; Khadwal, Alka; Nahar Saikia, Uma; Malhotra, Pankaj

    2016-01-01

    Acute promyelocytic leukaemia is among the most curable haematological malignancies after the introduction of differentiating agents (arsenic trioxide (ATO) and all-trans-retinoic-acid (ATRA)). Despite excellent cure rates, approaching 85-95% in various series, APL is associated with significant early mortality and morbidity. ATRA-related side effects partly contribute to this morbidity, which commonly presents as differentiation syndrome, pseudo tumour cerebri, dermatitis, gastrointestinal disorders, liver dysfunction (raised transaminases) and dryness of skin/eyes. Rarely, ATRA can lead to hypercalcaemia, genital vasculitis, erythaema nodosum and Sweet's syndrome. We present two cases of ATRA-associated genital vasculitis while being managed with a combination of ATO with ATRA therapy. Both patients developed these lesions despite being on prophylactic steroids (0.5 mg/kg). We also discuss the pathophysiology, clinical manifestations, differential diagnosis and treatment of genital vasculitis as a rare adverse event of ATRA. PMID:26791116

  1. 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

  2. Endoscopic and radiographic features of gastrointestinal involvement in vasculitis

    PubMed Central

    Hokama, Akira; Kishimoto, Kazuto; Ihama, Yasushi; Kobashigawa, Chiharu; Nakamoto, Manabu; Hirata, Tetsuo; Kinjo, Nagisa; Higa, Futoshi; Tateyama, Masao; Kinjo, Fukunori; Iseki, Kunitoshi; Kato, Seiya; Fujita, Jiro

    2012-01-01

    Vasculitis is an inflammation of vessel walls, followed by alteration of the blood flow and damage to the dependent organ. Vasculitis can cause local or diffuse pathologic changes in the gastrointestinal (GI) tract. The variety of GI lesions includes ulcer, submucosal edema, hemorrhage, paralytic ileus, mesenteric ischemia, bowel obstruction, and life-threatening perforation.The endoscopic and radiographic features of GI involvement in vasculitisare reviewed with the emphasis on small-vessel vasculitis by presenting our typical cases, including Churg-Strauss syndrome, Henoch-Schnlein purpura, systemic lupus erythematosus, and Behets disease. Important endoscopic features are ischemic enterocolitis and ulcer. Characteristic computed tomographic findings include bowel wall thickening with the target sign and engorgement of mesenteric vessels with comb sign. Knowledge of endoscopic and radiographic GI manifestations can help make an early diagnosis and establish treatment strategy. PMID:22442741

  3. Mycoplasmal Upper Respiratory Infection Presenting as Leukocytoclastic Vasculitis

    PubMed Central

    Rao, Mana; Agrawal, Abhinav; Parikh, Manan; Banayat, Rikka; Thomas, Maria Joana; Guo, Tianhua; Lee, Andrew

    2015-01-01

    Mycoplasma is a virulent organism that is known to primarily infect the respiratory tract; however, affection of the skin, nervous system, kidneys, heart and bloodstream has been observed in various forms, which include Stevens Johnson syndrome, erythema multiforme, toxic epidermal necrolysis, encephalitis, renal failure, conduction system abnormalities and hemolytic anemia. Small vessel vasculitis is a lesser-known complication of mycoplasma pneumonia infection. We report a case of mycoplasmal upper respiratory tract infection with striking cutaneous lesions as the presenting symptom. Mycoplasmal infection was confirmed by serology testing, skin biopsy was suggestive of leukocytoclastic vasculitis. This case brings forth an uncommon manifestation of mycoplasmal infection with extra-pulmonary affection, namely small vessel vasculitis. PMID:25874067

  4. 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

  5. Choroidal vasculitis in acute posterior multifocal placoid pigment epitheliopathy.

    PubMed Central

    Spaide, R. F.; Yannuzzi, L. A.; Slakter, J.

    1991-01-01

    A 24-year-old Caucasian female presented with acute posterior multifocal placoid pigment epitheliopathy (APMPPE) and associated infiltration round some of the larger choroidal blood vessels. This infiltration dissipated as the patient's clinical condition improved and did not induce any permanent alteration of the overlying retinal pigment epithelium. We suggest that the infiltration round the choroidal vessels was due to a choroidal vasculitis. The finding of choroidal inflammation in this case lends support to the hypothesis that choroidal vasculitis is an underlying pathological process in APMPPE. Images PMID:1793461

  6. Ischemic Retinal Vasculitis Associated with Cataract Surgery and Intracameral Vancomycin

    PubMed Central

    Lenci, Lucas T.; Chin, Eric K.; Carter, Christi; Russell, Stephen R.; Almeida, David R. P.

    2015-01-01

    Recently, there have been reports suggesting that intracameral vancomycin has been associated with retinal vasculitis; some have described this phenomenon as postoperative hemorrhagic occlusive retinal vasculitis. We present a case of a 65-year-old woman who underwent uncomplicated phacoemulsification and posterior chamber intraocular lens implantation followed by intracameral antibiotic prophylaxis. Unlike prior reports, this report demonstrates a case of mild visual reduction and minimal inflammation with subtle but complete unilateral peripheral retinal ischemia associated with cataract surgery and intracameral vancomycin, suggesting a spectrum of toxicity that may be underrecognized. PMID:26618015

  7. 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

  8. Vasculitis related to viral and other microbial agents.

    PubMed

    Teng, Gim Gee; Chatham, W Winn

    2015-04-01

    Vasculitis due to infection may occur as a consequence of the inflammation of vessel walls due to direct or contiguous infection, type II or immune complex-mediated reaction, cell-mediated hypersensitivity, or inflammation due to immune dysregulation triggered by bacterial toxin and/or superantigen production. As immunosuppressive therapy administered in the absence of antimicrobial therapy may increase morbidity and fail to effect the resolution of infection-associated vascular inflammation, it is important to consider infectious entities as potential inciting factors in vasculitis syndromes. The causality between infection and vasculitis has been established in hepatitis B-associated polyarteritis nodosa (HBV-PAN) and hepatitis C-associated (cryoglobulinemic) vasculitis (HCV-CV). The review summarizes the recent literature on the pathophysiological mechanisms and the approaches to the management of HBV-PAN and HCV-CV. Roles of other viral and microbial infections, which either manifest as vasculitic syndromes or are implicated in the pathogenesis of primary vasculitides, are also discussed. PMID:26362741

  9. 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

  10. 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

  11. Single-organ gallbladder vasculitis: characterization and distinction from systemic vasculitis involving the gallbladder. An analysis of 61 patients.

    PubMed

    Hernndez-Rodrguez, Jos; Tan, Carmela D; Rodrguez, E Ren; Hoffman, Gary S

    2014-11-01

    Systemic vasculitis (SV) involving abdominal structures usually has a poor prognosis. Gallbladder vasculitis (GV) has been reported as part of SV (GB-SV) and focal single-organ vasculitis (GB-SOV). We analyzed clinical and histologic characteristics of patients with GV to identify features that differentiate GB-SOV from the systemic forms of GV. To identify affected patients with GV we used pathology databases from our institution and an English-language PubMed search. Clinical manifestations, laboratory and histologic features, treatment administered, and outcomes were recorded. Patients were divided in 2 groups, GB-SOV and GB-SV. As in previous studies of single-organ vasculitis, GB-SOV was only considered to be a sustainable diagnosis if disease beyond the gallbladder was not apparent after a follow-up period of at least 6 months. Sixty-one well-characterized patients with GV were included (6 from our institution). There was no significant sex bias (32 female patients, 29 male). Median age was 52 years (range, 18-94 yr). GB-SOV was found in 20 (33%) and GB-SV in 41 (67%) patients. No differences were observed in age, sex frequency, or duration of gallbladder symptoms between groups. Past episodes of recurrent right-upper quadrant or abdominal pain and lithiasic cholecystitis were more frequent in GB-SOV patients, whereas acalculous cholecystitis occurred more often in GB-SV. In GB-SV, gallbladder-related symptoms occurred more often concomitantly with or after the systemic features, but they sometimes appeared before SV was fully developed (13.5%). Constitutional and musculoskeletal symptoms were reported only in GB-SV patients. Compared to GB-SOV, GB-SV patients presented more often with fever (62.5% vs 20%; p?=?0.003) and exhibited higher erythrocyte sedimentation rate levels (80??28 vs 37??25?mm/h, respectively; p?=?0.006). All GB-SV patients required glucocorticoids and 50% of them also received cytotoxic agents. Mortality in GB-SV was higher than in GB-SOV (35.5% vs 10%; p?=?0.05). Nongranulomatous inflammation with fibrinoid necrosis of medium-sized vessels occurred equally in both groups (>90%). Forms of SV affecting the gallbladder included polyarteritis nodosa (n?=?10), hepatitis B virus-associated vasculitis (n?=?8), cryoglobulinemic (essential or hepatitis C virus-associated) vasculitis (n?=?6), vasculitis associated with autoimmune diseases (n?=?6), microscopic polyangiitis (n?=?4), eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (n?=?4), IgA vasculitis (Henoch-Schnlein) (n?=?2), and giant cell arteritis (n?=?1).GV is uncommon. Its histology most often consists of a nongranulomatous necrotizing vasculitis affecting medium-sized vessels. GB-SOV is usually discovered after routine cholecystectomy performed because of the presence of local symptoms, gallstone-associated cholecystitis, and contrary to GB-SV, GB-SOV is usually not associated with systemic symptoms. Acute phase reactants and surrogate markers of autoimmunity are usually normal or negative in GB-SOV. GB-SOV does not require systemic antiinflammatory or immunosuppressive therapy; surgery is adequate to achieve cure. GB-SV always warrants immunosuppressant therapy and is associated with high mortality. The finding of GV may precede the generalized manifestations of SV. Therefore, once GV is discovered, studies to determine disease extent and a vigilant follow-up are mandatory. PMID:25500710

  12. Postoperative leucocytoclastic vasculitis in free flap mimicking venous insufficiency.

    PubMed

    Pyon, Jai Kyong; Lee, Kyeong Tae; Lim, So Young; Bang, Sa Ik; Oh, Kap Sung; Mun, Goo-Hyun

    2011-12-01

    A 29-year-old man, who had been diagnosed with myelodysplastic syndrome 1 year before, developed a wound on his thigh and knee due to necrotising fasciitis. Forty h after a free thoracodorsal artery perforator flap transfer, the colour of the flap changed from pink to purple, starting from the peripheral margin and epitheliolysis proceeded. Within a week, the colour returned to normal spontaneously and the flap survived completely. On punch biopsy, the lesion was confirmed as leucocytoclastic vasculitis, necrotising inflammatory disease of the small vessels in the dermis. This case demonstrates that leucocytoclastic vasculitis mimics postoperative venous insufficiency of a free flap; hence, differential diagnosis by careful judgement of vascular status is required. This rare occurrence needs to be considered in free-flap monitoring, especially in immunosuppressed patients. PMID:21530432

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

    PubMed

    Chaigne, Benjamin; Terrier, Benjamin; Thieblemont, Nathalie; Witko-Sarsat, Vronique; 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

  14. 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

  15. Treatment of renal manifestations of ANCA-associated vasculitis

    PubMed Central

    Galesic, Kresimir; Ljubanovic, Danica; Horvatic, Ivica

    2013-01-01

    Context Vasculitis is a clinicopathological entity characterized by inflammation and necrosis of blood vessels. Evidence Acquisitions Directory of Open Access Journals (DOAJ), Google Scholar, Pubmed (NLM), LISTA (EBSCO) and Web of Science have been searched. Results Two major autoantigens for ANCA are myeloperoxidase (MPO) and proteinase 3 (PR3), which are proteins in the primary granules of neutrophils and in the lysosomes of monocytes. They are expressed in mature neutrophils of patients with ANCA, while absent in healthy subjects. Conclusions The kidney is the most commonly affected vital organ in ANCA-associated vasculitis, and patient outcomes are largely determined by the severity of renal disease at diagnosis and by its response to treatment. PMID:24475421

  16. Central Nervous System Vasculitis: Still More Questions than Answers

    PubMed Central

    Alba, Marco A; Espígol-Frigolé, Georgina; Prieto-González, Sergio; Tavera-Bahillo, Itziar; García-Martínez, Ana; Butjosa, Montserrat; Hernández-Rodríguez, José; Cid, Maria C

    2011-01-01

    The central nervous system (CNS) may be involved by a variety of inflammatory diseases of blood vessels. These include primary angiitis of the central nervous system (PACNS), a rare disorder specifically targeting the CNS vasculature, and the systemic vasculitides which may affect the CNS among other organs and systems. Both situations are severe and convey a guarded prognosis. PACNS usually presents with headache and cognitive impairment. Focal symptoms are infrequent at disease onset but are common in more advanced stages. The diagnosis of PACNS is difficult because, although magnetic resonance imaging is almost invariably abnormal, findings are non specific. Angiography has limited sensitivity and specificity. Brain and leptomeningeal biopsy may provide a definitive diagnosis when disclosing blood vessel inflammation and are also useful to exclude other conditions presenting with similar findings. However, since lesions are segmental, a normal biopsy does not completely exclude PACNS. Secondary CNS involvement by systemic vasculitis occurs in less than one fifth of patients but may be devastating. A prompt recognition and aggressive treatment is crucial to avoid permanent damage and dysfunction. Glucocorticoids and cyclophosphamide are recommended for patients with PACNS and for patients with secondary CNS involvement by small-medium-sized systemic vasculitis. CNS involvement in large-vessel vasculitis is usually managed with high-dose glucocorticoids (giant-cell arteritis) or glucocorticoids and immunosuppressive agents (Takayasu’s disease). However, in large vessel vasculitis, where CNS symptoms are usually due to involvement of extracranial arteries (Takayasu’s disease) or proximal portions of intracranial arteries (giant-cell arteritis), revascularization procedures may also have an important role. PMID:22379458

  17. Severe Small-Vessel Vasculitis Temporally Associated With Administration of Ustekinumab.

    PubMed

    MacArthur, Kelly M; Merkel, Peter A; Van Voorhees, Abby S; Nguyen, Jennifer; Rosenbach, Misha

    2016-03-01

    Vasculitis may be caused by infection, medications, systemic diseases, malignancy, or occur as an idiopathic condition. In cases of drug-induced vasculitis, it is essential to identify and discontinue the culprit medication. As novel agents are approved through clinical trials, some rare events, including vasculitis, may not become apparent until wider use, and rigorous post-marketing surveillance for new medications is important. Physicians should consider drug-induced vasculitis on the differential for all new vasculitis diagnoses, and if the potential triggering medication is a novel medication, it is essential to rigorously investigate the potential for emerging cases of medication-associated vasculitis in all available scientific literature.

    J Drugs Dermatol. 2016;15(3):359-362. PMID:26954323

  18. [Treatment of cryoglobulinemic vasculitis associated with hepatitis C virus infection].

    PubMed

    Retamozo, Soledad; Brito-Zerón, Pilar; Ramos-Casals, Manuel

    2015-05-01

    Cryoglobulinemia is a heterogeneous systemic autoimmune disease with a wide variety of causes, symptoms and outcomes, and different etiopathogenic pathways involved in the vasculitic organ damage. The discovery of the hepatitis C virus (HCV) in 1989 changed radically the focus of research of the so-called "essential" cryoglobulinemia. Cryoglobulins can be detected in 25-30% of patients with HCV, overwhelmingly representing mixed cryoglobulins. However, only 10-15% of patients present with cryoglobulinemic vasculitis, with a broad spectrum of symptoms including mild or life-threatening manifestations. Consequently, not all patients can be uniformly treated. The key therapeutic points in HCV+ patients with cryoglobulinemic vasculitis cover different aspects. The first is to treat the underlying cause of cryoglobulinemia whenever possible, hence the use of antiviral therapies must always be considered in these patients. An individualized diagnostic approach to assess the number of organs involved and the severity of organ involvement is also essential in the therapeutic planning. This complex clinical scenario leads to an equally complex therapeutic scenario. There are three main treatment strategies for HCV-associated cryoglobulinemic vasculitis: conventional immunosuppression, antiviral treatment and biological therapies. The most recent studies are suggesting a change from the classical therapeutic approach (monotherapeutic regimens) to combination/sequential regimens, including treatments targeting the virus and those directed against the induced autoimmune disease, with the aim of blocking the various etiopathogenic pathways involved. PMID:24787686

  19. Levamisole/Cocaine Induced Systemic Vasculitis and Immune Complex Glomerulonephritis

    PubMed Central

    Garg, Lohit; Gupta, Sagar; Swami, Abhishek; Zhang, Ping

    2015-01-01

    Levamisole is an antihelminthic and immunomodulator medication that was banned by the USFDA in 1998. It has been increasingly used to adulterate cocaine due to its psychotropic effects and morphological properties. Adverse reactions including cutaneous vasculitis, thrombocytopenia, and agranulocytosis have been well described. Despite systemic vasculitis in this setting, renal involvement is uncommon. We report here a case of ANCA positive systemic vasculitis with biopsy proven immune complex mediated glomerulonephritis likely secondary to levamisole/cocaine. A 40-year-old Caucasian male with no past medical history presented with 3-week history of fatigue, skin rash, joint pains, painful oral lesions, oliguria, hematuria, worsening dyspnea on exertion, and progressive lower extremity edema. He had a history of regular tobacco and cocaine use. Lab testing revealed severe anemia, marked azotemia, deranged electrolytes, and 4.7?gm proteinuria. Rheumatologic testing revealed hypocomplementemia, borderline ANA, myeloperoxidase antibody, and positive atypical p-ANCA. Infectious and other autoimmune workup was negative. Kidney biopsy was consistent with immune mediated glomerulonephritis and showed mesangial proliferation and immune complex deposition consisting of IgG, IgM, and complement. High dose corticosteroids and discontinuing cocaine use resulted in marked improvement in rash, mucocutaneous lesions, and arthritis. There was no renal recovery and he remained hemodialysis dependent. PMID:26290761

  20. Pharmacological approaches to CNS vasculitis: where are we at now?

    PubMed

    Pagnoux, Christian; Hajj-Ali, Rula A

    2016-01-01

    The diagnosis and treatment of central nervous system (CNS) vasculitis is extremely challenging. Several conditions can mimic CNS vasculitis and require totally different treatment. CNS vasculitis, once confirmed, may result from infections or systemic diseases that will warrant specific treatments, or, more rarely, be primary and isolated (PCNSV). Prospective trials to help determine the optimal treatment for PCNSV are lacking, but data from several cohorts have provided seminal data on its management. The consensus is to use glucocorticoids as first-line agents, combined with additional immunosuppressants for the most severe cases, mainly cyclophosphamide for induction, followed by less-toxic maintenance therapy with azathioprine, methotrexate, or mycophenolate mofetil. The recent identification of PCNSV subgroups and predictors of outcomes might help in deciding the adequate treatment for each patient, keeping in mind that these data are based on a small number of patients. Other agents and biologics can be considered for patients with relapsing and/or refractory disease, but evidence is limited. In practice, the diagnosis must be re-questioned in patients with PCNSV refractory to standard treatment, especially with diagnoses not based on pathology. PMID:26559201

  1. Primary testicular necrotizing vasculitis clinically presented as neoplasm of the testicle: a case report

    PubMed Central

    2011-01-01

    We present a case of necrotizing vasculitis with the testicle as the isolated affected organ. A 25-year-old man, pretreated for epididymo-orchitis, presented with a presumed testicular neoplasm. Radical orchiectomy was performed and diagnosis of necrotizing vasculitis was established. In the absence of any other sign of systemic disease, the diagnosis of isolated necrotizing vasculitis of the testis was confirmed. Two years after the operation, the patient showed no symptoms of systemic disease. PMID:21672251

  2. 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

  3. Recent pathogenetic advances in ANCA-associated vasculitis.

    PubMed

    Pendergraft, William F; Nachman, Patrick H

    2015-06-01

    Since the discovery of anti-neutrophil cytoplasmic autoantibodies (ANCA), great strides have been made in elucidating the etiology and pathogenesis of disease. In this article, we review recent published key breakthroughs in understanding the pathogenesis of ANCA vasculitis, including some that may lead to novel therapeutics. These breakthroughs have occurred in multiple areas of investigation. A European genome-wide association study (GWAS) revealed the importance of the genetic contribution of proteinase 3 (PR3) and its endogenous inhibitor, alpha (1)-antitrypsin as well as HLA risk. Epigenetic modification of autoantigen genes appears to contribute to perpetuation of disease and possibly relapse risk. Autoantigen excision, a novel method to detect autoantibody epitopes using mass spectrometry, not only revealed pathogenic epitopes in myeloperoxidase (MPO)-ANCA vasculitis and identified unique MPO-ANCA responsible for the majority of ANCA-negative small vessel vasculitis, but has vast applicability to other autoantibody-mediated diseases. An explosion of biomarker studies has revealed circulating cytokines and alternative complement pathway products that may predict active disease. Interestingly, alternative complement pathway blockade in the murine model of disease is protective and a clinical trial in humans using an oral alternative complement pathway inhibitor is underway. Increasing clarity of the role of B and T cells in disease pathogenesis is ongoing. B cell depleting agents have shown great utility in remission induction and maintenance, and monitoring specific B cell subsets during the disease course may have predictive power for remission maintenance. Despite these substantial advances, more research is needed including, but not limited to, validation of existing discoveries. As additional novel discoveries emerge, so will novel therapies, and it is with great hope that these collective insights will ultimately lead to prevention and cure. PMID:26033562

  4. Correlation between Fluorescein Angiographic Findings and Visual Acuity in Behçet Retinal Vasculitis

    PubMed Central

    Kim, Min; Kwon, Hee Jung; Choi, Eun Young; Kim, Sung Soo; Koh, Hyoung Jun

    2015-01-01

    Purpose To identify significant fluorescein angiographic (FA) characteristics associated with visual acuity (VA) in Behçet retinal vasculitis. Materials and Methods Retrospective review of 86 eyes of 48 patients (age: 35.6±10.2 years) with Behçet retinal vasculitis were performed. VA and FA findings as well as correlation between them were assessed. Results The mean initial VA of eyes with posterior pole-involved vasculitis (63 eyes; 73.3%) was significantly worse than that of those with peripheral vasculitis (23 eye; 26.7%) (logarithm of the minimum angle of resolution VA: 0.554±0.572 vs. 0.078±0.148; p<0.0001). Subgroup analysis revealed a more severe and diffuse pattern of vascular leakage in posterior pole-involved vasculitis compared to peripheral vasculitis (p<0.0001). Retinal vascular leakage (β=0.345; p<0.0001), optic disc hyperfluorescence (β=0.147; p=0.032), and macular leakage (β=0.107; p=0.047) were significantly associated with worse initial VA. During the follow up (mean: 33.3±17.9 months), the change of leakage showed no significant correlation with change of VA in posterior pole-involved vasculitis (τ=0.199, p=0.092). Conclusion Posterior pole involvement, the degree of retinal vascular leakage, optic disc hyperfluorescence, and macular leakage are significantly associated with VA in Behçet retinal vasculitis. PMID:26069134

  5. Refractory retinal vasculitis due to sarcoidosis successfully treated with infliximab.

    PubMed

    Cruz, Boris A; Reis, Dorothy D; Araujo, Clia Aparecida A

    2007-10-01

    Sarcoidosis is a chronic disease of unknown etiology characterized by granulomatous infiltration of several organs. Up to two- thirds of patients develop ocular inflammation eventually leading to significant visual loss. Treatment includes corticosteroids and immunosuppressive agents, but refractory cases do occur. In this report, the authors describe two patients with multi-systemic sarcoidosis and refractory retinal vasculitis who experienced an excellent response to infliximab. This report emphasizes the importance of TNF-alpha in the pathophysiology of this disease and warrants further studies with anti-TNF therapy in patients with refractory ocular sarcoidosis. PMID:17520259

  6. Corticosteroids in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.

    PubMed

    Keller, Sarah F; Miloslavsky, Eli M

    2016-02-01

    Glucocorticoids (GCs) have been the cornerstone of antineutrophil cytoplasmic antibody-associated vasculitis (AAV) therapy since their advent in the 1950s. There is considerable variation in their use, both with respect to dose and duration. Given considerable treatment-related morbidity and mortality, refining the role of GCs is becoming increasingly important. This article discusses the current role of GCs in various phases of AAV treatment, including remission induction, maintenance therapy, treatment of relapses, and the use of local GCs. It discusses current controversies relating to GC use as well as research efforts that seek to reduce GC toxicity in AAV. PMID:26611553

  7. Cutaneous vasculitis in equines: a retrospective study of 72 cases.

    PubMed

    White, Stephen D; Affolter, Verena K; Dewey, Jennifer; Kass, Philip H; Outerbridge, Catherine; Ihrke, Peter J

    2009-10-01

    Cutaneous vasculitis was identified by histopathological findings in 72 equines. The most frequent clinical findings were crusts/scales and oedema of the legs with the most common underlying disease being photo-aggravated dermatitis. Common laboratory findings were anaemia, neutrophilia, hyperglycaemia and hyperglobulinaemia. Histopathological patterns were most commonly cell-poor and lymphocytic/histiocytic. While statistically supported treatment recommendations could not be made due to the large numbers of confounding factors, trimethoprim-sulfa antibiotics, corticosteroids, and/or resolution or control of the underlying disease process were the most common treatment modalities. PMID:20178500

  8. 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-? 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

  9. T CellMacrophage Interactions and Granuloma Formation in Vasculitis

    PubMed Central

    Hilhorst, Marc; Shirai, Tsuyoshi; Berry, Gerald; Goronzy, Jrg 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 macrophageT 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 cellmacrophage interactions will redefine pathogenic models in the vasculitides and provide new avenues for immunomodulatory therapy. PMID:25309534

  10. 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

  11. Multiple evanescent white dot syndrome associated with retinal vasculitis

    PubMed Central

    Takahashi, Akihiro; Saito, Wataru; Hashimoto, Yuki; Ishida, Susumu

    2015-01-01

    Purpose A recent study revealed thickening of the inner retinal layers in acute stage of multiple evanescent white dot syndrome (MEWDS); however, the pathogenesis is still unknown. We report two cases with MEWDS whose funduscopy showed obvious retinal vasculitis. Methods Case reports. Results Healthy myopic 16- and 27-year-old women were the cases under study. In both cases, funduscopic examination revealed multiple, faint, small, subretinal white dots at the posterior pole to the midperiphery and macular granularity oculus dexter. Retinal vascular sheathing was also observed at midperiphery. Late-phase fluorescein angiography revealed leakages corresponding to the vascular sheathing. Enhanced depth imaging optical coherence tomography revealed the discontinuity of the ellipsoid zone corresponding to the white dots and increased macular choroidal thickness. One month later, these white dots and retinal sheathing spontaneously resolved in both cases. Three months later, impairments of the outer retinal morphology and the visual acuity were restored. Conclusion These results suggest that retinal vasculitis possibly plays a role in the pathogenesis of thickened inner retinal layers in acute stage of MEWDS. PMID:26451125

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

    PubMed Central

    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

  13. Gemcitabine-induced large vessel vasculitis demonstrated by PET CT: a rare, important side effect.

    PubMed

    Eyre, Toby A; Gooding, Sarah; Patel, Ishita; Moore, Niall; Hatton, Chris; Collins, Graham P

    2014-06-01

    Gemcitabine is a nucleoside analogue used widely across haemato-oncology. Side effects are generally predictable, and typically consist of cytopenia, nausea, and infection. As the present case clearly demonstrates, gemcitabine is in rare cases associated with life-threatening large vessel vasculitis, which can involve the aorta. It is important to consider gemcitabine-induced vasculitis in non-specifically unwell patients with raised inflammatory markers and fever of unknown origin, with or without signs of vascular compromise. Early recognition, cessation of gemcitabine therapy, and high-dose steroids are critical for a good outcome. PET CT is valuable to diagnose large vessel vasculitis and monitor treatment response. PMID:24584910

  14. A case report: Multaq-induced leukocytoclastic vasculitis.

    PubMed

    Smith, Suzanne M; Al-Bataineh, Mohammed; Iorfido, Stephen B; Macfarlane, Jarrod

    2014-01-01

    A 71-year-old white male presented to the emergency department complaining of a worsening lower extremity rash. Thirteen days before presentation, the patient was placed on Multaq (dronedarone) for his paroxysmal atrial fibrillation. Biopsy-proven leukocytoclastic vasculitis (LV) was diagnosed, and causes for the condition other than drug-induced were investigated and ruled out. Rash has been cited as a possible side effect of Multaq; however, a literature search has revealed this to be the first documented case of likely multaq-induced LV. Other patients on Multaq therapy should be monitored for signs and symptoms of LV and be referred to an acute care setting as indicated. PMID:23011162

  15. Inflammatory myopathy as the initial presentation of cryoglobulinaemic vasculitis

    PubMed Central

    Rodríguez-Pérez, Noelia; Rodríguez-Navedo, Yerania; Font, Yvonne M; Vilá, Luis M

    2013-01-01

    Cryoglobulinaemic vasculitis is characterised by immunoglobulin deposition at low temperatures. The most common manifestations are cutaneous involvement, arthralgias, Raynaud's phenomenon, peripheral neuropathy and renal disease. Myopathy is unusual and only a few cases have been reported. Here, we present a 31-year-old woman who developed progressive muscle weakness involving upper and lower extremities, dysphagia, paraesthesias and palpable purpura. Diagnostic studies revealed elevated creatine kinase, diffuse myopathic and sensorimotor axonal neuropathy on electromyography and nerve conduction studies, and inflammatory myopathy on muscle biospsy. Cryoglobulin levels were elevated on two occasions. She responded favourably to cyclophosphamide and high-dose corticosteroids. Cyclophosphamide was continued for 1 year followed by methotrexate. Prednisone was gradually tapered and discontinued 1 year later. She remained in clinical remission after 4 years of follow-up. This case suggests that cryoglobulinaemia should be considered in the differential diagnosis of a patient presenting with inflammatory myopathy. PMID:23737595

  16. Mesenteric vasculitis in children with systemic lupus erythematosus.

    PubMed

    Fotis, Lampros; Baszis, Kevin W; French, Anthony R; Cooper, Megan A; White, Andrew J

    2016-03-01

    Lupus mesenteric vasculitis (LMV) is a severe and potentially fatal complication of systemic lupus erythematosus (SLE). Although LMV is always a consideration in adolescents and adult patients with SLE, who present with acute abdominal pain, diagnosis and management remain a great challenge. We describe the cases of five patients age 14 to 21 years old diagnosed with LMV. All five patients had active SLE and typical clinical presentation suspicious of LMV. Abdominal CT was the preferred imaging modality and was useful in four patients. Corticosteroids were the mainstay of treatment for all five patients. All five patients survived and complete remission of symptoms was achieved in four out of five patients with the addition of cyclophosphamide and in one out of five with rituximab. A review of the literature was performed including a systemic review of the case reports and case series published in the English literature over the last 20 years. PMID:25687984

  17. [McDuffie hypocomplementemic urticarial vasculitis associated with Sjgren's syndrome].

    PubMed

    Abdallah, M; Darghouth, S; Hamzaoui, S; Ben Ahmed, M; Harmel, A; Ennafaa, M; Bouslama, K; M'rad, S

    2010-07-01

    We report a patient with Sjgren's syndrome who presented with urticarial hypocomplementemic vasculitis. A 46-year-old female was admitted for assessment of ascitis. Clinical examination and computed tomographic scan disclosed evidence of multiple peripheral and intra abdominal lymph nodes. During her admission, she developed several bouts of acute angioedema and urticarial skin lesions. Minor salivary gland biopsy showed focal sialadenitis, stage IV of Chisholm. Schirmer's test was positive. Laboratory examination found low levels of C1q and high levels of C1q antibodies. Therapy with prednisone and hydroxychloroquine was initiated. Six months later, the patient presented with lower limb oedema. Urinalysis showed proteinuria (1g/day) and renal biopsy revealed membranous nephropathy with favorable outcome with corticosteroids. PMID:20362362

  18. Theodore E. Woodward Award. Do ANCA cause small vessel vasculitis?

    PubMed Central

    Falk, R. J.

    2001-01-01

    ANCA may be a pathogenetic force, but to date, support for this contention remains indirect. Active immunization with antigen or passive transfer of ANCA has not reproduced small vessel vasculitis (SVV). It is more than likely that if ANCA are pathogenetic, they are involved as one of many simultaneously occurring mechanisms acting in concert with other synergistic inflammatory mediators of disease. These include not only environmental factors such as infection or environmental toxins such as silica, but also genetic factors that are only now being described. The paradigm for this autoimmune process must include several events that occur simultaneously or sequentially, including ANCA, leukocyte activation and injured endothelium. Images Fig. 1 Fig. 2 PMID:11413776

  19. Complement system activation in ANCA vasculitis: A translational success story?

    PubMed

    Kallenberg, Cees G M; Heeringa, Peter

    2015-11-01

    The ANCA-associated vasculitides (AAV) are characterized by pauci-immune necrotizing small to medium size vessel vasculitis frequently including necrotizing crescentric glomerulonephritis. Neutrophil activation by ANCA appears a primary pathogenic event. More recently, the complement system has been shown to be involved as well. Activation of the alternative pathway of complement, at least in part via activated neutrophils, results, amongst others, in the generation of C5a, a strong chemoattractant for neutrophils. C5a is also effective in neutrophil priming, a process leading to surface expression of the ANCA antigens so enabling neutrophils to be further activated by ANCA. Both in vitro and in vivo experimental data and histopathological studies from AAV patients underscore the role of complement, and particularly of C5a, in the pathophysiology of AAV. Preliminary data show that blocking of the C5a-receptor is a promising approach in the treatment of AAV. PMID:26597208

  20. Hepatitis C-Associated Mixed Cryoglobulinemic Vasculitis Induces Differential Gene Expression in Peripheral Mononuclear Cells

    PubMed Central

    Sidharthan, Sreetha; Kim, Cheol-Woo; Murphy, Alison A.; Zhang, Xiaozhen; Yang, Jun; Lempicki, Richard A.; Sneller, Michael C.; Kottilil, Shyam

    2014-01-01

    This study examines the distinct gene expression profile of peripheral blood mononuclear cells from patients with chronic hepatitis C infection and mixed cryoglobulinemic (MC) vasculitis. Our DNA microarray analysis indicates that hepatitis C virus (HCV)-associated MC vasculitis is characterized by compromised neutrophil function, impaired chemotaxis, and increased interferon-stimulated gene (ISG) expression, contributing to overall MC pathogenesis and end-organ damage. Increased ISG expression is suggestive of an enhanced endogenous interferon gene signature. PBMC depletion assays demonstrate that this increased expression is likely due to an activation of monocytes and not a direct result of B cell expansion. Notably, this monocyte activation of ISG expression in HCV-associated MC vasculitis suggests a poor predictor status of interferon-based treatment. Further analysis of PBMC gene expression profiles before and after in vivo B cell depletion therapy is critical to completely understanding the mechanisms of MC vasculitis pathogenesis. PMID:24904592

  1. Coronary artery vasculitis: assessment with cardiac multi-detector computed tomography.

    PubMed

    Jeon, Chang Ho; Kim, Yeo Koon; Chun, Eun Ju; Kim, Jeong A; Yong, Hwan Seok; Doo, Kyung Won; Choi, Sang Il

    2015-06-01

    Coronary artery vasculitis is rare and comprises an array of inflammatory diseases. It often results in severe and life-threatening complications, including coronary artery aneurysm, coronary artery stenosis, intraluminal thrombosis, and microcirculation abnormalities. These may occur at a young age and are often silent in the early phases. Invasive coronary angiography is the gold standard for diagnosing coronary artery disease (CAD); however, multi-detector computed tomography (MDCT) is now widely regarded as a powerful non-invasive tool for the detection of CAD. It is important for clinicians to recognize the various CT findings associated with coronary artery vasculitis in order to promote accurate diagnosis and proper patient management. The purpose of this article is to present an overview of the conditions associated with coronary artery vasculitis, with an emphasis on etiology and cardiac MDCT diagnosis of CAD. Cardiac MDCT is clinically useful and can provide information for the accurate diagnosis and treatment of coronary vasculitis. PMID:25841665

  2. 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.

  3. A case of vasculitis, retinitis and macular neurosensory detachment presenting post typhoid fever

    PubMed Central

    2014-01-01

    Background Ocular and extraocular immune-mediated phenomena are known to occur following febrile illness. Vasculitis, retinitis and neurosensory detachment are not well-recognized sequelae of typhoid fever. Findings We report a case of vasculitis, retinitis and macular neurosensory detachment presenting post typhoid fever. A 27-year-old female presented with decreased vision in right eye with history of typhoid fever (treated adequately 6 weeks prior). Her best corrected visual acuity in right eye was 20/125, N36. Fundus showed a patch of vasculitis and retinitis superior to the disc associated with macular neurosensory detachment and disc pallor. With oral steroids, the inflammation resolved and visual acuity improved to 20/20 at 6 weeks. Conclusions Immune-mediated vasculitis and retinitis following typhoid fever may respond well to systemic steroids. PMID:25246983

  4. Expression Profiling of PBMC-based Diagnostic Gene Markers Isolated from Vasculitis Patients

    PubMed Central

    Kobayashi, Shigeto; Ito, Akihiko; Okuzaki, Daisuke; Onda, Hiroaki; Yabuta, Norikazu; Nagamori, Ippei; Suzuki, Kazuo; Hashimoto, Hiroshi; Nojima, Hiroshi

    2008-01-01

    Vasculitis (angiitis) is a systemic autoimmune disease that often causes fatal symptoms. We aimed to isolate cDNA markers that would be useful for diagnosing not only vasculitis but also other autoimmune diseases. For this purpose, we used stepwise subtractive hybridization and cDNA microarray analyses to comprehensively isolate the genes whose expressions are augmented in peripheral blood mononuclear cells (PBMCs) pooled from vasculitis patients. Subsequently, we used quantitative real-time polymerase chain reaction (qRTPCR) to examine the mRNA levels of each candidate gene in individual patients. These analyses indicated that seven genes exhibit remarkably augmented expression in many vasculitis patients. Of these genes, we analyzed G0/G1 switch gene 2 (G0S2) further because G0S2 expression is also enhanced in the PBMCs of patients with systemic lupus erythematodes (SLE). We generated G0S2 transgenic mice that ubiquitously overexpress human G0S2. Although we did not observe any obvious vasculitis-related histopathologic findings in these mice, these mice are unhealthy as they produce only few offspring and showed elevated serum levels of two autoimmunity-related antibodies, anti-nuclear antibody, and anti-double strand DNA antibody. Thus, our large-scale gene profiling study may help finding sensitive and specific DNA markers for diagnosing autoimmune diseases including vasculitis and SLE. PMID:18562305

  5. 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 CYC might improve the outcome. PMID:25500703

  6. Tocilizumab improves systemic rheumatoid vasculitis with necrotizing crescentic glomerulonephritis.

    PubMed

    Iijima, Takashi; Suwabe, Tatsuya; Sumida, Keiichi; Hayami, Noriko; Hiramatsu, Rikako; Hasegawa, Eiko; Yamanouchi, Masayuki; Hoshino, Junichi; Sawa, Naoki; Takaichi, Kenmei; Oohashi, Kenichi; Fujii, Takeshi; Ubara, Yoshifumi

    2015-01-01

    We report a Japanese woman with systemic rheumatoid vasculitis (SRV) complicated by necrotizing crescentic glomerulonephritis (NCGN). Rheumatoid arthritis first occurred at the age of 19 years, followed by interstitial pneumonia, hepatitis, rheumatoid nodules, mononeuritis multiplex, and hypocomplementemia in chronological order. At the age of 51 years, rapidly progressive renal failure occurred with nephrotic proteinuria, and NCGN with subepithelial deposits was revealed by renal biopsy. Severe destructive changes of multiple joints and scleritis were detected, but anti-neutrophil cytoplasmic antibody was negative on enzyme-linked immunosorbent assays and indirect immunofluorescence. SRV was diagnosed due to involvement of multiple extra-articular organs. An anti-interleukin (IL)-6 receptor antibody (tocilizumab) was started at dosage of 280 mg (8 mg/kg) monthly. After 18 months, her serum creatinine decreased from 1.7 to 1.3 mg/dL, and urinary protein excretion declined from 5.2 to 1.2 g daily. Tocilizumab may be a therapeutic option for SRV associated with NCGN. PMID:24533557

  7. B-Cell Mediated Pathogenesis of ANCA-Mediated Vasculitis

    PubMed Central

    Jennette, J. Charles; Falk, Ronald J.

    2014-01-01

    B cells and their progeny that produce and release anti-neutrophil cytoplasmic autoantibodies (ANCA) are the primary cause for an aggressive form of necrotizing small vessel vasculitis. Cytoplasmic ANCA antigens are released at the surface and in the microenvironment of cytokine-primed neutrophils. Binding of ANCA to ANCA antigens activates neutrophils by both Fc receptor engagement and direct Fab2 binding to antigen on the cell surface. ANCA-activated neutrophils release factors that induce alternative complement pathway activation, which establishes a potent inflammatory amplification loop that causes severe necrotizing vascular inflammation. The origin of the ANCA autoimmune response is unknown but appears to involve genetically determined HLA specificities that allow the autoimmune response to develop. One putative immunogenic mechanisms begins with an immune response to a peptide that is complementary to the autoantigen and evolves through an anti-idiotypic network to produce autoantibodies to the autoantigen. Another putative immunogenic mechanism begins with an immune response to a microbe-derived molecular mimic of the autoantigen resulting in antibodies that cross-react with the autoantigen. Release of neutrophil extracellular traps, apoptosis and increased granule protein expression of ANCA antigens may facilitate the initiation of an ANCA autoimmune response, augment established pathogenic ANCA production, or both. The ANCA B cell autoimmune response is facilitated by quantitatively and qualitatively impaired T cell and B cell suppression, and by release from activated neutrophils of B cell activating factors that enhance B cell proliferation and retard B cell apoptosis. PMID:24777746

  8. Hepatitis C virus-induced vasculitis: therapeutic options.

    PubMed

    Cacoub, Patrice; Terrier, Benjamin; Saadoun, David

    2014-01-01

    Hepatitis C virus (HCV) is now well recognised as the main etiologic agent of mixed cryoglobulinaemia vasculitis (cryovas). New opportunities and problems in developing therapy have therefore emerged. Antiviral therapy with pegylated interferon-α and ribavirin (plus protease inhibitor in the case of HCV genotype 1 infection) should be considered as induction therapy for HCV-cryovas with mild to moderate disease severity and activity. An early virologic response to antiviral therapy is correlated with a complete clinical response of HCV-cryovas. In patients presenting with more severe disease (ie, worsening of renal function, mononeuritis multiplex, extensive skin disease including ulcers and distal necrosis), an immunosuppression induction phase is often necessary while awaiting the generally slow response to antiviral treatments. Combination therapy with rituximab plus an optimal antiviral agent is recommended, as it may target the downstream B cell arm of autoimmunity and the viral trigger. Careful monitoring for adverse effects is mandatory, since some manifestations of HCV-cryovas, such as peripheral neuropathy or skin ulcers, may worsen with interferon-based therapy. Clinicians should be aware of the possibility of malignant lymphoma when patients develop a relapse of cryovas without virological relapse. Room for other treatment strategies is very limited. Low-dose corticosteroids may help to control minor intermittent inflammatory signs such arthralgia but do not succeed in case of major organ involvement. Other immunosuppressants should be given only in case of refractory forms of HCV-cryovas, which are frequently associated with an underlying B cell lymphoma. PMID:23921995

  9. Propylthiouracil-induced lupus-like or vasculitis syndrome

    PubMed Central

    2012-01-01

    A 27 year old female with Graves’ disease presented with fever, exertional dyspnea and polyarthralgia. Erythema nodosum had occured three months earlier. The patient declared irregular use of propylthiouracil (PTU) for the last 8 months. Neutropenia and microscopic hematuria developed in the second week of admission. Chest X-ray showed inhomogenous pulmonary opacities, left pleural effusion and cardiomegaly. Computed tomography (CT) revealed multiple subpleural nodules, left pleural effusion, pericardial effusion, enlarged mediastinal and axillary lymph nodes. Bronchoalveolar lavage (BAL) cytology demonstrated hemosiderin laden macrophages. Histopathologic examination of the transbronchial biopsy specimen revealed a nonspecific inflammation. Serum was positive for ANA, P-ANCA, MPO-ANCA, PR3-ANCA and negative for anti-ds-DNA, C-ANCA, C3, C4 and anti-histone antibody. All symptoms resolved in two months after PTU withdrawal and starting steroid treatment. The same clinical manifestations recurred when the patient used PTU erronously one month after discharge. This is a case of PTU induced-autoimmune disease in whom the accurate distinction between drug-induced-lupus (DIL) and vasculitis was not possible due to the significant overlap of clinical and laboratory findings causing a significant diagnostic challenge for the chest physician. PMID:22958435

  10. 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

  11. 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

  12. Mast Cells Contribute to Peripheral Tolerance and Attenuate Autoimmune Vasculitis

    PubMed Central

    Gan, Poh-Yi; Summers, Shaun A.; Ooi, Joshua D.; OSullivan, Kim M.; Tan, Diana S.Y.; Muljadi, Ruth C.M.; Odobasic, Dragana; Kitching, A. Richard

    2012-01-01

    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 (KitW-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-10deficient mast cells led to enhanced autoimmunity to myeloperoxidase and greater disease severity compared with reconstitution with IL-10intact 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

  13. New Features of Disease after Diagnosis in Six Forms of Systemic Vasculitis

    PubMed Central

    Grayson, Peter C.; Cuthbertson, David; Carette, Simon; Hoffman, Gary S.; Khalidi, Nader A.; Koening, Curry L.; Langford, Carol A.; Maksimowicz-McKinnon, Kathleen; Monach, Paul A.; Seo, Philip; Specks, Ulrich; Ytterberg, Steven R.; Merkel, Peter A.

    2015-01-01

    Objective To quantify the occurrence of features of vasculitis that initially present after diagnosis in 6 types of primary vasculitis. Methods Standardized collection of data on 95 disease manifestations in 6 vasculitides, including granulomatosis with polyangiitis (Wegener's, GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (Churg-Strauss, EGPA), polyarteritis nodosa (PAN), giant cell arteritis (GCA), and Takayasu's arteritis (TAK), was performed within a set of multicenter longitudinal, observational cohorts. For each form of vasculitis, the frequency of disease-specific manifestations at diagnosis was compared to the cumulative frequency of each manifestation. The percentage of patients who initially developed severe manifestations after diagnosis, defined as organ- or life-threatening in the small and medium vessel vasculitides (GPA, MPA, EGPA, PAN) and as ischemic/vascular in the large vessel vasculitides (GCA, TAK), was reported. Results Out of 838 patients with vasculitis, 490 (59%) experienced ? 1 new disease manifestation after diagnosis. On average, patients with vasculitis experienced 1.3 new manifestations after diagnosis (GPA - 1.9, MPA - 1.2, EGPA - 1.5, PAN - 1.2, GCA - 0.7, TAK - 1.0). New severe manifestations occurred after diagnosis in 224 (27%) out of 838 patients (GPA - 26%, MPA - 19%, EGPA - 21%, PAN - 23%, GCA - 24%, and TAK - 44%). Timing of onset of new manifestations was not significantly associated with disease duration. Conclusion A majority of patients with vasculitis develop new disease features after diagnosis, including a substantial number of new, severe manifestations. Ongoing assessment of patients with established vasculitis should remain broad in scope. PMID:23908447

  14. Incidence of Leukocytoclastic Vasculitis, 19962010: A Population-Based Study in Olmsted County, Minnesota

    PubMed Central

    Arora, Amrita; Wetter, David A.; Gonzalez-Santiago, Tania M.; Davis, Mark D. P.; Lohse, Christine M.

    2014-01-01

    Objective To determine the population-based incidence of leukocytoclastic vasculitis (LCV). Patients and Methods Retrospective population-based study of all Olmsted County, Minnesota, residents with a skin biopsyproven diagnosis of LCV from January 1, 1996, through December 31, 2010. Results A total of 84 patients (mean age at diagnosis, 48.3 years) with newly diagnosed, skin biopsyproven LCV (43 women, 41 men) were identified. The incidence rate (age and sex adjusted to the 2000 US white population) was 4.5 per 100,000 person-years (95% confidence interval, 3.55.4). The incidence of LCV increased significantly with age at diagnosis (P<.001) and did not differ between female and male patients. Subtypes of LCV were cutaneous small-vessel vasculitis (CSVV), 38 patients (45%); IgA vasculitis, 25 (30%); urticarial vasculitis, 10 (12%); cryoglobulinemic vasculitis, 3 (4%); and antineutrophil cytoplasmic antibody (ANCA)associated vasculitis, 8 (10%). LCV was idiopathic in 29 of 38 CSVV patients (76%) and 24 of 25 IgA vasculitis patients (96%). Thirty-nine of 84 patients (46%) had systemic involvement, with the renal system most commonly involved (17/39; 44%). Twenty-four of 80 patients (30%) with follow-up data available had recurrent disease. Compared with the Minnesota white population, observed survival in the incident LCV cohort was significantly poorer than expected (P<.001), including the subset of patients with idiopathic CSVV (P=.03). Conclusion The incidence of LCV was higher than that in previously published studies. Idiopathic LCV was more common in our population-based cohort than previously described. Overall survival was significantly poorer and should be explored further in future studies. PMID:24981218

  15. Neuroborreliosis-associated cerebral vasculitis: long-term outcome and health-related quality of life.

    PubMed

    Back, Tobias; Grünig, Steffi; Winter, Yaroslav; Bodechtel, Ulf; Guthke, Kersten; Khati, Diana; von Kummer, Rüdiger

    2013-06-01

    Neuroborreliosis affects the nervous system after systemic infection with the spirochete Borrelia burgdorferi. Previously, cerebral vasculitis has been regarded as an extremely rare complication of neuroborreliosis. The data on the long-term outcome in patients with cerebral vasculitis due to neuroborreliosis are limited. The objective of this study was to perform a longitudinal analysis of cases of neuroborreliosis-associated cerebral vasculitis. We recruited all patients (n = 11) diagnosed with neuroborreliosis-associated in three neurological departments in an East German region. Inclusion criteria were sudden neurological deficits, magnetic resonance (MR) imaging findings that conform to cerebral ischemia or brain infarction, intrathecal synthesis of borrelia-specific antibodies, and non-atherosclerotic pathology of brain supplying arteries. Vasculitic changes were detected by digital subtraction angiography, MR angiography and/or transcranial Doppler ultrasound. Outcomes were measured by the modified Rankin scale (mRS) and EuroQoL Index. Cerebral vasculitis is a rare complication of Lyme disease (0.3% of all cases in the endemic area). Ten out of 11 patients diagnosed with neuroborreliosis-associated vasculitis cerebral vasculitis using clinical, radiological and immunological criteria developed ischemic stroke or transient ischemic attacks (TIA), 7 patients had recurrent stroke. Vasculitic alterations could be demonstrated in 8 patients that all except one developed ischemic lesions. The median mRS was 3 (range 0-4) at admission and 2 (range 0-6) at discharge. The posterior circulation was affected in 8 of 11 patients; thrombosis of the basilar artery was detected in 2 patients, one died in the acute stage. Neuroborreliosis can cause recurrent stroke or TIA on the basis of cerebral vasculitis. Lumbar puncture is needed for detection of this potentially life-threatening condition. Early recognition and adequate therapy would possibly improve outcome. PMID:23329377

  16. The Place of Immunotherapy in the Management of HCV-Induced Vasculitis: An Update

    PubMed Central

    Chiche, Laurent; Bataille, Stanislas; Kaplanski, Gilles; Jourde, Noemie

    2012-01-01

    Patients with chronic hepatitis C virus (HCV) can develop systemic cryoglobulinemic vasculitis. Combination of pegylated-interferon α and ribavirin is the first-line treatment of this condition. However, in case of severe or life-threatening manifestations, absence of a virological response, or autonomized vasculitis, immunotherapy (alone or in addition to the antiviral regimen) is necessary. Rituximab is to date the only biologic with a sufficient level of evidence to support its use in this indication. Several studies have demonstrated that rituximab is highly effective when cryoglobulinaemic vasculitis is refractory to antiviral regimen, that association of rituximab with antiviral regimen may induce a better and faster clinical remission, and, recently, that rituximab is more efficient than traditional immunosuppressive treatments. Some issues with regard to the optimal dose of rituximab or its use as maintenance treatment remain unsolved. Interestingly, in balance with this anti-inflammatory strategy, a recent pilot study reported the significant expansion of circulating regulatory T lymphocytes with concomitant clinical improvement in patients with refractory HCV-induced cryoglobulinaemic vasculitis using low dose of subcutaneous interleukin-2. This paper provides an updated overview on the place of immunotherapy, especially biologics, in the management of HCV-induced cryoglobulinaemic vasculitis. PMID:22927871

  17. Sjogren's syndrome complicated with IgA nephropathy and leukocytoclastic vasculitis.

    PubMed

    Tsai, Tsung-Chang; Chen, Chen-Yin; Lin, Wei-Tung; Lee, Wei-Jen; Chen, Hung-Chun

    2008-01-01

    We report a case of primary Sjogren's syndrome (SS) with cutaneous leukocytoclastic vasculitis and IgA nephropathy. The accurate diagnosis of SS was established based on objective signs and symptoms of ocular and oral dryness, a characteristic appearance of a biopsy sample from a minor salivary gland, and the presence of anti-SS-A autoantibody. A second autoimmune disorder was not present, so the diagnosis of primary SS was established. A histologic finding of skin biopsy of purpuric lesion was typical for leukocytoclastic vasculitis. Renal biopsy was performed for nephrotic range proteinuria. The pathologic finding of renal biopsy was IgA glomerulonephritis with crescent formation. The patient was treated with small doses of glucocorticoids and maintenance hemodialysis. Leukocytoclastic vasculitis is one of the most characteristic extraglandular manifestations of SS. However, IgA nephropathy associated with SS and leukocytoclastic vasculitis is a rare finding. SS patients with glomerulonephritis present a more diverse outcome, even requiring hemodialysis. Therefore, renal biopsy is warranted in SS with glomerulonephritis and systemic vasculitis. PMID:18704825

  18. Genome-Wide Association Study of Hepatitis C Virus- and Cryoglobulin-Related Vasculitis

    PubMed Central

    Zignego, Anna Linda; Wojcik, Genevieve L.; Cacoub, Patrice; Visentini, Marcella; Casato, Milvia; Mangia, Alessandra; Latanich, Rachel; Charles, Edgar; Gragnani, Laura; Terrier, Benjamin; Piazzola, Valeria; Dustin, Lynn B.; Khakoo, Salim I.; Busch, Michael P.; Lauer, George M.; Kim, Arthur Y.; Alric, Laurent; Thomas, David L.; Duggal, Priya

    2014-01-01

    Objectives The host genetic basis of mixed cryoglobulin vasculitis is not well understood and has not been studied in large cohorts. A genome-wide association study was conducted among 356 HCV RNA positive individuals with cryoglobulin-related vasculitis and 447 ethnically-matched, HCV RNA positive controls. Methods All cases had both serum cryoglobulins as well as a vasculitis syndrome. A total of 899,641 markers from the Illumina HumanOmni1-Quad chip were analyzed using logistic regression adjusted for sex, as well as genetically-determined ancestry. Replication of select single nucleotide polymorphisms (SNPs) was conducted using 91 cases and 180 controls, adjusting for sex and country of origin. Results The most significant associations were identified on chromosome 6 near the NOTCH4 and MHC class II genes. A genome-wide significant association was detected on chromosome 6 at SNP rs9461776 (OR= 2.16, p=1.16E-07) between HLA-DRB1 and DQA1: this association was further replicated in additional independent samples (meta-analysis p=7.1×10−9). Conclusions A genome-wide significant association with cryoglobulin related vasculitis was identified with SNPs near NOTCH4 and MHC Class II genes. The two regions are correlated and it is difficult to disentangle which gene is responsible for the association with MC vasculitis in this extended MHC region. PMID:25030430

  19. An Interferon-Induced Digital Vasculitis-Like Syndrome: A Case Report.

    PubMed

    Hamidi, Oksana; Reiser, Jochen; Hasler, Scott

    2014-04-11

    This report describes a patient with chronic hepatitis C undergoing therapy with interferon (IFN) alpha who developed bilateral ischemia of his fingers. We present a 43-year-old man with a failed renal transplant and chronic hepatitis C. He was treated with 6 months of IFN therapy with good reduction of his viral load. He presented with 2 days of pain and swelling in the second digits of both hands. Workup for extrahepatic manifestations of hepatitis C was initiated including assessment for vasculitis because of cryoglobulin- and noncryoglobulin-related causes. Extensive assessment with invasive and noninvasive vascular testing was performed. His workup for vasculitis did not reveal any specific reasons for the ischemic changes. Angiography of his fingers showed mild stenotic changes but no evidence of systemic vasculitis. IFN therapy was stopped and over several weeks his symptoms resolved. The ischemic changes were attributed to IFN therapy. The patient in this report is unique because although IFN has been historically reported to cause a variety of vascular syndromes, the reported experience in hepatitis C patients is small. In addition, the likelihood of encountering vasculitis and vasculitis-like syndromes in patients with hepatitis C is significant, and the increasing use of IFN in this population makes drug-induced vascular changes an essential consideration in this subset of patients. PMID:24732907

  20. Clinical and serological features of severe vasculitis in rheumatoid arthritis: prognostic implications.

    PubMed Central

    Geirsson, A J; Sturfelt, G; Truedsson, L

    1987-01-01

    Sixteen patients with classic rheumatoid arthritis (RA) complicated by severe vasculitis were studied and compared with a matched control group of 16 RA patients without vasculitis. Seven of the patients with vasculitis died within 4 to 120 months (median 32 months) after developing vasculitic symptoms. Gangrene of digits and extremities, bowel ulcers or bowel perforation, or both, and cardiac involvement were more common among the patients who died than among those with a more favourable course. The present data suggest that large vessel vasculitis in RA is associated with high frequency of arteriosclerotic vascular disease. The serum concentrations of complement components C3 and C4 were lower, and concentrations of IgM rheumatoid factor, complement activating rheumatoid factor, and C1q binding immune complexes (C1q solid and C1q fluid phase assay) were significantly higher among vasculitic patients than in the control group. Laboratory data provided little prognostic information with regard to rheumatoid vasculitis, with the exception that IgM and IgG rheumatoid factors were significantly higher among patients with fatal course of disease than in those who achieved remission. PMID:3689000

  1. Urinary Biomarkers in Relapsing Antineutrophil Cytoplasmic Antibody-associated Vasculitis

    PubMed Central

    Lieberthal, Jason G.; Cuthbertson, David; Carette, Simon; Hoffman, Gary S.; Khalidi, Nader A.; Koening, Curry L.; Langford, Carol A.; Maksimowicz-McKinnon, Kathleen; Seo, Philip; Specks, Ulrich; Ytterberg, Steven R.; Merkel, Peter A.; Monach, Paul A.

    2015-01-01

    Objective Glomerulonephritis (GN) is common in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), but tools for early detection of renal involvement are imperfect. We investigated 4 urinary proteins as markers of active renal AAV: alpha-1 acid glycoprotein (AGP), kidney injury molecule-1 (KIM-1), monocyte chemoattractant protein-1 (MCP-1), and neutrophil gelatinase-associated lipocalin (NGAL). Methods Patients with active renal AAV (n = 20), active nonrenal AAV (n = 16), and AAV in longterm remission (n = 14) were identified within a longitudinal cohort. Urinary biomarker concentrations (by ELISA) were normalized for urine creatinine. Marker levels during active AAV were compared to baseline remission levels (from 14 visits) for each patient. Areas under receiver-operating characteristic curves (AUC), sensitivities, specificities, and likelihood ratios (LR) comparing disease states were calculated. Results Baseline biomarker levels varied among patients. All 4 markers increased during renal flares (p < 0.05). MCP-1 discriminated best between active renal disease and remission: a 1.3-fold increase in MCP-1 had 94% sensitivity and 89% specificity for active renal disease (AUC = 0.93, positive LR 8.5, negative LR 0.07). Increased MCP-1 also characterized 50% of apparently nonrenal flares. Change in AGP, KIM-1, or NGAL showed more modest ability to distinguish active renal disease from remission (AUC 0.710.75). Hematuria was noted in 83% of active renal episodes, but also 43% of nonrenal flares and 25% of remission samples. Conclusion Either urinary MCP-1 is not specific for GN in AAV, or it identifies early GN not detected by standard assessment and thus has potential to improve care. A followup study with kidney biopsy as the gold standard is needed. PMID:23547217

  2. Vasculitic emergencies in the intensive care unit: a special focus on cryoglobulinemic vasculitis

    PubMed Central

    2012-01-01

    Vasculitis is characterized by the infiltration of vessel walls by inflammatory leukocytes with reactive damage and subsequent loss of vessel integrity. The clinical course of systemic vasculitis may be punctuated by acute life-threatening manifestations that require intensive care unit (ICU) admission. Furthermore, the diagnosis may be established in the ICU after admission for a severe inaugural symptom, mostly acute respiratory failure. Among the systemic vasculitides, cryoglobulinemic vasculitis (CV) has been rarely studied in an ICU setting. Severe CV-related complications may involve the kidneys, lungs, heart, gut, and/or central nervous system. The diagnosis of CV in the ICU may be delayed or completely unrecognized. A high level of suspicion is critical to obtain a timely and accurate diagnosis and to initiate appropriate treatment. We describe severe acute manifestations of CV based on six selected patients admitted to our ICU. That all six patients survived suggests the benefit of prompt ICU admission of patients with severe CV. PMID:22812447

  3. Intravascular immunity as a key to systemic vasculitis: a work in progress, gaining momentum

    PubMed Central

    Ramirez, G A; Maugeri, N; Sabbadini, M G; Rovere-Querini, P; Manfredi, A A

    2014-01-01

    Vascular inflammation contributes to the defence against invading microbes and to the repair of injured tissues. In most cases it resolves before becoming apparent. Vasculitis comprises heterogeneous clinical entities that are characterized by the persistence of vascular inflammation after it has served its homeostatic function. Most underlying mechanisms have so far remained elusive. Intravascular immunity refers to the surveillance of the vasculature by leucocytes that sense microbial or sterile threats to vessel integrity and initiate protective responses that entail most events that determine the clinical manifestations of vasculitis, such as end-organ ischaemia, neutrophil extracellular traps generation and thrombosis, leucocyte extravasation and degranulation. Understanding how the resolution of vascular inflammation goes awry in patients with systemic vasculitis will facilitate the identification of novel pharmacological targets and bring us a step closer in each patient to the selection of more effective and less toxic treatments. PMID:24128276

  4. 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

  5. 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

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

    PubMed Central

    Figueiredo, Lgia; Rothwell, Renata; Brando, 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

  7. Finger necrosis due to cryoglobulinemic vasculitis in association with membranous nephropathy

    PubMed Central

    Rodriguez-Castro, Carlos E.; Osvaldo, Padilla; Saifuddin, Fátima; Siddiqui, Tariq; Fan, Jerry; Mukherjee, Debabrata; Pema, Kanchan; Abbas, Aamer

    2015-01-01

    Cryoglobulinemic vasculitis is a small vessel vasculitis that has been associated with chronic infections and autoimmune, lymphoproliferative, and neoplastic disorders. When no significant etiological factors are identified, it is called essential mixed cryoglobulinemia. A detailed and thorough laboratory investigation is required to exclude all possible causes of cryoglobulin formation. Although cryoglobulin testing is simple, careful temperature regulation is needed to avoid false-negative results. Consensus diagnosis should be developed and implemented for appropriate cryoglobulin detection and accurate clinical diagnosis for cryoglobulinemic vasculitis. Here we present an interesting, first-ever case report of a 54-year-old Hispanic-American woman with essential mixed cryoglobulinemia presenting with significant digital necrosis in association with membranous nephropathy. PMID:25552807

  8. Transduction of a Foreign Histocompatibility Gene into the Arterial Wall Induces Vasculitis

    NASA Astrophysics Data System (ADS)

    Nabel, Elizabeth G.; Plautz, Gregory; Nabel, Gary J.

    1992-06-01

    Autoimmune vasculitis represents a disease characterized by focal inflammation within arteries at multiple sites in the vasculature. Therapeutic interventions in this disease are empirical and often unsuccessful, and the mechanisms of immune injury are not well-defined. The direct transfer of recombinant genes and their expression in the arterial wall provides an opportunity to explore the pathogenesis and treatment of vascular disease. In this report, an animal model for vasculitis has been developed. Inflammation has been elicited by direct gene transfer of a foreign class I major histocompatibility complex gene, HLA-B7, to specific sites in porcine arteries. Transfer and expression of this recombinant gene was confirmed by a polymerase chain reaction and immunohistochemistry, and cytolytic T cells specific for HLA-B7 were detected. These findings demonstrate that expression of a recombinant gene in the vessel wall can induce a focal immune response and suggest that vessel damage induced by cell-mediated immune injury can initiate vasculitis.

  9. Intravascular crystal deposition: an early clue to the diagnosis of type 1 cryoglobulinemic vasculitis.

    PubMed

    Gammon, Bryan; Longmire, Michelle; DeClerck, Brittney

    2014-09-01

    Cutaneous small vessel vasculitis (CSVV) is a nonspecific finding with an extensive differential diagnosis. It is critically important to distinguish skin-limited presentations of CSVV from severe life-threatening systemic vasculitides presenting with CSVV as an initial manifestation. It can be challenging to determine which patients presenting with CSVV are at risk for systemic disease. Standard histopathologic evaluation, direct immunofluorescence, and serologic evaluation is typically required to exclude a systemic vasculitis. Type 1 cryoglobulinemia may rarely present with CSVV. Herein, we report a case of type 1 cryoglobulinemia in the setting of occult multiple myeloma. CSVV with prominent intravascular crystal formation was noted. The presence of intravascular crystals in the setting of CSVV may represent an important early clue to the diagnosis of type 1 cryoglobulinemic vasculitis. PMID:25147987

  10. CMV disease complicating induction immunosuppressive treatment for ANCA-associated vasculitis.

    PubMed

    Tollitt, James; O'Riordan, Edmond; Poulikakos, Dimitrios

    2016-01-01

    We present a case of a 71-year-old woman who initially presented with renal-limited antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Following standard therapy with cyclophosphamide, steroids and plasma exchange, her renal function began to improve. However, despite appropriate treatment, her renal function subsequently deteriorated and she suffered haemoptysis. Owing to diagnostic uncertainty, bronchoscopy and a repeat renal biopsy were performed. The bronchoscopy washings demonstrated positivity for cytomegalovirus (CMV) DNA, and in combination with a positive serum CMV PCR, immunosuppression was withheld. Treatment with ganciclovir was started. Repeat renal biopsy demonstrated active vasculitis and, following successful treatment of CMV disease, immunosuppression was re-started alongside prophylactic valganciclovir. This resulted in a successful outcome for the patient. Pulmonary CMV disease may mimic pulmonary disease associated with vasculitis, posing a diagnostic challenge to clinicians. We recommend a low threshold when testing for CMV in these patients. PMID:26907821

  11. IgA vasculitis associated with anti-TNF-? inhibitors in a patient with Crohn's disease.

    PubMed

    Nishikawa, Jun; Hosokawa, Ayumu; Akashi, Momoko; Mihara, Hiroshi; Nanjo, Sohachi; Yoshita, Hiroki; Ando, Takayuki; Kajiura, Shinya; Fujinami, Haruka; Sugiyama, Toshiro

    2015-01-01

    Anti-TNF-? inhibitors have been widely used in the treatment of inflammatory bowel disease. Although they have good clinical efficacy and tolerance, they remain a matter of concern because they cause drug-induced autoimmune disorders as side effects. Here, we report a case of a patient with Crohn's disease who developed IgA vasculitis after infliximab and adalimumab treatment. A 17-year-old male with Crohn's disease who had received scheduled infliximab treatment for the preceding 19 months complained of purpura on his lower limbs. He was diagnosed with infliximab-induced IgA vasculitis. Switching infliximab to adalimumab resulted in rapid improvement of the condition. However, 21 months after switching to adalimumab, his purpura recurred. Drug-induced IgA vasculitis is a rare complication caused by infliximab and adalimumab; however, diagnosis in the early phase and appropriate management of patients receiving anti-TNF-? inhibitors is critical to a successful patient outcome. PMID:26440688

  12. 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

  13. VZV retinal vasculitis without systemic infection: diagnosis and monitoring with quantitative Polymerase Chain Reaction.

    PubMed

    Wimmersberger, Yves; Gervaix, A; Baglivo, Edoardo

    2010-02-01

    To report a case of unilateral varicella zoster virus (VZV) retinal vasculitis aspect in an immunocompetent child without systemic infection. Clinically, no signs of retinal necrosis or frosted branch vasculitis were present. This is an observational case report. Quantitative PCR was performed on the aqueous humor (AH) using primers specific for herpes virus (cytomegalovirus, Epstein-Barr virus, herpes simplex virus 1-2, and VZV). The patient was treated with intravenous acyclovir, intravitreous ganciclovir, and oral valacyclovir. A positive quantitative PCR result was found for VZV DNA (1.72 x 10(6) viral copies/ml) in the AH. After 6 months, PCR of the AH was negative. Herpes viruses are involved in the pathogenesis of isolated retinal vasculitis. This case demonstrates that quantitative PCR is useful to detect viral DNA in AH and to monitor the viral activity and the therapeutic response. PMID:18853105

  14. 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

  15. Etanercept treatment-related c-ANCA-associated large vessel vasculitis.

    PubMed

    Ginsberg, Shira; Rosner, Itzhak; Slobodin, Gleb; Boulman, Nina; Rozenbaum, Michael; Kaly, Lisa; Beyar, Ofrat Katz; Rimar, Doron

    2016-01-01

    Anti-tumor necrosis factor (TNF) agents have become central players in the management of autoimmune and rheumatic disease. With the wide use of anti-TNF agents today, we have become aware of rare autoimmune complications as systemic lupus erythematosus and psoriasis, yet rarely has large vessels vasculitis been described. We herein describe a case of cytoplasmic anti-neutrophil cytoplasmic antibody (c-ANCA) (with myeloperoxidase (MPO) antibodies)-associated large vessel vasculitis (aortitis) that developed during anti-TNF treatment for ankylosing spondylitis. Awareness of this rare, but serious, adverse event of these commonly used agents in rheumatic diseases is of importance. PMID:26626631

  16. Secondary Syphilis with Nodular Vasculitis Mimicking Behçet's Disease

    PubMed Central

    Jo, Jaemin; Kim, Jae Wang; Kim, Jinseok; Yu, Jung Re

    2013-01-01

    Although, erythema nodosum is a common skin manifestation associated with syphilis, nodular vasculitis is a rare feature. Here, we describe a case of a 22-year-old, human immunedeficiency virus negative, non-immunocompromised man who developed recurrent oral and scrotal ulcers with nodular lesions of the lower extremitie. Behçet's disease was initially suspected, however, his serologic test for syphilis was positive, and he was thus diagnosed with secondary syphilis, with a skin biopsy showing nodular vasculitis. The patient was treated with benzathine penicillin, and the skin lesions disappeared after treatment. PMID:24475361

  17. Pachymeningitis as a manifestation of ANCA-associated vasculitis: a care report and literature review

    PubMed Central

    Li, Sheyu; Tang, Honghu; Rong, Xia; Huang, Xiangyang; Li, Qianrui

    2015-01-01

    Pachymeningitis is a rare cause of headache characterized by dura mater thickening with various origins. We present a 67-year-old male with cranial and spinal pachymeningitis and MPO-ANCA-associated vasculitis, which is considered as the cause of pachymeningitis of this patient. After revision of other 32 reported cases, our study suggested cranial and spinal pachymeningitis could be a manifestation of ANCA-associated vasculitis. Thus, it is important to screen other manifestations of ANCA-associated vasulitis as soon as pachymeningitis was suspected. PMID:26131256

  18. Management of Large-Vessel Vasculitis With FDG-PET

    PubMed Central

    Soussan, Michael; Nicolas, Patrick; Schramm, Catherine; Katsahian, Sandrine; Pop, Gabriel; Fain, Olivier; Mekinian, Arsene

    2015-01-01

    Abstract We aimed to clarify the role of 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) in the management of large-vessel vasculitis (LVV), focusing on 3 issues which are as follows: describe and determine the different FDG-PET criteria for the diagnosis of vascular inflammation, the performance of FDG-PET for the diagnosis of large-vessel inflammation in giant cell arteritis (GCA) patients, and the performance of FDG-PET to evaluate the disease inflammatory activity in Takayasu arteritis (TA) patients. MEDLINE, Cochrane Library, and EMBASE database were searched for articles that evaluated the value of FDG-PET in LVV, from January 2000 to December 2013. Inclusion criteria were American College of Rheumatology criteria for GCA or TA, definition PET positivity threshold, and >4 cases included. Sensitivity (Se) and specificity (Sp) of FDG-PET for the diagnosis of large-vessel inflammation were calculated from each included individual study, and then pooled for meta-analysis with a random-effects model. Twenty-one studies (413 patients, 299 controls) were included in the systematic review. FDG-PET showed FDG vascular uptake in 70% (288/413) of patients and 7% (22/299) of controls. Only vascular uptake equal to or higher than the liver uptake was significantly different between GCA/TA patients and controls (P?

  19. Life-threatening ANCA-positive vasculitis associated with rickettsial infection.

    PubMed

    Nickerson, Ashley; Marik, Paul Ellis

    2012-01-01

    We present a 47-year-old Caucasian fire fighter who developed multisystem organ failure in the setting of a positive antineutrophil cytoplasmic autoantibody (myeloperoxidase) as well as confirmed Rocky Mountain spotted fever by skin biopsy PCR. This case provided a diagnostic challenge, a rare association of a Rickettsia infection and autoimmune vasculitis as well as a unique management approach. PMID:22669870

  20. Cocaine-Induced Delayed Recurrent Vasculitis: A 4-Year Follow-Up

    PubMed Central

    Yogarajah, Meera; Pervil-Ulysse, Mona; Sivasambu, Bhradeev

    2015-01-01

    Patient: Female, 51 Final Diagnosis: Cocaine induced vasculitis Symptoms: Medication: Clinical Procedure: None Specialty: Rheumatology Objective: Rare disease Background: Cocaine is a highly abused substance in United States with almost 70 % of cocaine adulterated with levamisole. It is known to cause vasculitis involving multiple organs due to its direct toxic effect and by the contribution of levamisole or a combined effect of both. Case Report: A 51-year-old woman complained of painful erythematous rash in her hands and lower extremities that started few hours after smoking cocaine and progressed to blistering dark lesions in her lower extremities. She denied any other systemic complaints. Although she has been smoking cocaine for more than 35 years, these skin eruptions started only 4 years ago. Examination revealed tender retiform purpura in the hand and tender retiform purpura with hemorrhagic bulla in the legs. Initially, she had only a significantly positive atypical p-ANCA and later developed combined positivity of both Myeloperoxidase (MPO) and Anti-proteinase-3(PR3) antibodies with a p-ANCA pattern on immunofluorescence. We report a unique case of cocaine (likely contaminated with levamisole)-induced delayed recurrent vasculitis with varying vasculitic antibodies over the years. Conclusions: This case highlights the fact that patients can develop cocaine-related vasculitis after many years of uneventful abuse. Cocaine, with its adulterant levamisole, has the propensity to trigger diverse immunological reactions, which is evident by the varying antibody profile seen in the same patient over time. PMID:26003170

  1. [Central nervous system vasculitis and of the peripheral nerves in the elderly].

    PubMed

    Boddaert, Jacques; Verny, Marc

    2002-11-01

    Vasculitis of the nervous system are rare in the elderly. When present, they may constitute an urgent diagnosis and a therapeutic emergency. Clinical expression is rich and without specificity. Atypical signs (unusual course of dementia, systemic signs) or atypical laboratory results (inflammatory syndrome) may suggest the diagnosis of vasculitis. However, as multiple comorbidity is the rule in elderly subjects, searching for intercurrent factors (e.g. atrial fibrilation due to infectious disease causing embolic stroke) may be more contributive than searching for proof of a rare disease (vasculitis) with invasive procedures in this population. Giant cell (temporal) arteritis is the only vasculitis specifically related with age; the vital prognosis of vision may be compromised. Corticosterid therapy must be instituted without delay. Periartritis nodosa begins in 30% of cases after 60 years of age. The clinical features are the same as in younger subjects. Other vasculidis are rare in the elderly. In absence of specific studies in this population, therapeutic protocols are the same as in younger subjects but may have to be adjusted. PMID:12598831

  2. Vasculitis with panniculitis following botulinum toxin A injection for cosmetic use

    PubMed Central

    Namazi, Nastaran; Robati, Reza M.; Dadkhahfar, Sahar; Shafiee, Anoush; Bidari-Zerehpoush, Farahnaz

    2016-01-01

    Botulinum toxin A injection is performed most frequently for facial rejuvenation all over the world. Some bizarre and severe side effects have been reported after botulinum toxin A injection. Herein, we present a case of vasculitis with panniculitis after a patient underwent a cosmetic procedure that included injection of purported botulinum toxin A. PMID:26937303

  3. 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...

  4. Systemic lupus erythematosus and antiphospholipid syndrome related retinal vasculitis mimicking ocular cysticercosis: a case report.

    PubMed

    Wu, Chan; Dong, Fang-tian; Chen, You-xin; Wang, Qian; Dai, Rong-ping; Zhang, Hua

    2015-03-01

    Making accurate and timely diagnosis is often challenging when patients with a systemic disease first present with ocular manifestations. The possibility that vasculitis associated with systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS) can be misdiagnosed as cysticercosis has not been discussed in the literatures. PMID:25837363

  5. eNOS inhibits the development of autoimmune-mediated vasculitis

    PubMed Central

    Schoeb, Trenton R.; Jarmi, Tambi; Hicks, M. John; Henke, Scott; Zarjou, Abolfazl; Suzuki, Hitoshi; Kramer, Philip; Novak, Jan; Agarwal, Anupam; Bullard, Daniel C.

    2012-01-01

    Objective Many different genes or mediators have been implicated in promoting the development of vasculitis, although little is known regarding the mechanisms that normally act to suppress lesion formation. eNOS (NOS3) has been shown to inhibit vascular inflammation in many different model systems, but its roles in the pathogenesis of vasculitis have not been elucidated. The aim of this study was to determine the functions of eNOS in the initiation and progression of vasculitic lesion formation. Methods Nos3 mutant MRL/MpJ-Faslpr mice were generated and comprehensively evaluated and compared to controls for the development of autoimmune disease, including vasculitic lesion formation and glomerulonephritis. Results Nos3?/? MRL/MpJ-Faslpr mice had accelerated onset and increased incidence of renal vasculitis compared to Nos3+/+ controls. In contrast, no significant differences in severity of glomerulonephritis were observed between groups. Vasculitis was also observed in eNOS deficient mice in other organs, including increased expression in the lung. Ultrastructural analyses of renal lesions revealed the presence of electron dense deposits in affected arteries, while IgG, IgA, and C3 deposition was observed in some vessels in Nos3?/? kidneys. In addition, eNOS deficient mice showed increased levels of circulating IgG-IgA immune complexes at 20 weeks of age compared to Nos3+/+ MRL/MpJ-Faslpr and Nos3?/? C57BL/6 mice. Conclusion These findings strongly indicate that eNOS serves as a negative regulator of vasculitis in MRL/MpJ-Faslpr mice, and further suggest that NO produced by this enzyme may be critical for inhibiting lesion formation and vascular damage in human vasculitic diseases. PMID:22933338

  6. Increased monocyte transcription of the proteinase 3 gene in small vessel vasculitis

    PubMed Central

    Ohlsson, S; Hellmark, T; Pieters, K; Sturfelt, G; Wieslander, J; Segelmark, M

    2005-01-01

    Proteinase 3 (PR3) is a pleiotropic and destructive serine protease and it is also a major target for autoantibodies in systemic small vessel vasculitis. We have shown recently that patients in stable remission have increased circulating levels of PR3, independent of autoantibody titre, inflammation, neutrophil degranulation and renal function. Here we explore the possibility of increased PR3 gene transcription. RNA was purified from peripheral blood monocytes from vasculitis patients and controls. Specific mRNA was measured by TaqMan real-time polymerase chain reaction (PCR). The monocyte-like cell lines THP-1 and U937 and human peripheral blod monocytes from healthy controls were stimulated with cytokines and lipopolysaccharide (LPS) for different time periods. PR3 protein was measured in plasma with enzyme-linked immunosorbent assay (ELISA). The median result for PR3 mRNA was 96 (18680) for 22 patients, compared to 1 (0128) for the 15 healthy controls. Elastase expression was also significantly increased, whereas myeloperoxidase and interleukin-8 were not. Stimulation of monocytes with tumour necrosis factor (TNF)-?, interferon (IFN)-? or LPS did not result in any increase of PR3 or elastase transcription, whereas interleukin (IL)-8 transcription was increased 10-fold. Circulating monocytes from patients with systemic vasculitis display increased PR3 gene transcription compared to healthy controls and patients with sytemic lupus erythematosus (SLE). This may be important for the development of vasculitis. Our results do not favour a role for cytokines, antineutrophil cytoplasmic antibodies (ANCA) or immunosuppressive medication in the upregulation of PR3 transcription in vasculitis. PMID:15958084

  7. Giant cell vasculitis is a T cell-dependent disease.

    PubMed Central

    Brack, A.; Geisler, A.; Martinez-Taboada, V. M.; Younge, B. R.; Goronzy, J. J.; Weyand, C. M.

    1997-01-01

    BACKGROUND: Giant cell arteritis (GCA) is a systemic vasculitis that preferentially targets medium-sized and large arteries. The etiopathogenesis of the syndrome is not known, and because of the paucity of information concerning the mechanisms of blood vessel wall damage, treatment options are limited. Clues to pathogenic events in this arteritis may derive from understanding the function of tissue-infiltrating cells. Arterial injury in GCA is associated with the formation of granulomas that are composed of T cells, activated macrophages, and multinucleated giant cells. To examine the role of T cells, we implanted inflamed temporal arteries from patients with GCA into severe combined immunodeficiency (SCID) mice and studied whether the vascular lesions were T cell-dependent. MATERIALS AND METHODS: Temporal artery specimens from patients with GCA were engrafted into SCID mice. The histomorphologic appearance of fresh arteries and grafts retrieved from the mice was compared by two-color immunohistochemistry, and the functional profile of tissue-infiltrating cells was analyzed by semiquantifying cytokine transcription with a polymerase chain reaction (PCR)-based assay system. The repertoire of tissue-infiltrating T cells was assessed for the presence of dominant T cell populations by using T cell receptor beta-chain-specific PCR followed by sequencing. To investigate the role of T cells in the activation of tissue-infiltrating macrophages, T cells were depleted from the arterial grafts by treating the mice with T cell-specific antibodies and the production of monokines was monitored. To demonstrate the disease relevance of T cells expanding in the implants, T cells were isolated from tissue segments and adoptively transferred into mice implanted with syngeneic arteries. The in situ production of lymphokines was then determined. RESULTS: The inflammatory infiltrate penetrating all layers of the arterial wall persisted in the xenotransplants, indicating that the inflammatory foci represent independent functional units. Similar quantities of T cell- and macrophage-derived cytokines were detected in fresh and engrafted tissue. However, the diversity of tissue-infiltrating T cells decreased following implantation. T cells with identical T cell receptors were expanded in different mice that had been engrafted with tissue fragments from the same patient, indicating that T cell survival in the arterial wall was a nonrandom process. To confirm the disease relevance of these T cells, T cell depletion and reconstitution experiments were performed. Antibody-mediated elimination of T cells from the xenotransplants resulted in the attenuation of the production of the monokines, IL-1 beta and IL-6. Adoptive transfer of syngeneic tissue-derived T cells, but not of peripheral blood T cells, into engrafted SCID mice enhanced the transcription of IL-2 and IFN-gamma in the implanted arteries. CONCLUSIONS: The vascular lesions of GCA are maintained in human artery-mouse chimeras, indicating that all cellular and noncellular components necessary for the disease are present in the temporal artery. Activation of tissue-infiltrating T cells and macrophages depends upon an infrequent subpopulation of lesional T cells that have a survival advantage in the xenotransplants. The selective proliferation of these T cells in the arteries suggests that there is recognition of a locally expressed antigen. Therefore, these T cells should be candidate targets for the development of novel therapeutic strategies in GCA. Images FIG. 1 FIG. 3 FIG. 5 PMID:9307981

  8. Coexistent granulomatous vasculitis and leukaemia cutis in a patient with resolving herpes zoster.

    PubMed

    Elgoweini, M; Blessing, K; Jackson, R; Duthie, F; Burden, A D

    2011-10-01

    An 80-year-old man presented with a 6-month history of indurated tender purple papules. These had coalesced to form plaques with some central scarring and a dermatomal distribution on the left arm, immediately following herpes zoster (HZ) infection at this site. The patient had a 5-year history of small lymphocytic lymphoma (SLL), which was being managed conservatively under a 'watch and wait' protocol. On histological examination of a skin biopsy, marked interstitial granulomas and prominent granulomatous vasculitis were seen, supporting the clinical impression of a post-HZ granulomatous reaction. In addition, there was a dense monoclonal small B-cell lymphocytic infiltrate indicating koebnerization by SLL (a finding that has not been reported previously with concurrent postherpetic granulomatous vasculitis). Although benign pseudolymphomas occur in postherpetic cases, this case shows that even in association with benign vasculitic features true lymphomas can occur. Furthermore, this case highlights the importance of immunocytochemistry, molecular studies and clinicopathological correlation. PMID:21883389

  9. Primary mediastinal (thymic) B-cell lymphoma presenting as cutaneous vasculitis.

    PubMed

    De Sousa Lages, Adriana; Esperto, Helder; Santos, Llita; Carvalho, Armando

    2015-01-01

    Cutaneous small vessel vasculitis is a severe neutrophilic vascular inflammation mediated by immune complexes that involves the dermal postcapillary venules. Neoplasms represent 2-5% of all causes of secondary cutaneous vasculitis. We present a case of a 52-year-old man who was admitted due to a 10-day history of respiratory symptoms and myalgia. From the third day of symptoms onwards, the patient noticed the appearance of cutaneous lesions in the lower limbs with palpable purpura and erythematous papules. Additionally, he reported of asthenia, anorexia and weight loss during the prior month. Chest radiography showed an enlarged mediastinum and thoracoabdominal-pelvic CT scan revealed a bulky left hilar mass. Biopsy of the left superior lobar bronchus' mucosa allowed for the histological diagnosis of diffuse large B-cell lymphoma (DLBL) of the thymus. The patient received symptomatic treatment with improvement of the purpuric lesions and a multiagent chemotherapy regimen was initiated. PMID:25935914

  10. Systemic antineutrophil cytoplasmic antibody vasculitis in a patient with chronic lymphocytic leukemia: quite a rare diagnosis.

    PubMed

    Pamuk, Glsm Emel; Uyanik, Mehmet Sevki; Demir, Muzaffer; Tekgndz, Emre; Turgut, Burhan; Soy, Mehmet

    2007-08-01

    There might be rheumatic manifestations of malignant diseases, especially those of the hematological type. Until now, antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis in chronic lymphocytic leukemia (CLL) has been reported on only very few occasions. Here, we present our patient with Rai stage II CLL who came to us with constitutional symptoms. She turned out to have hematuria with dysmorphic erythrocytes and developed hemoptysis. She had pulmonary-renal syndrome and was diagnosed with p-ANCA positive microscopic polyangiitis. She is currently using prednisolone and cyclophosphamide and undergoing regular hemodialysis. Constitutional symptoms in patients with hematological malignancies should make the physicians consider systemic vasculitis after exclusion of disease-related complications. PMID:17010431

  11. Systemic vasculitis and aneurysm formation in the Wiskott-Aldrich syndrome.

    PubMed Central

    McCluggage, W G; Armstrong, D J; Maxwell, R J; Ellis, P K; McCluskey, D R

    1999-01-01

    A 24 year old male who suffered from the Wiskott-Aldrich syndrome developed intra-abdominal bleeding on two occasions. Radiological investigations showed aneurysmal dilatation of branches of the hepatic and superior mesenteric arteries. The second abdominal bleed necessitated laparotomy and the bleeding was localised to the kidneys. Right nephrectomy was performed and histological examination showed a necrotising vasculitis, mainly involving medium and small sized renal blood vessels. Steroids, immunosuppressive treatment, and control of blood pressure resulted in resolution of the vasculitic process and prevented further haemorrhage. Vasculitis and aneurysm formation are rarely described complications of Wiskott-Aldrich syndrome and may account for the life threatening haemorrhage which occurs in this condition. Images PMID:10560364

  12. [Implication of dexamethasone adjunctive therapy after the onset of cerebral vasculitis in Streptococcus pneumoniae meningitis].

    PubMed

    Lefebvre, N; Carre, A-C; Delabranche, X; Guiot, P; Mootien, Y

    2007-02-01

    Few adverse effects have been reported with adjunctive dexamethasone treatment in pneumococcal meningitis. Nevertheless, we report a case of cerebral vasculitis. A 49-year-old man was admitted for fever and altered mental status. Lumbar puncture revealed a high inflammatory response and Streptococcus pneumoniae was identified by culture. Antibacterial therapy and adjunctive dexamethasone treatment were initiated as recommended. The immediate outcome was favorable but due to the onset of focal cerebral abnormalities, a CT scan was performed on the ninth day showing cerebral vasculitis. The patient died on the thirteenth day despite antibacterial therapy and resuscitation. In our case, a secondary neurological worsening appeared when adjunctive dexamethasone treatment was stopped suggesting a rebound effect. Observation of similar cases may lead to modifying adjunctive dexamethasone treatment protocol in bacterial meningitis. PMID:17267155

  13. 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

  14. Comparability of patients with ANCA-associated vasculitis enrolled in clinical trials or in observational cohorts

    PubMed Central

    Pagnoux, Christian; Carette, Simon; Khalidi, Nader A.; Walsh, Michael; Hiemstra, Thomas F.; Cuthbertson, David; Langford, Carol; Hoffman, Gary S.; Koening, Curry L.; Monach, Paul A.; Moreland, Larry; Mouthon, Luc; Seo, Phil; Specks, Ulrich; Ytterberg, Steven; Westman, Kerstin; Hoglund, Peter; Harper, Lorraine; Flossmann, Oliver; Luqmani, Raashid; Savage, Caroline; Rasmussen, Niels; de Groot, Kirstin; Tesar, Vladimir; Jayne, David; Merkel, Pater A.; Guillevin, Loic

    2015-01-01

    Objective To analyse the differences between patients with granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) entered into randomised clinical trials (RCTs) and those followed in large observational cohorts. Methods The main characteristics and outcomes of patients with generalised and/or severe GPA or MPA with a five-factor score ?1 enrolled in the French Vasculitis Study Group (FVSG) or the US-Canadian-based Vasculitis Clinical Research Consortium cohorts were compared to those enrolled in one of 2 FVSG clinical RCTs (WEG91, WEGENT) or 3 European Vasculitis Society clinical trials (CYCLOPS, CYCAZAREM, IMPROVE). Results 657 patients (65.3% with GPA) in RCTs were compared to 437 in cohorts (90.6% with GPA). RCT patients were older at diagnosis than the cohort patients (56.613.9 vs. 46.817.3 years), had higher Birmingham vasculitis activity score (19.59.1 vs. 16.97.4), and more frequent kidney disease (84.0% vs. 54.9%) but fewer ear, nose, and throat symptoms (56.8% vs. 72.2%). At 56 months post-diagnosis, mortality and relapse rates, adjusted for age and renal function, were higher for patients with GPA in RCTs vs. cohorts (10.7% vs. 2.5% [p=0.001] and 22.5% vs. 15.6% [p=0.03], respectively) but similar for patients with MPA (6.2% vs. 6.6% [p=0.92] and 16.6% vs. 10.1% [p=0.39], respectively). Conclusion Patients with GPA or MPA in RCTs and those in observational cohorts show important differences that should be remembered when interpreting results based on these study populations. PMID:26016754

  15. Risks of treatments and long-term outcomes of systemic ANCA-associated vasculitis.

    PubMed

    Flossmann, Oliver

    2015-06-01

    Patients with ANCA-associated vasculitis (AAV) have an increased risk of premature death and organ failure. Treatment strategies with immune-suppressive drugs need to balance disease control and prevention of relapse against the risk of side effects in particular infection and malignancy. The longer-term outcome of patients with AAV who participated in several randomised controlled trials has been published in recent years. The results of these and other newer studies will be the focus of this review. PMID:26009242

  16. Pulmonary renal syndrome in a patient with vasculitis: Case report and review of literature

    PubMed Central

    Chachar, Aijaz Zeeshan Khan; Sabir, Omer; Haider, Irfan; Tanvir, Imrana; Rafique, Kashif; Tarif, Nauman

    2015-01-01

    Granulomatosis with polyangiitis (GPA) previously known as Wegners granulomatosis, is a small vessel vasculitis that preferentially involves capillaries, arterioles and venules, presenting as multisystemic disease classically with alveolar haemorrhage and renal insufficiency. We report a case of GPA diagnosed on history, clinical findings and supported by imaging and very high levels of C-ANCA. Renal biopsy confirmed the typical histopathological findings. We discuss herein the management of the case and review of literature. PMID:26870133

  17. Ureteric vasculitis, an unusual presentation of polyarteritis nodosa: a case report.

    PubMed

    Jois, Ramesh; Gupta, Anant; Krishnamurthy, Santosh

    2015-06-01

    A 22 year old female presented with vasculitic skin rash. She was incidentally found to be hypertensive and had proteinuria. Skin biopsy showed leuco-cytoclastic vasculitis. On Imaging, there was left hydronephrosis, hydroureter with bilateral multiple foci of ureteric stenosis and micro-aneurysms in relation to interlobar arteries of kidney. A diagnosis of classical Polyarteritis Nodosa (cPAN) with multi-level ureteric stenosis was made. She was treated with Glucocorticoids, Cyclophosphamide, following which Azathioprine was given. PMID:25925589

  18. 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 (2.5mg/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 34 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 patients 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

  19. Diffuse dermal angiomatosis: a clinical mimicker of vasculitis associated with calciphylaxis and monoclonal gammopathy.

    PubMed

    Ferreli, C; Atzori, L; Pinna, A L; Pau, M; Aste, N; Ricotti, C; Rongioletti, F

    2015-02-01

    Diffuse dermal angiomatosis is a form of cutaneous reactive angiomatosis characterized clinically by painful erythematous or violaceous lesions with ulcers that may mimic cutaneous vasculitis/vasculopathy. Histologically it shows a benign, diffuse proliferation of endothelial cells with tiny blood vessels in the papillary and reticular dermis. Herein, we report four patients with diffuse dermal angiomatosis in the setting of calciphylaxis and monoclonal gammopathy and review the cases previously published in the literature. Comorbidities and management will also be discussed. PMID:25502367

  20. 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

  1. 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

  2. Kidney lesions associated with systemic vasculitis: report of 25 cases in morocco.

    PubMed

    Abdou, N; El Mustapha, F; Ghislaine, M; Khalid, Z; Benyounes, R; Mohamed, B G; Khadija, H; Squalli, S; Driss, Z

    2001-01-01

    In this retrospective study, we report 25 patients with renal injury caused by systemic vasculitis. These patients were hospitalized at the department of nephrology-hemodialysis in Ibn Rochd hospital from 1985 to 1998. The mean age of the patients was 36 years (range 3 to 57 years) with male predominance (68%). The clinical presentations included cutaneous purpura in 80% of the patients, arthritis in 60% and pulmonary hemorrhage in 20%. The renal injury manifested as abnormal urinary sediment in 20 of the study patients (80%), nephritic syndrome in 14 (56%), renal failure in nine (36%). Three patients (12%) had renal failure as the initial presentation followed by the other manifestations of vasculitis. The anti-neutrophil cytoplasmic antibodies (ANCA) have been checked in only six patients of whom four were positive. Henoch-Schonlein purpura was the most common clinical sign in 11 patients (44%) followed by Behcet's disease in five (20%). The treatment was variable in type and duration but generally included corticoids alone or in combination with immunosuppressants. The prognosis of the renal injury was variable. The renal function remained stable in those with Henoch-Schonlein purpura, but was less favorable with the other etiologies with more predominance of renal failure and end-stage renal disease. This retrospective analysis of our experience is given to throw light on the pattern of vasculitis in our region. PMID:18209403

  3. 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

  4. 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

  5. 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

  6. 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

  7. 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

  8. IgG autoantibody to the collagen-like region of Clq in hypocomplementemic urticarial vasculitis syndrome, systemic lupus erythematosus, and 6 other musculoskeletal or rheumatic diseases.

    PubMed

    Wisnieski, J J; Jones, S M

    1992-06-01

    We previously found that the Clq precipitin in sera from patients with hypocomplementemic urticarial vasculitis syndrome is an IgG autoantibody to Clq. We report here a prevalence study of this autoantibody in 162 patients with musculoskeletal or rheumatic diseases including hypocomplementemic urticarial vasculitis syndrome, systemic lupus erythematosus (SLE), and rheumatoid arthritis uncomplicated by vasculitis. The autoantibody, which binds only to the collagen-like region of Clq, was found almost exclusively in hypocomplementemic urticarial vasculitis syndrome (100%) and SLE (35%) sera. Our results support the idea that among rheumatic diseases, anti-Clq autoantibody develops in disorders characterized by immune complex mediated injury, particularly of cutaneous and glomerular microvasculature. PMID:1404125

  9. Patient-reported outcomes in ANCA-associated vasculitis. A comparison between Birmingham Vasculitis Activity Score and routine assessment of patient index data 3.

    PubMed

    Annapureddy, Narender; Elsallabi, Osama; Baker, Joshua; Sreih, Antoine G

    2016-02-01

    The objective of this study was to determine health-related quality of life (HRQoL) in patients with ANCA-associated vasculitis (AAV) as measured by the "routine assessment of patient index data 3" (RAPID3) and whether RAPID3 is correlated with disease activity as determined by the Birmingham Vasculitis Activity Score (BVAS). Data from patients at an academic institution vasculitis clinic seen between Jan 2010 and Jan 2012 were collected using chart review. BVAS and RAPID3 scores were calculated at each patient visit. RAPID3 was compared between patients in remission (BVAS = 0) and patients with active disease (BVAS > =1) at all visits for four consecutive visits, when data available, at least 3 months apart during the period mentioned. Robust generalized estimating equations (GEE) in linear regression models evaluated associations between the RAPID3 and BVAS over all available observations, adjusting for intra-subject correlations. Thirty-four patients were included in the study, 26 had granulomatosis with polyangiitis (GPA), five microscopic polyangiitis (MPA), and three eosinophilic granulomatosis with polyangiitis (EGPA). Patients at first visit had impaired HRQoL as measured by RAPID3 [6.8 (3.1-12.6)]. The median RAPID3 scores were higher in patients with active disease as compared to patients in remission (7.0 vs. 3.0, p = 0.115; 8.8 vs. 1.0, p = 0.011; 6.1 vs. 2.0, p = 0.032; and 11.7 vs. 2.0, p = 0.128 for visits 1, 2, 3, and 4, respectively). In longitudinal GEE models incorporating all observations there was a strong association between the RAPID3 (per 1 unit) and BVAS (per 1 unit) [β 0.21 (0.10, 0.32) p < 0.001]. RAPID3 can be used to measure HRQoL in patients with AAV. RAPID3 correlated significantly with BVAS. RAPID3 can discriminate between disease states in AAV. This instrument may help document patient experience and add to clinical decisions. PMID:25822996

  10. 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

  11. Antineutrophil cytoplasmic autoantibodies specific for myeloperoxidase cause glomerulonephritis and vasculitis in mice.

    PubMed

    Xiao, Hong; Heeringa, Peter; Hu, Peiqi; Liu, Zhi; Zhao, Minglang; Aratani, Yasuaki; Maeda, Nobuyo; Falk, Ronald J; Jennette, J Charles

    2002-10-01

    Antineutrophil cytoplasmic autoantibodies (ANCAs) are identified in the circulation of approximately 80% of patients with pauci-immune necrotizing and crescentic glomerulonephritis and systemic small vessel vasculitis, such as microscopic polyangiitis and Wegener granulomatosis. The most common antigen target for ANCAs is myeloperoxidase (MPO), which is found in neutrophils and monocytes. We report definitive experimental animal evidence that ANCAs are pathogenic. MPO knockout (Mpo(-/-)) mice were immunized with mouse MPO. Splenocytes from these mice or from control mice were injected intravenously into recombinase-activating gene-2-deficient (Rag2(-/-)) mice, which lack functioning B lymphocytes and T lymphocytes. All mice that received splenocytes developed mild to moderate glomerular immune deposits, but only mice that received 1 x 10(8) or 5 x 10(7) anti-MPO splenocytes developed severe necrotizing and crescentic glomerulonephritis, granulomatous inflammation, and systemic necrotizing vasculitis, including necrotizing arteritis and hemorrhagic pulmonary capillaritis. To test the pathogenic potential of antibodies alone, purified anti-MPO IgG or control IgG was injected intravenously into Rag2(-/-) mice and wild-type mice. Mice that received anti-MPO IgG but not mice that received control IgG developed focal necrotizing and crescentic glomerulonephritis with a paucity of glomerular Ig deposition. Thus, anti-MPO IgG alone was able to cause pauci-immune glomerular necrosis and crescent formation in the absence of functional T or B lymphocytes in Rag2(-/-) mice and in the presence of an intact immune system in wild-type C57BL/6J mice. This animal model offers strong support for a direct pathogenic role for ANCA IgG in human glomerulonephritis and vasculitis. PMID:12370273

  12. 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

  13. [Autopsy case of PR3-ANCA-associated vasculitis complicated with rectus muscle hematoma].

    PubMed

    Sakaguchi, Yusuke; Niihata, Kakuya; Yasuda, Keiko; Shimomura, Akihiro; Uehata, Takuya; Inoue, Kazunori; Kaneko, Tetsuya; Shoji, Tatsuya; Tsubakihara, Yoshiharu; Okada, Noriyuki

    2009-01-01

    A 80-year-old man was admitted to our hospital because of coughing, hemosputum and dyspnea. As a chest X-ray showed infiltrates of the right lung, he was diagnosed as bacterial pneumonia and treated with antibiotics. However, after a few days, he exhibited hemoptysis and developed severe dyspnea, while laboratory findings showed rapid elevation of the serum creatinine level (5.55 mg dL). Computed tomography (CT) revealed large areas of ground glass opacity in the right lung, hence the hemoptysis was considered to be due to alveolar hemorrhage. As he had been diagnosed as chronic renal failure a few years before this admission and we also noticed that interstitial pneumonia with a slightly elevated level of C-reactive protein had existed from that time, ANCA-associated vasculitis was suspected to be the underlying pathogenesis. Accordingly, he was started on methylprednisolone pulse therapy and temporary hemodialysis resulted in improvement of dyspnea and renal function. PR3-ANCA was 12.4 EU, so he was diagnosed as PR3-ANCA-associated vasculitis. After a few days, he suddenly complained of abdominal pain, developing hypotension and anemia. Abdominal CT showed an irregular low-density mass in the right muscle, so he was diagnosed as rectus muscle hematoma. Surgery was performed and a massive hematoma was found in the rectus muscle without any ruptures of macroscopic vessels in the abdomen. Bleeding could not be stopped followed by multiple organ failure and the patient died four days postoperatively. Rectus muscle hematoma is an uncommon cause of acute abdomen, and has been reported in about 100 cases in Japan. It occurs because of a tear in epigastric vessels and is usually managed conservatively with a good prognosis, although hemodynamically unstable cases require surgery. To the best of the authors' knowledge, this is the first case of rectus muscle hematoma complicated with ANCA-associated vasculitis. PMID:19715163

  14. Septic vasculitis and vasculopathy in some infectious emergencies: the perspective of the histopathologist.

    PubMed

    Tomasini, C

    2015-02-01

    Sepsis is a potentially life-threatening complication of an infection where cutaneous lesions often represent one of the early signs. A myriad of microorganisms including bacteria, fungi, yeasts, viruses, protozoas, helminths and algae can be implicated. A broad spectrum of clinical and histopathologic findings can be observed in the skin and the common denominator is a thrombotic vasculopathy. The pathogenesis of cutaneous septic vasculitis (SV)/vasculopathy is complex and includes five main mechanisms: disseminated intravascular coagulation, direct invasion and occlusion of blood vessel walls by microorganisms, hypersensitivity reaction with immune complex deposition into blood vessel walls, embolism from a distant infectious site and vascular effects of toxins. Herein we describe the clinicopathologic findings of some selected cases of SV recently observed in our hospital, including purpura fulminans, necrotizing fasciitis, cutaneous meningococcemia, malignant syphilis and disseminated alternaria infection. Histopathologically, a wide spectrum of histopathologic changes was observed in skin specimens from the various entities, involving the intensity and composition of the inflammatory infiltrate, the degree of vascular changes and the presence of microorganisms, that ranged from a predominant not inflammatory, thrombotic-occlusive vasculopathy in purpura fulminans to leukocytoclastic vasculitis like changes in cutaneous meningococcemia to a dermal angiomatosis-like pattern in disseminated Alternaria infection. The different pathologic presentations may be related to the microorganism involved, the main pathogenetic mechanism that induced the vascular injury and the individual immunologic burden. Early skin biopsy for histopathologic examination and microbiologic culture is a cornerstone in the diagnosis of life-threatening diseases that present with cutaneous septic vasculitis. Ancillary techniques, such as immunohistochemistry and polymerase chain reaction are additional novel and helpful tools to identify pathogens, leading to definite diagnosis in cases with challenging or ambiguous clinical and/or pathologic findings. PMID:25592669

  15. 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.

  16. 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

  17. Bortezomib in type I cryoglobulinemic vasculitis: are we acting too late?

    PubMed

    Ramirez, Giuseppe A; Campochiaro, Corrado; Salmaggi, Chiara; Pagliula, Gaia; D'Aliberti, Teresa; Marcatti, Magda; Tresoldi, Moreno; Praderio, Luisa

    2015-01-01

    Type II and type III cryoglobulinemic vasculitis (CV) are characterized by a deranged immune function due to concomitant chronic infections or rheumatic disorders. Conversely, type I CV is caused by plasma cell dyscrasia. Bortezomib is a proteasome inhibitor that is largely employed as a first-line treatment for multiple myeloma. The use of bortezomib in cases of monoclonal gammopathy of undetermined significance (MGUS)-related refractory type I CV has been reported in only four patients. In the current report, we discuss the efficacy of bortezomib treatment in a patient with type I CV, with a focus on the suitability and early application of this drug. PMID:25948361

  18. [Systemic necrotizing vasculitis presenting as gangrene combined with diabetes insipidus: a case report].

    PubMed

    Huang, Qing; Liu, Yu-lan

    2015-12-18

    The male patient reported here presented as gangrene and central diabetes insipidus (CDI), who had characteristics of vasculitis. The patient complained about polydipsia and polyuria half a year ago, and then developed tingling, pain and blackish discoloration of some fingers and toes 3 month ago. He also had Raynaud's phenomenon. After admission, his laboratory examination showed the rise of erythrocyte sedimentation rate, C-reactive protein, immunoglobulin, β2-glycoprotein I and the activity of rheumatoid factors, lupus anticoagulant test. his pituitary gland showed loss of posterior signal on magnetic resonance imaging. In addition, his vasopressin test was active. However, there was no sufficient evidence to diagnose any specific disease; as a consequence the patient was diagnosed as idiopathic systemic necrotizing vasculitis (SNV). For SNV, the patient was treated with glucocorticoid 40 mg/d and impact therapy of cyclophosphamide 0.4 g every 2 weeks. He also received symptomatic treatment for gangrene and CDI. Cutaneous involvement leading to gangrene was widely reported in SNV, however pituitary involvement in SNV leading to CDI was rare. The prognosis of this patient was poor. PMID:26679670

  19. Coronary vasculitis with acute myocardial infarction in a young woman with systemic lupus erythematosus.

    PubMed

    Caracciolo, Eugene A; Marcu, Constantin B; Ghantous, Andre; Donohue, Thomas J; Hutchinson, Gordon

    2004-04-01

    Myocardial infarction in patients with systemic lupus erythematosus (SLE) is most commonly a consequence of atherosclerosis. Coronary vasculitis with aneurysms is a rare cause of myocardial ischemia in SLE. We present a case of a 22-year-old woman with a 4-year history of SLE who was admitted with acute onset of chest pain. Although initially treated for lupus pericarditis, she was subsequently found to have an acute myocardial infarction. Cardiac catheterization revealed multiple areas of aneurysmal coronary dilatation and only moderate stenoses of the secondary branches.In view of the angiographic findings, coronary revascularization was not indicated. Anticoagulant therapy was initiated as a result of the presence of large aneurysmal coronary dilatations, which are predisposed to in situ thrombosis and distal embolization. The coronary vasculitis was treated with immunosuppressive therapy. Measures aimed at secondary prevention of coronary artery disease, including optimization of lipid profile, blood pressure control, and prevention of left ventricular postinfarct remodeling, were initiated and continued indefinitely. PMID:17043468

  20. 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

  1. 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

  2. Emerging role of high mobility group box 1 in ANCA-associated vasculitis.

    PubMed

    Wang, Chen; de Souza, Alexandre W S; Westra, Johanna; Bijl, Marc; Chen, Min; Zhao, Ming-Hui; Kallenberg, Cees G M

    2015-11-01

    High mobility group box 1 (HMGB1) has been suggested to be involved in the pathogenesis of many autoimmune diseases. In addition to its nuclear functions, extracellular HMGB1 released from activated, injured or dying cells becomes a proinflammatory mediator via binding to various receptors on the surface of responding cells. HMGB1 interacts with various systems involved in inflammation, such as the complement system and the coagulation system. Thus, HMGB1 could amplify inflammation and enhance immune responses in pathophysiology of certain diseases. In the past years, HMGB1 has been studied in several vasculitides including antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, Kawasaki disease, Henoch-Schnlein purpura, Takayasu arteritis and giant cell arteritis. Several studies showed that circulating HMGB1 levels are higher in patients with active disease compared with healthy controls, and levels are associated with disease severity. Further studies on pathogenetic mechanisms revealed pathogenic roles of HMGB1 in some vasculitides. Here we review clinical and experimental studies dealing with the role of HMGB1 in vascular inflammation, and its relation to the manifestations and prognosis of specific vasculitides, in particular ANCA-associated vasculitis. PMID:26209906

  3. 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.

  4. 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

  5. Rheumatoid Vasculitis

    MedlinePLUS

    ... Hypocomplementemia, anti-nuclear antibodies (ANA), and atypical anti-neutrophil cytoplasmic antibodies (ANCA) are common. Rheumatoid factor levels ... Hypocomplementemia, anti-nuclear antibodies (ANAs), and atypical anti-neutrophil cytoplasmic antibodies (atypical ANCAs) are common. Rheumatoid factor ...

  6. Rituximab for remission induction in elderly patients with ANCA-associated vasculitis

    PubMed Central

    Timlin, H.; Lee, S. M.; Manno, R.L.; Seo, P.; Geetha, Duvura

    2015-01-01

    Objectives Advancing age is a risk factor for treatment-related side effects and mortality in AAV patients treated with cyclophosphamide (CYC) and glucocorticoids (GC) for remission induction. The efficacy and safety of rituximab (RTX) in elderly AAV patients has not been well described. Methods We performed a single center retrospective review of 31 consecutive AAV patients aged 60 or more at the time of RTX use for remission induction. All patients received RTX with GC for remission induction. Four patients received concomitant CYC for a mean duration of 52 days. We evaluated clinical and laboratory variables at diagnosis, rates of complete remission defined as Birmingham Vasculitis Activity Score/Wegener's Granulomatosis (BVAS/WG) = 0 and patient survival, renal survival, infections requiring hospitalization, and vasculitis relapse 24 months following RTX use. Results Of the 31 patients, 77% were Caucasian, 68% female, mean age was 71 6 years, 58% were MPO ANCA positive, and 42% had relapsing disease. The mean BVAS/WG score entry was 4.4 1.5, 71% had glomerulonephritis (GN) and 10% had alveolar hemorrhage. The mean baseline e-GFR was 40 28 ml/ min/1.73 m2. Thirty patients achieved remission with a mean time to remission of 57 27 days. The single patient with refractory vasculitis responded to CYC. The mean prednisone dose at 6 months was 5.6 4 mg. Remission maintenance therapy was started within 12 months of RTX induction in 6 patients (4 with RTX, 1 with azathioprine, and 1 with mycophenolate mofetil). One patient suffered a limited relapse 10 months post RTX use. Among the 22 patients with GN at baseline, 1 developed ESRD. One-year patient survival among 25 patients with at least 1 year of follow-up was 100%. There were no episodes of infusion reaction or leukopenia. There were 3 episodes of bacterial pneumonia, 1 episode of candida pneumonia, and 1 episode of disseminated cutaneous zoster. Conclusions This study demonstrates that rituximab is effective for remission induction in elderly patients with AAV. Furthermore, we observed a high incidence of infectious complications. Our experience was limited by its retrospective design, and further studies are needed to evaluate the efficacy and safety of RTX in elderly AAV patients. PMID:25796088

  7. PF-1355, a mechanism-based myeloperoxidase inhibitor, prevents immune complex vasculitis and anti-glomerular basement membrane glomerulonephritis.

    PubMed

    Zheng, Wei; Warner, Roscoe; Ruggeri, Roger; Su, Chunyan; Cortes, Christian; Skoura, Athanasia; Ward, Jessica; Ahn, Kay; Kalgutkar, Amit; Sun, Dexue; Maurer, Tristan S; Bonin, Paul D; Okerberg, Carlin; Bobrowski, Walter; Kawabe, Thomas; Zhang, Yanwei; Coskran, Timothy; Bell, Sammy; Kapoor, Bhupesh; Johnson, Kent; Buckbinder, Leonard

    2015-05-01

    Small vessel vasculitis is a life-threatening condition and patients typically present with renal and pulmonary injury. Disease pathogenesis is associated with neutrophil accumulation, activation, and oxidative damage, the latter being driven in large part by myeloperoxidase (MPO), which generates hypochlorous acid among other oxidants. MPO has been associated with vasculitis, disseminated vascular inflammation typically involving pulmonary and renal microvasculature and often resulting in critical consequences. MPO contributes to vascular injury by 1) catabolizing nitric oxide, impairing vasomotor function; 2) causing oxidative damage to lipoproteins and endothelial cells, leading to atherosclerosis; and 3) stimulating formation of neutrophil extracellular traps, resulting in vessel occlusion and thrombosis. Here we report a selective 2-thiouracil mechanism-based MPO inhibitor (PF-1355 [2-(6-(2,5-dimethoxyphenyl)-4-oxo-2-thioxo-3,4-dihydropyrimidin-1(2H)-yl)acetamide) and demonstrate that MPO is a critical mediator of vasculitis in mouse disease models. A pharmacokinetic/pharmacodynamic response model of PF-1355 exposure in relation with MPO activity was derived from mouse peritonitis. The contribution of MPO activity to vasculitis was then examined in an immune complex model of pulmonary disease. Oral administration of PF-1355 reduced plasma MPO activity, vascular edema, neutrophil recruitment, and elevated circulating cytokines. In a model of anti-glomerular basement membrane disease, formerly known as Goodpasture disease, albuminuria and chronic renal dysfunction were completely suppressed by PF-1355 treatment. This study shows that MPO activity is critical in driving immune complex vasculitis and provides confidence in testing the hypothesis that MPO inhibition will provide benefit in treating human vasculitic diseases. PMID:25698787

  8. The two-edged sword: vasculitis associated with HIV and hepatitis C coinfection.

    PubMed

    Ojaimi, Samar; Lin, Ming-Wei; Singh, Kasha P; Woolley, Ian

    2014-02-01

    Vasculitis has long been associated with chronic viral infections, thus the twin perils of the infection and the immune response against it that bedevils the specialties of infection and immunity. After HIV was identified, it too became associated with vasculitic syndromes. Later, hepatitis C virus was also isolated, identified and described with its own spectrum of vasculitic diseases, including hepatitis C virus-associated cryoglobulinaemia. With the increasing prevalence of HIV and hepatitis C virus coinfection, there has come an increasing recognition of the range of vasculitides that can occur in this population leading to significant morbidity, diagnostic and treatment challenges. In this review, we examine the epidemiology, pathogenesis and general principles of treatment of these systemic diseases in HIV/hepatitis C virus coinfected individuals. PMID:23970639

  9. ANCA-Associated Vasculitis in a Patient with Systematic Sclerosis and Sjgren's Syndrome: A Case Report

    PubMed Central

    Kubota, Keiichi; Ueno, Toshiharu; Mise, Koki; Hazue, Ryo; Suwabe, Tatsuya; Kikuchi, Koichi; Hoshino, Junichi; Sumida, Keiichi; Hayami, Noriko; Takaichi, Kenmei; Fujii, Takeshi; Ohashi, Kenichi; Nonomura, Yoshinori; Ubara, Yosifumi

    2015-01-01

    A 65-year-old woman with a limited form of systematic sclerosis (SSc) and Sjgren's syndrome (SS) was admitted to our hospital for the evaluation of renal dysfunction. Her serum creatinine was 1.6 mg/dl, proteinuria was 1.6 g/day, and the urine sediment contained 2029 erythrocytes/high-power field. Myeloperoxidase anti-neutrophil cytoplasmic antibodies, anti-SS-A/SS-B antibodies and anti-centromere antibodies were positive. A renal biopsy showed focal necrotizing glomerulonephritis with focal interstitial lymphoplasmacytic infiltration. A diagnosis of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) was made. A steroid therapy was initiated and AAV subsided. This is a rare case of AAV in a patient with anti-centromere-positive limited SSc and SS. PMID:26120576

  10. ANCA-Associated Vasculitis in a Patient with Systematic Sclerosis and Sjgren's Syndrome: A Case Report.

    PubMed

    Kubota, Keiichi; Ueno, Toshiharu; Mise, Koki; Hazue, Ryo; Suwabe, Tatsuya; Kikuchi, Koichi; Hoshino, Junichi; Sumida, Keiichi; Hayami, Noriko; Takaichi, Kenmei; Fujii, Takeshi; Ohashi, Kenichi; Nonomura, Yoshinori; Ubara, Yosifumi

    2015-01-01

    A 65-year-old woman with a limited form of systematic sclerosis (SSc) and Sjgren's syndrome (SS) was admitted to our hospital for the evaluation of renal dysfunction. Her serum creatinine was 1.6 mg/dl, proteinuria was 1.6 g/day, and the urine sediment contained 20-29 erythrocytes/high-power field. Myeloperoxidase anti-neutrophil cytoplasmic antibodies, anti-SS-A/SS-B antibodies and anti-centromere antibodies were positive. A renal biopsy showed focal necrotizing glomerulonephritis with focal interstitial lymphoplasmacytic infiltration. A diagnosis of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) was made. A steroid therapy was initiated and AAV subsided. This is a rare case of AAV in a patient with anti-centromere-positive limited SSc and SS. PMID:26120576

  11. Non-infectious cryoglobulinemia vasculitis (CryoVas): update on clinical and therapeutic approach.

    PubMed

    Perez-Alamino, Rodolfo; Espinoza, Luis R

    2014-05-01

    CryoVas is a small vessel vasculitis associated with the presence of circulating cryoglobulins. In the absence of HCV infection, several disorders have been identified in association of CryoVas. Although evidence is limited, a few studies have recently described the clinical presentation, prognosis, and therapeutic management of non-infectious CryoVas. Patients with type I CryoVas, especially associated with hematologic malignancies, have shown a worse clinical presentation. Recent studies have also identified prognostic factors in mixed CryoVas. Therapeutic management in non-infectious CryoVas remains to be defined. Overall, treatment options should be individualized based on severity of involvement. In this setting, new data have emerged regarding the role of biologic therapy in non-infectious CryoVas. Off-label use of rituximab should be highlighted, based on the assessment of benefits and risks, especially infections. PMID:24647999

  12. 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

  13. Anti-neutrophil cytoplasmic antibody-associated systemic vasculitis: nature or nurture?

    PubMed

    Gatenby, P A

    2012-04-01

    This review examines the environmental and genetic contributions to the anti-neutrophil cytoplasmic antibody-associated systemic vasculitides. The dominant environmental risk factors appear to be silica exposure for all three syndromes, and vitamin D deficiency is strongly suggested by the latitude and ultraviolet radiation gradient observed for Wegener's granulomatosis and Churg-Strauss syndrome. Genetic factors are generally not very strong, consistent with the rarity of these conditions in children. However, multiple genetic factors, each with a relatively small effect, may combine to create a state of susceptibility towards autoimmunity. With infection as a triggering agent, it is possible to synthesise a pathogenetic hypothesis that accounts for both environmental and genetic effects in regard to both necrotising vasculitis and granuloma formation. PMID:22212903

  14. 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

  15. Is Levamisole-Induced Vasculitis a Relegated Diagnostic Possibility? A Case Report and Review of Literature

    PubMed Central

    Patnaik, Soumya; Balderia, Percy; Vanchhawng, Lisa; Markazi, Peyman; Wykretowicz, Jedrzej; Perloff, Sarah

    2015-01-01

    Patient: Female, 38 Final Diagnosis: Levamisole induced vasculitis Symptoms: Skin and joint Medication: — Clinical Procedure: None Specialty: Toxicology Objective: Unusual clinical course Background: Levamisole, a veterinary anti-helminthic, is a common adulterant in cocaine. Levamisole-induced vasculopathy (LIV) is a relatively new entity, and is being increasingly recognized since it was first reported in 2010. Although cutaneous findings, agranulocytosis, and positive antineutrophil cytoplasmic antibodies (ANCA) are characteristic, the full clinical picture and appropriate management remain unclear. Case Report: A 38-year-old woman presented with malaise and a pruritic, painful rash on all extremities, right ankle pain, and effusion and necrosis of the right 2nd and 3rd finger tips. After extensive work-up, we determined that she had LIV. Conclusions: Arthritis-dermatitis syndrome in cocaine users should raise suspicion for LIV. Although some features are characteristic, the full clinical spectrum is yet to be described. Management is supportive. PMID:26406869

  16. ANCA-associated vasculitis and pauci-immune glomerulonephritis in HIV disease

    PubMed Central

    Evans, Rhys; Gueret-Wardle, Alexander; Edwards, Simon; Salama, Alan

    2014-01-01

    We report the case of a 51-year-old African woman with longstanding well-controlled HIV who developed relapsing systemic antineutrophil cytoplasm antibody (ANCA)-associated vasculitis and glomerulonephritis. She presented with an acute kidney injury and heavy proteinuria in the setting of a prolonged history of diffuse arthralgias and transient ocular symptoms. Antimyeloperoxidase (MPO) antibody titre was >100 IU/mL. Subsequent renal biopsy demonstrated a focal necrotising pauci-immune glomerulonephritis and a diagnosis of MPO-ANCA-associated microscopic polyangiitis was made. The patient was treated with tapering glucocorticoids and rituximab with resolution of her acute kidney injury and reduction in her proteinuria. Treatment was complicated by the development of steroid-induced diabetes and one mild clinical relapse, but was otherwise well tolerated without infectious complications or deterioration in her HIV disease. PMID:24692374

  17. Treatment of vasculitis and dermatitis in a 59-yr-old Nile hippopotamus (Hippopotamus amphibius).

    PubMed

    Spriggs, Maria; Reeder, Chris

    2012-09-01

    A 59-yr-old female Nile hippopotamus (Hippopotamus amphibius) was diagnosed and treated for severe dermatitis. Lesions included large areas of depigmentation, erosions, and ulcerations on glabrous skin areas, limbs, and perineal region. Histopathologic lesions included a markedly edematous, focally eroded, ulcerative to necrotic epidermis; foci of keratinocyte apoptosis; and a mixed suppurative dermatitis. Most of the dermal vessels had variable hyalinized walls with plump endothelial cells and frequent intramural neutrophils, and some vessels had vascular thrombi consistent with vasculitis. Culture of the lesions yielded beta-hemolytic Streptococcus, Morganella morgannii, and Enterococcus sp. The hippopotamus was successfully treated with sulfamethoxazole and trimethoprim, amoxicillin, and pentoxifylline for more than 2 mo, and the condition did not recur over the subsequent 16 mo. PMID:23082536

  18. Chronic localized leukocytoclastic vasculitis: clinicopathological spectrum of granuloma faciale with and without extrafacial and mucosal involvement.

    PubMed

    Vassallo, C; Derlino, F; Croci, G A; Brazzelli, V; Borroni, G

    2015-02-01

    Granuloma faciale (GF) is a rare cutaneous condition of unknown origin, that usually presents as one or more brown-purple papules, plaques and/or nodules, localized mostly on the face, although extrafacial lesions can also occur. Eosinophilic angiocentric fibrosis (EAF) is regarded as the mucosal counterpart of GF. Histologically, it has been described as a persistent leukocytoclastic vasculitis, with a dense polymorphous inflammatory infiltrate in the superficial and mid dermis, typically sparing the subpapillary dermis, the so called grenz zone. The presence of eosinophils is considered a characteristic feature of the disease. All the cases of GF seen at the Dermatology Unit from 2002 to 2013 were considered and reviewed, both clinically and histopathologically. Only cases with consistent clinical findings of GF, and accurate patient's history were considered. Ten cases of GF were reviewed for both histological specificity and clinico-pathological correlation. Two patients presented extrafacial lesions. One patient had involvement of nasal mucosa. Two patients suffered from associated rheumatological diseases. The most frequent histopathologic features were the presence of a grenz zone and eosinophils in the infiltrate, but also adnexal involvement was often present; vascular changes were constant, yet leukocytoclastic vasculitis could be recorded only in four cases. Fibrosis or sclerosis were always absent. Clinical pictures of the patients treated demonstrated a complete remission of the lesions, without scarring. However, a complete enduring healing was observed only in two patients, and relapse or incomplete remission of the disease was the rule. In conclusion a review of clinicopathological findings of ten patients affected by GF was made and new details of the disease presented. PMID:25592670

  19. 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

  20. Presentation and prognosis of cardiac involvement in hepatitis C virus-related vasculitis.

    PubMed

    Terrier, Benjamin; Karras, Alexandre; Cluzel, Philippe; Collet, Jean-Philippe; Sne, Damien; Saadoun, David; Cacoub, Patrice

    2013-01-15

    Cardiac manifestation in primary systemic vasculitides is associated with poor outcomes, leading to the use of immunosuppressive therapy. In contrast, the spectrum and the outcome of cardiac involvement in the setting of mixed cryoglobulinemia vasculitis (CryoVas) have never been evaluated. To describe the clinical presentation and to evaluate clinical outcomes of cardiac manifestations during hepatitis C virus (HCV)-related mixed CryoVas, the clinical records of 165 consecutive patients with HCV-related mixed CryoVas followed from January 1, 1993, to January 1, 2010, were reviewed. Of the 165 patients with HCV-related mixed CryoVas, 7 (4%) had cardiac manifestations. Thoracic pain and congestive heart failure manifestations were the main clinical manifestations (n= 4 [57%] each). Cardiac imaging showed dilated cardiomyopathy in 5 patients and hypertrophic cardiomyopathy in 1. In multivariate analysis, patients with cardiac manifestations had more frequent B-cell lymphoma (odds ratio 18.1, 95% confidence interval 2.8 to 116.7, p= 0.0023) and gastrointestinal involvement (odds ratio 14.6, 95% confidence interval 2.0 to 104.9, p= 0.0078). All cardiac manifestations were reversible early after the initiation of corticosteroids and aggressive immunosuppressive therapy. However, after a median follow-up period of 19 months, 3 patients (43%) had died. Respective 6-month, 1-year, and 2-year survival rates in patients with and without cardiac involvement were 86% and 99%, 71% and 96%, and 48% and 90% (hazard ratio 5.01, p= 0.003). In conclusion, cardiac damage is a rare manifestation of HCV-related mixed cryoglobulinemia vasculitis. Cardiac involvement is associated with B-cell lymphoma and life-threatening manifestations. Despite favorable early outcomes, patients with cardiac damage had poorer survival than those without. PMID:23116612

  1. A Case of Rituximab Use as an Induction and Maintenance of Remission in ANCA-Associated Vasculitis.

    PubMed

    Awad, Neveen; Hafiz, Shahd; Albeity, Abdurahman; Almoallim, Hani

    2016-01-01

    Antineutrophil cytoplasmic antibody- (ANCA-) associated vasculitis (AAV) is a multisystem autoimmune disease affecting mainly microscopic blood vessels due to circulating autoantibodies against neutrophil cytoplasmic antigens. We report a case of a 57-year-old female patient presenting with hemoptysis, sinusitis, and conjunctivitis. Based on lung biopsy, the diagnosis of antineutrophil cytoplasmic antibody- (ANCA-) associated vasculitis (AAV) was established. She was put on rituximab as induction and maintenance therapy. She responded initially to rituximab as induction therapy but failed to respond in the maintenance course of the drug. Rituximab was stopped and mycophenolate mofetil was administered. She responded as laboratory c-ANCA titers turned negative and symptoms subsided. There are no randomized clinical trials addressing rituximab effect in induction and remission at the same time. This case report doubts the efficacy of the use of rituximab therapy for both induction and maintenance of remission at the same time, waiting for the results of the ongoing trials. PMID:27006851

  2. 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

  3. Mononeuritis multiplex due to thrombotic ischemia of primary antiphospholipid antibody syndrome without vasculitis: an autopsy case report.

    PubMed

    Takahashi, Masatoshi; Katada, Fumiaki; Sato, Susumu; Shibayama, Hidehiro; Fukutake, Toshio; Murayama, Shigeo

    2015-12-23

    The patient was a 78-year-old man. Three years before admission, he developed transient peripheral neuropathy and purpura, and at admission, he presented with livedo reticularis of both his lower extremities and with mononeuritis multiplex. Vasculitis was not observed, and antiphospholipid antibodies were detected. The nerve and skin biopsies revealed no inflammation; axonal degeneration accompanied by thrombi was found in his arterioles and venules. Based on these findings, he was diagnosed with ischemic peripheral neuropathy due to primary antiphospholipid syndrome. Administration of anticoagulant therapy resulted in an improvement in symptoms; however, two months later, a relapse occurred, and the patient contracted an infection while undergoing immunosuppressive therapy. The infection became fulminant, and the patient succumbed to multiple organ failure. The autopsy revealed a systemic arterial and venous embolism; however, no vasculitis was observed. Antiphospholipid syndrome, which is responsive to antithrombotic treatment, should be considered as a differential diagnosis of mononeuritis multiplex. PMID:26511022

  4. Relapsed Brucellosis Presenting as Neurobrucellosis with Cerebral Vasculitis in a Patient Previously Diagnosed with Brucellar Spondylitis: A Case Report

    PubMed Central

    Ahn, Eun Young; Ryu, Dae Gon; Choi, Yu Hee; Kim, Tae Hyun

    2015-01-01

    Brucellosis is a multisystem disease with various clinical symptoms. Neurobrucellosis is a rare but serious manifestation of brucellosis. A 60-year-old man with a previous diagnosis of brucellar spondylitis presented with sudden onset of aphasia and numbness of the right upper extremity. Cerebral angiography showed diffuse narrowing and dilatation on the distal branches of both the anterior cerebral artery (ACA) and the left middle cerebral artery (MCA) which indicated cerebral vasculitis, and the patient's Brucella agglutinin titer was 1:1280. After combined antimicrobial and steroid therapy was started, the patient's condition improved significantly, and he was discharged after 1 month. Antimicrobial therapy was continued for 16 months on an outpatient basis, and the last Brucella agglutinin titer was 1:40. To our knowledge, this is the first case of relapsed neurobrucellosis with vasculitis in Korea to have been treated successfully. PMID:26788412

  5. Relapsed Brucellosis Presenting as Neurobrucellosis with Cerebral Vasculitis in a Patient Previously Diagnosed with Brucellar Spondylitis: A Case Report.

    PubMed

    Kim, Eun Jung; Lee, Su Jin; Ahn, Eun Young; Ryu, Dae Gon; Choi, Yu Hee; Kim, Tae Hyun

    2015-12-01

    Brucellosis is a multisystem disease with various clinical symptoms. Neurobrucellosis is a rare but serious manifestation of brucellosis. A 60-year-old man with a previous diagnosis of brucellar spondylitis presented with sudden onset of aphasia and numbness of the right upper extremity. Cerebral angiography showed diffuse narrowing and dilatation on the distal branches of both the anterior cerebral artery (ACA) and the left middle cerebral artery (MCA) which indicated cerebral vasculitis, and the patient's Brucella agglutinin titer was 1:1280. After combined antimicrobial and steroid therapy was started, the patient's condition improved significantly, and he was discharged after 1 month. Antimicrobial therapy was continued for 16 months on an outpatient basis, and the last Brucella agglutinin titer was 1:40. To our knowledge, this is the first case of relapsed neurobrucellosis with vasculitis in Korea to have been treated successfully. PMID:26788412

  6. Novel CFI mutation in a patient with leukocytoclastic vasculitis may redefine the clinical spectrum of Complement Factor I deficiency.

    PubMed

    Bay, Jakob Thaning; Katzenstein, Terese Lea; Kofoed, Kristian; Patel, Dustin; Skjoedt, Mikkel-Ole; Garred, Peter; Schejbel, Lone

    2015-10-01

    Factor I is an important regulator of the complement system. Lack of Factor I causes uncontrolled activation of the complement system leading to consumption of C3. Complete deficiency of Factor I is a rare condition and only around 40 cases has been reported in the literature. The clinical presentation of Factor I deficiency varies and includes severe recurrent bacterial infections, glomerulonephritis and autoimmune diseases. The patient, a 28-years old woman with consanguineous parents, presented with recurrent leukocytoclastic vasculitis in the lower extremities with no associated systemic involvement, and without increased infection tendency. Initial testing showed low C3 concentration and a detailed complement evaluation absence of complement Factor I. Sequencing revealed a homozygous missense mutation in exon 2 of the CFI gene (SCV000221312). Even though the clinical symptoms of CFI mutations vary among patients sole association with leukocytoclastic vasculitis redefines the clinical spectrum of complete Factor I deficiency. PMID:25988862

  7. A Case of Rituximab Use as an Induction and Maintenance of Remission in ANCA-Associated Vasculitis

    PubMed Central

    Hafiz, Shahd; Albeity, Abdurahman; Almoallim, Hani

    2016-01-01

    Antineutrophil cytoplasmic antibody- (ANCA-) associated vasculitis (AAV) is a multisystem autoimmune disease affecting mainly microscopic blood vessels due to circulating autoantibodies against neutrophil cytoplasmic antigens. We report a case of a 57-year-old female patient presenting with hemoptysis, sinusitis, and conjunctivitis. Based on lung biopsy, the diagnosis of antineutrophil cytoplasmic antibody- (ANCA-) associated vasculitis (AAV) was established. She was put on rituximab as induction and maintenance therapy. She responded initially to rituximab as induction therapy but failed to respond in the maintenance course of the drug. Rituximab was stopped and mycophenolate mofetil was administered. She responded as laboratory c-ANCA titers turned negative and symptoms subsided. There are no randomized clinical trials addressing rituximab effect in induction and remission at the same time. This case report doubts the efficacy of the use of rituximab therapy for both induction and maintenance of remission at the same time, waiting for the results of the ongoing trials. PMID:27006851

  8. 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

  9. Hepatotropic Viral Infection Associated Systemic Vasculitides—Hepatitis B Virus Associated Polyarteritis Nodosa and Hepatitis C Virus Associated Cryoglobulinemic Vasculitis

    PubMed Central

    Sharma, Aman; Sharma, Kusum

    2013-01-01

    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

  10. 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

  11. 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.

  12. 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

  13. Peripheral capillary nonperfusion and full-field electroretinographic changes in eyes with frosted branch-like appearance retinal vasculitis

    PubMed Central

    Matsui, Yoshitsugu; Tsukitome, Hideyuki; Uchiyama, Eriko; Wada, Yuko; Yagi, Tatsuya; Matsubara, Hisashi; Kondo, Mineo

    2013-01-01

    We report a patient with frosted branch-like appearance retinal vasculitis associated with peripheral capillary nonperfusion and full-field electroretinographic changes. A 62-year-old man presented with sudden bilateral decreased vision accompanied by headaches. His best-corrected visual acuity was 0.01 in both eyes. Fundus examination and fluorescein angiography showed bilateral frosted branch-like appearance retinal vasculitis, and spectral-domain optical coherence tomography showed severe macular edema in both eyes. The cerebrospinal fluid analyses showed an increased lymphocyte count and protein levels. He was treated with systemic corticosteroid therapy, and his best-corrected visual acuity improved to 0.8 OD and 1.0 OS at 6 months after onset. However, fluorescein angiography showed a lack of capillary perfusion in the periphery, and the oscillatory potentials on full-field electroretinography were severely reduced in both eyes. These findings indicated extensive retinal ischemia and inner retinal dysfunction, and that fluorescein angiography and full-field electroretinograms can be useful during follow-up of eyes with frosted branch-like appearance retinal vasculitis. PMID:23355770

  14. Spotlight on rituximab in the treatment of antineutrophil cytoplasmic antibody-associated vasculitis: current perspectives

    PubMed Central

    Moog, Philipp; Thuermel, Klaus

    2015-01-01

    A 54-year-old patient presented to his general practitioner because of strong muscle pain in both thighs. Inflammatory parameters (CRP 16.3 mg/dL) and white blood cells (15 g/L) were elevated. The patient reported a weight loss of 10 kg in 4 weeks. There was no fever or any other specific symptoms. Urine dipstick examination and computed tomography of the chest were unremarkable. Because of increasing symptoms, the patient was referred to our department. Magnetic resonance tomography showed diffuse inflammatory changes of the muscles of both thighs. Neurological examination and electrophysiology revealed axonal sensorimotor neuropathy and ground-glass opacities of both lungs had occurred. Serum creatinine increased to 229 μmol/L within a few days, with proteinuria of 3.3 g/g creatinine. Kidney biopsy showed diffuse pauci-immune proliferative glomerulonephritis. Proteinase 3-specific antineutrophil cytoplasmic antibodies were markedly increased. Birmingham Vasculitis Activity Score was 35. Within 2 days, serum creatinine further increased to 495 μmol/L. Plasma exchange, high-dose glucocorticosteroids, and hemodialysis were started. The patient received cyclophosphamide 1 g twice and rituximab 375 mg/m2 four times according to the RITUXVAS protocol. Despite ongoing therapy, hemodialysis could not be withdrawn and had to be continued over 3 weeks until diuresis normalized. Glucocorticosteroids were tapered to 20 mg after 2 months, and serum creatinine was 133 μmol/L. However, nephritic urinary sediment reappeared. Another dose of 1 g cyclophosphamide was given, and glucocorticosteroids were raised for another 4 weeks. After 6 months, the daily prednisolone dose was able to be tapered to 5 mg. Serum creatinine was 124 μmol/L, proteinuria further decreased to 382 mg/g creatinine, and the Birmingham Vasculitis Activity Score was 0. Maintenance therapy with rituximab 375 mg/m2 every 6 months was started. At the last visit after 8 months, the patient was still in remission, with only minor persistent dysesthesia of the left foot and a persistent serum creatinine of 133 μmol/L. PMID:26664125

  15. HCV-related cryoglobulinemic vasculitis: an update on its etiopathogenesis and therapeutic strategies.

    PubMed

    Ferri, C; Giuggioli, D; Cazzato, M; Sebastiani, M; Mascia, M T; Zignego, A L

    2003-01-01

    Cryoglobulinemic vasculitis (CV) is an immune-complex-mediated systemic vasculitis involving small-medium sized vessels. A causative role of hepatitis C virus (HCV) in over 4/5 patients has been definitely established on the basis of epidemiological, pathological, and laboratory studies. There is great geographical heterogeneity in the prevalence of CV as well as other HCV-related immuno-lymphoproliferative disorders. Thus, unknown environmental and/or genetic co-factors should contribute to the pathogenesis of these conditions. Due to the biological properties, HCV genomic sequences cannot be integrated into the host genome; the virus could trigger the immunological alterations only indirectly by exerting a chronic stimulus to the immune system. Recent laboratory observations gave us new important insights on the complex pathogenetic mechanism(s) of HCV-related CV. Firstly, the HCV envelop protein E2, able to bind CD81 molecule expressed on B-lymphocytes, might be involved in the first steps of HCV-driven autoimmune and lymphoproliferative phenomena. The interaction between HCV-E2 and CD81 may increase the frequency of VDJ rearrangement in antigen-reactive B-cell. One possible consequence may be the activation of anti-apoptotic Bcl-2 protoncogene that leads to extended B-cell survival. Interestingly, t(14, 18) translocation along with Bcl-2 activation have been demonstrated in B-lymphocytes of 80% HCV-related CV. The B-lymphocyte expansion is responsible for a wide autoantibody and immune-complex production, including mixed cryoglobulins. CV shows a relatively benign clinical course; however, its cumulative survival is significantly worse if compared to general population. For a correct therapeutic approach to HCV-related CV we must deal with conflicting conditions: HCV infection, autoimmune, and lymphoproliferative alterations. Therapeutic strategy of CV includes etiologic, pathogenetic, and/or symptomatic therapies, which should be tailored for the single patient according to the severity of clinical symptoms. A careful clinical monitoring of patients with HCV-related CV is mandatory in all cases, with particular attention to neoplastic complications. PMID:14740431

  16. Peripheral CD5+ B Cells in Antineutrophil Cytoplasmic AntibodyAssociated Vasculitis

    PubMed Central

    Unizony, Sebastian; Lim, Noha; Phippard, Deborah J.; Carey, Vincent J.; Miloslavsky, Eli M.; Tchao, Nadia K.; Ikl, David; Asare, Adam L.; Merkel, Peter A.; Monach, Paul A.; Seo, Philip; St Clair, E. William; Langford, Carol A.; Spiera, Robert; Hoffman, Gary S.; Kallenberg, Cees G. M.; Specks, Ulrich; Stone, John H.

    2015-01-01

    Objective CD5+ B cells have been conceptualized as a possible surrogate for Breg cells. The aim of the present study was to determine the utility of CD5+ B cells as biomarkers in antineutrophil cytoplasmic antibodyassociated vasculitis (AAV). Methods The absolute and relative numbers (percentages) of CD5+ B cells (explanatory variables) were measured longitudinally during 18 months in 197 patients randomized to receive either rituximab (RTX) or cyclophosphamide (CYC) followed by azathioprine (AZA) for the treatment of AAV (Rituximab in ANCA-Associated Vasculitis [RAVE] trial). Outcome variables included disease activity (status of active disease versus complete remission), responsiveness to induction therapy, disease relapse, disease severity, and, in RTX-treated patients, relapse-free survival according to the percentage of CD5+ B cells detected upon B cell repopulation. Results CD5+ B cell numbers were comparable between the treatment groups at baseline. After an initial decline, absolute CD5+ B cell numbers progressively increased in patients in the RTX treatment arm, but remained low in CYC/AZA-treated patients. In both groups, the percentage of CD5+ B cells increased during remission induction and slowly declined thereafter. During relapse, the percentage of CD5+ B cells correlated inversely with disease activity in RTX-treated patients, but not in patients who received CYC/AZA. No significant association was observed between the numbers of CD5+ B cells and induction treatment failure or disease severity. The dynamics of the CD5+ B cell compartment did not anticipate disease relapse. Following B cell repopulation, the percentage of CD5+ B cells was not predictive of time to flare in RTX-treated patients. Conclusion The percentage of peripheral CD5+ B cells might reflect disease activity in RTX-treated patients. However, sole staining for CD5 as a putative surrogate marker for Breg cells did not identify a subpopulation of B cells with clear potential for meaningful clinical use. Adequate phenotyping of Breg cells is required to further explore the value of these cells as biomarkers in AAV. PMID:25332071

  17. IgA vasculitis (formerly Henoch-Schnlein purpura) in an adult with systemic lupus erythematosus.

    PubMed

    Bernardino, Vera; Mendes-Bastos, Pedro; Rodrigues, Ana; Riso, Nuno

    2015-01-01

    We report a case of a 65-year-old man with systemic lupus erythematosus (SLE) and antiphospholipid syndrome, presenting palpable purpuric lesions, necrotic blisters and swelling ankles, after a previous tracheobronchitis episode. Laboratory data were remarkable for mild proteinuria and imaging studies were normal. A skin biopsy showed IgA deposits on superficial dermal capillaries and IgA vasculitis (IgAV) (former Henoch-Schnlein purpura) was assumed. The patient was treated with colchicine, deflazacort and azathioprine, but as a regression in the purpuric lesions was noted, a decline in renal function was detected. A kidney biopsy revealed mesangial proliferation with IgA deposition and IgAV nephritis was considered. Immunosuppressive treatment was adjusted, with progressive normalisation of renal function and disappearance of proteinuria over a monthly follow-up; after 6?months, total remission was achieved. To the best of our knowledge, this is the first reported case of IgAV in an adult patient with SLE. PMID:26354836

  18. Genetic Susceptibility to ANCA-Associated Vasculitis: State of the Art

    PubMed Central

    Bonatti, Francesco; Reina, Michele; Neri, Tauro Maria; Martorana, Davide

    2014-01-01

    ANCA-associated vasculitis (AAV) is a group of disorders that is caused by inflammation affecting small blood vessels. Both arteries and veins are affected. AAV includes microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) renamed from Wegeners granulomatosis, and eosinophilic granulomatosis with polyangiitis (EGPA), renamed from ChurgStrauss syndrome. AAV is primarily due to leukocyte migration and resultant damage. Despite decades of research, the mechanisms behind AAV disease etiology are still not fully understood, although it is clear that genetic and environmental factors are involved. To improve the understanding of the disease, the genetic component has been extensively studied by candidate association studies and two genome-wide association studies. The majority of the identified genetic AAV risk factors are common variants. These have uncovered information that still needs further investigation to clarify its importance. In this review, we summarize and discuss the results of the genetic studies in AAV. We also present the novel approaches to identifying the causal variants in complex susceptibility loci and disease mechanisms. Finally, we discuss the limitations of current methods and the challenges that we still have to face in order to incorporate genomic and epigenomic data into clinical practice. PMID:25452756

  19. Rapidly progressive IgA nephropathy: a form of vasculitis or a complement-mediated disease?

    PubMed

    Rojas-Rivera, Jorge; Fernndez-Jurez, Gema; Praga, Manuel

    2015-10-01

    A rapidly progressive and crescentic IgA nephropathy (IgAN) is uncommon, but it has a high risk of progression to end-stage renal disease and variable response to immunosuppression. The importance of a positive anti-neutrophil cytoplasmic antibody (ANCA) serology in this group of patients is not fully understood but may have prognostic significance. On the other hand, there is growing evidence of the role of complement in the pathogenesis of IgAN, especially in cases of crescentic IgAN. Therapies directed against the complement system are a potential and rational therapeutic approach. In this issue, two clinical studies of crescentic IgAN are presented. The first work, is a retrospective case-control study describing clinical presentation, histological findings and response to treatment of crescentic IgAN/positive ANCA patients, comparing them with IgAN/negative ANCA patients and ANCA vasculitis patients. The second is a case report showing the effect of eculizumab, a humanized monoclonal antibody that is a terminal cascade complement inhibitor, as salvage therapy for crescentic IgAN resistant to conventional immunosuppression. Both studies broaden our approach to patients with aggressive forms of IgAN. PMID:26413269

  20. 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

  1. 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-Surez, 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

  2. 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

  3. Activation-induced cytidine deaminase in B cells of hepatits C virus-related cryoglobulinaemic vasculitis.

    PubMed

    Russi, S; Dammacco, F; Sansonno, S; Pavone, F; Sansonno, D

    2015-12-01

    Immunoglobulin variable region heavy chain (IgVH ) somatic gene diversification is instrumental in the transformation process that characterizes hepatitis C virus (HCV)-related B cell lymphoproliferative disorders. However, the extent to which activation-induced cytidine deaminase (AID), an enzyme essential for IgV gene somatic hypermutation (SHM), is active in cryoglobulinaemic vasculitis (CV) remains unclear. AID mRNA expression in the peripheral blood of 102 chronically hepatitis C virus (HCV)-infected patients (58 with and 44 without CV) and 26 healthy subjects was investigated using real-time reverse transcription-polymerase chain reaction (RT-PCR). The features of activation-induced cytidine deaminase (AID) protein and mRNA transcripts were explored in liver tissue biopsies and portal tracts isolated using laser capture microdissection. In chronically HCV-infected patients, AID mRNA expression was almost threefold higher in those with than in those without CV and sevenfold higher than in healthy subjects (median-fold: 6.68 versus 2.54, P = 0.03 and versus 0.95, P = 0.0003). AID transcript levels were significantly higher in polyclonal than in clonally restricted B cell preparations in either CV or non-CV patients (median-fold, 15.0 versus 2.70, P = 0.009 and 3.46 versus 1.58, P = 0.02, respectively). AID gene expression was found to be related negatively to age and virological parameters. AID protein was found in portal tracts containing inflammatory cells that, in several instances, expressed AID mRNA transcripts. Our data indicate that the aberrant expression of AID may reflect continuous B cell activation and sustained survival signals in HCV-related CV patients. PMID:26219420

  4. Positron Emission Tomography scanning in Anti-Neutrophil Cytoplasmic Antibodies-Associated Vasculitis

    PubMed Central

    Kemna, Michael J.; Vandergheynst, Frdric; V, Stefan; Blocklet, Didier; Nguyen, Thomas; Timmermans, Sjoerd A.M.E.G.; van Paassen, Pieter; Cogan, Elie; van Kroonenburgh, Marinus J.P.G.; Tervaert, Jan Willem Cohen

    2015-01-01

    Abstract Tools for evaluation of disease activity in patients with anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) include scoring clinical manifestations, determination of biochemical parameters of inflammation, and obtaining tissue biopsies. These tools, however, are sometimes inconclusive. 2-deoxy-2-[18F]-fluoro-D-glucose (FDG) positron emission tomography (PET) scans are commonly used to detect inflammatory or malignant lesions. Our objective is to explore the ability of PET scanning to assess the extent of disease activity in patients with AAV. Consecutive PET scans made between December 2006 and March 2014 in Maastricht (MUMC) and between July 2008 and June 2013 in Brussels (EUH) to assess disease activity in patients with AAV were retrospectively included. Scans were re-examined and quantitatively scored using maximum standard uptake values (SUVmax). PET findings were compared with C-reactive protein (CRP) and ANCA positivity at the time of scanning. Forty-four scans were performed in 33 patients during a period of suspected active disease. All but 2 scans showed PET-positive sites, most commonly the nasopharynx (n?=?22) and the lung (n?=?22). Forty-one clinically occult lesions were found, including the thyroid gland (n?=?4 patients), aorta (n?=?8), and bone marrow (n?=?7). The amount of hotspots, but not the highest observed SUVmax value, was higher if CRP levels were elevated. Seventeen follow-up scans were made in 13 patients and showed decreased SUVmax values. FDG PET scans in AAV patients with active disease show positive findings in multiple sites of the body even when biochemical parameters are inconclusive, including sites clinically unsuspected and difficult to assess otherwise. PMID:25997040

  5. Peg-IFNα/ribavirin/protease inhibitor combination in hepatitis C virus associated mixed cryoglobulinemia vasculitis: results at week 24

    PubMed Central

    Saadoun, David; Resche Rigon, M; Thibault, V; Longuet, M; Pol, S; Blanc, F; Pialoux, G; Karras, A; Bazin-Karra, D; Cazorla, C; Vittecoq, D; Musset, L; Decaux, O; Ziza, J M; Lambotte, O; Cacoub, Patrice

    2014-01-01

    Background The standard-of-care treatment of patients with hepatitis C virus (HCV)-mixed cryoglobulinemia (MC) vasculitis includes pegylated interferon α (PegIFN)-α plus ribavirin and/or rituximab. About 30–40% of patients are non-responders or relapsers to such combination. Objective To analyse the safety and efficacy of Peg-IFNα/ribavirin/protease inhibitor combination in HCV-MC vasculitis. Patients and methods Open-label, prospective, cohort study including 23 patients with HCV-MC vasculitis. Peg-IFNα/ribavirin was associated to telaprevir (375 mg three times daily, for 12 weeks, (n=15)) or boceprevir (800 mg three times daily, for 44 weeks, (n=8)) for 48 weeks. Results The median age was 59 (52.5–66) years, with 48.8% women. Thirteen patients (56.5%) were complete clinical responders, and 10 (43.5%) were partial responders at week 24. The virological response (ie, HCV RNA negativation) was of 69.6% at week 24 (p=0.005). The cryoglobulin level decreased from 0.44 to 0.06 g/l (p=0.0006) and the C4 level increased from 0.09 to 0.15 g/l (p=0.045). Grades 3 and 4 adverse events (mainly anaemia, neutropenia and thrombocytopenia) were observed in 10 cases (43.5%). Twenty patients (87%) received erythropoietin, 9 (39.1%) had red cell transfusion, and 2 (8.7%) had granulocyte stimulating agents. Antiviral therapy discontinuation was required in 8 (34.7%) patients for virological non-response (n=5), virological relapse (n=2) and depression (n=1). Conclusions Peg-IFNα/ribavirin/protease inhibitor combination seems highly effective in HCV-MC. Such therapeutic regimen should be administered cautiously considering the high rate of side effects. PMID:23606708

  6. High Prevalence of Autoantibodies to hLAMP-2 in Anti–Neutrophil Cytoplasmic Antibody–Associated Vasculitis

    PubMed Central

    Tadema, Henko; McKinney, Eoin F.; Benharkou, Alexandra; Brandes, Ricarda; Peschel, Andrea; Hubert, Virginie; Feenstra, Tjerk; Sengölge, Gürkan; Stegeman, Coen; Heeringa, Peter; Lyons, Paul A.; Smith, Kenneth G.C.; Kallenberg, Cees; Rees, Andrew J.

    2012-01-01

    The involvement of autoantibodies to human lysosome-associated membrane protein-2 (hLAMP-2) in anti–neutrophil cytoplasmic antibody (ANCA)–associated vasculitis is controversial because of the absence of confirmatory data subsequent to the initial reports of their high prevalence in this disease. We characterized three assays for anti-hLAMP-2 antibodies: ELISA and Western blotting assays using unglycosylated recombinant hLAMP-2 expressed in Escherichia coli, and an indirect immunofluorescence assay using stably transfected ldlD cells that expressed glycosylated full-length hLAMP-2 on the plasma membrane. The assays detected autoantibodies to hLAMP-2 in human sera reproducibly and with comparable sensitivity and the assays gave the same results in 80.5% of the test panel of 40 selected positive and negative sera. In untreated patients at presentation, the frequencies of autoantibodies to LAMP-2 were 89%, 91%, and 80%, respectively, among three groups of patients with ANCA-associated vasculitis from Vienna, Austria (n=19); Groningen, the Netherlands (n=50) and Cambridge, United Kingdom (n=53). Prevalence of LAMP-2 autoantibodies was similar in both those with myeloperoxidase-ANCA and proteinase 3-ANCA. Furthermore, we detected LAMP-2 autoantibodies in two ANCA-negative patients. LAMP-2 autoantibodies rapidly became undetectable after the initiation of immunosuppressive treatment and frequently became detectable again during clinical relapse. We conclude that when robust assays are used, circulating autoantibodies to hLAMP-2 can be detected in most European patients with ANCA-associated vasculitis. Large-scale prospective studies are now needed to determine whether they are pathogenic or merely an epiphenomenon. PMID:22323643

  7. Anti-neutrophil cytoplasmic antibody (ANCA) associated small-vessel vasculitis in a patient with diabetic nephropathy and autoimmune polyendocrinopathy syndrome (APS) Type 2: a case report.

    PubMed

    Murray, Jonathan S; Baines, Laura A; Pearce, Simon H S; Ball, Steve; Leech, Nicola; Wood, Katrina M; Kanagasundaram, Nigel S

    2013-09-01

    We present a 42-year-old woman with pre-existing autoimmune polyendocrinopathy syndrome (APS) Type 2 and chronic kidney disease due to Type 1 diabetic nephropathy, who developed a rapid deterioration in renal function due to perinuclear anti-neutrophil cytoplasmic antibody (pANCA)-associated vasculitis. Although possibly a chance occurrence, ANCA have been detected more frequently in patients with a history of certain autoimmune diseases. Such an association may simply reflect an underlying tendency to immune system dysfunction in these patients and the finding of positive ANCA serology does not reliably herald the development of ANCA-associated vasculitis. However, our case illustrates that positive ANCA serology in such circumstances is not always a benign phenomenon and should still be interpreted within the clinical context. Moreover, clinicians managing patients with pre-existing autoimmune disease should maintain a low threshold for appropriate assessment should such patients develop evidence suggestive of vasculitis. PMID:22541677

  8. 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

  9. 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 ofTrypanosoma 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, andT. cruzitetramer-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. cruziinfection 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

  10. Successful outcome of pregnancy in patients with anti-neutrophil cytoplasm antibody-associated small vessel vasculitis.

    PubMed

    Croft, Adam P; Smith, Stuart W; Carr, Sue; Youssouf, Sajeda; Salama, Alan D; Burns, Aine; Pusey, Charles D; Hamilton, Patrick; Brown, Nina; Venning, Michael; Harper, Lorraine; Morgan, Matthew D

    2015-04-01

    Pregnancy in patients with anti-neutrophil cytoplasm antibody-associated vasculitis is reportedly associated with a high risk of fetal and maternal complications. Here we describe the outcome of pregnancies in patients with granulomatosis with polyangiitis and microscopic polyangiitis at five centers in the United Kingdom using a retrospective case review of all women who became pregnant following diagnosis. We report 15 pregnancies in 13 women resulting in 15 live births including one twin pregnancy and 13 singleton pregnancies. One patient had an unplanned pregnancy and a first trimester miscarriage while taking methotrexate. All other pregnancies were planned following a minimum of 6 months clinical remission. Eleven successful pregnancies were delivered vaginally at full term, whereas three were delivered by cesarean section. All infants were healthy with no neonatal complications on their initial health check within the first 24?h of delivery and no evidence of neonatal vasculitis. One relapse occurred during pregnancy and was successfully treated with an increased dose of azathioprine and corticosteroids, intravenous immunoglobulin, and plasma exchange therapy. One patient developed tracheal crusting and subglottic stenosis of infective etiology in the third trimester requiring tracheal debridement post delivery. No patient had a relapse in the first 12 months postpartum. Thus, successful pregnancy outcomes can occur following planned pregnancy in women in sustained remission on non-teratogenic therapies. PMID:25272233

  11. 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

  12. 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 = 1.372.21), 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 = 3.477.73), 4.89 (95%CI = 2.938.16) and 3.84 (95%CI = 2.725.42) respectively. There was no significant increase in the risk of kidney cancer (SIR = 2.12, 95%CI = 0.666.85), prostate cancer (SIR = 1.45, 95%CI = 0.872.42), colon cancer (SIR = 1.26, 95%CI = 0.702.27), and breast cancer (SIR = 0.95, 95%CI = 0.501.79). Among these site-specific cancers, only NMSC showed moderate heterogeneity (I2 = 55.8%, P = 0.06). No publication bias was found by using the Beggs 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 association between AASV and kidney cancer, prostate cancer, colon cancer and breast cancer. These findings emphasize monitoring and preventative management in AASV patients after cessation of CYC therapy is momentous. PMID:25973882

  13. 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 cerebral amyloid angiopathy. The annual incidence rate of PCNSV was estimated at 2.4 cases per 1,000,000 person-years. PCNSV appears to consist of several subsets defined by the size of the vessels involved, the clinical characteristics at presentation, MRI findings, and histopathological patterns on biopsy. Early recognition and treatment may reduce poor outcomes. PMID:26020379

  14. Is serum HMGB1 a biomarker in ANCA-associated vasculitis?

    PubMed Central

    2013-01-01

    Background Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are systemic inflammatory disorders that include granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), Churg-Strauss syndrome and renal limited vasculitis (RLV). Extracellular high-mobility group box 1 (HMGB1) acts as an alarmin and has been shown to be a biomarker of disease activity as well as an autoantigen in systemic lupus erythematosus (SLE) and, possibly, in AAV. This study aims to assess antibodies against HMGB1 and HMGB1 levels as biomarkers for AAV disease activity and predictors of relapsing disease. Methods AAV patients with active disease and healthy controls (HC) were evaluated for anti-HMGB1 antibodies while serum HMGB1 levels were measured longitudinally in AAV patients at presentation, during remission, prior to and at relapses. Results HMGB1 levels were similar between AAV patients at presentation (n = 52) and HC (n = 35) (2.64 1.80 ng/ml vs. 2.39 1.09 ng/ml; P = 0.422) and no difference regarding HMGB1 levels could be found among AAV disease subsets (GPA: 2.66 1.83 ng/ml vs. MPA: 3.11 1.91 ng/ml vs. RLV: 1.92 1.48 ng/ml; P = 0.369). AAV patients with renal involvement had lower HMGB1 levels than patients without renal involvement at presentation (2.35 1.48 ng/ml vs. 3.52 2.41 ng/ml; P = 0.042). A negative correlation was observed between HMGB1 levels and 24-hour proteinuria (? = -0.361, P = 0.028). Forty-nine AAV patients were evaluated for HMGB1 levels during follow-up and no differences were observed between relapsing and nonrelapsing patients (P = 0.350). No significant increase in HMGB1 levels was observed prior to a relapse compared with the remission period and changes in HMGB1 levels were not associated with an increased risk for relapse in AAV. Positivity for anti-HMGB1 antibodies was low in patients with active AAV (three out of 24 patients). Conclusions Serum HMGB1 levels at presentation are not increased and are lower in patients with renal involvement. Relapses are not preceded or accompanied by significant rises in HMGB1 levels and changes in HMGB1 levels are not related to ensuing relapses. Anti-HMGB1 antibodies are present in only a few patients in AAV. In contrast to SLE, HMGB1 is not a useful biomarker in AAV. PMID:24007972

  15. CD4+ T Cells Sensitized by Vascular Smooth Muscle Induce Vasculitis, and Interferon Gamma Is Critical for the Initiation of Vascular Pathology

    PubMed Central

    Baiu, Dana Carina; Sandor, Matyas; Hart, Michael

    2010-01-01

    Primary vasculitis is the result of idiopathic inflammation in blood vessel walls. T cells are believed to play a critical role, but the nature of the pathological T-cell response remains obscure. In this study, we provide evidence that CD4+ T lymphocytes, activated in the presence of syngeneic vascular smooth muscle cells, were sufficient to induce vasculitic lesions after adoptive transfer to recipient mice. Additionally, the disease is triggered in the absence of antibodies in experiments in which both the donors of stimulated lymphocytes and the transfer recipients were mice that were deficient in B cells. Tracking and proliferation of the transferred cells and their cytokine profiles were assessed by fluorescence tagging and flow cytometry. Proliferating CD4+ T cells were evident 3 days after transfer, corresponding to the occurrence of vasculitic lesions in mouse lungs. The transferred T lymphocytes exhibited Th1 and Th17 cytokine profiles and minimal Th2. However, 1 week after vasculitis induction, effector functions could be successfully recalled in Th1 cells, but not in Th17 cells. Additionally, in the absence of constitutive interferon-? expression, T cells sensitized by vascular smooth muscle cells failed to induce vasculitis. In conclusion, our results show that Th1 cells play a key role in eliciting vasculitis in this murine model and that induction of the disease is possible in the absence of pathogenic antibodies. PMID:20971729

  16. Fibroblasts express OvHV-2 capsid protein in 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), a '-herpesvirus, is an often fatal disease characterized by lymphoproliferation, vasculitis, and mucosal ulceration in American bison (Bison bison), cattle (Bos taurus), and other clinically susceptible speci...

  17. Increased circulating levels of proteinase 3 in patients with anti-neutrophilic cytoplasmic autoantibodies-associated systemic vasculitis in remission

    PubMed Central

    Ohlsson, S; Wieslander, J; Segelmark, M

    2003-01-01

    In systemic small vessel vasculitides, patients form autoantibodies against neutrophil granular proteins, anti-neutrophilic cytoplasmic autoantibodies (ANCA). Some correlation is seen between ANCA titre and disease activity, but whether this is cause or effect is still unknown. It has been reported that levels of proteinase 3 (PR3), one of the main ANCA antigens, are increased in patients with active disease. An increased level of circulating antigen could mean a predisposition to autoimmunity. In order to explore this we measured PR3 levels in patients with stable disease. In addition we measured neutrophil gelatinase-associated lipocalin (NGAL) as a specific marker of neutrophil degranulation, cystatin C as a marker of renal function as well as C-reactive protein (CRP), IL-6 and sTNFr1 as markers of inflammation. PR3, NGAL, IL-6 and sTNFr1 were measured in plasma by the ELISA technique. In the PR3 ELISA, we used anti-PR3 monoclonal antibodies as capture-antibodies and affinity-purified rabbit-anti-PR3 antibodies for detection. PR3-ANCA, myeloperoxidase (MPO)-ANCA, CRP and cystatin C were measured by routine methods. PR3 was significantly raised (P < 00001) in vasculitis patients (median 560 g/l, range 1103940, n = 59) compared with healthy blood donors (350 g/l, 110580, n = 30) as well as disease controls (360, 110580, n = 46). No correlation was seen with disease activity, inflammation or renal function. The raised NGAL levels correlated strongly with decreased renal function (r = 08, P < 0001). After correcting for this, slightly increased levels (110, 42340, n = 59) were observed compared with healthy blood donors (81, 38130, n = 25), but not compared with the disease controls (120, 57260, n = 48). In the disease controls, there was a significant correlation between NGAL and proteinase 3 (r = 03, p < 005), but this was not the case in the vasculitis patients. Whether patients had PR3-ANCA or MPO-ANCA was of no significance. In our measurements, we found significantly raised levels of PR3 in plasma from patients with small vessel vasculitis, regardless of ANCA specificity. This was not due to decreased renal function, ongoing inflammation or neutrophil activation. Plausible mechanisms for this include defects in the reticuloendothelial system, genetic factors and selective neutrophil degranulation or leakage. PMID:12605707

  18. 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

  19. Current and emerging treatment options for ANCA-associated vasculitis: potential role of belimumab and other BAFF/APRIL targeting agents

    PubMed Central

    Lenert, Aleksander; Lenert, Petar

    2015-01-01

    Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) comprises several clinical entities with diverse clinical presentations, outcomes, and nonunifying pathogenesis. AAV has a clear potential for relapses, and shows unpredictable response to treatment. Cyclophosphamide-based therapies have remained the hallmark of induction therapy protocols for more than four decades. Recently, B-cell depleting therapy with the anti-CD20 antibody rituximab has proved beneficial in AAV, leading to Food and Drug Administration approval of rituximab in combination with corticosteroids for the treatment of AAV in adults. Rituximab for ANCA-associated vasculitis and other clinical trials provided clear evidence that rituximab was not inferior to cyclophosphamide for remission induction, and rituximab appeared even more beneficial in patients with relapsing disease. This raised hopes that other B-cell-targeted therapies directed either against CD19, CD20, CD22, or B-cell survival factors, B-cell activating factor of the tumor necrosis factor family (BAFF) and a proliferation-inducing ligand could also be beneficial for the management of AAV. BAFF neutralization with the fully humanized monoclonal antibody belimumab has already shown success in human systemic lupus erythematosus and, along with another anti-BAFF reagent blisibimod, is currently undergoing Phase II and III clinical trials in AAV. Local production of BAFF in granulomatous lesions and elevated levels of serum BAFF in AAV provide a rationale for BAFF-targeted therapies not only in AAV but also in other forms of vasculitis such as Behcet’s disease, large-vessel vasculitis, or cryoglobulinemic vasculitis secondary to chronic hepatitis C infection. BAFF-targeted therapies have a very solid safety profile, and may have an additional benefit of preferentially targeting newly arising autoreactive B cells over non-self-reactive B cells. PMID:25609919

  20. Primary malignant lymphoma of the CNS and polyneuropathy in a patient with necrotizing vasculitis treated with immunosuppression.

    PubMed

    Jellinger, K; Kothbauer, P; Weiss, R; Sunder-Plassmann, E

    1979-01-01

    A woman, aged 58, who had undergone prolonged treatment with corticosteroids for generalized necrotizing vasculitis, and had received azathioprine (Imurel) for 18 months, 4 years after discontinuing the latter treatment, developed peripheral neuropathy and a rapidly progressing cerebral disorder suggesting a basal meningeal process. CSF cytology suggested malignant lymphoma with meningeal involvement. Immunological studies showed an increase of Null-lymphocytes in the peripheral blood. Autopsy disclosed a primary malignant lymphoma of the CNS with the histological appearance of a multilocular immunoblastoma showing almost ubiquitous meningocerebral involvement. Clinical and postmortem examinations failed to demonstrate any systemic extraneural lymphoproliferative disorder. In addition, there was peripheral polyneuropathy of the axonal type with denervation atrophy of skeletal muscle, but without lymphomatous involvement of the neuromuscular system. The possible relations between primary malignant lymphomas of the CNS and previous immunosuppressive treatment of immunoinflammatory disease are discussed in view of the concept of impaired immunoregulation. The pathogenetic background of peripheral polyneuropathy is unknown. PMID:90135

  1. Urinary Levels of High Mobility Group Box-1 Are Associated with Disease Activity in Antineutrophil Cytoplasmic Autoantibody-Associated Vasculitis

    PubMed Central

    Wang, Chen; Chang, Dong-Yuan; Zhao, Ming-Hui; Chen, Min

    2015-01-01

    Background High mobility group box-1 (HMGB1), a kind of pro-inflammatory mediator, is associated with inflammatory conditions and tissue damage. Our previous study demonstrated that the circulating levels of HMGB1 correlated with disease activity of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). In the current study, we aimed to measure urinary levels of HMGB1 in AAV patients, correlated them to clinical activity index and analysed the immunohistochemical HMGB1 staining in kidney specimens. Methods 50 patients with AAV in active stage and 56 patients with AAV in remission were recruited. The urinary levels of HMGB1 were determined by enzyme-linked immunosorbent assay. Moreover, renal biopsy specimens from 27 patients with active AAV were randomly collected to evaluate the deposition of HMGB1. Results Urinary HMGB1 levels in AAV patients in active stage were significantly higher than those in AAV patients in remission and healthy controls (1.46 [0.56-3.43] versus 0.38 [0.10-1.35] mg/μmolCr, P=0.001; 1.46 [0.56-3.43] versus 0.48 [0.40-0.60] mg/μmolCr, P=0.000, respectively). Further analysis found that urinary levels of HMGB1 correlated with erythrocyte sedimentation rate (r=0.354, p=0.012), C-reactive protein (r=0.289, p=0.042), and Birmingham Vasculitis Activity Score (r=0.350, p=0.013). Renal tissue of active AAV patients showed HMGB1 was mainly expressed in the cytoplasm and the extracellular space. The percentage of HMGB1-negative nuclei in renal tissue of patients with active AAV was significantly higher than that in normal controls (60.6±20.2 % versus 2.7±0.6 %, p<0.01). Conclusion Urinary levels of HMGB1 may be associated with the disease activity in AAV patients. PMID:25884225

  2. 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

  3. Diagnostic Value of Procalcitonin in ANCA-Associated Vasculitis (AAV) to Differentiate Between Disease Activity, Infection and Drug Hypersensitivity

    PubMed Central

    Herrmann, K; Schinke, S; Csernok, E; Moosig, F; Holle, J.U

    2015-01-01

    Objective: Procalcitonin (PCT) is considered to be a specific marker for severe bacterial infections and sepsis. Elevated PCT levels have been reported in active autoimmune diseases without infection. The aim of this study was to assess the diagnostic value of PCT serum levels in ANCA-associated vasculitis (AAV) patients with respect to infection, disease activity and drug fever using a high sensitive PCT detection method. Methods: In 53 AAV patients with elevated C-reactive protein (CRP) PCT was determined by the Thermo Scientific BRAHMS PCT sensitive KRYPTOR assay. Patients underwent standardized diagnostic procedures for evaluation of disease activity and infection. Results: 53 patients with AAV and elevated CRP (7.76.9 mg/dl, PCT 0.341.02 ng/ml) were assessed, 10 had infection with elevated CRP levels of 11.210.2 mg/dl and PCT levels of 1.062.07 ng/dl. 43 patients had no evidence of infection, 36 of them were presented with AAV with normal or only slightly positive PCT levels in active disease (n=36) (PCT 0.060.06 ng/ml). 7 patients had increased PCT levels due to azathioprine hypersensitivity (0.761.01 ng/ml). For discrimination between infection and vasculitis activity PCT was more useful than CRP with the best cut-off at 0.1 ng/ml (sensitivity 60%, specificity 92%). Conclusion: In contrast to previous studies using semiquantitative PCT assays, the KRYPTOR performs better with respect to discrimination of infection from active AAV. In all patients assessed with active AAV (and without infection) PCT levels remained below the PCT reference limit (0.5 ng/ml) for infections. Drug hypersensitivity seems to be an important differential diagnosis in the setting of elevated CRP and PCT in patients who receive azathioprine. PMID:26535070

  4. Long-Term Follow-Up of Cyclophosphamide Compared with Azathioprine for Initial Maintenance Therapy in ANCA-Associated Vasculitis

    PubMed Central

    Faurschou, Mikkel; Berden, Annelies; Flossmann, Oliver; Bajema, Ingeborg; Hoglund, Peter; Smith, Rona; Szpirt, Wladimir; Westman, Kerstin; Pusey, Charles D.; Jayne, David R.W.

    2014-01-01

    Background and objectives Treatment with azathioprine within 3 months of remission induction with cyclophosphamide is a common treatment strategy for patients with ANCA-associated vasculitis. This study comprised patients undergoing long-term follow-up who were randomly allocated to azathioprine after 36 months or after 12 months of cyclophosphamide treatment. Design, setting, participants, & measurements Patients from 39 European centers between 1995 and 1997 with a new diagnosis of ANCA-associated vasculitis that involved the kidneys or another vital organ were eligible. At the time of diagnosis, participants were randomly allocated to convert to azathioprine after 36 months (the azathioprine group) or after 12 months of cyclophosphamide (the cyclophosphamide group). Patients who did not achieve a remission within 6 months were excluded. This study assessed relapses, ESRD, and death during long-term follow-up. Results Patients were allocated to the azathioprine group (n=71) and the cyclophosphamide group (n=73). Of these patients, 63 (43.8%) developed a relapse, 35 (24.3%) developed a renal relapse, 13 (9.0%) developed ESRD, and 21 (14.6%) died. Although there were worse outcomes in the azathioprine group, none were statistically significant. The subdistribution hazard ratio [sHR] for relapse was 1.63 (95% confidence interval [95% CI], 0.99 to 2.71), the composite of relapse or death hazard ratio [HR] was 1.59 (95% CI, 1.00 to 2.54), the ESRD sHR was 1.71 (95% CI, 0.56 to 5.19), and the death HR was 0.75 (95% CI, 0.32 to 1.79). Conclusions It remains uncertain whether converting to azathioprine after 36 months of induction cyclophosphamide therapy is as effective as converting after 12 months. Outcomes are still poor for this group of patients and further research is required to determine the optimal timing of maintenance therapy. PMID:24970876

  5. 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

  6. 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

  7. 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 doseresponse 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

  8. 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

  9. Persistent fever, neck swelling, and small vessel vasculitis following tonsillectomy in a patient with Behets disease: a case report

    PubMed Central

    2012-01-01

    Introduction Behets disease commonly presents with recurrent oral and genital mucocutaneous ulcerations, uveitis and various skin manifestations. Other clinical symptoms include gastrointestinal ulcerations, arthritis, venous thrombosis, arterial aneurysms and central nervous system affection. Vasculitis underlies most clinical symptoms of Behets disease. Case presentation We report the case of a 62-year-old European Caucasian woman with Behets disease who presented with persistent fever and neck soft-tissue swelling, despite broad antibiotic treatment, two weeks after acute tonsillitis and a tonsillectomy. Diffuse epi- and mesopharyngeal swelling shown on a computed tomography scan of her neck and persistently elevated serum markers of inflammation initially prompted suspicion of an infectious etiology. Magnet resonance imaging of her neck and a neck tissue biopsy finally confirmed small vessel vasculitis involving skin, subcutaneous tissue and muscle. Considering the clinical presentation, past medical history and histological findings, we interpreted our patients symptoms as a flare of Behets disease. Immunosuppressive treatment led to rapid clinical improvement. Conclusion A patient with Behets disease developed small vessel vasculitis of the soft tissue of her neck after tonsillitis and a tonsillectomy. Infection and surgery probably triggered a flare of Behets disease. PMID:23110825

  10. 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

  11. Hydralazine Induces Myeloperoxidase and Proteinase 3 Anti-Neutrophil Cytoplasmic Antibody Vasculitis and Leads to Pulmonary Renal Syndrome

    PubMed Central

    Sultan, Ghayyath; Werner, Sherry L.; Hura, Claudia

    2014-01-01

    We report a case of hydralazine-induced ANCA-associated glomerulonephritis with pulmonary hemorrhage. A 62-year-old Hispanic man with hypertension, who was being treated with hydralazine 100?mg three times a day for four and half years, presented to the hospital with severe anemia. He had acute kidney injury and urinalysis showed proteinuria, dysmorphic RBCs, and rare RBC cast. CT scan of the chest revealed bilateral pulmonary ground-glass infiltrates. Transbronchial biopsy was consistent with pulmonary hemorrhage. Serologic tests showed high titer PR3 ANCA and, to a lesser extent, MPO ANCA. Kidney biopsy revealed focal segmental necrotizing glomerulonephritis with crescents, without evidence of immune complex deposits. Hydralazine was discontinued and the patient was treated with corticosteroids and intravenous cyclophosphamide. At one-year follow-up, he had no symptoms and anemia had resolved. Kidney function improved dramatically. Serology showed undetectable PR3 ANCA and minimally elevated MPO ANCA. To our knowledge, hydralazine-associated PR3 ANCA has not been previously reported. The possibility of ANCA systemic vasculitis should be included in the differential diagnosis of any patient with hydralazine use and pulmonary renal syndrome. This is a potentially life threatening condition requiring prompt cessation of the drug and treatment with glucocorticoids and immunosuppression. PMID:25210633

  12. Mapping of the Outcome Measures in Rheumatology Core Set for Antineutrophil Cytoplasmic AntibodyAssociated 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 antibodyassociated 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

  13. Lymphocytic Vasculitis Involving the Central Nervous System Occurs in Patients with X-linked Lymphoproliferative Disease in the Absence of Epstein-Barr Virus Infection

    PubMed Central

    Talaat, Kawsar R.; Rothman, Jennifer A.; Cohen, Jeffrey I.; Santi, Mariarita; Rorke-Adams, Lucy B.; Choi, John K.; Guzman, Miguel; Zimmerman, Robert; Nallasamy, Sudha; Brucker, Alexander; Quezado, Martha; Pittaluga, Stefania; Patronas, Nicholas J.; Klion, Amy D.; Nichols, Kim E.

    2009-01-01

    X-linked lymphoproliferative disease (XLP) is an immunodeficiency caused by defects in the adaptor molecule SAP. The manifestations of XLP generally occur following Epstein-Barr virus (EBV) infection and include fulminant mononucleosis, hypogammaglobulinemia and lymphoma. In this report, we describe two unrelated patients with fatal T cell-mediated central nervous system vasculitis for whom repeated serologic and molecular testing for EBV was negative. In both patients, clonal T cell populations were observed, but neither demonstrated evidence of lymphoma. Thus, loss of SAP function can lead to dysregulated immune responses characterized by the uncontrolled expansion and activation of T cells independent of EBV infection. PMID:19621458

  14. 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

  15. Intermediate monocytes in ANCA vasculitis: increased surface expression of ANCA autoantigens and IL-1? secretion in response to anti-MPO antibodies

    PubMed Central

    OBrien, Ein C.; Abdulahad, Wayel H.; Rutgers, Abraham; Huitema, Minke G.; OReilly, Vincent P.; Coughlan, Alice M.; Harrington, Mark; Heeringa, Peter; Little, Mark A.; Hickey, Fionnuala B.

    2015-01-01

    ANCA vasculitis encompasses several autoimmune conditions characterised by destruction of small vessels, inflammation of the respiratory tract and glomerulonephritis. Most patients harbour autoantibodies to myeloperoxidase (MPO) or proteinase 3 (PR3). Clinical and experimental data suggest that pathogenesis is driven by ANCA-mediated activation of neutrophils and monocytes. We investigated a potential role for distinct monocyte subsets. We found that the relative proportion of intermediate monocytes is increased in patients versus control individuals, and both MPO and PR3 are preferentially expressed on these cells. We demonstrate that MPO and PR3 are expressed independently of each other on monocytes and that PR3 is not associated with CD177. MPO expression correlates with that of Fc receptor CD16 on intermediate monocytes. Monocyte subsets respond differently to antibodies directed against MPO and PR3, with anti-MPO but not anti-PR3 leading to increased IL-1?, IL-6 and IL-8 production. In concordance with the observed higher surface expression of MPO on intermediate monocytes, this subset produces the highest quantity of IL-1? in response to anti-MPO stimulation. These data suggest that monocytes, specifically, the intermediate subset, may play a role in ANCA vasculitis, and also indicate that substantial differences exist between the effect of anti-MPO and anti-PR3 antibodies on these cells. PMID:26149790

  16. Circulating Level of Neutrophil Extracellular Traps Is Not a Useful Biomarker for Assessing Disease Activity in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis

    PubMed Central

    Ma, Tian-Tian; Zhang, Lu-Xia; Chen, Min; Zhao, Ming-Hui

    2016-01-01

    Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of life-threatening disorders, and frequently affects the kidneys. This study investigated whether the circulating neutrophil extracellular traps (NETs) levels were associated with disease activity of AAV. We collected serum samples from 34 patients with AAV in active stage and 62 patients with AAV in remission. Cell free DNA in serum was quantified using the Quant-iT PicoGreen assay. NETs associated MPO-DNA complexes, citrullinated-histone H3-DNA (cit-H3-DNA) complexes and the concentration of deoxyribonuclease I (DNase I) were quantified using ELISA. The activity of DNase I was quantified using radial enzyme-diffusion method. Associations between circulating levels of NETs with clinico-pathological parameters were analyzed. Serum levels of NETs in active AAV patients were significantly higher than those in healthy controls, and the level of cell free DNA correlated with C-reactive protein (CRP). However, no correlation was found between MPO-DNA complexes or cit-H3-DNA complexes level and CRP. Also there was no significant correlation between NETs level and initial serum creatinine, estimated glomerular filtration rate (eGFR), crescents formation or Birmingham Vasculitis Activity Score (BVAS). Furthermore, there was no significant difference of serum levels of cell free DNA or MPO-DNA complexes between active stage and remission of AAV. In conclusion, circulating levels of NETs cannot be used as a biomarker to assess disease activity in AAV patients. PMID:26840412

  17. Viral RNA in the influenza vaccine may have contributed to the development of ANCA-associated vasculitis in a patient following immunisation.

    PubMed

    Jeffs, Lisa S; Nitschke, Jodie; Tervaert, Jan Willem Cohen; Peh, Chen Au; Hurtado, Plinio R

    2016-04-01

    A number of large studies have demonstrated influenza vaccinations to be safe and effective. However, there have been some sporadic case reports, describing a temporal association of influenza vaccination with onset or relapse of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis. The nature of this association, beyond time of occurrence, remains unknown. The presentation of a previously healthy patient who developed ANCA-associated vasculitis (AAV) shortly after influenza vaccination provided us with the rare opportunity to study the possible mechanisms behind this observation. We tested the ability of different types and batches of influenza vaccines to stimulate proteinase-3 ANCA (PR3-ANCA) production in vitro. We found that only some influenza vaccines stimulated PR3-ANCA production in this patient. We demonstrated that this unusual response was associated with those vaccines that contained viral ribonucleic acid (RNA), the natural ligand for Toll-like receptor-7. Exome sequencing of the patient's DNA did not show any mutation in any of the molecules associated with Toll-like receptor signalling. We propose that hyper-reaction to viral RNA in the influenza vaccine may have contributed to the development of AAV following influenza vaccination in this patient. PMID:26361945

  18. Fibroblasts express OvHV-2 capsid protein in vasculitis lesions of American bison (Bison bison) with experimental sheep-associated malignant catarrhal fever.

    PubMed

    Nelson, Danielle D; Taus, Naomi S; Schneider, David A; Cunha, Cristina W; Davis, William C; Brown, Wendy C; Li, Hong; O'Toole, Donal; Oaks, J Lindsay

    2013-10-25

    American bison (Bison bison) are particularly susceptible to developing fatal sheep-associated malignant catarrhal fever (SA-MCF) caused by ovine herpesvirus-2 (OvHV-2), a ?-herpesvirus in the Macavirus genus. This generally fatal disease is characterized by lymphoproliferation, vasculitis, and mucosal ulceration in American bison, domestic cattle (Bos taurus), and other clinically susceptible species which are considered non-adapted, dead-end hosts. The pathogenesis and cellular tropism of OvHV-2 infection have not been fully defined. An earlier study detected OvHV-2 open reading frame 25 (ORF25) transcripts encoding the viral major capsid protein in tissues of bison with SA-MCF, and levels of viral transcript expression positively correlated with lesion severity. To further define the cellular tropism and replication of OvHV-2 infection in vascular lesions of bison, immunofluorescence studies were performed to identify cell type(s) expressing ORF25 protein within tissues. Cytoplasmic and not nuclear ORF25 protein was demonstrated in predominantly perivascular fibroblasts in six bison with experimentally-induced SA-MCF, and there was no evidence of immunoreactivity in vascular endothelium, smooth muscle, or infiltrating leukocytes. The cytoplasmic distribution of viral major capsid protein suggests that viral replication in perivascular fibroblasts may be abortive in this dead-end host. These findings provide a novel foundation for defining the pathogenesis of vasculitis in non-adapted hosts with SA-MCF. PMID:23953727

  19. Circulating Level of Neutrophil Extracellular Traps Is Not a Useful Biomarker for Assessing Disease Activity in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.

    PubMed

    Wang, Huan; Sha, Li-Li; Ma, Tian-Tian; Zhang, Lu-Xia; Chen, Min; Zhao, Ming-Hui

    2016-01-01

    Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of life-threatening disorders, and frequently affects the kidneys. This study investigated whether the circulating neutrophil extracellular traps (NETs) levels were associated with disease activity of AAV. We collected serum samples from 34 patients with AAV in active stage and 62 patients with AAV in remission. Cell free DNA in serum was quantified using the Quant-iT PicoGreen assay. NETs associated MPO-DNA complexes, citrullinated-histone H3-DNA (cit-H3-DNA) complexes and the concentration of deoxyribonuclease I (DNase I) were quantified using ELISA. The activity of DNase I was quantified using radial enzyme-diffusion method. Associations between circulating levels of NETs with clinico-pathological parameters were analyzed. Serum levels of NETs in active AAV patients were significantly higher than those in healthy controls, and the level of cell free DNA correlated with C-reactive protein (CRP). However, no correlation was found between MPO-DNA complexes or cit-H3-DNA complexes level and CRP. Also there was no significant correlation between NETs level and initial serum creatinine, estimated glomerular filtration rate (eGFR), crescents formation or Birmingham Vasculitis Activity Score (BVAS). Furthermore, there was no significant difference of serum levels of cell free DNA or MPO-DNA complexes between active stage and remission of AAV. In conclusion, circulating levels of NETs cannot be used as a biomarker to assess disease activity in AAV patients. PMID:26840412

  20. Management of large-vessel vasculitis with FDG-PET: a systematic literature review and meta-analysis.

    PubMed

    Soussan, Michael; Nicolas, Patrick; Schramm, Catherine; Katsahian, Sandrine; Pop, Gabriel; Fain, Olivier; Mekinian, Arsene

    2015-04-01

    We aimed to clarify the role of 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) in the management of large-vessel vasculitis (LVV), focusing on 3 issues which are as follows: describe and determine the different FDG-PET criteria for the diagnosis of vascular inflammation, the performance of FDG-PET for the diagnosis of large-vessel inflammation in giant cell arteritis (GCA) patients, and the performance of FDG-PET to evaluate the disease inflammatory activity in Takayasu arteritis (TA) patients. MEDLINE, Cochrane Library, and EMBASE database were searched for articles that evaluated the value of FDG-PET in LVV, from January 2000 to December 2013. Inclusion criteria were American College of Rheumatology criteria for GCA or TA, definition PET positivity threshold, and >4 cases included. Sensitivity (Se) and specificity (Sp) of FDG-PET for the diagnosis of large-vessel inflammation were calculated from each included individual study, and then pooled for meta-analysis with a random-effects model. Twenty-one studies (413 patients, 299 controls) were included in the systematic review. FDG-PET showed FDG vascular uptake in 70% (288/413) of patients and 7% (22/299) of controls. Only vascular uptake equal to or higher than the liver uptake was significantly different between GCA/TA patients and controls (P < 0.001). The meta-analysis of GCA patients (4 studies, 57 patients) shows that FDG-PET has high Se and Sp for the diagnosis of large-vessel inflammation in GCA patients in comparison to controls, with a pooled Se at 90% (95% confidence interval [CI], 79%-93%) and a pooled Sp at 98% (95% CI, 94%-99%). The meta-analysis of TA patients (7 studies, 191 patients) shows that FDG-PET has a pooled Se at 87% (95% CI, 78%-93%) and Sp at 73% (95% CI, 63%-81%) for the assessment of disease activity in TA, with up to 84% Sp, with studies using National Institutes of Health criteria as the disease activity assessment scale. FDG-PET showed good performances in the diagnosis of large-vessel inflammation, with higher accuracy in GCA patients than in TA patients. Although a vascular uptake equal to or higher than the liver uptake appears to be a good criterion for the diagnosis of vascular inflammation, further studies are needed to define the threshold of significance as well as the clinical significance of the vascular uptake. PMID:25860208

  1. 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...

  2. What is Vasculitis?

    MedlinePLUS

    ... more about the condition and its various types, causes, and treatments. Rate This Content: NEXT >> Updated: September 23, 2014 Twitter Facebook YouTube Google+ SITE INDEX ACCESSIBILITY PRIVACY STATEMENT FOIA ...

  3. Living with Vasculitis

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    ... and friends also can help relieve stress and anxiety. Let your loved ones know how you feel and what they can do to help you. Rate This Content: NEXT >> Updated: September 23, 2014 Twitter Facebook YouTube Google+ SITE INDEX ACCESSIBILITY PRIVACY STATEMENT FOIA ...

  4. 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

  5. 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

  6. Gastric presentation (vasculitis) mimics a gastric cancer as initial symptom in granulomatosis with polyangiitis: a case report and review of the literature.

    PubMed

    Zheng, Zhaohui; Ding, Jin; Li, Xueyi; Wu, Zhenbiao

    2015-11-01

    Granulomatosis with polyangiitis (GPA), formerly called Wegener's Granulomatosis, is characterized by necrotizing granulomatous inflammation and belongs to the family of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides. The main clinical symptoms of GPA are vasculitis primarily involving upper and lower respiratory tracts, as well as kidneys. Gastrointestinal manifestations of GPA are less common (0-20 %), with gastric presentation mimicking a gastric cancer as an initial symptom. This is a descriptive case report of one patient, together with systematic review of the literature. We described a 31-year-old Chinese woman who presented with complaints of abdominal distention, anorexia for 2 months. Gastroscopy was carried out for three times, and stomach cancer was suspected. However, histopathology of gastric biopsy revealed a chronic inflammation with mucosal ulceration, frequent neutrophils and lymphocytes infiltration, and local granulomatous formation, whereas no sign of stomach carcinoma was observed. In view of the positive cANCA test, a diagnosis of GPA was considered. From the onset of the GPA in the patients, no other organs have been involved in the disease. The patient was successfully treated with corticosteroids and cyclophosphamide. As shown in the report, patients who present only with gastrointestinal manifestations represent challenges to diagnosis. ANCA testing can serve as a decisive diagnostic tool. Although uncommon, GI involvement may be a major feature in GPA, sometimes presenting as gastric tumor-like lesions. Diagnosis should be considered in patients presenting with GI symptoms accompanied by evidence of systemic vasculitis, and ANCA test should be used as a diagnostic measurement to clarify differential diagnosis. PMID:26248531

  7. 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

  8. BRONCHIAL LESIONS OF MOUSE MODEL OF ASTHMA ARE PRECEDED BY IMMUNE COMPLEX VASCULITIS AND INDUCED BRONCHIAL ASSOCIATED LYMPHOID TISSUE (iBALT)

    PubMed Central

    Guest, Ian; Sell, Stewart

    2015-01-01

    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 hours after a single challenge there is fibrinoid necrosis of arterial walls with deposition of immunoglobulin 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. After 2 challenges, severe eosinophilic vasculitis is present at 6 hours, increases by 72 hours and then declines; B-cell proliferation and significant goblet cell hypertrophy and hyperplasia (GCHTH) and bronchial smooth muscle hypertrophy recur more prominently. After 3 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 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

  9. Haematopoietic stem cell transplantation for vasculitis including Behet's disease and polychondritis: a retrospective analysis of patients recorded in the European Bone Marrow Transplantation and European League Against Rheumatism databases and a review of the literature

    PubMed Central

    Daikeler, Thomas; Ktter, Ina; Tyndall, Chiara Bocelli; Apperley, Jane; Attarbaschi, Andishe; Guardiola, Philippe; Gratwohl, Alois; Jantunen, Esa; Marmont, Alberto; Porretto, Ferdinando; Musso, Maurizio; Maurer, Britta; Rinaldi, Nadia; Saccardi, Riccardo; Tyndall, Alan

    2007-01-01

    Objective To evaluate the feasibility of haematopoietic stem cell transplantation (HSCT) in vasculitis. Methods This is a retrospective analysis of patients who had received HSCT for vasculitic diseases and have been reported to the European League Against Rheumatism autoimmune disease or European Bone Marrow Transplantation ProMISe databases. Information about the disease and outcome was obtained by a questionnaire sent to the referring centres. Response of the disease to HSCT was defined as partial or complete responses according to the ability to reduce immunosuppression after HSCT. In addition, the Medline database was searched for reports on HSCT in patients with vasculitis. Results Detailed information was obtained for 15 patients, whose median age at HSCT was 37?years. The diagnoses were cryoglobulinaemia in four patients, Behet's disease in three patients, Wegener's granulomatosis in three patients, and undifferentiated vasculitis, ChurgStrauss angiitis, polychondritis, Takayasu arteritis and polyarteritis nodosa in one patient each. 14 patients received autologous HSCT and 1 an allogeneic HSCT as the first transplant. In three patients, further transplantation was given because of relapse. The overall response, including all consecutive transplantations (HSCT/patient, n?=?13, median 1.3) to HSCT, was 93%, with 46% complete responses and 46% partial responses; median (range) duration of response at the time of reporting was 45 (1684)?months. Three patients died, one from advanced disease, one from cancer and one from graft?versus?host disease. The Medline search showed five other patients who were effectively treated with HSCT for vasculitic diseases. Conclusion This retrospective study suggests that autologous HSCT is feasible for vasculitis. Its value remains to be tested in prospective controlled studies. PMID:16950809

  10. High-mobility group box-1 protein (HMGB1) is increased in antineutrophilic cytoplasmatic antibody (ANCA)-associated vasculitis with renal manifestations.

    PubMed

    Bruchfeld, Annette; Wendt, Mrten; Bratt, Johan; Qureshi, Abdul R; Chavan, Sangeeta; Tracey, Kevin J; Palmblad, Karin; Gunnarsson, Iva

    2011-01-01

    High-mobility group box 1 (HMGB1) is a nuclear and cytosolic protein that is increasingly recognized as an important proinflammatory mediator actively secreted from monocytes and macrophages and passively released from necrotic cells. In antineutrophilic cytoplasmatic antibody (ANCA)-associated vasculitis (AAV), the kidneys are commonly affected vital organs, characterized by focal necrotizing and/or crescentic pauci-immune glomerulonephritis. The aim of the study was to determine whether HMGB1 serum levels are elevated in AAV with renal manifestations. A total of 30 AAV patients (16 female and 14 male; median age 59 years, range 17-82) with Wegener granulomatosis, microscopic polyangiitis and Churg-Strauss syndrome with available renal biopsies and serum samples were included. In seven cases, serum was also obtained at rebiopsy in remission. HMGB1 was analyzed with Western blot. Birmingham Vasculitis Activity Score (BVAS, version 2003), C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), urinanalysis, creatinine, estimated glomerular filtration rate, sex and age were included in the analysis. Twenty-five episodes of biopsy-proven active disease with BVAS 17.9 4.6 and 13 cases with inactive biopsies and BVAS 2.3 3.7 (P = 0.0001) were identified. CRP, ESR, hematuria and proteinuria were significantly higher in active cases. HMGB1 was significantly elevated (P = 0.01) comparing active with inactive cases (120 48 versus 78 46 ng/mL) and significantly lower in the seven control patients (P = 0.03) at rebiopsy in remission. HMGB1 remained higher in inactive cases compared with historic healthy controls (10.9 10.5 ng/mL). HMGB1 levels did not differ significantly between AAV subgroups. CRP and ESR did not correlate with HMGB1. HMGB1 is significantly increased in AAV with renal involvement. Residual HMGB1 elevation in remission could possibly reflect low-grade inflammatory activity or tissue damage. Future studies may further reveal whether HMGB1 is useful as a marker of disease activity and a predictor of outcome in AAV. PMID:20844833

  11. Giant Cell Arteritis: A Systematic Review of the Qualitative and Semiquantitative Methods to Assess Vasculitis with 18F-Fluorodeoxyglucose Positron Emission Tomography

    PubMed Central

    Puppo, Cristina; Massollo, Michela; Paparo, Francesco; Camellino, Dario; Piccardo, Arnoldo; Shoushtari Zadeh Naseri, Mehrdad; Villavecchia, Giampiero; Rollandi, Gian Andrea; Cimmino, Marco Amedeo

    2014-01-01

    Giant cell arteritis (GCA) is the most common vasculitis affecting medium and large vessels. It shows a close clinical association with polymyalgia rheumatica (PMR), a musculoskeletal inflammatory disorder, which is clinically characterized by girdles pain and stiffness. 18F-Fluorodeoxyglucose (18F-FDG) positron emission tomography (PET) is an effective tool for the diagnosis, grading, and follow-up of patients affected by GCA involving the aorta and its proximal branches, but the lack of a standardized method for the assessment of vascular inflammation remains a critical issue, potentially leading to misclassification. In our systematic review, including 19 original articles for a total of 442 GCA patients (with or without PMR symptoms) and 535 healthy controls, we described the different qualitative, semiquantitative and combined methods that have been proposed throughout the literature for assessing the presence and grading the severity of GCA-related vascular inflammation on 18F-FDG PET scans, focusing on the diagnostic performance and examining their respective advantages and limitations. The majority of the included studies adopted qualitative methods of PET image analysis, which are less sensitive but more specific than semiquantitative ones. Among the semiquantitative approaches, the aortic-to-blood pool uptake ratio of the aortic arch seems to be the most accurate method. PMID:25254211

  12. Leukocyte and serum S100A8/S100A9 expression reflects disease activity in ANCA-associated vasculitis and glomerulonephritis

    PubMed Central

    Pepper, Ruth J; Hamour, Sally; Chavele, Konstantia-Maria; Todd, Sarah K; Rasmussen, Niels; Flint, Shaun; Lyons, Paul A; Smith, Kenneth G C; Pusey, Charles D; Cook, H Terence; Salama, Alan D

    2013-01-01

    Antineutrophil cytoplasm antibody (ANCA)–associated vasculitis (AAV) commonly results in glomerulonephritis, in which neutrophils and monocytes have important roles. The heterodimer calprotectin (S100A8/S100A9, mrp8/14) is a Toll-like receptor-4 ligand found in neutrophils and monocytes and is elevated in inflammatory conditions. By immunohistochemistry of renal biopsies, patients with focal or crescentic glomerular lesions were found to have the highest expression of calprotectin and those with sclerotic the least. Serum levels of calprotectin as measured by ELISA were elevated in patients with active AAV and the levels decreased but did not normalize during remission, suggesting subclinical inflammation. Calprotectin levels in patients with limited systemic disease increased following treatment withdrawal and were significantly elevated in patients who relapsed compared with those who did not. As assessed by flow cytometry, patients with AAV had higher monocyte and neutrophil cell surface calprotectin expression than healthy controls, but this was not associated with augmented mRNA expression in CD14+ monocytes or CD16+ neutrophils. Thus, serum calprotectin is a potential disease biomarker in patients with AAV, and may have a role in disease pathogenesis. PMID:23423260

  13. [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

  14. What Everybody is Doing but No One is Talking About: Use of Complementary and Alternative Medicine in the ANCA Associated Vasculitis Population

    PubMed Central

    Berg, Elisabeth A.B.; McGregor, JulieAnne G.; Burkart, Madelyn E.; Poulton, Caroline J.; Hu, Yichun; Falk, Ronald J.; Hogan, Susan L.

    2014-01-01

    The use and impact of complementary and alternative medicine (CAM) for anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) has not been reported. AAV patients seeking care at our center inquired about CAM, prompting a formal study. Study objectives were to discern how many AAV patients used CAM and its perceived helpfulness in disease management. Methods AAV patients completed a CAM questionnaire between July 2011 and May 2012. Patients were 18 years or older and had biopsy proven and/or clinical evidence of AAV. Medical record abstraction supplemented data. Classification detailed CAM type including Mind or Mind-Body. Perceived helpfulness of CAM was assessed as very, somewhat or not at all/dont know. Results A total of 107 patients participated and were a mean age of 53 (range: 1885), 62% female; 48% proteinase 3 (PR3)-ANCA, 44% myeloperoxidase (MPO)-ANCA and 8% ANCA-negative. Top organs involved included kidney (87%), joints (55%), lung (53%) and upper respiratory (53%). At least one type of CAM treatment or self-help practice was reported by 81% of study participants, with the most frequent being prayer (64%), exercise (27%) and massage therapy (19%). Mind-based practices were used by 28% (excluding prayer) and Mind-Body practices by 14%. Most practices were used to improve wellbeing, and Mind and Mind-Body were deemed very helpful by 83% and 87% respectively. Only 24% of study participants discussed CAM with their physician. Conclusion CAM practices were commonly used to improve well-being and found to be beneficial among AAV patients, but more open discussion is needed about CAM between physicians and patients. PMID:25599078

  15. The effects of duration of glucocorticoid therapy on relapse rate in anti-neutrophil cytoplasm antibody associated vasculitis: A meta-analysis

    PubMed Central

    Walsh, Michael; Merkel, Peter A.; Mahr, Alfred; Jayne, David

    2010-01-01

    Objective Disease relapses are common for patients with anti-neutrophil cytoplasm antibody associated vasculitis (AAV). The role of low-dose glucocorticoids (GC) in relapse prevention is controversial. We undertook a systematic review and meta-analysis to determine if GC target doses influence relapses of AAV. Methods Medline, EMBASE and Cochrane databases were searched for observational studies and randomized controlled trials of treatment of AAV that included a predefined GC treatment plan. The association of GC target dose with the proportion of relapses in studies was assessed using meta-regression and multi-level generalized linear modeling. Results Thirteen studies (983 patients) were identified for inclusion. There were no studies directly comparing GC regimens. We classified 288 patients as having a non-zero GC target dose by study end and 695 patients as having a zero GC target dose by study end. The pooled proportion of patients with a relapse was 36% (95% confidence interval [CI] 25 to 47%). GC regimen was the most significant variable explaining the variability between the proportions of patients with relapses. The proportion of patients with a relapse was 14% (95% CI 10 to 19%) in non-zero GC target dose and 43% (95% CI 33 to 52%) in zero GC target dose studies. Differences other than GC regimens exist between studies that complicate the comparability of trials and isolation of the variability in relapses due to GC target alone. Conclusions Studies with longer courses of GC in AAV are associated with fewer relapses. These results have implications for study design and outcome assessment in clinical trials of AAV. PMID:20235186

  16. Use of SNOMED CT to Represent Clinical Research Data: A Semantic Characterization of Data Items on Case Report Forms in Vasculitis Research

    PubMed Central

    Richesson, Rachel L.; Andrews, James E.; Krischer, Jeffrey P.

    2006-01-01

    Objective To estimate the coverage provided by SNOMED CT for clinical research concepts represented by the items on case report forms (CRFs), as well as the semantic nature of those concepts relevant to post-coordination methods. Design Convenience samples from CRFs developed by rheumatologists conducting several longitudinal, observational studies of vasculitis were selected. A total of 17 CRFs were used as the basis of analysis for this study, from which a total set of 616 (unique) items were identified. Each unique data item was classified as either a clinical finding or procedure. The items were coded by the presence and nature of SNOMED CT coverage and classified into semantic types by 2 coders. Measurements Basic frequency analysis was conducted to determine levels of coverage provided by SNOMED CT. Estimates of coverage by various semantic characterizations were estimated. Results Most of the core clinical concepts (88%) from these clinical research data items were covered by SNOMED CT; however, far fewer of the concepts were fully covered (that is, where all aspects of the CRF item could be represented completely without post-coordination; 23%). In addition, a large majority of the concepts (83%) required post-coordination, either to clarify context (e.g., time) or to better capture complex clinical concepts (e.g., disease-related findings). For just over one third of the sampled CRF data items, both types of post-coordination were necessary to fully represent the meaning of the item. Conclusion SNOMED CT appears well-suited for representing a variety of clinical concepts, yet is less suited for representing the full amount of information collected on CRFs. PMID:16799121

  17. A myelopoiesis gene signature during remission in anti-neutrophil cytoplasm antibody-associated vasculitis does not predict relapses but seems to reflect ongoing prednisolone therapy

    PubMed Central

    Kurz, T; Weiner, M; Skoglund, C; Basnet, S; Eriksson, P; Segelmark, M

    2014-01-01

    A myelopoiesis gene signature in circulating leucocytes, exemplified by increased myeloperoxidase (MPO) and proteinase 3 (PR3) mRNA levels, has been reported in patients with active anti-neutrophil cytoplasm antibody-associated vasculitis (AAV), and to a lesser extent during remission. We hypothesized that this signature could predict disease relapse. mRNA levels of PR3, MPO, selected myelopoiesis transcription factors [CCAAT/enhancer binding protein α (CEBP-α), CCAAT/enhancer binding protein β (CEBP-β), SPI1/PU.1-related transcription factor (SPIB), spleen focus forming virus proviral integration oncogene, PU.1 homologue (SPI1)] and microRNAs (miRNAs) from patient and control peripheral blood mononuclear cells (PBMC) and polymorphonuclear cells (PMN) were analysed and associated with clinical data. Patients in stable remission had higher mRNA levels for PR3 (PBMC, PMN) and MPO (PBMC). PR3 and SPIB mRNA correlated positively in controls but negatively in patient PBMC. Statistically significant correlations existed between PR3 mRNA and several miRNAs in controls, but not in patients. PR3/MPO mRNA levels were not associated with previous or future relapses, but correlated with steroid treatment. Prednisolone doses were negatively linked to SPIB and miR-155-5p, miR-339-5p (PBMC) and to miR-221, miR-361 and miR-505 (PMN). PR3 mRNA in PBMC correlated with time since last flare, blood leucocyte count and estimated glomerular filtration rate. Our results show that elevated leucocyte PR3 mRNA levels in AAV patients in remission do not predict relapse. The origin seems multi-factorial, but to an important extent explainable by prednisolone action. Gene signatures in patients with AAV undergoing steroid treatment should therefore be interpreted accordingly. PMID:24215168

  18. Plasma exchanges for the treatment of severe systemic necrotizing vasculitides in clinical daily practice: Data from the French Vasculitis Study Group.

    PubMed

    de Luna, Gonzalo; Chauveau, Dominique; Aniort, Julien; Carron, Pierre-Louis; Gobert, Pierre; Karras, Alexandre; Marchand-Adam, Sylvain; Maurier, Franois; Hatron, Pierre-Yves; Mania, Alexandre; le Guenno, Guillaume; Bally, Stphane; Bienvenu, Boris; Cardineau, Eric; Goulenok, Tiphaine; Jourde-Chiche, Nomie; Samson, Maxime; Huart, Antoine; Pourrat, Jacques; Tiple, Aurlien; Aumaitre, Olivier; Puchal, Xavier; Heshmati, Farhad; le Jeunne, Claire; Mouthon, Luc; Guillevin, Loc; Terrier, Benjamin

    2015-12-01

    The use of plasma exchanges (PLEX) in systemic necrotizing vasculitides (SNV) still need to be codified. To describe indications, efficacy and safety of PLEX for the treatment of SNV, we conducted a multicenter retrospective study on patients with ANCA-associated vasculitis (AAV) or non-viral polyarteritis nodosa (PAN) treated with PLEX. One hundred and fifty-two patients were included: GPA (n=87), MPA (n=56), EGPA (n=4) and PAN (n=5). PLEX were used for rapidly progressive glomerulonephritis (RPGN) in 126 cases (86%), alveolar hemorrhage in 64 cases (42%), and severe mononeuritis multiplex in 23 cases (15%). In patients with RPGN, there was a significant improvement in renal function compared to baseline value (P<0.0001), the plateau being reached at month 3 after PLEX initiation, and estimated glomerular filtration rate improved especially as the number of PLEX increased. In patients with alveolar hemorrhage, mechanical ventilation was discontinued in all patients after a median time of 15 days. Patients treated for mononeuritis multiplex showed improvement of severe motor weakness. After a median follow of 22 months, 18 deaths (12%) were recorded, mainly in patients with RPGN and within the first 6 months. Incidence of end-stage renal disease and/or death was similar between groups of different baseline renal function, but was increased in MPO-ANCA compared to PR3-ANCA. Adverse events attributable to PLEX were recorded in 63%. No death occurred during PLEX. This large series describes indications, efficacy and safety of PLEX in daily practice. Randomized controlled studies are ongoing to define optimal indications, PLEX regimen and concomitant medications. PMID:26330347

  19. Biotherapies in large vessel vasculitis.

    PubMed

    Pazzola, G; Muratore, F; Pipitone, N; Salvarani, C

    2016-04-01

    The mainstay of therapy of large vessel vasculitides (LVV) remains glucocorticoids (GC). Although most patients initially achieve disease remission, relapses and GC dependence are seen in more than two-thirds of cases. Conventional synthetic disease-modifying antirheumatic drugs (DMARDs) showed little or no steroid sparing effects, while biological agents represent a valid therapeutic option in patients with severe and/or relapsing LVV. PMID:26383765

  20. Cerebral vasculitis presenting as a stroke

    PubMed Central

    Montefort, Maxine; Dashora, Umesh; Chowdhury, Muhammad

    2012-01-01

    A 57-year-old man was admitted with right arm weakness and numbness on the background of intermittent headaches. On examination he was found to have mildly decreased sensation, power was 4/5 on the right side. He had dyspraxia in the right hand and was unable to spell his name. His speech was hesitant and he had left-sided visual field impairment as well as some photophobia. MRI and CT revealed multiple areas of haemorrhage and infarctions raising the possibility of primary angitis of brain. The biopsy confirmed the diagnosis. The patient responded to steroids and immunosuppressants partially. PMID:23188852

  1. Biologics for ANCA-associated vasculitis.

    PubMed

    Murgia, Giuseppe; Firinu, Davide; Manconi, Paolo E; Del Giacco, Stefano R

    2014-01-01

    The antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are a group of necrotizing vasculitides with a potential fatal outcome. Conventional therapy is based on the use of glucocorticoids (GCs) and cyclophosphamide (CYC), which is associated with severe toxic effects and is unable to control the disease activity in some refractory and relapsing cases. Several authors focused their efforts on the identification of safe and more efficient drugs, primarily investigating biological agents. Rituximab (RTX) demonstrated to be an alternative to CYC as remission-induction therapy for microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) in two clinical controlled randomized trials. Contrasting data emerged regarding anti-TNF-? agents, and their use should be limited to some selected refractory or relapsing cases. Mepolizumab (MPZ) and Omalizumab (OMZ) are potentially beneficial treatments for patients with eosinophilic granulomatosis with polyangiitis (EGPA). Hereby, we perform a review focused on the use of biological drugs for AAV treatment. PMID:24998311

  2. Vasculitis Syndromes of the Central and Peripheral Nervous Systems

    MedlinePLUS

    ... to prevent blindness. Most individuals respond well to steroid drugs, such as prednisone and methylprednisolone, but they ... promptly and carefully monitored. Long-term use of steroids can cause harmful side effects, such as collapsing ...

  3. Macrophages in vascular inflammation--From atherosclerosis to vasculitis.

    PubMed

    Shirai, Tsuyoshi; Hilhorst, Marc; Harrison, David G; Goronzy, Jrg J; Weyand, Cornelia M

    2015-05-01

    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

  4. Macrophages in Vascular Inflammation From Atherosclerosis to Vasculitis

    PubMed Central

    Shirai, Tsuyoshi; Hilhorst, Marc; Harrison, David G.; Goronzy, Jrg 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

  5. What Are the Signs and Symptoms of Vasculitis?

    MedlinePLUS

    ... of your body. You also may have a loss of feeling or strength in your hands and feet and shooting pains in your arms and legs. Rate This Content: NEXT >> Updated: January 29, 2016 Twitter Facebook YouTube Google+ SITE INDEX ACCESSIBILITY PRIVACY STATEMENT FOIA OIG CONTACT US National Institutes of ...

  6. 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...

  7. Vasculitis Syndromes of the Central and Peripheral Nervous Systems

    MedlinePLUS

    ... Disorders (NORD) 55 Kenosia Avenue Danbury, CT 06810 orphan@rarediseases.org http://www.rarediseases.org Tel: 203- ... 2015 National Institute of Neurological Disorders and Stroke Home | About NINDS | Disorders A - Z | Research Funding | News ...

  8. Extra- and Intracranial Cerebral Vasculitis in Giant Cell Arteritis

    PubMed Central

    Larivière, Delphine; Sacre, Karim; Klein, Isabelle; Hyafil, Fabien; Choudat, Laurence; Chauveheid, Marie-Paule; Papo, Thomas

    2014-01-01

    Abstract Recognizing giant cell arteritis (GCA) in patients with stroke may be challenging. We aimed to highlight the clinical spectrum and long-term follow-up of GCA-specific cerebrovascular accidents. Medical charts of all patients followed in a French Department of Internal Medicine for GCA between January 2008 and January 2014 were retrospectively reviewed. Patients with cerebrovascular accidents at GCA diagnosis were included. Diagnosis of GCA was based on American College of Rheumatology criteria. Transient ischemic attacks and stroke resulting from an atherosclerotic or cardioembolic mechanism were excluded. Clinical features, GCA-diagnosis workup, brain imaging, cerebrospinal fluid (CSF) study, treatment, and follow-up data were analyzed. From January 2008 to January 2014, 97 patients have been followed for GCA. Among them, 8 biopsy-proven GCA patients (mean age 70 ± 7.8 years, M/F sex ratio 3/1) had stroke at GCA diagnosis. Six patients reported headache and visual impairment. Brain MR angiography showed involvement of vertebral and/or basilar arteries in all cases with multiple or unique ischemic lesions in the infratentorial region of the brain in all but one case. Intracranial cerebral arteries involvement was observed in 4 cases including 2 cases with cerebral angiitis. Long lasting lesions on diffusion-weight brain MRI sequences were observed in 1 case. All patients received steroids for a mean of 28.1 ± 12.8 months. Side effects associated with long-term steroid therapy occurred in 6 patients. Relapses occurred in 4 patients and required immunosuppressive drugs in 3 cases. After a mean follow-up duration of 36.4 ± 16.4 months, all but 1 patient achieved complete remission without major sequelae. The conjunction of headache with vertebral and basilar arteries involvement in elderly is highly suggestive of stroke associated with GCA. Intracranial cerebral arteries involvement with cerebral angiitis associated with long lasting brain lesions on diffusion-weight brain MRI sequences may occur in GCA. Both frequent relapses and steroid-induced side effects argue for the use of immunosuppressive agents combined with steroids as first-line therapy. PMID:25526454

  9. Vasculitis: Wegener granulomatosis, Churg-Strauss syndrome, microscopic polyangiitis, polyarteritis nodosa, and Takayasu arteritis.

    PubMed

    Frankel, Stephen K; Sullivan, Eugene J; Brown, Kevin K

    2002-10-01

    Identification, diagnosis, and management of the primary vasculitides and their attendant complications is a challenging task for the critical care physician. However, with appropriate therapy, the morbidity and mortality of these diseases can be markedly improved and allow the individual patient to return to their previous functional state. PMID:12418444

  10. Monocyte Chemoattractant Protein 1 is a Prognostic Marker in ANCA-Associated Small Vessel Vasculitis

    PubMed Central

    Ohlsson, Sophie; Bakoush, Omran; Tencer, Jan; Torffvit, Ole; Segelmark, Mrten

    2009-01-01

    Background. The (anti neutrophil cytoplasmatic autoantibody ANCA), associated small vessel vasculitides (ASVV) are relapsing-remitting inflammatory disorders, involving various organs, such as the kidneys. (Monocyte chemoattractant protein 1 MCP-1) has been shown to be locally up regulated in glomerulonephritis and recent studies have pointed out MCP-1 as a promising marker of renal inflammation. Here we measure urinary cytokine levels in different phases of disease, exploring the possible prognostic value of MCP-1, together with (interleukin 6 IL-6), (interleukin 8 IL-8) and (immunoglobulin M IgM). Methods. MCP-1, IL-6 and IL-8 were measured using commercially available ELISA kits, whereas IgM in the urine was measured by an in-house ELISA. Results. The MCP-1 levels in urine were significantly higher in patients in stable phase of the disease, compared with healthy controls. Patients in stable phase, with subsequent adverse events; had significantly higher MCP-1 values than patients who did not. MCP-1 and IgM both tended to be higher in patients relapsing within three months, an observation, however, not reaching statistical significance. Urinary levels of IL-6 correlated with relapse tendency, and IL-8 was associated with disease outcome. Conclusions. Patients with ASVV have raised cytokine levels in the urine compared to healthy controls, even during remission. Raised MCP-1 levels are associated with poor prognosis and possibly also with relapse tendency. The association with poor prognosis was stronger for U-MCP-1 than for conventional markers of disease like CRP, BVAS, and ANCA, as well as compared to candidate markers like U-IgM and U-IL-8. We thus consider U-MCP-1 to have promising potential as a prognostic marker in ASVV. PMID:19587833

  11. [Renal damage caused by Rhupus syndrome associated with anti-neutrophil cytoplasmic antibodies vasculitis and cryoglobulinemia].

    PubMed

    Zhao, Xin-ju; Wei, Tao; Dong, Bao; Jia, Yuan; Wang, Mei

    2015-10-18

    We analyzed the clinicopathological characteristics of one patient with Rhupus syndrome associated nephropathy in Peking University People's Hospital, and reviewed the related literature. The patient was a middle aged female. She developed rheumatoid arthritis first, and then manifested mild systemic lupus erythematosus together with positive anti-neutrophil cytoplasmic antibodies (ANCA) and cryoglobulinemia several years later. The renal biopsy was performed and manifested as lupus nephritis. The transmission electron microscopy revealed cryoglobulinemia associated renal damage. This report shows that the clinicopathological characteristics in patients with Rhupus syndrome associated nephropathy are complicated. The renal pathology can be used as a diagnostic tool. PMID:26474633

  12. Vasculitis with renal involvement in essential mixed cryoglobulinemia: Case report and mini-review.

    PubMed

    Anis, Sabiha; Abbas, Khawar; Mubarak, Mohammad; Ahmed, Ejaz; Bhatti, Sajid; Muzaffar, Rana

    2014-05-16

    The discovery of a strong association between hepatitis C virus (HCV) infection and mixed cryoglobulinemia (MC) has led to an increasingly rare diagnosis of idiopathic essential MC (EMC). The incidence of EMC is high in regions where there is a comparatively low HCV infection burden and low in areas of high infection prevalence, including HCV. The diagnosis of EMC requires an extensive laboratory investigation to exclude all possible causes of cryoglobulin formation. In addition, although cryoglobulin testing is simple, improper testing conditions will result in false negative results. Here, we present a 46-year-old female patient with a case of EMC with dermatological and renal manifestations, highlighting the importance of extensive investigation to reach a proper diagnosis. We review the need for appropriate laboratory testing, which is often neglected in clinical practice and which can result in false negative results. This review also emphasizes the significance of an extended testing repertoire necessary for better patient management. Despite a strong association of MC with HCV infection and other causes that lead to cryoglobulin formation, EMC remains a separate entity. Correct diagnosis requires proper temperature regulation during sample handling, as well as characterization and quantification of the cryoprecipitate. Inclusion of rheumatoid factor activity and complement levels in the cryoglobulin test-panel promotes better patient management and monitoring. Consensus guidelines should be developed and implemented for cryoglobulin detection and the diagnosis of cryoglobulinemic syndrome, which will reduce variability in inter-laboratory reporting. PMID:24868518

  13. EMC viral infection of the coronary blood vessels in newborn mice: viral vasculitis.

    PubMed Central

    Burch, G. E.; Rayburn, P.

    1977-01-01

    The coronary arteries, veins and capillaries of newborn mice experimentally infected with encephalomyocarditis (EMC) virus were studied histologically and electronmicroscopically. Damage to these coronary vessels was patchy. Some lesions were extensive, particularly when adjacent to areas of myocardial damage. The histological findings consisted of swelling of adventitial and endothelial cells pyknosis, and polymorphonuclear leucocyte and round cell infiltration. Histological and ultrastructural changes occurred in all three layers of the vessels. EMC viral crystals were found electronmicroscopically in the adventitia of the coronary arteries and veins. Ultrastructural lesions typical of virocytonecrosis were frequently noted in association with viral crystals, establishing the viral etiology of the lesions. These findings lend support to the concept that the patchy atherosclerotic and arteriosclerotic lesions found in the coronary vessels of man may represent healed lesions of previous viral damage. Images Fig. 1 Fig. 2 Fig. 3 Fig. 4 Fig. 5 Fig. 6 Fig. 7 PMID:201264

  14. ANCA negative eosinophilic granulomatosis with polyangiitis: sometimes it really IS vasculitis.

    PubMed

    Tyagi, Niharika; Maheswaran, Tim; Wimalaratna, Sunil

    2015-01-01

    A 21-year-old woman, with a background of asthma, presented to medical admissions ward, with diarrhoea and vomiting; the clinical picture during her admission evolved to include acute shortness of breath, seizures, unsteadiness, low mood and apathy. Investigations revealed pericardial, pleural effusions, myocardial infiltration and vasculitic lesions in the brain. Although serological tests for autoantibodies were negative, an eventual diagnosis of eosinophilic granulomatosis with polyangiitis, formerly known as Churg-Strauss syndrome, was performed based on the clinical picture. The multiorgan involvement meant that the initial diagnosis and effective management required multidisciplinary input from cardiology, neurology, rheumatology, psychiatry, immunology and occupational and physiotherapy. PMID:26698203

  15. Recent Understanding on Diagnosis and Management of Central Nervous System Vasculitis in Children

    PubMed Central

    Iannetti, Ludovico; Zito, Roberta; Bruschi, Simone; Papetti, Laura; Ulgiati, Fiorenza; Nicita, Francesco; Del Balzo, Francesca; Spalice, Alberto

    2012-01-01

    Central nervous system vasculitides in children may develop as a primary condition or secondary to an underlying systemic disease. Many vasculitides affect both adults and children, while some others occur almost exclusively in childhood. Patients usually present with systemic symptoms with single or multiorgan dysfunction. The involvement of central nervous system in childhood is not frequent and it occurs more often as a feature of subtypes like childhood polyarteritis nodosa, Kawasaki disease, Henoch Schnlein purpura, and Bechet disease. Primary angiitis of the central nervous system of childhood is a reversible cause of severe neurological impairment, including acute ischemic stroke, intractable seizures, and cognitive decline. The first line therapy of CNS vasculitides is mainly based on corticosteroids and immunosuppressor drugs. Other strategies include plasmapheresis, immunoglobulins, and biologic drugs. This paper discusses on current understanding of most frequent primary and secondary central nervous system vasculitides in children including a tailored-diagnostic approach and new evidence regarding treatment. PMID:23008735

  16. Intestinal Behet's Disease: A True Inflammatory Bowel Disease or Merely an Intestinal Complication of Systemic Vasculitis?

    PubMed

    Kim, Duk Hwan; Cheon, Jae Hee

    2016-01-01

    Behet'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 ? 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

  17. Acute necrotising pulmonary vasculitis and pulmonary hypertension in a juvenile dog.

    PubMed

    Russell, N J; Irwin, P J; Hopper, B J; Olivry, T; Nicholls, P K

    2008-07-01

    A five-month-old female Jack Russell terrier was presented for investigation of acute lethargy, anorexia, coughing, respiratory distress and weakness. Examination findings included cyanosis, a grade 3 of 6 systolic heart murmur and prolonged capillary refill time. Radiography and echocardiography revealed severe pulmonary hypertension, cor pulmonale and right-sided heart failure. Indirect measurement of the systolic pulmonary artery pressure estimated pressures over 100 mmHg. Despite treatment the patient died. Post-mortem examination did not identify a congenital cardiovascular anomaly. Histopathology confirmed acute necrotising pulmonary arteritis and immunohistochemistry failed to identify any immune complex or complement deposition. PMID:18638059

  18. Hypogalactosylation of serum IgG in patients with ANCA-associated systemic vasculitis

    PubMed Central

    HOLLAND, M; TAKADA, K; OKUMOTO, T; TAKAHASHI, N; KATO, K; ADU, D; BEN-SMITH, A; HARPER, L; SAVAGE, C O S; JEFFERIS, R

    2002-01-01

    The triad of small vessel vasculitides (SVV) comprise Wegener's granulomatosis (WG), microscopic polyangiitis (MPA) and Churg–Strauss syndrome (CS). All three are associated with presence of circulating IgG antineutrophil cytoplasm antibodies (ANCA) which target autoantigens contained, primarily, within neutrophil azurophilic granules. The widely accepted model of pathogenesis suggests that ANCA activate cytokine-primed neutrophils within the microvasculature, leading to by-stander damage to endothelial cells, and rapid escalation of inflammation with recruitment of mononuclear cells. Activation may be initiated, in vitro, by the coligation of the PR3 or MPO antigen, translocated to the cell surface, and FcγRIIa/FcγRIIIb receptors. This suggests that the IgG subclass profile of ANCA and, possibly, its glycosylation status could influence the inflammatory mechanisms activated. The glycosylation status of total IgG isolated from the sera of patients with WG (13), MPA (6) and CSS (1) was determined by analysis of the released oligosaccharides. A deficit in IgG galactosylation is demonstrated for all patient samples, compared to controls. The mean percentage values for the agalactosylated (G0) oligosaccharides were 57% (SD ± 9·71), 47% (SD ± 4·25) and 28% (SD ± 4·09) for WG, MPO and control samples, respectively. The G0 levels for polyclonal IgG isolated from the sera of both WG and MPA patients were significantly increased compared to controls (P < 0·0001). The major glycoform present therefore is agalactosylated (G0) IgG. In previous studies the G0 glycoform of IgG has been shown to bind and activate mannan binding lectin, and hence to activate the complement cascade, and to facilitate mannose receptor binding and the uptake of IgG complexes by macrophages and dendritic cells. Both of these activities could impact on the processing and presentation of self-antigens in autoimmune disease. PMID:12100039

  19. Extra- and intracranial cerebral vasculitis in giant cell arteritis: an observational study.

    PubMed

    Larivire, Delphine; Sacre, Karim; Klein, Isabelle; Hyafil, Fabien; Choudat, Laurence; Chauveheid, Marie-Paule; Papo, Thomas

    2014-12-01

    Recognizing giant cell arteritis (GCA) in patients with stroke may be challenging. We aimed to highlight the clinical spectrum and long-term follow-up of GCA-specific cerebrovascular accidents. Medical charts of all patients followed in a French Department of Internal Medicine for GCA between January 2008 and January 2014 were retrospectively reviewed. Patients with cerebrovascular accidents at GCA diagnosis were included. Diagnosis of GCA was based on American College of Rheumatology criteria. Transient ischemic attacks and stroke resulting from an atherosclerotic or cardioembolic mechanism were excluded. Clinical features, GCA-diagnosis workup, brain imaging, cerebrospinal fluid (CSF) study, treatment, and follow-up data were analyzed. From January 2008 to January 2014, 97 patients have been followed for GCA. Among them, 8 biopsy-proven GCA patients (mean age 707.8 years, M/F sex ratio 3/1) had stroke at GCA diagnosis. Six patients reported headache and visual impairment. Brain MR angiography showed involvement of vertebral and/or basilar arteries in all cases with multiple or unique ischemic lesions in the infratentorial region of the brain in all but one case. Intracranial cerebral arteries involvement was observed in 4 cases including 2 cases with cerebral angiitis. Long lasting lesions on diffusion-weight brain MRI sequences were observed in 1 case. All patients received steroids for a mean of 28.112.8 months. Side effects associated with long-term steroid therapy occurred in 6 patients. Relapses occurred in 4 patients and required immunosuppressive drugs in 3 cases. After a mean follow-up duration of 36.416.4 months, all but 1 patient achieved complete remission without major sequelae. The conjunction of headache with vertebral and basilar arteries involvement in elderly is highly suggestive of stroke associated with GCA. Intracranial cerebral arteries involvement with cerebral angiitis associated with long lasting brain lesions on diffusion-weight brain MRI sequences may occur in GCA. Both frequent relapses and steroid-induced side effects argue for the use of immunosuppressive agents combined with steroids as first-line therapy. PMID:25526454

  20. 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

  1. The Role of Biological Agents in the Management of Large Vessel Vasculitis (LVV): A Systematic Review and Meta-Analysis

    PubMed Central

    Osman, Mohammed; Pagnoux, Christian; Dryden, Donna M.; Storie, Dale; Yacyshyn, Elaine

    2014-01-01

    Background Giant cell arteritis (GCA) and Takayasu's arteritis (TAA) are large vessel vasculitides (LVV) for which corticosteroids (CS) are the mainstay for treatment. In patients with LVV unable to tolerate CS, biological agents have been used with variable effectiveness. Objective To systematically review the effectiveness and safety of biological agents in patients with LVV. Methods We searched 5 electronic databases (inception to October 2012) and conference abstracts with no language restrictions. Two reviewers independently selected studies, extracted data and assessed methodological quality. Our protocol was registered in PROSPERO. Results We included 25 studies (3 RCTs and 22 case series with ?2 cases). 95 GCA and 98 TAA patients received biological agents. The RCTs using anti-TNF agents (infliximab, etanercept and adalimumab) did not suggest a benefit in GCA. GCA patients receiving tocilizumab, in case series, achieved remission (19 patients) and reduction of corticosteroid dose (mean difference, 16.55 mg/day (95% CI: 26.24, 6.86)). In case series, 75 patients with refractory TAA treated with infliximab discontinued CS 32% of the time. Remission was variably defined and the studies were clinically heterogeneous which precluded further analysis. Conclusion This systematic review demonstrated a weak evidence base on which to assess the effectiveness of biological treatment in LVV. Evidence from RCTs suggests that anti-TNF agents are not effective for remission or reduction of CS use. Tocilizumab and infliximab may be effective in the management of LVV and refractory TAA, respectively, although the evidence comes from case series. Future analytical studies are needed to confirm these findings. PMID:25517966

  2. 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

  3. Genetics Home Reference: Adenosine deaminase 2 deficiency

    MedlinePLUS

    ... inflammation of various tissues, particularly the blood vessels (vasculitis). Signs and symptoms can begin anytime from early ... inflammation of blood vessels throughout the body (systemic vasculitis). However, not all researchers classify ADA2 deficiency as ...

  4. Henoch-Schönlein purpura

    MedlinePLUS

    Leukocytoclastic vasculitis; Anaphylactoid purpura; Vascular purpura ... Ardoin SP, Fels E. Vasculitis syndromes. In: Kliegman RM, Stanton B, St. Geme J, Schor N, Behrman RE, eds. Nelson Textbook of Pediatrics . 19th ed. ...

  5. Granulomatosis with polyangiitis

    MedlinePLUS

    ... Savage COS, Harper L. Antineutrophil Cytoplasm Antibody Associated Vasculitis. In: Firestein GS, Budd RC, Gabriel SE, et ... et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med . 2010;363(3):221. ...

  6. ESR (Erythrocyte Sedimentation Rate) Test

    MedlinePLUS

    ... diagnose certain specific inflammatory diseases, temporal arteritis , systemic vasculitis and polymyalgia rheumatica . A significantly elevated ESR is ... individual has symptoms that suggest polymyalgia rheumatica , systemic vasculitis , or temporal arteritis , such as headaches, neck or ...

  7. Skin Complications of IBD

    MedlinePLUS

    ... chronic illnesses such as IBD. PYODERMA VEGETANS and VASCULITIS are other rare skin disorders, believed to be ... become darkened areas of skin as they heal. Vasculitis, which means “inflammation of the blood vessels,” is ...

  8. Genetics Home Reference: Granulomatosis with polyangiitis

    MedlinePLUS

    ... feature of GPA is inflammation of blood vessels (vasculitis), particularly the small- and medium-sized blood vessels ... of vessels, such as small arteries and veins. Vasculitis causes scarring and tissue death in the vessels ...

  9. Isolated Aortitis

    MedlinePLUS

    The Vasculitis Foundation supports and empowers our community through education, awareness, and research. Subscribe for the VF E-News HERE A A A Youtube LinkedIn Twitter Facebook Vasculitis Awareness Month + Vasculitis Behcet’s Disease Central Nervous System ( ...

  10. Cryoglobulinemia

    MedlinePLUS

    The Vasculitis Foundation supports and empowers our community through education, awareness, and research. Subscribe for the VF E-News HERE A A A Youtube LinkedIn Twitter Facebook Vasculitis Awareness Month + Vasculitis Behcet’s Disease Central Nervous System ( ...

  11. 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

  12. Systemic vasculitides: a critical digest of the most recent literature.

    PubMed

    Stagnaro, C; Cioffi, E; Talarico, R; Della Rossa, A

    2015-01-01

    Herewith we provide our annual digest of the recent literature on systemic vasculitides. In this manuscript, we reviewed all the articles published during the last 12 months on large-, medium- and small-vessel vasculitis and selected the most relevant studies regarding the epidemiology, pathogenesis and management of systemic vasculitis. In particular, we focused the attention on giant cell arteritis, ANCA-associated vasculitis and cryoglobulinaemia. PMID:26016767

  13. [Cutaneous symptoms in vasculitides].

    PubMed

    Pock, L; Stork, J; Becvr, R

    1995-10-01

    Vasculitis has a very variable dermal manifestation. It includes purpura, pustules, bullae, ulcers, nodosits, pomphi etc. Dermal manifestation can represent the initial signs in systemic vasculitis and therefore its early clinical and histopathologic evaluation represents a presupposition for the determination of the subsequent examination route. The study gives information on general morphogenesis, principles of the correctly performed probatory excision, and clinical and histopathologic patterns of individual vasculitis types. (Tab. 2, Ref. 8.). PMID:8620327

  14. Muscle biopsy

    MedlinePLUS

    ... dystrophy Inflammation of the muscle Muscular dystrophy Myopathic changes (destruction of the muscle) Necrosis (tissue death) of muscle Necrotizing vasculitis Traumatic muscle damage Polymyositis ...

  15. 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

  16. Pregnancy and Medically Assisted Conception in Rare Diseases

    ClinicalTrials.gov

    2015-12-15

    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

  17. 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

  18. Developing of Granulomatosis with Polyangiitis during Etanercept Therapy

    PubMed Central

    Ortiz-Sierra, Mara Clara; Echeverri, Andrs Felipe; Tobn, Gabriel J.; Caas, Carlos Alberto

    2014-01-01

    We describe a 67-year-old woman who developed c-ANCA positive vasculitis with involvement in eyes, skin, kidney, peripheral nerves, and upper and lower airway during treatment with etanercept therapy for rheumatoid arthritis. A diagnosis of Granulomatosis with Polyangiitis was done. Thus, anti-TNF therapy may be associated with the development of ANCA positive vasculitis. PMID:24707429

  19. 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

  20. 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

  1. Cold hard facts of cryoglobulinemia: updates on clinical features and treatment advances.

    PubMed

    Ghetie, Daniela; Mehraban, Navid; Sibley, Cailin H

    2015-01-01

    Cryoglobulins are immunoglobulins that precipitate at temperatures less than 37°C. They occur secondary to infectious, autoimmune, and malignant processes. In the Brouet classification, type I cryoglobulinemia is caused by hyperviscosity, whereas type II and III manifestations are caused by vasculitis in target organs (primarily skin, peripheral nerves, and kidney). New classification criteria were recently proposed that may help with study and treatment of cryoglobulinemic vasculitis (CryoVas). Hepatitis C virus is the most common cause of CryoVas and treatment with antivirals can be curative in mild cases, whereas rituximab is highly effective in treating active vasculitis in more severe cases. PMID:25399942

  2. How Is Kawasaki Disease Diagnosed?

    MedlinePLUS

    ... Related Topics Cardiac Catheterization Chest X Ray Echocardiography Electrocardiogram Vasculitis Send a link to NHLBI to someone ... whether Kawasaki disease has affected the heart. EKG (electrocardiogram). This simple test detects and records the heart's ...

  3. 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

  4. A Real World, Observational Registry of Chronic Wounds and Ulcers

    ClinicalTrials.gov

    2014-10-29

    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

  5. Giant Cell Arteritis

    MedlinePLUS

    ... Patient / Caregiver Diseases & Conditions Giant Cell Arteritis Giant Cell Arteritis Fast Facts Giant cell arteritis (GCA) is ... polymyalgia rheumatica (also called PMR). What is giant cell arteritis? GCA is a type of vasculitis or ...

  6. Olecranon nodules in a case of Behet's disease.

    PubMed Central

    Yurdakul, S; Yazici, H; Tzner, N; Aytac, S; Mfto?lu, A U

    1981-01-01

    A 33-year-old male with definite Behet's disease had rheumatoid-like nodules at his elbows. This finding, we believe, represents another manifestation of vasculitis in Behet's disease. Images PMID:7224690

  7. IgA nephropathy

    MedlinePLUS

    ... worse slowly over many years (chronic glomerulonephritis). Risk factors include: A personal or family history of IgA nephropathy or Henoch Schonlein purpura , a form of vasculitis that affects many parts of the body Caucasian or Asian ...

  8. 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.

  9. 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...

  10. 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

  11. What Causes Raynaud's?

    MedlinePLUS

    ... Living With Clinical Trials Links Related Topics Atherosclerosis Pulmonary Hypertension Vasculitis Send a link to NHLBI to someone ... o-SI-tis) Buerger's disease Thyroid problems and pulmonary hypertension also may cause Raynaud's. Repetitive Actions Repetitive actions ...

  12. Methotrexate

    MedlinePLUS

    ... You are here: Home / Vasculitis Treatments / Methotrexate (MTX) Methotrexate (MTX) Methotrexate (MTX) is used to treat forms ... your primary care provider. Frequently Asked Questions Regarding Methotrexate How does MTX work? MTX works by dampening ...

  13. Cerebral angiography

    MedlinePLUS

    ... Vasculitis It is sometimes used to: Confirm a brain tumor Evaluate the arteries of the head and neck ... of place blood vessels may be due to: Brain tumors Bleeding within the skull Aneurysm (bulging of the ...

  14. 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

  15. 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

  16. Isolated polyarteritis nodosa of the large bowel: a case report.

    PubMed

    Gambino, Giovanni; Rizzuto, Maria Rosa; Spallitta, Ivan Salvatore; Rizzo, Aroldo; Branca, Massimo; Guccione, Marzio; Air Farulla, Marco; Scio, Antonio; Nicoli, Nicola

    2008-01-01

    Polyarteritis nodosa is a form of vasculitis that affects several organs. Gastrointestinal involvement is frequent, but cases in which the gastrointestinal tract is the only site of disease are rare. In this paper we report a case of a 70-year-old patient with polyarteritis nodosa restricted to the large intestine, who underwent a total colectomy. The patient had no other signs of chronic vasculitis and for this reason surgical treatment resolved the clinical symptoms. PMID:18709790

  17. 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

  18. [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

  19. Spontaneous closure of patent ductus arteriosus after an episode of Kawasaki disease: a case report

    PubMed Central

    2012-01-01

    Introduction Kawasaki disease is regarded as systemic vasculitis. Many experts believe that not only coronary arteries but also other small arteries are involved during the period of systemic inflammation. However, the evidence to support this point view is limited. Case presentation We report the case of a one-year-four-month-old Taiwanese girl whose patent ductus arteriosus was incidentally found during an episode of Kawasaki disease. The ductus closed spontaneously after the acute phase of Kawasaki disease. Conclusions In this patient, the patent ductus arteriosus may have closed spontaneously after Kawasaki disease due to its involvement in the generalized vasculitis that this disease incurs. This would support the theory that the vasculitis of Kawasaki disease is limited not only to coronary arteries but also to all medium- sized arteries. PMID:22277763

  20. Severe Henoch-Schnlein purpura with infliximab for ulcerative colitis

    PubMed Central

    Song, Yang; Shi, Yan-Hong; He, Chong; Liu, Chang-Qin; Wang, Jun-Shan; Zhao, Yu-Jie; Guo, Yan-Min; Wu, Rui-Jin; Feng, Xiao-Yue; Liu, Zhan-Ju

    2015-01-01

    Infliximab (IFX) is an anti-tumor necrosis factor chimeric antibody that is effective for treatment of autoimmune disorders such as Crohns disease and ulcerative colitis (UC). IFX is well tolerated with a low incidence of adverse effects such as infections, skin reactions, autoimmunity, and malignancy. Dermatological manifestations can appear as infusion reaction, vasculitis, cutaneous infections, psoriasis, eczema, and skin cancer. Here, we present an unusual case of extensive and sporadic subcutaneous ecchymosis in a 69-year-old woman with severe UC, partial colectomy and cecostomy, following her initial dose of IFX. The reaction occurred during infliximab infusion, and withdrawal of IFX led to gradual alleviation of her symptoms. We concluded that Henoch-Schnlein purpura, a kind of leukocytoclastic vasculitis, might have contributed to the development of the bruising. Although the precise mechanisms of the vasculitis are still controversial, such a case highlights the importance of subcutaneous adverse effects in the management of UC with IFX. PMID:26019477

  1. Clinical management of infectious cerebral vasculitides.

    PubMed

    Carod Artal, Francisco Javier

    2016-02-01

    A wide range of infections (virus, bacteria, parasite and fungi) may cause cerebral vasculitides. Headache, seizures, encephalopathy and stroke are common forms of presentation. Infection and inflammation of intracranial vessels may cause pathological vascular remodelling, vascular occlusion and ischemia. Vasculitis in chronic meningitis may cause ischemic infarctions, and is associated with poor outcome. Appropriate neuroimaging (CT-angiography, MR-angiography, conventional 4-vessel angiography) and laboratory testing (specific antibodies in blood and CSF, CSF culture and microscopy) and even brain biopsy are needed to quickly establish the aetiology. Enhancement of contrast, wall thickening and lumen narrowing are radiological signs pointing to an infectious vasculitis origin. Although corticosteroids and prophylactic antiplatelet therapy have been used in infectious cerebral vasculitis, there are no randomized clinical trials that have evaluated their efficacy and safety. Stable mycotic aneurysms can be treated with specific antimicrobial therapy. Endovascular therapy and intracranial surgery are reserved for ruptured aneurysms or enlarging unruptured aneurysms. PMID:26689107

  2. Severe Henoch-Schnlein purpura with infliximab for ulcerative colitis.

    PubMed

    Song, Yang; Shi, Yan-Hong; He, Chong; Liu, Chang-Qin; Wang, Jun-Shan; Zhao, Yu-Jie; Guo, Yan-Min; Wu, Rui-Jin; Feng, Xiao-Yue; Liu, Zhan-Ju

    2015-05-21

    Infliximab (IFX) is an anti-tumor necrosis factor chimeric antibody that is effective for treatment of autoimmune disorders such as Crohn's disease and ulcerative colitis (UC). IFX is well tolerated with a low incidence of adverse effects such as infections, skin reactions, autoimmunity, and malignancy. Dermatological manifestations can appear as infusion reaction, vasculitis, cutaneous infections, psoriasis, eczema, and skin cancer. Here, we present an unusual case of extensive and sporadic subcutaneous ecchymosis in a 69-year-old woman with severe UC, partial colectomy and cecostomy, following her initial dose of IFX. The reaction occurred during infliximab infusion, and withdrawal of IFX led to gradual alleviation of her symptoms. We concluded that Henoch-Schnlein purpura, a kind of leukocytoclastic vasculitis, might have contributed to the development of the bruising. Although the precise mechanisms of the vasculitis are still controversial, such a case highlights the importance of subcutaneous adverse effects in the management of UC with IFX. PMID:26019477

  3. Vessel involvement in giant cell arteritis: an imaging approach.

    PubMed

    Holm, Pieter W; Sandovici, Maria; Slart, Riemer H; Glaudemans, Andor W; Rutgers, Abraham; Brouwer, Elisabeth

    2016-04-01

    Vasculitis is classified based on the size of the involved vessels. The two major forms are small vessel vasculitis and large vessel vasculitis (LVV). Main forms of LVV are Takayasu arteritis, giant cell arteritis (GCA), isolated aortitis and chronic periaortitis. This manuscript will focus on GCA, named after the presence of giant cells in the artery vessel wall. A positive biopsy of the temporal artery is the gold standard for making a diagnosis of GCA. In the past 10 years the introduction of new imaging techniques in GCA patients has revealed a variable prevalence of extra cranial involvement, challenging the temporal artery biopsy as gold standard. Also, imaging has become important not only for diagnosing GCA but also for assessment of vascular damage in GCA and for the evaluation of treatment. PMID:26756121

  4. Budd-chiari syndrome and renal arterial neurysms due to behcet disease: a rare association

    PubMed Central

    Batur, Abdussamet; Dorum, Meltem; Yksekkaya, Hasan Ali; Koc, Osman

    2015-01-01

    Behcet's disease is a multisystemic vasculitis of unknown etiology with a chronic relapsing course. Vasculitis in Behcet's disease with predominant vascular involvement is the only vasculitis that affects both arteries and veins of any size. Involvement of the renal artery and inferior vena cava is rare among the arteries and veins, respectively. When disease affect the veins, it is in the form of thrombosis. Arterial complications include aneurysms, stenosis and occlusions. Both rupture of arterial aneurysm and occlusion of suprahepatic veins, causing Budd-Chiari syndrome, are associated with a high mortality rate. Vascular involvement is more common in male patients than in female patients. Men and patients with a younger age of onset present with a more severe prognosis. In this case report, we describe a very rare cause of intrarenal arterial aneurysm's rupture with previous Budd-Chiari syndrome due to Behcet's disease and successful angiographic embolization of actively bleeding aneurysm. PMID:26491527

  5. Life-Threatening Cryoglobulinemic Patients With Hepatitis C

    PubMed Central

    Retamozo, Soledad; Díaz-Lagares, Cándido; Bosch, Xavier; Bové, Albert; Brito-Zerón, Pilar; Gómez, Maria-Eugenia; Yagüe, Jordi; Forns, Xavier; Cid, Maria C.; Ramos-Casals, Manuel

    2013-01-01

    Abstract Cryoglobulinemia is characterized by a wide range of causes, symptoms, and outcomes. Hepatitis C virus (HCV) infection is detected in 30%–100% of patients with cryoglobulins. Although more than half the patients with cryoglobulinemic vasculitis present a relatively benign clinical course, some may present with potentially life-threatening situations. We conducted the current study to analyze the clinical characteristics and outcomes of HCV patients presenting with life-threatening cryoglobulinemic vasculitis. We evaluated 181 admissions from 89 HCV patients diagnosed with cryoglobulinemic vasculitis consecutively admitted to our department between 1995 and 2010. In addition, we performed a systematic analysis of cases reported to date through a MEDLINE search. The following organ involvements were considered to be potentially life-threatening in HCV patients with cryoglobulinemic vasculitis: cryoglobulinemic, biopsy-proven glomerulonephritis presenting with renal failure; gastrointestinal vasculitis; pulmonary hemorrhage; central nervous system (CNS) involvement; and myocardial involvement. A total of 279 patients (30 from our department and 249 from the literature search) fulfilled the inclusion criteria: 205 presented with renal failure, 45 with gastrointestinal vasculitis, 38 with CNS involvement, 18 with pulmonary hemorrhage, and 3 with myocardial involvement; 30 patients presented with more than 1 life-threatening cryoglobulinemic manifestation. There were 146 (52%) women and 133 (48%) men, with a mean age at diagnosis of cryoglobulinemia of 54 years (range, 25–87 yr) and a mean age at life-threatening involvement of 55 years (range, 25–87 yr). In 232 (83%) patients, life-threatening involvement was the first clinical manifestation of cryoglobulinemia. Severe involvement appeared a mean of 1.2 years (range, 1–11 yr) after the diagnosis of cryoglobulinemic vasculitis. Patients were followed for a mean of 14 months (range, 3–120 mo) after the diagnosis of life-threatening cryoglobulinemia. Sixty-three patients (22%) died. The main cause of death was sepsis (42%) in patients with glomerulonephritis, and cryoglobulinemic vasculitis itself in patients with gastrointestinal, pulmonary, and CNS involvement (60%, 57%, and 62%, respectively). In conclusion, HCV-related cryoglobulinemia may result in progressive (renal involvement) or acute (pulmonary hemorrhage, gastrointestinal ischemia, CNS involvement) life-threatening organ damage. The mortality rate of these manifestations ranges between 20% and 80%. Unfortunately, this may be the first cryoglobulinemic involvement in almost two-thirds of cases, highlighting the complex management and very elevated mortality of these cases. PMID:23974248

  6. A rare case of Behet disease with generalised myositis, cardiomyositis and necrotising fasciitis.

    PubMed

    Ng, Felix; Chiong, Fabian Joon Kiong; Buchanan, Russell; Burrell, Louise M

    2016-01-01

    Behet disease (BD) is a rare relapsing, multisystem vasculitis characterised by recurrent oral and genital ulcers, and uveitis. As an autoimmune small vessel vasculitis, BD can involve other organs including the skin, joints, nervous system, kidney and the gastrointestinal tract. This report describes a 40-year-old woman who presented with an uncommon feature of BD, namely myositis, and who went on to develop myocarditis and polymicrobial necrotising fasciitis. To the best of our knowledge, this is the first reported case of an immunocompromised-associated infection occurring in BD without concurrent immunosuppressive therapy. PMID:26740268

  7. Kawasaki disease.

    PubMed

    Sundel, Robert P

    2015-01-01

    Kawasaki disease (KD) is the archetypal pediatric vasculitis, exemplifying the unique aspects and challenges of vascular inflammation in children. The condition is almost unheard of in adults, is closely associated with infections, and is self-limited, with fever resolving after an average of 12 days even without treatment. Yet KD is also a potentially fatal disease and the most common cause of acquired heart disease in the developed world. Unraveling of the developmental, immunologic, and genetic secrets of Kawasaki disease promises to improve our understanding of vasculitis in particular, and perhaps also to provide a window on the fundamental mysteries of inflammatory diseases in general. PMID:25399940

  8. Giant aortic aneurysm in a child with Takayasu arteritis.

    PubMed

    Halaweish, Ihab; Patel, Himanshu; Si, Ming-Sing

    2016-03-01

    Takayasu arteritis is a chronic, idiopathic, granulomatous vasculitis involving the aorta, its major branches, and occasionally the pulmonary arteries. Although rare in children, it is the third most common vasculitis in the paediatric population. Although aneurysmal disease has been reported in adults with Takayasu arteritis, it is a rare entity in children. We present the case of a 10-year-old boy with a giant ascending and arch aneurysm that necessitated follow-up surgery for a new aneurysm and occlusive disease. This is also the first published case involving endovascular aortic graft placement for the management of vascular sequela of Takayasu arteritis in a child. PMID:26365418

  9. The safety and tolerability of an HIV-1 DNA prime-protein boost vaccine (DP6-001) in healthy adult volunteers.

    PubMed

    Kennedy, Jeffrey S; Co, Mary; Green, Sharone; Longtine, Karen; Longtine, Jaclyn; O'Neill, Melissa A; Adams, Janice P; Rothman, Alan L; Yu, Qiao; Johnson-Leva, Renita; Pal, Ranajit; Wang, Shixia; Lu, Shan; Markham, Phillip

    2008-08-18

    This report describes the safety observations following administration of a polyvalent DNA prime-protein boost HIV-1 vaccine formulated with adjuvant QS21. Local injection site reactions were the most common (65% of subjects), and included type IV delayed-type hypersensitivity (DTH) reactions at prior DNA inoculation sites in 12 of 28 (43%) subjects following protein vaccination. Systemic reactions revealed two cases of vasculitis temporally related to inoculation with recombinant Env protein+QS21 adjuvant. Questions remain regarding the cause of the vasculitis, but the unique DTH observation may have contributed to the high level of immune responses previously reported for this vaccine. PMID:18588934

  10. Recurrent angioedema and urticaria.

    PubMed Central

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

    1993-01-01

    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

  11. The first case of Henoch-Schönlein purpura associated with rosuvastatin: colonic involvement coexisting with small intestine

    PubMed Central

    Gonen, Korcan Aysun; Erfan, Gamze; Oznur, Meltem; Erdogan, Cuneyt

    2014-01-01

    Henoch-Schönlein purpura (HSP) is a systemic vasculitis affecting small vessels. It is the most common systemic vasculitis in children, and is rare in adults. Serious gastrointestinal complications are more common in childhood. Infections and drugs are the most prominent factors in the aetiology. Wall thickening in segments of the small intestine is commonly seen in imaging studies in gastrointestinal system (GIS) involvement. Simultaneous involvement of small intestine and colon is rare. An HSP case involving small intestine and colon in an adult patient due to the use of rosuvastatin, an antihyperlipidaemic agent, is presented, and is first of its kind reported in the literature. PMID:24648473

  12. CanVasc recommendations for the management of antineutrophil cytoplasm antibody (ANCA)-associated vasculitides - Executive summary.

    PubMed

    McGeoch, Lucy; Twilt, Marinka; Famorca, Leilani; Bakowsky, Volodko; Barra, Lillian; Benseler, Susan; Cabral, David A; Carette, Simon; Cox, Gerald P; Dhindsa, Navjot; Dipchand, Christine; Fifi-Mah, Aurore; Goulet, Michele; Khalidi, Nader; Khraishi, Majed M; Liang, Patrick; Milman, Nataliya; Pineau, Christian A; Reich, Heather; Samadi, Nooshin; Shojania, Kam; Taylor-Gjevre, Regina; Towheed, Tanveer E; Trudeau, Judith; Walsh, Michael; Yacyshyn, Elaine; Pagnoux, Christian

    2015-01-01

    The Canadian Vasculitis research network (CanVasc) is composed of physicians from different medical specialties, including rheumatology and nephrology and researchers with expertise in vasculitis. One of its aims was to develop recommendations for the diagnosis and management of antineutrophil cytoplasm antibody (ANCA)-associated vasculitides in Canada. This executive summary features the 19 recommendations and 17 statements addressing general AAV diagnosis and management, developed by CanVasc group based on a synthesis of existing international guidelines, other published supporting evidence and expert consensus considering the Canadian healthcare context. PMID:26557369

  13. The clinical implications of adult-onset henoch-schonelin purpura

    PubMed Central

    2011-01-01

    Henoch-Schonlein Purpura (HSP) is a small vessel vasculitis mediated by IgA-immune complex deposition. It is characterized by the clinical tetrad of non-thrombocytopenic palpable purpura, abdominal pain, arthritis and renal involvement. Pathologically, it can be considered a form of immune complex-mediated leukocytoclastic vasculitis (LCV) involving the skin and other organs. Though it primarily affects children (over 90% of cases), the occurrence in adults has been rarely reported. Management often involves the use of immunomodulatory or immune-suppressive regimens. PMID:21619657

  14. Rituximab Induced Left Bundle Branch Block

    PubMed Central

    Sheikh, Mujeeb; Moza, Ankush; Grubb, Blair P.

    2015-01-01

    Rituximab (a monoclonal antibody directed against CD 20) therapy can be acutely complicated by infusion reactions and cardiac arrhythmia on rare occasions. We report the first case of a new onset left bundle branch block (LBBB) after rituximab therapy for Wegener's vasculitis. PMID:25838875

  15. Atypical Endobronchial Carcinoid with Postobstructive Pneumonia Obscuring the Diagnosis of Granulomatosis with Polyangiitis.

    PubMed

    Ali, Robert; Baldeo, Candice; Onyenekwe, Jesse; Lala, Roshan; Landa, Cristian; Siddiqi, Anwer

    2015-01-01

    Granulomatosis with polyangiitis (GPA), previously termed Wegener's Granulomatosis, is an autoimmune small vessel vasculitis which is highly associated with antineutrophil cytoplasmic antibodies (ANCA) and has varied clinical manifestations. Diagnosis hinges on identifying a combination of clinical features of systemic vasculitis, positive ANCA serology, and histological evidence of necrotizing vasculitis, necrotizing glomerulonephritis, or granulomatous inflammation from a relevant organ biopsy. The American College of Rheumatology has also developed a classification criteria focusing specifically on nasal or oral inflammation, abnormal chest radiograph, and abnormal urinary sediment, along with granulomatous inflammation, which helps to distinguish GPA from other forms of systemic vasculitis. In the case presented below, the diagnosis of GPA was delayed as the patient had a concomitant atypical endobronchial carcinoid which predisposed to postobstructive pneumonia. Fortunately, the papular lesions that developed across her lower limbs prompted further investigations. The return of appropriate serology coincided with progression to alveolar hemorrhage, offering a more complete clinical picture, and when she responded to the combination of steroid, cyclophosphamide, and plasma exchange, the diagnosis of GPA was cinched. PMID:26347844

  16. Severe drug-induced dermatoses.

    PubMed

    Ahronowitz, Iris; Fox, Lindy

    2014-03-01

    A variety of common dermatoses are known to have drug-induced variants. This article discusses the clinical presentation, time frames, reported culprit medications, pathophysiology and management of drug-induced lupus, cutaneous vasculitis, pemphigus, pemphigoid, linear IgA bullous dermatosis, Sweet's syndrome, erythema nodosum, pyoderma gangrenosum, pseudolymphoma, lichen planus, and psoriasis. PMID:25037258

  17. Confusing presentation of chaetomium brain abscess.

    PubMed

    Waqas, Muhammad; Waheed, Shahan; Mangrio, Salman Ahmed; Rashid, Sana; Qadeer, Mohsin; Bari, Ehsan

    2014-12-01

    We put forth the case of a young immunocompetent female who presented with fever and left basal ganglia bleed secondary to vasculitis. After 2 weeks of prednisolone therapy, she continued to deteriorate. MRI scan revealed enhancement of previous hematoma, pus was aspirated surgically and fungal culture grew Chaetomium. PMID:24977715

  18. Panniculitis caused by Mycobacterium monacense mimicking erythema induratum: a case in Ecuador

    PubMed Central

    Romero, J.J.; Herrera, P.; Cartelle, M.; Barba, P.; Tello, S.; Zurita, J.

    2016-01-01

    We report the first case of recently characterized species M. monacense associated with chronic nodular vasculitis, infecting a young woman. This case represents the first isolation of M. monacense from Ecuador. The isolate was identified by conventional and molecular techniques. PMID:26933504

  19. Erythema elevatum diutinum in association with dermatitis herpetiformis.

    PubMed

    Chandrasekaran, Shanmuga Sekar; Rai, Reena; Vedachalam, Sandhya; Dorairaj, Lathika; Palaniraman, Surendran

    2014-01-01

    Erythema elevatum diutinum (EED) is a rare skin disease that initially presents as leucocytoclastic vasculitis and later resolves with fibrosis. Dermatitis herpetiformis is an autoimmune blistering disease characterized by granular deposits of immunoglobulin A (IgA) in dermal papillae. We report a rare association of these two disorders. PMID:24616856

  20. Erythema elevatum diutinum in association with dermatitis herpetiformis

    PubMed Central

    Chandrasekaran, Shanmuga Sekar; Rai, Reena; Vedachalam, Sandhya; Dorairaj, Lathika; Palaniraman, Surendran

    2014-01-01

    Erythema elevatum diutinum (EED) is a rare skin disease that initially presents as leucocytoclastic vasculitis and later resolves with fibrosis. Dermatitis herpetiformis is an autoimmune blistering disease characterized by granular deposits of immunoglobulin A (IgA) in dermal papillae. We report a rare association of these two disorders. PMID:24616856

  1. Benzylthiouracil-Induced Glomerulonephritis

    PubMed Central

    Trimeche Ajmi, Sihem; Braham, Rim; Toumi, Sarra; Chadli Chaieb, Molka; Maaroufi, Amel; Ach, Koussay; Chaieb, Larbi

    2009-01-01

    Vasculitis is a rare complication of antithyroid drugs (ATDs). It was first described with Propylthiouracil (PTU). We report a new case of antineutrophil cytoplasmic antibody (ANCA) vasculitis with glomerulonephritis induced by Benzylthiouracile (BTU). A 50-year-old man with Graves disease treated with BTU developed general malaise and haematuria without skin rash or respiratory involvement. Laboratory data revealed acute renal failure with proteinuria and haematuria. An indirect immunofluorescence test for ANCA was positive, showing a perinuclear pattern with specificity antimyeloperoxidase (MPO). A renal biopsy was performed and revealed pauci-immune extracapillary glomerular nephropathy and necrotic vasculitis lesions. Based on these findings we concluded to the diagnosis of rapidly progressive glomerulonephritis associated with ANCA induced by BTU therapy. The drug was therefore discontinued and the patient was treated with steroids and immunosuppressive treatment during 3 months. Renal failure, proteinuria and haematuria significantly improved within 2 months. However, P-ANCA remained positive until 10 months after drug withdrawal. Thyroid function was kept within normal range using iodine solution. We demonstrated clearly that BTU may induce severe forms of vasculitis with glomerulonephritis. Thus, the ANCA must be measured when confronted to systemic manifestation during treatment. PMID:19721711

  2. Pancreatic mass as an initial manifestation of polyarteritis nodosa: A case report and review of the literature

    PubMed Central

    Yokoi, Yoshihiro; Nakamura, Ippei; Kaneko, Takeshi; Sawayanagi, Tomoki; Watahiki, Youichi; Kuroda, Makoto

    2015-01-01

    Classic polyarteritis nodosa (PAN) that targets medium-sized muscular arteries and microscopic polyangiitis (MPA), characterized by inflammation of small-caliber vessels and the presence of circulating myeloperoxidase anti-neutrophil cytoplasmic antibodies (MPO-ANCA), are distinct clinicopathological entities of systemic vasculitis. A 66-year-old woman presented with fever, cholestasis and positive MPO-ANCA. Radiological examination showed a pancreatic mass compressing the bile duct. Therefore, we performed pancreatoduodenectomy. Histopathological examination revealed that necrotizing vasculitis predominantly affecting the medium-sized vessels, spared arterioles or capillaries in the pancreas, a finding consistent with PAN. Unexpectedly, renal biopsy revealed small-caliber vasculitis and glomerulonephritis, supporting MPA. The initial manifestation of a pancreatic mass associated with vasculitis has only been reported in 7 articles. Its diagnosis is challenging because no reliable clinico-radiological findings have been observed. Clinicians should be aware of such cases and early diagnosis followed by immunosuppression is mandatory. Our findings may reflect a polyangiitis overlap syndrome coexisting between pancreatic PAN and renal MPA. PMID:25624739

  3. Emphysema in nonsmokers: alpha 1-antitrypsin deficiency and other causes.

    PubMed

    Lee, Pyng; Gildea, Thomas R; Stoller, James K

    2002-12-01

    Nonsmokers with signs of emphysema at an earlier age than is typical for emphysema deserve a workup for one of the less common causes of emphysema, which include alpha 1-antitrypsin deficiency, connective tissue diseases, hypocomplementemic urticarial vasculitis syndrome, intravenous drug use, human immunodeficiency virus infection, and several rare metabolic disorders. PMID:12546267

  4. What the Cardiologist Should Know About Cardiac Involvement in Behet Disease.

    PubMed

    Veilleux, Simon-Pierre; O'Connor, Kim; Couture, Christian; Pag, Sylvain; Voisine, Pierre; Poirier, Paul; Dubois, Michelle; Snchal, Mario

    2015-12-01

    Behet disease (BD) is a chronic multisystem inflammatory vasculitis affecting mainly young adults and is characterized by a remitting-relapsing course. In North America, the prevalence is 5.2 per 100,000 population. It is believed that cardiac involvement is one of the most severe complications in patients with BD despite its sporadic occurrence, being greatly correlated with mortality. PMID:26215987

  5. [An unusual course in hairy-cell leukemia with marked abdominal lymphadenopathy, leukemic infiltration of the cornea and skin changes].

    PubMed

    Zák, P; Chrobák, L; Podzimek, K; Hejcmanová, D; Voglová, J; Dulícek, P; Mirová, S

    1996-07-01

    The authors describe a female patient suffering from hairy-cell leukaemia. Already at the onset of the disease, apart from marked splenomegaly, sonography revealed marked retroperitoneal lymphadenopathy. During the subsequent course skin changes developed such as vasculitis and leukaemic infiltrates of the cornea on both eyes. The patient was successfully treated with 2-chlorodeoxyadenosine (2-CdA, Leustatin). PMID:8928422

  6. Textbook of rheumatology

    SciTech Connect

    Turner, R.A.; Wise, C.M.

    1986-01-01

    This book contains 23 papers. Some of the titles are: Diagnostic Radiology in the Rheumatic Diseases; Laboratory Testing in Rheumatology; Arthritis Nursing and the Team Approach in the Management of Rheumatic Disease; The Surgical Management of Arthritis; Vasculities; Neoplasms of Bone and Joints; and Rheumatic Disease of Childhood.

  7. Etiopathogenesis of Behcet's disease.

    PubMed

    Mendoza-Pinto, Claudia; García-Carrasco, Mario; Jiménez-Hernández, Mario; Jiménez Hernández, Cesar; Riebeling-Navarro, Carlos; Nava Zavala, Arnulfo; Vera Recabarren, Mauricio; Espinosa, Gerard; Jara Quezada, Javier; Cervera, Ricard

    2010-02-01

    Bechet's disease (BD) is an inflammatory, multi systemic disease with spontaneous remissions and relapses similar to various autoimmune diseases. BD leads to organ damage, including the eyes, skin, joints, etc., which produces various clinical manifestations. The central histopathologic characteristic is systemic vasculitis with perivascular inflammatory infiltrates. The etiopathogenesis is unknown, although immunological abnormalities, possibly induced by susceptible microbiological pathogens, have been postulated. PMID:19879978

  8. Multiorgan dysfunction syndrome secondary to joint supplement overdosage in a dog.

    PubMed

    Nobles, Irma J; Khan, Safdar

    2015-04-01

    A 5-year-old spayed female Bernese mountain dog, with a chief complaint of vomiting and melena ingested approximately 200 nutritional joint supplement tablets. Despite aggressive therapy, the patient developed a coagulopathy, pancreatitis, peritonitis, acute kidney injury, and was euthanized. Postmortem examination revealed myocardial necrosis, pneumonia, centrilobular hemorrhage and necrosis of the liver, vasculitis, and acute tubular necrosis. PMID:25829554

  9. BRIEF REPORT: ODOCOILEUS HEMIONUS DEER ADENOVIRUS IS RELATED TO THE MEMBERS OF ATADENOVIRUS GENUS

    Technology Transfer Automated Retrieval System (TEKTRAN)

    The Odocoileus hemionus deer adenovirus (OdAdV-1) causes systemic and local vasculitis and proves extremely lethal for mule deer. To characterize the virus, part of the genome flanking the fiber gene was cloned and sequenced. The sequence revealed two open-reading frames that mapped to pVIII hexon...

  10. Phenomics in Autoimmune and Inflammatory Diseases

    ClinicalTrials.gov

    2015-09-04

    Healthy Volunteer; Rheumatoid Arthritis; Ankylosing Spondylitis; Systemic Lupus Erythematosus/Antiphospholipid Syndrome; FMF; Cryopyrin-Associated Periodic Syndromes /TNF-receptor Associated Periodic Syndrome; Vasculitis; Uveitis; Myositis; Crohn's Disease; Ulcerative Rectocolitis; Type 1 Diabetes; Unclassified IAD Knee and/or Hip Arthritis, Muscular Dystrophy

  11. Naturally occurring sheep-associated malignant catarrhal fever in North American pigs

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Two cases of sheep-associated malignant catarrhal fever (MCF) in pigs were diagnosed on a small farm in New York State, and in Kentucky, U.S.A. In both cases initial diagnosis was based on histopathological changes representing typical lymphoproliferative vasculitis in multiple tissues of the affect...

  12. OVINE HERPESVIRUS 2 MRNA EXPRESSION IN CATTLE AND BISON WITH MALIGNANT CATARRHAL FEVER

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Malignant catarrhal fever (MCF) is a fulminant disease of certain susceptible ruminants caused by ovine herpesvirus 2 (OvHV-2). The characteristic lesions of MCF include lymphoproliferation, vasculitis and mucosal ulceration. The pathogenesis of these lesions is very poorly understood, but is most ...

  13. Autologous Peripheral Blood Stem Cell Transplantation in Patients With Life Threatening Autoimmune Diseases

    ClinicalTrials.gov

    2005-06-23

    Purpura, Schoenlein-Henoch; Graft Versus Host Disease; Anemia, Hemolytic, Autoimmune; Rheumatoid Arthritis; Churg-Strauss Syndrome; Hypersensitivity Vasculitis; Wegener's Granulomatosis; Systemic Lupus Erythematosus; Giant Cell Arteritis; Pure Red Cell Aplasia; Juvenile Rheumatoid Arthritis; Polyarteritis Nodosa; Autoimmune Thrombocytopenic Purpura; Takayasu Arteritis

  14. Comparison of PR3-ANCA and MPO-ANCA Epitope Specificity upon Disease Relapse

    EPA Science Inventory

    BACKGROUND Relapse is a major clinical problem in ANCA vasculitis that causes increased morbidity and mortality. Compared to MPO-ANCA patients, patients with PR3-ANCA run a significantly increased risk of experiencing relapses. We hypothesized that a relapsing patient is produ...

  15. Churg-Strauss syndrome: a case report*

    PubMed Central

    Fernandes, Gabriel Lacerda; Teixeira, Arivaldo Arajo; Antn, Ana Graziela Santana; Reis, Alan Timteo Rodrigues; de Freitas, Ana Carolina Rezende; Baslio, Dunya Bachour

    2014-01-01

    Churg-Strauss syndrome is a rare systemic disease primarily characterized by hypereosinophilia, asthma and vasculitis. The lung is the organ most frequently involved. In the present report, the authors describe a relatively rare finding in this disease - the presence of a pulmonary nodule -, while recalling the main radiological findings and the most relevant differential diagnoses. PMID:25741095

  16. Cryoglobulinaemic neuropathy: a further cause of bilateral sciatic neuropathy

    PubMed Central

    Prez, Desire; de la Torre, Ricardo Gmez; Carrio, Isabel; Pinto, Jess; Mors, Germn

    2008-01-01

    Bilateral sciatic neuropathy is a rare condition and it has been described as a compression or entrapment neuropathy but it is an uncommon clinical manifestation due to necrotizing vasculitis. We report an unusual case of cryoglobulinaemic neuropathy in an elderly woman with no underlying infectious or neoplastic cause; acute bilateral sciatic mononeuropathy was the presenting clinical manifestation of the cryoglobulinaemia. PMID:18834546

  17. [What's new in internal medicine?].

    PubMed

    Doutre, M-S

    2013-11-01

    In this paper based on a review of medical articles from September 2012 to September 2013, new data were selected about IgG4-related disease, connections between vitamin D and systemic lupus erythematosus, revised nomenclature of vasculitis, effects of salt on autoimmunity, new autoinflammatory syndromes and some diseases as systemic sclerosis and thrombangiitis obliterans. PMID:24365498

  18. Toxocariasis After Slug Ingestion Characterized by Severe Neurologic, Ocular, and Pulmonary Involvement

    PubMed Central

    Fellrath, Jean-Marc; Magnaval, Jean-François

    2014-01-01

    Human toxocariasis is generally a benign, self-curing disease, and neurologic involvement is quite exceptional. In this study, we report a case of toxocariasis caused by ingestion of an unusual transport host, namely live slugs. The clinical picture comprised eosinophilic lung involvement with severe neurologic disorders in relation to vasculitis as well as retinal detachment. PMID:25734133

  19. Bilateral borderzone brain infarctions in association with heroin abuse.

    PubMed

    Niehaus, L; Meyer, B U

    1998-10-01

    A 25-year-old drug abuser who developed an unusual pattern of cerebral ischemic lesions is presented. Cerebral magnetic resonance imaging revealed bilateral borderzone infarctions which were attributed to a heroin-associated vasculitis of the basal cerebral arteries. Under probatory corticosteroid medication the mild neurological deficits completely disappeared. PMID:9849803

  20. [A cause of palatal necrosis not to ignore].

    PubMed

    Boulagnon, Camille; Kovacs, Ovidiu-Bujor; Patey, Martine

    2015-04-01

    We report a case of pseudotumoral nasal septum and hard palate perforation in a 42-years-old man. The diagnosis retained after differential diagnosis exclusion was necrotic midfacial lesion due to chronic inhalation of cocaine. This condition can mimic vasculitis, primary tumors and granulomatous infections. Differential diagnosis and pathophysiology of this condition will be discussed in this anatomo-clinical case. PMID:25778762

  1. Wegener’s granulomatosis mimicking inflammatory bowel disease and presenting with chronic enteritis

    PubMed Central

    Shahedi, Kamyar; Hanna, Ramy Magdy; Melamed, Oleg; Wilson, James

    2013-01-01

    Wegener’s granulomatosis, also known as anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, is a small vessel vasculitis with primarily pulmonary, renal, and sinus disease manifestations. The prevalence of Wegener’s granulomatosis is three cases per 100,000 patients. Cardiovascular, neurologic, cutaneous, and joint manifestations have been reported in many case reports and case series. Gastrointestinal manifestations are less noted in Wegener’s granulomatosis, although they have been previously reported in the form of intestinal perforation and intestinal ischemia. Additionally, there are characteristic findings of vasculitis that are noted with active Wegener’s granulomatosis of the small bowel. We report a case of an elderly patient who presented with weight loss, diarrhea, and hematochezia. His symptoms were chronic and had lasted for more than 1 year before diagnosis. Inflammatory bowel disease or chronic enteritis due to Salmonella arizonae because of reptile exposure originally were suspected as etiologies of his presentation. The findings of proteinuria, renal failure, and pauci-immune glomerulonephritis on renal biopsy, in conjunction with an elevated c-ANCA titer, confirmed the diagnosis of Wegener’s granulomatosis with associated intestinal vasculitis. This case demonstrates an atypical presentation of chronic duodenitis and jejunitis secondary to Wegener’s granulomatosis, which mimicked inflammatory bowel disease. PMID:24124396

  2. Atypical Endobronchial Carcinoid with Postobstructive Pneumonia Obscuring the Diagnosis of Granulomatosis with Polyangiitis

    PubMed Central

    Ali, Robert; Baldeo, Candice; Onyenekwe, Jesse; Lala, Roshan; Landa, Cristian; Siddiqi, Anwer

    2015-01-01

    Granulomatosis with polyangiitis (GPA), previously termed Wegener's Granulomatosis, is an autoimmune small vessel vasculitis which is highly associated with antineutrophil cytoplasmic antibodies (ANCA) and has varied clinical manifestations. Diagnosis hinges on identifying a combination of clinical features of systemic vasculitis, positive ANCA serology, and histological evidence of necrotizing vasculitis, necrotizing glomerulonephritis, or granulomatous inflammation from a relevant organ biopsy. The American College of Rheumatology has also developed a classification criteria focusing specifically on nasal or oral inflammation, abnormal chest radiograph, and abnormal urinary sediment, along with granulomatous inflammation, which helps to distinguish GPA from other forms of systemic vasculitis. In the case presented below, the diagnosis of GPA was delayed as the patient had a concomitant atypical endobronchial carcinoid which predisposed to postobstructive pneumonia. Fortunately, the papular lesions that developed across her lower limbs prompted further investigations. The return of appropriate serology coincided with progression to alveolar hemorrhage, offering a more complete clinical picture, and when she responded to the combination of steroid, cyclophosphamide, and plasma exchange, the diagnosis of GPA was cinched. PMID:26347844

  3. Anthrax vaccine associated deaths in miniature horses.

    PubMed

    Wobeser, Bruce K

    2015-04-01

    During a widespread anthrax outbreak in Canada, miniature horses were vaccinated using a live spore anthrax vaccine. Several of these horses died from an apparent immune-mediated vasculitis temporally associated with this vaccination. During the course of the outbreak, other miniature horses from different regions with a similar vaccination history, clinical signs, and necropsy findings were found. PMID:25829553

  4. Multiorgan dysfunction syndrome secondary to joint supplement overdosage in a dog

    PubMed Central

    Nobles, Irma J.; Khan, Safdar

    2015-01-01

    A 5-year-old spayed female Bernese mountain dog, with a chief complaint of vomiting and melena ingested approximately 200 nutritional joint supplement tablets. Despite aggressive therapy, the patient developed a coagulopathy, pancreatitis, peritonitis, acute kidney injury, and was euthanized. Postmortem examination revealed myocardial necrosis, pneumonia, centrilobular hemorrhage and necrosis of the liver, vasculitis, and acute tubular necrosis. PMID:25829554

  5. [Vasculitic peripheral neuropathy].

    PubMed

    Oya, Yasushi

    2013-11-01

    The typical clinical manifestation of vasculitic peripheral neuropathy is sensory-dominant multiple mononeuropathy, although it can progress to distal-dominant sensorimotor polyneuropathy. It is painful in most cases. Peripheral nerves may be the most prone to produce symptoms of the vasculitis. Nerve conduction studies show reduced amplitude of M wave or sensory nerve action potential, which depends on the degree of injury of a nerve examined. Wallerian degeneration can cause pseudo-conduction block in the acute stage and temporal dispersion in the chronic stage. However, a definite diagnosis requires histological confirmation. Combined biopsy of the sural nerve and the peroneus brevis muscle can be performed by a single incision. Skin biopsy can also be performed. To increase the diagnostic yield, biopsy specimens are prepared in different manners to observe as many cross sections as possible: frozen unfixed, formalin-fixed paraffin-embedded, and glutaraldehyde-fixed epon embedded specimens, as well as teased fiber preparation of a nerve. Vasculitic peripheral neuropathy usually results from small-vessel vasculitis. There are still controversies regarding the classification of vasculitides. Differential diagnosis of vasculitis includes infection and lymphoma. Delayed diagnosis and treatment of neuropathy result in the impairment of ADL and QOL. Recovery from axonal degeneration usually takes time and is not always possible. Treatment includes corticosteroid, cyclophosphamide, and intravenous immunoglobulin administration; however, the intensity of treatment depends on the disease activity of vasculitis. PMID:24200608

  6. Cutaneous pseudovasculitis.

    PubMed

    Carlson, J Andrew; Chen, Ko-Ron

    2007-02-01

    Cutaneous pseudovasculitis represents a heterogeneous collection of disorders that are capable of simulating cutaneous vasculitis and can be broadly classified into diseases that produce hemorrhage (petechiae, purpura, and ecchymoses) or vessel occlusion with resultant livedo, cyanosis, ulcers, digital necrosis, and/or gangrene. Overlap is not uncommon, but if present, one mechanism dominates. Hemorrhagic pseudovasculitis is due to vessel wall dysfunction (incompetence), which can be related to diverse factors that include vessel wall deposition of metabolic substances (amyloid, calcium), nutritional deficiencies (scurvy), nonvasculitic inflammatory purpura (pigmented purpuric dermatitis, arthropod, viral and drug reactions), degeneration of the vessel wall and supporting stroma (senile/solar purpura), direct vessel wall invasion of infective organisms, coagulation-fibrinolytic disorders (eg, thrombocytopenia), and vessel wall trauma. Cyanotic-infarctive pseudovasculitis is due vaso-occlusion by emboli, thrombi, or fibrointimal hyperplasia (endarteritis obliterans) and includes varied conditions such as purpura fulminans, Coumadin necrosis, antiphospholipid antibody syndrome, cardiac myxoma, cholesterol embolization, calciphylaxis, and radiation arteritis. Delayed and inappropriate diagnosis of pseudovasculitis leads to incorrect management and exposure to potentially deleterious treatment modalities such as corticosteroids and cytotoxic agents. The diagnosis of a pseudovasculitic disorder requires a high index of suspicion and should always be part of the differential diagnosis of vasculitis. Skin biopsy is a crucial step in differentiating pseudovasculitis from authentic vasculitis; absence of histologic evidence of vasculitis, particularly after multiple biopsies, should direct evaluation and diagnosis towards pseudovasculitis. PMID:17284961

  7. Takayasu's Arteritis

    MedlinePLUS

    ... across the world, have been conducting a “Surrogate Markers Study” for the past several years. In this study, the investigators examine blood specimens from patients with vasculitis for the purpose of identifying proteins and other molecules whose presence indicates ongoing inflammation. ...

  8. Vancomycin-induced Henoch-Schnlein purpura: a case report

    PubMed Central

    2012-01-01

    Introduction Henoch-Schnlein purpura is a small-vessel systemic vasculitis. Although its exact pathophysiology remains unknown, Henoch-Schnlein purpura has been reported in association with various medical conditions including hypersensitivity. We report the case of a patient with vancomycin-induced Henoch-Schnlein purpura. Case presentation A 42-year-old Caucasian man who had previously undergone a heart transplant was diagnosed as having an intra-abdominal abscess after he underwent a Hartmann procedure. At 15 days after initiation of antibiotic therapy including vancomycin, he developed a purpuric rash of the lower limbs, arthralgia, and macroscopic hematuria. At that time, our patient was already on hemodialysis for end-stage renal disease. Henoch-Schnlein purpura was diagnosed. After a second 15-day course of vancomycin, a second flare of Henoch-Schnlein purpura occurred. Skin biopsies showed leucocytoclastic vasculitis with IgA deposits and eosinophils in the peri-capillary inflammatory infiltrate, suggesting an allergic mechanism. After vancomycin was stopped, we did not observe any further flares. Only five cases of isolated cutaneous vasculitis, one case of lupus-like syndrome and one case of Henoch-Schnlein purpura after vancomycin treatment have been described to date in the literature. Conclusions Clinicians should be aware that systemic vasculitis can be induced by some treatments. Vancomycin is a widely prescribed antibiotic. Occurrence of rare but serious Henoch-Schnlein purpura associated with vancomycin requires its prompt discontinuation. PMID:22490390

  9. Churg-Strauss syndrome.

    PubMed

    Greco, Antonio; Rizzo, Maria Ida; De Virgilio, Armando; Gallo, Andrea; Fusconi, Massimo; Ruoppolo, Giovanni; Altissimi, Giancarlo; De Vincentiis, Marco

    2015-04-01

    Churg-Strauss syndrome (CSS), alternatively known as eosinophilic granulomatosis with polyangiitis (EGPA), was first described in 1951 by Churg and Strauss as a rare disease characterized by disseminated necrotizing vasculitis with extravascular granulomas occurring exclusively among patients with asthma and tissue eosinophilia. EGPA is classified as a small-vessel vasculitis associated with antineutrophil cytoplasmic antibodies (ANCAs) and the hypereosinophilic syndromes (HESs) in which vessel inflammation and eosinophilic proliferation are thought to contribute to organ damage. Although still considered an idiopathic condition, EGPA is classically considered a Th2-mediated disease. Emerging clinical observations provide compelling evidence that ANCAs are primarily and directly involved in the pathogenesis of AASVs, although recent evidence implicates B cells and the humoral response as further contributors to EGPA pathogenesis. EGPA has traditionally been described as evolving through a prodromic phase characterized by asthma and rhino-sinusitis, an eosinophilic phase marked by peripheral eosinophilia and organ involvement, and a vasculitic phase with clinical manifestations due to small-vessel vasculitis. The American College of Rheumatology defined the classification criteria to distinguish the different types of vasculitides and identified six criteria for EGPA. When four or more of these criteria are met, vasculitis can be classified as EGPA. The French Vasculitis Study Group has identified five prognostic factors that make up the so-called five-factor score (FFS). Patients without poor prognosis factors (FFS=0) have better survival rates than patients with poor prognosis factors (FFS?1). The treatment of patients with CSS must be tailored to individual patients according to the presence of poor prognostic factors. A combination of high-dose corticosteroids and cyclophosphamide is still the gold standard for the treatment of severe cases, but the use of biological agents such as rituximab or mepolizumab seems to be a promising therapeutic alternative. PMID:25500434

  10. State of the Art in the Treatment of Systemic Vasculitides

    PubMed Central

    Luqmani, Raashid Ahmed

    2014-01-01

    Anti-neutrophil cytoplasm antibodies (ANCA) are associated with small vessel vasculitides (AASV) affecting the lungs and kidneys. Structured clinical assessment using the Birmingham Vasculitis Activity Score and Vasculitis Damage Index should form the basis of a treatment plan and be used to document progress, including relapse. Severe disease with organ or life threatening manifestations needs cyclophosphamide or rituximab, plus high dose glucocorticoids, followed by lower dose steroid plus azathioprine, or methotrexate. Additional plasmapheresis is effective for very severe disease, reducing dialysis dependence from 60 to 40% in the first year, but with no effect on mortality or long-term renal function, probably due to established renal damage. In milder forms of ANCA-associated vasculitis, methotrexate, leflunomide, or mycophenolate mofetil are effective. Mortality depends on initial severity: 25% in patients with renal failure or severe lung hemorrhage; 6% for generalized non-life threatening AASV but rising to 30–40% at 5 years. Mortality from GPA is four times higher than the background population. Early deaths are due to active vasculitis and infection. Subsequent deaths are more often due to cardiovascular events, infection, and cancer. We need to improve the long-term outcome, by controlling disease activity but also preventing damage and drug toxicity. By contrast, in large vessel vasculitis where mortality is much less but morbidity potentially greater, such as giant cell arteritis (GCA) and Takayasu arteritis, therapeutic options are limited. High dose glucocorticoid results in significant toxicity in over 80%. Advances in understanding the biology of the vasculitides are improving therapies. Novel, mechanism based therapies such as rituximab in AASV, mepolizumab in eosinophilic granulomatosis with polyangiitis, and tocilizumab in GCA, but the lack of reliable biomarkers remains a challenge to progress in these chronic relapsing diseases. PMID:25352843

  11. [Skin diseases associated with chronic hepatitis C].

    PubMed

    Podnyi, B; Lengyel, G; Hrsing, J; Becker, K; Horvth, A

    1998-11-01

    The authors are discussing hepatic and extrahepatic pathologic processes caused by hepatitis C virus (HCV) infection and they focus their interest to the skin disorders appearing in the presence of chronic, active HCV infections. The trigger of the immunologic processes leading to dermatologic manifestations are the activated T cells (CD8 + cytotoxic T lymphocytes), cytokins, and also the expansion of certain B cells. Pathologic immunologic phenomena may initiate various dermatologic manifestations. Immunoglobulins, immuncomplexes generated by the disease itself are manifested as various forms of cutan vasculitis. In the present series of patients (pts), HCV related skin disorders known from the literature were diagnosed in eleven cases and they were representing 7 different disease entities. These were palpable purpura (3 pts), urticaria, prurigo and alopecia areata (2-2 pts), lichen ruber planus, pruritus and vitiligo (1-1 patient respectively). The case reports of 2 pts, one with palpable purpura (vasculitis purpurica), one with prurigo and vitiligo are presented in details. PMID:9842236

  12. Vasculitic small bowel perforation masquerading as spontaneous bacterial peritonitis in a patient with decompensated liver disease.

    PubMed

    Verma, S; Giles, T E; Tsai, H H

    1999-04-01

    We report on a young patient with decompensated alcohol-induced liver disease (Child-Pugh score C) who presented with clinical, biochemical and radiological evidence suggestive of spontaneous bacterial peritonitis. She was however subsequently found to have multiple small bowel perforations, which were diagnosed only at laparotomy. The histology of the bowel showed evidence of vasculitis. This case illustrates two important points. Firstly, even if a patient has all the prerequisites to develop spontaneous bacterial peritonitis, a secondary cause of peritonitis (eg. bowel perforation or intra-abdominal abscess) must always be considered as a differential diagnosis and a repeat ascitic tap is mandatory after 48 h of antibiotic therapy to confirm a decrease in the white cell count. Secondly, it shows the rare co-existence of alcoholic liver disease and vasculitis. PMID:10321767

  13. Cardiac manifestations in Behcet's disease

    PubMed Central

    Demirelli, Selami; Degirmenci, Husnu; Inci, Sinan; Arisoy, Arif

    2015-01-01

    Summary Behcet's disease (BD) is a chronic inflammatory disorder, with vasculitis underlying the pathophysiology of its multisystemic effects. Venous pathology and thrombotic complications are hallmarks of BD. However, it has been increasingly recognised that cardiac involvement and arterial complications are also important aspects of the course of the disease. Cardiac lesions include pericarditis, endocarditis, intracardiac thrombosis, myocardial infarction, endomyocardial fibrosis, and myocardial aneurysm. Treatment of cardiovascular involvement in BD is largely empirical, and is aimed towards suppressing the vasculitis. The most challenging aspect seems to be the treatment of arterial aneurysms and thromboses due to the associated risk of bleeding. When the prognosis of cardiac involvement in BD is not good, recovery can be achieved through oral anticoagulation, immunosuppressive therapy, and colchicine use. In this review, we summarise the cardiovascular involvement, different manifestations, and treatment of BD. PMID:25984424

  14. Churg-Strauss Syndrome Presented With Hearing Impairment and Facial Palsy

    PubMed Central

    Byun, Jeong-Hyun; Lee, Jong-Hoo

    2014-01-01

    Churg-Strauss syndrome (CSS) is a rare systemic necrotizing vasculitis. Cranial nerve involvement is very rare in CSS. A 59-year-old woman had complained of both hearing impairments for eight months and left facial palsy for three months. Left facial and cochlear neuropathies were detected in electrodiagnostic studies. Paranasal sinus computed tomography (CT) showed chronic pansinusitis. Chest CT revealed eosinophilic infiltration in the right upper lobe. Tissue biopsy of the right inferior turbinate displayed necrotizing vasculitis with eosinophilic infiltration. She was diagnosed as CSS, based on the presence of eosinophilia, pulmonary infiltration, paranasal sinusitis, and biopsy containing blood vessels with extravascular eosinophils. She was treated with intravenous and oral steroids and azathioprine, showing relatively good prognosis on facial palsy and hearing impairment. We report a very rare case of CSS presented with hearing impairment and facial palsy. PMID:25566487

  15. Erythema elevatum diutinum and hypothyroidism: coincidence or causal relationship?*

    PubMed Central

    Cirvidiu, Denise Camargo; Elias, Beatriz Lopes Ferraz; Jorge, Juliana Chaib Ferraira; Lira, Mrcia Lanzoni de Alvarenga; Mandelbaum, Samuel Henrique

    2015-01-01

    Erythema elevatum diutinum is a rare chronic leukocytoclastic vasculitis of unknown etiology. It is believed to be due to deposition of immune complexes in the vessels. Clinically it is manifested as erythematous violaceous papules and nodules, isolated or confluent with hardened consistency, symmetrical, usually located on the extensor surface of the extremities, particularly over the joints. Diagnosis is based on clinical and histological findings. We report the case of a woman, 71 years old, with erythematous violaceous nodules on the hands, elbows, back and legs, beginning two years ago, with pain and itching. Histopathological analysis revealed leukocytoclastic vasculitis, confirming the clinical suspicion. Laboratory tests revealed hypothyroidism. We report the case because of its rarity, with subsequent review of the literature. PMID:26375227

  16. Progressive Hemifacial Atrophy With Contralateral Uveitis: A Case Report

    PubMed Central

    Ayyildiz, Onder; Ayyildiz, Simel; Durukan, Ali Hakan; Sobaci, Gungor

    2015-01-01

    Introduction: Progressive hemifacial atrophy, known as Parry-Romberg syndrome (PRS), was first described by Parry in 1825. There is a progressive atrophy of facial tissues including skin, bones and muscles. Ophthalmic disorders are common and include keratitis, uveitis, cataract, ipsilateral enophthalmos, optic neuritis, retinal vasculitis and scleral melting. Case Presentation: We describe a patient with progressive hemifacial atrophy at right facial side who developed granulomatous uveitis and periferic retinal vasculitis in his left eye. We started topical and systemic steroid therapy. Uveitic reaction had regressed almost entirely after a 3-month steroid treatment. Conclusions: The individuals should have multidisciplinary approach for the variety of disorders to maintain the appropriate treatment for a better appearance of the patients. PMID:26473067

  17. Do neutrophil extracellular traps contribute to the heightened risk of thrombosis in inflammatory diseases?

    PubMed

    Rao, Ashish N; Kazzaz, Nayef M; Knight, Jason S

    2015-12-26

    Thrombotic events, both arterial and venous, are a major health concern worldwide. Further, autoimmune diseases, such as systemic lupus erythematosus, anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, and antiphospholipid syndrome, predispose to thrombosis, and thereby push the risk for these morbid events even higher. In recent years, neutrophils have been identified as important players in both arterial and venous thrombosis. Specifically, chromatin-based structures called neutrophil extracellular traps (NETs) play a key role in activating the coagulation cascade, recruiting platelets, and serving as scaffolding upon which the thrombus can be assembled. At the same time, neutrophils and NETs are emerging as important mediators of pathogenic inflammation in the aforementioned autoimmune diseases. Here, we first review the general role of NETs in thrombosis. We then posit that exaggerated NET release contributes to the prothrombotic diatheses of systemic lupus erythematosus, ANCA-associated vasculitis, and antiphospholipid syndrome. PMID:26730289

  18. Necrolytic acral erythema: an expanding spectrum.

    PubMed

    Halpern, Analisa V; Peikin, Steven R; Ferzli, Pascal; Heymann, Warren R

    2009-12-01

    Hepatitis C virus (HCV) infection is the most common chronic blood-borne viral infection in the United States. Well-described cutaneous manifestations of HCV infection include polyarteritis nodosa, porphyria cutanea tarda, type II cryoglobulinemia-associated vasculitis, pruritus, erythema nodosum, urticaria and urticarial vasculitis, lichen planus, and erythema multiforme. First described in 1996, necrolytic acral erythema (NAE) is now recognized as a cutaneous acral eruption uniquely associated with HCV infection. Most patients present with chronic, acral, erythematous, and psoriasiform lesions. Acute presentations of NAE are rare and patients may present with atypical clinical features; in these cases, suspicion for HCV infection may be delayed for weeks to months until more classic chronic lesions develop. In many cases, NAE presents before the patient has been diagnosed with HCV infection, which allows dermatologists the unique opportunity to suspect and diagnose HCV infection based on skin findings alone. PMID:20166571

  19. Terminal Ileitis as a Feature of Henoch-Schnlein Purpura Masquerading as Crohn Disease in Adults.

    PubMed

    Sampat, Hemal N; McAllister, Brian P; Gaines, Darryl D; Ostrov, Barbara

    2016-03-01

    Henoch-Schnlein purpura (HSP), more recently termed immunoglobulin A (IgA) vasculitis, is a systemic small-vessel vasculitis characterized by perivascular IgA deposition. This disease manifests clinically as palpable purpura, arthralgia, gastrointestinal symptoms, and renal dysfunction. Although ileitis can be seen in HSP, terminal ileitis is virtually pathognomonic for Crohn disease. We present a comprehensive review of the literature on this association, including 2 cases of our own, to demonstrate the importance of considering HSP in the differential diagnosis of ileitis suggestive of Crohn disease. We review the growing body of literature suggesting a pathophysiologic link between the conditions, possibly through an IgA-mediated mechanism. PMID:26906301

  20. Do neutrophil extracellular traps contribute to the heightened risk of thrombosis in inflammatory diseases?

    PubMed Central

    Rao, Ashish N; Kazzaz, Nayef M; Knight, Jason S

    2015-01-01

    Thrombotic events, both arterial and venous, are a major health concern worldwide. Further, autoimmune diseases, such as systemic lupus erythematosus, anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, and antiphospholipid syndrome, predispose to thrombosis, and thereby push the risk for these morbid events even higher. In recent years, neutrophils have been identified as important players in both arterial and venous thrombosis. Specifically, chromatin-based structures called neutrophil extracellular traps (NETs) play a key role in activating the coagulation cascade, recruiting platelets, and serving as scaffolding upon which the thrombus can be assembled. At the same time, neutrophils and NETs are emerging as important mediators of pathogenic inflammation in the aforementioned autoimmune diseases. Here, we first review the general role of NETs in thrombosis. We then posit that exaggerated NET release contributes to the prothrombotic diatheses of systemic lupus erythematosus, ANCA-associated vasculitis, and antiphospholipid syndrome. PMID:26730289

  1. Bilateral Facial Palsy in Rapidly Progressive Course of Wegener's Granulomatosis: A Case Report

    PubMed Central

    Roszkowska, Anna; Morawska-Kochman, Monika; Temporale, Hanna; Sikorska-?uk, Ma?gorzata; Kr?cicki, Tomasz

    2013-01-01

    Introduction. Wegener's granulomatosis belongs to a group of systemic vasculitis diseases, which is characterized by necrotizing vasculitis and presence of granulomas. In a lot of cases, the first symptoms of the disease are observed in the head and neck region, but the bilateral facial nerve palsy occurs very rarely. Objective. The objective of our report was to describe the unusual course of Wegener's granulomatosis with the bilateral facial nerve paralysis, which subsided after application of steroids and immunosuppressive therapy in combination with surgical treatment. Results and Conclusions. Hearing loss may precede other symptoms in Wegener's granulomatosis. Ear pain and otorrhea may suggest the diagnosis of bacterial purulent otitis media and delay the proper diagnosis. In the presented case, considering the clinical course, it was necessary to apply both pharmacological and surgical treatments. PMID:24187641

  2. Extralimbic autoimmune encephalitis associated with glutamic acid decarboxylase antibodies: an underdiagnosed entity?

    PubMed

    Najjar, Souhel; Pearlman, Daniel; Najjar, Amanda; Ghiasian, Vahid; Zagzag, David; Devinsky, Orrin

    2011-07-01

    Nonparaneoplastic glutamic acid decarboxylase antibody (GADAb)-related autoimmune encephalitis is a syndrome characterized by refractory seizures, progressive cognitive deficits, and psychiatric manifestations. The limbic subtype is well described, has characteristic affective and memory disturbances, and typical mesial temporal MRI abnormalities. We found only one single case report of the extralimbic subtype. We report clinical, radiological, and pathological findings of two additional cases with contrast-enhancing lesions. One of our cases presented as vasculitis, and the other imitated a tumor. Pathological evidence of both vasculitis and encephalitis has never been previously reported in any inflammatory condition affecting the brain. Our cases confirm prior reports that immune therapy can better control seizures associated with GADAb autoimmune encephalitis, and support the rationale for assaying for GADAb titers in patients with etiologically unclear extralimbic lesions and refractory epilepsy, independent of seizure types. PMID:21620774

  3. Challenges with Takayasu's arteritis: a case study.

    PubMed

    Strider, D; Robinson, T; Guarini, J; Ivey, J

    1996-03-01

    Takayasu's arteritis (TA) is a systemic vasculitis that involves an autoimmune-mediated transmural degeneration of the aorta and its major branches, leading to severe arterial stenoses or occlusions with subsequent cerebral, cardiopulmonary, mesenteric, renal, or limb ischemia. Patient history and physical examination, arteriography, magnetic resonance imaging, and specific laboratory tests facilitate the diagnosis of TA. Major treatments for the condition include the use of corticosteroids, cytotoxic agents, antiplatelet drugs, percutaneous transluminal angioplasty (PTA), and surgical revascularization. Major nursing interventions for TA include teaching the patient and family about the progression and treatments of TA and performing periodic patient assessments for manifestations of systemic vasculitis, alterations in tissue perfusion, and alterations in coping. PMID:8703796

  4. Detection of circulating immune complexes by Raji cell assay: comparison of flow cytometric and radiometric methods

    SciTech Connect

    Kingsmore, S.F.; Crockard, A.D.; Fay, A.C.; McNeill, T.A.; Roberts, S.D.; Thompson, J.M.

    1988-01-01

    Several flow cytometric methods for the measurement of circulating immune complexes (CIC) have recently become available. We report a Raji cell flow cytometric assay (FCMA) that uses aggregated human globulin (AHG) as primary calibrator. Technical advantages of the Raji cell flow cytometric assay are discussed, and its clinical usefulness is evaluated in a method comparison study with the widely used Raji cell immunoradiometric assay. FCMA is more precise and has greater analytic sensitivity for AHG. Diagnostic sensitivity by the flow cytometric method is superior in systemic lupus erythematosus (SLE), rheumatoid arthritis, and vasculitis patients: however, diagnostic specificity is similar for both assays, but the reference interval of FCMA is narrower. Significant correlations were found between CIC levels obtained with both methods in SLE, rheumatoid arthritis, and vasculitis patients and in longitudinal studies of two patients with cerebral SLE. The Raji cell FCMA is recommended for measurement of CIC levels to clinical laboratories with access to a flow cytometer.

  5. Structural brain lesions in inflammatory bowel disease

    PubMed Central

    Dolapcioglu, Can; Dolapcioglu, Hatice

    2015-01-01

    Central nervous system (CNS) complications or manifestations of inflammatory bowel disease deserve particular attention because symptomatic conditions can require early diagnosis and treatment, whereas unexplained manifestations might be linked with pathogenic mechanisms. This review focuses on both symptomatic and asymptomatic brain lesions detectable on imaging studies, as well as their frequency and potential mechanisms. A direct causal relationship between inflammatory bowel disease (IBD) and asymptomatic structural brain changes has not been demonstrated, but several possible explanations, including vasculitis, thromboembolism and malnutrition, have been proposed. IBD is associated with a tendency for thromboembolisms; therefore, cerebrovascular thromboembolism represents the most frequent and grave CNS complication. Vasculitis, demyelinating conditions and CNS infections are among the other CNS manifestations of the disease. Biological agents also represent a risk factor, particularly for demyelination. Identification of the nature and potential mechanisms of brain lesions detectable on imaging studies would shed further light on the disease process and could improve patient care through early diagnosis and treatment. PMID:26600970

  6. Pulsed-laser therapy (GA-As) in combined treatment of post-traumatic swellings and some dermatological disorders

    NASA Astrophysics Data System (ADS)

    Antipa, Ciprian; Dona, Dumitru; Podoleanu, Adrian Gh.

    1994-02-01

    The effect of a pulsed gallium arsenide infrared laser radiation was studied on 64 patients with post traumatic swellings, allergic vasculitis and varicose crural ulcers, therapy resistant. The soft laser therapy was conducted in combination with classical therapy and was compared with a non irradiated control group of 52 patients treated only by classical therapy. Laser irradiation was directed to the skin damage by laser scanning. Segmental and dermatomic areas of the skin lesions were irradiated by laserpuncture. Therapeutic protocol included an average of nine sessions. The statistical analysis shows a significant difference of the efficiency between Ga-As pulsed laser treated group and the control group, especially in the case of post-traumatic swellings and less in the case of allergic vasculitis. The effects reported by this study are relevant for clinical application of infrared pulsed low lasers in dermatology.

  7. Adult Dermatomyositis with Bleeding Ulcer in the Pharynx

    PubMed Central

    Kusano, Junko; Takahashi, Yuka

    2014-01-01

    Dermatomyositis (DM) is one of the idiopathic inflammatory myopathies caused by complement-mediated vasculopathy or vasculitis in the muscle. Although the gastrointestinal (GI) mucosa has been reported to be involved as a result of vasculitis or vasculopathy, ulceration in the pharynx is a rare manifestation of DM. A 54-year-old woman complaining of muscle weakness in the extremities, low-grade fever, and dysphagia was diagnosed as having DM. Despite medical treatment with corticosteroids and immunosuppressive agents, her DM progressed rapidly, leading to exacerbation of the dysphagia. About 3 weeks after undergoing tracheostomy as a preventive measure against aspiration, the patient developed intractable respiratory tract hemorrhage. Repeated laryngoendoscopy revealed a bleeding ulceration in the pharynx that required hemostasis with electric cautery under general anesthesia. No bleeding recurred thereafter. Histopathologically, the pharynx exhibited nonspecific inflammatory cell infiltration in the muscle tissue. This rare manifestation may be considered in cases of DM with unexplainable airway bleeding. PMID:25328739

  8. A Clinical Picture of the Visual Outcome in Adamantiades-Behçet's Disease

    PubMed Central

    Figus, Michele; Posarelli, Chiara; Albert, Timothy G.; Talarico, Rosaria; Nardi, Marco

    2015-01-01

    Adamantiades-Behçet's disease is a multisystemic vasculitis with multiorgan involvement. Ocular disorders occur often in this syndrome typically in the form of a relapsing-remitting panuveitis and vasculitis and can lead to blindness as one of its most disabling complications if left untreated. There are known risk factors related with the worst visual prognosis, which require early and intensive treatment in order to obtain a rapid suppression of inflammation and to prevent future relapses. The management strategy to avoid vision loss and blindness currently involves the use of local and systemic drugs including steroids and immunosuppressive and biologic agents. This review aims to demonstrate how the introduction and the use of biologic agents improves the visual outcome of patients with Adamantiades-Behçet's disease. PMID:26558256

  9. Resolution of neurological deficits secondary to spontaneous intracranial haemorrhage and posterior reversible encephalopathy syndrome (PRES) in a patient with hepatitis C-associated cryoglobulinaemia: a role for plasmapheresis

    PubMed Central

    Ahmad, Delshad; Ilias Basha, Haseeb; Towfiq, Basim; Bachuwa, Ghassan

    2014-01-01

    Essential mixed cryoglobulinaemia or type II cryoglobulinaemia is an important extrahepatic manifestation of chronic hepatitis C. Cryoglobulinaemia results in the deposition of immune complexes in small or medium-sized blood vessels leading to palpable purpura, arthralgia, renal disease and peripheral neuropathy. Posterior reversible encephalopathy syndrome (PRES) is a distinct phenomenon characterised by vasogenic oedema in the posterior circulation of brain. Cryoglobulinaemic vasculitis leading to spontaneous intracranial haemorrhage and PRES syndrome is rarely reported in the medical literature. In this report, we present an unusual case of spontaneous intracranial haemorrhage and PRES secondary to hepatitis C-associated cryoglobulinaemia presenting as right dense hemiplegia. Prompt institution of plasmapheresis resulted in successful resolution of symptoms in our patient, followed by full neurological recovery. To the best of our knowledge, this case describes the first successful use of plasmapheresis in alleviating neurological complications resulting from cryoglobulinaemic vasculitis and PRES secondary to chronic hepatitis C. PMID:24445850

  10. Henoch Schonlein purpura associated with bee sting: case report.

    PubMed

    Gálvez-Olortegui, José; Álvarez-Vargas, Mayita; Durand-Vergara, Juan; Díaz-Lozano, Marisol; Gálvez-Olortegui, Tomas; Armas-Ramírez, Indira; Hilario-Vargas, Julio

    2015-01-01

    Henoch Schonlein purpura (HSP) is a common childhood vasculitis, characterized by a non-thrombocytopenic palpable purpura and systemic features. It can be triggered by conditions like infections and insect bites. We present the case of a six-year-old girl with palpable maculopapular lesions on the lower limbs, itching, mild pain, swelling of feet, limitation of limb mobility, and a history of bee sting. Thigh skin biopsy was performed, with a report of leucocytoclastic vasculitis, and was diagnosed as HSP. She was prescribed bed rest, and was given oral hydration. The patient outcome was favorable and was discharged after five days. This is the fifth report of a HSP case associated with a bee sting with an uncomplicated course, which is in contrast to previous case reports. PMID:26610057

  11. [Classification of dermatologic manifestations in lupus erythematosus].

    PubMed

    Francs, Camille; Barete, Stphane; Ayoub, Nakhle; Piette, Jean-Charles

    2003-02-01

    Various dermatologic manifestations are observed in the different lupus subsets. Lupus lesions are characterized by a dermo-epidermal dermatitis. Other lesions, vascular or non vascular, are essentially present in association with systemic lupus erythematosus. Acute, subacute and chronic lupus erythematosus are distinguishable according to their clinical aspects, pathological features and evolution. Acute lesions are either localized to the midface or widespread. Subacute lesions may be annular or psoriasiform. Chronic lupus erythematosus includes localized or widespread discoid lupus, lupus tumidus, chilblain lupus and panniculitis. Therapy of cutaneous lupus is mainly based on antimalarials and avoidance of sun irradiation. In refractory cutaneous lupus, no universal guidelines are currently available. Except for acrosyndromes and urticaria-like lesions, vascular lesions may be due to vasculitis or thrombosis. An accurate diagnosis is absolutely necessary since therapy is different in thrombosis and vasculitis. Non vascular and non lupus lesions are numerous, some of them require special treatment such as dapsone for bullous lupus. PMID:12746657

  12. Neurorickettsioses: A Rare Presentation with Stroke in a Young Adult

    PubMed Central

    K, Pramod

    2014-01-01

    Acute stroke-like presentations due to rickettsial infections have been sparsely reported in literature. We report a young patient who presented with high grade fever and acute encephalopathy with right hemiplegia. CT head showed left cerebral, bilateral thalamic and midbrain infarcts. The stroke in young work-up for conventional and non-conventional risk factors including few infections known to cause cerebral vasculitis was negative. He did not respond to empirical antimalarials and antibiotics. With a high index of suspicion of his febrile cerebrovasculitis like presentation, serological test for rickettsia was done and found to be positive. He responded to doxycycline. This case expands the spectrum of presentation of neurorickettsioses and highlights the importance of considering rickettsial vasculitis as one of the potentially treatable causes of infections causing stroke. PMID:25478386

  13. What's in a name?

    PubMed

    Chu, Chia-Kai; Morgan, Michael L; Almarzouqi, Sumayya; Lee, Andrew G; Newman, Steven A

    2016-01-01

    A 51-year-old man with rheumatoid arthritis and diabetes mellitus presented with new onset left-sided hemiparesis, left-sided neglect, and left-sided incongruous, denser inferiorly, homonymous hemianopsia. Magnetic resonance image of the brain showed prominent swelling of the right frontal, parietal, and occipital lobes greater than on the left with significant change in the fluid-attenuated inversion recovery signal in the gray matter. Perinuclear antineutrophil cytoplasmic antibodies titers were elevated, and skin biopsy demonstrating leukocytoclastic vasculitis. He showed marked clinical and radiographic improvement in association with recovery of vascular abnormalities after the initiation of prednisone, pulse therapy with methylprednisolone, and methotrexate. Clinicians should be aware of the possibility of neuro-ophthalmic manifestations of rheumatoid arthritis, including rheumatoid arthritis-related vasculitis causing homonymous hemianopsia. PMID:26212152

  14. ANA Negative Systemic Lupus Erythematosus Leading to CTEPH, TTP-Like Thrombocytopenia, and Skin Ulcers

    PubMed Central

    Sofi, Fayaz; Altaf, Sheikh Shoaib; Raina, Adnan; Raina, Ab. Hameed

    2016-01-01

    SLE affects almost every organ system, with differing degrees of severity. During its clinical course periods of flares may alternate with periods of remission culminating in disease and therapy related damage. We describe a case of ANA negative SLE with severe thrombocytopenia, cutaneous vasculitis, antiphospholipid antibody syndrome, and pulmonary artery hypertension. As there is no definitive cure for SLE the treatment lies in caring for the individual organ systems involved and simultaneously taking care of the patient as a whole. PMID:27006850

  15. Granulomatosis with Polyangiitis Presenting as a Parotid Gland Abscess

    PubMed Central

    Dias, Blenda; Soares, Daniela; Sampaio, Patrick; Santiago, Mittermayer

    2015-01-01

    Granulomatosis with polyangiitis (GPA) is a small-vessel vasculitis consisting of necrotizing granulomatous lesions in airways and focal necrotizing glomerulonephritis. However, it may affect other sites such as the skin, central nervous system, eyes, heart, gastrointestinal tract, and liver. We describe a rare case of GPA in which the initial manifestation was the involvement of the parotid gland mimicking a pyogenic abscess. PMID:25815014

  16. Skin diseases as extrahepatic manifestations of HCV. Review of some clinical cases.

    PubMed

    Paoletti, V; Parlapiano, C; Labbadia, G; Cavina, G; Marziali, M; Donnarumma, A; Paoletti, F

    2002-09-01

    Among extra-hepatic manifestations of hepatitis C virus (HCV) infection particular interest is focused on some dermatological diseases such as: leukocytoclastic vasculitis, oral lichen planus, pruritus-urticaria, psoriasis. Aim of this paper is to analyze these typical dermatoses in a population of patients with HCV infection and describe the characteristic clinical pictures. These clinical pictures confirm the importance of liver examination in presence of skin diseases not related to other pathogenetic mechanisms. PMID:16491052

  17. Facial ulcerations due to Acinetobacter baumannii: Vessel thrombosis with bacterial mycelia

    PubMed Central

    Li, Dong Ming; Sun, Ting Ting

    2014-01-01

    A 14-year-old girl presented with a 2-week history of progressive facial ulcerations that did not respond to cephalexin and topical dexamethasone. Biopsy on the ulcer showed rod-shaped bacteria and actinomycetes-like mycelia in the vessel walls and within thrombi. Tissue culture yielded Acinetobacter baumannii, which was resistant to cephalexin. A favourite outcome was achieved with minocycline treatment. This is the first case report of A. baumannii-related vasculitis.

  18. Lyme disease in central Europe.

    PubMed

    Hercogova, J; Brzonova, I

    2001-04-01

    Lyme borreliosis is a fascinating disease, the aetiopathology of which is not yet completely known. Different subspecies of Borrelia burgdorferi sensu lato are responsible for the variable clinical course of the disease. Some new cutaneous (alopecia) and ocular (photophobia and retinal vasculitis) manisfestations have been described and the largest prospective study on erythema migrans during pregnancy was published during the last year. Optimal therapy of Lyme borreliosis is still lacking, but doxycycline, amoxicillin, penicillin, and ceftriaxone are recommended most frequently. PMID:11979122

  19. [Thromboangiitis obliterans (Buerger's disease): update 2015].

    PubMed

    Klein-Weigel, Peter; Volz, Theresa Sophie; Richter, Jutta

    2015-10-01

    Thromboangiitis obliterans (Buerger's disease) is a vasculitis with undulating clinical course multisegmentarily affecting small and medium-sized arteries and veins. The disease is closely linked to tobacco-use. Increasing knowledge of autoimmunologic mechanisms in the complex pathophyiology of the disease let to the formulation of an autoimmunity-hypothesis now serving as a new paradigma. New treatment options comprise progenitor-cell-therapy, immunoadsorption, use of sendothelin-receptor-blocking agent Bosentan, and prescriptions of antiphosphodiesterase-V-inhibitors. PMID:26445249

  20. Rapid progressive bilateral hearing loss due to granulomatous otitis media in Lyme disease.

    PubMed

    Maniu, Alma; Damian, Laura

    2013-01-01

    We report on a patient with Lyme disease who presented with chronic bilateral otitis media and cranial neuropathy with rapid progressive hearing loss. After ceftriaxone and high-dose intravenous immunoglobulins, the disease was controlled only with methylprednisolone and cyclophosphamide. The relationship between Lyme disease and granulomatous vasculitis is discussed. Lyme disease should be kept in mind in the differential diagnosis of various otolaryngological and neurological presentations. PMID:23313123

  1. Single Bilateral Dexamethasone Implant in Addition to Panretinal Photocoagulation and Oral Azathioprine Treatment in IRVAN Syndrome.

    PubMed

    Saatci, Ali Osman; Ayhan, Ziya; Take?, mer; Yaman, Aylin; Bajin, F Meltem Sylev

    2015-01-01

    The idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) syndrome is a disease characterized by multiple retinal macroaneurysms, neuroretinitis and peripheral capillary nonperfusion. Visual loss may result from either ischemia-related complications or macular involvement. Treatment is not always rewarding. We report a case with stage 2 IRVAN syndrome who was successfully treated with a single bilateral intravitreal dexamethasone implant in addition to panretinal photocoagulation and systemic azathioprine treatment. PMID:25802506

  2. Single Bilateral Dexamethasone Implant in Addition to Panretinal Photocoagulation and Oral Azathioprine Treatment in IRVAN Syndrome

    PubMed Central

    Saatci, Ali Osman; Ayhan, Ziya; Take?, mer; Yaman, Aylin; Bajin, F. Meltem Sylev

    2015-01-01

    The idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) syndrome is a disease characterized by multiple retinal macroaneurysms, neuroretinitis and peripheral capillary nonperfusion. Visual loss may result from either ischemia-related complications or macular involvement. Treatment is not always rewarding. We report a case with stage 2 IRVAN syndrome who was successfully treated with a single bilateral intravitreal dexamethasone implant in addition to panretinal photocoagulation and systemic azathioprine treatment. PMID:25802506

  3. Human recombinant interferon alfa-2a for the treatment of Behet's disease with sight threatening posterior or panuveitis

    PubMed Central

    Ktter, I; Zierhut, M; Eckstein, A K; Vonthein, R; Ness, T; Gnaydin, I; Grimbacher, B; Blaschke, S; Meyer-Riemann, W; Peter, H H; Stbiger, N

    2003-01-01

    Background: Behet's disease is a multisystem vasculitis of unknown origin. Standard treatment mainly comprises systemic immunosuppressive agents. Ocular involvement, mostly posterior uveitis with retinal vasculitis, leads to blindness in 2050% of the involved eyes within 5 years. The efficacy of interferon alfa-2a was studied in patients with sight threatening posterior uveitis or retinal vasculitis. Methods: 50 patients were included in this open, non-randomised, uncontrolled prospective study. Recombinant human interferon alfa-2a (rhIFN?-2a) was applied at a dose of 6 million units subcutaneously daily. Dose reduction was performed according to a decision tree until discontinuation. Disease activity was evaluated every 2 weeks by the Behet's disease activity scoring system and the uveitis scoring system. Results: Response rate of the ocular manifestations was 92% (three non-responder, one incomplete response). Mean visual acuity rose significantly from 0.56 to 0.84 at week 24 (p<0.0001). Posterior uveitis score of the affected eyes fell by 46% every week (p<0.001). Remission of retinal inflammation was achieved by week 24. Mean Behet's disease activity score fell from 5.8 to 3.3 at week 24 and further to 2.8 at week 52. After a mean observation period of 36.4 months (range 1272), 20 patients (40%) are off treatment and disease free for 758 months (mean 29.5). In the other patients maintenance IFN dosage is three million units three times weekly. Conclusions: rhIFN?-2a is effective in ocular Behet's disease, leading to significant improvement of vision and complete remission of ocular vasculitis in the majority of the patients. PMID:12642304

  4. Polyarteritis nodosa and acute abdomen: A role for laparoscopy?

    PubMed Central

    Asti, Emanuele; Pogliani, Luca; Tritella, Stefania; Bonavina, Luigi

    2015-01-01

    Mesenteric vasculitis secondary to polyarteritis nodosa represents an atypical but potentially life-threatening cause of bowel ischemia and acute abdomen. The patient presented with severe abdominal pain of recent onset, pitting edema of the legs, renal failure and bowel wall thickening suggestive of mesenteric ischemia on CT scan. Early laparoscopy allowed to rule out proximal bowel necrosis and resection was avoided. The patient was successfully managed with corticosteroid therapy and repeated hemodialysis sessions. PMID:26656589

  5. Small bowel involvement documented by capsule endoscopy in Churg-Strauss syndrome

    PubMed Central

    Beye, Birane; Lesur, Gilles; Claude, Pierre; Martzolf, Lionel; Kieffer, Pierre; Sondag, Daniel

    2015-01-01

    Churg-Strauss syndrome is a small and medium vessel vasculitis and is also known as allergic granulomatous angiitis. Gastrointestinal involvement is common in patients with Churg-Strauss syndrome (20-50%). The most common symptoms are abdominal pain, diarrhoea and occasionally gastrointestinal bleeding and perforation. We present a case of Churg-Strauss syndrome with small bowel lesions documented by video capsule endoscopy. PMID:26664542

  6. Microscopic polyangiitis causing diffuse alveolar hemorrhage and rapidly progressive glomerulonephritis

    PubMed Central

    Lababidi, Mohamad Hani; Okolo, Chukwuka; Elhassan, Ahmed; Iroegbu, Nkemakolam

    2015-01-01

    Diffuse alveolar hemorrhage complicating small vessel vasculitis is a life-threatening emergency and should be considered in the differential diagnosis of patients who develop rapidly progressive dyspnea with alveolar opacities on chest imaging. In these patients, the coexistence of pulmonary and renal involvement suggests a multisystem disease. We present a case of a man who presented to our hospital with diffuse alveolar hemorrhage, severe anemia, and rapidly progressive glomerulonephritis. PMID:26424944

  7. Role of neutrophils in systemic autoimmune diseases

    PubMed Central

    2013-01-01

    Neutrophils have emerged as important regulators of innate and adaptive immune responses. Recent evidence indicates that neutrophils display marked abnormalities in phenotype and function in various systemic autoimmune diseases, and may play a central role in initiation and perpetuation of aberrant immune responses and organ damage in these conditions. This review discusses the putative roles that neutrophils and aberrant neutrophil cell death play in the pathogenesis of various systemic autoimmune diseases, including systemic lupus erythematosus, small vessel vasculitis and rheumatoid arthritis. PMID:24286137

  8. [Acute myocardial infarction as Eosinophilic granulomatosis with polyangiitis (formerly Churg Strauss syndrome) initial presentation].

    PubMed

    Sulaiman, Wahinuddin; Seung, Ong Ping; Noor, Sabariah Mohd

    2014-01-01

    Eosinophilic granulomatosis with polyangiitis is a rare primary vasculitic disease characterized by hypereosinophilia, late onset asthma and extravascular eosinophil granulomas. We report a case presented initially with acute myocardial infarction which later only proceed with asthma, skin manifestations and peripheral neuropathy. Laboratory parameters showed hypereosinohpilia with negative perinuclear pattern of antineutrophil cytoplasmic autoantibodies (p-ANCA). Skin biopsy showed leucocytoclastic vasculitis with eosinophilic infiltration while coronary angiography was normal. The patient's symptoms improved with IV methylprednisolone, pulse cyclophosphamide and azathioprine. PMID:25627304

  9. [Febrile torticollis: an atypical presentation of Kawasaki disease].

    PubMed

    Runel-Belliard, C; Lasserre, S; Quinet, B; Grimprel, E

    2009-02-01

    Kawasaki disease is a form of idiopathic systemic vasculitis. Diagnosis is based upon specific clinical parameters. Cardiac manifestations explain the mortality rate. They can be reduced by early treatment using intravenous immunoglobulin. Atypical Kawasaki disease is difficult to diagnose and can delay diagnosis. We report a case of Kawasaki disease with arthritis in a 4-year-old girl whose initial presentation was a febrile torticollis. A literature review details the atypical early signs of Kawasaki disease revealed by torticollis. PMID:19112010

  10. Systemic amyloidosis of beta 2-microglobulin type: a complication of long-term haemodialysis.

    PubMed

    Theaker, J M; Raine, A E; Rainey, A J; Heryet, A; Clark, A; Oliver, D O

    1987-10-01

    A patient receiving long-term haemodialysis developed systemic amyloidosis, which was shown immunohistochemically to be of beta 2-microglobulin type, a previously unrecognised form of systemic amyloidosis. Histologically, the amyloid deposits were closely associated with foci of acute and granulomatous inflammation and vasculitis, although it was not clear if the amyloid deposits directly caused the inflammatory process, or if amyloid was deposited preferentially in areas of inflammation of uncertain aetiology. PMID:3119676

  11. Henoch-Schönlein Purpura Associated with Gangrenous Appendicitis: A Case Report

    PubMed Central

    SEMEENA, NK; ADLEKHA, Shashikant

    2014-01-01

    Henoch-Schönlein Purpura (HSP) is a leucocytoclastic vasculitis of unclear aetiology characterised by symmetrical, non-traumatic, nonthrombocytopenic purpura mostly involving the lower limbs and buttocks, as well as arthritis, gastrointestinal manifestations, and occasional nephritis. A 35 years old male presented with purpuric rash on the lower extremities, abdominal pain, fever, arthralgia, and melaena. A diagnosis of HSP with appendicitis was made, which is an exceedingly rare phenomenon. PMID:24876811

  12. Angiocentric CD3+ T-Cell Infiltrates in Human Immunodeficiency Virus Type 1-Associated Central Nervous System Disease in Children

    PubMed Central

    Katsetos, Christos D.; Fincke, John E.; Legido, Agustin; Lischner, Harold W.; de Chadarevian, Jean-Pierre; Kaye, Edward M.; Platsoucas, Chris D.; Oleszak, Emilia L.

    1999-01-01

    A significant proportion of brain tissue specimens from children with AIDS show evidence of vascular inflammation in the form of transmural and/or perivascular mononuclear-cell infiltrates at autopsy. Previous studies have shown that in contrast to inflammatory lesions observed in human immunodeficiency virus type 1 (HIV-1) encephalitis, in which monocytes/macrophages are the prevailing mononuclear cells, these infiltrates consist mostly of lymphocytes. Perivascular mononuclear-cell infiltrates were found in brain tissue specimens collected at autopsy from five of six children with AIDS and consisted of CD3+ T cells and equal or greater proportions of CD68+ monocytes/macrophages. Transmural (including endothelial) mononuclear-cell infiltrates were evident in one patient and comprised predominantly CD3+ T cells and small or, in certain vessels, approximately equal proportions of CD68+ monocytes/macrophages. There was a clear preponderance of CD3+ CD8+ T cells on the endothelial side of transmural infiltrates. In active lesions of transmural vasculitis, CD3+ T-cell infiltrates exhibited a distinctive zonal distribution. The majority of CD3+ cells were also CD8+ and CD45RO+. Scattered perivascular monocytes/macrophages in foci of florid vasculitis were immunoreactive for the p24 core protein. In contrast to the perivascular space, the intervening brain neuropil was dominated by monocytes/macrophages, microglia, and reactive astrocytes, containing only scant CD3+ CD8+ cells. Five of six patients showed evidence of calcific vasculopathy, but only two exhibited HIV-1 encephalitis. One patient had multiple subacute cerebral and brainstem infarcts associated with a widespread, fulminant mononuclear-cell vasculitis. A second patient had an old brain infarct associated with fibrointimal thickening of large leptomeningeal vessels. These infiltrating CD3+ T cells may be responsible for HIV-1-associated CNS vasculitis and vasculopathy and for endothelial-cell injury and the opening of the blood-brain barrier in children with AIDS. PMID:9874673

  13. Atherosclerosis associated with vasculopathic lesions in a golden retriever with hypercholesterolemia

    PubMed Central

    Boynosky, Nicole A.; Stokking, Laura

    2014-01-01

    A 2-year-old neutered male golden retriever dog presented for lameness secondary to ulcerations of multiple digital paw pads was diagnosed with vasculitis and hypercholesterolemia. Despite treatment, ischemic necrosis progressed to include all distal extremities and the dog eventually expired due to myocardial infarction secondary to severe atherosclerosis. The rapid demise and the dermatologic lesions may have been secondary to cholesterol embolism syndrome which has never before been reported in a dog. PMID:24790237

  14. Ulcerative colitis complicating seronegative HLA-A2-B27 rheumatoid arthritis with sacroiliitis.

    PubMed Central

    Klausen, T; Amris, K; Helin, P

    1992-01-01

    The case is reported of a 50 year old man with longstanding seronegative rheumatoid arthritis who developed ulcerative colitis. The patient also had sacroiliitis and his tissue was typed as HLA-A2-B27 several years before the bowel disease began. A possible overlap between primary inflammatory bowel disease, complications to the treatment of rheumatoid arthritis with drugs, and gastrointestinal rheumatoid vasculitis is discussed. Images PMID:1417108

  15. Amnesia or fugue state: a diagnostic dilemma.

    PubMed

    Keller, R; Shaywitz, B A

    1986-04-01

    We report the case of a 16-year-old boy whose presenting symptoms were total retrograde amnesia. After finding no evidence for organic causes, including toxic-metabolic derangements, epilepsy, encephalitis, vasculitis, trauma, or CNS neoplasm, it was determined that the child experienced a psychogenic fugue state with a spontaneous recovery in memory over several days. Although rare, fugue state should be considered in the differential diagnosis of amnesia. PMID:3700661

  16. Role of Advanced MRI Brain Sequences in Diagnosing Neurological Complications of Scrub Typhus

    PubMed Central

    Sood, Shikha; Sharma, Sanjeev; Khanna, Shweta

    2015-01-01

    Scrub typhus is a rare disease affecting many organs and causing vasculitis by affecting the endothelium of blood vessels. Review of literature shows that there are only a few case reports describing the neuroradiological manifestations of scrub typhus. This case report describes how newer and advanced MRI sequences are able to diagnose neurological complications of scrub typhus, such as hemorrhages, meningoencephalitis, infarctions, cranial nerve involvement, thrombosis, and hypoperfusion, that are not picked up on routine magnetic resonance imaging (MRI) sequences. PMID:25861545

  17. Acute myocardial infarction and cerebrovascular accident in a young girl after a viper bite.

    PubMed Central

    Aravanis, C; Ioannidis, P J; Ktenas, J

    1982-01-01

    A 17-year-old girl developed an acute myocardial infarction immediately after being bitten by a viper and four days later she had a cerebrovascular accident. The close clinical and laboratory follow-up of this case suggested that myocardial damage could be attributed to a direct cardiotoxic effect of the venom, while the brain injury that subsequently appeared was probably the result of a disseminated intravascular coagulopathy, possibly in conjunction with vasculitis. Images PMID:7073914

  18. Histopathologic Study of Pathergy Test in Behet's Disease

    PubMed Central

    Ozluk, Ekin; Balta, Ilknur; Akoguz, Ozlem; Kalkan, Goknur; Astarci, Muzeyyen; Akbay, Gulfer; Eksioglu, Meral

    2014-01-01

    Background: The pathergy test (PT) is important in the diagnosis of Behet's disease (BD). However, misinterpretation of the test might cause false-positive or false-negative results. Therefore, immunopathologic and histopathologic tests are recommended with PT. Aims and Objectives: The aim of this study is to determine histopathologic findings of positive pathergy reaction at BD. Materials and Methods: This study was performed on 23 patients with BD. All patients were in active period of the disease. After 48 h from the injection, biopsy was performed on positive pathergy lesions. Results: Of the specimen from positive PT lesions of patients with BD, nine revealed mixed type inflammatory cell infiltration (39.1%), two revealed lobular panniculitis without vasculitis (8.7%), two revealed neutrophil rich infiltration (8.7%), and five revealed lymphocyte rich infiltration (21.7%) at the subcutaneous tissue. Mixed type inflammatory cell infiltration (43.4%), endothelial swelling and thickening (17.3%), erythrocyte extravasation (26.0%), perivascular cell infiltration (13.0%), lymphocytic vascular reaction (8.6%), lymphocytic vasculitis (13.0%), and leukocytoclastic vasculitis (21.7%) were detected in dermis by histopathologic examinations. There was no statistically significant difference between histopathologic findings and sex, family history, and systemic involvement except uveitis. Conclusion: This is an exceptional study since it is the first study that determines subcutaneous tissue findings of positive pathergy reaction in Behet patients. In our study, uveitis was found to be statistically significant in the patients who had vasculitis in dermis. It can be a clue for prediction of disease severity and course. Further, studies that include wide number of patients will better illuminate the correlation between subcutaneous tissue findings and disease severity and clinical course. PMID:25484413

  19. [Takayasu arteritis].

    PubMed

    Mirault, T; Messas, E

    2016-04-01

    Takayasu arteritis is a chronic inflammatory vasculitis of unknown origin affecting large vessels, predominantly the aorta and its main branches. Vessel inflammation leads to wall thickening, fibrosis, and stenosis. The lesions are often asymptomatic leading to limb numbness, transient ischemic attack, cardiovascular event and renovascular hypertension. Treatment is based on corticosteroids, immunosuppressant and biologics if necessary. Endovascular treatment and open-surgery can be useful for end-organ ischemia relief. PMID:26827273

  20. Extracellular vesicles as mediators of vascular inflammation in kidney disease

    PubMed Central

    Helmke, Alexandra; von Vietinghoff, Sibylle

    2016-01-01

    Vascular inflammation is a common cause of renal impairment and a major cause of morbidity and mortality of patients with kidney disease. Current studies consistently show an increase of extracellular vesicles (EVs) in acute vasculitis and in patients with atherosclerosis. Recent research has elucidated mechanisms that mediate vascular wall leukocyte accumulation and differentiation. This review addresses the role of EVs in this process. Part one of this review addresses functional roles of EVs in renal vasculitis. Most published data address anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis and indicate that the number of EVs, mostly of platelet origin, is increased in active disease. EVs generated from neutrophils by activation by ANCA can contribute to vessel damage. While EVs are also elevated in other types of autoimmune vasculitis with renal involvement such as systemic lupus erythematodes, functional consequences beyond intravascular thrombosis remain to be established. In typical hemolytic uremic syndrome secondary to infection with shiga toxin producing Escherichia coli, EV numbers are elevated and contribute to toxin distribution into the vascular wall. Part two addresses mechanisms how EVs modulate vascular inflammation in atherosclerosis, a process that is aggravated in uremia. Elevated numbers of circulating endothelial EVs were associated with atherosclerotic complications in a number of studies in patients with and without kidney disease. Uremic endothelial EVs are defective in induction of vascular relaxation. Neutrophil adhesion and transmigration and intravascular thrombus formation are critically modulated by EVs, a process that is amenable to therapeutic interventions. EVs can enhance monocyte adhesion to the endothelium and modulate macrophage differentiation and cytokine production with major influence on the local inflammatory milieu in the plaque. They significantly influence lipid phagocytosis and antigen presentation by mononuclear phagocytes. Finally, platelet, erythrocyte and monocyte EVs cooperate in shaping adaptive T cell immunity. Future research is needed to define changes in uremic EVs and their differential effects on inflammatory leukocytes in the vessel wall. PMID:26981436

  1. The epidemiology of Kawasaki disease: a global update.

    PubMed

    Singh, Surjit; Vignesh, Pandiarajan; Burgner, David

    2015-11-01

    Kawasaki disease (KD) is a childhood vasculitis and the most frequent cause of paediatric acquired heart disease in North America, Europe and Japan. It is increasingly recognised in rapidly industrialising countries such as China and India where it may replace rheumatic heart disease as the most common cause of acquired heart disease in children. We review the current global epidemiology of KD and discuss some public health implications. PMID:26111818

  2. Pseudomonas bacteremia as an initial presentation of SLE

    PubMed Central

    Gill, Veenu; Patel, Jatinbhai; Koshy, Sanjana; Gomez, Tessa

    2014-01-01

    Infections have been commonly implicated in lupus relapses and in some cases as initiating the diagnostic work up of systemic lupus erythematosus (SLE). We describe here the case of a young patient who presented with Pseudomonas aeruginosa bacteremia and was found to have a new diagnosis of SLE. 53% of patients with active SLE and abdominal pain have intestinal vasculitis. These vasculitic changes can cause intestinal ischemia with consequent translocation of pathogens from the gastrointestinal tract to the bloodstream causing sepsis.

  3. Juvenile dermatomyositis in South African children is characterised by frequent dystropic calcification: a cross sectional study

    PubMed Central

    2014-01-01

    Background To describe Juvenile dermatomyositis (JDM) that has rarely been reported in Sub-Saharan Africa in children. Methods Retrospective record review of children with JDM attending a tertiary hospital in South Africa. Results Twenty-one children (16 female, five male) with JDM had a mean (SD) age at presentation of 9.8 (3.3) years. Mean follow-up period was 2.6 (2.2) years. The commonest presenting features were skin rash (71%), muscle weakness (71%), inflammatory arthritis (42%) and calcinosis (29%). The cumulative frequency of calcinosis was 71%. Skin vasculitis was present in 9(43%), and 7 (33%) had Staphylococcus aureus infections. Calcinosis was strongly associated with vasculitis; 11/15 (73.3%) with calcinosis had vasculitis versus 0/6 without vasculitis (p?=?0.003). Patients with calcinosis had significantly lower creatinine kinase (CK) levels compared to those without calcinosis [mean (SD) 272 U/L (401) vs. 2414 U/L (3201), respectively, p?=?0.016]. All children with calcinosis had Staphylococcus aureus infection, but there was no significant difference in their duration of symptoms to presentation. Joint contractures, occurring in eight patients (38%), were associated with a significantly lower age at presentation [mean (SD) 6.8(2.8) vs. 11.6(2.1) years (no contractures) p?=?0.0003], and significantly higher CRP and ESR levels. Three patients were lost to follow-up, two died. In the remaining 16 patients: 10 (47%) experienced remission, 2 relapsed and 4 persistent active disease. Conclusion African children with JDM have increased vasculitic disease and high levels of calcinosis with low muscle enzymes, particularly CK. Younger children are at higher risk of contractures and disability. Patients are at high risk of developing Staphylococcus aureus infection. Rapid and aggressive therapy is necessary. PMID:24397895

  4. Granuloma annularis revealing Wegener's granulomatosis.

    PubMed

    Del Porto, F; Proietta, M; Muscianese, M; Tamburi, F; Cifani, N; Ferri, L; Nistic, S; Bottoni, U; Bruno, G; Pranteda, G

    2014-01-01

    Skin manifestations are often associated with systemic autoimmune diseases (SAD). Some SAD, such as systemic lupus erythematosus, psoriatic arthritis and scleroderma display pathognomonic dermatological features, whereas other systemic diseases such as sarcoidosis, vasculitis and rheumatoid arthritis can present with non-specific skin manifestations that range from erythema nodosum to necrotic lesions. Here we report the case of a 25-year-old man with uveitis, polyarthrirtis, pulmonary involvement, nephrotic syndrome, cutaneous granuloma and pneumonia by E. coli. PMID:25033485

  5. Henipavirus Encephalitis: Recent Developments and Advances.

    PubMed

    Ong, Kien Chai; Wong, Kum Thong

    2015-09-01

    The genus Henipavirus within the family Paramyxoviridae includes the Hendra virus (HeV) and Nipah virus (NiV) which were discovered in the 1990s in Australia and Malaysia, respectively, after emerging to cause severe and often fatal outbreaks in humans and animals. While HeV is confined to Australia, more recent NiV outbreaks have been reported in Bangladesh, India and the Philippines. The clinical manifestations of both henipaviruses in humans appear similar, with a predominance of an acute encephalitic syndrome. Likewise, the pathological features are similar and characterized by disseminated, multi-organ vasculopathy comprising endothelial infection/ulceration, vasculitis, vasculitis-induced thrombosis/occlusion, parenchymal ischemia/microinfarction, and parenchymal cell infection in the central nervous system (CNS), lung, kidney and other major organs. This unique dual pathogenetic mechanism of vasculitis-induced microinfarction and neuronal infection causes severe tissue damage in the CNS. Both viruses can also cause relapsing encephalitis months and years after the acute infection. Many animal models studied to date have largely confirmed the pathology of henipavirus infection, and provided the means to test new therapeutic agents and vaccines. As the bat is the natural host of henipaviruses and has worldwide distribution, spillover events into human populations are expected to occur in the future. PMID:26276024

  6. Effects of nutrition on disease and life span. I. Immune responses, cardiovascular pathology, and life span in MRL mice.

    PubMed Central

    Mark, D. A.; Alonso, D. R.; Quimby, F.; Thaler, H. T.; Kim, Y. T.; Fernandes, G.; Good, R. A.; Weksler, M. E.

    1984-01-01

    Mice of the autoimmune, lymphoproliferative strain MRL/lpr and the congenic, nonlymphoproliferative strain MRL/n were fed one of six diets from weaning on-ward. These mice were sacrificed at 3 or 5 months of age. Low fat diets resulted in lower cholesterol and higher triglyceride levels than did cholesterol-containing high-fat diets. Caloric restriction of MRL/lpr mice was associated with an increased plaque-forming cell response to trinitrophenylated polyacrylamide beads, less lymphoproliferation, and less severe glomerulonephritis. Diet did not affect the incidence of autoimmune vasculitis in MRL/lpr mice sacrificed at 5 months. MRL/lpr mice fed a low-fat, calorically restricted diet from 5 months of age to death lived longer than mice which were fed ad libitum a cholesterol-containing, high-fat diet. At death, MRL/lpr mice fed the former diet had the autoimmune vasculitis which had been evident in mice killed at 5 months, whereas mice fed the latter diet, in addition to the vasculitis, had a high incidence of atherosclerotic lesions of intrarenal and aortic branch arteries. Images Figure 5 Figure 6 Figure 7 Figure 8 Figure 9 Figure 10 PMID:6333184

  7. Development of Canadian Recommendations for the Management of ANCA-Associated Vasculitides: Results of the National Needs Assessment Questionnaire

    PubMed Central

    Famorca, Leilani; Twilt, Marinka; Barra, Lillian; Bakowsky, Volodko; Benseler, Susanne; Cabral, David; Carette, Simon; Dhindsa, Navjot; Fifi-Mah, Aurore; Goulet, Michelle; Khalidi, Nader; Khraishi, Majed; McGeoch, Lucy; Milman, Nataliya; Pineau, Christian; Shojania, Kam; Taylor-Gjevre, Regina; Towheed, Tanveer; Trudeau, Judith; Yacyshyn, Elaine; Liang, Patrick; Pagnoux, Christian

    2015-01-01

    Objectives : To study variations in Canadian clinical practice patterns for the management of ANCA-associated vasculitis (AAV) and identify points to consider for the development of national recommendations. Material and Methodology : A 30-item needs assessment questionnaire was sent to all members of the Canadian Vasculitis network (CanVasc), Canadian Rheumatology Association (CRA), Canadian Thoracic Society (CTS) and Canadian Society of Nephrology (CSN). Respondent characteristics, practice patterns, concerns and expectations were analyzed. Results : Among 132 physicians who followed at least 1 vasculitis patient and responded to the survey, 39% stated that they felt confident in their management of AAV. Several variations in practice were observed regarding diagnostic procedure, induction and maintenance treatments and use of biologics; some were due to logistic constraints (difficulties in access to some specific tests, drugs or care; lack of health care coverage for the costs). The top 5 topics for which recommendations are expected involve treatment for remission induction, maintenance, refractory disease, and relapse as well as biologics. Conclusion : Practice variations identified in this needs assessment survey will serve to formulate key questions for the development of CanVasc recommendations. PMID:25893028

  8. Granulomatosis with Polyangiitis Presenting with Coronary Artery and Pericardial Involvement

    PubMed Central

    Dewan, Rohit; Trejo Bittar, Humberto E.; Lacomis, Joan; Ocak, Iclal

    2015-01-01

    Granulomatosis with polyangiitis is a systemic disease resulting in necrotizing vasculitis of small- and medium-sized vessels. Cardiac involvement is rare and when present usually manifests with pericarditis and coronary artery vasculitis. We report here a case of granulomatosis with polyangiitis involving the native coronary arteries, bypass graft, and pericardium with interesting imaging findings on contrast-enhanced CT and MRI. A 57-year-old man with a history of chronic headaches presented to the emergency room with syncope. Contrast-enhanced CT demonstrated extensive soft tissue attenuation around the native coronary arteries and bypass graft. Contrast-enhanced MRI demonstrated enhancing nodular soft tissue surrounding the coronary arteries, bypass graft, and pericardium. Pericardial biopsy revealed a necrotizing granulomatous pericarditis with vasculitis concerning for granulomatosis with polyangiitis. The patient demonstrated MPO-positive and PR-3 negative serologies. After being discharged on rituximab and prednisone, follow-up CT 3 years later showed significant improvement of the soft tissue thickening surrounding the coronary arteries, bypass graft, and pericardium. PMID:26798536

  9. Nitric oxide as an inflammatory mediator in autoimmune MRL-lpr/lpr mice.

    PubMed Central

    Weinberg, J B

    1998-01-01

    Nitric oxide (.NO) may exhibit proinflammatory features. .NO synthase type 2 (NOS2) is overexpressed and .NO overproduced in rodent models of induced inflammation. Blockage of .NO production by administration of NOS inhibitors prevents or reduces various types of induced inflammation in mice and rats. We have shown that autoimmune MRL-lpr/lpr mice overexpress NOS2 and overproduce .NO in an age-dependent fashion that parallels expression of arthritis, glomerulonephritis, and vasculitis. Blocking .NO production by oral administration of the NOS inhibitor NG-monomethyl-L-arginine reduced the arthritis, glomerulonephritis, and vasculitis, but it did not modify serum anti-DNA antibody levels or glomerular deposition of immune complexes. When mice with genetically disrupted NOS2 were backcrossed to MRL-lpr/lpr mice, the resultant (-/-) mice expressed no NOS2 and produced no .NO, the wild-type (+/+) mice overexpressed NOS2 and overproduced .NO (in comparison to normal, control mice), and the heterozygous (+/-) mice expressed and produced intermediate levels. Nephritis and arthritis in the (-/-) mice were comparable to that in MRL-lpr/lpr mice, but vasculitis was markedly decreased. Levels of anti-DNA antibodies were comparable in all mice, but IgG rheumatoid factor production was markedly reduced in the (-/-) mice. These results of studies in MRL-lpr/lpr mice with genetically disrupted NOS2 highlight the heterogeneity and complexity of the role of NOS2 and .NO in inflammation. PMID:9788887

  10. Functional and morphologic changes during experimental Rocky Mountain spotted fever in guinea pigs.

    PubMed

    Moe, J B; Mosher, D F; Kenyon, R H; White, J D; Stookey, J L; Bagley, L R; Fine, D P

    1976-09-01

    Experimental Rocky Mountain spotted fever was studied in guinea pigs following intraperitoneal inoculation of 10(7) Rickettsia rickettsii. After a 2-day incubation period, animals developed fever, progressive emaciation, and scrotal swelling with necrosis. Vasculitis, with increased small vessel permeability for colloidal carbon, was evident in cremaster muscles as early as 1 day after inoculation. Inflammatory changes in vessels became progressively more severe as numbers of circulating rickettsiae increased. Thrombosis and vascular occlusion were first evident on day 4. Mild thrombocytopenia developed, coinciding with the development of vasculitis, and preceding the appearance of either fibrin-split products in blood or thrombi in vessels. Rickettsiae were first detected in blood on day 2; peak rickettsemia occurred on days 5 to 8. Rickettsiae were demonstrated in inflamed vessels on day 5 and later, but not at earlier stages. Serum lysozyme concentration was moderately elevated and hemolytic complement was moderately depressed throughout the illness. Agglutinating antibody was present in low titers on days 3 to 10. Antibody titers increased on days 12 to 16 after the rickettsiae were cleared from blood. These studies indicate that vasculitis seen early in the course of Rocky Mountain spotted fever is the result of rickettsial infection, but is not dependent on the presence of rickettsiae in endothelial cells or other blood vessel components. PMID:822237

  11. Biopsy findings in primary angiitis of the central nervous system.

    PubMed

    Miller, Dylan V; Salvarani, Carlo; Hunder, Gene G; Brown, Robert D; Parisi, Joseph E; Christianson, Teresa J; Giannini, Caterina

    2009-01-01

    Primary angiitis of the central nervous system (PACNS) is a form of vasculitis restricted to the brain and spinal cord, with protean clinical manifestations and often slowly progressive course. Outcomes vary, ranging from spontaneous resolution to rapid decline and death. Diagnosis of PACNS is based on angiography and/or biopsy. We reviewed surgical biopsies from 46 patients (53 biopsies) with PACNS, including 25 men and 21 women (median age 46, range: 25 to 84 y) and correlated the findings with relevant clinical parameters. Biopsies (51 brain, 2 spinal) were diagnostic of vasculitis in 29 (63%) patients. Three morphologic patterns of vasculitis were observed: acute necrotizing (n=4, 14%); purely lymphocytic (n=8, 28%); and granulomatous (n=17, 58%), 8 associated with deposition of beta-A4 amyloid. Biopsies not diagnostic of PACNS (n=17, 37%) showed nonspecific gliosis (53%), mild perivascular mononuclear inflammation (18%), and parenchymal ischemic damage/infarct (18%). All positive biopsies were among those directed to an imaging abnormality (targeted biopsies) and biopsies including leptomeninges were more often positive than those that did not. Thus, where possible, a targeted biopsy that includes leptomeninges is recommended to maximize diagnostic potential. No statistically significant differences in outcome were noted among the 3 histopathologic groups or when comparing biopsy positive versus biopsy negative PACNS groups. Overall the outcomes were relatively favorable, with only 14% mortality or severe morbidity at 1.14 years (mean) after biopsy. PMID:18941399

  12. Mycoplasma pneumoniae as a trigger for Henoch-Schnlein purpura in children.

    PubMed

    Ku?ma-Mroczkowska, El?bieta; Pa?czyk-Tomaszewska, Ma?gorzata; Szmigielska, Agnieszka; Szymanik-Grzelak, Hanna; Roszkowska-Blaim, Maria

    2016-01-01

    Mycoplasma pneumoniae is one of the most common causes of respiratory tract infections in children. Extrapulmonary manifestations are seen in up to 25% of infected patients. Extrapulmonary complications are associated with the central nervous system, gastrointestinal tract, skin changes, myocarditis, pericarditis, hemolytic anemia, thrombocytopenia and thrombosis. The majority of extrapulmonary symptoms are associated with skin changes such as exanthematous skin eruptions, erythema nodosum, urticaria, Stevens-Jonson syndrome. M. pneumoniae stimulates production of the interleukins and tumor necrosis factor (TNF) ? and can cause vasculitis. Henoch-Schnlein purpura (HSP) is a leucoclastic vasculitis that affects small vessels. Clinical manifestations of HSP include typical rash, arthritis, gastrointestinal and sometimes renal involvement. The main feature in HSP is abnormal IgA deposits in vessel walls. Circulating abnormal glycosylated IgA 1 and IgG antibodies form immune complexes: IgA1-IgG and anti-IgA 1. Immune complexes activate cytokines, parts of complement and influence directly the endothelium. We report cases of three children with Henoch-Schnlein purpura with prolonged and recurrent skin and joint changes. The serological analysis (positive serum IgM) confirmed Mycoplasma pneumoniae infection. Treatment with clarithromycin caused complete regression of disease. We suggest that in the case of prolonged symptoms of vasculitis due to Henoch-Schnlein purpura, Mycoplasma pneumonia infection may be a potential cause of exacerbation of the disease. PMID:26862316

  13. Pathology of Experimental Encephalitozoon cuniculi Infection in Immunocompetent and Immunosuppressed Mice in Iraq

    PubMed Central

    Al-Sadi, Hafidh I.; Al-Mahmood, Saevan S.

    2014-01-01

    This study was performed to evaluate pathology of experimental Encephalitozoon cuniculi (Iraqi isolate) infection in normal and immunosuppressed mice. Pathological changes were not seen in negative control mice while secondary bacterial infections were noted in the lungs, kidneys, and heart of mice given dexamethasone. Typical E. cuniculi infection lesions were found in brain, livers, lungs, and kidneys of mice given 107  E. cuniculi spores/mouse orally. These lesions were in the form of nonsuppurative meningoencephalitis with vasculitis in brain, interstitial inflammation with infiltration of both lymphocytes and plasma cells in lung tissue, and nonsuppurative interstitial (focal and diffuse) nephritis, presence of vacuole containing mature and immature spores in enterocytes within the tips of villi, and lymphoiod hyperplasia of the white pulp and vasculitis of the intratrabecular vessels. Mice that were given 107  E. cuniculi spores/mouse orally showed lesions similar to those observed in the previous group (vasculitis and granulomas) but the lesions were more severe and widespread. In conclusion, this is the first report of experimental E. cuniculi infection induced by E. cuniculi isolated from a naturally infected rabbit in Iraq and that infection became more severe and widespread upon the administration of dexaethasone. PMID:24772366

  14. Kidney and Urinary Tract Involvement in Kawasaki Disease

    PubMed Central

    Watanabe, Toru

    2013-01-01

    Kawasaki disease (KD) is a systemic vasculitis and can develop multiple organ injuries including kidney and urinary tract involvement. These disorders include pyuria, prerenal acute kidney injury (AKI), renal AKI caused by tubulointerstitial nephritis (TIN), hemolytic uremic syndrome (HUS), and immune-complex mediated nephropathy, renal AKI associated with either Kawasaki disease shock syndrome or unknown causes, acute nephritic syndrome (ANS), nephrotic syndrome (NS), renal tubular abnormalities, renal abnormalities in imaging studies, and renal artery lesions (aneurysms and stenosis). Pyuria is common in KD and originates from the urethra and/or the kidney. TIN with AKI and renal tubular abnormalities probably result from renal parenchymal inflammation caused by T-cell activation. HUS and renal artery lesions are caused by vascular endothelial injuries resulting from vasculitis. Some patients with ANS have immunological abnormalities associated with immune-complex formation. Nephromegaly and renal parenchymal inflammatory foci are detected frequently in patients with KD by renal ultrasonography and renal scintigraphy, respectively. Although the precise pathogenesis of KD is not completely understood, renal vasculitis, immune-complex mediated kidney injuries, or T-cell immune-regulatory abnormalities have been proposed as possible mechanisms for the development of kidney and urinary tract injuries. PMID:24288547

  15. Eosinophilic Granulomatosis With Polyangiitis With Thrombotic Microangiopathy: Is Simultaneous Systemic Lupus Erythematosus Associated With Clinical Manifestations?: A Case Report and Review of the Literature.

    PubMed

    Fukui, Shoichi; Iwamoto, Naoki; Tsuji, Sosuke; Umeda, Masataka; Nishino, Ayako; Nakashima, Yoshikazu; Suzuki, Takahisa; Horai, Yoshiro; Koga, Tomohiro; Kawashiri, Shin-Ya; Ichinose, Kunihiro; Hirai, Yasuko; Tamai, Mami; Nakamura, Hideki; Origuchi, Tomoki; Kinoshita, Nae; Kawakami, Atsushi

    2015-11-01

    Eosinophilic granulomatosis with polyangiitis (EGPA) is one of the antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, which is characterized by vasculitis of the small to medium-sized vessels. On the contrary, thrombotic microangiopathy (TMA) is a life-threatening condition which can cause ischemic organ injury. Although several case reports have described patients with TMA associated with ANCA-associated vasculitis except for EGPA, there are no previous case reports of EGPA associated with TMA.A 71-year-old Japanese man was diagnosed with EGPA based on his asthma, eosinophilia, lung opacity, refractory sinusitis, and positive myeloperoxidase-ANCA. He was also diagnosed with TMA based on peripheral schizocytes and hemolytic anemia. We performed plasmapheresis and started high-dose corticosteroid therapy; thereafter, he improved promptly. His case also fulfilled the classification criteria of systemic lupus erythematosus (SLE) based on the pleural effusion, renal disorder, anemia, thrombocytopenia, positive antidouble-stranded DNA antibody, and low complement. Elements of SLE were thought to affect his clinical course.We reviewed 11 patients with EGPA or hypereosinophilic syndrome (HES) associated with SLE, including our case. Patients with EGPA or HES associated with SLE had more heart complications than patients with simple EGPA or simple HES did. Patients with EGPA or HES associated with SLE had more pleural effusion than patients with simple SLE did.Clinical manifestations of eosinophilia with SLE or SLE with eosinophilia may differ from simple SLE or simple eosinophilia. PMID:26559264

  16. Mycoplasma pneumoniae as a trigger for Henoch-Schönlein purpura in children

    PubMed Central

    Pańczyk-Tomaszewska, Małgorzata; Szmigielska, Agnieszka; Szymanik-Grzelak, Hanna; Roszkowska-Blaim, Maria

    2016-01-01

    Mycoplasma pneumoniae is one of the most common causes of respiratory tract infections in children. Extrapulmonary manifestations are seen in up to 25% of infected patients. Extrapulmonary complications are associated with the central nervous system, gastrointestinal tract, skin changes, myocarditis, pericarditis, hemolytic anemia, thrombocytopenia and thrombosis. The majority of extrapulmonary symptoms are associated with skin changes such as exanthematous skin eruptions, erythema nodosum, urticaria, Stevens-Jonson syndrome. M. pneumoniae stimulates production of the interleukins and tumor necrosis factor (TNF) α and can cause vasculitis. Henoch-Schönlein purpura (HSP) is a leucoclastic vasculitis that affects small vessels. Clinical manifestations of HSP include typical rash, arthritis, gastrointestinal and sometimes renal involvement. The main feature in HSP is abnormal IgA deposits in vessel walls. Circulating abnormal glycosylated IgA 1 and IgG antibodies form immune complexes: IgA1-IgG and anti-IgA 1. Immune complexes activate cytokines, parts of complement and influence directly the endothelium. We report cases of three children with Henoch-Schönlein purpura with prolonged and recurrent skin and joint changes. The serological analysis (positive serum IgM) confirmed Mycoplasma pneumoniae infection. Treatment with clarithromycin caused complete regression of disease. We suggest that in the case of prolonged symptoms of vasculitis due to Henoch-Schönlein purpura, Mycoplasma pneumonia infection may be a potential cause of exacerbation of the disease. PMID:26862316

  17. Spontaneous and antiviral-induced cutaneous lesions in chronic hepatitis B virus infection

    PubMed Central

    Grigorescu, Ioana; Dumitrascu, Dan Lucian

    2014-01-01

    AIM: To describe spontaneous, or interferon (IFN)- or immunization-induced skin lesions in hepatitis B virus (HBV) infection. METHODS: A comprehensive literature search of all the papers presenting case reports of dermatological lesions in patients with chronic HBV infection was carried out. We included only patients with histologically proven skin lesions that appeared in the normal course of hepatitis B infection, or after immunization for hepatitis B or antiviral treatment. RESULTS: We found 44 papers on this topic, reporting 151 cases. About 2% of patients with hepatitis B infection, mainly men, presented with skin lesions. Among patients with chronic hepatitis B, vasculitis and essential mixed cryoglobulinemia seemed to be the most frequent skin lesion (53.3%), followed by papular changes, rashes and Gianotti-Crosti syndrome, skin carcinoma and Henoch-Schnlein purpura were rare. IFN treatment seemed to be effective against HBV-associated and immunoglobulin-complex-mediated disease (vasculitis). Two cutaneous lesions (lichen planus and granuloma annulare) were described after hepatitis B vaccination. Systemic lupus and lupus-like lesions were the most frequently encountered lesions after antiviral treatment. Immunosuppressive and steroid therapy ameliorates lichen planus lesions in 50% of cases. CONCLUSION: Vasculitis was the most frequent spontaneous skin lesion found in chronic hepatitis B. Lichen planus was most frequent after immunization and lupus/lupus-like lesions after IFN. PMID:25400473

  18. P-ANCA cranial pachymeningitis: a case report.

    PubMed

    Jacobi, D; Maillot, F; Hommet, C; Arsne, S; Cottier, J P; Lamisse, F; Guillevin, L

    2005-04-01

    Pachymeningitis is an inflammatory process that thickens the dura mater. This disease has various etiologies including infectious, neoplastic, or autoimmune diseases. We present the case of a patient who developed cranial pachymeningitis with a clinical and biological picture suggestive of a neurological form of vasculitis. A 51-year-old woman developed rhinitis, otitis media, headaches, and deterioration of her condition after a course of recombinant hepatitis B vaccine. After a booster dose of the vaccine, she developed unilateral visual loss and impairment of multiple cranial nerves. Blood analysis showed inflammation and presence of antimyeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA). Cranial magnetic resonance imaging (MRI) showed pachymeningitis. A complete remission was obtained with immunosuppressive therapy. The initial clinical presentation and subsequent remission under immunosuppressive therapy were suggestive of a vasculitis with nervous system involvement. Though vasculitis was not proven histologically in this patient, we believe that MPO-ANCA-related autoimmunity provoked the patient's disease as already reported in similar cases. As pachymeningitis is a fibrosing process, early recognition and treatment of an autoimmune etiology, even in the absence of previous pulmonary or renal involvement, is required to prevent definitive neurological impairment. PMID:15578248

  19. The Influence and Role of Microbial Factors in Autoimmune Kidney Diseases: A Systematic Review

    PubMed Central

    2015-01-01

    A better understanding of the pathophysiology of autoimmune disorders is desired to allow tailored interventions. Despite increased scientific interest a direct pathogenic factor in autoimmune renal disease has been described only in a minority like membranous nephropathy or ANCA-associated vasculitis. Nonetheless the initial step leading to the formation of these antibodies is still obscure. In this review we will focus on the possible role of microbial factors in this context. Staphylococcus aureus may be a direct pathogenetic factor in granulomatosis with polyangiitis (GPA). Chronic bacterial colonization or chronic infections of the upper respiratory tract have been proposed as trigger of IgA vasculitis and IgA nephropathy. Interventions to remove major lymphoid organs, such as tonsillectomy, have shown conflicting results but may be an option in IgA vasculitis. Interestingly no clear clinical benefit despite similar local colonization with bacterial strains has been detected in patients with IgA nephropathy. In systemic lupus erythematosus injection of bacterial lipopolysaccharide induced progressive lupus nephritis in mouse models. The aim of this review is to discuss and summarize the knowledge of microbial antigens in autoimmune renal disease. Novel methods may provide insight into the involvement of microbial antigens in the onset, progression, and prognosis of autoimmune kidney disorders. PMID:26078982

  20. Eosinophilic Fasciitis and Acute Encephalopathy Toxicity from Pembrolizumab Treatment of a Patient with Metastatic Melanoma.

    PubMed

    Khoja, Leila; Maurice, Catherine; Chappell, MaryAnne; MacMillan, Leslie; Al-Habeeb, Ayman S; Al-Faraidy, Nada; Butler, Marcus O; Rogalla, Patrik; Mason, Warren; Joshua, Anthony M; Hogg, David

    2016-03-01

    Anti-PD-1 inhibitors have significant activity in metastatic melanoma. Responses often occur early and may be sustained. The optimal duration of treatment with these agents is unknown. Here, we report the case of a 51-year-old woman treated with pembrolizumab, as part of the Keynote-001 trial, as first-line treatment for metastatic disease. She experienced a complete response after 13.8 months of treatment with no adverse events. One month after the last drug infusion and 18 months from starting treatment, the patient presented with eosinophilic fasciitis. She then developed acute confusion and weakness, thought to be due to intracranial vasculitis. High-dose steroids were initiated with resolution of the fasciitis. Aspirin was commenced for presumed vasculitis with resolution of the neurologic symptoms. To our knowledge, there are no previous reports of eosinophilic fasciitis or cerebral vasculitis due to anti-PD-1 agents. This case demonstrates that toxicity may occur in association with pembrolizumab treatment after a prolonged period of treatment without toxicity. Future trials should explore the optimal duration of treatment with pembrolizumab. Cancer Immunol Res; 4(3); 175-8. 2016 AACR. PMID:26822024

  1. A challenging twist in pulmonary renal syndrome.

    PubMed

    Namas, Rajaie; Rubin, Bernard; Adwar, Wamidh; Meysami, Alireza

    2014-01-01

    Case. We report a rare case of hydralazine-induced anti-neutrophil cytoplasmic antibody-associated vasculitis. A 75-year-old African American woman with history of high blood pressure on hydralazine for 3 years presented with acute onset of shortness of breath and hemoptysis. Lab workup revealed a severe normocytic anemia and a serum creatinine of 5.09?mg/dL (baseline 0.9). Bronchoscopy demonstrated active pulmonary hemorrhage. A urine sample revealed red cell casts and a renal biopsy demonstrated pauci-immune, focally necrotizing glomerulonephritis with small crescents consistent with possible anti-neutrophil cytoplasmic antibody-positive renal vasculitis. Serologies showed high-titer MPO-ANCA and high-titer anti-histone antibodies. She was treated with intravenous steroids and subsequently with immunosuppression after cessation of hydralazine. The patient was subsequently discharged from hospital after a rapid clinical improvement. Conclusion. Hydralazine-induced anti-neutrophil cytoplasmic antibody-positive renal vasculitis is a rare adverse effect and can present as a severe vasculitic syndrome with multiple organ involvement. Features of this association include the presence of high titer of anti-myeloperoxidase anti-neutrophil cytoplasmic antibody with multiantigenicity, positive anti-histone antibodies, and the lack of immunoglobulin and complement deposition. Prompt cessation of hydralazine may be sufficient to reverse disease activity but immunosuppression may be needed. PMID:25525550

  2. Pathologic Evaluation of Type 2 Porcine Reproductive and Respiratory Syndrome Virus Infection at the Maternal-Fetal Interface of Late Gestation Pregnant Gilts

    PubMed Central

    Novakovic, Predrag; Harding, John C. S.; Al-Dissi, Ahmad N.; Ladinig, Andrea; Detmer, Susan E.

    2016-01-01

    The pathogenesis of fetal death caused by porcine reproductive and respiratory syndrome virus (PRRSV) remains unclear. The objective of this study was to improve our understanding of the pathogenesis by assessing potential relationships between specific histopathological lesions and PRRSV RNA concentration in the fetuses and the maternal-fetal interface. Pregnant gilts were inoculated with PRRSV (n = 114) or sham inoculated (n = 19) at 85±1 days of gestation. Dams and their litters were humanely euthanized and necropsied 21 days later. PRRSV RNA concentration was measured by qRT-PCR in the maternal-fetal interface and fetal thymus (n = 1391). Presence of fetal lesions was positively related to PRRSV RNA concentration in the maternal-fetal interface and fetal thymus (P<0.05 for both), but not to the distribution or severity of vasculitis, or the severity of endometrial inflammation. The presence of fetal and umbilical lesions was associated with greater odds of meconium staining (P<0.05 for both). The distribution and severity of vasculitis in endometrium were not significantly related to PRRSV RNA concentration in maternal-fetal interface or fetal thymus. Endometrial inflammation severity was positively related to distribution and severity of vasculitis in endometrium (P<0.001 for both). Conclusions from this study suggest that type 2 PRRSV infection in pregnant gilts induces significant histopathological lesions at maternal-fetal interface, but they are not associated with presence of PRRSV in the maternal-fetal interface at 21 days post infection. Conversely, fetal pathological lesions are associated with presence of PRRSV in the maternal-fetal interface and fetal thymus, and meconium staining is significantly associated with the presence of both fetal and umbilical lesions observed 21 days post infection. PMID:26963101

  3. Early-Onset Stroke and Vasculopathy Associated with Mutations in ADA2

    PubMed Central

    Zhou, Q.; Yang, D.; Ombrello, A.K.; Zavialov, Andrey V.; Toro, C.; Zavialov, Anton V.; Stone, D.L.; Chae, J.J.; Rosenzweig, S.D.; Bishop, K.; Barron, K.S.; Kuehn, H.S.; Hoffmann, P.; Negro, A.; Tsai, W.L.; Cowen, E.W.; Pei, W.; Milner, J.D.; Silvin, C.; Heller, T.; Chin, D.T.; Patronas, N.J.; Barber, J.S.; Lee, C.-C.R.; Wood, G.M.; Ling, A.; Kelly, S.J.; Kleiner, D.E.; Mullikin, J.C.; Ganson, N.J.; Kong, H.H.; Hambleton, S.; Candotti, F.; Quezado, M.M.; Calvo, K.R.; Alao, H.; Barham, B.K.; Jones, A.; Meschia, J.F.; Worrall, B.B.; Kasner, S.E.; Rich, S.S.; Goldbach-Mansky, R.; Abinun, M.; Chalom, E.; Gotte, A.C.; Punaro, M.; Pascual, V.; Verbsky, J.W.; Torgerson, T.R.; Singer, N.G.; Gershon, T.R.; Ozen, S.; Karadag, O.; Fleisher, T.A.; Remmers, E.F.; Burgess, S.M.; Moir, S.L.; Gadina, M.; Sood, R.; Hershfield, M.S.; Boehm, M.; Kastner, D.L.; Aksentijevich, I.

    2014-01-01

    BACKGROUND We observed a syndrome of intermittent fevers, early-onset lacunar strokes and other neurovascular manifestations, livedoid rash, hepatosplenomegaly, and systemic vasculopathy in three unrelated patients. We suspected a genetic cause because the disorder presented in early childhood. METHODS We performed whole-exome sequencing in the initial three patients and their unaffected parents and candidate-gene sequencing in three patients with a similar phenotype, as well as two young siblings with polyarteritis nodosa and one patient with small-vessel vasculitis. Enzyme assays, immunoblotting, immunohistochemical testing, flow cytometry, and cytokine profiling were performed on samples from the patients. To study protein function, we used morpholino-mediated knockdowns in zebrafish and short hairpin RNA knockdowns in U937 cells cultured with human dermal endothelial cells. RESULTS All nine patients carried recessively inherited mutations in CECR1 (cat eye syndrome chromosome region, candidate 1), encoding adenosine deaminase 2 (ADA2), that were predicted to be deleterious; these mutations were rare or absent in healthy controls. Six patients were compound heterozygous for eight CECR1 mutations, whereas the three patients with polyarteritis nodosa or small-vessel vasculitis were homozygous for the p.Gly47Arg mutation. Patients had a marked reduction in the levels of ADA2 and ADA2-specific enzyme activity in the blood. Skin, liver, and brain biopsies revealed vasculopathic changes characterized by compromised endothelial integrity, endothelial cellular activation, and inflammation. Knockdown of a zebrafish ADA2 homologue caused intracranial hemorrhages and neutropenia phenotypes that were prevented by coinjection with nonmutated (but not with mutated) human CECR1. Monocytes from patients induced damage in cocultured endothelial-cell layers. CONCLUSIONS Loss-of-function mutations in CECR1 were associated with a spectrum of vascular and inflammatory phenotypes, ranging from early-onset recurrent stroke to systemic vasculopathy or vasculitis. (Funded by the National Institutes of Health Intramural Research Programs and others.) PMID:24552284

  4. [Infective endocarditis due to high level aminoglycoside resistant Enterococcus faecalis and methicillin resistant coagulase-negative staphylococci presenting with rheumatic manifestations].

    PubMed

    Pi?kin, Nihal; Akduman, Deniz; Aydemir, Hande; Celebi, Gven; Oztoprak, Nefise; Akta?, Elif

    2008-07-01

    Infective endocarditis has variable clinical presentations and may present with rheumatologic manifestations. Infective endocarditis due to high level aminoglycoside resistant enterococci represents a severe therapeutic challenge as none of the currently recommended treatment regimens are bactericidal against these isolates. In this report, a case of infective endocarditis with double aetiology, high level aminoglycoside resistant Enterococcus faecalis together with methicillin-resistant coagulase-negative staphylococci (MR-CNS), presenting with leukocytoclastic vasculitis and rapidly progressive glomerulonephritis, has been presented. A 48-years-old woman was admitted to our hospital with malaise and non-pruritic purpural rush on her lower extremities. On admission she had no fever or leukocytosis. Skin biopsy showed leukocytoclastic vasculitis and steroid therapy was started. On 12th day of admission rapidly progressive glomerulonephritis was diagnosed and she received plasmapheresis and haemodialysis support. Transthoracic echocardiography (TTE) demonstrated 1 x 1.5 cm vegetation on the mitral valve. An initial diagnosis of infective endocarditis was made and empirical treatment with vancomycin and gentamicin was started. All blood cultures yielded high level aminoglycoside resistant E. faecalis and additionally two of them yielded MR-CNS. Vancomycin was administered in combination with high dose ampicillin and repeated blood cultures taken after administration of ampicillin, revealed no growth. The patient remained afebrile, renal functions improved and a repeat TTE done on 20th day of ampicillin therapy showed waning of the vegetation. On 42nd day of treatment repeat TTE showed new vegetation on the mitral valve and severe valve insufficiency, so the patient was scheduled for mitral valve replacement. She was treated for 12 weeks with vancomycin and ampicillin and recovered successfully. In conclusion; infective endocarditis should be considered in the differential diagnosis of leukocytoclastic vasculitis and rapidly progressive glomerulonephritis. Physicians should document their treatment outcomes and experience with high level aminoglycoside resistant enterococcal infective endocarditis, which is a therapeutic challenge, so that the best therapeutic options can be identified. PMID:18822897

  5. Severe deterioration of newly diagnosed Takayasu arteritis in a patient re-treated with interferon beta-1? for concomitant longstanding multiple sclerosis.

    PubMed

    Diamantopoulos, Andreas P; Hetland, Helene; Hansen, Atle E; Myklebust, Geirmund

    2012-06-01

    A 52-year-old woman with Takayasu arteritis and a known history of multiple sclerosis had been treated with subcutaneous interferon (IFN) beta-1?. After the re-introduction of the IFN beta-1?, the patient had a gradual worsening of the arteritis, with claudication symptoms in the left arm and increased inflammation markers. An evaluation using Doppler ultrasound of the supra-aortic vessels revealed severe stenosis of the left axillary artery. The IFN beta-1? was withdrawn, with prompt clinical and laboratory improvement of the vasculitis. PMID:21928043

  6. Acute Esophageal Necrosis Presenting With Henoch-Schönlein Purpura

    PubMed Central

    Bernstein, Gregory R.; Malik, Zubair; Schey, Ron

    2015-01-01

    A 63-year-old woman with abdominal pain and melena developed a palpable, purpuric rash and acute kidney injury. Skin and kidney biopsy confirmed Henoch-Schönlein purpura. Upper endoscopy revealed diffuse, circumferential, black-appearing mucosa of the esophagus consistent with acute esophageal necrosis (AEN), also known as black esophagus. AEN is a very rare cause of gastrointestinal hemorrhage with a high mortality risk. To our knowledge, there have been no prior reports of AEN associated with Henoch-Schonlein purpura or other vasculitis. PMID:26504868

  7. Histopathologic features in a case of hyperimmunoglobulinemia D syndrome

    PubMed Central

    Pace, Sarah; Bingham, Jonathan; Royer, Michael

    2015-01-01

    We describe a case of Mevalonate Kinase Deficiency (MKD) also known as Hyperimmunoglobulinemia D Syndrome (HIDS) presenting as a Sweet-like syndrome in a 5-week-old with multiple erythematous plaques, fever, aseptic meningitis, and bronchiolitis. The locations of the predominant plaques were periumbilical and periocular, which originally prompted concern for omphalitis and preseptal cellulitis. Histopathology demonstrated a neutrophilic and histiocytic dermatitis with prominent squamous syringometaplasia and leukocytoclasis in the absence of a vasculitis. This case is reported here due to the unique findings of a prominent histiocytic component in addition to the typically described neutrophilic infiltrate. PMID:26904447

  8. [Skin manifestations related to hepatitis C viruses].

    PubMed

    Doutre, M S; Beylot, C; Beylot-Barry, M; Couzigou, P; Beylot, J

    1995-01-01

    The hepatitis C virus causes both hepatic and extrahepatic disorders, particularly as regards dermatology. The link between essential mixed cryoglobulinemias and the C virus infection has been clearly evidenced., whereas its frequency seems low in other systemic vasculitis such as polyarteritis nodosa. Similarly, the link between C virus hepatopathy and porphyria cutanea tarda is now proven. Lichen planus is also described as being associated with this virus, but further epidemiological studies are required to determine the exact prevalence of lichen in C virus hepatopathy cases. Finally, various cutaneous disorders, such as urticaria, erythema multiforme, dermo-hypodermitis, etc, occasionally arise during acute or chronic hepatitis C. PMID:7481154

  9. Extrahepatic manifestations of infection with hepatitis C virus.

    PubMed

    Mehta, S; Levey, J M; Bonkovsky, H L

    2001-11-01

    Chronic hepatitis C is associated with, and may trigger or exacerbate, an extraordinary variety of extrahepatic manifestations. Most of these manifestations affect the skin, the most frequent and important of which are the leukocytoclastic vasculitis of MC type II and PCT. The former is an example of an autoimmune disorder triggered by HCV infection, whereas PCT is a skin disease caused by hepatic overproduction of uro- and 7-carboxyl porphyrins caused by increased oxidative stress in hepatocytes. Currently available effective therapies of CHC (IFN, ribavirin) may also trigger or exacerbate extrahepatic manifestations, especially including autoimmune thyroiditis, skin rashes, and hemolytic anemia. PMID:11685805

  10. Central nervous system granulomastous phlebitis with limited extracranial involvement of the heart and lungs: An autopsy case.

    PubMed

    Mlakar, Jernej; Zorman, Jerneja Vide?nik; Mati?i?, Mojca; Vrabec, Matej; Alibegovi?, Armin; Popovi?, Mara

    2016-02-01

    Primary angiitis of the central nervous system is a rare condition, usually with an insidious onset. There is a wide variety of histological types (granulomatous, lymphocytic or necrotizing vasculitis) and types of vessel involved (arteries, veins or both). Most cases are idiopathic. We describe a first case of idiopathic granulomatous central nervous system phlebitis with additional limited involvement of the heart and lung, exclusively affecting small and medium sized veins in a 22-year-old woman, presenting as a sub acute headache. The reasons for this peculiar limitation of inflammation to the veins and the involvement of the heart and lungs are unknown. PMID:26198847

  11. Massive pulmonary hemorrhage in neonatal infection.

    PubMed Central

    Yeung, C. Y.

    1976-01-01

    Of 35 newborn infants who died from an infection 19 had postmortem evidence of massive pulmonary hemorrhage. All but 1 of the 19 had evidence of antimortem formation of intravascular fibrin clots in lung tissue. Seventeen infants had low platelet counts. Of the 11 infants in whom coagulation studies were done, 8 had evidence of disseminated intravascular coagulation (DIC) during life. Vasculitis in the lungs, associated with fibrin clots and hemorrhages, was detected in two infants. It is postulated that sepsis is an important cause of hemorrhage in the newborn, probably as a result of the development of DIC. Images FIG. 1 FIG. 2 PMID:766937

  12. Rheumatic manifestations of hepatitis C virus chronic infection: Indications for a correct diagnosis.

    PubMed

    Palazzi, Carlo; D'Amico, Emilio; D'Angelo, Salvatore; Gilio, Michele; Olivieri, Ignazio

    2016-01-28

    Hepatitis C virus (HCV) is a hepato- and lymphotropic agent that is able to induce several autoimmune rheumatic disorders: vasculitis, sicca syndrome, arthralgias/arthritis and fibromyalgia. The severity of clinical manifestations is variable and sometimes life-threatening. HCV infection can mimic many primitive rheumatic diseases, therefore, it is mandatory to distinguish HCV-related manifestations from primitive ones because the prognosis and therapeutic strategies can be fairly dissimilar. The new direct-acting antivirals drugs can help to avoid the well-known risks of worsening or new onset of autoimmune diseases during the traditional interferon-based therapies. PMID:26819509

  13. Pseudotumor Formation in a Metal-on-polyethylene Total Hip Arthroplasty Due to Trunnionosis at the Head-neck Taper.

    PubMed

    Stahnke, Jake T; Sharpe, Kipling P

    2015-11-01

    We present here a case of pseudotumor formation likely due to metal wear debris generated at the head-neck taper (trunnion) of the femoral stem and head components in a metal-on-polyethylene (MOP) total hip arthroplasty. Over the last few years, this recently described diagnosis, trunnionosis, has been recognized and reported more frequently. This patient presented with a rather large (12-cm diameter) pseudotumor with accompanying loss of abductors and a pelvic discontinuity making reconstruction more challenging. We believe the psuedotumor in this patient developed from trunnionosis. This is an interesting case of aseptic lymphocyte-dominated vasculitis-associated lesions (ALVAL) in a MOP total hip arthroplasty. PMID:26680405

  14. Birdshot retinochoroidopathy review.

    PubMed

    Freitas-Neto, Clovis Arcoverde; Boonsopon, Sutasinee; Dhanireddy, Swetha; Maghsoudlou, Armin; Silpa-Archa, Sukhum; Foster, C Stephen

    2015-01-01

    Birdshot retinochoroidopathy (BSRC) is a distinct type of posterior uveitis originally described in the 1940s. Its characteristics include minimal anterior segment inflammation and diffuse posterior choroidopathy with vitritis and retinal vasculitis. The precise etiology of this disease is yet to be elucidated. However, various treatment modalities have been employed with the ultimate goal of durable remission of this vision threatening intraocular disease. The purpose of this review is not only to emphasize the importance of recognizing BSRC, but also to discuss the new discoveries, immune mediators, current and new therapies, and techniques applied to monitor and accomplish disease remission. PMID:25714542

  15. Aggressive eosinophilic granulomatosis with polyangiitis and transverse sinus thrombosis.

    PubMed

    Ananth, Snegha; Sankaralingam, Rajeswari; Manoj, Manesh

    2016-01-01

    We report a case of a patient with eosinophilic granulomatosis with polyangiitis (EGPA, previously known as Churg-Strauss syndrome) who succumbed to respiratory failure complicated by sepsis, pneumonitis and transverse sinus thrombosis. In the absence of antineutrophilic cytoplasmic antibodies positivity, a clinical diagnosis was made based on The American College of Rheumatology-1990 criteria. Glucocorticoids, cyclophosphamide, rituximab and intravenous immunoglobulin were administered at different stages. The patient recovered momentarily after each cycle of treatment, but his vasculitis progressed and he eventually died. Cardiac or renal involvement, which is more commonly associated with an adverse outcome, was not present. PMID:26759439

  16. Syphilitic Scleritis.

    PubMed

    Fnolland, Jean-Rmi; Bonnel, Samantha; Rambaud, Camille; Froussart-Maille, Franoise; Rigal-Sastourn, Jean-Claude

    2016-02-01

    A 47-year-old man developed a painful right red eye for 72 hours with a 20/25 decreased visual acuity. He had no medical history. Slit-lamp examination revealed a painful nodular scleritis at the equator of the globe in the infero-temporal quadrant. There was a moderate intraocular inflammation in the anterior segment. Fundus examination revealed a grade 1 hyalitis and a focal retinitis with vasculitis and arterio-veinous occlusion toward the scleritis zone. Syphilis and HIV serology were positive and the scleritis resolved 5 days after a penicillin G medication. Syphilitic scleritis are relatively uncommon. PMID:24833404

  17. Birdshot chorioretinopathy in a male patient with facioscapulohumeral muscular dystrophy.

    PubMed

    Papavasileiou, Evangelia; Lobo, Ann-Marie

    2015-01-01

    We report a case of birdshot chorioretinopathy (BSCR) in a patient with facioscapulohumeral muscular dystrophy (FSHD). A 40-year-old male with history of facioscapulohumeral muscular dystrophy with significant facial diplegia and lagophthalmos presents for an evaluation of bilateral choroiditis with vasculitis and optic disc edema. Clinical examination included fundus and autofluorescence photographs, fluorescein angiography, and optical coherence tomography. To our knowledge, this patient represents the first reported case of birdshot chorioretinopathy with facioscapulohumeral muscular dystrophy. Patients with FSHD can present with ocular findings and should be screened with dilated fundus examinations for retinal vascular changes and posterior uveitis. PMID:25861398

  18. Intranasal eosinophilic angiocentric fibrosis: a case report and review.

    PubMed

    Sunde, Jumin; Alexander, Kathrina A; Reddy, Vishnu V B; Woodworth, Bradford A

    2010-09-01

    Eosinophilc angiocentric fibrosis is a rare fibrosing vasculitis of unknown etiology that is progressive and potentially disfiguring. It has a predilection for the upper airways presenting most commonly as an obstructing mass lesion that is diagnosed histologically. Thus far, it has been nonfatal in the more than 40 reported cases; however, subglottic and ocular involvement has resulted in significant morbidity in several patients. Treatment has been challenging with persistent disease in most cases. The current case is a prototypical presentation with a limited nasal septal lesion providing the opportunity to discuss clinically relevant issues and increase awareness. PMID:20533005

  19. Endovascular Treatment of Left Subclavian Artery Pseudoaneurysm after Clavicle Fracture in an Elderly Adult with a 40-Year History of Behets Disease

    PubMed Central

    Hamasaki, Takafumi; Yamamoto, Shuichi; Kawai, Tsuyoshi; Sugiura, Kimihiko

    2015-01-01

    Behets disease (BD) is a rare chronic systemic vasculitis of unknown etiology that presents in young adults. We report a very rare case of a left subclavian artery pseudoaneurysm identified after a left clavicle fracture in a 70-year-old man with a 40-year history of intestinal BD. The patient received fixation by banding to treat a fracture after falling from a bed about 5 months previously, and he was referred to our hospital due to the finding of a left subclavian pulsatile mass. As enhanced computed tomography showed a left subclavian artery pseudoaneurysm, it was repaired using an endovascular stent graft.

  20. Takayasu's Arteritis with Systemic Lupus Erythematosus: A Rare Association

    PubMed Central

    Bandyopadhyay, Dhrubajyoti; Ganesan, Vijayan; Bhar, Debarati; Bhowmick, Diptak; Sasmal, Sibnarayan; Choudhury, Cankatika; Mukhopadhyay, Sabyasachi; Hajra, Adrija; Layek, Manas; Karmakar, Partha Sarathi

    2015-01-01

    We report the case of a 24-year-old nondiabetic, nonhypertensive lady with history of fatigue, dyspnoea and limb claudication. She has been diagnosed with Takayasu's arteritis. Subsequently she developed rash, alopecia, joint pain, and various other laboratory abnormalities which led to a diagnosis of SLE. Takayasu's arteritis (TA) rarely coexists with systemic lupus erythematosus (SLE). The absence of specific SLE markers in patients with TA who subsequently develop SLE suggests that the coexistence of these conditions may be coincidental. The antiphospholipid syndrome in patients with SLE may mimic the occlusive vasculitis of TA. PMID:26167325