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... pains, are common among people who have vasculitis. Outlook There are many types of vasculitis, but overall ... condition is rare. If you have vasculitis, the outlook depends on: The type of vasculitis you have ...



PubMed Central

The vasculitides are a large and diverse group of disorders, often confusing for the clinician in terms of diagnosis, investigation, and treatment. As most patients will have cutaneous lesions, it is useful for the physician to be aware of the dermatologic manifestations that may help in differentiating these diseases. This review covers both the common and not so common form of vasculitis, including both cutaneous and systemic disorders, an approach to investigation, and brief discussion of therapeutic approaches.

Ongley, Roberta C.



Hypersensitivity Vasculitis  


Hypersensitivity Vasculitis joseph July 18, 2012 No Comments What is Hypersensitivity vasculitis? Hypersensitivity vasculitis (HV) is often used to ... blood vessels, called a leukocytoclastic vasculitis. What causes Hypersensitivity vasculitis? HV may be caused by a specific ...


Cutaneous vasculitis.  


Cutaneous vasculitis comprises a wide spectrum of clinical syndromes and histopathologic findings that share the common theme of vascular inflammation and blood vessel damage. Clinical pattern and proper histopathologic studies are essential to the proper classification and evaluation of vasculitis. This article discusses the major types of cutaneous vasculitis typified by necrotizing or leukocytoclastic vasculitis and includes discussions of Henoch-Schönlein purpura, urticarial vasculitis, erythema elevatum diutinum, cutaneous polyarteritis nodosa, livedoid vasculitis, and cutaneous granulomatous vasculitis. PMID:8592739

Gibson, L E; Su, W P



Cutaneous vasculitis.  


Cutaneous vasculitis comprises a wide spectrum of clinical syndromes and histopathologic findings which share the common theme of vascular inflammation and changes in the adjacent tissue. This article discusses several types of cutaneous vasculitides including leukocytoclastic vasculitis, Henoch-Schönlein purpura, urticarial vasculitis, livedoid vasculitis, and granulomatous vasculitides. The clinical patterns of these types of vasculitic syndromes as well as associated diseases and histopathology are discussed. PMID:2189153

Gibson, L E; Su, W P



Urticarial Vasculitis  


... Vasculitis Foundation Fellowship is possible thanks to the generous support of Genentech, Inc., and Biogen Idec and many other generous individual and corporate donors. For more information on ...


[Livedo vasculitis].  


Twenty one patients with livedoid vasculitis are presented. Diagnosis was based on the clinical, histopathologic and direct immunofluorescent findings. The majority of patients were women (2:1), aged up to 30 years. Except for two patients with chronic venous insufficiency, the rest were with idiopathic-primary livedoid vasculitis. They all reacted favorably to the therapy with pentoxifylline, dipyridamole and acetylsalicylic acid. It is necessary to observe the difference to the "real" vasculitis of the skin, which requires more aggressive therapy. PMID:11548552

Zecevi?, R D


Pediatric Vasculitis  

PubMed Central

Synopsis Childhood vasculitis is a challenging and complex group of conditions that are multisystem in nature and often require integrated care from multiple subspecialties including rheumatology, dermatology, cardiology, nephrology, neurology, and gastroenterology. Vasculitis is defined as the presence of inflammation in the blood vessel wall. The site of vessel involvement, size of the affected vessels, extent of vascular injury, and underlying pathology determine the disease phenotype and severity. This review explores the classification and general features of pediatric vasculitis as well as the clinical presentation, diagnostic evaluation, and therapeutic options for the most common vasculitides.

Weiss, Pamela F.



Childhood vasculitis.  


Vasculitis can and does occur in childhood. Apart from the relatively common vasculitides (Henoch-Schönlein purpura, Kawasaki disease and in world wide terms Takayasu disease) there are a number of important but comparatively rare disorders affecting children. These include macroscopic and microscopic polyarteritis, cutaneous polyarteritis, Wegener's granulomatosis, Churg-Strauss syndrome, primary angiitis of the central nervous system, hypersensitivity angiitis, hypocomplimentaemic urticarial vasculitis, vasculitis associated with various connective tissue disorders and vasculitis associated with conditions such as Behçets syndrome, familial Mediterranean fever and Cogan's syndrome. Distinguishing these conditions from other disorders is often difficult and requires clinical acumen and appropriate investigative procedures. With modern therapeutic agents, it is possible to implement appropriate therapy but in spite of this, there remains a not inconsequential morbidity and mortality. PMID:9643315

Dillon, M J



Fortuitous vasculitis.  


A 43-year-old man with a cardiac device for dilated cardiomyopathy presented with fever, night sweats, and weight loss. Investigations revealed pancytopenia, acute renal failure, abnormal lung function, and raised inflammatory markers. A renal biopsy demonstrated pauci-immune necrotizing crescentic glomerulonephritis. He was diagnosed with pulmonary-renal antineutrophil cytoplasmic antibody-negative systemic small vessel vasculitis. He commenced immunosuppression with prednisolone and cyclophosphamide with recovery from pancytopenia and improvement in renal function 3 months later. Subsequently, a bone marrow culture grew Mycobacterium fortuitum. Isolation on repeat peripheral mycobacterial blood cultures prompted treatment with ciprofloxacin and clarithromycin. Four months later, he presented with neutropenic sepsis, influenza A/H1N1, and Aspergillus flavus pneumonia. Despite treatment he deteriorated. A transthoracic echocardiogram revealed a vegetation on the right ventricular pacing wire. The device was removed. The vegetation revealed acid and alcohol fast bacilli on Ziehl-Neelsen staining and grew M. fortuitum on culture, sensitive to ciprofloxacin and clarithromycin. Despite device removal and antimicrobial therapy, the patient succumbed to treatment-related complications. The association between glomerulonephritis and endocarditis is well known; however, this is the first case to our knowledge describing pauci-immune necrotizing crescentic glomerulonephritis in the context of M. fortuitum endocarditis. Clinicians should maintain a high index of suspicion for endocarditis in patients with a cardiac device who present with fever and pauci-immune necrotizing crescentic glomerulonephritis. Patients should be investigated with mycobacterial blood cultures, at least three sets of standard blood cultures and transthoracic and transesophageal echocardiography. Clinicians should beware the perils of immunosuppression in the face of an occult sepsis. PMID:22250755

Sharma, Hema; Keshavan, Ashvini; Little, Mark Alan; Cross, Jennifer; Lipman, Marc C; Talukdar, Sabrina; Hopkins, Susan



[Secondary vasculitides and vasculitis mimics].  


Secondary vasculitis is a form of vasculitis for which an underlying disease is known. Diseases associated with secondary vasculitis include infections, drug hypersensitivity, malignancy, rheumatoid arthritis, collagen vascular disease and sarcoidosis. Moreover, there are numerous conditions that can mimic vasculitis clinically, in laboratory testing, radiographically and in histopathology. It is evident that distinguishing primary vasculitis from secondary vasculitis and also vascular inflammation from non-vasculitic disorders (vasculitis mimics) has significant therapeutic implications. PMID:22956168

de Groot, K; Märker-Hermann, E



Vasculitis in Systemic Sclerosis  

PubMed Central

Systemic sclerosis (SSc) is a multiorgan connective tissue disease characterized by autoantibody production and fibroproliferative stenosis of the microvasculature. The vascoluopathy associated with SSc is considered to be noninflammatory, yet frank vasculitis can complicate SSc, posing diagnostic and therapeutic challenges. Here, we have reviewed the literature for reports of small-, medium-, and large-vessel vasculitis occurring in SSc. Amongst 88 reported cases of vasculitis in SSc, patients with ANCA-associated vasculitis appear to present a unique subclass in that they combined typical features of SSc with the renal manifestation of ANCA-associated glomerulonephritis. Other vasculitic syndromes, including large-vessel vasculitis, Behcet's disease, cryoglobulinemia, and polyarteritis nodosa, are rarely encountered in SSc patients. ANCA-associated vasculitis needs to be considered as a differential diagnosis in SSc patients presenting with renal insufficiency, as renal manifestations may result from distinct disease processes and require appropriate diagnostic testing and treatment.

Kao, Lily; Weyand, Cornelia



Nutritional Issues in Vasculitis  

Microsoft Academic Search

• Gastrointestinal involvement is common in systemic vasculitis.\\u000a \\u000a • Chronic systemic inflammation is common in vasculitis and frequently leads to weight loss and cachexia.\\u000a \\u000a \\u000a • Immunosuppressive medications can cause gastrointestinal toxicity.\\u000a \\u000a \\u000a • There is little evidence of any dietary manipulations or supplementations having any impact on the course of vasculitis.

Paul F. Dellaripa; Donough Howard


Central Nervous System Vasculitis  


... Vasculitis Foundation Fellowship is possible thanks to the generous support of Genentech, Inc., and Biogen Idec and many other generous individual and corporate donors. For more information on ...


Hypocomplementemic Urticarial Vasculitis Syndrome  

PubMed Central

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

Christensen, Jim; McCarty, Morgan



Primary cutaneous small vessel vasculitis  

Microsoft Academic Search

Opinion statement  Disorders associated with cutaneous vasculitis include numerous well-described etiologies. Primary cutaneous vasculitis limits\\u000a discussion to primary leukocytoclastic vasculitis, essential mixed cryoglobulinemia, urticarial vasculitis, Henoch-Schönlein\\u000a purpura, and erythema elevatum diutinum. Although the therapeutics for these disorders are based on limited data, we attempt\\u000a to construct a consensus opinion on the management of primary cutaneous vasculitis. Therapy of primary cutaneous vasculitis

James P. Russell; Roger H. Weenig



Panniculitis with vasculitis.  


Panniculitides encompass a great number of different entities; however, once a vasculitis has been detected histopathologically within the subcutaneous tissue, the differential diagnosis is mainly restricted to polyarteritis (panarteritis) nodosa (PAN), nodular vasculitis (NV), and Bazin's erythema induratum (EI). Patients with PAN may have the disease confined to the skin, but must be followed over a long period because many of them develop late systemic disease. The NV/EI group represents by far the most common type of lobular panniculitis with vasculitis; we prefer keeping the distinction between the two entities by underlining the equation NV positive tuberculin skin test = EI. Other lobular panniculitides with vasculitis are exceedingly rare and set in a systemic background which can be infectious (lepromatous leprosy panniculitides) or autoimmune/dysreactive (neutrophilic lobular panniculitis in rheumatoid arthritis, lobular panniculitis in inflammatory bowel disease). PMID:23900160

Ferrara, G; Stefanato, C M; Gianotti, R; Kubba, A; Annessi, G



Vasculitis: Classification and Management  

PubMed Central

Vasculitis, which is pathologically an inflammatory or necrotic state of blood vessels, consists of a diverse group of disesases. The clinical manifestations of vasculitis vary greatly in severity. The simplified classification described in this article is geared toward recognizing the degree of severity of the vasculitic process so that appropriate management can be initiated expeditiously. Emphasis is placed on the vasculitides likely to be encountered in general practice. ImagesFigure 1Figure 2Figure 3Figure 4Figure 5

Anastassiades, T.P.



Retinal vasculitis--a primer.  

PubMed Central

Retinal vasculitis is a diagnosis that is generally suggested by an ophthalmologist. Frequently patients with the disorder are referred to nonophthalmologists for further diagnostic evaluation or treatment. The criteria for defining vasculitis differ greatly between ophthalmologists and other physicians. To facilitate collaboration between ophthalmologists and their colleagues, we have sought to clarify the term "retinal vasculitis" by discussing its subcategories, the potential role of antiphospholipid antibodies, and the etiology of retinal vasculitis. We offer guidelines for evaluating the disorder and treating patients. Images

Rosenbaum, J. T.; Robertson, J. E.; Watzke, R. C.



Headache and vasculitis.  


Although headaches are common in the general population and have many causes, headaches secondary to inflammatory processes in the blood vessels in the Central Nervous System (CNS) are not so common. The most common types of vasculitis that are associated with headaches include primary CNS vasculitis, systemic necrotizing arteritis, granulomatous vasculitis, and systemic collagen diseases. It is important to differentiate between "true" vasculitides and a condition known and reversible cerebral vasoconstriction syndrome (RCVS). While treatment for many of the vasculitides consists of anti-inflammatory medications, this approach may produce significant complications in RCVS. It is up to the clinician to judiciously use imaging and laboratory data to reach the proper diagnosis and therefore offer the correct treatment to these patients. PMID:23338772

Lopez, J Ivan; Holdridge, Ashley; Chalela, Julio



Small vessel vasculitis and vasculitis confined to skin.  


Cutaneous vasculitis is a heterogeneous group of disorders, which can be confined to the skin or may be part of an associated systemic disease. Various aetiological agents as well as conditions that mimic skin vasculitis, usually present with similar clinical features; mainly palpable purpura. The skin biopsies usually show leukocytoclastic vasculitis. This poses a great diagnositc and therapeutic challenge for the physician. The aetiologies, clinical features, diagnosis and treatment modalities for each form (drugs, infections, malignancies, systemic vasculitides, connective tissue disorders. Schönlein-Henoch purpura, cryoglobulinaemia, cutaneous periarteritis nodosa, livedoid vasculitis, erythema elevatum diutinum and urticarial vasculitis) are reviewed. PMID:9220077

Mat, C; Yurdakul, S; Tüzüner, N; Tüzün, Y



Renal disease and vasculitis  

Microsoft Academic Search

Vasculitis is rare in childhood, apart from Henoch-Schönlein purpura, and paediatric nephrologists see few cases of Wegener's granuloma or microscopic polyarteritis. In contrast, this pattern is reversed in adults. Recently much information about the presentation and longer-term course of both these conditions has been described in adults, and more aggressive treatment of severe cases with renal failure has improved the

J. Stewart Cameron



Imaging in vasculitis.  


Vasculitides may involve small, medium-sized or large arteries. In small-vessel vasculitides imaging studies of lungs, nasal sinuses, cerebrum, heart and other organs are important for determining disease extension and disease activity. Aneurysms are a hallmark of medium-sized artery vasculitides. In large-vessel vasculitis, imaging studies depict homogeneous, circumferential wall swelling and smoothly tapered luminal narrowing. Ultrasound and high-resolution magnetic resonance imaging (MRI) show characteristic wall abnormalities in temporal and occipital arteries whereas ultrasound, MRI, MR angiography, computed tomography (CT), CT angiography and positron emission tomography delineate characteristic features in extra-cranial arteries that are affected in large-vessel giant cell arteritis, Takayasu arteritis and idiopathic aortitis. Conventional angiography has still its place for therapeutic interventions. Imaging has led to a better understanding of the nature and distribution of vasculitides. It significantly facilitates diagnosis of patients with suspected vasculitis. PMID:23507061

Schmidt, Wolfgang A



Imaging findings in pulmonary vasculitis.  


Vasculitis is a destructive inflammatory process affecting blood vessels. Pulmonary vasculitis may develop secondary to other conditions or constitute a primary idiopathic disorder. Thoracic involvement is most common in primary idiopathic large-vessel vasculitides (Takayasu arteritis, giant cell arteritis, Behçet disease) and primary antineutrophil cytoplasmic autoantibody-associated small-vessel vasculitides (Wegener granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome). Primary pulmonary vasculitides are rare, and their signs and symptoms are nonspecific, overlapping with those of infections, connective tissue diseases, and malignancies. The radiologic findings in primary pulmonary vasculitis vary widely and can include vessel wall thickening, nodular or cavitary lesions, ground-glass opacities, and consolidations, among others. Diffuse alveolar hemorrhage usually results from primary small-vessel vasculitis in the lungs. To diagnose vasculitis, medical teams must recognize characteristic combinations of clinical, radiologic, laboratory, and histopathologic features. PMID:23168065

Castańer, Eva; Alguersuari, Anna; Andreu, Marta; Gallardo, Xavier; Spinu, Cristina; Mata, Josep M



CMR in inflammatory vasculitis  

PubMed Central

Vasculitis, the inflammation of blood vessels, can produce devastating complications such as blindness, renal failure, aortic rupture and heart failure through a variety of end-organ effects. Noninvasive imaging with cardiovascular magnetic resonance (CMR) has contributed to improved and earlier diagnosis. CMR may also be used in serial evaluation of such patients as a marker of treatment response and as an indicator of subsequent complications. Unique strengths of CMR favoring its use in such conditions are its abilities to noninvasively visualize both lumen and vessel wall with high resolution. This case-based review focuses on the large- and medium-vessel vasculitides where MR angiography has the greatest utility. Because of increasing recognition of cardiac involvement in small-vessel vasculitides, this review also presents evidence supporting greater consideration of CMR to detect and quantify myocardial microvascular disease. CMR’s complementary role amidst traditional clinical, serological and other diagnostic techniques in personalized care for patients with vasculitis is emphasized. Specifically, the CMR laboratory can address questions related to extent and severity of vascular involvement. As ongoing basic and translational studies better elucidate poorly-defined underlying molecular mechanisms, this review concludes with a discussion of potential directions for the development of more targeted imaging approaches.



Neurological complications of vasculitis.  


The vasculitides are a group of disorders that include the polyarteritis nodosa group of systemic necrotizing vasculitides, hypersensitivity vasculitis, Wegener's granulomatosis, lymphomatoid granulomatosis, giant cell arteritis, Behçet's disease, and isolated angiitis of the central nervous system. Classification is based on clinical, angiographic, and histological features. The frequency and distribution of neurological involvement vary with the underlying disorder and may provide the initial symptoms. Polyarteritis nodosa and Wegener's granulomatosis may affect both the central and peripheral nervous systems, whereas isolated angiitis of the central nervous system and Behçet's disease affect the central nervous system alone. Neurological dysfunction occurs in 80% of patients with polyarteritis nodosa and fewer than 10% of patients with hypersensitivity vasculitis. The mechanism of neurological dysfunction in the vasculitides is tissue ischemia. The clinical effects of ischemia vary, and symptoms may be transient or prolonged. Mononeuritis multiplex, polyneuropathy, and stroke are frequent complications, but encephalopathies, cranial neuropathies, and brachial plexopathies are seen as well. The occurrence of symptoms late in the course of a disease suggests ischemia resulting from healed, scarred vessels as well as from those that are acutely inflamed; this is the case in Takayasu's arteritis and possibly also in polyarteritis nodosa. Treatment is based on identifying and removing the sensitizing agent when possible. Wegener's granulomatosis requires therapy with cyclophosphamide; temporal arteritis, with corticosteroids. In other vasculitides a balance must be reached between the progression of the disease and the side effects of immunosuppression. PMID:6137994

Moore, P M; Cupps, T R



Fertility and pregnancy in vasculitis.  


Despite the rarity of vasculitides, fertility and pregnancy outcome in the setting of vasculitis have become a major topic of interest within the past decade. The potential impact of vasculitis therapies, particularly cyclophosphamide, has been examined to some extent, but data are limited on the possible impact of the disease itself on fertility. Ideally, pregnancy should be planned when the vasculitis is in remission. The outcome for mothers and newborns is usually good when vasculitis is known before the pregnancy and is in remission, but every pregnant woman must be monitored by a specialised health-care team consisting of obstetricians specialised in high-risk births and internists/rheumatologists with expertise in managing these rare conditions. Most maternal complications during pregnancy are indeed due to vasculitis damage: hypertension in Takayasu arteritis (TAK) or granulomatosis with polyangiitis (GPA)/microscopic polyangiitis (MPA) with renal insufficiency, asthma or cardiac damage in eosinophilic granulomatosis with polyangiitis (EGPA) and subglottic and/or bronchial stenosis(es) in GPA. Pregnancy loss can occur in about 10% of cases in GPA, up to 20% in EGPA, 20-30% in Behçet's disease and up to 25% in TAK, and several studies found high rates of preterm births, at least with some vasculitides. Vasculitis manifestations in newborns from mothers with known vasculitis are very rare and usually transient. PMID:23507059

Pagnoux, Christian; Mahendira, Dharini; Laskin, Carl A



[Therapeutic study of intractable vasculitis].  


Vasculitis is a clinicopathological entity characterized by primary inflammation and necrosis of blood vessels. Vasculitis virtually involves any size or type of blood vessels in any organ systems. Within a category of vasculitis, there are several of the more serious vasculitides, such as polyarteritis nodosa, Wegener's granulomatosis and the systemic necrotizing vasculitides of the polyarteritis nodosa group are known to be extremely poor prognosis. However, early diagnosis and early treatment with steroid and/or immunosuppressant has been shown to prolong extraordinarily their survival. In this sense, appropriate agents where efficacy has been proven, such as corticosteroid in allergic granulomatous angiitis; cyclophosphamide in Wegener's granulomatosis should be chosen for the proper treatment, which in turn prolong survival of patients with serious vasculitis. PMID:7933606

Abe, T



Vasculitis of the nervous system  

Microsoft Academic Search

Vasculitis is inflammation of the blood vessels, which may involve either the central nervous system (CNS), or the peripheral\\u000a nervous system (PNS), or both. This involvement may be primary and restricted to the CNS, and rarely to the PNS. Vasculitis\\u000a is inflammation of the blood vessels, which may involve either the central nervous system (CNS), or the peripheral nervous\\u000a system

Aksel Siva



Damage assessment in systemic vasculitis  

Microsoft Academic Search

Systemic vasculitides were initially reported as acute, progressive, severe, and life-threatening diseases. The introduction\\u000a of glucocorticoids and cyclophosphamide for the treatment of vasculitis improved survival dramatically, but morbidity has\\u000a remained high. Damage develops as a consequence of recurrent or persistent active vasculitis or its treatment. It is defined\\u000a as the accumulation of nonhealing scars that are unlikely to respond to

Luis H. Silveira



Myelodysplasia and malignancy-associated vasculitis.  


While the link between malignancy and vasculitis has been known for some time, the association of vasculitis and myelodysplastic syndrome (MDS) has only recently been reported. This article reviews the most current and landmark publications regarding MDS, as well as malignancy-associated vasculitis. We include theories of paraneoplastic associations, immune pathogenesis including an associated cytokine transcriptional factor (interferon regulatory factor-1 [IFN-1]), and the relationship to treatment. Key clinical features that suggest underlying malignancy in patients with vasculitis are highlighted. Although the association between vasculitis and malignancy is rare, leukocytoclastic vasculitis is the most common vasculitis associated with MDS, hematologic malignancies as well as solid tumors. We review several articles that demonstrate a paraneoplastic association between vasculitis and various malignancies, but overall, the connection is still unclear and not well defined. Certain features that suggest a true paraneoplastic association are outlined. Further studies are needed to advance our understanding of this complex topic. PMID:22821200

Agha, Amir; Bateman, Helen; Sterrett, Ashley; Valeriano-Marcet, Joanne



Infectious etiology of vasculitis: Diagnosis and management  

Microsoft Academic Search

Vasculitides are a heterogeneous group of syndromes characterized by inflammation of the vessel wall. Several microbial pathogens\\u000a have been known or suspected to cause vasculitis, and the development of molecular biology has promoted the search and confirmation\\u000a of infectious agents in idiopathic vasculitis. Though several agents present primarily as an infectious process with vasculitis\\u000a as an occasional manifestation, vasculitis may

Niveditha Mohan; Gail Kerr



Cerebral vasculitis associated with cocaine abuse.  


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. We now include cocaine with methamphetamines, heroin, and ephedrine as illicit drugs that can cause cerebral vasculitis. PMID:3656626

Kaye, B R; Fainstat, M



Cutaneous vasculitis: a rheumatologist perspective.  


Cutaneous vasculitis (CV) comprises a wide spectrum of diseases and displays a variety of clinical lesions, the most common being a palpable purpura. CV may be a process confined exclusively to the skin or be a manifestation of a more widespread entity associated to a variable grade of visceral involvement. In this regard, CV may occur as part of the clinical spectrum of primary systemic vasculitis, autoimmune diseases or less commonly as presenting manifestation of cancer or severe infections. An adequate clinical approach is required to establish optimal management of this condition. PMID:23832592

Pina, Trinitario; Blanco, Ricardo; González-Gay, Miguel A



Prognosis of vasculitis associated myelodysplasia.  


Systemic and immune manifestations have been reported in patients with MDS. The correlation between immunological abnormalities and prognosis in myelodysplastic syndrome patients remains controversial. Most of the authors agree that the median survival in myelodysplastic syndrome is not related to the presence of systemic and immune manifestations, but only with the existence of a systemic vasculitis. PMID:23542504

Belizna, C; Subra, J F; Henrion, D; Ghali, A; Renier, G; Royer, M; Corre, Y Le; Martin, L; Voswinkel, J; Ifrah, N



Circulating Immune Complexes in Cutaneous Vasculitis  

PubMed Central

To investigate the pathogeneic significance of immune complexes in cutaneous vasculitis, 107 patients with various forms of cutaneous vasculitis, including 59 patients with necrotizing (leukocytoclastic) vasculitis (group 1), and 48 patients with lymphocytic vasculitis, or a predominately lymphocytic perivascular infiltrate (group 2), were studied. Immunoglobulins or complement components in cutaneous blood vessels were detected by direct immunofluorescence in high frequency in both groups (91 and 88%, respectively). Using two radioassays for circulating immune complexes, Clq or monoclonal rheumatoid factor (mRF) reactive material was detected in 68% of the patients with necrotizing vasculitis but only 44% of the patients in the lymphocytic-perivascular group. The mRF radioassay was elevated in 58% of the first group of patients and 41% of the patients in group 2, although Clq binding activity was increased in 54% of the patients with necrotizing vasculitis but only in 9% of the patients with a lymphocytic vasculitis or lymphocytic perivascular infiltrate. By using both sucrose density gradient ultracentrifugation and Sepharose 6B gel filtration, the Clq and mRF reactive material detected in some patients with necrotizing vasculitis eluted in high molecular weight fractions that were also anticomplementary. In one patient with necrotizing vasculitis and hepatitis B antigenemia, these heavy molecular weight Clq and mRF reactive fractions contained a two- to three-fold increase in hepatitis B surface antigen when compared with lighter molecular weight fractions. Heavy and light molecular weight mRF reactive material could be detected in selected patients in the lymphocytic-perivascular group as well as in the necrotizing vasculitis group. These studies suggest that cutaneous vasculitis, including acute necrotizing (leukocytoclastic) vasculitis and some forms of lymphocytic vasculitis, and perhaps some diseases characterized by a lymphocytic perivascular infiltrate, may represent cutaneous expressions of immune complex disease. Images

Mackel, Susan E.; Tappeiner, Gerhard; Brumfield, Hilton; Jordon, Robert E.



Leg ulcerations in livedoid vasculitis.  


Leg ulcerations associated with livedoid vasculitis may undergo a benign or very serious course depending on the presence of underlying disease. The disease has fairly distinctive clinical and histopathologic features, and it should be considered in cases of ulcerations refractory to conventional treatment. Most of these cases may eventually heal after a long course of intermittent eruptions and ulcerations. However, in rare instances, the loss of limbs may be unavoidable. PMID:3889951

Cabbabe, E B; Clift, S D



Silica exposure and systemic vasculitis.  

PubMed Central

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.

Mulloy, Karen B



Lessons from animal models of vasculitis  

Microsoft Academic Search

Vasculitis can occur as a primary disease or as a secondary manifestation of either another illness or a type-III hypersensitivity\\u000a response to a foreign antigen. Over the past four decades, a number of animal models of vasculitis have been described. These\\u000a models have served as important tools for enhancing our understanding of the basic mechanisms underlying the pathogenesis\\u000a of vasculitis.

Irina G. Luzina; Barry S. Handwerger



[Cutaneous leukocytoclastic vasculitis and renal carcinoma].  


The association between leukocytoclastic vasculitis and solid tumors like renal carcinoma has been infrequently described. We present the case of a 76-year-old woman who began with skin lesions on the legs that were clinically and histologically diagnosed as leukocytoclastic vasculitis. During analysis of the symptoms, she was diagnosed with renal carcinoma. After the tumor was excised, the vasculitis lesions disappeared, with no recurrence during the follow-up period. PMID:16801023

Hernández-Núńez, Almudena; Córdoba, Susana; Arias, Dolores; Romero, Alberto; García-Donoso, Carmen; Mińano, Román; Martínez, Diego; Borbujo, Jesús



Vasculitis in Sjögren’s Syndrome  

Microsoft Academic Search

Sjögren’s syndrome is a chronic autoimmune disease that is commonly manifested by immune attack on the exocrine glands with\\u000a resultant dry eyes and dry mouth. Sjögren’s syndrome patients also have disease in other organs. One of the most common extraglandular\\u000a manifestations is vasculitis. Skin vasculitis, with palpable purpura clinically and leukocytoclastic vasculitis on pathological\\u000a examination, is common. Although half of

R. Hal Scofield


Ischemic Neuropathy Associated with Livedoid Vasculitis  

PubMed Central

Background Livedoid vasculitis is a chronic dermatological problem with an unclear etiology. Clinical findings are petechiae with painful ulcers in both lower extremities, which heal to become hyperpigmented and porcelain-white satellite lesions. There are only a few reported cases of livedoid vasculitis presenting in combination with peripheral neuropathy. Case Report We report the first case of a Korean patient presenting with mononeuritis multiplex combined with livedoid vasculitis, which was confirmed by electrophysiological and pathological studies. Conclusions Our report supports the possible vaso-occlusive etiology of livedoid vasculitis in multifocal ischemic neuropathy.

Kim, Jee-Eun; Park, Su-Yeon; Sinn, Dong In; Kim, Sung-Min; Hong, Yoon-Ho; Park, Kyung Seok; Lee, Kwang-Woo



Goodpasture's syndrome associated with pulmonary eosinophilic vasculitis.  


Lung hemorrhage and antiglomerular basement membrane (anti-GBM) antibody mediated nephritis define Goodpasture's syndrome. We present the case of a 19-year-old Caucasian woman with unique clinical findings of Goodpasture's syndrome. Our patient initially presented with leukocytoclastic vasculitis of the skin followed by the development of nephritis and lung hemorrhage. An open lung biopsy done prior to diagnosing anti-GBM antibody disease demonstrated an intense eosinophilic vasculitis. Skin vasculitis has only been rarely reported, and to our knowledge this is the first reported case of pulmonary eosinophilic vasculitis associated with Goodpasture's syndrome. PMID:3184080

Komadina, K H; Houk, R W; Vicks, S L; Desrosier, K F; Ridley, D J; Boswell, R N



Retinal vasculitis with multifocal retinochoroiditis.  


We found a multifocal retinochoroiditis in 16 patients (13 female, 3 male). Patients also showed vitreous cells, in some cases pronounced retinal vasculitis, cystoid macular edema and papilledema. The patients ranged in age from 62 to 77 years. The anterior segment was involved in 13 cases. One patient demonstrated subretinal neovascularisation in both eyes. Fluorescein angiograms showed leakage from the retinal vessels, papilledema, macular edema and subretinal neovascularisation. A convincing classification of the described changes within any known disease entity was impossible. The findings most closely resembled a disease described by Dreyer and Gass in 1984 as 'multifocal choroiditis and panuveitis'. PMID:2249919

Schenck, F; Böke, W



Update on systemic necrotizing vasculitis.  


The systemic necrotizing vasculitides are classified into vasculitic syndromes on the basis of the pattern of clinical and pathologic involvement. The vasculitides have certain common clinical and laboratory abnormalities. Systemic necrotizing vasculitis is diagnosed on the basis of clinical features, and the vascular nature of the disease is determined by biopsy of involved tissue or angiography. The outcome is dependent on the extent of visceral involvement. Vascular inflammation influences the physiologic features of the vessel and may trigger vasoconstriction. Although glucocorticoids combat the inflammation, they may augment vasoconstriction and platelet aggregation. These effects must be considered in designing a management approach and in evaluating the cause and management of ischemic complications. PMID:2657234

Conn, D L



Isolated nail fold vasculitis in rheumatoid arthritis.  

PubMed Central

OBJECTIVES--To establish the clinical significance of isolated nail fold vasculitis in patients with rheumatoid arthritis (RA). METHODS--Patients attending hospital with established RA were assessed by a single observer using unaided vision. Isolated nail fold vasculitis (NFV) was diagnosed as small nail edge or nail fold lesions without any evidence of other extra-articular or systemic vasculitis. Patients were followed prospectively. RESULTS--Thirty patients with isolated NFV were identified and followed for a median interval of 22 months. Three patients died within three months of diagnosis and six developed complications possibly indicative of extra-articular disease: xerostomia (three), pericarditis (one), pleural effusion and empyaema (one), pulmonary fibrosis (one). No patient developed systemic vasculitis. CONCLUSION--Isolated NFV has a favourable prognosis compared with systemic vasculitis. There is a low risk of developing systemic or extra-articular disease.

Watts, R A; Carruthers, D M; Scott, D G



Vasculitis Associated With Tumor Necrosis Factor-? Inhibitors  

PubMed Central

Objective To describe the clinical characteristics, histopathologic features, and outcomes of patients in whom vasculitis developed in association with use of tumor necrosis factor-? (TNF-?) inhibitors. Patients and Methods This is a retrospective review of patients evaluated at Mayo Clinic, Rochester, Minnesota, from January 1, 1998, through March 31, 2011, with a diagnosis of vasculitis induced by anti–TNF-? therapy. Results Of 8 patients with vasculitis associated with anti–TNF-? therapy (mean age, 48.5 years), 6 (75%) were female. Four (50%) had rheumatoid arthritis, 1 (13%) had Crohn disease, and 3 (38%) had ulcerative colitis. Five (63%) were treated with infliximab, 2 (25%) with etanercept, and 1 (13%) with adalimumab. The mean duration of treatment before development of vasculitis was 34.5 months. The skin was the predominant organ affected (5 patients [63%]), with the most common cutaneous lesion being palpable purpura (4 of 5 [80%]). Two organs involved in systemic vasculitis were the peripheral nervous system (4 patients [50%]) and kidney (1 patient [13%]). All cases of vasculitis were histopathologically confirmed. Seven of 8 patients improved with discontinuation of therapy (mean time to resolution, 6.9 months) and adjuvant treatment (all 8 received prednisone; another agent was also used in 7); rechallenge with anti–TNF-? therapy was not attempted in any patient. At last follow-up, no patients had experienced a recurrence of vasculitis after therapy discontinuation. Conclusion Cutaneous small-vessel vasculitis was the most common finding, but systemic vasculitis, including peripheral nerve and renal vasculitis, was also frequently observed.

Sokumbi, Olayemi; Wetter, David A.; Makol, Ashima; Warrington, Kenneth J.



Biologic agents in systemic vasculitis  

PubMed Central

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-? 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.

Henderson, Charles F; Seo, Philip



Fulminant Vasculitis: A Rare Fatal Complication of Lymphoma  

Microsoft Academic Search

Vasculitis is rarely associated with lymphoma; however, most cases associated with lymphoma are cutaneous. Systemic vasculitis in association with lymphoma is usually an indolent and non-fatal complication. Two patients presented to our department with fulminant vasculitis with a fatal course and were later diagnosed with lymphoma. A search of the literature for systemic fulminant vasculitis in association with lymphoma disclosed

Eyal Leshem; Yaron Davidovitz; Eyal Meltzer; Paul Fefer; Efrat Ofek; Yechezkel Sidi



MRI and angiographic diagnosis of lupus cerebral vasculitis  

Microsoft Academic Search

The authors present a patient with striking angiographic findings of systemic lupus erythematosus (SLE) cerebral vasculitis. Although lupus is often cited as part of the differential diagnosis for angiographically apparent cerebral vasculitis, true SLE cerebral vasculitis is rare in pathological and radiological series. The clinical spectrum and pathological findings of CNS lupus with particular attention to cerebral vasculitis are briefly

M. D. Liem; D. J. Gzesh; A. E. Flanders



Retinal vasculitis in Toxocara canis neuroretinitis  

PubMed Central

Background The purpose of this case report is to describe clinical and angiographic findings of retinal vasculitis in acute Toxocara canis neuroretinitis associated with systemic infection. Findings A 16-year-old male presented with a 1 week history of left eye pain, floaters, and decreased visual acuity. Ocular examination was consistent with neuroretinitis and retinal vasculitis. Fluorescein angiography demonstrated leakage of fluorescein from the optic nerve and the retinal veins. Clinical and laboratory evaluation were consistent with systemic Toxocara canis infection. Conclusions Ocular T. canis may present with retinal vasculitis in young patients in the setting of acute systemic infection.



[Vasculitis and hereditary small vessel diseases].  


In younger patients with stroke, cerebral vasculitis and hereditary small vessel diseases should be considered as important differential diagnoses. Since the clinical course of cerebral vasculitis is highly variable, diagnostic workup, which includes laboratory tests, CSF analysis, cranial magnetic resonance imaging and biopsy, is often challenging. Therapy should be initiated on an interdisciplinary basis and includes immunosuppressive induction and maintenance regimes. Hereditary small vessel diseases, e.g. CADASIL or Fabry's disease, can mimic clinical features of cerebral vasculitis. Their diagnosis which is based on family history, typical clinical features and genetic analysis often has implications for treatment and genetic counselling. PMID:19838657

Opherk, C; Peters, N; Dichgans, M



Response of livedoid vasculitis to intravenous immunoglobulin.  


Livedoid vasculitis is a chronic condition characterized by recurrent painful ulceration of the lower limbs, which heals to leave atrophie blanche surrounded by hyperpigmentation and telangiectasia. We report two patients with livedoid vasculitis who, after failure of conventional therapies, responded to intravenous immunoglobulin (IVIg). There was healing of areas of active ulceration and improvement of erythema, swelling and pain. IVIg has been used successfully to treat a variety of vasculitic disorders and appears to be well tolerated. We suggest that this treatment is offered to patients who have livedoid vasculitis that is unresponsive to other therapies. PMID:12100203

Ravat, F E; Evans, A V; Russell-Jones, R



[Retinal vasculitis with multifocal chorioretinitis].  


Multifocal retinochoroiditis was diagnosed in 16 patients (13 female, 3 male). The patients also had cells in the vitreous, and in some cases pronounced retinal vasculitis, cystoid macular edema and papilledema. The patients' ages ranged from 62 to 77 years. There was anterior segment involvement in 13 cases. One patient had subretinal neovascularization in both eyes. Fluorescein angiograms revealed leaks from the retinal vessels, papilledema, and macular edema. So far, the authors have not succeeded in classifying the changes described as belonging to a known disease entity. The findings most closely resembled a disease described by Dreyer and Gass in 1984 as "multifocal choroiditis and panuveitis". Similar changes were seen by Tiedemann in 1987, in patients with Epstein-Barr viral antibodies. PMID:2290294

Schenck, F; Böke, W



Churg-Strauss vasculitis and ascaris infection.  

PubMed Central

A patient with Churg-Strauss vasculitis presenting with mononeuritis multiplex, who developed obstructive jaundice, is described. On investigation the jaundice proved to be due to ascaris infestation. As the immune abnormalities associated with ascaris infection are also typical of those seen in the Churg-Strauss syndrome it is speculated that the vasculitis occurred because of a failure to regulate the anti-ascaris immune response. Images

Chauhan, A; Scott, D G; Neuberger, J; Gaston, J S; Bacon, P A



Hansen's disease mimicking a systemic vasculitis.  


Hansen's disease, caused by Mycobacterium leprae, classically presents with cutaneous and neurological manifestations. Rheumatologic manifestations present in 1 to 5% of the patients, and include arthritis, arthralgias, Charcot arthropathy, erythema nodosum and vasculitis. We report a case of a 86 year old woman with polyarthritis, subcutaneous nodules and leg ulcers whose differential diagnosis included primary vasculitis and diffuse connective tissue diseases and ended to be leprosy in a non endemic country. PMID:21483282

Sampaio, L; Silva, L; Terroso, G; Pimenta, S; Brandăo, F; Pinto, J; Prisca, A; Brito, J; Ventura, F


Large artery vasculitis following recombinant hepatitis B vaccination: 2 cases.  


We describe 2 women who developed large artery vasculitis shortly after receiving recombinant hepatitis B vaccination. One patient developed Takayasu's arteritis, the other a vasculitis involving subclavian and renal arteries. Both developed renal failure. Whether the vasculitis was caused by the vaccination is not known. Although small vessel vasculitis following hepatitis B vaccination has been reported a number of times, large vessel vasculitis associated with hepatitis B vaccination has been reported only once. These cases suggest that large artery vasculitis should be added to the list of possible side effects of hepatitis B vaccination. PMID:11361200

Zaas, A; Scheel, P; Venbrux, A; Hellmann, D B



Cerebral rheumatoid vasculitis: a case report  

PubMed Central

Introduction Central nervous system involvement in rheumatoid arthritis is infrequent. The most frequent neurological manifestations of rheumatoid arthritis are peripheral neuropathy and cervical spinal cord compression due to subluxation of the cervical vertebrae. Cerebral rheumatoid vasculitis is an uncommon and serious complication which can be life-threatening. Case presentation A 52-year-old North African Tunisian Caucasian woman presented with a six-week history of headache. She had suffered seropositive and destructive rheumatoid arthritis for nine years without any extra-articular complications. Magnetic resonance imaging of the brain with the T2 sequence showed high-intensity signal images at the frontal and parietal cortico-subcortical junction suggesting hemispheric vasculitis. Conclusions Cerebral vasculitis is an infrequent complication in rheumatoid arthritis which is associated with high morbidity and in some cases can be life-threatening. Early assessment and a high index of suspicion to recognize such complications are essential in managing these patients.



Isolated testicular vasculitis mimicking a testicular neoplasm.  

PubMed Central

A 19 year old man presented with unilateral testicular swelling and pain. An initial diagnosis of epididymo-orchitis was modified to a presumed testicular neoplasm following ultrasonography. The final diagnosis of isolated testicular vasculitis was established following histological examination of the orchidectomy specimen. Staining for antineutrophil cytoplasmic antibodies was negative. Despite immunosuppressive treatment, the patient developed further symptoms affecting the remaining testis one year later. He responded well to an increase in immunosuppressive therapy and has remained asymptomatic 18 months from diagnosis. Symptomatic vasculitis confined to the testis is extremely rare, but must be considered in the differential diagnosis of testicular swelling and may be the presenting feature of a systemic vasculitis such as polyarteritis nodosa. The risk of progression to systemic disease in such cases is unknown. Immunosuppressive therapy must be considered carefully and long term follow up is important. Images

Warfield, A T; Lee, S J; Phillips, S M; Pall, A A



The vasculitis of atrophie blanche (livedoid vasculitis) and abdominal aortic pathology.  


Seven of forty-two patients with livedoid vasculitis whose cases were reviewed at the Mayo Clinic were found to have abdominal aortic disease, in the form of aortic calcification and atherosclerosis or aneurysm. None of these patients had any associated systemic mesenchymal disease (lupus erythematosus, periarteritis nodosa, rheumatoid arthritis, Sjögren's syndrome, or carcinomatous diseases). Three patients received graft replacements for aneurysm or vascular occlusive disease. Removal of the aneurysm in one case was associated with resolution of the livedoid vasculitis. Abdominal aortic changes should be considered in patients with diagnosed livedoid vasculitis. PMID:6488886

Schroeter, A L; Harris, R B



Antineutrophil Cytoplasmic Antibodies, Autoimmune Neutropenia, and Vasculitis  

PubMed Central

Objectives Reports of an association between antineutrophil cytoplasmic antibodies (ANCA) and autoimmune neutropenia have rarely included cases of proven vasculitis. A case of ANCA-associated vasculitis (AAV) with recurrent neutropenia is described and relevant literature on the association between ANCA, neutropenia, and vasculitis is reviewed. Methods Longitudinal clinical assessments and laboratory findings are described in a patient with AAV and recurrent episodes of profound neutropenia from December 2008 – October 2010. A PubMed database search of the medical literature was performed for papers published from 1960 through October 2010 to identify all reported cases of ANCA and neutropenia. Results A 49 year-old man developed recurrent neutropenia, periodic fevers, arthritis, biopsy-proven cutaneous vasculitis, sensorineural hearing loss, epididymitis, and positive tests for ANCA with specificity for antibodies to both proteinase 3 and myeloperoxidase. Antineutrophil membrane antibodies were detected during an acute neutropenic phase and were not detectable in a post-recovery sample, whereas ANCA titers did not seem to correlate with neutropenia. An association between ANCA and neutropenia has been reported in 74 cases from 24 studies in the context of drug/toxin exposure, underlying autoimmune disease, or chronic neutropenia without underlying autoimmune disease. In these cases, the presence of atypical ANCA patterns and other antibodies were common; however, vasculitis was uncommon and when it occurred was usually limited to the skin and in cases of underlying toxin exposure. Conclusions ANCA is associated with autoimmune neutropenia, but systemic vasculitis rarely occurs in association with ANCA and neutropenia. The interaction between neutrophils and ANCA may provide insight into understanding both autoimmune neutropenia and AAV.

Grayson, Peter C.; Sloan, J. Mark; Niles, John L.; Monach, Paul A.; Merkel, Peter A.



Primary systemic vasculitis: treatment of difficult cases.  


The primary systemic necrotizing vasculitides are a severe group of diseases, which untreated have a high mortality. The majority respond to treatment with high dose steroids and cyclophosphamide, however a significant proportion of those treated suffer morbidity due to the side effects of these agents, and a number of patients are refractory to treatment. We review and discuss alternative and emerging treatment options for patients who fail or cannot tolerate conventional therapy. An interesting subgroup of patients with systemic vasculitis and antiphospholipid antibodies provides an additional diagnostic and therapeutic challenge. We review what is known about this subgroup, and suggest screening for antiphospholipid antibodies in all patients with systemic vasculitis. PMID:16634367

Foster, R; Rosenthal, E; Marques, S; Vounotrypidis, P; Sangle, S; D'Cruz, D



Mononeuropathy multiplex in association with livedoid vasculitis.  


Livedoid vasculitis is a chronic dermatological disorder associated with petechiae and recurrent, unusually shaped ulcers that heal to form hyperpigmentated areas and atrophie blanche. This condition is more correctly termed a vasculopathy, rather than a vascultis, and is often associated with an underlying hypercoagulable disorder. We report a patient with livedoid vasculitis and mononeuropathy multiplex. We propose that peripheral nervous system involvement arises from multifocal areas of ischemia due to fibrin and thrombin deposition within both the wall and lumen of vasa nervorum. PMID:14571469

Toth, Cory; Trotter, Martin; Clark, Arthur; Zochodne, Douglas



Hypocomplementemic urticarial vasculitis syndrome in three siblings.  


Hypocomplementemic urticarial vasculitis syndrome (HUVS) is relatively uncommon and generally seen in the fourth decade of life. There are very few pediatric cases with the diagnosis of HUVS in the literature. In this report, we describe the first familial cases of HUVS in three siblings. The disease onset was during childhood period in all patients. One of them developed severe renal involvement and died. The other two had ongoing skin and eye manifestations and the elder one developed lupus. Presence of these three patients is a strong evidence for the role of genetic factors in the pathogenesis of this rare vasculitis. PMID:21113808

Ozçakar, Z Birsin; Yalç?nkaya, Fato?; Altugan, F Semsa; Kavaz, Asl?; Ensari, Arzu; Ekim, Mesiha



Plasmapheresis in a patient with "refractory" urticarial vasculitis.  


Immune complexes are found in the circulation of 30%-75% of patients with urticarial vasculitis and much evidence supports the role of these immune complexes in the pathogenesis of urticarial vasculitis. Plasmapheresis is effective for removing these immune complexes; however, there are few reports on the use of plasmapheresis in the treatment of urticarial vasculitis. We describe a case of "refractory" urticarial vasculitis in which the symptoms improved after plasmapheresis treatment. We suggest that plasmapheresis be considered as an option in patients with severe or treatment-resistant urticarial vasculitis. PMID:22754719

Kartal, Ozgur; Gulec, Mustafa; Caliskaner, Zafer; Nevruz, Oral; Cetin, Turker; Sener, Osman



Plasmapheresis in a Patient With "Refractory" Urticarial Vasculitis  

PubMed Central

Immune complexes are found in the circulation of 30%-75% of patients with urticarial vasculitis and much evidence supports the role of these immune complexes in the pathogenesis of urticarial vasculitis. Plasmapheresis is effective for removing these immune complexes; however, there are few reports on the use of plasmapheresis in the treatment of urticarial vasculitis. We describe a case of "refractory" urticarial vasculitis in which the symptoms improved after plasmapheresis treatment. We suggest that plasmapheresis be considered as an option in patients with severe or treatment-resistant urticarial vasculitis.

Gulec, Mustafa; Caliskaner, Zafer; Nevruz, Oral; Cetin, Turker; Sener, Osman



Pathology of ANCA-associated vasculitis.  


According to the recent 2012 Chapel Hill Consensus Conference, ANCA-associated vasculitis is classified in the group of small vessel vasculitis. Pauci-immune necrotizing crescentic glomerulonephritis is the morphological hallmark of ANCA-associated vasculitis. Crescentic damage of the glomerular tuft is characterized by macrophage accumulation through vascular cell adhesion molecule-1 (VCAM-1) activation. Macrophages have a key role in chronic progression of renal damage due to production of substances involved in matrix remodelling [transforming growth factor-beta (TGF?)]. Diffuse interstitial infiltration of T and B lymphocytes and macrophages is another frequent morphological feature. Tubulitis is considered an important marker of worse prognosis. Unfortunately, the renal changes on long-term follow-up are largely unknown due to the small number of studies with repeat biopsy in these disorders. Although a standardized score for renal biopsies was developed previously, a final histopathologic classification is still lacking. The European Vasculitis Study Group (EUVAS) proposed a classification system based on glomerular pathology as assessed by light microscopy. PMID:23180037

Ferrario, Franco; Vanzati, Alice; Pagni, Fabio



Propylthiouracil induced leukocytoclastic vasculitis: A rare manifestation  

PubMed Central

Propylthiouracil (PTU) is a common drug used in patients with hyperthyroidism. It may cause perinuclearantineutrophil cytoplasmic antibodies (p-ANCA) in few patients with Graves’ disease. This antibody has been associated with different forms of vasculitis. We report a patient who presented with cutaneous manifestations of leukocytoclasticvasculitis with simultaneous development of p-ANCAs during PTU therapy for Graves’ disease.

Ayturk, Semra; Demir, Mustafa Volkan; Yaylac?, Selcuk; Tamer, Ali



Paraneoplastic vasculitis and paraneoplastic vascular syndromes.  


Paraneoplastic syndromes are localized or diffuse pathologic manifestations that may occur in subjects affected by neoplastic diseases, even occult ones. Among the many clinical manifestations of paraneoplastic syndromes, cutaneous ones are quite common. It is estimated that skin manifestations may represent the very first diagnostic sign of a neoplastic disease in about 1% of patients. Many paraneoplastic syndromes with skin manifestations are caused by vascular alterations. In case of solid tumors, migrant thrombophlebitis and blood hypercoagulability can be seen, whereas in case of hematological neoplasms, vasculitis, and erythromelalgia can occur. Paraneoplastic vasculitis and paraneoplastic vascular syndromes are challenging issues in dermatology and general medicine. The present article will review the actual knowledge in the argument, together with providing hints for its diagnosis and management. PMID:21054706

Buggiani, Gionata; Krysenka, Alena; Grazzini, Marta; Vašk?, Vladimír; Hercogová, Jana; Lotti, Torello


Preliminary classification criteria for the cryoglobulinaemic vasculitis  

Microsoft Academic Search

BackgroundTo develop preliminary classification criteria for the cryoglobulinaemic syndrome or cryoglobulinaemic vasculitis (CV).MethodsStudy 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

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



Antiproteinase 3- and antimyeloperoxidase-associated vasculitis  

Microsoft Academic Search

Antiproteinase 3- and antimyeloperoxidase-associated vasculitis. Wegener's granulomatosis, microscopic polyangiitis, and idiopathic pauci-immune necrotizing crescentic glomerulonephritis (NCGN) are strongly associated with antineutrophil cytoplasmic autoantibodies (ANCAs) directed against either proteinase 3 (anti-PR3) or myeloperoxidase (anti-MPO). This has led some investigators to prefer combining these diseases under the common heading of ANCA-associated vasculitides. However, it is increasingly recognized that there are characteristic differences

Casper F. M. Franssen; Coen A. Stegeman; Cees G. M. Kallenberg; Reinold O. B. Gans; Paul E. De Jong; Steven J. Hoorntje; Jan Willem Cohen Tervaert



Biomarkers in ANCA-Associated Vasculitis.  


Despite recent advances in the treatment of antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV), relapse remains common and patients often experience a variable clinical course after initial treatment. New biomarkers are needed to aid the management of these complex diseases. Discoveries regarding the pathogenesis of AAV, from the importance both of activated B and T cells and the alternative complement pathway to genomic data, may lay the groundwork for identification of novel biomarkers. PMID:23955064

Lally, Lindsay; Spiera, Robert F



Cerebral vasculitis in a patient with rheumatoid arthritis  

Microsoft Academic Search

Inflammatory vasculitis of the central nervous system is exceedingly rare in patients with rheumatoid arthritis (RA). The symptoms may be misleading. Most of the reported cases occurred in males with long-standing, nodular, destructive, rheumatoid factor-positive disease. Severe constitutional symptoms and prominent extraarticular manifestations of vasculitis were usually present. We report a case of cerebral vasculitis in a 59-year-old woman with

Dalila Mrabet; Nihel Meddeb; Houda Ajlani; Hela Sahli; Slaheddine Sellami



Systemic antineutrophil cytoplasmic antibody vasculitis associated with lymphoid neoplasia  

PubMed Central

Two cases of systemic antineutrophil cytoplasmic antibody (ANCA) vasculitis in the setting of chronic lymphocytic leukaemia and angioimmunoblastic lymphadenopathy type T cell lymphoma are reported. The two patients had fever of unknown origin associated with cutaneous vasculitis and "pulmonary-renal syndrome" with alveolar haemorrhage. Despite anti-infectious treatments, steroids, and chemotherapy, the vasculitis had a fatal paraneoplastic course in several weeks. When infection is excluded in patients with malignancy, atypical features should be promptly investigated for systemic vasculitis, and an ANCA test performed.??

Hamidou, M; El Kouri, D; Audrain, M; Grolleau, J



Complement in antineutrophil cytoplasmic antibody-associated vasculitis.  


Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a group of autoimmune disorders. It was previously assumed that the complement system is not involved in the development of ANCA-associated vasculitis due to its "pauci-immune" feature in renal histology. However, increasing evidence indicates that activation of the complement system, especially via the alternative complement pathway, plays a crucial role in the pathogenesis of ANCA-associated vasculitis. In this brief review, we discuss the evidence, including in vivo, in vitro, and clinical studies, for complement system activation in ANCA-associated vasculitis. PMID:23180036

Yuan, Jun; Chen, Min; Zhao, Ming-Hui



Clinicopathologic study of 43 patients with sural nerve vasculitis  

Microsoft Academic Search

Vasculitis is a relatively uncommon finding in sural nerve biopsy specimens and is associated with significant morbidity. This study retrospectively reviewed the clinicopathologic features of 43 patients (44 sural nerve biopsy specimens) with sural nerve vasculitis, defined as infiltration of vessel walls by inflammatory cells. These biopsy specimens were obtained over a 19-year period, during which 1503 nerve specimens were

Richard A Prayson; David J Sedlock



Limited Cutaneous Vasculitis Associated With Levamisole-Adulterated Cocaine  

PubMed Central

Levamisole is among the many contaminants that have been detected in seized cocaine throughout North America and Europe. Little is known about the association between levamisole-adulterated cocaine and vasculitis. Herein we describe a case of limited cutaneous vasculitis manifested as retiform purpura and skin necrosis in a user of cocaine contaminated with levamisole.

Yachoui, Ralph; Kolasinski, Sharon L; Eid, Hala



The impact of vasculitis on patients' social participation and friendships  

PubMed Central

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 participant’s 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.

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



Renal involvement in HCV-related vasculitis.  


Renal involvement has been frequently reported in the setting of hepatitis C virus (HCV) infection. The most common renal pathology associated with chronic HCV infection is type I membranoproliferative glomerulonephritis associated with type II mixed cryoglobulinemia, while membranoproliferative glomerulonephritis without cryoglobulinemia and membranous nephropathy were less frequently reported. Rarely, focal segmental glomerulosclerosis, fibrillary and immunotactoid glomerulopathies, and thrombotic microangiopathies were described during the course of HCV infection. In the present review, we have focused on renal involvement in HCV-related vasculitis. PMID:23562337

Terrier, Benjamin; Cacoub, Patrice



Leukocytoclastic vasculitis caused by drug additives.  


Chronic cutaneous small vessel (leukocytoclastic) vasculitis (LCV) is a process believed to be related to the presence of circulating immune complexes. The most frequent causes and associated disorders are medications, infections, collagen vascular disorders, paraproteinemias, and, rarely, neoplasia. Reports of food or food additives as a causative factor for LCV have appeared but are rare. We report a patient with chronic cutaneous LCV in whom the presumed cause was an excipient (a dye) used in the capsule form of lithium carbonate. Furthermore, ingestion of foods containing dyes results in a disease flare in our patient. PMID:8169261

Lowry, M D; Hudson, C F; Callen, J P



Livedoid vasculitis: a manifestation of the antiphospholipid syndrome?  


Livedoid vasculitis, otherwise known as segmental hyalinizing vasculitis or livedo reticularis with summer ulceration, is a chronic disease with lesions affecting the feet and lower legs. Early lesions show petechiae, but characteristic features are recurrent, bizarrely shaped ulcers that heal to leave hyperpigmentation and atrophie blanche. The aetiology of the disorder is unknown, but the histology shows fibrin deposition within both the wall and lumen of affected vessels. The absence of a sufficient perivascular infiltrate or leucocytoclasia argues against a vasculitis, being more in keeping with a thrombo-occlusive process. Four patients with livedoid vasculitis with ulceration are described, all of whom had associated raised anticardiolipin antibodies but no other evidence of systemic disease. We suggest that livedoid vasculitis may be a manifestation of the antiphospholipid syndrome and recommend that all patients are screened for this. We also discuss treatment options for this often resistant condition. PMID:10215783

Acland, K M; Darvay, A; Wakelin, S H; Russell-Jones, R



Diagnosis and treatment of cerebral vasculitis  

PubMed Central

Vasculitides are characterized by inflammation and necrosis of the blood vessel wall. Large vessels including the aorta are affected in giant-cell arteritis, medium-size arteries in classic polyarteritis nodosa. The small-vessel vasculitides are separated in those with antineutrophil cytoplasm antibodies (ANCA) and those without. The primary angiitis of the central nervous system (PACNS) is a rare disorder affecting both medium- and small-sized vessels. Major symptoms of cerebral vasculitis are stroke, headache and encephalopathy. Diagnosis is based on laboratory and imaging findings. When cerebral affection occurs in systemic vasculitis an acute inflammatory response with raised erythrocyte sedimentation rate and increased values of C-reactive protein is present. In many cerebral vasculitides including PACNS, CSF studies reveal inflammatory findings. Magnetic resonance imaging, including ADC maps, diffusion and gradient echo sequences, is the investigation of choice to detect and monitor cerebral involvement. Certain MRI techniques and 18-fluorodeoxyglucose positron emission tomography allow the visualization of vessel wall inflammation when the lumen is still unaffected on angiography. The treatment recommendations for cerebral angitis are derived from protocols for systemic vasculitides. In general, a combination of steroids and pulse cyclophosphamide (CYC) is recommended for induction treatment. An alternative option is the use of the anti- CD20 antibody rituximab. Methotrexate, azathioprine and mycophenolate mofetil are recommended as alternatives to CYC once remission is achieved.

Berlit, Peter



Wünderlich syndrome from lupus-associated vasculitis.  


Spontaneous perinephric hemorrhage is a clinically rare life-threatening condition, also known as Wünderlich syndrome. Presentations can be variable, but patients typically display symptoms and signs including flank or abdominal pain, abdominal mass, and hypovolemia. It is important to diagnose the syndrome early because untreated cases carry a high mortality risk, and prompt intervention with an endovascular procedure or surgery is life saving. Causes range from anatomical anomalies, including vascular diseases (vasculitides and aneurysms) and renal tumors, to functional coagulation defect (bleeding tendency). The most common causes of Wünderlich syndrome are renal angiomyolipoma and renal cell carcinoma, constituting 60%~70% of cases. Vascular causes of Wünderlich syndrome are infrequent, and the culprit most frequently is vasculitis resulting from polyarteritis nodosa. Other vasculitides presenting as Wünderlich syndrome are infrequent. We describe a 39-year-old woman with end-stage renal disease from lupus nephritis and spontaneous renal hemorrhage, ascribed to lupus-related vasculitis after serologic testing, computed tomography, and angiographic studies. PMID:22981757

Chao, Chia-Ter; Wang, Wei-Jie; Ting, Jui-Tsung



Neoplastic and paraneoplastic vasculitis, vasculopathy, and hypercoagulability.  


It is essential to be aware of both neoplastic and paraneoplastic vasculitides, vasculopathy, and hypercoagulability, considering the importance of an accurate diagnosis and timely treatment of the underlying malignancy. Characteristics such as the type of vasculitis, age, gender, atypical presentation, and lack of response to common therapies should prompt investigation for an occult malignancy, whereas vasculitis such as GPA require due malignancy vigilance given a significantly increased risk of malignancy at the time of diagnosis and in the following years. Vasculopathies are rarer than vasculitides, but are associated with specific malignancies and, in the context of such malignancies, should be kept in mind. Hypercoagulability is a well-documented neoplastic phenomenon with an increased risk of thrombosis in the setting of positive aPLs. Most neoplastic and paraneoplastic vascular syndromes require no specific treatment outside of treatment of the underlying malignancy. The two key exceptions are PACNS, because of its poor prognosis, and erythromelalgia, in which aspirin is an effective agent. PMID:22075199

Park, Hyon Ju; Ranganathan, Prabha



Cochlear implants in systemic autoimmune vasculitis syndromes.  


The concept that autoimmunity may damage the inner ear was introduced by McCabe in 1979. Audiovestibular symptoms may occur in isolation or may be mediated by vasculitis in patients affected by systemic autoimmune disorders. Sensorineural hearing loss (SNHL) is typical in Cogan's syndrome but occurs less frequently in Beçhet's syndrome and in systemic necrotizing vasculitides. Patients affected by immune-mediated profound SNHL represent ideal candidates for cochlear implantation as these patients become deaf after years of hearing. The disease itself and the medication taken may, however, influence the prognosis of cochlear implantation in these patients. We retrospectively evaluated the pre- and intraoperative findings as well as the postoperative course and performance of a group of five patients affected by a systemic vasculitis syndrome who received a cochlear implant. Implantation was successful in all patients, no complications occurred and excellent postoperative speech perception was achieved. We conclude that cochlear implantation in patients affected by immune-mediated inner ear disorders is effective although the long-term results remain to be evaluated. PMID:12211357

Quaranta, Nicola; Bartoli, Roberto; Giagnotti, Francesca; Di Cuonzo, Franca; Quaranta, Antonio



Severe leukocytoclastic vasculitis secondary to the use of a naproxen and requiring amputation: a case report  

Microsoft Academic Search

INTRODUCTION: Leukocytoclastic vasculitis (also known as hypersensitivity vasculitis and cutaneous necrotizing vasculitis) can present with various manifestations, which often delays the diagnosis and treatment. In order to show the importance of the early recognition of leukocytoclastic vasculitis, we present a case which occurred secondary to the use of a common pharmaceutical, naproxen. We were unable to find a case of

Keri Brown; Jeanine Martin; Susan Zito



Cocaine Induced Vasculitis: Have We Found a Culprit?  

PubMed Central

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.

Sanchez-Cruz, Alfredo; Marrero, Sylmarie; Betancourt, Jose; Andino, Myrna; Lopez, Adolfo; Gutierrez-Nunez, Jose



Livedo reticularis and livedoid vasculitis responding to PUVA therapy.  


Livedo reticularis is a mottled blue discoloration of the skin, which occurs in a netlike pattern. Livedoid vasculitis is a chronic disorder clinically manifested by recurrent painful ulcerations of the lower extremities and is characterized by the presence of smooth or depressed ivory-white lesions surrounded by hyperpigmentation and telangiectasia. We describe two patients with livedo reticularis and livedoid vasculitis who responded to PUVA therapy and propose that systemic PUVA with methoxsalen undergo further investigation as an alternative therapy for drug-resistant patients with livedo reticularis and livedoid vasculitis. PMID:10025746

Choi, H J; Hann, S K



[The aspects of the problem of systemic vasculitis open to discussion].  


Some controversial problems in the diagnosis of systemic vasculitis are discussed. The importance of the morphological studies is assessed. The data are provided on the interrelations between skin and systemic vasculitis, on the existence of the local forms of vasculitis, the presence of the crisscross, mixed and combined forms of vasculitis, and on the possibility of the primary chronic disease course. The data obtained are based on the results of the prolonged observation over 325 patients suffering from systemic vasculitis. PMID:2697950

Shilkina, N P



[Renal disease in ANCA-associated vasculitis].  


Renal disease in ANCA-associated vasculitis (AAV) is characterized by a pauci-immune necrotizing glomerulonephritis. Renal biopsy is essential for diagnosis, treatment strategy and also for the long-term prognosis of AAV. The prospective randomized trials conducted by the EUVAS and GFEV have contributed to define therapeutic guidelines. The current standard of care can be divided into two phases: initial immunosuppression for rapid and effective onset of remission and subsequent maintenance therapy to prevent relapses. Despite these recent therapeutic advances, mortality and morbidity, such as end-stage renal failure, remain high, related to diagnosis delay, drug-related toxicity, refractory disease and propensity of AAV to relapse. Conventional therapies need to be optimized, especially in high-risk patients. Targeting B-cells with rituximab is a new and attractive therapeutic option, but long term benefits and safety are still unknown. PMID:22265953

Vanhille, Philippe; Vrigneaud, Laurence; Quéméneur, Thomas



[Secondary vasculitis to infection by Fasciola hepatica].  


There is a case of a 38 year-old male patient coming from an area where hepatic fascioliasis is endemic in Peru. He was hospitalized because he showed 4 weeks of symptoms like fever, intense myalgias, erythematous and painful injuries on limb extensions. The electromyography and nerve conduction velocity showed a global inflammatory myopathy. A skin biopsy showed polyarteritis nodosa-type vasculitis. During the evaluation process prior to the immunosuppressive therapy, hepatic Fasciola eggs were found in the parasitological examination of stools. The fascioliasis diagnosis was confirmed by fas2-ELISA: 0.46 (VN <0.20). Clinical symptoms started to subside after treatment with ticlabendazol. Contact with the patient was maintained for a year and there was no evidence of disease recurrence, and he was asymptomatic. PMID:23085802

Málaga, Germán; Taco-Palma, Rubén; Cáceres-Pizarro, Jaime; Lazo, María de los Angeles; Castaneda-Guarderas, Ana; Ticse, Ray


Kaposi's sarcoma following immunosuppressive therapy for vasculitis.  


Kaposi's sarcoma (KS) is widely reported to develop after renal transplantation and is induced by activation of a latent human herpes virus 8. We report the clinical features and outcome of a 50-year-old woman who presented with KS 18 weeks after starting immunosuppressive therapy for vasculitis. She had positive-titer IgG antibody to human herpes virus 8. Cyclophosphamide pulses were interrupted, and prednisone was decreased gradually to 10 mg/day. Skin lesions showed important regression with stabilization of the general state and renal function. Eight months later, the patient presented with a diffuse cutaneous KS that required the discontinuation of steroids. Within 1 month, her general status and renal function deteriorated, and she died with a disseminated intravascular coagulation syndrome. PMID:21422634

Bouattar, Tarik; Kazmouhi, Laila; Alhamany, Zaitouna; Beqqal, Kawtar; Haffane, Laila; Houssaini, Tarik Sqalli; Rhou, Hakima; Benamar, Loubna; Senouci, Karima; Bayahia, Rabia; Ouzeddoun, Naima



[Vasculitis : New nomenclature of the Chapel Hill consensus conference 2012].  


In recent years, many advances have been made in our understanding of vasculitis etiopathology as well as of different disease courses. The revised Chapel Hill Consensus Conference (CHCC) 2012 nomenclature reflects current knowledge about etiopathology, in addition to the descriptive principles of vessel size and type of inflammation. Anti-neutrophil cyptoplasmic antibody (ANCA)-associated vasculitides have been classified as a separate group, as opposed to immune complex small vessel vasculitis. In cases where consensus was achieved, eponyms have been replaced by systematic names, such as granulomatosis with polyangiitis (Wegener's) or eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). Moreover, clinically important but less well-known types of vasculitis have now been included in the CHCC nomenclature. This article presents the changes, focussing on those types that are relevant to the histopathologist, and summarizes the results of important new articles on morphology and clinical picture of vasculitis. PMID:24154756

Holl-Ulrich, K



The hairy-print for levamisole-induced vasculitis.  


Levamisole-induced vasculitis is a well-characterised antineutrophil cytoplasm antibodies (ANCA)-positive vasculitis in cocaine abuser patients. However, due to the short half-life of levamisole in serum and urine, the causal role of levamisole is not established. Here we report the detection of both levamisole and cocaine in hair samples of a patient who presented with an ANCA-positive vasculitis. The higher concentration of levamisole in proximal sample of the hair confirms that the patient abused of cocaine added with levamisole in the days preceding the development of skin lesions. Although a direct causative role has not been established, our report strongly suggests that levamisole may have triggered vasculitis in this case. PMID:22879002

Lazareth, Hélčne; Peytavin, Gilles; Polivka, Laura; Dupin, Nicolas



Vasculitis Syndromes of the Central and Peripheral Nervous Systems  


... Systems Information Page Synonym(s): Temporal Arteritis, Cranial Arteritis, Giant Cell Arteritis Condensed from Vasculitis Syndromes of the ... better understood syndromes are: temporal arteritis (also called giant cell arteritis or cranial arteritis), Primary angiitis of ...


Clinicopathologic study of 43 patients with sural nerve vasculitis.  


Vasculitis is a relatively uncommon finding in sural nerve biopsy specimens and is associated with significant morbidity. This study retrospectively reviewed the clinicopathologic features of 43 patients (44 sural nerve biopsy specimens) with sural nerve vasculitis, defined as infiltration of vessel walls by inflammatory cells. These biopsy specimens were obtained over a 19-year period, during which 1503 nerve specimens were reviewed. The study group comprised 29 females and 14 males, ranging in age from 19 to 94 years (mean, 72.5 years) at the time of biopsy. The most frequent clinical presentations included paresthesias in 19 patients (61%), pain in 17 patients (57%), weakness in 10 patients (32%), and weight loss in 9 patients (29%). Histologically, 26 of 44 biopsy specimens (59%) demonstrated necrotizing vasculitis. The remaining 18 biopsy specimens demonstrated a nonnecrotizing lymphocytic vasculitis. Eosinophils were identified in 4 biopsy specimens, intravascular thrombi in 10 (22%) specimens, and granulomatous inflammation in 1 specimen. In 39 biopsy specimens (89%), multiple vessels were involved by vasculitis. Epineurial vessels were the most common target of vasculitis, (n = 42; 95%). Evidence of vascular wall scarring, indicative of healed vasculitis, was observed in 13 biopsy specimens (30%). All biopsy specimens showed evidence of axonopathy, with mild axonal loss noted in 14 specimens (32%), moderate loss in 18 specimens (41%), and severe loss in 12 specimens (27%). Concomitant muscle biopsy was performed in 31 patients. Fifteen muscle biopsy specimens demonstrated evidence of vasculitis (48%), which was necrotizing in 11 cases. All muscle biopsy specimens demonstrated features of neurogenic atrophy. Twenty-five out of 32 patients were known to have been treated with steroids and demonstrated some degree of clinical improvement. In conclusion, sural nerve vasculitis is a relatively uncommon pathological finding (prevalence of 2.9% in this study). In most cases, multiple vessels were involved. Concomitant vasculitis was also identified in about half of the muscle biopsy specimens obtained at the time of nerve biopsy. In most patients, the vasculitis appeared to be at least partially responsive to immunosuppressive therapy. PMID:12792923

Prayson, Richard A; Sedlock, David J



Severe food-induced vasculitis in two children.  


Food-induced vasculitis seems to be rare and is considered by some as controversial. The reported cases in the literature are few and mostly on adult patients. Described in this report are two children with severe vasculitis caused by specific foods. They were diagnosed at two separate allergy centers that have a special interest in food allergies. Case 1 was an 8-year-old girl with a 9-month history of cutaneous vasculitis with large joints involvement. Case 2 was a 23-month-old girl with an 8-month history of multiple hospitalizations for recurrent acute severe cutaneous and mucous membrane vasculitis with large joints involvement. In both patients, skin biopsy showed leucocytoclastic vasculitis. In neither of the patients could the symptoms be attributed to drug intake, infection, autoimmunity, or other systemic disease. Case 1 had a moderately elevated serum total immunoglobulin E (IgE) level and strongly positive skin test and radioallergosorbent test (RAST) to cow's milk and hen's egg, both of which were proven to be the cause by elimination-challenge tests. Case 2 had a slightly elevated serum total IgE level, but negative skin tests to foods, including chocolate that was suspected by the mother. Avoidance of chocolate resulted in remission, except following accidental ingestion of cocoa-containing products. These findings support the few previous reports on food-induced vasculitis, an entity that seems rare but may be more common than currently realized. PMID:12000502

Businco, Luisa; Falconieri, Paola; Bellioni-Businco, Barbara; Bahna, Sami L



Urinary neopterin in idiopathic retinal vasculitis  

PubMed Central

AIMS—To determine whether urinary neopterin:creatinine (UNC) ratios relate to disease activity in idiopathic retinal vasculitis (RV).?METHODS—18 patients with RV were prospectively recruited into a year long longitudinal study. Patients collected first morning urine samples on a weekly basis and on the same day completed a diary which documented their subjective view of RV activity and any concurrent infection. They were examined in clinic on a 6-8 weekly basis and an objective assessment was made of RV disease activity. 14 healthy controls collected urine samples in the same way.?RESULTS—UNC ratios were significantly higher in patients than in controls (p=0.004, Mann-Whitney U test). UNC ratios were significantly higher when, according to their diaries, the patients had a subjective flare up of RV (p=0.001, Mann-Whitney U test). Subjective increased RV activity occurred more often when the patients had a concurrent infection (p<0.0001, ?2 test). There was no significant difference in the UNC ratio between objective clinical relapse and non-relapse of RV. There was moderate agreement between the clinical assessment and patients' subjective impression of RV activity (?=0.48).?CONCLUSIONS—Higher neopterin levels reflect cell mediated disease that occurs in RV, but UNC ratios are not recommended as a means of monitoring clinical disease activity in RV.??

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



Long- term outcome of paediatric patients with ANCA vasculitis  

PubMed Central

Background Primary systemic vasculitis presenting in childhood is an uncommon but serious condition. As these patients transfer to adult clinics for continuing care, defining long term outcomes with emphasis on disease and treatment- related morbidity and mortality is important. The aim of this study is to describe the long- term clinical course of paediatric patients with ANCA vasculitis. Methods The adult patients in our vasculitis clinics who had presented in childhood, with a follow up time of greater than 10 years were included. We also reviewed the literature for articles describing the clinical outcome of paediatric patients with ANCA vasculitis. Results We describe the clinical course of 8 adults who presented in childhood with ANCA vasculitis. 7 patients had Wegener's granulomatosis and 1 had microscopic polyangiitis. The median age at presentation was 11.5 years, and follow up time ranged form 11 to 30 years. Induction therapy for all patients was steroids and/or cyclophosphamide. Maintenance therapy was with azathioprine or mycophenolate mofetil. Biological agents were used in 3 patients for relapsed disease in adulthood only. Seven patients achieved complete remission. All patients experienced disease relapse, with a median of 4 episodes. Kidney function was generally well preserved, with median eGFR 76 ml/min. Only one patient developed end-stage renal failure and one patient died after 25 years of disease. Treatment-related morbidity rates were high; 7 suffered from infections, 4 were infertile, 2 had skeletal complications, and 1 developed malignancy. Conclusion Close long- term follow up of paediatric patients with ANCA vasculitis is imperative, as this patient cohort is likely to live long enough to develop significant treatment and disease- related morbidities. Prospective cohort studies with novel therapies including paediatric patients are crucial to help us determine the best approach to managing this complex group of patients. In addition, although not yet observed in our series, late cardiovascular morbidity remains a major longer-term potential concern for adult survivors of paediatric vasculitis.



Shedding new light on vasculitis: how the LAMP story unfolded.  


The last couple of years have brought some major advances both in our understanding of antineutrophil cytoplasmic antibody (ANCA)-positive vasculitis pathogenesis mechanisms and in its treatment options. Recent discoveries of completely new antigens such as lysosome-associated membrane protein-2 (LAMP-2) have meant a huge step forward, and the fact that this antigen is homologous to proteins of bacterial fimbria caused a shift in the focus regarding underlying pathomechanisms of ANCA vasculitis toward bacterial infections, mainly with Klebsiella or Escherichia species, possibly playing a role in triggering the disease. So nephrology has seen real progress in understanding of glomerulonephritis disease mechanisms - not only regarding primary membranous glomerulonephritis (with the recent identification of the phospholipase A2 receptor being the underlying antigen) but also regarding secondary pauci-immune glomerulonephritis due to ANCA-positive vasculitis. PMID:22684653

Rothe, Hansjörg


Isolated rheumatoid arthritis-associated cerebral vasculitis: a diagnostic challenge.  


Central nervous system involvement in rheumatoid arthritis is uncommon. In order of frequency, published cases have reported rheumatoid nodules, meningeal vasculitis, and cerebral vasculitis (CV). The frequency of vasculitic cerebral involvement in rheumatoid arthritis is unknown. However, it is known that it is more common in patients with seropositive, long-standing rheumatoid arthritis, and in those with concomitant systemic vasculitis. We report the case of a 47-year-old woman with an 11-year history of seropositive rheumatoid arthritis without any extra-articular manifestations, with the exception of secondary Sjogren's syndrome, presenting with mental status changes and tonic-clonic seizures as symptoms of isolated CV. Magnetic resonance imaging (T2) showed hyperintense white-matter lesions in the frontal and temporal lobes, as well as in the hippocampus and cerebellum. Transcranial Doppler ultrasound findings were consistent with CV. Other differential diagnoses were ruled out. The patient responded favorably to methylprednisolone and intravenous gammaglobulin therapy. PMID:22858148

Guadalupe Loya-de la Cerda, Denisse; Avilés-Solís, Juan Carlos; Delgado-Montemayor, María José; Camara-Lemarroy, Carlos Rodrigo; Galarza-Delgado, Dionicio Ángel



Endoscopic and radiographic features of gastrointestinal involvement in vasculitis  

PubMed Central

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-Schönlein purpura, systemic lupus erythematosus, and Behçet’s 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.

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



Active necrotizing cerebral vasculitis in systemic lupus erythematosus.  


Systemic lupus erythematosus (SLE) is a multisystemic disease with varied clinical manifestations. Focal cortical brain infarcts and CNS infections are the most common neuropathological features reported in most studies. This report describes a 32-year-old woman who had repeated episodes of strokes over 5 years. In view of polyarthritis, oral ulcers, presence of high titres of serum antinuclear antibodies, high titres of double-stranded DNA and strokes, she was treated as SLE. Despite prolonged immunosuppressive therapy with azathioprine and pulse cyclophosphamide, she succumbed to a brainstem stroke. Complete body autopsy showed multiple cerebral cortical and brainstem infarcts with fibrinoid necrosis of the vessel wall. Renal infarction with healed vasculitis and systemic vasculitis involving small vessels was seen. Extensive thrombosis was remarkable by its absence. Active necrotizing vasculitis of cerebral and renal vessels is a rare complication of SLE, which contributed to a fatal outcome in this patient. PMID:18021377

Goel, Deepa; Reddy, S Rajashekhar; Sundaram, Challa; Prayaga, Aruna K; Rajasekhar, Liza; Narsimulu, Gumdal



Does vasculitis alone cause AVN? A review of literature.  


AVN is caused by a disease, or severe trauma that affects the blood supply to the bone or in many cases may be idiopathic, with no known cause. AVN pathophysiology is most closely linked to SLE literature, and there is a strong cause and effect relationship between corticosteroid intake and AVN development in SLE patients, and AVN is extremely rare in the absence of steroid use. Apart from few anecdotal reports, there is no data on exact pathophysiologic mechanisms responsible for AVN in the setting of vasculitis. We saw a 69-year-old man with femoral AVN and a possibility of vasculitis as the underlying cause was raised by the radiologist, and hence we present this literature search on vasculitis per se causing AVN of the bone. PMID:23011085

Abraham, Rtika R; Meyerhoff, John O



Long term outcome of paediatric patients with ANCA vasculitis  

Microsoft Academic Search

Background  Primary systemic vasculitis presenting in childhood is an uncommon but serious condition. As these patients transfer to adult\\u000a clinics for continuing care, defining long term outcomes with emphasis on disease and treatment- related morbidity and mortality\\u000a is important. The aim of this study is to describe the long- term clinical course of paediatric patients with ANCA vasculitis.\\u000a \\u000a \\u000a \\u000a \\u000a Methods  The adult patients

Nishkantha Arulkumaran; Susan Jawad; Stuart W Smith; Lorraine Harper; Paul Brogan; Charles D Pusey; Alan D Salama



Leukocytoclastic Vasculitis as an Onset Symptom of Crohn's Disease  

PubMed Central

We report the case of an octagenerian who presented with leukocytoclastic vasculitis as the first symptom of Crohn's disease. The patient was admitted with skin rash on the lower extremities and ankles and episodes of bloody diarrhea. Skin and colon biopsies revealed acute leukocytoclastic vasculitis and moderate Crohn's disease, respectively. The patient was treated with intravenous corticosteroids in conjunction with antibiotics and per os mesalazine. Symptoms resolved rapidly within 5 days, and the patient was still asymptomatic on follow-up 3 months later.

Tsiamoulos, Z.; Karamanolis, G.; Polymeros, D.; Triantafyllou, K.; Oikonomopoulos, T.



Vasculitis as the basis of cutaneous lesions in Reiter's disease  

Microsoft Academic Search

The cutaneous lesions of Reiter's disease (RD) and pustular psoriasis (PP) are said to be histologically similar and often indistinguishable. We encountered three cases of RD in which biopsy specimens of lesions clinically compatible with keratoderma blenorrhagicum showed a pustular psoriasiform tissue reaction in conjunction with a subjacent superficial leukocytoclastic vasculitis (LCV). In an attempt to ascertain if these changes

C. M Magro; A. N Crowson; R Peeling



Ocular disease in patients with ANCA-positive vasculitis  

PubMed Central

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

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



Central nervous system vasculitis after chickenpox--cause or coincidence?  

Microsoft Academic Search

A 7.5 year old boy, known to have a seizure disorder, presented with an infarct in the left middle cerebral artery territory, 10 weeks after severe chickenpox. Immunofluorescent antibody titre to the varicella zoster virus in the cerebrospinal fluid was 1:32. Cerebral angiography showed evidence of focal vasculitis. He presented again seven months later with an acute exacerbation of seizures.

A Shuper; E P Vining; J M Freeman



Cutaneous vasculitis and IgA glomerulonephritis in ankylosing spondylitis.  

PubMed Central

Two patients with ankylosing spondylitis were found to have IgA nephropathy and leucocytoclastic cutaneous vasculitis. Immunofluorescence showed perivascular deposition of IgA in the skin of one patient and in the mesangium of both patients. Such an association has been reported only once before. This supports the concept of abnormal IgA immune stimulation in the pathogenesis of ankylosing spondylitis.

Beauvais, C; Kaplan, G; Mougenot, B; Michel, C; Marinho, E



The spectrum of vasculitis: clinical, pathologic, immunologic and therapeutic considerations.  


Vasculitis is a clinicopathologic process characterized by inflammation and necrosis of blood vessels. Certain disorders have vasculitis as the predominant and most obvious manifestation, whereas others have various degrees of vasculitis in association with other primary disorders. Within the entire spectrum of vasculitis virtually any size or type of blood vessel in any organ system can be involved. Most of the vasculitides can be associated directly or indirectly with immunopathogenic mechanisms. In this regard, immune complex mediation is being increasingly recognized as the underlying mechanism in several of the vasculitides. With clinical, pathologic, and immunologic criteria, certain vasculitic disorders can be clearly recognized and categorized as distinct entities, whereas in others there is an overlap of different diseases within a broader category. In recent years, several of the more serious vasculitides, such as Wegener's granulomatosis and the systemic necrotizing vasculitides of the polyarteritis nodosa group, which formerly had extremely poor prognoses, have been shown to be extraordinarily responsive to chronic low-dose cytotoxic therapy, particularly cyclophosphamide. PMID:31121

Fauci, A S; Haynes, B; Katz, P



Cutaneous leukocytoclastic vasculitis in the presence of methimazole therapy*  

PubMed Central

Treatment with antithyroid drugs may be accompanied by side effects. We present a patient diagnosed with Grave's Disease who developed extensive vasculitis in the lower limbs during methimazole use. After suspension of the methimazole and the introduction of prednisone in immunesupressor doses the cutaneous lesions started to involute.

Ribeiro, Carla de Oliveira; Magrin, Paula Ferrazzi; Vilar, Enoi Aparecida Guedes; Duraes, Sandra Maria Barbosa; Estrella, Rogerio Ribeiro



Autoantibodies to Vascular Smooth Muscle Are Pathogenic for Vasculitis  

PubMed Central

We have previously shown that microvascular smooth muscle activates CD4+ T lymphocytes in sterile co-culture, presents antigen, and produces inflammatory cytokines. Adoptive transfer of lymphocytes co-cultured with syngeneic smooth muscle cells to healthy recipient mice results in vasculitic lesions predominantly in postcapillary venules. The present study assessed the pathogenic role of immunoglobulin and B cells in a murine model of vasculitis. Here, we show that transferred B cells, including plasmablast cells, accumulated, persisted, and proliferated in lung and secondary lymphoid organs of recipient mice. The induction of vasculitis was accompanied by production of IgM and IgG2a autoantibodies specific for vascular smooth muscle intracellular antigens. Circulating immunoglobulin had a pathogenic role in this vasculitis model, because the disease could be induced by transfer of serum from vasculitic mice to untreated animals but not by transfer of serum depleted of anti-smooth muscle autoantibodies. Additionally, the pathogenic mechanisms triggered by the transfer of vasculitogenic serum were dependent on T lymphocytes because both wild-type and B cell-deficient mice developed the disease after serum transfer, whereas RAG2-deficient mice did not. Thus, immunoglobulin and cell-mediated pathways work in concert to produce vasculitis in this model.

Baiu, Dana Carina; Barger, Brittany; Sandor, Matyas; Fabry, Zsuzsa; Hart, Michael Noel



Progress in treatment of ANCA-associated vasculitis  

PubMed Central

Autoantibodies to neutrophil cytoplasmic antigen-associated vasculitis (AAV) is characterised by inflammation of blood vessels. The introduction of immunosuppressive therapy with glucocorticoids and cyclophosphamide transformed AAV from a fatal condition to a largely treatable condition. Over the past 30 years, considerable progress has been made refining immunosuppressive regimens with a focus on minimising toxicity. There is, however, a high unmet need in the treatment of AAV. A proportion of patients are refractory to current therapies; 50% experience a relapse within 5 years and treatment toxicity contributes to mortality and chronic disability. As knowledge of the pathogenesis of vasculitis grows, it is mirrored by the availability of biological agents, which herald a revolution in the treatment of vasculitis. Lymphocyte-targeted and cytokine-targeted agents have been evaluated for the treatment of AAV and are entering the routine therapeutic arena with the potential to improve patient outcomes. As rare diseases, treatment advances in vasculitis depend on international collaborative research networks both to establish an evidence base for newer agents and to develop recommendations for patient management.



The median arcuate ligament syndrome: a mimicker of mesenteric vasculitis.  


The median arcuate ligament syndrome is an uncommon condition characterized by the triad of postprandial abdominal pain, unintentional weight loss, and an epigastric bruit. This condition is diagnostically challenging and patients often undergo extensive laboratory, radiographic, and invasive evaluations before it is identified. Physicians should consider this syndrome in the differential diagnoses of chronic abdominal pain and mesenteric vasculitis. Once diagnosed, treatment is generally surgical with known predictors of favorable and unfavorable outcomes. Surgical candidates should be selected carefully. We describe the cases of two young active duty patients diagnosed with median arcuate ligament syndrome after suffering from chronic abdominal pain. Both were referred to our rheumatology department to evaluate for mesenteric vasculitis. Each had a different therapeutic outcome. PMID:23929065

Kay, Johnson C; Arroyo, Ramon A



Treatment of retinal vasculitis in Behçet's disease with rituximab.  


Behçet's disease (BD) is more common in eastern than western countries. Physicians have frequently encountered problems in its treatment, especially eye involvement. Recurrent oral and genital aphthous ulcerations are the hallmarks of Behçet's disease but other organs can be involved and ocular disease is one of the most disabling manifestations. Up to now, there are some problems in treatment of the retinal vasculitis due to Behçet's disease. We reported one patient, with visual loss due to retinal vasculitis that was resistant to prednisolone and azathioprine. Our patient was treated successfully with rituximab and his remission was sustained for 24 months of follow-up. Rituximab is a chimeric monoclonal antibody that acts against the specific B cell antigen, CD20. The recent success of rituximab in autoimmune diseases, which is considered to be T cell-mediated, indicates that B cells must have a much broader role in the pathogenesis of autoimmune diseases than generally appreciated. PMID:18438602

Sadreddini, Shahram; Noshad, Hamid; Molaeefard, Mahshid; Noshad, Rahim



Immunoregulatory gene polymorphisms are associated with ANCA-related vasculitis  

Microsoft Academic Search

T cell activation is regulated by inhibitory molecules such as PD-1 and CTLA-4, whose expression may be affected by gene polymorphisms. Increased T cell activation is present in patients with ANCA-associated vasculitis (AAV). We investigated two single-nucleotide polymorphisms (SNPs) in PDCD1 and five polymorphisms in CTLA4 in 102 patients with AAV and 188 healthy controls (HC). The distributions of the

Marjan C. Slot; Milena G. Sokolowska; Kim G. Savelkouls; Rob G. J. H. Janssen; Jan G. M. C. Damoiseaux; Jan Willem Cohen Tervaert



Hypocomplementemic urticarial vasculitis: report of a pediatric case  

Microsoft Academic Search

Hypocomplementemic urticarial vasculitis syndrome (HUVS) is well described in adults but is quite rare in children. We report\\u000a a pediatric case of HUVS initially diagnosed as juvenile rheumatoid arthritis and then as Henoch-Schnlein purpura. Beginning\\u000a at 3 years of age, our patient developed polyarthritis with hypocomplementemia. She subsequently experienced an intermittent\\u000a purpuric rash beginning at age 4 years, and she

Melissa A. Cadnapaphornchai; Frank T. Saulsbury; Victoria F. Norwood



[Livedoid vasculitis in a patient with antiphospholipid syndrome].  


The authors present a clinical case of a 30 year old male patient admitted to the hospital for recurrent cyanosis and feet pain lasting for one year. In addition he presented bilateral purpuric lesions in the lateral maleolar region, one of which with ulceration. The finding of anticardiolipin antibodies associated with intraluminal thrombosis in the dermal vessels, allowed to conclude for Antiphospholipid syndrome. The cutaneous changes identified are named livedoid vasculitis. PMID:20711098

Serra, Sara; Saavedra, M Joăo; Salvador, M Joăo; Reis, J Pedro; Malcata, A


Small vessel vasculitis as first manifestation of paracoccidioidomycosis - case report.  


Paracoccidioidomycosis is a systemic disease caused by inhalation of conidia of Paracoccidioides brasiliensis, a dimorphic fungus that initially affects the airway, spreading by lymphatic and hematogenous routes to various organs and systems, and that can be fatal if the diagnosis and treatment are not correct. The authors describe a patient who presents small vessel vasculitis as an initial manifestation of the disease, which is an atypical clinical form with a single description in the literature. PMID:22281916

Caldeira, Alessandra Cristiana de Barros Figueiredo; Cas, Kelly da; Pinto, Taili Pedroso Lemes; Zômpero, Camila Machado; Pés, Cacinele; Guolo, Cristian Eduardo


Livedoid vasculitis and central nervous system involvement in systemic lupus erythematosus.  


Of 66 patients with systemic lupus erythematosus (SLE), 11 (16.7%) had livedoid vasculitis. The sites of predilection included the elbows, knees, fingers, soles, and back, which are not those of common livedoid vasculitis. Livedoid vasculitis developed in ten (90.9%) of the 11 patients with SLE who eventually developed central nervous system (CNS) lupus. In contrast, such preceding livedoid vasculitis occurred in only three (11.5%) of the 26 patients with severe nephrotic lupus nephritis. Livedoid vasculitis did not occur in any patient with neither CNS lupus nor severe lupus nephritis. These results indicate that livedoid vasculitis in SLE is a very important cutaneous manifestation that may possibly be prodromal to CNS lupus. PMID:3942409

Yasue, T



Remarks on the Asia Pacific Meeting of Vasculitis and ANCA Workshop 2012.  


An international meeting, The Asia Pacific Meeting of Vasculitis and ANCA Workshop 2012, was held on March 28-31, 2012 at Shinagawa, Tokyo, Japan. The meeting focused on vasculitis and anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis in the Asia-Pacific area. This was a high-profile event and attracted scientists and physicians from around the world, all of whom are committed to the care of patients with vasculitis, ANCA and related diseases. The Asia Pacific international meeting was able to extend insights into Kawasaki disease, Takayasu arteritis, and myeloperoxidase ANCA-associated vasculitis. The program addressed the multidisciplinary nature of vasculitis, including genomics, genetics, pathogenesis, epidemiology, biomarkers, clinical features, and therapeutic trials. PMID:23615782

Suzuki, Kazuo



Clinical features and outcomes of ANCA-associated renal vasculitis.  


To determine the patterns and outcomes of the pauci-immune vasculitis in the nephrology department at hospital La Conception in Marseille, we conducted a retrospective study including all patients with diagnosis of pauci-immune renal vasculitis between January 1, 2000 and December 31, 2007. Among 33 cases, 25 were diagnosed as Wegener granulomatosis (WG), seven as microscopic polyangitis (MPA) and one as Churg-Strauss syndrome (SCS). The median age of the patients was 57.7 years and the sex-ratio (M/F) was 1.6. The visceral manifestations included kidneys (100% of patients), lungs (75%), ENT (52% of WG), and nervous system (57% of MPA). The mean serum creatinine at admission was 3.3 mg/dL. Renal biopsies revealed a pauci-immune crescentic gromerulonephritis in 96% of the cases. Two patients with WG received plasmapheresis and seven patients required emergency hemodialysis. Induction therapy comprised cyclophosphamide IV and corticosteroids, while maintenance therapy included azathioprine for the majority of patients. Eighty four percent of the patients experienced complete remission after induction therapy. During maintenance therapy relapses were more frequent among patients with MPA (28%) compared to WG cases (12%). After 35 months of follow-up, eight patients ended on chronic hemodialysis, and five patients died. ANCA associated vasculitis are frequent in our patients. Long-term outcomes are relatively good despite a mortality rate of 15% and 25% of the patients entering dialysis after three years of follow-up. PMID:22382223

Seck, Sidy Mohamed; Dussol, Bertrand; Brunet, Philippe; Burtey, Stephane



Cutaneous Small Vessel Vasculitis Accompanied by Pustulosis Palmaris et Plantaris  

PubMed Central

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

Kosaka, Motoko; Kato, Tokue; Kawana, Seiji



Tissue plasminogen activator for treatment of livedoid vasculitis.  


Livedoid vasculitis, a hyalinizing vasculopathy, is characterized by extensive formation of microthrombi and deposition of fibrin in the middermal vessels, which result in epidermal infarction, ulceration, and formation of stellate scars. In a prospective study of nonhealing ulcers in patients with livedoid vasculitis, we found a high incidence of anticardiolipin antibodies, lupus anticoagulants, increased levels of plasminogen activator inhibitor, and low levels of endogenous tissue plasminogen activator (t-PA) activity. This procoagulant tendency and decreased fibrinolysis may provide an explanation for the occlusive vasculopathy often noted in biopsy specimens from these patients. On the basis of these findings, we proposed that fibrinolysis with recombinant t-PA would lyse microvascular thrombi, restore circulation, and promote wound healing. In six patients who had nonhealing ulcers caused by livedoid vasculitis and in whom numerous conventional therapies had failed, low-dose t-PA (10 mg) was administered intravenously during a 4-hour period daily for 14 days. Five of the six patients had dramatic improvement; almost complete healing of the ulcers occurred during hospitalization, and tissue oxygenation, as measured by transcutaneous oximetry, increased. The one treatment failure was due to rethrombosis of the microvasculature; this patient was subsequently re-treated but with concurrent anticoagulation, and her leg ulcers healed. We conclude that daily administration of a low dose of t-PA is safe and effective treatment for nonhealing ulcers due to occlusive vasculopathy. PMID:1434852

Klein, K L; Pittelkow, M R



Recurrence of ANCA-associated vasculitis following renal transplantation in the modern era of immunosupression  

Microsoft Academic Search

Progressive glomerulonephritis and attendant end-stage renal disease (ESRD) result from antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. The optimum time of kidney transplantation in patients with ESRD due to ANCA-associated vasculitis (AAV) and the risk of renal or nonrenal recurrence of vasculitis after transplantation are unknown. To answer some of these questions, we followed 35 transplant recipients with diagnoses of microscopic polyangiitis

M Gera; M D Griffin; U Specks; N Leung; M D Stegall; F C Fervenza



Recognizing calcific uremic arteriolopathy in autoimmune disease: An emerging mimicker of vasculitis  

Microsoft Academic Search

Calcinosis has long been associated with autoimmune disease and has a distinctive profile in scleroderma, dermatomyositis, systemic lupus erythematosus, and overlap syndromes. However, there have also been a number of case studies of calcific uremic arteriolopathy, or calciphylaxis, described within vessels, including patients with chronic renal insufficiency and several forms of vasculitis. Interestingly, the calciphylaxis associated with vasculitis appears to

Jennifer L. Lee; Gurtej Cheema; M. Eric Gershwin



Vasculitis complicating treatment with intravenous anisoylated plasminogen streptokinase activator complex in acute myocardial infarction.  

PubMed Central

Vasculitis developed in six of 253 patients treated with intravenous anisoylated plasminogen streptokinase activator complex (APSAC) after acute myocardial infarction. All patients recovered spontaneously with no evidence of renal impairment and no long term sequelae. Although leucocytoclastic vasculitis and serum sickness have been reported after streptokinase treatment, such allergic reactions have not been described as a complication of other thrombolytic agents. Images Figure

Bucknall, C; Darley, C; Flax, J; Vincent, R; Chamberlain, D



Refractory livedoid vasculitis responding to PUVA: a report of four cases.  


Livedoid vasculitis is a chronic disease characterized by recurrent painful irregularly shaped ulcers, which heal with scars, most commonly located on feet or lower extremities. This condition is often resistant to the therapy. We report four cases with refractory livedoid vasculitis that responded to systemic psoralens and ultraviolet A radiation therapy. PMID:15888134

Tuchinda, C; Leenutaphong, V; Sudtim, S; Lim, H W



Livedo (livedoid) vasculitis and the factor V Leiden mutation: additional evidence for abnormal coagulation.  


We report the case of a patient with livedo vasculitis associated with the factor V Leiden mutation. This association provides additional support for abnormalities of coagulation in patients with this disorder. The spectrum of platelet, coagulation, and fibrinolytic disorders reported with livedo vasculitis is reviewed. PMID:11756961

Calamia, Kenneth T; Balabanova, Maria; Perniciaro, Charles; Walsh, John S



Performance of the BioPlex 2200 Autoimmune Vasculitis kit.  


The BioRad BioPlex 2200 Vasculitis kit demonstrates excellent relative sensitivity and relative specificity for the semi-quantitative detection of IgG autoantibodies to MPO, PR3 and GBM. The fully-automated platform simultaneously measures three analytes in a single tube, offering superior advantage in speed and ease of use over current assays. The availability of a fully-automated platform with 24-hour availability for these three antibodies may be of considerable value in the differential diagnosis of patients with rapidly progressive glomerulonephritis. PMID:18718876

Kaul, Ravi; Johnson, Kim; Scholz, Heidi; Marr, Greg



Leukocytoclastic vasculitis presenting with acute haemorrhagic oedema of the scrotum  

PubMed Central

Fournier’s gangrene is a form of rapidly progressive necrotising fasciitis involving the soft tissues of the male genitalia. The treatment for this urologic emergency is immediate surgical debridement. Misdiagnosis of Fournier’s may lead to unnecessary surgical interventions, making careful recognition of systemic signs of illness crucial. The authors present a case of a 48-year-old patient who presented emergently with massive scrotal oedema, erythema and pain suspicious for Fournier’s gangrene and systemic signs of illness, including palpable purpura and leukocytoclastic vasculitis on biopsy.

Reyner, Karina; Dahm, Philipp



587 Plasmapheresis in a Patient with "Refractory" Urticarial Vasculitis  

PubMed Central

Background Immune complexes have been found in the circulation approximately 30 to 75% of patients with urticarial vasculitis and much evidence supports the role of these immune complexes in the pathogenesis of urticarial vasculitis. Plasmapheresis is effective in removal of these immune complexes. However, few cases have been reported regarding the use of plasmapheresis in the treatment of urticarial vasculitis. Methods A 35-year-old woman presented with history of recurrent episodes of generalized painful urticarial plaques often lasting 9 years associated with swelling of her parts of body. Examination revealed multiple urticarial plaques distributed all over the body (particularly in the extremities, palms and soles). The initial laboratory studies, including a complete blood count, thyroid function tests - thyroid autoantibodies, erythrocyte sedimentation rate, hepatitis markers, liver and renal function tests, urinary analysis, stool analysis for parasite ova, total IgE, C3, C4, C1q, CH50, C1 inhibitor levels and antinuclear antibodies were found to be within normal range. Skin prick tests were performed with commonly consumed foods in Turkey found to be negative. A biopsy from an affected area of skin revealed an urticarial vasculitis. Based on the biopsy results, the patient was diagnosed with UV. Treatment with H1/ H2-antihistamines and oral corticosteroids (1 mg/kg/day) had been unsuccessful; therefore hydroxychloroquine 400 mg/day was added. Unfortunately hydroxychloroquine was stopped in the second month due to the emergence of an adverse event (keratopathy). The patient underwent plasma exchange 2 times with an interval of 6 months. Five percent albumin solution as replacement fluid was used. One plasma volume was processed in each session. Apheresis procedure was performed with the “Cell Separator” device. The plasmapheresis procedures were completed without any adverse events. At 13 months after the plasmapheresis, the urticarial plaques were reappeared, but the severity and duration of symptoms were lower than before the plasmapheresis. The newly lesions were re-treated with short-term oral antihistamine regimen. Conclusions In conclusion, the presented report supports the usability of plasmapheresis in patients with “refractory” UV. Further clinical studies are needed to confirm our experience.

Kartal, Ozgur; Gulec, Mustafa; Caliskaner, Zafer; Nevruz, Oral; Cetin, Turker; Sener, Osman



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


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. PMID:11413776

Falk, R J



Leflunomide-induced DRESS syndrome with renal involvement and vasculitis.  


DRESS or drug reaction (or rash) with eosinophilia and systemic symptoms belongs to the severe cutaneous adverse reaction group and is characterized by hematological abnormalities and visceral organ involvement. Although most often related with anticonvulsant and sulfonamide use, it is reported with numerous other drugs. We report an unusual case of DRESS syndrome due to Leflunomide, also complicated by renal involvement in the form of granulomatous interstitial nephritis and vasculitis. On a review of the literature, eight similar cases were found, and these are discussed. PMID:23271613

Pinto, Benzeeta; Dhir, Varun; Krishnan, Sabari; Nada, Ritambhra



Three-phase abdominal scintigraphy in lupus vasculitis of the gastrointestinal tract.  


Gastrointestinal vasculitis is a recognized but frequently misdiagnosed complication of systemic lupus erythematosus. The authors investigated the usefulness of three-phase Tc-99m pyrophosphate abdominal scintigraphy for identifying areas of vasculitis in patients with systemic lupus erythematosus who have gastrointestinal symptoms (diarrhea or abdominal pain). Among patients with gastrointestinal symptoms, 62 of 82 (75%) patients showed positive scan results, suggesting gastrointestinal vasculitis. Among patients without gastrointestinal symptoms, 6 of 43 (13%) patients showed positive scan results. Although three-phase Tc-99m pyrophosphate abdominal scintigraphy cannot provide a definite diagnosis of vasculitis of the gastrointestinal tract, it is a noninvasive and convenient method for suggesting and monitoring gastrointestinal vasculitis. PMID:7586871

Wang, S J; Lan, J L; Lin, W Y; Liu, R S; Yeh, S H



Antineutrophil cytoplasmic antibody vasculitis associated with influenza vaccination.  


Background: Administration of influenza vaccines has been associated with the development of autoantibodies and autoimmune rheumatic disease. Patients: We discuss 2 patients who developed antineutrophil cytoplasmic antibody-associated vasculitis (AAV) in temporal association with influenza immunization. AAV was diagnosed 2 and 4 weeks after immunization in these patients. Both patients had renal involvement with one requiring dialysis. Both patients were treated with cyclophosphamide and corticosteroids, and plasmapheresis was added to the immunosuppressive regimen in one patient with dialysis-dependent renal failure. Both patients achieved disease remission. The patient with initial dialysis-dependent renal failure reached end-stage renal disease. There are 6 previous cases of AAV in the literature described in temporal association with administration of influenza vaccines. Conclusion: A causal role of vaccines in AAV cannot be confirmed with these case reports. The temporality suggests that the influenza vaccine may be a triggering factor for induction of vasculitis in predisposed individuals. We review the literature on reported cases of AAV following influenza vaccine administration and discuss possible mechanisms for influenza vaccine-associated AAV. PMID:23941822

Duggal, Tanu; Segal, Paul; Shah, Megha; Carter-Monroe, Naima; Manoharan, Pradeep; Geetha, Duvuru



TLR4 and TLR5 induce distinct types of vasculitis  

PubMed Central

Large vessel vasculitides, such as Takayasu arteritis and giant cell arteritis (GCA), affect vital arteries and cause clinical complications by either luminal occlusion or vessel wall destruction. Inflammatory infiltrates, often with granulomatous arrangements, are distributed as a panarteritis throughout all of the artery’s wall layers or cluster in the adventitia as a perivasculitis. Factors determining the architecture and compartmentalization of vasculitis are unknown. Human macrovessels are populated by indigenous dendritic cells (DC) positioned in the adventitia. Herein, we report that these vascular DC sense bacterial pathogens and regulate the patterning of the emerging arteritis. In human temporal artery-SCID chimeras, lipopolysaccharides stimulating Toll-like receptor (TLR) 4 and flagellin stimulating TLR5 trigger vascular DC and induce T-cell recruitment and activation. However, the architecture of the evolving inflammation is ligand specific; TLR4 ligands cause transmural panarteritis and TLR5 ligands promote adventitial perivasculitis. Underlying mechanisms involve selective recruitment of functional T cell subsets. Specifically, TLR4-mediated DC stimulation markedly enhances production of the chemokine CCL20, biasing recruitment towards CCL20-responsive CCR6+ T cells. In adoptive transfer experiments,CCR6+ T cells produce an arteritis pattern with media-invasive T cells damaging vascular smooth muscle cells. Also, CCR6+ T cells dominate the vasculitic infiltrates in patients with panarteritic GCA. Thus, depending on the original danger signal, vascular DC edit the emerging immune response by differentially recruiting specialized T effector cells and direct the disease process toward distinct types of vasculitis.

Deng, Jiusheng; Ma-Krupa, Wei; Gewirtz, Andrew T.; Younge, Brian R.; Goronzy, Jorg J.; Weyand, Cornelia M.



Antineutrophil cytoplasmic antibody--a useful serological marker for vasculitis.  


Systemic necrotizing vasculitides, including polyarteritis nodosa, Churg-Strauss syndrome, "overlap" systemic vasculitis, Wegener's granulomatosis, and idiopathic crescentic glomerulonephritis, are frequent clinical diagnostic problems. These diseases have diverse presentations and are often rapidly progressive, causing irreversible injury to the vessels of the kidneys and lungs before effective immunosuppressive therapy is instituted. Even in their less fulminant forms, they are a cause of significant morbidity and mortality. Antineutrophil cytoplasmic antibody, a recently identified autoantibody, has a high sensitivity and specificity for this spectrum of diseases. The clinical and pathological similarities, the high frequency of antineutrophil cytoplasmic antibody expression, and the similar good response to immunosuppressive therapy suggest that these diseases may be linked by a common pathophysiological mechanisms. Evidence is growing that antineutrophil cytoplasmic antibody plays a central role in this mechanisms. A revision in the classification scheme of vasculitides to recognize that the polyarteritis group (polyarteritis nodosa, Churg-Strauss syndrome, and "overlap" systemic vasculitis), Wegener's granulomatosis, and idiopathic crescentic glomerulonephritis are closely related diseases may be warranted. The clinical and pathological features of systemic necrotizing vasculitides and the current knowledge concerning antineutrophil cytoplasmic antibodies are reviewed. PMID:1918264

Goeken, J A



Myocardial Ischemia in Wegener's Granulomatosis: Coronary Atherosclerosis Versus Vasculitis  

PubMed Central

Wegener’s granulomatosis (WG) is one of the most common small- and medium-sized necrotizing vasculitides that mainly affects the upper and lower respiratory tract and the kidneys. Cardiac manifestations in WG are relatively rare, and their role and place among different causes of mortality remain largely unknown. Substantially increased number of reports describing involvement of all structures of the heart, which underlie conduction disturbances, valvular disease, ischemic heart disease and other potentially serious conditions, underscores importance of comprehensive cardiovascular investigations and monitoring of patients with WG. The majority of previous reports and our current observation distinguish coronary vasculitis and thrombosis as a cause of myocardial ischemia and cardiovascular co-morbidities in WG. It seems plausible that inflammatory processes in this disease, like in some other systemic vasculitidies, do not predispose to accelerated atherogenesis. However, characteristic small- and medium-sized vasculitis still can manifest as myocardial ischemia and infarction. We overview diverse cardiac manifestations and present our own rare case of angina in the oligosymptomatic debut of WG. Importantly, in this case, coronarography failed to reveal atherosclerotic disease or thrombotic occlusion. However, magnetic resonance imaging (MRI) with adenosine test revealed subendocardial ischemia. As a result of immunosuppressive therapy with a steroid and cyclophosphamide, myocardial ischemia disappeared.

Cocco, Giuseppe; Gasparyan, Armen Yuri



[Intractable vasculitis: general consideration-concept and classification: pathological aspects].  


Vasculitis may be defined as an inflammatory process, primarily affecting the vascular wall which possesses an architecture, such as organ or tissue, and accordingly may not include the capillary. Since the walls are destroyed, secondary circulatory disturbances may be associated and produce vasculitic symptoms, which are nonspecific in most instances. Although the pathogenesis of most vasculitides is well understood, the lack of knowledge of the etiology makes it difficult to establish a satisfactory classification of vasculitis. The inflammatory lesions tend to be distributed along the course of the vessels throughout the body in most systemic vasculitic syndromes but long segments are frequently affected in the arteritides of large arteries. In recent years, a classification, useful for understanding the clinical manifestations of these vasculitic disorders, is based on the predominant size of the involved vessels, which may be categorized into large arteries (aorta and its primary branches), medium-sized to small muscular arteries and small vessels (arterioles and venules). Certain vasculitides may occur as a primary disease process, whereas others may be associated with other primary disorders. In general, idiopathic primary vasculitides have an extremely poor prognosis and are intractable. However, recent advances of drug therapies in the early stage of the systemic necrotizing vasculitides, such as Wegener's granulomatosis, has bettered prognosis. In the patients with Takayasu's arteritis, life has been prolonged over more than ten years. As a result, additional problems, such as luminal stenosis or dilatation of the involved vessels, have occurred. PMID:7933570

Hotchi, M



[Necrotizing vasculitis as a manifestation of hypersensitivity to propylthiouracil].  


Propylthiouracil (PTU), a thyonamide class drug commonly used to treat hyperthyroidism has been reported to cause adverse reactions in 3% to 12% of patients. The side effects have been described more frequently as mild, but ocasionally severe fatal reactions may occur. We report the case of a fourteen years old patient in use of PTU for the last three years who presented with fever, hemorrhagic blisters, necrotic ulcers, and that developed purpuric lesions and nodules in lower extremities. Laboratory and histopathologic findings were compatible with skin leukocytoclastic vasculitis, a pattern found in hypersensitivity reaction vasculitis. Suspension of PTU and introduction of prednisone, induced complete remission of symptoms and healing of the skin lesions. The importance of this study is to call attention to the occurrence of serious cutaneous manifestation with a mortality rate that might reach 10%, associated with a systemic drug frequently used in internal medicine. Early diagnosis and withdrawal of the suspected medication is mandatory. Administration of corticosteroids and/or immunosuppressives agents must be considered. PMID:19578601

Dinato, Sandra Lopes Mattos e; Dinato, Mauro Cesar; Dinato, Marcelo Mattos e; Ribeiro, Marcela Castro M; Yamashiro, Cintia Yukimi; Romiti, Ney



MRI in human immunodeficiency virus-associated cerebral vasculitis.  


Cerebral ischaemia caused by inflammatory vasculopathies has been described as complication of human immunodeficiency virus (HIV) infection. Imaging studies have shown ischaemic lesions and changes of the vascular lumen, but did not allow demonstration of abnormalities within the vessel wall itself. Two HIV-infected men presented with symptoms of a transient ischaemic attack. Initial MRI of the first showed no infarct; in the second two small lacunar lesions were detected. In both cases, multiplanar 3-mm slice contrast-enhanced T1-weighted images showed aneurysmal dilatation, with thickening and contrast enhancement of the wall of the internal carotid and middle cerebral (MCA) arteries. These findings were interpreted as indicating cerebral vasculitis. In the first patient the vasculopathy progressed to carotid artery occlusion, and he developed an infarct in the MCA territory, but then remained neurologically stable. In the second patient varicella zoster virus (VZV) infection was the probable cause of vasculitis. The clinical deficits and vasculitic MRI changes regressed with antiviral and immunosuppressive therapy. PMID:10952187

Berkefeld, J; Enzensberger, W; Lanfermann, H



Refractory anemia as a presenting feature of microscopic polyangiitis: a rare vasculitis in children.  


Microcytic anemia refractory to usual supplementation is an important clue to an alternative diagnosis. Accompanying pulmonary and renal involvement during the disease course suggests a multisystem disease. Small-vessel vasculitis should be suspected in any patient who presents with a multisystem disease that is not caused by an infectious or malignant process. Among these, antineutrophilic cytoplasmic antibodies (ANCA)-associated vasculitis is a distinct subclass involving anti-neutrophil cytoplasmic antibody (ANCA) as the common pathogenesis. Microscopic polyangiitis is a rare form of such vasculitis in children characterized by pulmonary-renal syndrome with pauci-imune rapidly progressive glomerulonephritis and a poor outcome. PMID:21630073

Jindal, Geetanjali; Cruz, Sanjay D; Punia, R P S; Kaur, Ravinder



Recalcitrant Granulomatous Sclerouveitis in a Patient with Granulomatous ANCA-associated Vasculitis  

PubMed Central

Purpose To report an unusual case of granulomatous sclerochoroiditis. Design Interventional case report. Methods A patient with ANCA-associated granulomatous vasculitis presented with nodular necrotizing scleritis, which was recalcitrant to multiple systemic immunosuppressive therapies and progressed to a blind painful eye, which was enucleated. Results Histopathology revealed extensive occlusive vasculitis, diffuse T- and B- cellular infiltration, and lymphoid granulomatous formation. Enhanced MHC class II antigens, adhesion molecules, and Fas (CD95) and FasL (CD95L) were detected in the lesion. Conclusion Granulomatous sclerochoroiditis with aggressive immune reaction can be a complication of ANCA-associated granulomatous vasculitis.

Levy-Clarke, Grace; Ding, Xiaoyan; Gangaputra, Sapna; Yeh, Steven; Goodglick, Todd; Byrnes, Gordon; Nussenblatt, Robert; Chan, Chi-Chao



Exercise-induced vasculitis associated with autoimmune disease.  


Exercised-induced vasculitis (EIV) is an underreported and frequently misdiagnosed condition that occurs on the lower extremities shortly after exercise. Most reported cases have presented in healthy-appearing individuals, but some cases have been linked to other disease processes. A case report is presented of recurring EIV in a 65-year-old woman with a history of dermatitis herpetiformis; chronic, mildly elevated liver transaminases of unknown cause; microscopic colitis; celiac disease; multiple miscarriages; and heart block who was found to have autoimmune hepatitis upon workup of her rash. Both EIV and autoimmune hepatitis were misdiagnosed over many years by several clinicians in various specialties. Her family history was remarkable for 2 sisters with systemic lupus erythematosus and similar recurring exercise-induced rashes of the lower extremities, suggesting a familial link for this condition. Clinicians should recognize EIV and consider the possibility that this disorder may be the presenting sign of subclinical connective-tissue diseases. PMID:19681343

Knoell, Keith Allen



Maintenance of clinical remission in ANCA-associated vasculitis.  


A fundamental change in management of antineutrophil cytoplasmic antibody-associated vasculitis in the past 10 years is the early focussed use of aggressive immunosuppression, using regimens comprised of widely available medications. Using a clinical framework to quantify morbidity, we can induce remission in most patients within 3-6 months using glucocorticoids plus methotrexate, cyclophosphamide or rituximab, with additional plasmapheresis when indicated. Difficulty in maintaining remission probably relates to the difference between true pathophysiological remission and the absence of clinical, serological or radiological evidence of disease activity. For surviving patients, the cumulative problems of relapse, burden of disease, or its treatment are coupled with pre-existing diseases or new conditions arising since diagnosis. Initial early control should reduce subsequent damage, but what effect it will have on relapse is not clear. In the absence of a cure, future trials should focus on reducing toxicity and comorbidity as well as controlling disease. PMID:23147895

Luqmani, Raashid



Angiotropic large cell lymphoma with mononeuritis multiplex mimicking systemic vasculitis.  

PubMed Central

Angiotropic large cell lymphoma (ALCL), the so-called malignant angioendotheliomatosis, is characterised by proliferation of tumorous cells within small vessels. Manifestations in the CNS and cutaneous lesions prevail in the clinical presentation, although any organ can be involved. The recent classification of this lymphoma as part of the large cell lymphomas has modified the therapeutic approaches employed. This should improve the prognosis of this usually fatal disease. An unusual case presenting with fever, mononeuritis multiplex, and cutaneous lesions is reported. Peripheral neuropathy without other neurological symptoms is uncommon, and, to our knowledge, such isolated mononeuritis multiplex with nerve lesions has not been previously reported in ALCL. The clinical diagnosis was a systemic necrotising vasculitis and it is considered that its differential diagnosis must include angiotropic large cell lymphoma. Images

Roux, S; Grossin, M; De Bandt, M; Palazzo, E; Vachon, F; Kahn, M F



Familial immunodeficiency with cutaneous vasculitis, myoclonus, and cognitive impairment.  


We report a family with five of six siblings (including identical male twins) with a novel constellation of immunologic and neurologic impairments. Affected subjects experienced severe dermatitis starting around 9 months of age, Stevens-Johnson syndrome in early childhood, and extreme elevations of IgE (9,400-43,000 IU/ml). The oldest sibling died at age 27 of respiratory failure following recurrent, severe pneumonias. All four surviving affected siblings have had chronic sinusitis or otitis, cutaneous vasculitis, and recurrent bacterial pneumonias leading to bronchiectasis. Neurologic features in all five siblings included oral motor deficits, dysarthria, low average IQ (70-80), and essential myoclonus. Four had documented ataxia and/or mild sensory loss with increased patellar but diminished ankle reflexes. The nonconsanguineous parents and one sibling had none of the above findings, consistent with autosomal recessive inheritance. This primary immunodeficiency with distinctive neurological impairments represents a new syndrome. Published 2003 Wiley-Liss, Inc. PMID:14981714

Hay, Beverly N; Martin, Julie E; Karp, Barbara; Davis, Joie; Darnell, Dirk; Solomon, Beth; Turner, Maria; Holland, Steven M; Puck, Jennifer M



Paraneoplastic cutaneous leukocytoclastic vasculitis disclosing multiple myeloma: a case report.  


Cutaneous lesions are unusual during the course of multiple myeloma. In rare cases, multiple myeloma may be associated to skin involvement secondary to amyloidosis, cryoglobulinemia, and POEMS syndrome. Paraneoplastic skin involvement occurs preferentially during solid neoplasms. We report the case of an uncommon presentation of an IgA lambda multiple myeloma in a 58 year-old woman preceded by vascular purpura with cutaneous leukocytoclastic vasculitis (LV) and intense deposit of IgA and kappa light chains in the dermal vessels. Purpura resolved after specific treatment of multiple myeloma and diagnosis of paraneoplastic purpura was asserted. We propose a brief review of the literature about skin involvement during multiple myeloma. This case highlights the necessity to search for MM when we are facing a LV. PMID:21729687

Peterlin, Pierre; Ponge, Thierry; Blin, Nicolas; Moreau, Philippe; Hamidou, Mohamed; Agard, Christian



Vasculitis Mimics: Cocaine-induced Midline Destructive Lesions.  


: It has been recognized that nasal cocaine abuse can induce midline destructive lesions that can mimic different disorders, including small-vessel vasculitis. The authors reported 2 cases of patients referred to the rheumatology clinic with a previous diagnosis of granulomatosis with polyangiitis (Wegener's granulomatosis), presenting with chronic perforation in the palate, refractory to immunosuppressant therapy. In both patients, laboratory investigation revealed antineutrophil cytoplasmic antibody positivity. A differential diagnosis between cocaine-induced midline destructive lesions and granulomatosis with polyangiitis is also difficult to establish because of the presence of antineutrophil cytoplasmic antibody in both disorders. Given the high prevalence of cocaine use, awareness of this mimic is essential to avoid a misdiagnosis and the use of unnecessary and potential toxic therapies. PMID:23811575

Perez Alamino, Rodolfo; Espinoza, Luis R



Cerebral vasculitis complicating postoperative meningitis: the role of steroids revisited.  


Meningitis due to Streptococcus pneumoniae is a rare complication of trans-sphenoidal surgery. We present the case of a patient who developed pneumococcal meningitis with associated bacteraemia after elective endoscopic trans-sphenoidal resection of a pituitary macro-adenoma. After initial treatment with ceftriaxone and dexamethasone, the patient made a good recovery and dexamethasone was discontinued. Two days later the patient's condition deteriorated rapidly, presenting focal and diffuse neurological deficits. Cerebral MRI revealed widespread punctate ischaemic-type lesions affecting both anterior and posterior vascular territories bilaterally and involving features consistent with cerebral vasculitis. Antibiotic treatment was broadened to include meropenem and dexamethasone was restarted, but the patient remained in a comatose state and died 14 days later. Steroid treatment may play a dual role in this poorly characterised infectious complication of trans-sphenoidal pituitary surgery. This possibility is discussed and the options for prophylaxis are reviewed. PMID:23135730

Darling, Katharine E A; Niederhauser, Julien; Bervini, David; Giulieri, Stefano; Daniel, Roy T; Bille, Jacques; Erard, Veronique



Chronic leg ulceration with livedoid vasculitis, and response to oral ketanserin.  


We report a patient with a 6-year history of recalcitrant painful ulceration of both lower legs, diagnosed as being due to livedoid vasculitis. The lesions healed rapidly and remained healed on treatment with oral ketanserin. PMID:2638912

Rustin, M H; Bunker, C B; Dowd, P M



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

SciTech Connect

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.

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



The contribution of genetic variation and infection to the pathogenesis of ANCA-associated systemic vasculitis  

PubMed Central

The aetiology of anti-neutrophil cytoplasmic antibody (ANCA)-associated systemic vasculitis has not been well defined. Here we review two factors which may play a role in the pathogenesis of the disease: genetics and infection. In particular, we discuss the role of autoantibodies to LAMP-2, which may arise following infection with Gram-negative bacteria, and may contribute to the development of ANCA-associated systemic vasculitis in genetically susceptible individuals.



Behçet’s disease: an immune-mediated vasculitis involving vessels of all sizes  

Microsoft Academic Search

Behçet’s disease is an immune-mediated vasculitis affecting both small and large vessels. Small-vessel vasculitis is the pathological\\u000a basis of the multiorgan involvement that results in protean clinical features. However, relapsing aphthous ulcers in the mouth\\u000a are considered the clinical hallmark and are often also observed over the genitalia. Both manifestations, in association with\\u000a uveitis, form the typical clinical triad. In

R. Rizzi; S. Bruno; R. Dammacco



Cutaneous vasculitis as the sole manifestation of disseminated gonococcal infection: case report.  

PubMed Central

One of the possible systemic complications of gonorrhoea is disseminated gonococcal infection (DGI), which is usually characterised by both skin and joint lesions. While joint involvement ranges from tenosynovitis to suppurative arthritis, cutaneous involvement features varied non-specific patterns often clinically and histologically consistent with vasculitis. We report a case of DGI in which an extensive, vesicobullous, haemorrhagic, and necrotic cutaneous vasculitis was the sole manifestation of the disease. Images

Mastrolonardo, M; Loconsole, F; Conte, A; Rantuccio, F



ACR/EULAR-endorsed study to develop Diagnostic and Classification Criteria for Vasculitis (DCVAS).  


The systemic vasculitides are a group of uncommon diseases characterized by blood vessel inflammation. There are currently no diagnostic criteria for the primary systemic vasculitides and physicians must rely on experience and disease definitions. The absence of validated criteria can result in delays in making the correct diagnosis and starting appropriate therapy. With the increased understanding of the pathophysiology of vasculitis and newer diagnostic tests in widespread clinical use, it is an appropriate time for classification criteria for primary vasculitis to be revised. The Diagnostic and Classification Criteria for Vasculitis (DCVAS) study is a multinational observational study designed to develop and validate diagnostic criteria and to improve and validate classification criteria for primary systemic vasculitis. The analytic approach will be based on the traditional approach of vessel size for classification of vasculitis but will also incorporate detailed clinical data, evaluation of anti-neutrophil cytoplasm antibody diagnostic testing, biopsy and imaging data. The study is following the guidelines for the development of classification criteria established by the American College of Rheumatology and the European League against Rheumatism. The study will incorporate the use of pre-defined cases of each condition to reduce the inherent circularity when developing new classification criteria and will explore alternative approaches to deriving reference standards by creating data-driven classification algorithms. We anticipate recruiting >2,000 patients with primary systemic vasculitis and 1,500 patients with autoimmune diseases and other conditions that mimic vasculitis. As of June 2013, >100 medical centers across 31 countries in Asia, Australasia, Europe, North America, and South America were contributing data to the study. The DCVAS study provides a unique opportunity to increase generalizability and collate a large dataset on the occurrence, presentation, and outcome of vasculitis in different populations. PMID:23996327

Craven, Anthea; Robson, Joanna; Ponte, Cristina; Grayson, Peter C; Suppiah, Ravi; Judge, Andrew; Watts, Richard; Merkel, Peter A; Luqmani, Raashid A



Clinical Outcome and Prognosis of Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis in Japan  

Microsoft Academic Search

Background\\/Aims: We conducted a broad survey of 99 patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis and investigated both prognosis and outcomes. Methods: Clinical data evaluated were age, sex, patient survival, renal survival, serum albumin, serum creatinine, urinary protein, hematuria, C-reactive protein (CRP), ANCA titer, IgG and the Birmingham Vasculitis Activity Score (BVAS). Results: The patient survival rate at 6 months

Mitsuyo Itabashi; Takashi Takei; Yasuko Yabuki; Hitoe Suzuki; Minoru Ando; Mayuko Akamatsu; Mayuko Yamazaki; Michihiro Mitobe; Yoshihiko Watanabe; Takahiro Mochizuki; Kosaku Nitta



[First manifestation of a leucocytoclastic vasculitis in the colon: case report and differential diagnostic aspects].  


This report presents a female patient suffering from chronic diarrhea, who developed palpable purpura on the lower extremities 8 weeks after onset of the gastrointestinal symptoms. Biopsies obtained from the colon and skin showed leukocytoclastic vasculitis. Possible triggers or underlying diseases could not be found, and the patient recovered without specific treatment for vasculitis. Possible differential diagnoses and the difficulties in classifying vasculitides are discussed in the present report. PMID:23315650

Schartner, E; Höflmayer, D; Müller-Knespel, E-M; Dzirlo, L; Haunold, I; Dawid, M; Stanek-Kalchhauser, D; Weiss, P; Klimpfinger, M



Vasculitis restricted to the lower limbs: a clinical and histopathological study  

PubMed Central

Background Rare cases of vasculitis restricted to the lower limbs have been reported, but the characteristics, outcome and response to treatment of this entity are not well known. Objective To describe the clinical, complementary examinations and response to treatment of this rare entity in the first retrospective series, and to compare data with historical pooled cases. Methods Retrospective analysis of all biopsy?proven cases observed over a 10?year period in four French tertiary medical units. Diagnosis of vasculitis restricted to the lower limb required the absence of any clinical symptom and complementary test finding, suggesting major extramuscular visceral involvement. Results 11 patients were included. Vasculitis restricted to the lower limb was associated with disabling muscle pain of the calves. Fever was present in 50% of cases; ankle arthralgia in 50% and skin involvement in 40%. MRI was the cornerstone of the diagnosis, showing hyperintense signal in T2 weight and in T1 weight after gadolinium injection. MRI findings correlated well with clinical outcome and were useful in guiding biopsy. Muscle biopsy was consistent with a polyarteritis nodosa?type vasculitis in only 40% cases, whereas a leucocytoclastic vasculitis was seen for all other cases. Treatment with corticosteroids was effective in all cases, but there were relapses requiring immunosuppressive agents in 54% of cases. Conclusion Vasculitis of the calf muscles must be considered for patients with calf pain and with a biological inflammatory syndrome.

Khellaf, M; Hamidou, M; Pagnoux, C; Michel, M; Brisseau, J M; Chevallier, X; Cohen, P; Guillevin, L; Godeau, B



Advances in the use of biologic agents for the treatment of systemic vasculitis  

PubMed Central

Purpose of review Due to the well-known toxicities of cyclophosphamide, substantial interest exists in finding other therapies to treat primary systemic vasculitis. Biologic agents have been proposed as an alternative to cyclophosphamide for these disorders because of their recent success in treating other rheumatic diseases. This article reviews the current state-of-the-art with regards to the use of biologic agents as a treatment for systemic vasculitis. Recent findings The greatest amount of experience with these agents for the treatment of systemic vasculitis is with anti-tumor necrosis factor agents, pooled intravenous immunoglobulin, and anti-B cell therapies such as rituximab. Intravenous immunoglobulin is already a standard therapy for Kawasaki's disease, but should also be considered for the treatment of ANCA-associated vasculitis when standard therapies are either ineffective or contraindicated. Early experience with tumor necrosis factor inhibitors indicates that they may be effective for the treatment of Takayasu's arteritis, but their role in the treatment of other forms of vasculitis remains controversial. Early experience with rituximab for the treatment of several forms of vasculitis has been quite promising, but must be confirmed by ongoing randomized clinical trials. Summary Biologic agents represent the next evolution in treatment for the primary systemic vasculitides. Greater understanding of these diseases has allowed use to move further away from non-specific, highly toxic therapies towards a more directed approach. As our experience with these agents increases, they will likely form the keystone of treatment in the near future.

Chung, Sharon A.; Seo, Philip



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

PubMed Central

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 (qRT–PCR) 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.

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



Elevated serum levels of macrophage migration inhibitory factor and their significant correlation with rheumatoid vasculitis disease activity  

Microsoft Academic Search

Macrophage migration inhibitory factor (MIF) is recognized to be an important mediator in several inflammatory disorders,\\u000a including rheumatoid arthritis (RA) and vasculitis. To evaluate the role of MIF in rheumatoid vasculitis (RV), we determined\\u000a serum levels of MIF by enzyme-linked immunosorbent assay in RA patients with and without vasculitis and assessed their relationship\\u000a to disease activity. Serum was obtained from

Kuninobu WakabayashiKumiko; Kumiko Otsuka; Michihito Sato; Ryo Takahashi; Tsuyoshi Odai; Takeo Isozaki; Nobuyuki Yajima; Yusuke Miwa; Tsuyoshi Kasama


Serum sFAS Levels Are Elevated in ANCA-Positive Vasculitis Compared with Other Autoimmune Diseases  

Microsoft Academic Search

The role of the Fas\\/FasL system in ANCA-associated vasculitis is unclear. We therefore assessed levels of soluble Fas (sFas) in sera and Fas expression on mononuclear cells from patients with ANCA-positive vasculitis and compared the results with those found in other rheumatic diseases. Serum levels of sFas were determined by ELISA. The ANCA-positive vasculitis patients studied included 29 at onset,

Marta Christensson; Erna Pettersson; Kjell Eneslätt; Birger Christensson; Johan Bratt; Solbritt Rantapää-Dahlqvist; Karl-Gösta Sundqvist



Circovirus in tissues of dogs with vasculitis and hemorrhage.  


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

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



Propylthiouracil-induced lupus-like or vasculitis syndrome  

PubMed Central

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.



Profile of retinal vasculitis in a tertiary eye care center in Eastern India  

PubMed Central

Aims: To provide a fact file on the etiology, clinical presentations and management of retinal vasculitis in Eastern India. Materials and Methods: Retrospective, record based analysis of retinal vasculitis cases in a tertiary care center in Eastern India from January 2007 to December 2009. Results: One hundred and thirteen eyes of 70 patients of retinal vasculitis were included in this study. Sixty (85.7%) patients were male (mean age 33± 11.1 years) and 10 (14.3%) were female (mean age 32.4 ± 13.6 years). Vasculitis was bilateral in 43 (61.4%) and unilateral in 27 (38.6%) patients. Commonest symptoms were dimness of vision (73; 64.6%) and floaters (36; 31.9%). Vascular sheathing (82; 72.6%) and vitritis (51; 45.1%) were commonest signs. Mantoux test was positive in 21 (30%) patients but tuberculosis was confirmed in only four (5.71%) patients. Raised serum angiotensin-converting enzyme level and positive antinuclear antibody level were reported in four (5.71%) patients each. Human leukocyte antigen B5 (HLA B5) marker was present in one (1.4%) patient. However, none of the total 70 patients were found to have a conclusively proven systemic disease attributable as the cause of retinal vasculitis. Oral corticosteroid (60; 85.7%) was the mainstay of treatment. Forty-eight (42.5%) eyes maintained their initial visual acuity and 43 (38%) gained one or more line at mean follow-up of 16.6± 6.3 months. Conclusion: Retinal vasculitis cases had similar clinical presentations and common treatment plan. There was no systemic disease association with vasculitis warranting a careful approach in prescribing investigations.

Saurabh, Kumar; Das, Radha R; Biswas, Jyotirmay; Kumar, Amitabh



Are classification criteria for vasculitis useful in clinical practice? Observations and lessons from Colombia  

PubMed Central

Introduction Idiopathic systemic vasculitis represents a group of clinical entities having non-specific etiology with the common characteristic of acute or chronic inflammatory compromise of the small and large vessels walls, associated with fibrinoid necrosis. Objectives To describe the most common inflammatory vascular diseases in a long historical cohort of patients from San Juan de Dios Hospital located in Bogota, Colombia using two different systems and a clinical histopathological correlation format, and to make a comparison between them. Methods We reviewed all previously ascertained cases of vasculitis confirmed by biopsy processed between 1953 and 1990, and systematically collected data on all new cases of vasculitis from 1991 to 1997 at the Hospital San Juan de Dios (Bogota – Colombia). The cases were classified in accordance with the Chapel Hill Consensus criteria, and the system proposed by J.T. Lie. Results Of 165,556 biopsy tissue specimens obtained during this period from our hospital, 0.18% had vasculitis, perivasculitis or vasculopathy. These included 304 histopathological biopsies from 292 patients. Cutaneous leukocytoclastic vasculitis (64 histological specimens) was the most frequently encountered type of "primary" vasculitis followed by thromboangiitis obliterans (38 specimens), and polyarteritis nodosa (24 specimens). Vasculitis associated with connective tissue diseases (33 specimens) and infection (20 specimens) were the main forms of secondary vasculitis, a category that was omitted from the Chapel Hill consensus report. We found that 65.8% of our histopathological diagnoses could not be classified according to the Chapel Hill classification, and 35.2% could not be classified according to the classification of Lie. Only 8.9% of cases remained unclassified by our system after clinical and histological correlation. Conclusion Current vasculitis classification schemes are designed for classification, rather that diagnosis of disease and do not adequately address some common forms of inflammatory vascular diseases, including those of infectious etiology and unusual etiology seen in clinical practice. Based on our clinical experience, we suggest a classification outline which practitioners can use which emphasizes correlation of the clinical picture to the histopathology findings for diagnosis and therapy, which may promote better clinical practice and standardization for clinical trials.

Patarroyo, Paul Alejandro Mendez; Restrepo, Jose Felix; Rojas, Samanda Adriana; Rondon, Federico; Matteson, Eric L; Iglesias-Gamarra, Antonio



Metastatic Crohn's disease accompanying granulomatous vasculitis and lymphangitis in the vulva  

PubMed Central

Metastatic Crohn’s disease (CD) is an extremely rare extragastrointestinal manifestation of CD, and is characterized histopathologically by the presence of non-caseating granulomatous inflammation. Granulomatous vasculitis and lymphangitis have rarely been documented in metastatic CD. Herein, we report the first documented case of metastatic CD accompanied by both granulomatous vasculitis and lymphangitis in the vulva. A 35-year-old Japanese female with CD presented with multiple small nodules in her vulva. Biopsy was performed under a clinical diagnosis of genital warts. A histopathological study revealed marked lymphangiectasia in the papillary dermis. Within the dilated lymphatics, lymphocytes and aggregates of macrophages were present, which are typical features of granulomatous lymphangitis. Tiny non-caseating granulomas and granulomatous vasculitis were also observed. Accordingly, a diagnosis of metastatic CD accompanied by both granulomatous vasculitis and lymphangitis was made. The occurrence of cutaneous lesions in patients with CD is well known. Albeit extremely rare, lymphangiectasia has been reported in the vulva of CD patients that clinically mimicked viral warts, as in the present case. The diagnosis of metastatic CD in the present case was not difficult because characteristic histopathological features were present, and a clinical history of CD was available. However, a few cases of genital swelling associated with granulomatous inflammation prior to a diagnosis of gastrointestinal CD have been documented. Therefore, granulomatous vasculitis and lymphangitis in the external genitals should be considered as potential indication of metastatic CD even in cases without a history of gastrointestinal CD.

Ishida, Mitsuaki; Iwai, Muneo; Yoshida, Keiko; Kagotani, Akiko; Okabe, Hidetoshi



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

PubMed Central

Patients with chronic hepatitis C virus (HCV) can develop systemic cryoglobulinemic vasculitis. Combination of pegylated-interferon ? 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.

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



Recurrence of anti-neutrophil cytoplasmic antibody vasculitis in the kidney allograft.  


We report a case of recurrent anti-cytoplasmic neutrophil antibody (ANCA)-associated vasculitis post kidney transplantation. A 60-year-old woman underwent uncomplicated deceased-donor kidney transplantation for end-stage renal disease (ESRD) secondary to myeloperoxidase-specific ANCA-associated vasculitis, after six years of haemodialysis, and clinical remission. Immunosuppression was with tacrolimus/mycophenolate and prednisolone after basiliximab induction therapy. Five weeks post-transplantation, an allograft biopsy, done for a rising creatinine and glomerular haematuria, revealed pauci-immune crescentic glomerulonephritis. This was treated with pulse methylprednisolone, increase in maintenance prednisolone, 7 sessions of plasma exchange, and replacement of mycophenolate with cyclophosphamide. Tacrolimus was continued throughout. After 3 months of therapy a repeat allograft biopsy showed quiescent vasculitis. The cyclophosphamide was then ceased, and mycophenolate reinstituted. The patient has maintained clinical and histological stability. Reported rates of ANCA-associated vasculitis recurrence post-kidney transplantation have varied but are low compared with other types of glomerulonephritis and seemed to have further declined in the era of modern immunosuppression. Given the low recurrence rate and excellent outcomes in suitable patients, kidney transplantation remains the optimal form of renal replacement therapy for ESRD due to ANCA-associated vasculitis. Whilst re-introduction of cyclophosphamide has been the mainstay of therapy, additional reported successful therapeutic strategies have included pulse methylprednisolone, plasma exchange and rituximab. Further study on the most effective and safest treatment options would be of use given the current paucity of data in this area. PMID:22497649

Lau, David; Summers, Shaun; Amos, Liv; Simpson, Ian; Mulley, William



Decreased Neutrophil Apoptosis in Quiescent ANCA-Associated Systemic Vasculitis  

PubMed Central

Background ANCA-Associated Systemic Vasculitis (AASV) is characterized by leukocytoclasis, accumulation of unscavenged apoptotic and necrotic neutrophils in perivascular tissues. Dysregulation of neutrophil cell death may contribute directly to the pathogenesis of AASV. Methods Neutrophils from Healthy Blood Donors (HBD), patients with AASV most in complete remission, Polycythemia Vera (PV), Systemic Lupus Erythematosus (SLE), Rheumatoid Arthritis (RA) and renal transplant recipients (TP) were incubated in vitro, and the rate of spontaneous apoptosis was measured by FACS. Plasma levels of cytokines and sFAS were measured with cytometric bead array and ELISA. Expression of pro/anti-apoptotic factors, transcription factors C/EBP-?, C/EBP-? and PU.1 and inhibitors of survival/JAK2-pathway were measured by real-time-PCR. Results AASV, PV and RA neutrophils had a significantly lower rate of apoptosis compared to HBD neutrophils (AASV 50±14% vs. HBD 64±11%, p<0.0001). In RA but not in AASV and PV, low apoptosis rate correlated with increased plasma levels of GM-CSF and high mRNA levels of anti-apoptotic factors Bcl-2A1 and Mcl-1. AASV patients had normal levels of G-CSF, GM-CSF and IL-3. Both C/EBP-?, C/EBP-? were significantly higher in neutrophils from AASV patients than HBD. Levels of sFAS were significantly higher in AASV compared to HBD. Conclusion Neutrophil apoptosis rates in vitro are decreased in AASV, RA and PV but mechanisms seem to differ. Increased mRNA levels of granulopoiesis-associated transcription factors and increased levels of sFAS in plasma were observed in AASV. Additional studies are required to define the mechanisms behind the decreased apoptosis rates, and possible connections with accumulation of dying neutrophils in regions of vascular lesions in AASV patients.

Abdgawad, Mohamed; Pettersson, Asa; Gunnarsson, Lena; Bengtsson, Anders A.; Geborek, Pierre; Nilsson, Lars; Segelmark, Marten; Hellmark, Thomas



Cutaneous necrotizing vasculitis after low dose methotrexate therapy for rheumatoid arthritis: A possible manifestation of methotrexate hypersensitivity  

Microsoft Academic Search

Large haemorrhagic and necrotic cutaneous lesions developed after two low dose (5mg) methotrexate injections in a patient suffering from long standing rheumatoid arthritis. Differential clinical diagnosis included factitia dermatitis, infectious processes, pyoderma gangrenosum, rheumatoid neutrophilic dermatitis, necrotizing arteritis and vasculitis. Histological and direct immunofluo-rescent examinations of skin biopsies supported the diagnosis of leucocytoclastic vasculitis. We discuss the respective roles of

Th. Simonart; P. Durez; J. Margaux; J. Van Geertruyden; D. Goldschmidt; D. Parent



ELISA is the superior method for detecting antineutrophil cytoplasmic antibodies in the diagnosis of systemic necrotising vasculitis.  

PubMed Central

BACKGROUND: Antineutrophil cytoplasmic antibodies (ANCA) have been used as a diagnostic marker for systemic necrotising vasculitis, a disease classification which includes Wegener granulomatosis, microscopic and classic polyarteritis nodosa, and Churg Strauss disease. OBJECTIVE: To compare the diagnostic value of the two methods for detecting these antibodies--immunofluorescence and enzyme linked immunosorbent assay (ELISA)--with respect to biopsy proven active systemic necrotising vasculitis in a clinically relevant population. METHODS: A prospective study to ascertain the patient's diagnosis at the time of each of the 466 requests for ANCA received at one laboratory over a nine month period, and allocate each to one of five diagnostic groups: active and inactive biopsy proven systemic necrotising vasculitis, suspected systemic necrotising vasculitis, low probability systemic necrotising vasculitis, and not systemic necrotising vasculitis. RESULTS: ELISA was superior to immunofluorescence in the diagnosis of systemic necrotising vasculitis because it was less likely to detect other diseases. This was reflected in its specificity of 97% and positive predictive value of 73%, compared with 90% and only 50% for immunofluorescence (p = 0.0006 and p = 0.013, respectively). ELISA had a negative predictive value of 98% which was not significantly different to immunofluorescence. ELISA was technically superior. CONCLUSIONS: ELISA is the superior method of ANCA detection in the diagnosis of systemic necrotising vasculitis and should be used in conjunction with a compatible clinical picture and histological evidence.

Harris, A; Chang, G; Vadas, M; Gillis, D



Progress Towards a Core Set of Outcome Measures in Small-vessel Vasculitis. Report from OMERACT 9  

PubMed Central

The past decade has seen a substantial increase in the number and quality of clinical trials of new therapies for vasculitis, including randomized, controlled, multicenter trials that have successfully incorporated measures of disease activity and toxicity. However, because current treatment regimens for severe disease effectively induce initial remission and reduce mortality, future trials will focus on any of several goals including: (a) treatment of mild—moderate disease; (b) prevention of chronic damage; (c) reduction in treatment toxicity; or (d) more subtle differences in remission induction or maintenance. Thus, new trials will require outcome measure instruments that are more precise and are better able to detect effective treatments for different disease states and measure chronic manifestations of disease. The OMERACT Vasculitis Working Group comprises international clinical investigators with expertise in vasculitis who, since 2002, have worked collaboratively to advance the refinement of outcome measures in vasculitis, create new measures to address domains of illness not covered by current research approaches, and harmonize outcome assessment in vasculitis. The focus of the OMERACT group to date has been on outcome measures in small-vessel vasculitis with an overall goal of creating a core set of outcome measures for vasculitis, each of which fulfills the OMERACT filter of truth, discrimination, feasibility, and identifying additional domains requiring further research. This process has been informed by several ongoing projects providing data on outcomes of disease activity, disease-related damage, multidimensional health-related quality of life, and patient-reported ratings of the burden of vasculitis.




Levamisole-induced occlusive necrotising vasculitis in cocaine abusers: an unusual cause of skin necrosis and neutropenia.  


We present three cases describing the various skin manifestations of presumed levamisole-contaminated cocaine use. Antibody-mediated vasculitis and neutropenia were consistent findings in these cases and repeat exposure resulted in distinct dermatologic complications. This phenomenon of levamisole-induced vasculitis and neutropenia is being increasingly described and has characteristic wound manifestations that must be recognised and treated early. PMID:22716045

Belfonte, Cassius D; Shanmugam, Victoria K; Kieffer, Nicole; Coker, Shodeinde; Boucree, Suelyn; Kerr, Gail



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

PubMed Central

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.

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



Intravitreal Injection of Bevacizumab in a Case of Occlusive Retinal Vasculitis Accompanied by Syphilitic Intraocular Inflammation  

PubMed Central

Background We report a rare case of syphilitic intraocular inflammation with occlusive retinal vasculitis treated with intravitreal injection of bevacizumab (IVB) in addition to conventional therapy for syphilis. Case A 24-year-old woman who complained floaters in both her eyes showed occlusive retinal vasculitis OU. According to the high titer of the Treponema antigen and characteristic cutaneous eruption, she was diagnosed as secondary syphilis. Observation She was treated with oral amoxicillin and retinal photocoagulation OU. Then, administration of prednisolone was required to the intraocular inflammations considered as Jarisch-Herxheimer reactions. IVB was also performed toward exacerbated retinal neovascularization and showed transient effects. Conclusion We experienced a rare case of occlusive retinal vasculitis accompanied by syphilis intraocular inflammation. IVB was considered to be effective as an adjunctive therapy for inflammatory neovascularizations.

Ishijima, Kan; Namba, Kenichi; Ohno, Shigeaki; Mochizuki, Kiyofumi; Ishida, Susumu



Serum alkaline ribonuclease derived from vascular endothelial cells is raised in patients with rheumatoid vasculitis.  

PubMed Central

We investigated the mechanisms of the marked increase of alkaline ribonuclease (RNase) in the sera of patients with rheumatoid arthritis associated with vasculitis. Among various tissues examined, blood vessels contained higher levels of alkaline RNase than acid RNase. Cultured human endothelial cells contained significantly higher amounts of alkaline RNase than acid RNase. In contrast, acid RNase was predominant in most other tissues and cells. Endothelial cells cultured with sera from patients with vasculitis released alkaline RNase into the extracellular medium. The phosphocellulose chromatographic profile of these sera differed from that of sera from healthy subjects. These results imply that the alkaline RNase in sera of patients with vasculitis is derived from blood vessels, probably from endothelial cells.

Oribe, M; Shingu, M; Nobunaga, M



Cutaneous leukocytoclastic vasculitis: clinical and laboratory features of 45 patients seen in Ramathibodi Hospital.  


Forty-five patients with histologically proved cutaneous leukocytoclastic vasculitis were studied with regard to the clinical features, laboratory findings and etiology. There were 12 males and 33 females, with an age range of 13 to 64 years. The most common skin lesions were palpable purpura which appeared mostly on the lower part of the legs. Renal involvement was the most common systemic manifestation, which occurred in 45 per cent of the patients. Abdominal pain occurred in 42 per cent of the male patients while none of the female patients had this symptom. Arthralgia occurred in 20 per cent of the patients. The most common laboratory abnormalities were elevation of ESR, which was significantly more common in females than in males (P = 0.047). The possible etiology of leukocytoclastic vasculitis was identified in 5 patients, these included streptococcal infection in 2 patients, in the other patients the possible causes were penicillin hypersensitivity, systemic lupus erythematosus and livedoid vasculitis, respectively. PMID:2212916

Puavilai, S; Timpatanapong, P; Rajatanavin, N; Charuwichitratana, S; Polnikorn, N



[Intravenous therapy with methylprednisolone and cyclophosphamide in vasculitis of peripheral nervous system. Evaluation of 8 patients].  


The peripheral nervous system is frequently involved in systemic vasculitis and it may be helpful in the disease diagnosis. We report on eight patients: seven women and one man; five white, two black and one yellow; age mean 55.9 years; four had polyarteritis nodosa, one had systemic lupus erythematosus, one had isolated peripheral nerve vasculitis and one had livedoid vasculitis. All of them received endovenous therapy with "pulse" of methylprednisolone (1 g/day/3 days) and cyclophosphamide (1 g/1 day). Five patients improved, two remained unchanged and one died. The neurological improvement occurred after the third or fourth pulse and in the patients who have had a shorter time of disease. PMID:9698740

Lino, A M; Hirata, M T; Baęta, A M; Tsanaclis, A M; Scaff, M; Marchiori, P E



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

PubMed Central

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.



Necrotizing vasculitis in a patient affected by autoimmune hyperthyroidism treated with propylthiouracil.  


Necrotizing vasculitis is a complex phenomenon because of an inflammation of small and larger vessels with polymorph infiltration within the vessel walls and leukocytoclasis, occurring in several autoimmune diseases. Propylthiouracil (PTU) is a medication commonly used to treat hyperthyroidism, but it is associated with various rare side effects, such as antineutrophil cytoplasm antibody-positive vasculitis. In the last decades, multiple cases of PTU causing antineutrophilic cytoplasmic antibody have been reported, some of them fatal. The present authors report the case of a 34-year-old Caucasian female affected by autoimmune hyperthyroidism treated with PTU, presenting an antineutrophil cytoplasm antibody-positive necrotizing vasculitis, with high levels of anticardiolipin antibodies that involved the upper arms and buttocks. The clinical manifestations improved after discontinuing of PTU and immunosuppressant treatment. PMID:20482568

Antonucci, Angela; Bardazzi, Federico; Iozzo, Ivano; Patrizi, Annalisa


Clinical findings on ANCA-associated renal vasculitis from the Japan RPGN registry obtained via a questionnaire survey.  


Renal involvement with significant organ damage is common in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). As a result, it is independently referred to ANCA-associated renal vasculitis. Clinically, ANCA-associated renal vasculitis is characterized by rapidly progressive glomerulonephritis. Pathologically, it is defined by pauci-immune type necrotizing and crescentic glomerulonephritis. According to previous reports from all over the world, the etiology, prevalence, and prognosis of RPGN including ANCA-associated renal vasculitis varies among races and periods. To elucidate the clinical characteristics of Japanese RPGN patients, a registry derived from a questionnaire survey was established in 1999 and maintained until 2006. As a result, 1,772 cases were collected, analyzed, and reported previously. In this mini-review, we outline the characteristic clinical findings of Japanese patients (Asian) with ANCA-associated renal vasculitis, based on the registry data. PMID:23239119

Yamagata, Kunihiro; Usui, Joichi; Sugiyama, Hitoshi; Nitta, Kosaku; Wada, Takashi; Muso, Eri; Arimura, Yoshihiro; Koyama, Akio; Makino, Hirofumi; Matsuo, Seiichi



A case of ANCA-associated vasculitis with glomerular eosinophilic infiltration: a possible pathogenic implication.  


We present a 58-year-old male patient with myeloperoxidase antineutrophil cytoplasmic antibody-associated vasculitis with rapidly progressive glomerulonephritis. He failed to fulfill the common American College of Rheumatology criteria for eosinophilic granulomatosis with polyangiitis and was tentatively diagnosed with microscopic polyangiitis. Kidney biopsy showed pauci-immune crescentic necrotizing glomerulonephritis with neutrophilic and eosinophilic infiltration. Previous reports implicate eosinophils in the pathogenesis of this disease. Therefore, this case suggests that infiltrated eosinophils as well as neutrophils might play roles in the development of tissue injury in systemic vasculitis. PMID:22710834

Suzuki, Hiroko; Fujita, Takayuki; Fuke, Yoshinobu; Yabuki, Minako; Kajiwara, Mamiko; Ishihara, Yuko; Hemmi, Seiichiro; Soma, Masayoshi



Large- and small-vessel vasculitis: a critical digest of the 2010-2011 literature.  


The last two years have been marked by significant achievement in the identification of the basic mechanisms of systemic vasculitis and in the translation of these mechanisms into targeted therapies. More specifically, new insights into the environmental, cellular, and genetic factors involved in the pathogenesis of systemic vasculitis have been provided. Consequently, several studies focused on the development of novel strategies to achieve and maintain clinical remission in small- and large-vessel vascultis, including relevant large multicentre trials, have been promoted. The highlights of these studies, their potential clinical implications and the unmet needs, which are still to be addressed, are summarised in this review. PMID:22640656

Talarico, Rosaria; Baldini, Chiara; Della Rossa, Alessandra; Stagnaro, Chiara; Ferrari, Claudia; Luciano, Nicoletta; Bombardieri, Stefano



Efficacy of rituximab in severe and mild abdominal vasculitis in the course of mixed cryoglobulinemia.  


Abdominal vasculitis represents a rare, but life-threatening manifestation in mixed cryoglobulinemic syndrome (MCsn), despite aggressive immunosuppressive treatments. Anti-CD20 monoclonal antibody, rituximab (RTX) has already been used with good results in MC in preliminary studies. No data have been provided, however, on the efficacy of RTX in gastrointestinal involvement of MCsn. Herein, we report the favourable outcomes of the gastrointestinal manifestations in five patients treated with RTX, where the diagnosis of abdominal vasculitis was confirmed by histopathological findings in 2 out of 5 patients, while in the other three patients the diagnosis was made on the basis of positive endoscopy or by integrating clinical and laboratory data. PMID:20412709

Quartuccio, Luca; Petrarca, Antonio; Mansutti, Elisa; Pieroni, Stefano; Calcabrini, Lucia; Avellini, C; Zignego, Annalinda; De Vita, Salvatore


Acute bilateral brachial plexus neuritis associated with hypersensitivity vasculitis. A case report and review of literature.  


The occurrence of brachial plexus neuritis during the acute phase of vasculitis is uncommon. We describe a patient with a long history of rhinitis and a recent onset of asthma, who developed purpuric skin lesions, abdominal pain, eosinophilia and brachial neuritis along with evidence of sacral plexus neuropathy. High dose steroids and cyclophosphamide induced a remission. He stopped all medications after 3 years and after 6 years the patient has some fixed minimal residual neurological deficit. The importance of aggressive therapy in treating extensive polyneuropathy during the acute phase of hypersensitivity vasculitis is raised. A possible cytotoxic role of eosinophils in the pathological process is suggested. PMID:4032989

Raz, I; Leitersdorf, E; Kleinman, Y



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


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

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




Microsoft Academic Search

Vasculitides can be divided into primary forms, for which the etiology is unknown, and secondary forms that result from an autoimmune condition or known infection. The interest in infection-related vasculitides has been boosted for the last two decades by the development of new molecular techniques and the proof of true associations between viral hepatitis C and systemic vasculitis. A wide

Patrice Cacoub


Leukocytoclastic Vasculitis and Acute Renal Failure after Influenza Vaccination in an Elderly Patient with Myelodysplastic Syndrome  

Microsoft Academic Search

Influenza vaccination is recommended for individuals over 65 years of age and for all patients with chronic diseases who are at risk. Side effects which are seen in 1-10% of the vaccinated individuals are usually mild and consist of local reactions and constitutional symptoms. Since 1974, about 30 cases of vasculitis following influenza vaccination have been reported. Case Report: We

Silke Ulm; Margit Hummel; Michael Emig; Manuel Barreto-Miranda; Walter Back; Andreas Reiter; Rüdiger Hehlmann; Andreas Willer



Double ANCA-positive vasculitis in a patient with infective endocarditis  

PubMed Central

The most common pattern of renal involvement in infective endocarditis is infection-associated glomerulonephritis. Due to clinical symptoms and signs that overlap with vasculitis, the diagnosis of infective endocarditis may be delayed. The unusual combination of reduced complement with positive antineutrophil cytoplasmic antibody should raise the suspicion of infections such as infective endocarditis.

Veerappan, I.; Prabitha, E. N.; Abraham, A.; Theodore, S.; Abraham, G.



Hydralazine-induced ANCA vasculitis with pulmonary renal syndrome: a rare clinical presentation.  


Hydralazine is a commonly used drug for treatment of hypertension and is known to cause drug-induced lupus erythematosus. It has rarely been reported to cause anti neutrophil cytoplasmic antibody positive vasculitis, a life-threatening complication. Presentation could be extremely variable delaying diagnosis. Although drug-induced vasculitis has been infrequently associated with rapidly progressing glomerulonephritis, pulmonary involvement presenting as pulmonary renal syndrome is extremely rare. We report a case of hydralazine-induced vasculitis presenting as pulmonary renal syndrome with fatal outcome even after aggressive treatment. Numerous antibodies are associated with hydralazine including anti myeloperoxidase antibody, anti-nuclear antibody, anti-histone antibody, and anti-elastase antibody. Additionally, we also report the presence of anti-phospholipid antibodies specific to anti-cardiolipin, anti-beta2 glycoprotein, and anti-phosphatid that have not been previously reported. We conclude that early diagnosis and prompt discontinuation of the drug is necessary for the treatment of hydralazine-induced anti neutrophil cytoplasmic antibody vasculitis. PMID:21590349

Marina, Vamsee Priya; Malhotra, Deepak; Kaw, Dinkar



Focal retrograde amnesia for autobiographical events following cerebral vasculitis: A connectionist account  

Microsoft Academic Search

We describe a patient who developed a focal retrograde amnesia in the absence of any measurable anterograde memory deficit following recovery from an acute confusional state arising from a primary cerebral vasculitis. Brain imaging revealed bilateral frontal and parietal atrophic changes and a well circumscribed lesion of the left superior temporal gyrus. The patient has normal Intellect and well above

J. J. Evans; E. K. Breen; N. Antoun; J. R. Hodges



Successful management of cryoglobulinemia-induced leukocytoclastic vasculitis with thalidomide in a patient with multiple myeloma  

Microsoft Academic Search

Leukocytoclastic vasculitis (LV) is a systemic inflammatory disorder involving mostly the small vessels. It is characterised by segmental angiocentric neutrophilic inflammation, endothelial cell damage and fibrinoid necrosis. LV is related to a variety of clinical disorders including cryoglobulinemia and, very rarely, multiple myeloma (MM), among many others. The development of LV in patients with MM has been linked to cryoglobulinemia,

M. Cem Ar; Teoman Soysal; Gulen Hatemi; Ayse Salihoglu; Hasan Yazici; Birsen Ulku



Immune-mediated vasculitis in a shar-pei with swollen hock syndrome.  

PubMed Central

A castrated male shar-pei was presented for episodes of lethargy, swelling of the tarsal joints, and polydipsia with polyuria. Histological examination of biopsies from skin overlying the tarsi and direct immunoperoxidase immunohistochemical staining confirmed immune complex vasculitis, suggesting a role for immune complex deposition in the pathogenesis of shar-pei fever.

Tellier, L A



Development of leukocytoclastic vasculitis in a patient with multiple myeloma during treatment with thalidomide  

Microsoft Academic Search

Thalidomide, an agent with antiangiogenic and immunomodulatory properties, is therapeutically effective in multiple myeloma, leprosy, and autoimmune diseases. The most common clinical toxicities of thalidomide are constipation, neuropathy, fatigue, sedation, rash, tremor, and edema. We here describe for the first time a patient who developed leukocytoclastic vasculitis during therapy with thalidomide. Of the 260 patients treated with thalidomide in our

Mathias Witzens; Thomas Moehler; Kai Neben; Stefan Fruehauf; Wolfgang Hartschuh; Anthony D. Ho; Hartmut Goldschmidt



Increased hydroxyl radical generation by normal polymorphonuclear leukocytes incubated in sera from patients with leukocytoclastic vasculitis  

Microsoft Academic Search

The effect of sera from patients with untreated leukocytoclastic vasculitis was investigated on the generation of oxygen intermediates by normal polymorphonuclear leukocytes. Sera from untreated patients induced increased hydroxyl radical generation, which is one of the most potent oxidants capable of causing tissue damage. It is suggested that vascular injury may be mediated in part by enhanced production of hydroxyl

Yoshiki Miyachi; Keiko Yanase; Sadao Imamura; Yukie Niwa



Establishment of Experimental Eosinophilic Vasculitis by IgE-Mediated Cutaneous Reverse Passive Arthus Reaction  

PubMed Central

Prominent eosinophil infiltration is a characteristic of some forms of vasculitis, such as Churg-Strauss syndrome, also known as allergic granulomatous vasculitis. In the current study, we established a mouse model of cutaneous eosinophilic vasculitis by the cutaneous reverse passive Arthus reaction using IgE injection instead of IgG. Wild-type C57BL/6 mice were injected with IgE anti-trinitrophenyl antibodies, followed immediately by intravenous administration of trinitrophenyl bovine serum albumin. IgE-mediated immune complex challenge induced substantial hemorrhage with marked infiltration of eosinophils in which neutrophils, mast cells, and macrophages were also mixed. This finding contrasted remarkably with the neutrophil-dominant infiltration pattern in IgG-mediated immune complex challenge. In the lesion, the expression level of monocyte chemotactic protein-3 was increased, and anti-monocyte chemotactic protein-3 treatment resulted in a significant but incomplete blockade of eosinophil recruitment. Furthermore, mice lacking E-selectin, P-selectin, L-selectin, or intercellular adhesion molecule-1, as well as wild-type mice that received anti-vascular cell adhesion molecule-1-blocking antibodies were assessed for the IgE-mediated Arthus reaction. After 24 hours, the loss of P-selectin resulted in a significant reduction in eosinophil accumulation compared with both wild-type mice and other mouse mutants. Collectively, the Fc class of immunoglobulins, which forms these immune complexes, critically determines the disease manifestation of vasculitis. The IgE-mediated cutaneous reverse passive Arthus reaction may serve as an experimental model for cutaneous eosinophilic infiltration in vasculitis as well as in other diseases.

Ishii, Takayuki; Fujita, Tomoyuki; Matsushita, Takashi; Yanaba, Koichi; Hasegawa, Minoru; Nakashima, Hiroko; Ogawa, Fumihide; Shimizu, Kazuhiro; Takehara, Kazuhiko; Tedder, Thomas F.; Sato, Shinichi; Fujimoto, Manabu



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

PubMed Central

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 9·6 (1·8–680) for 22 patients, compared to 1 (0·1–2·8) 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.

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



Cutaneous granulomatous vasculitis associated with chronic lymphocytic leukemia: case report and review of literature of a rare entity.  


Cutaneous granulomatous vasculitis associated with lymphoproliferative disorders is a rare entity, with only 14 cases previously reported in the English literature. Patients generally present with nodules or ulcers involving the extremities, which can appear months or years before or after onset of systemic disease. Granulomatous vasculitis has a poor prognosis when associated with underlying lymphoproliferative disorders, with the majority of reported cases fatal. Knowledge of this unusual entity is important to allow for proper clinical evaluation, follow-up, and therapy. We report a 77-year-old female with chronic lymphocytic leukemia and granulomatous vasculitis, which highlights the features of this association, and expands the clinical data. PMID:22483523

Gaertner, Erich M; Switlyk, Stephen A



Phenylpropanolamine: an over-the-counter drug causing central nervous system vasculitis and intracerebral hemorrhage. Case report and review.  


Phenylpropanolamine (PPA) is the major ingredient in more than 70 over-the-counter preparations including diet pills, nasal decongestants, and the legal "look-alike" stimulants. Structurally and functionally similar to amphetamine and ephedrine, PPA has recently been associated with several neurological manifestations including psychosis, stroke, severe headache, seizures, and intracerebral hematoma. We report a case of intracerebral hematoma and subarachnoid hemorrhage in a young woman with angiographic and biopsy-proven vasculitis of the central nervous system (CNS) induced by PPA in her diet pills. From review of the literature, we distinguish drug-induced vasculitis as a separate entity from primary CNS vasculitis, both clinically and pathologically. This report should alert physicians, in general, to this potentially fatal side effect of PPA, a commonly used over-the-counter drug. Also, neurosurgeons in particular should consider the possibility of drug-induced vasculitis when faced with cases of intracerebral or subarachnoid hemorrhage without apparent cause. PMID:2956531

Glick, R; Hoying, J; Cerullo, L; Perlman, S



Fulminant amoebic colitis mimicking intestinal vasculitis in a patient with systemic lupus erythematosus.  


Colitis in patients with systemic lupus erythematosus (SLE) is quite rare. It can be caused by intestinal vasculitis, mesenteric vascular thrombosis, concomitant inflammatory bowel disease or infectious colitis. It is important to make an accurate and early diagnosis as the treatments for each condition differ and a delayed diagnosis can result in life-threatening complications. However, non-specific gastrointestinal symptoms make a timely diagnosis challenging. Amoebic colitis is a rare condition in patients with SLE. Here we present a case of fulminant amoebic colitis in a patient with SLE which was initially misdiagnosed as ischemic colitis due to intestinal vasculitis. Her colitis was complicated with multiple intestinal perforations, disseminated intravascular coagulation and acute respiratory distress syndrome; but in the end, the patient was successfully treated with metronidazole and paromomycin. PMID:22570337

Lee, J; Jung, H-S; Nam, H-C; Kwok, S-K; Ju, J H; Park, K-S; Kim, H-Y; Park, S-H



[Vasculitis 2013. What are the changes introduced in the 2012 Chapel Hill Consensus Conference?].  


Vasculitis is a heterogeneous group of rare disorders in which inflammation of blood vessels is the common feature. Due to the increasing number of diseases as well as overlaps and gaps in the definition and nomenclature, the classification criteria were constantly changing in the past decades. The classifications were based essentially on the size of affected blood vessels and pathologic characteristics of inflamed vessel walls. The standard procedures and validated diagnostic criteria are missing from the diagnostics of vasculitis, thus in clinical practice the classification criteria are applicable. The 2012 Chapel Hill Consensus Conference brought a change in the definition, nomenclature and classification of previously uncategorized diseases. The definitions of subgroups accurately determine the diagnosis of the specific disease, and they are suitable for establishing homogeneous disease groups. By better understanding of the etiopathogenetic factors, further diseases and subgroups may be defined in the near future. Orv. Hetil., 2013, 154, 1571-1578. PMID:24077160

Zeher, Margit



Recurrent Bilateral Retinal Vasculitis as a Manifestation of Post-streptococcal Uveitis Syndrome  

PubMed Central

We report a case of post-streptococcal uveitis mainly presenting with bilateral recurrent retinal vasculitis in Korea. A 14-year-old Asian female presented with decreased visual acuity of 20 / 30 in the right eye and 20 / 25 in the left eye. The patient had a history of glomerulonephritis nine months before onset of uveitis. The manifestation of uveitis was predominantly retinal vasculitis. We presumed post-streptococcal uveitis because probable streptococcal infection was confirmed by anti-streptolysin O titer elevation. With topical and oral steroid treatments, the patient experienced complete vision recovery. Post-streptococcal uveitis occurs rarely and mostly involves young patients in the form of non-granulomatous anterior uveitis. However, as this case shows, it may primarily involve the posterior uvea without anterior inflammation and may recur.

Han, Jinu; Lee, Sung Chul



p-ANCA-Associated Vasculitis Caused by Levamisole-Adulterated Cocaine: A Case Report  

PubMed Central

A rare case of a patient with an unusual skin rash after using cocaine is presented. A clinical diagnosis of levamisole-induced vasculitis was made based on initial presentation of purpuric skin lesions involving the ears and positive cocaine on urine toxicology screening. The diagnosis was confirmed after laboratory findings of neutropenia, positive serum levamisole, and a histopathologic test of the skin lesions. The pathophysiology of this condition is discussed. Treatment with methylprednisolone and prednisone led to the resolution of the neutropenia and complete recovery of the skin lesions. With the growing use of levamisole-tainted cocaine, clinicians should be aware of the symptoms of vasculitis and neutropenia induced by this combination of drugs to avoid unnecessary tests and delayed diagnosis.

Carter, Michelle R.



p-ANCA-Associated Vasculitis Caused by Levamisole-Adulterated Cocaine: A Case Report.  


A rare case of a patient with an unusual skin rash after using cocaine is presented. A clinical diagnosis of levamisole-induced vasculitis was made based on initial presentation of purpuric skin lesions involving the ears and positive cocaine on urine toxicology screening. The diagnosis was confirmed after laboratory findings of neutropenia, positive serum levamisole, and a histopathologic test of the skin lesions. The pathophysiology of this condition is discussed. Treatment with methylprednisolone and prednisone led to the resolution of the neutropenia and complete recovery of the skin lesions. With the growing use of levamisole-tainted cocaine, clinicians should be aware of the symptoms of vasculitis and neutropenia induced by this combination of drugs to avoid unnecessary tests and delayed diagnosis. PMID:23476825

Carter, Michelle R; Amirhaeri, Sorour



B-cell-targeted therapies in systemic lupus erythematosus and ANCA-associated vasculitis: current progress.  


B cells play a central role in the pathogenesis of systemic lupus erythematosus and anti-neutrophil cytoplasmic antibody-associated vasculitis. There are various strategies for targeting B cells including depletion, inhibition of survival factors, activation and inhibition of co-stimulatory molecules. Controlled trials in systemic lupus erythematosus have shown positive results for belimumab, promising results for epratuzumab and negative results for rituximab. The failure of rituximab in controlled trials has been attributed to trial design, sample size and outcome measures rather than true inefficacy. In anti-neutrophil cytoplasmic antibody-associated vasculitis, rituximab is effective for remission induction and in relapsing disease. However, the optimal long-term re-treatment strategy remains to be determined. Over the next 5 years, evidence will be available regarding the clinical efficacy of these novel therapies, biomarkers and their long-term safety. PMID:23971754

Md Yusof, Md Yuzaiful; Vital, Edward Mj; Emery, Paul



Breast vasculitis presenting as a tumor-like lesion. A case report.  


Breast vasculitis presenting as a tumor-like lesion is rare. The differential diagnosis in these cases can be extremely difficult but is very important for treatment and follow-up. We report the case of a 80-year-old woman who was admitted to our service with a lesion resembling an inflammatory carcinoma of the breast. We discuss the pathological and clinical characteristics, the presentation, assessment and treatment of this case and its resolution. Few reports in the literature have addressed the possible occurrence of systemic vasculitis mimicking cancerous lesions. The most common location of such lesions was shown to be the breast in one review. Although rare, such manifestations can be effectively recognized and treated. PMID:16277108

Famŕ, Fausto; Piquard, Arnaud; Fedele, Francesco; Gioffrč Florio, Maria Antonietta


Animal models of anti-neutrophil cytoplasmic antibody-associated vasculitis  

PubMed Central

OTHER ARTICLES PUBLISHED ON ANCA IN THIS ISSUE How anti-neutrophil cytoplasmic autoantibodies activate neutrophils. Clinical and Experimental Immunology 2012, 169: 220–8. Antibodies against neutrophil proteins myeloperoxidase (MPO) and proteinase 3 are thought to cause disease in anti-neutrophil cytoplasmic antibody (ANCA) vasculitis. There have been a number of recent developments in the animal models of ANCA vasculitis in both mice and rats. These include models based on an immune response to MPO generated in MPO-deficient mice, with other models using MPO-sufficient mice and rats. In addition, there is a report of the use of humanized mice where immunodeficient mice have been engrafted with human haematopoietic stem cells and injected with patient ANCA. Antibodies to another protein lysosomal-associated protein-2 have been found in patients with ANCA vasculitis, and evidence from a rat model suggests that they are also pathogenic. These models all have advantages and disadvantages, which are discussed. We also consider what these models have taught us about the pathogenesis of ANCA vasculitis. Experiments using genetically modified mice and pharmacological inhibition have given insights into disease mechanisms and have identified potential therapeutic targets. Toll-like receptor stimulation modifies disease by acting both at the level of tissue injury and in the generation of the autoimmune response. Complement is also potentially important with data to support the role of the alternative pathway and C5a in particular. Intracellular pathways have been examined, with a role showing p38 mitogen-activated protein kinase and phosphatidylinositol 3-kinase ?. Serine proteases are now known to contribute to disease by release of interleukin-1? in ANCA-activated neutrophils and monocytes. Other potential therapies studied in these models include the use of bortezemib and strategies to modify antibody glycosylation.

Coughlan, A M; Freeley, S J; Robson, M G



[Prostatic vasculitis, an unusual onset of panarteritis nodosa: presentation of 2 cases].  


Prostatic involvement in nodose panarteritis is considered as exceptional, especially as the onset of the disease. We discuss two cases which made their debut with fever, weight loss and urinary symptomatology, simulating a prostatic neoplasia. The pathology study of the ressected prostatic sample showed in both cases a necrotizing vasculitis which yielded the diagnosis of nodose panarteritis, and to establish the specific treatment with steroids an immunosuppressants. PMID:8101993

Corralés Torres, A J; el Amrani, A; Aranegui Lasuen, P; Rivera Cívico, F; Arrebola Nacle, J P; Jiménez-Alonso, J



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

Microsoft Academic Search

Although infections are a major concern in patients with primary systemic vasculitis, actual knowledge about risk factors\\u000a and evidence concerning the use of anti-infective prophylaxis from clinical trials are scarce. The use of high dose glucocorticoids\\u000a and cyclophosphamide pose a definite risk for infections. Bacterial infections are among the most frequent causes of death,\\u000a with Staphylococcus aureus being the most

Frank Moosig; Julia U Holle; Wolfgang L Gross



Direct Immunofluorescence in Cutaneous Vasculitis: Experience from a Referral Hospital in India  

PubMed Central

Background: Cutaneous vasculitis is commonly recognized and biopsied, owing to ease of access. Most biopsies are also subjected to direct immunofluorescence (DIF), though the rates of positivity vary. This is an attempt to assess the utility of DIF and glean data that will help optimize the test. Objective: To assess the diagnostic utility of DIF in cutaneous vasculitis. Materials and Methods: All cases of suspected cutaneous vasculitis submitted for DIF between 2004 and 2010 were included. Clinical data, histopathologic diagnosis, DIF findings and additional tests such as anti nuclear antibody (ANA), anti neutrophil cytoplasmic antibody (ANCA) (where done) were noted. Results: There were 198 patients in the study group, with a female predominance. Purpura was the commonest clinical presentation. Extracutaneous involvement was noted in 29% of patients’ i.e., joint pain, abdominal pain and hematuria. Leukocytoclastic vasculitis was the commonest histologic diagnosis. DIF showed an overall positivity of 39% (n = 77) with C3 in 26% (n = 52) and IgA in 23% (n = 46) cases. Forty one cases of suspected Henoch Schonlein Purpura (HSP) showed IgA positivity. The timing of biopsy ranged from <3 days to six months, with 38% being done within seven days. DIF was positive in 86% of biopsies performed within seven days of onset of lesions. Sixty percent of patients with extracutaneous manifestations showed deposits. Vascular deposits were also noted in dermatitis herpetiformis, dematomyositis and prurigo. Conclusion: DIF positivity is strongly influenced by the timing of the biopsy and the presence of extracutaneous features. Its clinical value is greatest in patients with HSP, being contributory in 90% of cases. Vascular deposits may be seen in non-vasculitic conditions and need clinicopathologic correlation.

Nandeesh, BN; Tirumalae, Rajalakshmi



Simultaneous presentation of hemophagocytic syndrome and mesenteric vasculitis in a patient with systemic lupus erythematosus.  


We report an 18-year old female patient with systemic lupus erythematosus (SLE), who developed fever, pancytopenia, abdominal pain, and watery diarrhea. Computed tomography (CT) and bone marrow aspirate revealed lupus mesenteric vasculitis (LMV) and hemophagocytic syndrome (HPS). Serologic tests for Epstein-Barr virus (EBV) indicated its reactivation. This case demonstrates that HPS and concomitant LMV associated with viral reactivation can occur as clinical manifestations of SLE flare. PMID:21229376

Moon, Su-Jin; Kwok, Seung-Ki; Park, Kyung-Su; Kim, Wan-Uk; Park, Sung-Hwan; Kim, Ho-Youn



Juvenile systemic lupus erythematosus and dermatomyositis associated with urticarial vasculitis syndrome: a unique presentation  

Microsoft Academic Search

To report a case of triple association of juvenile systemic lupus erythematosus (SLE), juvenile dermatomyositis and urticarial\\u000a vasculitis as well as a review of the relevant literature. A 12-year-old male patient diagnosed with overlap syndrome between\\u000a SLE and juvenile dermatomyositis since 2004 evolved with erythematous plaques, which were compatible with an urticarial rash.\\u000a Clinical, laboratory and histopathological findings indicated a

Patrícia A. Macędo; Carolina B. Garcia; Monique K. Schmitz; Levi H. Jales; Rosa M. R. Pereira; Jozélio F. Carvalho


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

PubMed Central

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.

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



Revisiting ANCA-associated vasculitis in systemic sclerosis: clinical, serological and immunogenetic factors.  


Objectives. To define the clinical, serological, histological and immunogenetic features of patients with scleroderma and ANCA-associated vasculitis (AAV). Methods. We examined a clinical database of 2200 patients with either limited or diffuse cutaneous systemic sclerosis (SSc). Patients with a confirmed diagnosis of vasculitis who were ANCA positive with either MPO or PR3 reactivity had their clinical features, serology, histology and HLA haplotypes examined in detail. Results. From this SSc cohort, 35 patients (1.6%) had evidence of vasculitis, and the SSc autoantibody profiles in this group were comparable to those previously published from the whole cohort. Of these 35 patients, 8 (0.4% of whole SSc cohort) had either anti-MPO or anti-PR3 antibodies and two further patients were ANCA positive without defined specificities. Of the eight ANCA-positive patients, seven had limited cutaneous SSc and anti-MPO antibodies and only one had anti-PR3 antibodies, associated with diffuse cutaneous SSc. Two ANCA-positive patients had anti-U3RNP antibodies, usually associated with overlap disease. None of the patients had granulomatous disease. The majority had glomerulonephritis, renal arteritis and pulmonary fibrosis. There were several shared HLA haplotypes from the DP and DQ loci in these overlap patients. Conclusion. SSc in overlap with ANCA-associated vasculitis is rare, and clinical features are more mixed than when either of these two conditions occurs separately. From our database, U3RNP antibodies may be more associated with overlap AAV than the other scleroderma-specific antibodies. PMID:23832959

Derrett-Smith, Emma C; Nihtyanova, Svetlana I; Harvey, Jennifer; Salama, Alan D; Denton, Christopher P



Addition of Infliximab to Standard Therapy for ANCA-Associated Vasculitis  

Microsoft Academic Search

Background: Tumour necrosis factor-? (TNF) is implicated in the pathogenesis of anti-neutrophil cytoplasm antibody-associated vasculitis (AAV). Current immunosuppressive therapy is associated with considerable morbidity and mortality. Anti-TNF antibody therapy (infliximab) may help control AAV by providing more targeted immunosuppression and allow reductions in the use of corticosteroids and cyclophosphamide, thereby reducing the burden of immunosuppression with its associated morbidity and

Matthew D. Morgan; Mark T. Drayson; Caroline O. S. Savage; Lorraine Harper



Leucocytoclastic vasculitis associated with Staphylococcus intermedius in the pastern of a horse.  


A pregnant quarterhorse mare became acutely lame as a result of severe swelling of its right hind leg, thought to have been caused by a fracture or a muscle tear. Diagnostic procedures ruled out a traumatic musculoskeletal cause and a physical examination revealed chronic pastern dermatitis ('scratches'/'grease heel'). Histopathological evaluation of biopsy samples from the right hind leg was consistent with a leucocytoclastic vasculitis, and culture yielded Staphylococcus intermedius. The treatment and infectious causes of pastern dermatitis are discussed. PMID:15937241

Risberg, A I; Webb, C B; Cooley, A J; Peek, S F; Darien, B J



Necrotizing peripheral vasculitis/vasculopathy following the use of cocaine laced with levamisole.  


The objective of this study was to describe a novel presentation of peripheral vasculitis associated with levamisole-adulterated cocaine. Cocaine abuse is widespread in the United States with 5.3 million people using cocaine in 2008. Over the past decade, drug enforcement officials have noticed the presence of levamisole in confiscated cocaine samples as an adulterant. Known side effects of cocaine-related levamisole ingestion have included agranulocytosis and a cutaneous acral purpura that is histopathologically characterized by a mixture of inflammation (vasculitis) and occlusion (vasculopathy). A 54-year-old man who nasally ingested cocaine laced with levamisole developed widespread necrotic/purpuric skin lesions on approximately 20% of his body with an acral accentuation. These lesions were complicated by multiple areas of sloughing and necrosis. He was initially treated with topical silver sulfadiazine dressing changes but progressed to require debridement and split-thickness skin grafting. Peripheral vasculitis/vasculopathy with severe necrosis resembling Coumadin necrosis is a relatively recently recognized sequelae from levamisole-adulterated cocaine use. PMID:22138808

Farmer, Russell W; Malhotra, Paula S; Mays, Michael P; Egger, Michael E; Smith, Jason W; Jortani, Saeed A; Spiller, Henry; Bosse, George M; Callen, Jeffrey P; Franklin, Glen A


ANCA-associated vasculitis in systemic sclerosis report of 3 cases.  


The aim of the study was to describe the occurrence of anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) in systemic sclerosis (SSc) patients. SSc patients who developed biopsy-proven AAV were identified. Their clinical manifestations, autoantibodies, presentation with vasculitis, treatment and outcome were described and compared with previously reported patients with these two conditions. Of 985 patients, 3 were identified. All patients had interstitial lung disease, and all presented with acute renal failure, proteinuria and hematuria, and were P-ANCA- and anti-Scl-70-positive. One required hemodialysis. Two were hypertensive; additionally, one patient had sinusitis, and another had monoarthritis and a macular rash. All were treated with high-dose corticosteroids and responded to therapy and attained remission at 6 months. At 1 year, one patient died of pneumonia. ANCA-associated vasculitis is a rare but serious finding in SSc patients. Positive anti-Scl-70 antibody is found commonly in these patients. Different treatment modalities are effective. Serious infections can complicate therapy and lead to death. PMID:22238029

Omair, Mohammed A; Mohamed, Nasreen; Johnson, Sindhu R; Ahmad, Zareen; Lee, Peter



Recurrent ALK-negative anaplastic large T-cell lymphoma presenting as necrotizing vasculitis.  


Anaplastic large cell lymphoma (ALCL) is a T-cell lymphoma histologically characterized by expression of CD30, a cell surface receptor present on activated T cells and B cells. ALCL may occur in a primary cutaneous form or as systemic ALCL with lymph node involvement. Anaplastic lymphoma kinase (ALK) is a tyrosine kinase that induces neoplastic transformation as a result of translocational fusion with an activating promoter. The presence of ALK can be used to distinguish between primary cutaneous ALCL and systemic nodal ALCL in certain cases. Primary cutaneous and systemic ALCL metastatic to the skin are histologically indistinguishable. "Leukemic vasculitis"--an uncommon finding in cases of cutaneous leukemia and even more exceptional in cutaneous lymphoma--refers to a pattern of vasculitis occurring as a direct result of infiltrating neoplastic cells. We report a fatal case of recurrent ALK-negative ALCL presenting as ulcerating skin lesions in a patient previously treated with the new anti-CD30 agent brentuximab vedotin. Biopsy revealed a necrotizing vasculitis resulting from the infiltration of neoplastic cells reminiscent of the patient's primary malignancy. We review the clinical and pathological findings of ALCL and present this case to highlight a subtle diagnostic clue in assessing recurrence of cutaneous lymphoma. PMID:23291583

Nambudiri, Vinod E; Aboutalebi, Amir; Granter, Scott R; Saavedra, Arturo



Percutaneous Management of Occlusive Arterial Disease Associated with Vasculitis: A Single Center Experience  

SciTech Connect

The purpose of this study was to evaluate the safety and effectiveness of percutaneous transluminal angioplasty for occlusive arterial disease associated with vasculitis. Eleven patients(10 women, 1 man; ages 35-82 years) with the diagnosis of vasculitis of the large vessels underwent interventional treatment during intraarterial angiography. The causes included giant cell arteritis(n = 8) and Takayasu arteritis (n = 3).Thirty-three occlusive lesions (including brachiocephalic and renalarteries, and arteries of upper and lower extremities) were treated with balloon angioplasty and/or stent placement. Follow-up included clinical examination, angiography, and color duplex ultrasound.Technical success was 100% (25/25) for stenoses and 50% (4/8) for occlusive lesions, representing all lesions combined from different anatomic locations. Dissection (n = 3) and arterial rupture with retroperitoneal hematoma (n = 1) was found in three patients. During follow-up (mean 12 months), restenoses(n = 8) and re-restenoses (n = 1)occurred in 8 vascular areas. Three of these lesions were treated with repeated PTA (n = 4). The cumulative primary clinical success rate was 67.6%, cumulative secondary success rate 74.4%, and cumulative tertiary success rate 75.9%. Interventional therapy in systemic vasculitis provides promising results in technical success rates and followup. Angioplasty may result in arterial injury, but the rate of complications is low.

Both, M.; Jahnke, T. [Department of Radiology, Christian-Albrechts-University of Kiel, Kiel(Germany); Reinhold-Keller, E. [Department of Rheumatology, University of Luebeck, Rheumaklinik Bad Bramstedt (Germany); Reuter, M.; Grimm, J.; Biederer, J.; Brossmann, J. [Department of Radiology, Christian-Albrechts-University of Kiel, Kiel (Germany); Gross, W.L. [Department of Rheumatology, University of Luebeck, Rheumaklinik Bad Bramstedt (Germany); Heller, M.; Mueller-Huelsbeck, S. [Department of Radiology, Christian-Albrechts-University of Kiel, Kiel (Germany)



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

PubMed Central

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.



CpG oligodeoxynucleotide stimulates production of anti-neutrophil cytoplasmic antibodies in ANCA associated vasculitis  

PubMed Central

Background Wegener's Granulomatosis and Microscopic Polyangiitis are life-threatening systemic necrotizing vasculitides of unknown aetiology. The appearance of circulating antibodies to neutrophil cytoplasmic antigens (ANCA) is strongly associated with the development of the disease. A link between infection and disease has long been suspected, and the appearance of ANCA antibodies has been reported following bacterial and viral infections. The depletion of circulating B cells with monoclonal antibody therapy can induce remission, and this observation suggests a pathogenic role for B cells in this disease. As bacterial DNA is known to induce B cell proliferation and antibody production via TLR-9 stimulation, we have explored the possibility that unmethylated CpG oligodeoxynucleotide, as found in bacterial and viral DNA, may play a role in stimulating circulating autoreactive B cells to produce ANCA in patients with vasculitis. Results We have confirmed that unmethylated CpG oligonucleotide is a potent stimulator of antibody production by PBMC in vitro. The stimulation of PBMC with CpG oligonucleutides resulted in the production of similar amounts of IgG in both ANCA+ patients and normal controls. In spite of this, PR3 ANCA+ patients synthesised significantly higher amount of IgG ANCA than normal controls. In MPO ANCA+ patients, there was a tendency for patients to produce higher amount of ANCA than controls, however, the difference did not reach significance. Furthermore, we were able to detect circulating MPO-reactive B cells by ELISpot assay from the peripheral blood of 2 MPO+ ANCA vasculitis patients. Together, this indicates that circulating anti-neutrophil autoreactive B cells are present in ANCA+ vasculitis patients, and they are capable of producing antibodies in response to CpG stimulation. Of note, CpG also induced the production of the relevant autoantibodies in patients with other types of autoimmune diseases. Conclusion Circulating ANCA autoreactive B cells are present in patients with ANCA+ vasculitis. The production of ANCA from these cells in response to unmethylated CpG stimulation lead us to propose that stimulation of these cells by immunostimulatory DNA sequences such as CpG oligodeoxynucleotide during infection may provide a link between infection and ANCA associated vasculitis. This phenomenon may also apply to other antibody mediated autoimmune diseases.

Hurtado, Plinio R; Jeffs, Lisa; Nitschke, Jodie; Patel, Mittal; Sarvestani, Ghafar; Cassidy, John; Hissaria, Pravin; Gillis, David; Au Peh, Chen



Anti-proteinase 3 anti-neutrophil cytoplasm autoantibodies recapitulate systemic vasculitis in mice with a humanized immune system.  


Evidence is lacking for direct pathogenicity of human anti-proteinase-3 (PR3) antibodies in development of systemic vasculitis and granulomatosis with polyangiitis (GPA, Wegener's granulomatosis). Progress in study of these antibodies in rodents has been hampered by lack of PR3 expression on murine neutrophils, and by different Fc-receptor affinities for IgG across species. Therefore, we tested whether human anti-PR3 antibodies can induce acute vasculitis in mice with a human immune system. Chimeric mice were generated by injecting human haematopoietic stem cells into irradiated NOD-scid-IL2R??/? mice. Matched chimera mice were treated with human IgG from patients with: anti-PR3 positive renal and lung vasculitis; patients with non-vasculitic renal disease; or healthy controls. Six-days later, 39% of anti-PR3 treated mice had haematuria, compared with none of controls. There was punctate bleeding on the surface of lungs of anti-PR3 treated animals, with histological evidence of vasculitis and haemorrhage. Anti-PR3 treated mice had mild pauci-immune proliferative glomerulonephritis, with infiltration of human and mouse leukocytes. In 3 mice (17%) more severe glomerular injury was present. There were no glomerular changes in controls. Human IgG from patients with anti-PR3 autoantibodies is therefore pathogenic. This model of anti-PR3 antibody-mediated vasculitis may be useful in dissecting mechanisms of microvascular injury. PMID:22247758

Little, Mark A; Al-Ani, Bahjat; Ren, Shuyu; Al-Nuaimi, Hamad; Leite, Maurilo; Alpers, Charles E; Savage, Caroline O; Duffield, Jeremy S



Autoantibodies against endothelial cells, extracellular matrix, and human collagen type IV in patients with systemic vasculitis.  


Endothelial cells and subendothelial matrix (ECM) are involved in the pathogenesis of vasculitis. Exposure of the ECM following vascular damage may promote further immune and inflammatory response. To investigate this, we studied the prevalence of antibodies against endothelial cells (AECA), ECM, and its major component collagen type IV in systemic vasculitis patients. Seventy-one percent of patients had AECA (binding index, means +/- SD: 64.8 +/- 48.1%; normal controls: 8.9 +/- 6.9%, P < 0.001). Anti-ECM and anti-collagen type IV antibodies were also significantly higher in patients compared to normals (anti-ECM: 28.6 +/- 29.6% vs 9.0 +/- 11.3%, P < 0.002; anti-collagen type IV: 23.5 +/- 20.3% vs 8.1 +/- 9.1%, P < 0.002). AECA correlated with anti-ECM (r = 0.75, P < 0.0001) but not with anti-collagen type IV. Anti-ECM correlated with anti-collagen type IV (r = 0.45, P < 0.01). Positivity of cytoplasmic anti-neutrophil cytoplasmic antibodies (cANCA) was significantly lower in patients positive for anti-ECM and/or anti-collagen type IV antibodies (58% vs 11%, P = 0.048). AECA binding was partially reduced with ECM incubation by 25.1%. The addition of heparin caused a dose-dependent inhibition of binding activity (19.2-30.6%) in the AECA ELISA. These results support the hypothesis that there is a humoral response against ECM components in addition to endothelial cells in systemic vasculitis patients which might have pathological significance in vascular damage. PMID:8313657

Direskeneli, H; D'Cruz, D; Khamashta, M A; Hughes, G R



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

PubMed Central

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

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



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

PubMed Central

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

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



gammadelta T-cells: basic features and potential role in vasculitis.  


gammadelta T-cells are a numerically small subset of T-cells with distinct features. They recognise antigens that are not seen by other immune cells. At the functional level, gammadelta T-cells share some features with alphabeta T-cells but also exert functions that are otherwise performed by specialised subsets of alphabeta T-cells (e.g. IL-17 production, regulatory activity). We discuss the potential role of gammadelta T-cells in various clinical forms of vasculitis. PMID:20412714

Kabelitz, Dieter; Fazio, Juliane; Adam-Klages, Sabine; Marget, Matthias; Oberg, Hans Heinrich; Wesch, Daniela; Lamprecht, Peter


Mixed connective tissue disease associated with skin defects of livedoid vasculitis.  


A 21-year-old woman who had a 2-year history of mixed connective tissue disease (MCTD) developed rapidly evolving ulcers consistent with livedoid vasculitis (LV) in all distal extremities. She presented clinically with Raynaud's phenomenon, polyarthritis and swollen hands; serologically with high titres of ANA and anti-nRNP; and immunogenetically with HLA-DR4 and HLA-DR53. Although there was initial success in treatment except for the skin defects over the ankles, the patient died from disseminated intravascular coagulation. We suggest that LV may be a poor prognostic manifestation in MCTD. PMID:11055828

Oh, Y B; Jun, J B; Kim, C K; Lee, C W; Park, C K; Kim, T Y; Yoo, D H; Kim, S Y



Muscle restricted vasculitis causing dropped head syndrome: a case report and review of the literature.  


A 52-year-old man presented with a severe head drop and proximal extremity weakness. Magnetic resonance imaging of the cervical spine showed T2 hyperintensity in cervical paraspinal muscles. Electrodiagnostic studies revealed an axial myopathy isolated to paraspinal muscles. A splenius capitis muscle biopsy confirmed an acute myopathy associated with nonsystemic vasculitis. The patient improved on steroids, intravenous immunoglobulin, and monthly pulse doses of cyclophosphamide. Our case emphasizes that a subgroup of patients with dropped head syndrome have treatable conditions. PMID:22538306

Nielsen, Amy Almaraz; Smith, Benn E; Engel, Andrew G; Bosch, Erich Peter



alpha 1-Antitrypsin (AAT) deficiency and ANCA-positive systemic vasculitis: genetic and clinical implications.  


A high incidence of alpha 1-antitrypsin (AAT) deficiency has been reported in patients with C-ANCA systemic vasculitis in association with antibodies against proteinase-3 (PR3). To clarify the role of AAT deficiency in the acute vasculitic process as well as in progression of the disease, we studied 84 patients with either C-ANCA or P-ANCA vasculitis with special reference to: (a) the AAT gene, (b) the phenotypic (Pi) variants and (c) the serum levels during both acute illness and remission. The PiZ gene was found in six patients (8% vs. 1.5% controls) irrespective of the type of autoantibodies (C-ANCA vs. P-ANCA). All PiZ patients displayed the ability to raise their AAT serum levels up to the normal range during acute illness. In contrast, 24 patients with the PiM phenotype presented low AAT serum levels during acute illness. In all these patients, the AAT levels returned to normal values during the remission. Low AAT levels were associated with low levels of C-reactive protein (PCR) (P < 0.001), with a less severe renal involvement or a minor risk of death, and, in one tested patient, with a novel point mutation (TCGA-->TCAA) at the enhancer-promoter region of the AAT gene. Low AAT serum levels did not correlate with either type/titre of autoantibody or distribution/severity of the vasculitis process. In the case-control study, high AAT levels emerged as a major determinant of progression towards end-stage renal failure [odds ratio 3 (95% CI 1.1-8.4)]. These results indicate: (a) a high incidence of the PiZ gene of AAT in systemic vasculitis irrespective of the type of autoantibodies; (b) a novel form of AAT deficiency associated with the normal PiM phenotype becoming manifest only during acute illness; (c) dysregulation of the acute-phase response affecting selectively AAT or both AAT and PCR; (d) correlation between low plasma levels of AAT and less severe renal involvement or risk of death. PMID:9279535

Callea, F; Gregorini, G; Sinico, A; Consalez, G G; Gonzales, G; Bossolasco, M; Salvidio, G; Radice, A; Tira, P; Candiano, G; Rossi, G; Petti, A; Ravera, G; Ghiggeri, G; Gusmano, R



[A case of a patient with rheumatoid vasculitis who was successfully transferred to home-care services through regional cooperation].  


We investigated a case of a patient with rheumatoid vasculitis who was successfully transferred to home-care services through regional cooperation. The patient was a 63-year-old woman with rheumatoid vasculitis complicated by small bowel necrosis, cerebral infarction, heart failure, scleritis, etc. In the present system, admission of such patients to acute or long-term care hospitals is difficult. It is also difficult to provide care at welfare facilities.Although symptom control and prognosis have improved owing to medical advances, the care system lags behind in terms of improvement. In the future, strengthening the seamless coordination between the medical and care systems is needed. PMID:23268916

Ichihara, Toshiaki; Sato, Kohei; Goto, Kazuya



Vasculitis secondary to presumptive leptospirosis treated with long-term corticosteroids in a captive lesser kudu (Tragelaphus imberbis australis).  


A 2-yr-old female lesser kudu (Tragelaphus imberbis australis) was presented for lethargy. Empirical antibiotic treatment appeared to improve its clinical signs, although no etiology for the symptoms was determined. The kudu again presented with lethargy, diffusely swollen limbs, and subcutaneous ecchymoses of 1 day's duration after completion of the initial therapy. Vasculitis secondary to presumptive leptospirosis infection was diagnosed based on a skin biopsy and decreasing paired serologic titers for Leptospira grippotyphosa. The vasculitis was responsive to intramuscular antibiotic therapy and dexamethasone treatment. This case provides evidence that corticosteroids can be used in ruminants at moderate doses for chronic treatment without clinically relevant detrimental effects. PMID:20945659

Fogelberg, Katherine; Ferrell, Shannon T



Clinical course and outcome predictors in pauci-immune ANCA-positive renal-limited vasculitis.  


Our aim was to assess the clinical course and outcome of ANCA-positive, pauci-immune renal limited vasculitis, their correlation with laboratory and histopathologic parameters recorded at initial and follow up testing, and to identify the possible outcome predictors. The study included 17 patients with renal biopsy, clinical, serologic and histopathologic parameters meeting the criteria for pauci-immune ANCA-positive glomerulonephritis without extrarenal manifestations of the disease. Creatinine clearance, 24-hour proteinuria and ANCA titer by ELISA method were determined at disease onset, during treatment and at the end of follow up. In 15 patients, the diagnosis was verified by kidney biopsy. Data were processed by Spearman correlation coefficient and Mann-Whitney test, and survival by Kaplan-Meier test. Lower percentage of glomeruli affected with vasculitis, better initial renal function as measured by serum creatinine or creatinine clearance, and lower chronicity on biopsy were identified as favorable indicators of kidney function outcome. Therapy responders had highest initial and lowest final 24-h proteinuria. The highest level of final 24-h proteinuria was recorded in dialysis dependent patients. The cumulative one-year and two-year patient and kidney survival rate was 64% and 50%, and 64% and 38%, respectively. PMID:22649876

Rupci?, Vesna; Jaki?, Marko; Rupci?, Vinka; Vizjak, Vedrana; Fijacko, Mirjana



Primary intraocular central nervous system lymphoma masquerading as diffuse retinal vasculitis.  


A 49-year-old woman had blurred vision and floaters of 4 days duration in the right eye. Ocular examination revealed granulomatous panuveitis, vitritis and diffuse retinal vasculitis. Following a strongly positive tuberculin skin test, she received antitubercular therapy with oral steroids and immunosuppressants. A year later, despite therapy, vitritis and vasculitis persisted. Additionally, yellowish white lesions appeared beneath the retinal pigment epithelium. Fluorescein angiography revealed a leopard skin appearance. Following a negative vitreous biopsy, she was subjected to a chorioretinal biopsy which revealed non-Hodgkin's lymphoma. MRI was normal. The ocular lesions resolved following intravitreal methotrexate injections. MRI of the brain was repeated every 3 months to rule out central nervous system (CNS) involvement. About 2.5 years after initial presentation, she complained of ataxia, hypersomnia and speech difficulty. MRI of the brain now showed lesions in the thalamocapsular region and the corpus callosum splenium suggestive of CNS lymphoma. She underwent a whole brain radiation with symptomatic improvement followed by chemotherapy. PMID:23632616

Katoch, Deeksha; Bansal, Reema; Nijhawan, Raje; Gupta, Amod



Rapidly progressive glomerulonephritis in a child with Henoch-Sch?nlein Vasculitis and familial Mediterranean fever  

PubMed Central

Henoch-Schonlein Vasculitis (HSV) is systemic small vessel vasculitis involving the skin, kidney, joints, and gastrointestinal tract. The proportion of patients reported to have renal involvement varies between 20% and 80%. Rapidly progressive glomerulonephritis (RPGN)is rare syndrome in children, characterized by clinical features of glomerulonephritis (GN) and rapid loss of renal function. We present a severe kidney involvement in a 14 year old boy with HSV in who is carring MEFV mutation. A 14 year old boy had developed sudden onset of palpable purpuric rash on his extensor surfaces of lower extremities. He had elevated an erythrocyte sedimentation rate (ESR) (45 mm/h), C-reactive protein (3.74 mg/dl), serum urea 66 mg/dl, serum creatinine 1.8 mg/dl. Also, he had hypocomplementemia. Antinuclear antibody, anti ds DNA, antineutrophil cytoplasmic antibody, anticardiolipine antibodies were negative. Urinalysis revealed macroscopic hematuria and proteinuria with a 24-h urinary protein excretion of 55 mg/m2/h. The renal biopsy specimen showed crescentic and necrotizing glomerulonephritis. He had also M694V/E148Q compound heterozygote mutation. Clinical symptoms and renal failure resolved with intermittant hemodialysis and medical therapy.

Sozeri, Betul; Mir, Sevgi; Ertan, Pelin; Kara, Orhan Deniz; Sen, Sait



T cell receptor (TCR) V gene usage in patients with systemic necrotizing vasculitis.  


Wegener's granulomatosis (WG) and polyarteritis nodosa (PAN) are systemic necrotizing vasculitides of unknown etiology. These disorders run a fatal course if untreated. T lymphocytes are implicated in the pathogenesis of WG, since they have been found to infiltrate affected organs, and sIL-2R correlates with disease activity. To elucidate further the role of T cells in necrotizing vasculitis, we have used a panel of 12 TCR V-specific MoAbs to investigate the number of cells expressing certain V alpha and V beta gene segments in the CD4+ and CD8+ subsets of altogether 11 patients with WG or PAN. In the group of patients, we found abnormal expansions of T cells using particular TCR V alpha or V beta gene products. These T cell expansions were more numerous, of a dramatically higher magnitude, and frequently more often found in the CD4 subset, compared with T cell expansions identified in healthy individuals. In long-term studies of the T cell expansions for up to 18 months, a heterogeneous pattern was revealed, with no obvious correlation to clinical features such as disease activity or treatment. Studies of TCR V gene usage in this group of patients may help in understanding the pathogenesis of necrotizing vasculitis, and in the identification of unknown antigens, and may open the possibility to a highly selective immunotherapy by targeting disease-mediating T cells. PMID:7648706

Giscombe, R; Grunewald, J; Nityanand, S; Lefvert, A K



T cell receptor (TCR) V gene usage in patients with systemic necrotizing vasculitis.  

PubMed Central

Wegener's granulomatosis (WG) and polyarteritis nodosa (PAN) are systemic necrotizing vasculitides of unknown etiology. These disorders run a fatal course if untreated. T lymphocytes are implicated in the pathogenesis of WG, since they have been found to infiltrate affected organs, and sIL-2R correlates with disease activity. To elucidate further the role of T cells in necrotizing vasculitis, we have used a panel of 12 TCR V-specific MoAbs to investigate the number of cells expressing certain V alpha and V beta gene segments in the CD4+ and CD8+ subsets of altogether 11 patients with WG or PAN. In the group of patients, we found abnormal expansions of T cells using particular TCR V alpha or V beta gene products. These T cell expansions were more numerous, of a dramatically higher magnitude, and frequently more often found in the CD4 subset, compared with T cell expansions identified in healthy individuals. In long-term studies of the T cell expansions for up to 18 months, a heterogeneous pattern was revealed, with no obvious correlation to clinical features such as disease activity or treatment. Studies of TCR V gene usage in this group of patients may help in understanding the pathogenesis of necrotizing vasculitis, and in the identification of unknown antigens, and may open the possibility to a highly selective immunotherapy by targeting disease-mediating T cells.

Giscombe, R; Grunewald, J; Nityanand, S; Lefvert, A K



The role of IgM rheumatoid factor in experimental immune vasculitis.  

PubMed Central

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.

Floyd, M; Tesar, J T



Clinical and Laboratory Double-blind Investigation on Effect of Fibrinolytic Therapy in Patients with Cutaneous Vasculitis  

Microsoft Academic Search

The effects of phenformin and ethyloestrenol and phenformin and stanozolol on the clinical state, plasma fibrinolytic activity, and fibrinogen-fibrin-related antigen (F.R.-antigen) were compared with placebo in 13 patients with cutaneous vasculitis. Eight patients showed considerable clinical improvement when taking phenformin and an anabolic steroid; an impaired fibrinolytic activity before treatment favoured clinical improvement.

B. Dodman; W. J. Cunliffe; B. E. Roberts; R. Sibbald



Vasculitis neuropathy mimicking lower limb mono-radiculopathy: a study and follow-up of 8 cases.  


The aim of this study was to describe misleading lower limb mono radiculopathy revealing peripheral nerve vasculitis. Retrospective review of eight patients with biopsy confirmed vasculitis presenting as mono-radicululopathy in a tertiary referral centre dedicated to patients with rare peripheral neuropathies. Patients presented with chronic (6/8) or acute (n = 2) radiculopathy in L4, L5 or S1 territories associated with subtle systemic signs. A diagnostic workup was performed because of secondary motor deficit, the absence of clear radicular compression or failure of initial treatment focused on pain relief. In all, nerve conduction studies showed signs of asymmetrical axonal peripheral neuropathy (mononeuritis multiplex). Necrotizing vasculitis was eventually confirmed by peripheral nerve biopsy. Biological markers of inflammation or eosinophilia were present in 5/8 and a progressive motor deficit (7/8) is suggestive of the diagnosis. Under steroid treatment, all patients improved during a mean of 2 years 6 months of follow-up (Mean Rankin score improvement 1.9 point), but five relapsed including three mononeuritis multiplex, and one had acral necrosis. Vasculitis presenting as LL radiculopathy is rare; EMG studies with signs of mononeuritis multiplex and nerve biopsy studies are useful for making the diagnosis. PMID:23054406

Lozeron, Pierre; Lacroix, Catherine; Michon, Mathilde; Theaudin, Marie; Petit Lacour, Marie-Christine; Denier, Christian; Adams, David



Rheumatoid Vasculitis  


... findings in RV – for example, elevations in the erythrocyte sedimentation rate or C-reactive protein – are non- ... findings in RV – for example, elevations in the erythrocyte sedimentation rate or C-reactive protein are non- ...


18F-Fludeoxyglucose PET/CT in the evaluation of large-vessel vasculitis: diagnostic performance and correlation with clinical and laboratory parameters  

PubMed Central

Objective To investigate the diagnostic performance of 18F-fludeoxyglucose (18F-FDG) positron emission tomography (PET)/CT in patients with suspected large-vessel vasculitis and its potential to evaluate the extent and activity of disease. Methods 78 consecutive patients (mean age 63 years; 53 females) with suspected large-vessel vasculitis were evaluated with 18F-FDG PET/CT. 18F-FDG uptake in the aorta and major branches was visually graded using a four-point scale and quantified with standardised uptake values (SUVmax). According to clinical diagnosis, patients were classified into three groups: (a) steroid-naďve, large-vessel vasculitis (16 patients), (b) vasculitis on steroid treatment (18 patients) and (c) no evidence of vasculitis (44 patients). Analysis of variance and linear regression were used to investigate the association of 18F-FDG uptake with clinical diagnosis and inflammatory markers. Results 18F-FDG PET/CT was positive (visual uptake ?2; equal to or greater than liver) in all patients with steroid-naďve, large-vessel vasculitis. The thoracic aorta, the carotid and the subclavian arteries were most frequently involved. In these patients, SUVmax values were significantly higher than in the other groups (analysis of variance; p<0.05). Linear regression showed a significant positive association (b-coefficients: 0.018–0.02; p<0.05) between SUVmax of the thoracic aorta and inflammatory markers in patients with vasculitis (Groups a and b). Patients on steroid treatment showed low visual scores (uptake <2) and significantly lower SUVmax values than steroid-naďve patients. Conclusion 18F-FDG PET/CT can detect the extent and activity of large-vessel vasculitis in untreated patients and is unreliable in diagnosing vasculitis in patients on steroids.

Papathanasiou, N D; Du, Y; Menezes, L J; Almuhaideb, A; Shastry, M; Beynon, H; Bomanji, J B



Current literature and imaging techniques of aseptic lymphocyte-dominated vasculitis-associated lesions (ALVAL).  


Aseptic lymphocyte-dominated vasculitis-associated lesions (ALVAL) are a recognized complication of metal-on-metal bearing hip prostheses. There is an impending concern regarding the future investigation and management of patients who have received such implants. The current literature is discussed, and the current guidelines for management of these patients in the UK are reviewed. The various imaging techniques available, such as computed tomography, metal artefact reduction magnetic resonance imaging, and ultrasound are discussed and evaluated with respect to the assessment of patients with suspected ALVAL. The histopathological findings are discussed with images of the tissue changes provided. Images of the radiological findings are also provided for all general radiological methods. ALVAL and its radiological presentation is an important issue that unfortunately may become a significant clinical problem. PMID:23932675

Duggan, P J; Burke, C J; Saha, S; Moonim, M; George, M; Desai, A; Houghton, R



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


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

Spriggs, Maria; Reeder, Chris



Systemic necrotizing vasculitis and glomerulonephritis in grower pigs in southwestern Quebec.  

PubMed Central

This purpose of this study was to describe the clinical features and the pathological findings of an unusual condition observed in pigs of a fattening unit in southwestern Quebec. Two of these pigs were submitted for complete postmortem examination. The disease was characterized by a systemic necrotizing vasculitis and an exudative and proliferative glomerulonephritis. In the skin, the vascular lesions produced a conspicuous papular dermatopathy with a characteristic distribution. Bacteriological and virological results were inconclusive. In the glomeruli, there were extensive granular complement deposits with scattered immunoglobulin M. Transmission electron microscopy did not reveal any dense deposits in glomerular basement membrane. The cause of this newly recognized and potentially lethal condition remains unknown, although histological and immunopathological observations suggest an immune-mediated process. Images Figure 1. Figure 2. Figure 3. Figure 4. Figure 5.

Helie, P; Drolet, R; Germain, M C; Bourgault, A



Streptococcus pneumoniae meningoencephalitis with bilateral basal ganglia necrosis: an unusual complication due to vasculitis.  


Streptococcus pneumoniae (S pneumoniae) is a common cause of bacterial meningitis, frequently leading to death or severe neurological impairment. We report an exceptional case of a 4-month-old boy with meningitis caused by S pneumoniae. Computed tomography (CT) and magnetic resonance imaging (MRI) examinations of the brain showed bilateral symmetrical necrosis involving the lentiform and caudate nuclei, as well as the thalamus. T1-weighted MR images showed patchy areas of increased signal intensity, consistent with hemorrhagic transformation of the lesions. Autopsy revealed widespread necrosis of the basal ganglia with clear signs of vasculitis. Severe complications of S pneumoniae meningoencephalitis are known in infants but to our knowledge, such lesions in the basal ganglia have only been reported thrice in adults and never in children. PMID:21677202

Magnus, Jessy; Parizel, Paul M; Ceulemans, Berten; Cras, Patrick; Luijks, Marloes; Jorens, Philippe G



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


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

Gatenby, P A



[Acute respiratory distress syndrome related to intra-alveolar hemorrhage revealing a vasculitis].  


Intra-alveolar hemorrhage (IAH) could be revealed by acute respiratory failure. The classic association of hemoptysis - anemia - radiological infiltrates is suggestive and has to be confirmed by broncho-alveolar lavage with Golde score. Etiologies included immune and non-immune diseases, with specific treatment for each. We report a case of IAH revealed by acute respiratory distress syndrome and anemia (3 g/dL), related to pulmonary and cerebral vasculitis without renal involvement. The patient was efficiently treated with corticosteroids and cyclophosphamide. This case highlights the critical role of BAL cytological analysis with Golde score, and the need for a rapid and accurate diagnosis in order to guide specific treatment. If histology is needed, renal biopsy even without renal involvement, or surgical lung biopsy is possible. PMID:22749619

Kerjouan, M; Gacouin, A; Gros, A; Caulet Maugendre, S; Le Tulzo, Y; Delaval, P; Jouneau, S



Retinal vasculitis in two pediatric patients with systemic lupus erythematosus: a case report  

PubMed Central

We report two pediatric female patients with systemic lupus erythematosus (SLE) who presented with decreased vision. Both patients were found to have retinal vasculitis and occlusive disease. The first patient also presented with vitreous hemorrhage and later non-arteritic ischemic optic neuropathy. She was treated with panretinal photocoagulation and steroid therapy and later in her disease course was treated with rituximab and cyclophosphamide. Her vision remained decreased. The second patient was treated with rituximab and monthly cyclophosphamide infusions early in her disease course, and her vision improved dramatically. The difference in the presentations and outcomes of these two pediatric patients with SLE highlights the spectrum of severity of SLE retinopathy. We suggest that early recognition of disease and early intervention with B-cell depletion therapy in addition to a traditional cytotoxic agent should be considered in pediatric patients with SLE and occlusive retinopathy.



alpha1-Antitrypsin deficiency associated with persistent cutaneous vasculitis. Occurrence in a child with liver disease.  


alpha1-Antitrypsin (alpha1-AT) deficiency is an autosomal recessive inherited disease. The serum concentration of the protease inhibitor (Pi) alpha1-AT is controlled by a set of codominant allelic genes, constituting the so-called Pi system. Abnormal conditions reported in connection with severe alpha1-AT deficiency of the PiZZ type have been, in the newborn, cholestasis and progressive juvenile cirrhosis, and in adults, panacinar pulmonary emphysema and liver disease. Skin changes have not been described previously in connection with this disease picture. The case is persistent cutaneous vasculitis in a 2-year-old child with alpha1-AT deficiency of the PiZZ type, heterozygosity for the Duarte variant of galactose-1-phosphate uridyl transferase, and neonatal liver disease. A pathogenetic relationship may exist between the biochemical defects and both the skin and liver diseases. PMID:352272

Brandrup, F; Ostergaard, P A



Four cases of MPO-ANCA-positive vasculitis with otitis media, and review of the literature.  


Otitis media is one of the common organ injuries that appear during the course of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). We experienced four patients with myeloperoxidase (MPO)-ANCA-positive AAV with otitis media. All were elderly Japanese women. MPO-ANCA in our patients was reminiscent of microscopic polyangiitis (MPA), although chest computed tomography (CT) scans revealed characteristics of both granulomatosis with polyangiitis (GPA), showing bronchial lesions and nodule formation, and MPA, showing interstitial changes. Whether our cases should be classified as GPA or MPA is a matter of discussion. We detail their profiles, and review previous literature on MPO-ANCA-positive AAV with otitis media. PMID:22706878

Ono, Nobuyuki; Yoshihiro, Kyoko; Oryoji, Daisuke; Matsuda, Motohiro; Ueki, Yoshihiro; Uezono, Shigehiro; Kai, Yasufumi; Himeji, Daisuke; Niiro, Hiroaki; Ueda, Akira



Does geographic location affect incidence of ANCA-associated renal vasculitis in northern Saskatchewan, Canada?  


Objectives. To compare the incidence of renal ANCA-associated vasculitis (AAV) in urban vs more rural populations in northern Saskatchewan, and as a secondary objective to compare time to diagnosis between these geographic areas. Methods. Northern Saskatchewan has a population of 562 882 of which approximately 260 600 live in the major urban area. A pathology database search for renal biopsy reports suggestive of AAV between January 2007 and December 2011 and subsequent chart review yielded 33 new diagnoses of granulomatosis polyangiitis (GPA) or microscopic polyangiitis (MPA). Data extraction included demographics, residential data, serological status, recorded symptom onset and estimations of BVAS, five-factor score (FFS) and vasculitis damage index (VDI). Results. Of 33 renal AAV cases, 24.2 % (n = 8) lived within the city. The incidence rate for urban residents was 6.1 cases/million/year, and for those residing elsewhere, 16.5 cases/million/year. The odds ratio for the more rural population was 2.69 (95% CI 1.3, 7.5). Mean time to diagnosis was 1.33 (s.d. 0.94) months for urban and 3.52 (s.d. 3.83) months for more rural patients (P = 0.002, 95% CI 0.7, 3.9). Secondary analysis supported these observations with higher BVAS, VDI and FFS scores in patients living outside the urban centre. Conclusion. The incidence of renal biopsy-proven AAV was higher in patients living in northern Saskatchewan smaller communities and rural settings. A significantly longer time to diagnosis existed for patients outside the urban centre and was associated with poorer BVAS, VDI and FFS scores. PMID:23838025

Anderson, Keltie; Klassen, Judy; Stewart, Samuel A; Taylor-Gjevre, Regina M



Pathways to renal biopsy and diagnosis among patients with ANCA small-vessel vasculitis  

PubMed Central

Objectives Antineutrophil cytoplasmic antibody small-vessel vasculitis (ANCA-SVV) is an autoimmune systemic process increasingly recognised since the advent of antibody testing for the disease. Prompt diagnosis and institution of immunosuppressive therapy has been shown to improve patient outcome. The goal of this study was to better understand how patients navigate the health care system from symptom presentation to biopsy diagnosis, and to study the effects of prompt versus delayed diagnosis. Methods Disease symptoms and number of physicians seen prior to renal biopsy were assessed for 127 ANCA-SVV patients. Direct, delayed, and quest pathways to diagnosis and treatment of vasculitis were defined for both patients and providers. Kruskal-Wallis and Fisher exact tests were used to evaluate continual measures and compare categorical variables across pathways. Results Among patients who sought direct care, physician delay in referral to a nephrologist was common (49/127, 71%, p=0.0023). Patients who delayed seeking care also experienced a delayed diagnosis 57% of the time (p=0.0023). Patients presenting with prodromal flu or upper respiratory involvement were more likely to have a delay/quest patient pathway (56% and 55%, respectively) than a direct patient pathway (44%, p=0.033 and 45%, p=0.019, respectively). There was a trend for patients with more severe loss of renal function to have a more direct referral to a nephrologist. Conclusion Delay in diagnosis of ANCA SVV may be due to lack of or non-specific symptoms, especially in patients who present with non-renal manifestations of disease. Better algorithms are needed to identify extra-renal manifestations, expedite diagnosis and improve patient outcomes.

Poulton, Caroline J.; Nachman, Patrick H.; Hu, Yichun; McGregor, JulieAnne G.; Jennette, J. Charles; Falk, Ronald J.; Hogan, Susan L.



Orbital apex syndrome as the presenting feature of large vessel vasculitis.  


Orbital apex syndromes are commonly due to metastases and orbital pseudotumour. We present a patient in whom an orbital apex syndrome was the initial manifestation of a large vessel vasculitis, which became apparent when she subsequently developed an aortic dissection. A 73-year-old lady presented with headaches for three days and high inflammatory markers (ESR 49, CRP 83). Scans showed extensive left sided sinusitis and she was given antibiotics. Despite antibiotics she developed a complete left external ophthalmoplegia, left visual loss, pyrexia and rising inflammatory markers. Steroids were added with clinical improvement and resolving inflammatory markers within ten days (CRP 18, ESR 10). Attempts at steroid withdrawal lead to recurrence of headache. She was discharged on Prednisolone and made a complete recovery with normal inflammatory markers. This was tapered to a maintainance dose of 2.5 mg. After ten months steroids were inadvertently stopped. She developed chest pain and was seen locally on a few occasions. Initial tests were negative for cardiac and respiratory causes, but in retrospect showed a small pleural effusion and rising inflammatory markers over this period (CRP 53 and rising). Two months after stopping steroids worsening chest pain let to a CT scan showing aortic dissection and pleural effusions (ESR 98, CRP 391). She was treated surgically. Aortic histology was consistent with aortitis, but it was not possible to exclude the changes being secondary to the dissection. High dose steroids were restarted with prompt normalisation of inflammatory markers and she has remained well on a small dose of prednisolone. Giant cell arteritis is a recognised cause of aortic dissection and can rarely mimic an orbital apex syndrome. Although the inflammatory changes in the aorta of our patient were not diagnostic, after exclusion of other causes of large vessel vasculitis, we suggest that the orbital apex syndrome, pleural effuions and subsequent aortic dissection were most likely an unusual presentation of giant cell arteritis.(1-5.) PMID:24108889

Garikipati, Venkat; Prevett, Martin



Evaluation of D-dimer serum levels among patients with chronic urticaria, psoriasis and urticarial vasculitis*  

PubMed Central

BACKGROUND It has been demonstrated that neutrophils, eosinophils and monocytes, under appropriated stimulus, may express tissue factor and therefore, activate the extrinsic pathway of coagulation. We performed a transversal and case-control study of patients with chronic urticaria and patients with psoriasis, in our outpatient clinic to evaluate the production of D-dimer. OBJECTIVE To evaluate D-dimer serum levels in patients with chronic urticaria and its possible correlation with disease activity. PATIENTS AND METHODS The study was conducted from October 2010 until March 2011. We selected 37 consecutive patients from our Allergy Unit and Psoriasis Unit, and divided them into three groups for statistical analysis: (i) 12 patients with active chronic urticaria (CU); (ii) 10 patients with chronic urticaria under remission and (iii) 15 patients with psoriasis (a disease with skin inflammatory infiltrate constituted by neutrophils, lymphocytes and monocytes). Another five patients with urticarial vasculitis were allocated in our study, but not included in statistical analysis. The serum levels of D-dimer were measured by Enzyme Linked Fluorescent Assay (ELFA), and the result units were given in ng/ml FEU. RESULTS Patients with active chronic urticaria had the highest serum levels of D-dimer (p<0.01), when compared to patients with CU under remission and the control group (patients with psoriasis). CONCLUSIONS Patients with active chronic urticaria have higher serum levels of D-dimer, when compared to patients with chronic urticaria under remission and patients with psoriasis. We found elevated serum levels of D-dimer among patients with urticarial vasculitis.

Criado, Paulo Ricardo; Antinori, Lidi Che Leon; Maruta, Celina Wakisaka; dos Reis, Vitor Manoel Silva



Anti-Plasminogen Antibodies Compromise Fibrinolysis and Associate with Renal Histology in ANCA-Associated Vasculitis  

PubMed Central

Antibodies recognizing plasminogen, a key component of the fibrinolytic system, associate with venous thrombotic events in PR3-ANCA vasculitis. Here, we investigated the prevalence and function of anti-plasminogen antibodies in independent UK and Dutch cohorts of patients with ANCA-associated vasculitis (AAV). We screened Ig isolated from patients (AAV-IgG) and healthy controls by ELISA. Eighteen of 74 (24%) UK and 10/38 (26%) Dutch patients with AAV had anti-plasminogen antibodies compared with 0/50 and 1/61 (2%) of controls. We detected anti-plasminogen antibodies in both PR3-ANCA– and MPO-ANCA–positive patients. In addition, we identified anti-tissue plasminogen activator (tPA) antibodies in 13/74 (18%) patients, and these antibodies were more common among patients with anti-plasminogen antibodies (P = 0.011). Eighteen of 74 AAV-IgG (but no control IgG) retarded fibrinolysis in vitro, and this associated with anti-plasminogen and/or anti-tPA antibody positivity. Only 4/18 AAV-IgG retarded fibrinolysis without harboring these antibodies; dual-positive samples retarded fibrinolysis to the greatest extent. Patients with anti-plasminogen antibodies had significantly higher percentages of glomeruli with fibrinoid necrosis (P < 0.05) and cellular crescents (P < 0.001) and had more severely reduced renal function than patients without these antibodies. In conclusion, anti-plasminogen and anti-tPA antibodies occur in AAV and associate with functional inhibition of fibrinolysis in vitro. Seropositivity for anti-plasminogen antibodies correlates with hallmark renal histologic lesions and reduced renal function. Conceivably, therapies that enhance fibrinolysis might benefit a subset of AAV patients.

Berden, Annelies E.; Nolan, Sarah L.; Morris, Hannah L.; Bertina, Rogier M.; Erasmus, Dianhdra D.; Hagen, E. Christiaan; Hayes, Donal P.; van Tilburg, Nico H.; Bruijn, Jan A.; Savage, Caroline O.S.; Bajema, Ingeborg M.



Production of antineutrophil cytoplasm antibodies derived from circulating B cells in patients with systemic vasculitis  

PubMed Central

The pathogenesis of systemic vasculitis is complex and is likely to involve many mechanisms. In certain systemic vasculitides, autoimmunity plays an important role with autoantibodies developing towards neutrophils, which are termed antineutrophil cytoplasm antibodies (ANCA). There is a growing body of evidence that T cells may contribute to the pathogenesis of ANCA-associated vasculitides. A system was set up to determine whether B cells require T cell help to produce antibodies in a peripheral blood lymphocyte (PBL) culture system enriched for B cells and dendritic cells (DC). As a control, tetanus toxoid (TT) antibody production was detected from individuals not recently immunized with tetanus vaccine when stimulated with TT antigen. Proteinase 3 (PR3) and myeloperoxidase (MPO) antibodies were produced from B cell and DC enriched cultures prior to the addition of antigen in some ANCA-positive patients with high ANCA titres, but not from patients with low ANCA titres or controls. PBMC from individuals recently immunized with tetanus vaccine were also maximally stimulated in that addition of antibody did not enhance antibody production. We conclude that this system supports a role for T cell help in the production of TT antibodies in individuals not immunized recently with tetanus vaccine. However, in patients with ANCA-associated vasculitis and controls recently immunized with tetanus vaccine, circulating B cells are apparently spontaneously producing autoantibody, possibly reflecting a system already maximally driven in vivo, and therefore masking underlying potential T cell-B cell collaboration. Such B cells may be less responsive to regulatory stimuli in vivo.




[A case of eosinophilic granulomatosis with polyangiitis with extra-vascular granuloma and eosinophilic vasculitis diagnosed by transbronchial lung biopsy].  


A 62-year-old man was suffering from bronchial asthma and referred to our institution with dry cough and dyspnea on exertion in November, 2010. He was diagnosed with eosinophilic granulomatosis with polyangiitis (EPGA, formerly Churg-Strauss syndrome) by chest radiographic findings, blood eosinophilia, mononeuritis multiplex and cardiomyopathy. Steroid therapy was started and he was rapidly improved. Steroid therapy had been tapered off by May, 2012. After 2 months, however, progressive dyspnea, neural symptoms, deafness, re-elevation of blood eosinophils and bilateral multifocal infiltrations appeared. He was re-admitted to our institution. Transbronchial lung biopsy (TBLB) specimens revealed extra-vascular granuloma, eosinophilic vasculitis and eosinophilic pneumonia and we diagnosed him with the reccurence of EGPA. He was improved by steroid pulse therapy, then tapered. This case was the antineutrophil cytoplasmic autoantibodies negative EGPA. The case of EGPA with granuloma and vasculitis diagnosed by TBLB was rare. PMID:23760204

Hara, Yu; Kanoh, Soichiro; Fujikura, Yuji; Kawano, Shuichi; Misawa, Kazuhisa; Kawana, Akihiko



Computed tomography of the brain in cases with venous vasculitis compared with an age-matched reference group.  


Patients with a particular, steroid-sensitive headache and often characteristic pathology at orbital phlebography, have been suggested to suffer from venous vasculitis. Fifty such patients were examined with computed tomography (CT) of the brain. The findings were compared with those of an age-matched reference group selected at random to represent normal subjects. The CT examinations were analyzed with respect to size of lateral ventricles and signs of atrophy. In both groups, there was a significant increase of atrophy with age. There was also a significantly higher degree of atrophy in the patient group as compared with the reference group. The findings indicate that the supposedly underlying venous vasculitis is related to early aging and atrophy of the brain. PMID:2964848

Hannerz, J; Ericson, K; Bergstrand, G; Berggren, B M; Edman, G


Ischaemic jejunal vasculitis during treatment with pegylated interferon-alpha 2b and ribavirin for hepatitis C virus related cirrhosis  

Microsoft Academic Search

A 53-year-old male with compensated cirrhosis (Child–Pugh class A5) and mixed cryoglobulinaemia (cryocrit: 2.0%), both hepatitis C virus-related, was treated with pegylated interferon-alpha 2b and ribavirin. After three months of therapy, he developed segmental jejunal vasculitis requiring emergency resection of an ischaemic intestinal loop 60cm long. Pathological examination of the surgical specimen revealed signs of ischaemic injury with haemorrhagic infarction

M. Pompili; F. Pizzolante; L. M. Larocca; M. Covino; G. L. Rapaccini; G. Gasbarrini



A case of polyarteritis nodosa complicated by left central retinal artery occlusion, ischemic optic neuropathy, and retinal vasculitis  

Microsoft Academic Search

A 23-year-old single female patient developed constitutional manifestations in the form of fever, weight loss, anorexia, malaise,\\u000a fatigue, and generalized aches in January 1995, 2 weeks after an attack of German measles. This was followed by painful, reddish,\\u000a macular skin lesions over both legs which healed by dark pigmentation (leucocytoclastic vasculitis), mononeuritis multiplex,\\u000a and Raynaud’s phenomena of both hands and feet.

Y. Emad; S. Basaffar; Y. Ragab; F. Zeinhom; T. Gheita



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

PubMed Central

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.

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



Systemic mononuclear-cell vasculitis in MRL/Mp-lpr/lpr mice. A histologic and immunocytochemical analysis.  

PubMed Central

The cellular mechanisms governing the expression of mononuclear cell vasculitis are poorly understood. For determination of the precise sequence of events in the development of vasculitis in autoimmune MRL/lpr mice, histologic sections from 4-20-week-old mice were evaluated with a panel of cytochemical and immunohistochemical stains. The results show that vascular disease in MRL/lpr mice develops as follows: Thy 1+, Ly 1+, L3T4- T cells assemble around predominantly small-to-medium muscular arteries at approximately 8 weeks of age. At 12 weeks of age, an adventitial inflammatory focus forms, composed of large "reactive" mononuclear inflammatory cells adjacent to hypertrophied vascular smooth muscle cells (VSMCs). Blastic Thy 1+, Ly 1+, L3T4- T cells subsequently infiltrate the tunica media, and selective VSMC karyolysis results. Occasional cytotoxic/suppressor T cells, macrophages, and possibly NK cells are noted primarily distal to the infiltration site. The outer zone of the inflammatory infiltrate is composed of mature B cells and occasional B-cell precursors. These findings suggest that cellular constituents of the immune response mediate mononuclear cell vasculitis in MRL/lpr mice. Images Figure 1 Figure 2 Figure 3 Figure 4 Figure 5 Figure 6 Figure 7 Figure 8 Figure 9 Figure 10

Moyer, C. F.; Strandberg, J. D.; Reinisch, C. L.



Pulmonary involvement in ANCA-associated vasculitis from the view of the pulmonologist.  


Microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA), and eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) are conditions classified under the general heading of antinuclear cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV). Lung lesion is a very common and important clinical feature in AAV. In MPA, diffuse alveolar hemorrhage and pulmonary fibrosis (PF) are the most frequent manifestations. High-resolution computed tomography (HRCT) chest findings associated with MPA in PF patients demonstrate a high frequency of usual interstitial pneumonia (UIP), fibrotic-nonspecific interstitial pneumonia (F-NSIP), and combined PF and emphysema (CPFE) pattern with honeycombing, traction bronchiectasis, ground-glass opacity, and emphysema. In most of these cases, the histologic pattern of PF has been classified as UIP and/or fibrotic NSIP. In addition, a high incidence of histological findings, such as extensive interstitial fibrosis, lymphoid hyperplasia, and bronchiolitis, are characteristics observed in PF associated with collagen vascular diseases and which are not observed in idiopathic PF (IPF). In some cases, PF precedes the development of MPA. Indeed, there are some cases of pulmonary-limited MPA in this group. Therefore, clinicians should be aware of MPA as an underlying feature of PF in order to avoid overlooking and misdiagnosing this condition as IPF. The median survival time (MST) in UIP pattern/MPA is comparable with that of IPF. In GPA, almost all patients have either upper airway or lower respiratory tract lesions. Solitary or multiple nodules (frequently cavitated) and masses are the most common findings on chest images. Asthma is a cardinal symptom of Churg-Straus syndrome, often preceded by allergic rhinitis. To induce remission, a severity-based regimen was given to patients according to the appropriate protocol of the Japanese patients with myeloperoxidase (MPO)-ANCA-associated vasculitis (JMAAV) study group: low-dose corticosteroid and, if necessary, cyclophosphamide or azathioprine in patients with mild form; high-dose corticosteroid and cyclophosphamide in those with severe form; severe-form regimen plus plasmapheresis in those with the most severe form. PMID:23188194

Homma, Sakae; Suzuki, Aika; Sato, Keita



[A case of Listeria rhombencephalitis with a secondary vasculitis suggested by MRI].  


We reported a rare case of Listeria rhombencephalitis with meningitis. A 48-year-old healthy man suddenly experienced high fever and headache, then he had lower cranial nerve's palsies and mental dysfunction developed during one week period. On admission, his temperature was 38 degrees C. He was slightly delirious and euphoric. He had nuchal rigidity, mild paresthesia over his left cheek to left upper lip, a right sixth nerve palsy, dysphagia, hiccup, nasal voice and left cerebellar ataxia. His tongue deviated toward the right side on protrusion. A CSF culture grew Listeria monocytogenes. Intravenous antibiotic therapy (PIPC, minocycline hydrochloride) produced improvement in one month except for mild paresthesia and dysphagia. He almost recovered after 7 months of illness. Brain MRI on T2 weighted image demonstrated multiple small ischemic lesions in the left lateral medulla, upper pontine tegmentum in the right side, and pontine tegmentum in the left side. These lesions enhanced by Gd. were assumed to be due to the secondary vasculitis. Listeria rhombencephalitis is extremely rare in human beings. To our knowledge only thirteen cases have been reported. In seven cases, post-mortem pathological findings confirmed necrotizing angitis in brainstem. Clinical aspects of Listeria rhombencephalitis were discussed, and the entity of this disease should be considered as a treatable cause of acute progressive brainstem meningoencephalitis. PMID:8403684

Tokonami, F; Imamura, S; Suga, M; Tokunaga, K; Fukuda, Y



Neutrophil and recombinant myeloperoxidase as antigens in ANCA positive systemic vasculitis.  

PubMed Central

Myeloperoxidase (MPO) is one of the major autoantigens recognized by anti-neutrophil cytoplasm antibodies. The association of this antigen with specific disease entities requires that there is a source of pure antigen present in large quantities. Further delineation of the molecular mechanisms involved in the antigen-antibody interaction requires the ability to manipulate the molecule. The expression of recombinant MPO in Chinese hamster ovary cells has produced a source of pure protein, suitable for molecular studies. We have shown that this protein is an antigen recognized by 95% of anti-MPO antibodies from patients with systemic vasculitis. This recombinant molecule will be of use in providing an additional specific solid-phase assay for these antibodies and further forms of this protein which mirror the antigenicity of native MPO more exactly may replace chemically purified antigen. It will also be of great value in studies examining the epitopes recognized by anti-MPO antibodies and in studies of immunoregulation and T cell activation. Images Fig. 2

Short, A K; Lockwood, C M; Bollen, A; Moguilevsky, N



[Internal carotid artery vasculitis originated by cervicofacial actinomycosis as a predisponing factor to stroke].  


Actinomycosis is a chronic, suppurative, fibrotic infection produced by a gram-positive anaerobic bacteria of the normal mouth flora, the infection having tendency to fistulate and to extend without respecting tissue layers. Initial diagnosis is usually delayed due to its painless evolution. Three clinical forms are known: cervicofacial, abdominal and thoracic. Occasionally, there is vascular involvement which is mainly associated with venous thrombotic phenomena with low clinical expresion and hematogenous dissemination that can result in a formation of abscess or meningitis in the central nervous system. We report the case of a 28 year old female patient with type I diabetes and microangiopathyc complication with a internal carotid artery stenosis adjacent to mandibular angle actinomicosis. A few months later and after a miscarriage she suffered from ischemic stroke. The anticardiolipin antibody were positive at the time of stroke with post-operative period and over the next two years. To our knowledge (Medline, 1984-2004), it is the first report of internal carotid vasculitis originated by cervicofacial actinomicosis. We discuss the neuroradiological aspects of our case. PMID:15954037

Aguirre-Sánchez, J J; Portilla-Cuenca, J C; Velicia Mata, M R; Querol-Pascual, R; Palacios Bote, R; Campos de Orellana, A



Evidence for a role of hydroxyl radical in immune-complex-induced vasculitis.  


Previously it was shown that tissue injury occurring in acute immune-complex-induced vasculitis, which is complement and neutrophil-dependent, is significantly attenuated by the presence of catalase, suggesting the pathogenic role of H2O2 generated from activated neutrophils. We now show that significant protection is also afforded by pretreatment of animals with apolactoferrin , a naturally occurring chelator of iron. Iron-saturated lactoferrin is devoid of protective effects. Deferoxamine mesylate, a synthetic iron chelator, also has protective effects. Infusion of ionic iron, especially Fe(III), potentiates the tissue injury. Significant protection from tissue injury is also produced by treatment of rats with dimethyl sulfoxide, a potent hydroxyl radical scavenger. Morphologically, animals treated with these protective interventions show the influx of neutrophils into sites of immune complex deposition, but there is markedly attenuated edema, little or no hemorrhage, and little evidence of endothelial cell injury, in contrast to the findings in nonprotected animals. These data support the suggestion that immune-complex-induced injury may be linked to generation of H2O2 from activated neutrophils and the subsequent conversion of H2O2 to the hydroxyl radical. PMID:6233906

Fligiel, S E; Ward, P A; Johnson, K J; Till, G O



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

PubMed Central

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.

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



Idiopathic retinal vasculitis, arteriolar macroaneurysms and neuroretinitis: clinical course and treatment  

PubMed Central

Background The purpose of the study is to describe the clinical course and treatment of idiopathic retinitis, vasculitis, aneurysms and neuroretinitis. The study utilized non-randomized, retrospective and interventional case series. The eight eyes of six patients were analysed. Testing included wide fluorescein angiography, indocyanine green angiography and systemic evaluation. Treatment involved observation, panretinal laser photocoagulation (PRP) for peripheral retinal ischemia, grid laser for macular oedema and focal laser on the macroaneurysms. The main outcome measures were initial visual acuity (VA), initial stage at diagnosis, clinical course, surgical intervention, final VA, final stage and complications of disease. Results Five out of eight eyes with retinal ischemia in more than two quadrants that were treated with PRP and grid laser for macular oedema maintained excellent VA and demonstrated no progression of retinal ischemia during follow-up. The two eyes which exhibited retinal ischemia in less than two quadrants and macular oedema were treated with grid laser and focal laser on the macroaneurysms, but did not undergo PRP. VA improved by two lines of the Snellen chart, and there was no progression of retinal ischemia during the 3 and 4 years of follow-up. One eye with neither retinal ischemia nor macular oedema was not treated, and the clinical picture remained stable during the follow-up. Conclusion Early PRP may be considered in the presence of angiographic evidence of peripheral retinal non-perfusion. However, treatment could be withheld until the patient develops retinal ischemia in more than two quadrants.



Hydralazine-induced anti-neutrophil cytoplasmic antibody-positive renal vasculitis presenting with a vasculitic syndrome, acute nephritis and a puzzling skin rash: a case report  

PubMed Central

Introduction Anti-neutrophil cytoplasmic antibody-associated vasculitis has been associated with many drugs and it is a relatively rare side effect of the antihypertensive drug hydralazine. The diagnosis and management of patients who have anti-neutrophil cytoplasmic antibody-associated vasculitis may be challenging because of its relative infrequency, variability of clinical expression and changing nomenclature. The spectrum of anti-neutrophil cytoplasmic antibody-associated vasculitis is wide and can be fatal. This case documents a 62-year-old woman who presented with hydralazine-induced anti-neutrophil cytoplasmic antibody-positive renal vasculitis with a puzzling cutaneous rash. Case presentation We report a rare case of hydralazine-induced anti-neutrophil cytoplasmic antibody-associated vasculitis in a 62-year-old Caucasian woman who presented with a vasculitic syndrome with a sore throat, mouth ulcers and otalgia after several months of constitutional symptoms. She then proceeded to develop a rash over her right lower limb. Clinically, the rash had features to suggest Sweet’s syndrome, but also had some appearances consistent with embolic phenomena and did not have the appearance of palpable purpure usually associated with cutaneous vasculitis. Differential diagnoses were hydralazine-associated Sweet’s syndrome, streptococcal-induced cutaneous eruption or an unrelated contact dermatitis. A midstream urine sample detected glomerular blood cells in the setting of anti-neutrophil cytoplasmic antibody-positive renal vasculitis and Streptococcus pyogenes bacteremia. A renal biopsy revealed a pauci-immune, focally necrotizing glomerulonephritis with small crescents. Her skin biopsy revealed a heavy neutrophil infiltrate involving the full thickness of the dermis with no evidence of a leucocytoclastic vasculitis, but was non-specific. She was initially commenced on intravenous lincomycin for her bloodstream infection and subsequently commenced on 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 with a severe vasculitic syndrome with multiple organ involvement. Features of this association include the presence of high titres of anti-myeloperoxidase-anti-neutrophil cytoplasmic antibody with multi-antigenicity, positive anti-histone antibodies and the lack of immunoglobulin and complement deposition histopathogically. A rash that is characteristic of Sweet’s syndrome has also been described as an association. Prompt cessation of hydralazine may be sufficient to reverse disease activity but immunosuppression may be needed for definite treatment.



[Safety and efficacy of biologics directed against TNF-alpha and CD20 in the therapy of vasculitis and systemic lupus erythematosus].  


While the inhibition of TNF-alpha has been shown to improve vasculitis in vitro and in animal models, the clinical evidence for the efficacy of TNF-alpha blockade in most forms of vasculitis is mainly based on case reports and case series. Randomised controlled studies have so far not shown superiority of anti-TNF-alpha agents for Wegener's granulomatosis and giant cell arteritis. Moreover, in the context of Wegener's granulomatosis, a higher frequency and severity of infections are to be expected. In refractory cases of Behçet's disease therapy of uveitis and other organ manifestations is promising. Rituximab has achieved good effects in case reports of vasculitis. Results from controlled trials are not available. Observational studies indicate that in refractory systemic lupus erythematosus, and possibly also in several instances of small vessel vasculitis, rituximab can achieve good responses. The increased frequency of severe infections under TNF-alpha blockade requires a stringent benefit and risk assessment in addition to a multidisciplinary analysis of follow-up parameters. A detailed information of the patient regarding symptoms and signs of a possible infection are a prerequisite. Due to the complexity of the field and the danger of morbidity and mortality as a consequence of vasculitis or systemic lupus erythematosus on the one hand, and of the therapy on the other, biologics should only be used to treat these disorders in institutions fully equipped and staffed for this purpose. PMID:18622936

Walker, Ulrich A; Hasler, Paul



Phagocytosis of apoptotic cells by macrophages in anti-neutrophil cytoplasmic antibody-associated systemic vasculitis  

PubMed Central

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of autoimmune diseases, including granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). It is not known why ANCA develop, but it has been shown that they participate in pathogenesis by activating polymorphonuclear neutrophils (PMNs). In this study we hypothesize that dysregulation of phagocytosis in AAV leads to the accumulation of apoptotic neutrophils seen in association with blood vessels in AAV. These cells progress into secondary necrosis, contributing to tissue damage and autoantibody formation. Peripheral blood cells were counted, and phagocytosis was investigated using monocyte-derived macrophages (MŘ) and PMNs from healthy blood donors (HBD), AAV patients and systemic lupus erythematosus (SLE) patients. Furthermore, the effect of serum was assessed. Phagocytosis was measured using flow cytometry. The results showed no deviation in monocyte subpopulations for AAV patients compared to HBDs, although there was a decrease in lymphocyte and pDC (plasmacytoid dendritic cell) populations (4·2 × 106 cells/l versus 10·4 × 106 cells/l, P < 0·001). The number of neutrophils was increased (6·0 × 109 cells/l versus 3·8 × 109 cells/l, P < 0·001). There were no differences found in the ability of MŘs to engulf apoptotic cells, nor when comparing apoptotic PMNs to become engulfed. However, serum from AAV donors tended to decrease the phagocytosis ability of MŘs (36%) compared to serum from HBDs (43%). In conclusion, there is no intrinsic dysfunction in the MŘs or in the PMNs that have an effect on phagocytic activity, but ANCA may play a role by decreasing phagocytic ability.

Ohlsson, S M; Pettersson, A; Ohlsson, S; Selga, D; Bengtsson, A A; Segelmark, M; Hellmark, T



Antibodies with Dual Reactivity to Plasminogen and Complementary PR3 in PR3-ANCA Vasculitis  

PubMed Central

Patients with inflammatory vascular disease caused by anti-neutrophil cytoplasmic autoantibodies (ANCA) can harbor antibodies not only to the autoantigen proteinase 3 (PR3) but also to complementary PR3 (cPR3105–201), a recombinant protein translated from the antisense strand of PR3 cDNA. The purpose of this study was to identify potential endogenous targets of anti-cPR3105–201 antibodies. Patients’ plasmapheresis material was tested for the presence of antigens reactive with affinity-purified rabbit and chicken anti-cPR3105–201 polyclonal antibodies. Antigen-containing fractions were tested with patients’ anti-cPR3105–201 affinity-purified IgG, and putative protein targets were sequenced by mass spectrometry. Unexpectedly, plasminogen was identified as a target of anti-cPR3105–201. Reactivity of affinity-purified antibodies from two patients was lost when plasminogen was converted to plasmin, indicating restricted specificity. Antiplasminogen antibodies from five patients bound plasminogen at a surface-exposed loop structure within the protease domain. This loop contains an amino acid motif that is also found in a portion of recombinant cPR3105–201; site-directed mutagenesis of this sequence decreased antibody reactivity by 30%. Functionally, antiplasminogen antibodies delayed the conversion of plasminogen to plasmin and increased the dissolution time of fibrin clots. Serologically, antiplasminogen antibody levels were higher in PR3-ANCA patients (n = 72) than healthy control subjects (n = 63), myeloperoxidase-ANCA patients (n = 34), and patients with idiopathic thrombosis (n = 57; P = 0.001). Of the patients with PR3-ANCA, nine had documented deep venous thrombosis events, five of whom were positive for antiplasminogen antibodies. In summary, capitalizing on interactions with complementary proteins, specifically complementary PR3, this study identified plasminogen as a previously undescribed autoantigen in PR3-ANCA vasculitis.

Bautz, David J.; Preston, Gloria A.; Lionaki, Sofia; Hewins, Peter; Wolberg, Alisa S.; Yang, Jia Jin; Hogan, Susan L.; Chin, Hyunsook; Moll, Stephan; Jennette, J. Charles; Falk, Ronald J.



Assessment of the Item Selection and Weighting in the Birmingham Vasculitis Activity Score for Wegener's Granulomatosis  

PubMed Central

Objective To assess the Birmingham Vasculitis Activity Score for Wegener's Granulomatosis (BVAS/WG) with respect to its selection and weighting of items. Methods This study used the BVAS/WG data from the Wegener's Granulomatosis Etanercept Trial. The scoring frequencies of the 34 predefined items and any “other” items added by clinicians were calculated. Using linear regression with generalized estimating equations in which the physician global assessment (PGA) of disease activity was the dependent variable, we computed weights for all predefined items. We also created variables for clinical manifestations frequently added as other items, and computed weights for these as well. We searched for the model that included the items and their generated weights yielding an activity score with the highest R2 to predict the PGA. Results We analyzed 2,044 BVAS/WG assessments from 180 patients; 734 assessments were scored during active disease. The highest R2 with the PGA was obtained by scoring WG activity based on the following items: the 25 predefined items rated on ?5 visits, the 2 newly created fatigue and weight loss variables, the remaining minor other and major other items, and a variable that signified whether new or worse items were present at a specific visit. The weights assigned to the items ranged from 1 to 21. Compared with the original BVAS/WG, this modified score correlated significantly more strongly with the PGA. Conclusion This study suggests possibilities to enhance the item selection and weighting of the BVAS/WG. These changes may increase this instrument's ability to capture the continuum of disease activity in WG.




Metastatic B-cell lymphoma masquerading as infectious retinitis and vasculitis  

PubMed Central

Intraocular lymphoma is a rare ocular malignancy that may occur in the retina or the uvea. Retina or vitreoretinal lymphoma accounts for the majority of cases and is often secondary to diffuse large B-cell lymphoma. In the present study, a 66-year-old Caucasian male with a history of Waldenstrom’s macroglobulinemia with diffuse large B-cell lymphoma, presented with blurred vision in the left eye one month following cycle 4 of an R-CHOP regimen. At the time of onset, the patient was being treated for bacterial pneumonia. Visual acuity was 20/25 in his right eye (OD) and 20/30 in the left (OS). Ophthalmologic examination showed intraretinal white infiltrates associated with hemorrhage in the superotemporal midperiphery of the retina and vitritis OS. Initial diagnostic considerations included infectious (cytomegalovirus retinitis, syphilis, toxoplasmosis, tuberculosis), inflammatory (retinal vasculitis associated with autoimmune disease or hypercoagulable states) or malignant (intraocular lymphoma) diseases. The patient did not respond to intravitreal injection of foscarnet and oral valgancyclovir. Systemic work-up and aqueous fluid biopsy were inconclusive. Diagnostic vitrectomy yielded inconclusive results and the patient continued to have progressive loss of vision. A repeat diagnostic vitrectomy with retinal and subretinal biopsy confirmed large B cells consistent with metastatic B-cell lymphoma. A concomitant PET/CT scan was performed that revealed bilateral new pulmonary nodules resulting in additional chemotherapy. Our case shows the diagnostic dilemmas in patients with systemic lymphoma and the possible role of concurrent systemic restaging in patients with ocular complaints, even when in systemic remission.




A case of ANCA-associated systemic vasculitis induced by atorvastatin.  


We present a 45-year-old male patient who presented to Accident and Emergency department with a 6-week history of pain and stiffness involving his bilateral legs. Both calves were markedly tender, and he was not able to bear weight. He also complained of numbness involving his left big toe for a few days, which later spread to involve his arms, and tinnitus and hearing loss in his left ear. There were no respiratory, gastrointestinal or urinary symptoms. He had a background history of hypercholesterolemia and was treated with atorvastatin 10 mg for 6 months. His initial investigations showed markedly increased inflammatory markers, and serum antineutrophil cytoplasmic antibody (ANCA) was markedly positive at a titre of 1:160 (P-ANCA). Electromyography and muscle biopsy showed myopathic features. A diagnosis of drug-induced ANCA-associated vasculitis (on the basis of mononeuritis multiplex, sensorineural hearing loss and markedly increased anti-myeloperoxidase (MPO) ANCA) and statin-induced distal myopathy was made. He was treated with three 500 mg doses of methylprednisolone, followed by slowly tapering dose of oral corticosteroids from 30 mg once daily (OD). He was also started on azathioprin (2.5 mg/kg). He had a dramatic improvement of his myalgia, hearing loss and sensory symptoms and went into complete clinical remission. His inflammatory markers rapidly returned to normal, and MPO-ANCA normalised within 3 months of starting immunosuppressive therapy and remained negative on further testing. He is currently on a tapering regimen of corticosteroids (7 mg OD), and after weaning him off corticosteroids, we plan to slowly taper his azathioprin. PMID:18839266

Haroon, Muhammad; Devlin, Joe



Anti-neutrophil cytoplasmic autoantibodies: methodological aspects and clinical significance in systemic vasculitis.  


Antineutrophil cytoplasmic antibodies (ANCA) are the serological hallmark of some idiopathic systemic vasculitides, such as granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and, to a lesser extent, Churg-Strauss syndrome (CCS), the so-called ANCA-associated vasculitides (AAV). ANCA were first detected by immunofluorescence (IIFT), subsequently the target antigens myeloperoxidase (MPO) and proteinase 3 (PR3) were identified, allowing the development of the quantitative, antigen-specific assays. According to the guidelines, combining IIFT and PR3-ANCA/MPO-ANCA assures the optimal diagnostic specificity. Antigen specificity does not effectively differentiate among the different AAV, however C-ANCA/PR3-ANCA are mainly found in GPA, while P-ANCA/MPO-ANCA are more prevalent in MPA and CSS. Despite their diagnostic value, the performance of the widespread immunometric assays for ANCA testing is disappointing, particularly for the low sensitivity. In recent years, more "sensitive" assays have been developed, using the microplate as well as fully the automated technologies, with promising preliminary results. ANCA, may be detected in a number of pathological conditions other than small vessel vasculitis. However, in most of these non-vasculitic patients ANCA do not recognize MPO or PR3 as target antigens, but other granulocyte components, often multiple or unknown specificities. A positive ANCA result by itself is not diagnostic for AAV, clinical evidence and possibly histological confirmation are always required. On the other hand, a negative test result cannot completely rule out a diagnosis of AAV, as AAV without detectable ANCA exist. The appropriate use of ANCA testing strongly improves the diagnostic accuracy and clinical usefulness of the results. PMID:22921790

Radice, Antonella; Bianchi, Laura; Sinico, Renato Alberto



High prevalence of autoantibodies to hLAMP-2 in anti-neutrophil cytoplasmic antibody-associated vasculitis.  


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

Kain, Renate; 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



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


BACKGROUND: The standard-of-care treatment of patients with hepatitis C virus (HCV)-mixed cryoglobulinemia (MC) vasculitis includes pegylated interferon ? (PegIFN)-? 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-IFN?/ribavirin/protease inhibitor combination in HCV-MC vasculitis. PATIENTS AND METHODS: Open-label, prospective, cohort study including 23 patients with HCV-MC vasculitis. Peg-IFN?/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-IFN?/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

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



A case of levamisole-induced systemic vasculitis and cocaine-induced midline destructive lesion: a case report.  


We describe a case of antineutrophil cytoplasmic antibody-positive vasculitis from levamisole-tainted cocaine with concomitant cocaine-induced midline destructive lesions of the palate and nasal septum. The diagnosis was confirmed after extensive clinical, laboratory, pathologic, and radiographic testing. Timely recognition of this clinical entity is critical to avoid misdiagnosis and unnecessary treatment with potentially harmful cytotoxic agents. Given the high rate of levamisole contamination within the nation's cocaine supply, clinicians should be alerted to this emerging health threat. PMID:21617545

Zwang, Nicholas A; Van Wagner, Lisa B; Rose, Shawn



Henoch-Schönlein purpura: clinicopathologic correlation of cutaneous vascular IgA deposits and the relationship to leukocytoclastic vasculitis.  


Significant cutaneous vascular IgA deposits are common in Henoch-Schönlein purpura but not in other vasculitides. The specificity for IgA vascular deposits for Henoch-Schönlein purpura is not well defined. To examine the specificity of IgA vascular deposits for this disease, we compared clinicopathologic features of 92 cases with IgA vascular deposits and a direct immunofluorescence impression of vasculitis with 90 similar cases without IgA deposits. Henoch-Schönlein purpura was diagnosed in 24% of cases with vascular IgA deposits on direct immunofluorescence examination. IgA deposits were frequent in erythema nodosum and venous stasis-related problems and in cryoglobulinemia, coagulopathic vasculopathies, and livedoid vasculitis. Of our cases, 78% exhibited vascular fluorescence with multiple conjugates. No histologic or immunofluorescence pattern alone was specific. The diagnostic specificity for Henoch-Schönlein purpura is improved if gastrointestinal involvement, upper respiratory infection, or age < 20 years is present. We propose diagnostic criteria for Henoch-Schönlein purpura incorporating clinical findings yielding sensitivity and specificity > 90%. PMID:7604640

Helander, S D; De Castro, F R; Gibson, L E



Cyclophosphamide and 4-hydroxycyclophosphamide pharmacokinetics in patients with glomerulonephritis secondary to lupus and small vessel vasculitis  

PubMed Central

AIMS Cyclophosphamide, the precursor to the active 4-hydroxycyclophosphamide, is used in active glomerulonephritis despite limited pharmacokinetics data. The pharmacokinetics of cyclophosphamide and 4-hydroxycyclophosphamide were evaluated. The influence of laboratory and pharmacogenomic covariates on pharmacokinetics was evaluated as a secondary aim. METHODS Glomerulonephritis patients (n = 23) participated in a pharmacokinetic evaluation. Blood was serially collected and assayed for cyclophosphamide and 4-hydroxycyclophosphamide by LC/MS methods. Kidney function, serum albumin and polymorphisms in drug metabolism or transport genes were evaluated. Analyses included non-compartmental pharmacokinetics and parametric and non-parametric statistics. RESULTS The mean area under the plasma concentration–time curve (AUC(0,?)) data were 110 100 ± 42 900 ng ml?1 h and 5388 ± 2841 ng ml?1 h for cyclophosphamide and 4-hydroxycyclophosphamide, respectively. The mean metabolic ratio was 0.06 ± 0.04. A statistically significant relationship was found between increased serum albumin and increased half-life (0.584, P = 0.007, 95% CI 0.176, 0.820) and a borderline relationship with AUC(0,?) (0.402, P = 0.079, 95% CI –0.064, 0.724) for 4-hydroxycyclophosphamide. Covariate relationships that trended toward significance for cyclophosphamide included decreased serum albumin and increased elimination rate constant (–0.427, P = 0.061, 95% CI 0.738, 0.034), increased urinary protein excretion and increased AUC(0,?) (–0.392, P = 0.064, 95% CI –0.699 to 0.037), decreased Cmax (0.367, P = 0.085, 95% CI –0.067, 0.684) and decreased plasma clearance (–0.392, P = 0.064, 95% CI –0.699, 0.037). CYP2B6*9 variants vs. wildtype were found to have decreased elimination rate constant (P = 0.0005, 95% CI 0.033, 0.103), increased Vd (P = 0.0271, 95% CI ?57.5, ?4.2) and decreased Cmax (P = 0.0176, 95% CI 0.696, 6179) for cyclophosphamide. ABCB1 C3435T variants had a borderline decrease in cyclophosphamide elimination rate constant (P = 0.0858; 95% CI –0.005, 0.102). CONCLUSIONS Pharmacokinetics of cyclophosphamide and 4-hydroxycyclophosphamide in patients with lupus nephritis and small vessel vasculitis are similar. Clinical and pharmacogenetic covariates alter disposition of cyclophosphamide and 4-hydroxycyclophosphamide. Clinical findings of worsened glomerulonephritis lead to increased exposure to cyclophosphamide vs. the active 4-hydroxycyclophosphamide, which could have relevance in terms of clinical efficacy. The CYP2B6*9 and ABCB1 C3435T polymorphisms alter the pharmacokinetics of cyclophosphamide and 4-hydroxycyclophosphamide in glomerulonephritis.

Joy, Melanie S; La, Mary; Wang, Jinzhao; Bridges, Arlene S; Hu, Yichun; Hogan, Susan L; Frye, Reginald F; Blaisdell, Joyce; Goldstein, Joyce A; Dooley, Mary Anne; Brouwer, Kim L R; Falk, Ronald J



Immune complexes activate human endothelium involving the cell-signaling HMGB1-RAGE axis in the pathogenesis of lupus vasculitis.  


Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by the formation of immune complexes (ICs), which contain a complex mixture of autoantigens nucleic acids, nucleic acids-associated proteins and corresponding autoantibodies. In SLE, ICs are deposited in multiple organs. Vasculopathy and vasculitis in SLE are typical complications and are associated with deposition of ICs on endothelium, endothelial activation and inflammatory cell infiltration. However, the effects of ICs on endothelial cells and the mechanisms involved remain unclear. In this study, we have demonstrated for the first time that ICs upregulated cell surface expression of the receptor for advanced glycation end products (RAGE), the expression of intercellular adhesion molecule-1 (ICAM-1), vascular cell adhesion molecule-1 (VCAM-1), increased the secretion of the chemokines interleukin 8 (IL-8), monocyte chemoattractant protein-1 (MCP-1), the proinflammatoy cytokines interleukin 6 (IL-6), tumor necrosis factor-? (TNF-?) and promoted the activation of the transcription factor NF-?B p65 in human endothelial cells (P<0.05). ICs also increased transendothelial migration of monocytes (P<0.05). One of the mechanisms underlying these activating effects of ICs on human endothelial cells involves cell signaling by high-mobility group box 1 protein (HMGB1)-RAGE axis, as these effects can be partially blocked by HMGB1 A-box, soluble RAGE (sRAGE), SB203580, PD98059, Bay 117082 (P<0.05) and co-treatment with these agents (P<0.05). In conclusion, ICs elicit proinflammatory responses in human endothelial cells and alter their function involving cellular signaling via the HMGB1-RAGE axis in the pathogenesis of SLE vasculitis. PMID:23628898

Sun, Wenping; Jiao, Yulian; Cui, Bin; Gao, Xuejun; Xia, Yu; Zhao, Yueran



Polymyalgia rheumatica as presenting manifestation of vasculitis involving the lower extremities in a patient with ulcerative colitis.  


Extraintestinal features may be observed in patients with ulcerative colitis (UC). We describe a 69-year-old woman who was initially diagnosed as having polymyalgia rheumatica (PMR). Prednisone was progressively tapered to complete discontinuation a year and a half after PMR diagnosis. However, at that time, she started to complain of asthenia, abdominal cramping and pain on the left side, weight loss and bloody diarrhoea. A colonoscopy confirmed a diagnosis of left-sided UC. She experienced several flares of the disease that required admission and treatment with high-dose corticosteroids and azathioprine. Colectomy was performed as the disease became refractory to these therapies. Four months after surgery, when the patient was not receiving any corticosteroid therapy, she started to feel dull and achy pain in the thighs along with claudication of the lower limbs. An 18F-fluorodeoxyglucosepositron emission tomography with CT (FDG PET/CT) disclosed an inflammatory process with mild-moderate diffuse increased metabolism in the thoracic aorta and markedly increased FDG uptake in the in the femoral and posterior tibial arteries on both sides. Treatment with the anti-TNF-alpha monoclonal antibody-adalimumab (40 mg every 2 weeks subcutaneously) along with prednisone (initial dose 15 mg/day) yielded rapid improvement of symptoms. Also, a new FDG PET/CT performed 4 months later disclosed marked decrease of FDG uptake in the involved arteries.This report emphasises the importance of suspecting the presence of large- and medium-vessel vasculitis in a patient with UC presenting with musculoskeletal features. It also highlights the beneficial effect of TNF-antagonists in vasculitis associated to UC. PMID:22640654

Bejerano, Carmen; Blanco, Ricardo; González-Vela, Carmen; Pérez-Martín, Inés; Martinez-Rodriguez, Isabel; Jimenez-Bonilla, Julio; González-Gay, Miguel A



C-ANCA-positive IgG fraction from patients with Wegener's granulomatosis induces lung vasculitis in rats.  


The aim of the present study was to analyse in rats the ability of C-ANCA-positive IgG fraction in triggering inflammatory response on pulmonary tissue. Wistar rats (n = 18) were injected via the the internal jugular vein with 20 mg of total C-ANCA-positive IgG fraction isolated from serum of three different Wegener's granulomatosis patients obtained before therapy. Similarly, control rats were treated with IgG fraction from two rheumatoid arthritis patients (n = 7), IgG from six normal human sera (n = 15) or saline (n = 18), respectively. Animals were sacrificed after 24h of injection for histological analysis of the lungs. Vasculitis and inflammatory infiltrate were consistently absent in rats injected with rheumatoid arthritis IgG or saline and in 14/15 of normal IgG treated animals. In contrast, marked vasculitis was observed in all 18 animals injected with C-ANCA-positive IgG fraction. The histological features were characterized by the presence of a perivascular pleomorphic cellular sheath, particularly around small vessels, endothelial adherence and diapedesis of polymorphonuclear leucocytes and presence of granuloma-like lesions. A dose-response relationship was observed between protein concentration of C-ANCA IgG sample and the intensity of the inflammatory response in the animals. In addition, IgG fraction with undetectable C-ANCA, obtained from one patient in remission after treatment, was not able to reproduce the pulmonary tissue alterations induced by its paired IgG that was positive for C-ANCA taken before therapy. The experimental model described herein may be useful to characterize more effectively the pathogenic mechanism of C-ANCA in Wegener's disease. PMID:12100022

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



18F-FDG PET as a diagnostic procedure in large vessel vasculitis—a controlled, blinded re-examination of routine PET scans  

Microsoft Academic Search

Large vessel vasculitis can be visualized by 18F-FDG positron emission tomography (PET). However, the diagnostic value of\\u000a 18F-FDG PET is yet to be determined. We therefore performed a study to evaluate this technique for the diagnosis of giant\\u000a cell arteritis (GCA) and Takayasu arteritis (TA). Patients with GCA or TA, who fulfilled the American College of Rheumatology\\u000a (ACR) criteria and

Petra Lehmann; Sarah Buchtala; Nelli Achajew; Peter Haerle; Boris Ehrenstein; Hamid Lighvani; Martin Fleck; Joerg Marienhagen



Systemic vasculitis with prolonged pyrexia, recurrent facial urticaria, skin nodules, pleural effusions and venous thrombosis: an unusual presentation of an uncommon disease  

PubMed Central

Classically presenting with multiple or single peripheral cytopenias of variable severity, the myelodysplastic syndromes may occasionally present with bizarre manifestations that confuse the clinical picture and result in significant delays in making the correct diagnosis. We describe the case of an elderly male patient whose presentation with prolonged unexplained fever coupled with cutaneous, pulmonary and other systemic features of inflammation was finally diagnosed as having a primary myelodysplastic syndrome with associated vasculitis after a delay of 4 years.

Hassan, Imad Salah; Dar, Javeed



Persistent fever, neck swelling, and small vessel vasculitis following tonsillectomy in a patient with Beh?et's disease: a case report  

PubMed Central

Introduction Behçet’s 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 Behçet’s disease. Case presentation We report the case of a 62-year-old European Caucasian woman with Behçet’s 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 patient’s symptoms as a flare of Behçet’s disease. Immunosuppressive treatment led to rapid clinical improvement. Conclusion A patient with Behçet’s 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 Behçet’s disease.



[Our experience in treatment of multicentric plasma-cell Castleman disease associated with vasculitis manifestations - case report and literature review].  


Castleman disease is a rare idiopathic non-neoplastic lymphoproliferative disorder with 2 clinical (unicentric and multicentric) and 3 histomorphological (hyaline-vascular, plasma-cell and mixed) forms identified. The case report given here describes the 3-year experience with therapy in a patient, male born 1961, diagnosed with multicentric plasma-cell Castleman disease (HIV and HHV-8 negative) with the finding of generalized lymphadenopathy and splenomegaly. During first line treatment (R-CHOP: rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone, 3 cycles in total, 12/2008-2/2009) the development of bilateral upper and lower limb edemas with clinical manifestation of vasculitis occurred and a restaging computed tomography (CT) examination revealed a stable finding of the lymphadenomegaly. Greater success was achieved with thalidomide regimen (CTD: cyclophosphamide, thalidomide, dexamethasone, 10 cycles, 3/2009-1/2010) leading to reduction in the size of the hypervascularized lymph nodes (almost by 50%) as well as their radiopharmaceutical (fluorodeoxyglucose) uptake as seen on a combined positron emission tomography and computed tomography (PET/CT) scan imaging. Thalidomide was given daily at doses between 100 and 200 mg. We returned to the CTD regimen again in April 2010 after a short period of monoclonal antibody tocilizumab treatment (400 mg intravenous in 2-week intervals with 50% dose reduction due to a limited supply of the drug, 5 doses in total) during which edemas reoccurred with a CT scan finding of stable lymphadenomegaly. However, the renewed regimen with thalidomide was stopped after 2.5 cycles due to adverse effects of thalidomide (neuropathy) and corticoids (Cushing syndrome). In September 2010, after enrollment in the Celgenes Compassionate Use Program we were able to start treating the patient with the derivative of thalidomide, lenalidomide, at a dosage of 25 mg on days 1-21 in a 28-day cycle, 15 cycles in total (10/2010-12/2011). The monotherapy with lenalidomide was very well tolerated by the patient without any effects of myelotoxicity, thromboembolism or relapses of edemas and vasculitis, additionally now with apparent improvement of fatic disorder and the patients motor abilities. Thus, lenalidomide represents an attractive alternative agent for patients with Castleman disease after rituximab and cytostatics failures. It has a favourable safety profile and could be therefore considered for administering in first line treatment. PMID:23094815

Szturz, P; Adam, Z; Moulis, M; Smardová, L; Klincová, M; Slaisová, R; Koukalová, R; Rehák, Z; Volfová, P; Chovancová, J; Stehlíková, O; Mayer, J



Renal-limited vasculitis in children: a single-center retrospective long-term follow-up analysis.  


Pauci-immune renal limited vasculitis (RLV) is a rare and aggressive autoimmune disease. We retrospectively analyzed the renal outcome of 6 children with biopsy proven RLV. Median age at diagnosis was 10.6 years (range 7.1 - 14.5) and the median follow-up was 4.4 years (range 2.3 - 6.6). At diagnosis, 5 patients were given induction therapy (methylprednisolone + cyclophosphamide pulses) followed by maintenance treatment (prednisolone + azathioprine) while 1 patient received maintenance treatment only. After induction, 4 patients either retained or recovered normal renal function, and 1 patient, in whom short-term plasma exchange was prescribed to try to rescue her renal function, became free from dialysis. Repeated biopsy showed no disease activity; however, renal scarring was evident in all renal specimens. At last follow-up, 2 patients had normal renal function, 3 patients had mild renal insufficiency, and 1 patient had advanced renal failure. In addition, 5 patients were treated for hypertension. Our case series suggests that an initial favorable response to immunosuppressive therapy might not necessarily prevent the occurrence of renal scarring and highlights the importance of regular follow-up. PMID:22541686

Krmar, Rafael T; Kĺgebrand, Mathias; A Hansson, Magnus E; Halling, Stella Edström; Asling-Monemi, Kajsa; Herthelius, Maria; Holtbäck, Ulla; Christensson, Marta; Wernerson, Annika; Bruchfeld, Annette



Cutaneous leukocytoclastic vasculitis due to erlotinib: just an adverse event or also a putative marker of drug efficacy?  


Erlotinib is a targeted anticancer therapy with selective inhibitory activity for tyrosine kinase of the epidermal growth factor receptor (EGFR). Different skin reactions have been described linked to these drugs. There are no other reports about erlotinib-induced leukocytoclastic vasculitis (LV) in the erlotinib-bevacizumab regimen for bone metastasis, from a relapsed hepatocellular carcinoma (HCC) in liver-transplanted patients. In our patient a dose reduction and then the suspension of erlotinib was required. After a 2 week withdrawal, the drug was re-challenged at a lower dose. The patient continued it without any skin recurrence, and resulted progression free for 16 months. Thus, we underline the possibility to avoid a permanent withdrawal of erlotinib and to rechallenge with it without any cutaneous toxicity, particularly in patients benefiting from this drug. Moreover, the median overall survival from the initial treatment of bone relapsed patients after liver transplant for HCC is found to be less than 5 months, while our patient died 5 years later. This longer survival encourages further investigations to assess also whether LV, even if rare, might be used as a marker of antitumor efficacy of EGFR inhibitors. PMID:23534992

Brandi, Giovanni; Venturi, Michela; Dika, Emi; Maibach, Howard; Patrizi, Annalisa; Biasco, Guido



TWEAK Enhances E-selectin and ICAM-1 Expression, and May Contribute to the Development of Cutaneous Vasculitis  

PubMed Central

Our previous work indicated that TWEAK is associated with various types of cutaneous vasculitis (CV). Herein, we investigate the effects of TWEAK on vascular injury and adhesion molecule expression in CV mice. We showed that TWEAK priming in mice induced a local CV. Furthermore, TWEAK priming also increased the extravasation of FITC-BSA, myeloperoxidase activity and the expression of E-selectin and ICAM-1. Conversely, TWEAK blockade ameliorated the LPS-induced vascular damage, leukocyte infiltrates and adhesion molecules expression in LPS-induced CV. In addition, TWEAK treatment of HDMECs up-regulated E-selectin and ICAM-1 expression at both mRNA and protein levels. TWEAK also enhanced the adhesion of PMNs to HDMECs. Finally, western blot data revealed that TWEAK can induce phosphorylation of p38, JNK and ERK in HDMECs. These data suggest that TWEAK acted as an inducer of E-selectin and ICAM-1 expression in CV mice and HDMECs, may contribute to the development of CV.

Chen, Tao; Guo, Zai-pei; Li, Li; Li, Meng-meng; Wang, Ting-ting; Jia, Rui-zhen; Cao, Na; Li, Jing-yi



Coping strategies and depressiveness in primary systemic vasculitis--what is their impact on health-related quality of life?  


Objective. To investigate the influence of disease-related coping strategies and depressiveness on health-related quality of life (HRQOL) in primary systemic vasculitis (PSV) patients. Methods. One hundred and twenty-two patients with definite diagnosis of PSV were examined in a cross-sectional study. HRQOL (SF-36), depressiveness (BDI), illness perception (B-IPQ) and coping strategies (FKV-LIS) were measured using validated instruments. Additional disease-related and demographic data were retrieved from the patients' records. Results. HRQOL in PSV patients was reduced compared with the SF-36 norm sample. Specific organ manifestation, size of vessel involvement and disease activity were not related to HRQOL. Linear regression modelling revealed a questionable relationship of emotional to physical HRQOL (P = 0.003, potential suppression effect of BDI), whereas both domains were influenced by depressiveness (P ? 0.001). Physical HRQOL was additionally related to fatigue and widowed marital status, while emotional HRQOL was associated with a depressive coping style. Conclusion. HRQOL is impaired in PSV as compared with the general population. Current depressiveness strongly affects physical as well as mental HRQOL. Cognitive intervention strategies should be established in order to improve quality of life in PSV patients. PMID:23843108

Brezinova, Petra; Englbrecht, Matthias; Lovric, Svjetlana; Sämann, Alexander; Strauss, Bernhard; Wolf, Gunter; Schett, Georg; Haubitz, Marion; Neumann, Thomas; Zwerina, Jochen



Genetics of ANCA-associated vasculitis in Japan: a role for HLA-DRB1*09:01 haplotype.  


The epidemiology of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is considerably different between European and Asian populations. Whereas granulomatosis with polyangiitis is the most common form of AAV in northern European populations, microscopic polyangiitis (MPA) accounts for the majority of AAV in Japan. This difference may at least in part derive from the difference in genetic background. In this review, I focus on our observation on HLA, an obvious candidate gene for immune disorders, and discuss its potential implication. In Japanese AAV, significant association was detected with HLA-DRB1*09:01, the carrier frequency of which was increased in MPA [P = 0.0087, odds ratio (OR) 1.90, 95 % confidence interval (CI) 1.17-3.08] and in myeloperoxidase (MPO)-ANCA-positive AAV (P = 0.0016, OR 2.05, 95 % CI 1.31-3.23) when compared with healthy Japanese controls. HLA-DRB1*09:01 is one of the most common HLA-DRB1 alleles in Asians but is rare in Caucasian populations. Interestingly, HLA-DRB1*09:01 has been shown to be associated with multiple autoimmune diseases, including type 1 diabetes, rheumatoid arthritis, and systemic lupus erythematosus, suggesting that either HLA-DRB1*09:01 itself or other genes in tight linkage disequilibrium may play a role in a molecular pathway shared by various autoimmune diseases in Japanese and possibly in other Asian populations. PMID:23180035

Tsuchiya, Naoyuki



Value of ANCA measurements during remission to predict a relapse of ANCA-associated vasculitis--a meta-analysis  

PubMed Central

Objective. The value of repeated ANCA measurements among patients with an established diagnosis of ANCA-associated vasculitis (AAV) remains controversial. The aim of this study was to explore whether either of the two distinct patterns of ANCA values during remission, a rise in ANCA or persistently positive ANCA, predicted future relapse. Methods. MEDLINE and EMBASE searches were performed. Studies with at least 10 subjects with AAV from which both sensitivity and specificity of a rise in ANCA and/or persistent ANCA for future disease relapse could be calculated were included. Likelihood ratios were calculated for each study and pooled to arrive at summary estimates. I2-values were calculated as a measure of heterogeneity and meta-regression was used to explore sources of heterogeneity. Results. Nine articles on a rise in ANCA and nine articles on persistent ANCA were included. The summary estimates for positive likelihood ratio (LR+) and negative likelihood ratio (LR?) of a rise in ANCA during remission on subsequent relapse of disease were 2.84 (95% CI 1.65, 4.90) and 0.49 (95% CI 0.27, 0.87), respectively. The summary estimates for LR+ and LR? of persistent ANCA during remission for subsequent disease relapse were 1.97 (95% CI 1.43, 2.70) and 0.73 (95% CI 0.50, 1.06), respectively. There was substantial between-study heterogeneity, which was partially explained by the frequency of ANCA measurements. Conclusion. Among patients with AAV, a rise in or persistence of ANCA during remission is only modestly predictive of future disease relapse. There is limited use to serial ANCA measurements during disease remission to guide treatment decisions for individual patients with AAV.

Grayson, Peter C.; Mahr, Alfred D.; LaValley, Michael; Merkel, Peter A.



Eosinophilic/T-cell chorionic vasculitis and chronic villitis involve regulatory T cells and often occur together.  


Eosinophilic/T-cell chorionic vasculitis (ETCV) is characterized by mixed T-cell, eosinophilic, and histiocytic infiltrates within the chorionic vessel wall. We sought to better characterize this lesion with respect to other pathologic correlates and the T-cell populations involved. Epidemiologic data and other pathologic diagnoses, including concurrent chronic villitis (CV), were tabulated for each case of ETCV diagnosed at our institution over a 6-year period. CD3, CD25, FOXP3, and dual FOXP3-CD3 immunostains were used to identify regulatory T-cell populations in ETCV and CV. Cells positive for CD3, FOXP3, and CD25 were quantitated by manual counts of ×40 fields at the sites of ETCV and CV, and FOXP3?CD3 and CD25?CD3 ratios were calculated. Digital analysis of ETCV and CV using the dual FOXP3-CD3 immunostain was also performed on select cases. Of 31 ETCV cases, 10 (32%) were accompanied by CV and 13 (42%) by a thrombus in the vessel affected by ETCV. The mean Treg cell marker?CD3 ratios in ETCV ranged from 0.18 to 0.26 by manual count and digital analysis, but the counts did not statistically differ by method. The mean Treg cell marker?CD3 ratios in CV ranged from 0.37 to 0.39 by manual count and 0.19 by digital analysis, but these counts also did not statistically differ by method. Chronic villitis was seen in one-third of ETCV cases. FOXP3+ and CD25+ regulatory T cells represent a significant subpopulation of T cells in ETCV and CV, suggesting that they may play a role in these entities. PMID:23600955

Katzman, Philip J; Oble, Darryl A



Health related quality of life in patients with newly diagnosed anti-neutrophil cytoplasm antibody associated vasculitis  

PubMed Central

Background Anti-neutrophil cytoplasm antibody-associated vasculitis (AAV) can present with a broad spectrum of signs and symptoms. The relative effects of different manifestations on health related quality of life (HRQOL) is unknown. Methods We conducted an individual patient data meta-analysis of baseline Short Form 36 (SF-36) scores from four randomized controlled trials of patients with newly diagnosed AAV. We determined the associations between organ manifestations at trial entry and the SF-36 Physical Composite Score (PCS) and Mental Composite Score (MCS) using mixed effects models adjusted for demographic factors. Associations with each of the 8 domains of the SF-36 were further explored using multivariate multiple regression. Results SF-36 data was available from 346 patients. Older age (?0.11 points/year; 95% Confidence Interval [CI] ?0.21 to ?0.012; p=0.029) and neurologic involvement (?5.84, p<0.001) at baseline were associated with lower Physical Composite Scores. Physical Function scores were the most affected and older age (?0.25 points per year, 95% Confidence Interval [CI] ?0.38 to ?0.11; p<0.001) scores and neurologic involvement (?8.48 points, 95% CI ?12.90 to ?4.06; p<0.001) had the largest effects. The MCS was negatively affected only by chest involvement (p=0.027) but this effect was not exerted in any particular domain. Conclusions HRQOL in patients with newly diagnosed AAV are complex and incompletely explained by their organ system manifestations.

Walsh, Michael; Mukhtyar, Chetan; Mahr, Alfred; Herlyn, Karen; Luqmani, Raashid; Merkel, Peter A.; Jayne, David R. W.



Decreased Numbers of Blood Dendritic Cells and Defective Function of Regulatory T Cells in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis  

PubMed Central

Background Dendritic cells (DC) and regulatory cells (Treg) play pivotal roles in controlling both normal and autoimmune adaptive immune responses. DC are the main antigen-presenting cells to T cells, and they also control Treg functions. In this study, we examined the frequency and phenotype of DC subsets, and the frequency and function of Treg from patients with ANCA-associated vasculitis (AAV). Methodology/Principal Findings Blood samples from 19 untreated patients with AAV during flares and before any immunosuppressive treatment were analyzed, along with 15 AAV patients in remission and 18 age-matched healthy controls. DC and Treg numbers, and phenotypes were assessed by flow cytometry, and in vitro suppressive function of Treg was determined by co-culture assay. When compared to healthy volunteers, absolute numbers of conventional and plasmacytoid DC were decreased in AAV patients. During the acute phase this decrease was significantly more pronounced and was associated with an increased DC expression of CD62L. Absolute numbers of Treg (CD4+CD25highCD127low/? Tcells) were moderately decreased in patients. FOXP3 and CD39 were expressed at similar levels on Treg from patients as compared to controls. The suppressive function of Treg from AAV patients was dramatically decreased as compared to controls, and this defect was more pronounced during flares than remission. This Treg functional deficiency occurred in the absence of obvious Th17 deviation. Conclusion In conclusion, these data show that AAV flares are associated with both a decrease number and altered phenotype of circulating DC and point to a role for Treg functional deficiency in the pathogenesis of AAV.

Rimbert, Marie; Hamidou, Mohamed; Braudeau, Cecile; Puechal, Xavier; Teixeira, Luis; Caillon, Helene; Neel, Antoine; Audrain, Marie; Guillevin, Loic; Josien, Regis



Standardised work-up programme for fever of unknown origin and contribution of magnetic resonance imaging for the diagnosis of hidden systemic vasculitis  

PubMed Central

Background: Fever of unknown origin (FUO) is a diagnostic challenge. Rheumatologists are often in charge of patients with FUO because the vasculitides, especially, are potential and common causes of FUO. Objective: To evaluate the value of a standardised investigation to identify the cause of FUO. Methods: A standardised work-up programme for patients with FUO was started at the beginning of September 1999. The rate of identified causes of FUO was compared between all patients with FUO admitted to a tertiary care centre of rheumatology between January 1996 and August 1999 (control group) and September 1999 and January 2003 (work-up group). In January 2002 magnetic resonance imaging (MRI) was added to the investigation. Results: 67 patients with FUO were identified—32 before and 35 after institution of the work-up programme. Before implementation 25% of all patients with FUO remained undiagnosed, after implementation 37%. After institution of the investigation the percentage of patients with vasculitides increased significantly from 6% (n = 2) to 26% (n = 9, p = 0.047, Fisher's exact test). This increase could be attributed to the addition of MRI in 2002. When all patients with FUO before 2002 (n = 55) and thereafter (n = 12) were compared the prevalence of systemic vasculitis increased from 11% (n = 6) to 42% (n = 5, p = 0.021). Conclusion: Implementation of a standardised work-up programme for FUO did not improve the overall rate of diagnosis. Addition of MRI significantly increased the diagnosis of systemic vasculitis as the underlying cause of FUO. MRI should be included in the investigation of patients with FUO when vasculitis is suspected.

Wagner, A; Andresen, J; Raum, E; Lotz, J; Zeidler, H; Kuipers, J; Jendro, M



Urocortin promotes the development of vasculitis in a rat model of thromboangiitis obliterans via corticotrophin-releasing factor type 1 receptors  

PubMed Central

Background and purpose: Urocortin is a locally expressed pro-inflammatory peptide. Here we have examined the effects of urocortin on sodium laurate-induced peripheral arterial vasculitis in rats, modelling the mechanisms of thromboangiitis obliterans (TAO). Experimental approach: Peripheral vasculitis in rats was induced by sodium laurate and graded by gross appearance on the 12th day after injection. Histological changes in rat femoral arteries were assessed by histopathology and transmission electron microscopy. Blood cell counts, blood rheology, blood coagulation and plasma urocortin, thromboxane B2, prostaglandin E2 and soluble intercellular adhesion molecule-1 levels were measured. Expression of urocortin, corticotrophin-releasing factor (CRF1/2) receptors, cyclooxygenase (COX)-2 and intercellular adhesion molecule-1 (ICAM-1) at both mRNA and protein levels were determined by RT-PCR and Western blot. Key results: Rats showed grossly visible signs and symptoms of TAO on the 12th day after sodium laurate injection. In these rats, blood was in a hypercoagulable state; plasma urocortin, prostaglandin E2 and soluble intercellular adhesion molecule-1 levels were elevated; and the expression of urocortin, CRF1 and CRF1?-receptors, COX-2 and ICAM-1 in rat femoral arteries were markedly increased. Exogenous urocortin, given for 12 days after sodium laurate, exacerbated the hypercoagulable state and augmented expression of CRF1?-receptors, COX-2 and ICAM-1. These effects were abolished by a CRF1-receptor antagonist, NBI-27914, or a non-selective CRF-receptor antagonist, astressin, but not by the CRF2-receptor antagonist, antisauvagine-30, given with exogenous urocortin. Conclusion and implications: Urocortin exacerbated the hypercoagulable state and vasculitis in a model of TAO induced by sodium laurate in rats, via CRF1-receptors. COX-2 and ICAM-1 might also have contributed to this exacerbation.

Xu, Youhua; Zhang, Rongjian; Chen, Jie; Zhang, Qichun; Wang, Juejin; Hu, Jue; Guan, Xiaowei; Jin, Lai; Fu, Hong; Gui, Bo; Guo, Yuanyuan; Li, Shengnan



Usefulness of whole-body fluorine-18-fluorodeoxyglucose positron emission tomography in patients with large-vessel vasculitis: a systematic review  

Microsoft Academic Search

The objective of this study is to systematically review the role of positron emission tomography (PET) and PET\\/computed tomography\\u000a (PET\\/CT) with fluorine-18-fluorodeoxyglucose (FDG) in patients with large-vessel vasculitis (LVV). A comprehensive literature\\u000a search of published studies through April 2011 in PubMed\\/MEDLINE and Scopus databases regarding whole-body FDG-PET and PET\\/CT\\u000a in patients with LVV was performed. We identified 32 studies including

Giorgio Treglia; Maria Vittoria Mattoli; Lucia Leccisotti; Gianfranco Ferraccioli; Alessandro Giordano


Mycobacteria other than Mycobacterium tuberculosis are not present in erythema induratum/nodular vasculitis: a case series and literature review of the clinical and histologic findings.  


Erythema induratum (EI)/nodular vasculitis (NV) is characterized by recurrent crops of tender oedematous nodules on the lower legs. A lobular panniculitis with granulomatous inflammation, vasculitis, focal necrosis and septal fibrosis is present. Mycobacterium tuberculosis DNA has been detected in some lesions by means of polymerase chain reaction (PCR). Ten cases of EI/NV were found. H&E slides were reviewed. PCR assays for M. tuberculosis and mycobacteria other than M. tuberculosis (MOTT) were performed. PCR did not reveal M. tuberculosis (0%) or MOTT (0%) DNA, with positive controls, indicating the reliability of the assays. Among the MOTT, cutaneous infections are most commonly caused by M. marinum. Subcutaneous tuberculoid granulomas may be seen with M. kansasii, M. marinum, M. scrofulaceum and M. avium complex. M. gordonae, M. szulgai and M. malmoense rarely cause cutaneous infections. M. simiae, M. gastri and M. asiaticum are probably not cutaneous pathogens. M. tuberculosis and MOTT DNA was not found in EI/NV. EI/NV has diverse aetiologies with varying pathogeneses leading to similar histologic changes. The cases analysed may not have had an infectious aetiology. However, in EI/NV, performance of PCR for MOTT as well as M. tuberculosis complex may still be beneficial, particularly in cases from immunocompromised hosts. PMID:15701084

Bayer-Garner, Ilene B; Cox, Mark D; Scott, Margie A; Smoller, Bruce R



Correlation of antineutrophil cytoplasmic antibodies with the extrarenal histopathology of Wegener's (pathergic) granulomatosis and related forms of vasculitis.  


We studied the histologic findings from extrarenal biopsies (especially of the lung or upper respiratory tract) or autopsies of 68 patients who were tested for serum antineutrophil cytoplasmic antibodies (ANCAs). We used antigen-specific assays to detect antibodies against proteinase 3 (PR3) and myeloperoxidase (MPO), the two types of ANCAs of proven diagnostic value for the spectrum of diseases that includes Wegener's (pathergic) granulomatosis, microscopic polyarteritis (microscopic polyangiitis), Churg-Strauss syndrome, idiopathic necrotizing and crescentic glomerulonephritis, and their variants. Twenty-eight patients had antibodies to PR3 and 16 had antibodies to MPO; no patient had antibodies to both. All 44 patients with ANCAs had histologic evidence of this spectrum of diseases. Thirteen patients without histologic evidence of this spectrum of diseases had negative tests for ANCAs. There were no pathologic features that reliably identified patients with one or the other type of ANCA. Eighteen of 31 patients with lesions of Wegener's granulomatosis had antibodies to PR3, seven had antibodies to MPO, and six had neither. Three of four patients with necrotizing arteries without granulomas had anti-MPO antibodies, but similar lesions were seen, together with extravascular granulomas, in three patients with anti-PR3 antibodies. Of 16 patients with alveolar hemorrhage, nine had anti-PR3 and five had anti-MPO antibodies. Two patients diagnosed clinically as having Churg-Strauss syndrome had anti-MPO antibodies. In 16 of the 25 patients with ANCAs and a histologic diagnosis of Wegener's granulomatosis the diagnosis was made on the basis of extravascular granulomatous lesions alone, which argues against the requirement for vasculitis. Of six patients with negative tests for ANCAs and histologically diagnosed Wegener's granulomatosis, none had evidence of renal involvement. We conclude that in the appropriate clinical setting the presence of anti-PR3 or anti-MPO antibodies provides reliable evidence of the above spectrum of diseases, but that subclassification (to the extent this is possible) depends on the presence of distinctive clinical or pathologic features. In patients with negative tests for ANCAs, interpretation of clinical and histologic findings remains the only definitive method of diagnosis. PMID:8381764

Fienberg, R; Mark, E J; Goodman, M; McCluskey, R T; Niles, J L



Large vessel vasculitis  

PubMed Central

Takayasu arteritis is a chronic granulomatous disease of the aorta and its major branches that usually affects women during the second and third decades of life, but it has been reported in young children. This review details the clinical, pathological and radiological features, differential diagnoses and management of the condition, focusing chiefly on the disease in children. The recent definition of Takayasu arteritis is discussed. The condition should be considered in patients with unexplained arterial hypertension or unexplained inflammatory syndromes without signs of localization. Since the disease may be life-threatening and progressive, early recognition is necessary to initiate appropriate therapy. Patients with persistent ischaemic symptoms including hypertension might benefit from revascularization procedures.

Gulati, Ashima



Recurrence of Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis in Appropriately Immunosuppressed Renal Transplant Patients: A Discussion of Two Cases  

PubMed Central

Background Granulomatosis with polyangiitis (GPA) (formerly known as Wegener's granulomatosis) is a multisystem autoimmune disease of unknown aetiology. Renal disease manifests as a crescentic glomerulonephritis, with varying degrees of renal failure. Ten percent of patients progress to end-stage kidney disease. Relapse of GPA in renal transplant patients is rare, with a rate of 0.09 relapses per patient per year. Patients and Methods We describe two cases of GPA relapse in immunosuppressed renal transplant patients. Results These patients presented with new-onset graft disfunction, having previously had an uncomplicated posttransplant course. Both patients were on appropriate doses of immunosuppressive agents at the time of relapse, with therapeutic target levels of tacrolimus. We describe the background history and management of both patients. Conclusion The cases described inform us that although recurrence of anti-neutrophil cytoplasmic antibody vasculitis in transplant patients is rare, it should remain on our list of differential diagnoses in allograft disfunction.

O'Brien, Frank J.; Abdalla, Ahad; Wong, Limy; Traynor, Carol A.; Cheriyan, Paul; Kok, Hong Kwan; Casserly, Liam; Dorman, Anthony; Conlon, Peter J.



Increased serum levels of the chemokine CXCL13 and up-regulation of its gene expression are distinctive features of HCV-related cryoglobulinemia and correlate with active cutaneous vasculitis.  


Chemokine CXCL13, also known as BCA-1 (B cell-attracting chemokine-1) or BLC (B-lymphocyte chemoattractant), is a major regulator of B-cell trafficking. Hepatitis C virus (HCV) infection may be associated with B-cell dysfunction and lymphoproliferative disorders, including mixed cryoglobulinemia (MC). This study evaluates circulating levels of CXCL13 protein and specific mRNA expression in chronically HCV-infected patients with and without MC. Compared with healthy controls and HCV-infected patients without MC, CXCL13 serum levels were significantly higher in MC patients. The highest CXCL13 levels strongly correlated with active cutaneous vasculitis. CXCL13 gene expression in portal tracts, isolated from liver biopsy tissues with laser capture microdissection, showed enhanced levels of specific mRNA in MC patients with active cutaneous vasculitis. Specific CXCL13 gene mRNA expression was also up-regulated in skin tissue of these patients. These findings paralleled specific deposits of CXCL13 protein both in the liver and in the skin. Our results indicate that up-regulation of CXCL13 gene expression is a distinctive feature of HCV-infected patients. Higher levels of this chemokine in the liver as well as in the skin of patients with active MC vasculitis suggest a possible interrelation between these biologic compartments. PMID:18550853

Sansonno, Domenico; Tucci, Felicia Anna; Troiani, Laura; Lauletta, Gianfranco; Montrone, Michele; Conteduca, Vincenza; Sansonno, Loredana; Dammacco, Franco



Neurologic manifestations of systemic vasculitis. A retrospective and prospective study of the clinicopathologic features and responses to therapy in 25 patients.  


A combined retrospective and prospective study of a patient population with either or both biopsy and angiographic evidence of systemic necrotizing vasculitis was undertaken in order to delineate the neurologic manifestations of this syndrome. The type, extent and natural history of the lesions in the peripheral and central nervous system were evaluated, together with the response fo the nervous system disease to corticosteroids or cytotoxic agents or both. In 60 percent of the patients, there was involvement of the peripheral nervous system. Four patterns of neuropathy were seen: mononeuritis multiplex, extensive mononeuritis, cutaneous neuropathy and polyneuropathy. The central nervous system was involved in 40 percent of the patients, manifested predominantly by diffuse and focal disturbances of cerebral, cerebellar and brain stem function. This study demonstrates the complexity and heterogeneity of the neurologic complications of the systemic necrotizing vasculitides. With treatment of the vasculitic process and prevention of further insult, both the peripheral and central nervous system make significant, and at times, dramatic, recovery. PMID:6269427

Moore, P M; Fauci, A S



Antineutrophil cytoplasmic antibody-associated renal vasculitis treated with autologous mesenchymal stromal cells: evaluation of the contribution of immune-mediated mechanisms.  


We report the first case of renal antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis treated with autologous mesenchymal stromal cells (MSCs). A 73-year-old man was admitted to the hospital for malaise, weight loss, and oliguria. His serum creatinine level was 2.7 mg/dL but it rapidly increased to 7.8 mg/dL; urinalysis showed proteinuria and hematuria, and the ANCA to myeloperoxidase with a perinuclear pattern (pANCA) titer was high (132 IU/mL). Renal biopsy showed necrotizing crescentic glomerulonephritis. Standard immunosuppressive therapy (cyclophosphamide and corticosteroids) was ineffective. Rituximab therapy was started, but it was discontinued after the third dose to minimize the risk of systemic spread of a severe oral Candida infection and to prevent superinfections that were facilitated by leukopenia. The patient received autologous MSCs, 1.5 × 10(6) cells/kg body weight, intravenously. After 7 days, his serum creatinine level decreased to 2.2 mg/dL, pANCA titer decreased to 75 IU/mL, and urinalysis findings normalized. Eight months later, he received a second MSC infusion because his serum creatinine level increased. In 1 week, his creatinine level decreased to 1.9 mg/dL and his pANCA titer decreased to 14 IU/mL. Immunosuppressive therapy was subsequently withdrawn. At the last follow-up visit, 12 months after the second MSC infusion, the patient remained in clinical remission without any therapy. Infusion of MSCs induced expansion of the T-lymphocyte subset expressing a regulatory T-cell phenotype (CD4(+)CD25(+)Foxp3(+)) and a notable reduction in interferon-?, interleukin 6, and tumor necrosis factor serum levels. PMID:24079687

Gregorini, Marilena; Maccario, Rita; Avanzini, Maria Antonietta; Corradetti, Valeria; Moretta, Antonia; Libetta, Carmelo; Esposito, Pasquale; Bosio, Francesca; Dal Canton, Antonio; Rampino, Teresa



A Bacterial Artificial Chromosome Transgene with Polymorphic Cd72 Inhibits the Development of Glomerulonephritis and Vasculitis in MRL-Faslpr Lupus Mice  

PubMed Central

Systemic lupus erythematosus is considered to be under the control of polygenic inheritance, developing according to the cumulative effects of susceptibility genes with polymorphic alleles; however, the mechanisms underlying the roles of polygenes based on functional and pathological genomics remain uncharacterized. In this study, we substantiate that a CD72 polymorphism in the membrane-distal extracellular domain impacts on both the development of glomerulonephritis and vasculitis in a lupus model strain of mice, MRL/MpJ-Faslpr, and the reactivity of BCR signal stimulation. We generated mice carrying a bacterial artificial chromosome transgene originating from C57BL/6 (B6) mice that contains the Cd72b locus (Cd72B6 transgenic [tg]) or the modified Cd72b locus with an MRL-derived Cd72c allele at the polymorphic region corresponding to the membrane-distal extracellular domain (Cd72B6/MRL tg). Cd72B6 tg mice, but not Cd72B6/MRL tg mice, showed a significant reduction in mortality following a marked improvement of disease associated with decreased serum levels of IgG3 and anti-dsDNA Abs. The number of splenic CD4?CD8? T cells in Cd72B6 tg mice was decreased significantly in association with a reduced response to B cell receptor signaling. These results indicate that the Cd72 polymorphism affects susceptibility to lupus phenotypes and that novel functional rescue by a bacterial artificial chromosome transgenesis is an efficient approach with wide applications for conducting a genomic analysis of polygene diseases.

Oishi, Hisashi; Tsubaki, Takahito; Miyazaki, Tatsuhiko; Ono, Masao



The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort.  


The 1996 Five-Factor Score (FFS) for systemic necrotizing vasculitides (polyarteritis nodosa [PAN], microscopic polyangiitis [MPA], and Churg-Strauss syndrome [CSS]) is used to evaluate prognosis at diagnosis. In the current study we revisited the FFS, this time including Wegener granulomatosis (WG).We analyzed clinical, laboratory, and immunologic manifestations present at diagnosis of systemic necrotizing vasculitides for 1108 consecutive patients registered in the French Vasculitis Study Group database. All patients met the American College of Rheumatology and Chapel Hill nomenclature criteria. Univariable and multivariable analyses yielded the 2009 FFS for the 4 systemic necrotizing vasculitides.Overall mortality was 19.8% (219/1108); mortality for each of the SNV is listed in descending order: MPA (60/218, 27.5%), PAN (86/349, 24.6%), CSS (32/230, 13.9%), and WG (41/311, 13.2%) (p < 0.001). The following factors were significantly associated with higher 5-year mortality: age >65 years, cardiac symptoms, gastrointestinal involvement, and renal insufficiency (stabilized peak creatinine ?150 ?mol/L). All were disease-specific (p < 0.001); the presence of each was accorded +1 point. Ear, nose, and throat (ENT) symptoms, affecting patients with WG and CSS, were associated with a lower relative risk of death, and their absence was scored +1 point (p < 0.001). Only renal insufficiency was retained (not proteinuria or microscopic hematuria) as impinging on outcome. According to the 2009 FFS, 5-year mortality rates for scores of 0, 1, and ?2 were 9%, 21% (p < 0.005), and 40% (p < 0.0001), respectively.The revised FFS for the 4 systemic necrotizing vasculitides now comprises 4 factors associated with poorer prognosis and 1 with better outcome. The retained items demonstrate that visceral involvement weighs heavily on outcome. The better WG prognosis for patients with ENT manifestations, even for patients with other visceral involvement, compared with the prognosis for those without ENT manifestations, probably reflects WG phenotype heterogeneity. PMID:21200183

Guillevin, Loďc; Pagnoux, Christian; Seror, Raphaele; Mahr, Alfred; Mouthon, Luc; Le Toumelin, Philippe



Presentation and outcome of gastrointestinal involvement in systemic necrotizing vasculitides: analysis of 62 patients with polyarteritis nodosa, microscopic polyangiitis, Wegener granulomatosis, Churg-Strauss syndrome, or rheumatoid arthritis-associated vasculitis.  


We reviewed the medical records of 62 patients with systemic small and medium-sized vessel vasculitides and gastrointestinal tract involvement followed at our institution between 1981 and 2002. This group included 46 men and 16 women (male:female ratio, 2.9), with a mean age of 48 +/- 18 years. Vasculitides were distributed as follows: 38 polyarteritis nodosa (21 related to hepatitis B virus), 11 Churg-Strauss syndrome, 6 Wegener granulomatosis, 4 microscopic polyangiitis, and 3 rheumatoid arthritis-associated vasculitis. Gastrointestinal manifestations were present at or occurred within 3 months of diagnosis in 50 (81%) patients and were mainly abdominal pain in 61 (97%), nausea or vomiting in 21 (34%), diarrhea in 17 (27%), hematochezia or melena in 10 (16%), and hematemesis in 4 (6%). Gastroduodenal ulcerations were detected endoscopically in 17 (27 %) patients, esophageal in 7 (11%), and colorectal in 6 (10%), but histologic signs of vasculitis were found in only 3 colon biopsies. Twenty-one (34%) patients had a surgical abdomen; 11 (18%) developed peritonitis, 9 (15%) had bowel perforations, 10 (16%) bowel ischemia/infarction, 4 (6%) intestinal occlusion, 6 (10%) acute appendicitis, 5 (8%) cholecystitis, and 3 (5%) acute pancreatitis. (Some patients had more than 1 condition.) Sixteen (26%) patients died.The respective 10-month and 5-year survival rates were 71% (95% confidence interval [CI], 52-90) and 56% (95% CI, 35-77) for the 21 surgical patients; and 94% (95% CI, 87-101) and 82% (95% CI, 70-94) for the 41 patients without surgical abdomen (p = 0.08). Peritonitis (hazard ratio [HR] = 4.3, p < 0.01), bowel perforations (HR = 5.7, p < 0.01), gastrointestinal ischemia or infarctions (HR = 4.1, p < 0.01), and intestinal occlusion (HR = 5.5, p < 0.01) were the only gastrointestinal manifestations significantly associated with increased mortality in multivariate analysis. For this subgroup of 15 patients, 6-month and 5-year survival rates were 60% (95% CI, 35-85) and 46% (95% CI, 19-73), respectively (p = 0.003). None of the other gastrointestinal or extraintestinal vasculitis-related symptoms, or angiographic abnormalities (seen in 67% of the 39 patients who underwent angiography), was predictive of surgical complications or poor outcome. However, prognosis has dramatically improved during the past 30 years, probably owing to better management of these more severely ill patients, with prompt surgical intervention when indicated, and the combined use of steroids and immunosuppressants. PMID:15758841

Pagnoux, Christian; Mahr, Alfred; Cohen, Pascal; Guillevin, Loďc



Longitudinal studies of patients with ANCA vasculitis demonstrate concurrent reactivity to complementary PR3 protein segments cPR3m and cPR3C and with no reactivity to cPR3N  

PubMed Central

Antibodies recognizing the complement of the middle of PR3 (cPR3m) occur in ~30% of PR3-ANCA-vasculitis patients and immunization of animals with a peptide complementary to the middle of PR3 (cPR3m) induces not only anti-complementary PR3 antibodies, but also anti-PR3 antibodies derived through an anti-idiotypic response. PR3 epitopes recognized by patient ANCA however, are not restricted to the middle of PR3. This prompted us to test for antibodies that react with proteins complementary to the terminal regions of PR3 (cPR3C and cPR3N) in PR3-ANCA patients. Anti-cPR3C reactivity was detected in 28% of patients but anti-cPR3N reactivity in only 15%. Ranked anti-cPR3C and anti-cPR3m reactivity correlated in the cohort, whereas there was no significant relationship between cPR3C and cPR3N reactivity. Serial samples from three patients’ revealed that anti-cPR3C and anti-cPR3m reactivity followed a similar pattern over time. Serial samples from a fourth patient demonstrated an anti-cPR3N response without concurrent cPR3m or cPR3C reactivity. Epitope determination by mass spectrometry identified a thirteen amino acid sequence on cPR3C that contained a common binding site recognized by antibodies from three patients. This peptide sequence contains a “PHQ” motif which was reported to be the basis for cross-reactivity of anti-cPR3m antibodies with plasminogen. Why these antibodies are detected in only ~30% of the patients remains unclear. The data reveal it is not due to lack of inclusion of flanking regions of complementary PR3 during screening. Instead, quite unexpectedly, the data demonstrate that patients’ antibodies react with a restricted epitope that exists in both cPR3m and cPR3C.

Hewins, Peter; Belmonte, Frances; Jennette, J Charles; Falk, Ronald J; Preston, Gloria A



Determinants of vessel targeting in vasculitis.  


Studies of autoimmune diseases have not yet elucidated why certain organs or vessels become the objects of injury while others are spared. This paper explores the hypothesis that important differences exist in regions of the aorta; these regional variations determine vulnerability to such diseases as atherosclerosis, aortitis, giant-cell arteritis, and Takayasu's disease. The reader is invited to reassess two issues: (1) whether the aorta is indeed a single homogeneous structure; and (2) whether the initial stage of aortitis (and indeed other diseases considered "autoimmune") may primarily be the result of acquired alterations of substrate that influence unique immune profiles, but that by themselves may not be pathogenic. Disease susceptibility and patterns are influenced by many factors that are either inborn or acquired. Examples include genetic background, gender, ethnicity, aging, prior and concomitant illnesses, habits, diet, and exposure to toxins and other environmental hazards. Studies of vascular diseases must assess how such variables affect regional anatomic differences in endothelial cells, subendothelial matrix, and vascular smooth muscle, as well as the response of each to a variety of stimuli. PMID:16126975

Hoffman, Gary S



Choroidal involvement in systemic necrotizing vasculitis.  


Wegener's granulomatosis and lymphomatosis are systemic necrotizing vasculitides of unknown cause. We report similar involvement of the choriocapillaris in these two diseases. A presumed choriocapillaritis caused ischemia and infarction of the choriocapillaris-retinal pigment epithelium-outer neurosensory retina complex. Systemic corticosteroid and immunosuppressive agents were successful in controlling systemic disease and preserving useful vision. At the time of this study, one patient had not had a recurrence for 12 years, and the other patient had been asymptomatic for seven years. PMID:3606453

Kinyoun, J L; Kalina, R E; Klein, M L



A case of isolated susac occlusive retinal vasculitis.  


: Susac syndrome is characterized by encephalopathy, sensorineural hearing loss, and branch retinal artery occlusion. Additional ocular findings include arteriolar wall hyperfluorescence and Gass plaques. We present a 51-year-old Caucasian woman with ophthalmologic findings indicative of Susac syndrome in the setting of tinnitus and migraine with aura. PMID:23612239

Seamone, Mark E; Fielden, Michael



Nasal tip necrosis—an unusual presentation of rheumatoid vasculitis  

Microsoft Academic Search

We describe an unusual case of cutaneous necrosis of the nasal tip presenting to a facial reconstructive surgeon. The patient\\u000a had developed this painless necrosis over a period of about 10 days. Her past medical history included rheumatoid arthritis.\\u000a She described an exacerbation of her arthritic symptoms in the weeks preceding the development of the nasal tip necrosis.\\u000a Her rheumatoid arthritis

G. E. Glass; A. V. Greig; J. Weir; N. Waterhouse



MR Imaging and Angiography of Primary CNS Vasculitis of Childhood  

Microsoft Academic Search

METHODS: We analyzed the MR imaging features of a single pediatric center cohort of 45 cPACNS patients. MR imaging studies were performed for all patients, and both MR imaging and MRA were performed for 42 patients, who formed the cohort for review of the presence and correlation of lesions. Proportions were calculated by using the Fisher exact test, and agreement

R. I. Aviv; S. M. Benseler; E. D. Silverman; P. N. Tyrrell; G. DeVeber; L. M. Tsang; D. Armstrong



Antiendothelial Cells Autoantibodies in Vasculitis-Associated Systemic Diseases  

Microsoft Academic Search

Antiendothelial cell antibodies (AECA) have been detected in healthy individuals, as well as in autoimmune and systemic inflammatory\\u000a diseases, including systemic vasculitides. AECA have been reported in large vessel vasculitides such as giant cell arteritis\\u000a and Takayasu arteritis; medium-sized vessel vasculitides, such as polyarteritis nodosa related to hepatitis B virus infection\\u000a and Kawasaki disease; and small-sized vessel vasculitides, such as

Philippe Guilpain; Luc Mouthon



MRI in human immunodeficiency virus-associated cerebral vasculitis  

Microsoft Academic Search

Cerebral ischaemia caused by inflammatory vasculopathies has been described as complication of human immunodeficiency virus\\u000a (HIV) infection. Imaging studies have shown ischaemic lesions and changes of the vascular lumen, but did not allow demonstration\\u000a of abnormalities within the vessel wall itself. Two HIV-infected men presented with symptoms of a transient ischaemic attack.\\u000a Initial MRI of the first showed no infarct;

J. Berkefeld; W. Enzensberger; H. Lanfermann



Therapy for Severe Vasculitis Shows Long-Term Effectiveness  


... sites by the National Center for Advancing Translational Sciences and the National Institute of Arthritis and Musculoskeletal and Skin Diseases, components of NIH, and by the Arthritis Foundation. The identifier for the study Rituximab ...


Therapy of resistant systemic necrotizing vasculitis. Polyarteritis, Churg-Strauss syndrome, Wegener's granulomatosis, and hypersensitivity vasculitis group disorders.  


The systemic necrotizing vasculitides represent a difficult group of diseases from a therapeutic perspective. Even with successful therapy patients often experience a high degree of disease and treatment morbidity. Refractory disease, or that which fails to respond to standard therapy, is particularly challenging. A clinical approach to several vasculitic syndromes is outlined in this article with emphasis given to therapies supported by controlled investigations and those supported by consensus opinion where available. PMID:7732173

Calabrese, L H; Hoffman, G S; Guillevin, L



Automated interpretation of ANCA patterns - a new approach in the serology of ANCA-associated vasculitis.  


ABSTRACT: INTRODUCTION: Indirect immunofluorescence (IIF) employing ethanol-fixed neutrophils (ethN) is still the method of choice for assessing antineutrophil cytoplasmic antibodies (ANCA) in ANCA-associated vasculitides (AAV). However, conventional fluorescence microscopy is subjective and prone to high variability. The objective of this study was to evaluate novel pattern recognition algorithms for the standardized automated interpretation of ANCA patterns. METHODS: Seventy ANCA-positive samples (20 antimyeloperoxidase ANCA, 50 antiproteinase3 ANCA) and 100 controls from healthy individuals analyzed on ethN and formalin-fixed neutrophils (formN) by IIF were used as a 'training set' for the development of pattern recognition algorithms. Sera from 342 patients ('test set') with AAV and other systemic rheumatic and infectious diseases were tested for ANCA patterns using the novel pattern recognition algorithms and conventional fluorescence microscopy. RESULTS: Interpretation software employing pattern recognition algorithms was developed enabling positive/negative discrimination and classification of cytoplasmic ANCA (C-ANCA) and perinuclear ANCA (P-ANCA). Comparison of visual reading of the 'test set' samples with automated interpretation revealed Cohen's kappa (?) values of 0.955 on ethN and 0.929 on formN for positive/negative discrimination. Analysis of the 'test set' with regard to the discrimination between C-ANCA and P-ANCA patterns showed a high agreement for ethN (? = 0.746) and formN (? = 0.847). There was no significant difference between visual and automated interpretation regarding positive/negative discrimination on ethN and formN, as well as ANCA pattern recognition (P > 0.05, respectively). CONCLUSIONS: Pattern recognition algorithms can assist in the automated interpretation of ANCA IIF. Automated reading of ethN and formN IIF patterns demonstrated high consistency with visual ANCA assessment. PMID:23241527

Knütter, Ilka; Hiemann, Rico; Brumma, Therese; Büttner, Thomas; Großmann, Kai; Cusini, Marco; Pregnolato, Francesca; Borghi, Maria Orietta; Anderer, Ursula; Conrad, Karsten; Reinhold, Dirk; Roggenbuck, Dirk; Csernok, Elena



[Case of trimethoprim-induced hyperkalemia complicating ANCA-associated vasculitis].  


A 76-year-old man was admitted to our hospital because of severe anemia. Routine screening revealed a sigmoid adenocarcinoma, and he underwent sigmoidectomy. Post-operatively, he developed rapidly progressive glomerulonephritis. He was positive for myeloperoxidase anti-neutrophil cytoplasmic antibody. A renal biopsy revealed idiopathic crescentic glomerulonephritis of the pauci-immune type. He was treated with methylprednisolone semi-pulse therapy with clinical improvement. After the steroid pulse therapy, he was given oral prednisolone, 40 mg per day, and oral trimethoprim (TMP), 160 mg, and sulfamethoxazole (SMX), 800 mg twice weekly for chemoprophylaxis against pneumocystis pneumonia. One month after the initiation of TMP/SMX, he developed hyperkalemia and hyponatremia. His transtubular K gradient was low, and urinary potassium excretion was decreased. On the other hand, plasma renin activity and plasma aldosterone concentrations were within normal limits. These results suggested that TMP acted similarly to a potassium-sparing diuretic amiloride and reduced renal potassium excretion. Administration of calcium polystyrene sulfonate resulted in correction of the hyperkalemia without discontinuation of TMP/SMX. We emphasize that patients with impaired renal function are at the significant risk of developing trimethoprim-induced hyperkalemia even with chemoprophylaxis. PMID:22991842

Shishido, Takashi; Ryuzaki, Munekazu; Futatsugi, Koji; Takimoto, Chie; Kobayashi, Emi; Handa, Michiko; Itoh, Hiroshi



Disseminated Herpes Zoster Mimicking Rheumatoid Vasculitis in a Rheumatoid Arthritis Patient on Etanercept  

Microsoft Academic Search

Tumor necrosis factor-? (TNF?)-blocking agents are immunomodulating agents introduced for treatment of a variety of chronic inflammatory disease conditions. Adverse effects include an increased incidence of infections. Clinically, these infections often have atypical presentations that may hamper prompt diagnosis. In our report of a patient on etanercept therapy for rheumatoid arthritis, the correct diagnosis was delayed because disseminated herpes zoster

S. Tresch; R. M. Trüeb; J. Kamarachev; L. E. French; G. F. L. Hofbauer



Cerebral vasculitis and obsessive-compulsive disorder following varicella infection in childhood.  


Varicella is largely a childhood disease, with more than 90% of cases occurring in children younger than 10 years. The primary infection is characterized by generalized vesicular dermal exanthemas, which are extremely contagious. Secondary bacterial infection and varicella pneumonia, usually seen in the immunocompromised or adult populations, may have high morbidity and mortality. Varicella in childhood is a generally benign and self-limited disorder; however, severe, life-threatening neurological complications may occur. We report a previously healthy eight-year-old boy who presented with acute hemiplegia and obsessive-compulsive disorder secondary to a lesion in lentiform nuclei associated with a history of recent varicella infection. The child was treated with sertraline for obsessive-compulsive disorder symptoms and made a full recovery. PMID:19378896

Yarami?, Ahmet; Hergüner, Sabri; Kara, Bülent; Tatli, Burak; Tüzün, Umran; Ozmen, Meral


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

PubMed Central

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 Fc?RIIa/Fc?RIIIb 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.




What is the best approach to diagnosing large-vessel vasculitis?  


Temporal arteritis, including large-vessel giant cell arteritis, and Takayasu's arteritis are the two primary large-vessel vasculitides. Patients with temporal arteritis often present with headache, swollen temporal arteries, impairment of vision or symptoms of polymyalgia rheumatica. Clinical examination includes palpation of the temporal arteries and radial pulses, auscultation of the subclavian and axillary region, and fundoscopy. The presence of jaw claudication, diplopia and temporal artery abnormalities correlates with a high probability of positive histology. Duplex ultrasonography of the temporal arteries delineates a characteristic hypoechoic, oedematous wall swelling, stenoses and occlusions. It detects the same pathologies in the axillary arteries and other arteries in large-vessel giant cell arteritis. Angiography, magnetic resonance imaging, magnetic resonance angiography, electron beam computed tomography, computed tomography angiography and positron emission tomography show characteristic changes in the aorta and its primary branches in large-vessel giant cell arteritis and Takayasu's arteritis. Takayasu's arteritis often begins with diffuse symptoms such as low-grade fever, arthralgia, fatigue and weight loss. Clinical examination is important to detect bruits, pulse reduction and blood pressure differences. Profound experience exists with angiography. Other imaging methods are interesting alternatives as they are less invasive and may depict the inflammatory wall swelling. PMID:15857793

Schmidt, Wolfgang A; Gromnica-Ihle, Erika



New insights into the pathogenesis of antineutrophil cytoplasmic autoantibody-associated vasculitis.  


Antineutrophil cytoplasmic autoantibodies (ANCA) directed to proteinase 3 (PR3-ANCA) or myeloperoxidase (MPO-ANCA) are closely associated with the idiopathic systemic necrotizing vasculitides, in particular Wegener's granulomatosis, microscopic polyangiitis and its renal limited manifestation, and Churg Strauss Syndrome. Many in vitro studies show that those ANCA have phlogistic potential, particularly at the interface of neutrophils and endothelial cells. A limited number of studies in experimental animals support their pathogenetic role. However, ANCA alone are not sufficient, as based on clinical and experimental data, and other, probably exogenous factors, seem necessary for disease induction and (re)activation. Among those silica and particularly, the carriage of Staphylococcus aureus have been proposed. Besides, various genetic factors are involved in disease susceptibility. The ANCA-associated vasculitides are systemic autoimmune diseases in which the interplay of autoimmunity with environmental and genetic factors determines their clinical expression. PMID:12849060

Kallenberg, Cees G M; Rarok, Agnieszka; Stegeman, Coen A; Limburg, Pieter C



[Steroid and non-steroidal anti-inflammatory drug therapy of vasculitis].  


Before corticosteroids were used in the treatment of the systemic necrotizing vasculitides of the polyarteritis nodosa, the disease almost always progressed with variable degree of fulminance leading to death. The use of corticosteroids improves the 5-year survival. The most dramatic break-through in the therapeutic approach to the vasculitides has been clinical experience with cyclophosphamides in treating Wegener's granulomatosis. With this therapeutic regimen, Wegener's granulomatosis can now be considered a curable disease, if treated early and appropriately with therapeutic regimen. These therapeutic strategy can be extended to that of polyarteritis nodosa group of vasculitides. Now, it is clear that cyclophosphamide, either alone or in combination with pre-existing corticosteroid therapy, can effect a dramatic response if instituted early in the course of the disease process. With the advent of deeper insight as to immuno-pathogenesis and pathophysiologic mechanism, the striking therapeutic success can be expected in near future. PMID:7933607

Abe, T



[Apheresis of euglobulins, cryoglobulins and the von Willebrand factor in vasculitis].  


A method of apheresis of plasma euglobulin fraction, cryoglobulins and Willebrand factor was developed. In one session of plasmapheresis 1500-1700 ml of patient's plasma were removed, fractionated and returned. The method was used in 2 patients with immune complex vasculitides. In one of them the disease developed against a background of chronic active hepatitis, in the other patient it manifested itself in cryoglobulin- and cryofibrinogenemia. Clinical improvement was noted in both cases: the absence of myalgia, arthralgia, hemorrhagic eruption, and ulcerative-necrotic skin changes. The normalization of increased ristomycin-cofactor activity of Willebrand factor and CIC levels was noted in one case. A decrease in the content of plasma cryoglobulins, cryofibrinogen, and urine protein concentration (from 1.5 up to 0.03%) was noted in the other case. A possibility of the use of the method in other pathological conditions (DIC-syndrome, unstable angina, atherosclerotic angiopathy) accompanied by endothelial damage, was discussed. Willebrand factor multimers form complexes with low density lipoproteins therefore the removal of these complexes may be useful in the treatment of hypercholesteremia and atherosclerosis. PMID:2957814

Savchenko, V G; Iakhnina, E I; Margulis, E Ia; Shklovskii-Kordi, N E; Kuznetsov, A I



EULAR points to consider in the development of classification and diagnostic criteria in systemic vasculitis  

Microsoft Academic Search

ObjectivesThe systemic vasculitides are multiorgan diseases where early diagnosis and treatment can significantly improve outcomes. Robust nomenclature reduces diagnostic delay. However, key aspects of current nomenclature are widely perceived to be out of date, these include disease definitions, classification and diagnostic criteria. Therefore, the aim of the present work was to identify deficiencies and provide contemporary points to consider for

Neil Basu; Richard Watts; Ingeborg Bajema; Bo Baslund; Thorsten Bley; Maarten Boers; Paul Brogan; Len Calabrese; Maria C Cid; Jan Willem Cohen-Tervaert; Luis Felipe Flores-Suarez; Shouichi Fujimoto; Kirsten de Groot; Loic Guillevin; Gulen Hatemi; Thomas Hauser; David Jayne; Charles Jennette; Cees G M Kallenberg; Shigeto Kobayashi; Mark A Little; Alfred Mahr; John McLaren; Peter A Merkel; Seza Ozen; Xavier Puechal; Niels Rasmussen; Alan Salama; Carlo Salvarani; Caroline Savage; David G I Scott; Mĺrten Segelmark; Ulrich Specks; Cord Sunderköetter; Kazuo Suzuki; Vladimir Tesar; Allan Wiik; Hasan Yazici; Raashid Luqmani



Automated interpretation of ANCA patterns - a new approach in the serology of ANCA-associated vasculitis  

PubMed Central

Introduction Indirect immunofluorescence (IIF) employing ethanol-fixed neutrophils (ethN) is still the method of choice for assessing antineutrophil cytoplasmic antibodies (ANCA) in ANCA-associated vasculitides (AAV). However, conventional fluorescence microscopy is subjective and prone to high variability. The objective of this study was to evaluate novel pattern recognition algorithms for the standardized automated interpretation of ANCA patterns. Methods Seventy ANCA-positive samples (20 antimyeloperoxidase ANCA, 50 antiproteinase3 ANCA) and 100 controls from healthy individuals analyzed on ethN and formalin-fixed neutrophils (formN) by IIF were used as a 'training set' for the development of pattern recognition algorithms. Sera from 342 patients ('test set') with AAV and other systemic rheumatic and infectious diseases were tested for ANCA patterns using the novel pattern recognition algorithms and conventional fluorescence microscopy. Results Interpretation software employing pattern recognition algorithms was developed enabling positive/negative discrimination and classification of cytoplasmic ANCA (C-ANCA) and perinuclear ANCA (P-ANCA). Comparison of visual reading of the 'test set' samples with automated interpretation revealed Cohen's kappa (?) values of 0.955 on ethN and 0.929 on formN for positive/negative discrimination. Analysis of the 'test set' with regard to the discrimination between C-ANCA and P-ANCA patterns showed a high agreement for ethN (? = 0.746) and formN (? = 0.847). There was no significant difference between visual and automated interpretation regarding positive/negative discrimination on ethN and formN, as well as ANCA pattern recognition (P > 0.05, respectively). Conclusions Pattern recognition algorithms can assist in the automated interpretation of ANCA IIF. Automated reading of ethN and formN IIF patterns demonstrated high consistency with visual ANCA assessment.



Gemcitabine-induced vasculitis in advanced transitional cell carcinoma of the bladder  

Microsoft Academic Search

Background Gemcitabine (GEM) is an alternative chemotherapeutic agent for patients with metastatic bladder cancer. It is believed to be a well-balanced agent, having acceptable toxicity and enhanced antitumor activity. The integration of GEM into the initial chemotherapy plan for these patients is still being developed.Case report The patient, male, aged 56 years, was suffering from a transitional cell carcinoma of the

M. Birlik; S. Akar; E. Tuzel; F. Onen; E. Ozer; M. Manisali; Z. Kirkali; N. Akkoc



In vivo approaches to investigate ANCA-associated vasculitis: lessons and limitations  

PubMed Central

Anti-neutrophil cytoplasm autoantibody (ANCA)-associated diseases are autoimmune conditions characterized by necrotizing inflammation of small blood vessels. The immunogenesis and etiology of these conditions are unknown, but our knowledge of the immunopathogenesis has increased considerably in recent years. In this review, we discuss the animal models currently used to investigate the mechanisms of vascular injury and to test novel therapies. We outline their advantages and limitations and propose potential directions for future research.



Vasculitis resulting from a superficial femoral artery angioplasty with a paclitaxel-eluting balloon.  


Drug-eluting balloons (DEBs) coated with the antiproliferative agent paclitaxel may improve primary patency by reducing recurrent luminal stenosis. A proportion of the active drug and excipient coating are known to embolize distally, but until now, there have been no reports of adverse events resulting from their use. We report an unusual case of a painful nodular, biopsy specimen-proven vasculitic rash that afflicted the ipsilateral lower limb of a patient after superficial femoral artery treatment with a DEB. This adverse event may have implications for the use of DEB in this and other vascular territories. PMID:23642919

Thomas, Shannon D; McDonald, Robert R A; Varcoe, Ramon L



Circulating Angiopoietin-2 as a Biomarker in ANCA-Associated Vasculitis  

PubMed Central

The endothelial-specific Angiopoietin-Tie2 ligand-receptor system is an important regulator of endothelial activation. Binding of angiopoietin-2 (Ang-2) to Tie2 receptor renders the endothelial barrier responsive to pro-inflammatory cytokines. We previously showed that circulating Ang-2 correlated with disease severity in a small cohort of critically ill patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis. The current study reassessed Ang-2 as a biomarker of disease activity and relapse in AAV. Circulating Ang-2 was measured in 162 patients with severe AAV (BVAS/WG?3, with or without glomerulonephritis) in a clinical trial. Ang-2 levels during active AAV were compared to levels in the same patients during remission (BVAS/WG?=?0). Levels in clinical subsets of AAV were compared, and association with future disease course was assessed. Ang-2 levels were elevated in severe disease (median 3.0 ng/ml, interquartile range 1.9–4.4) compared to healthy controls (1.2, 0.9–1.5). However, they did not reliably decline with successful treatment (median 2.6 ng/ml, interquartile range 1.9–3.8, median change ?0.1). Ang-2 correlated weakly with BVAS/WG score (r?=?0.17), moderately with markers of systemic inflammation (r?=?0.25–0.41), and inversely with renal function (r?=??0.36). Levels were higher in patients with glomerulonephritis, but levels adjusted for renal dysfunction were no different in patients with or without glomerulonephritis. Levels were higher in patients with newly diagnosed AAV and lower in patients in whom treatment had recently been started. Ang-2 levels during active disease did not predict response to treatment, and Ang-2 levels in remission did not predict time to flare. Thus, Ang-2 appears to have limited practical value in AAV as a biomarker of disease activity at time of measurement or for predicting future activity.

Monach, Paul A.; Kumpers, Philipp; Lukasz, Alexander; Tomasson, Gunnar; Specks, Ulrich; Stone, John H.; Cuthbertson, David; Krischer, Jeffrey; Carette, Simon; Ding, Linna; Hoffman, Gary S.; Ikle, David; Kallenberg, Cees G. M.; Khalidi, Nader A.; Langford, Carol A.; Seo, Philip; St. Clair, E. William; Spiera, Robert; Tchao, Nadia; Ytterberg, Steven R.; Haubitz, Marion; Merkel, Peter A.



Large vessel vasculitis without temporal artery involvement: Isolated form of giant cell arteritis?  

Microsoft Academic Search

Diffuse arterial involvement in giant cell arteritis (GCA) is well recognized. By contrast, GCA clinically isolated to large vessels without cephalic, rheumatologic or systemic symptoms represents a much rarer manifestation of the disease.

M. Lambert; A. Weber; B. Boland; J. F. Plaen; J. Donckier



[The relationships of rapidly progressive glomerulonephritis (RPGN), crescentic glomerulonephritis and vasculitis: the clinical, histopathological and therapeutic considerations].  


Rapidly progressive glomerulonephritis (RPGN) is a rare but severe condition, with a particular poor outcome in the absence of aggressive therapy. Our study describes all RPGN consecutive cases treated during the 1994-1995 period, with special interest in revealing negative prognostic features at presentation and the optimum therapeutic strategy. 14 (20% of all ARF for the same period) cases were classified as RPGN. Although rare (30%), extrarenal symptoms were related with a more unfavourable course. Creatinine clearance at presentation was not a reliable prognostic factor in our study. ANCA was found in 86% of our patients (p-ANCA/c-ANCA = 2/1), and therapeutic success was associated with ANCA disappearance. Crescentic glomerulonephritis was seen in 93% of all cases 77% of which were type III, pauciimune, ANCA positive. Vasculitic lesions and fibrous crescents, but not % of glomerular circumference or % of affected glomeruli were also related with a poor prognosis. Only 43% of our RPGN cases survived with a normal renal function. i.v. metil-prednisolone (at presentation, as soon as possible) followed by i.v. cyclophosphamide up to six months was the best therapeutic regimen, with no important side-effects. PMID:9455438

Covic, A; Marian, D; Florea, L; Mititiuc, I; C?runtu, I; Cotu?iu, C; Covic, M


Anticentromere antibody in patients without CREST and scleroderma: association with active digital vasculitis, rheumatic and connective tissue disease  

Microsoft Academic Search

This paper looks at the problem confronting a doctor evaluating a patient with anticentromere antibody who does not have evidence of disease along the spectrum from CREST (calcinosis, Raynaud's phenomenon, oesophageal dysmotility, sclerodactyly, telangiectasia) to progressive systemic sclerosis. Of 33 people with anticentromere antibody, 21 had CREST and two had scleroderma. Of the other 10 with a positive anticentromere antibody,

J A Goldman



Imaging of large vessel vasculitis with 18 FDG PET: illusion or reality? A critical review of the literature data  

Microsoft Academic Search

Fluorine-18 fluorodeoxyglucose positron emission tomography ( 18FDG PET) plays a major role in the management of oncology patients. Owing to the singular properties of the glucose tracer, many patients suffering from non-malignant diseases such as inflammatory or infectious diseases may also derive clinical benefit from the appropriate use of metabolic imaging. Large vessel vasculitides such as giant cell arteritis and

Tarik Belhocine; Daniel Blockmans; Roland Hustinx; Johan Vandevivere; Luc Mortelmans



Full panretinal photocoagulation and early vitrectomy improve prognosis of retinal vasculitis associated with tuberculoprotein hypersensitivity (Eales' disease)  

PubMed Central

Background/aims: Eales’ disease is an uncommon vasoproliferative retinal disease affecting otherwise healthy young men that is characterised by obliterative retinal periphlebitis, with sequelae such as recurrent vitreous haemorrhage and traction retinal detachment. This study was undertaken to determine whether visual prognosis of Eales’ disease could be improved by appropriate medical and surgical treatment. Methods: The authors retrospectively studied 30 patients (46 eyes) who were treated from 1992 to 2001. Recorded data included patient age, sex, race, medical history, medications, results of the ophthalmological examination, results of diagnostic laboratory evaluation, and details of systemic and surgical treatments. The mean follow up was 10.6 months. Results: 19 patients (23 eyes) who presented with active periphlebitis received systemic steroids and antituberculous therapy. Extensive full panretinal photocoagulation was performed in 21 eyes that presented with new vessel formation and peripheral capillary closure with or without vitreous haemorrhage. Vitrectomy and endolaser panretinal photocoagulation was necessary in 15 eyes, for severe non-clearing vitreous haemorrhage in 11 eyes and vitreous haemorrhage with traction retinal detachment in four eyes. Complete regression of the disease was achieved in all eyes. Vitrectomy resulted in a significant visual improvement with 14 of the 15 eyes (93.3%) achieving ?20/200 visual acuity. Overall, the distribution of visual acuities among eyes improved from presentation to final follow up, with 36.4% of eyes having 20/40 or better acuity at presentation compared with 63.6% of eyes by final follow up. Conclusions: These results suggest that aggressive treatment of Eales’ disease with systemic steroids and antituberculous therapy, full panretinal photocoagulation and early vitrectomy, when necessary, may result in improving the anatomic and visual outcome.

El-Asrar, A M Abu; Al-Kharashi, S A



Prevention of relapsing disease in anti-neutrophil cytoplasmic antibody related necrotizing small-vessel vasculitis: the role for autoantibody guided and anti-bacterial treatment  

Microsoft Academic Search

\\u000a Within the spectrum of primary vasculitic syndromes, the anti-neutrophil cytoplasmic antibody (ANCA) related syndromes form\\u000a a distinct group with overlapping features. ANCA related small-vessel vasculitides are potentially life-threatening diseases\\u000a with high mortality. Prolonged immunosuppressive therapy (> 1 year) with cyclophosphamide and steroids is effective in inducing\\u000a disease remission and preventing early relapses in most vasculitic disorders [1-6]. Continuous use of

Maarten M. Boomsma; Coen A. Stegeman; Cees G. M. Kallenberg; Jan W. Cohen Tervaert


Differential glycosylation of polyclonal IgG, IgG-Fc and IgG-Fab isolated from the sera of patients with ANCA-associated systemic vasculitis.  


Post-translational modifications (PTMs) of proteins produced in vivo may be tissue, developmentally and/or disease specific. PTMs impact on the stability and function of proteins and offer a challenge to the commercial production of protein biotherapeutics. We have previously reported a marked deficit in galactosylation of oligosaccharides released from polyclonal IgG isolated from sera of patients with the anti-neutrophil cytoplasmic antibodies (ANCA) associated vasculitides; Wegener's granulomatosis (WG) and microscopic polyangiitis (MPA). Whilst normal polyclonal IgG molecules are glycosylated within the IgG-Fc region, approximately 20% of molecules also bear oligosaccharides attached to the variable regions of the light or heavy chain IgG-Fab. It is of interest, therefore to compare profiles of oligosaccharides released from the IgG-Fc and IgG-Fab of normal IgG with that isolated from the sera of patients with WG or MPA. This study shows that whilst the oligosaccharides released from ANCA IgG-Fc are hypogalactosylated those released from IgG-Fab are galactosylated and sialylated. These results show that hypogalactosylation of IgG-Fc is not due to a defect in the glycosylation or processing machinery. It rather suggests a subtle change in IgG-Fc conformation that influences the addition of galactose. Remarkably, this influence is exerted on all plasma cells. Interestingly, a licensed monoclonal antibody therapeutic, produced in Sp2/0 cells, is also shown to be hypogalactosylated in its IgG-Fc but fully galactosylated in its IgG-Fab. PMID:16413679

Holland, M; Yagi, H; Takahashi, N; Kato, K; Savage, C O S; Goodall, D M; Jefferis, R



Acute orbital inflammatory syndrome secondary to an ANCA-positive small-vessel vasculitis: a case report and review of the literature.  


A 61-year-old man presents with sequential painful bilateral proptosis within 36 h and orbital compartment syndrome resulting in complete loss of vision bilaterally. Sequential urgent lateral canthotomy and cantholysis were performed to reverse the compartment syndrome. Orbital imaging showed non-specific orbital inflammation. Biopsies showed necrotizing inflammation and bloodwork was positive for c-ANCA. The patient was therefore treated with prednisone and cyclophosphomide and showed good recovery of vision in one eye, and had no recurrence of orbital inflammation. ANCA-associated orbital vasculitides are rare, but must be kept in mind in the differential diagnosis of acute orbital inflammatory syndromes. PMID:22642696

Maleki, Babak; Valenzuela, Alejandra A; Huang, Weei-Yuarn; Heathcote, J Godfrey



Determining drug causation from geriatric clinical observations: The case study of a suspected hypersensitivity vasculitis with glomerular involvement associated with lansoprazole  

Microsoft Academic Search

Drug causation assessment is a systematic process, designed to estimate the probability of a drug's implication in the onset of an adverse event. Postmarketing surveillance is very useful for geriatric medicine, because it gives a better idea of a drug's efficiency and safety in special populations, such as older adults. Although the probabilistic reasoning used in drug causation assessment often

D. Samaras; N. Samaras; P. Antonini; C. Ferrier; N. Vogt-Ferrier



p38 mitogen-activated protein kinase (p38 MAPK)-mediated autoimmunity: lessons to learn from ANCA vasculitis and pemphigus vulgaris.  


Evidence is beginning to accumulate that p38 mitogen activated protein kinase (p38 MAPK) signaling pathway plays an important role in the regulation of cellular and humoral autoimmune responses. The exact mechanisms and the degree by which the p38 MAPK pathway participates in the immune-mediated induction of diseases have started to emerge. This review discusses the recent advances in the molecular dissection of the p38 MAPK pathway and the findings generated by reports investigating its role in the pathogenesis of autoimmune diseases such as rheumatoid arthritis, systemic lupus erythematosus, and autoimmune hepatitis. Application of newly-developed protocols based on sensitive flow cytometric detection has proven to be a useful tool in the investigation of the phosphorylation of p38 MAPK within different peripheral blood mononuclear cell populations and may help us to better understand the enigmatic role of this signaling cascade in the induction of autoimmunity as well as its role in immunosuppressive-induced remission. Special attention is paid to reported data proposing a specific role for autoantibody-induced activation of p38 MAPK-mediated immunopathology in the pathogenesis of autoimmune blistering diseases and anti-neutrophilic antibody-mediated vasculitides. PMID:23207287

Mavropoulos, Athanasios; Orfanidou, Timoklia; Liaskos, Christos; Smyk, Daniel S; Billinis, Charalambos; Blank, Miri; Rigopoulou, Eirini I; Bogdanos, Dimitrios P



[The viewpoint of the dermatologist toward white atrophy].  


Milian's white atrophy (1929), linked by English authors with livedoid vasculitis and segmentary hyaline vasculitis, is "the microvascular expression of diseases of variable aetiologies". It can be isolated, without vasculitis (idiopathic or secondary to an inflammatory livedo); the variety with vasculitis, most often observed, stems from loco-regional causes (above all from post-phlebitic venous hypertension) but also from general causes (collagenoses, infections, carcinamatoses). The need for an attempt at a better approach to white atrophy is clear. PMID:3786432

Passas, H


Overview of the 2012 revised International Chapel Hill Consensus Conference nomenclature of vasculitides.  


The nomenclature and classification of vasculitis has been difficult and controversial for many decades. This is problematic both for research on vasculitis as well as clinical care of patients with vasculitis. The first (1994) International Chapel Hill Consensus Conference on the Nomenclature of Systemic Vasculitides (CHCC) proposed names and definitions for the most common forms of vasculitis. Since then, there have been substantial advances in our understanding of vasculitis and changes in medical terminology. In addition, CHCC 1994 did not propose a nomenclature for some relatively common forms of vasculitis, such as vasculitis secondary to other diseases. To address these issues, a second International Chapel Hill Consensus Conference was held in 2012. The goals were to change names and definitions as appropriate, and add important categories of vasculitis not included in CHCC 1994. This overview summarizes the 2012 CHCC and points out the changes compared to the 1994 CHCC. Notable changes include the introduction of new terms such as granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis and immunoglobulin A vasculitis and the inclusion of categories for variable vessel vasculitis and secondary forms of vasculitis. PMID:24072416

Jennette, J Charles





... Vasculitis Foundation Fellowship is possible thanks to the generous support of Genentech, Inc., and Biogen Idec and many other generous individual and corporate donors. For more information on ...


Observational Study of the Genetic Architecture of Neutrophil-Mediated Inflammatory Skin Diseases

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



How Is Raynaud's Diagnosed?  


... Causes Who Is at Risk Signs & Symptoms Diagnosis Treatments Living With Clinical Trials Links Related Topics Atherosclerosis Vasculitis Pulmonary Hypertension Related Media Videos Widgets Quizzes ...


January 28, 2011 Approval Letter - Menevo  

Center for Biologics Evaluation and Research (CBER)

... including ITP, Kawasaki's disease, myasthenia gravis, vasculitis, thrombocytopenia, arthritis, hemolytic anemia, and collagen-vascular disease ... More results from


Síntomas neurológicos de las enfermedades reumáticas  

Microsoft Academic Search

Resumen Una de las más importantes pero la menos co- mún de las manifestaciones de enfermedad autoinmune la constituye la vasculitis, la cual mo- difica la historia de la enfermedad, dependiendo del órgano afectado. Esta revisión incluye las más importantes manifestaciones y complicaciones de los sistemas nerviosos central y periférico que se desarrollan por vasculitis en las enfermedades reumáticas, ya



Hypergammaglobulinemic purpura of Waldenström.  


We report a case of a 33-year-old-woman with a one-year history of bilateral lower extremity vasculitis and laboratory evidence of hypergammaglobulinemia with otherwise unremarkable routine laboratory and rheumatologic studies. Her clinical picture, together with histopathologic evidence of leukocytoclastic vasculitis, favor a diagnosis of hypergammaglobulinemic purpura of Waldenström. PMID:23286792

Lewin, Jesse M; Hunt, Raegan; Fischer, Max; Kamino, Hidelo; Franks, Andrew G



Staphylococcus aureus and Wegener's granulomatosis  

Microsoft Academic Search

Wegener's granulomatosis (WG) is a form of systemic vasculitis. It is characterized by granulomatous inflammation in the upper and lower airways, vasculitis and necrotizing glomerulonephritis, and is strongly associated with antineutrophil cytoplasmic antibodies against proteinase 3. Since the etiology of the disease is not clear, treatment, consisting of corticosteroids and immunosuppressives, is nonspecific and associated with severe side effects. Pinpointing

Eliane R Popa; Coen A Stegeman; Cees GM Kallenberg; Jan Willem Cohen Tervaert



Haemorrhagic and perivenous encephalitis: a clinical-pathological review of 38 cases  

Microsoft Academic Search

Clinical and pathological data from eight cases of acute haemorrhagic leucoencephalitis (AHL) confirm the previously documented devastating features of this disease. Data from 30 cases of perivenous encephalitis (PVE) associated with viral diseases reveal pathological changes ranging from lymphocytic cuffing of vessels to severe vasculitis similar to the vasculitis of AHL. Relatively few cases show demyelination as a prominent feature.

M N Hart; K M Earle



Churg Strauss Syndrome after Introducing Oral Steroid to Inhaler - A Report of Three Cases  

Microsoft Academic Search

The tetrad of bronchial asthma, severe sinusitis, nasal polyp, eosinophilia, and systemic vasculitis is the main feature of allergic granulomatosis and angitis (Churg- Strauss Syndrome). This vasculitis is usually seen idiopathic in patients with a long history of asthma; oral steroids using steroid inhalers, vaccination and desensitization might be triggering factors. Drugs such as leukotriene receptor antagonists (LTRAS), penicillin, sulphonamides,

Saeid Fallah-Tafti; Davood Mansouri; Mohammad Reza Masjedi; Mehran Marashian; Delara Faridian


Acute posterior multifocal placoid pigment epitheliopathy with cerebral involvement  

Microsoft Academic Search

In a patient with angiographically proven cerebral vasculitis five months after acute posterior multifocal placoid pigment epitheliopathy (APMPPE) neurological symptoms promptly responded to steroid treatment. Cerebrospinal fluid (CSF) showed a lymphocytic pleocytosis. Magnetic resonance imaging (MRI) revealed multifocal white matter lesions in the hemispheres and the brain stem suggesting a diffuse subcortical vasculitis.

G Stoll; K Reiners; A Schwartz; F G Kaup; C Althaus; H J Freund



Correlations between plasma levels of a fibronectin isoform subpopulation and C-reactive protein in patients with systemic inflammatory disease  

PubMed Central

Surrogate markers to detect vasculitic processes prior to organ compromise are lacking. To determine if specific populations among the fibronectin (FN) family of alternatively spliced proteins correlate with parameters of vasculitis in at-risk patients, we retrospectively evaluated the association of plasma levels of total FN (TFN) and FN bearing the alternatively spliced EIIIA segment (A+FN) with clinical vasculitis status and with levels of two putative vasculitis markers (C-reactive protein [CRP] and von Willebrand factor) in a previously-studied cohort of 27 patients with systemic inflammatory disease. We found that the percentage of TFN comprised by A+FN (%A+) and A+FN, but not TFN, correlated with plasma levels of CRP, the prototypic inflammation biomarker used to detect vasculitis. These findings suggest that different FNs may confer distinct clinical information, and that their simultaneous measurement merits further investigation in our efforts to identify soluble biomarker systems to detect vasculitis.

Peters, John H.; Greasby, Tammy; Lane, Nancy; Woolf, Anthony



A diagnostic challenge: chronic myelomonocytic leukaemia and recurrent anterior ischaemic optic neuropathy.  


We report the first case of ischaemic optic neuropathy (ION) linked to chronic myelomonocytic leukaemia (CMML) and its associated vasculitis. We discuss the link between CMML and vasculitis and the evidence suggesting it can cause anterior ischaemic optic neuropathy through a vasculitic process. We highlight the difficulty and delay in diagnosis as the use of steroids masked an underlying systemic process. Recurrent ION and raised inflammatory markers should raise suspicion of vasculitis. Together with an elevated monocyte but low platelet count, CMML should be considered. PMID:23179230

De Smit, Elisabeth; O'Sullivan, Eoin



Vascular ulcers in scleroderma.  


Seven patients with scleroderma and either livedo reticularis or atrophie blanche lesions had ulcers of the lower extremity. Livedoid vasculitis, periarteritis nodosa, or endarteritis obliterans lesions were associated with the ulcers. Five patients had livedoid vasculitis, one patient had associated lupus panniculitis, and one patient had rheumatoid arthritis. Four patients had elevated ESRs, four had positive antinuclear antibody tests, and two had positive tests for rheumatoid factor. Patients with scleroderma and livedoid vasculitis or livedo reticularis and ulcers should be examined to rule out underlying vascular disease or endarteritis obliterans. PMID:6137196

Thomas, J R; Winkelmann, R K



Systemic Embolization and Myocardial Infarction due to Clinically Unrecognized Left Atrial Myxoma  

PubMed Central

Myxomas are the most common primary tumors of the heart. We report an extraordinary severe case of left atrial myxoma, presenting with stroke, myocardial infarction, and multiple arterial embolism including aorta, splenic and renal arteries, and several peripheral arteries. The patient had previously been diagnosed with systemic vasculitis, a typical but less common finding caused by multiple emboli mimicking vasculitis. The myxoma was removed and atrial septum reconstruction was performed. In summary, early diagnostic differentiation of myxoma from vasculitis is critical, and immediate surgical removal of myxoma is required as the probability of thromboembolic complications increases over time.

Vogel, Britta; Thomas, Dierk; Mereles, Derliz; Rottbauer, Wolfgang; Katus, Hugo A.



Briefing Package  

Center for Biologics Evaluation and Research (CBER)

Text Version... C) cerebellum including vermis and hemispheres, and medulla ... vasculitis observed in the brains of raxibacumab ... No brain lesions were observed in ... More results from


Treatment of Mycobacterium haemophilum infection with an antibiotic regimen including clarithromycin.  


A patient with rheumatoid arthritis developed ulcerated nodules predominantly on his legs. Skin biopsy and culture demonstrated rheumatoid vasculitis and infection with Mycobacterium haemophilum. Improvement was not seen until clarithromycin was added to his treatment regimen. PMID:7918014

Darling, T N; Sidhu-Malik, N; Corey, G R; Allen, N B; Kamino, H; Murray, J C



VRBPAC Meeting  

Center for Biologics Evaluation and Research (CBER)

Text Version... 2 - Osteoporosis and hypothyroidism diagnosed on the same date Q-Pan + AS03A Vasculitis, cutaneous 50 yo F 22 1 - More results from


Dynamics of Morphological Changes in Guinea Pig Organs after Injection of Penicillin.  

National Technical Information Service (NTIS)

A single parenteral injection of 30 guinea pigs with penicillin produced within 24 hours widespread lesions of the blood vessels in the form of vasculitis combined with focal myocarditis, focal encephalitis, bronchospasm, and liver necroses. These changes...

E. Y. Severova



Microscopic polyangiitis following recurrent Staphylococcus aureus bacteremia and infectious endocarditis.  


Secondary vasculitis resulting from unusual pathologic expressions of infections has been described and has important clinical significance. Infectious agents have also been implicated in the pathogenesis of different primary systemic necrotizing vasculitides. Infectious endocarditis is of particular importance in the differential diagnosis of a patient presenting with ANCA associated vasculitis. We report a well-documented case of a patient with recurrent Staphylococcus aureus bacteremia who developed bacterial endocarditis and also fulfilled the Chapel Hill Conference definitions for microscopic polyangiitis. To the best of our knowledge, it is the second case of bacterial endocarditis associated with both pANCA and anti-MPO specificity that fulfilled definitions for systemic necrotizing vasculitis. We emphasize the potential pathogenic role of infection as the trigger factor for the development of systemic vasculitis. PMID:17207390

Miranda-Filloy, J A; Veiga, J A; Juarez, Y; Gonzalez-Juanatey, C; Gonzalez-Gay, M A; Garcia-Porrua, C


Spontaneous ureteral rupture in a patient with systemic lupus erythematosus  

SciTech Connect

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.

Benson, C.H.; Pennebaker, J.B.; Harisdangkul, V.; Songcharoen, S.



Pharmacovigilance Review Memorandum, April 5, 2011 ...  

Center for Biologics Evaluation and Research (CBER)

Text Version... vasculitis, with an incidence of 10-30 cases per million people per year ... skin type, history of skin cancer, other co- morbidities, smoking status, and ... More results from


Polymyalgia Rheumatica and Giant Cell Arteritis  


... reasons for the patient's symptoms. The most typical laboratory finding in people with polymyalgia rheumatica is an ... Vasculitis Clinical Research Consortium are collecting clinical and laboratory information from patients with giant cell arteritis to ...


21 CFR 312.32 - IND safety reporting.  

Code of Federal Regulations, 2013 CFR

...would be unexpected (by virtue of greater severity) if the investigator brochure referred only to elevated hepatic enzymes or hepatitis. Similarly, cerebral thromboembolism and cerebral vasculitis would be unexpected (by virtue of greater specificity)...



21 CFR 600.80 - Postmarketing reporting of adverse experiences.  

Code of Federal Regulations, 2013 CFR

...necrosis would be unexpected (by virtue of greater severity) if the labeling only referred to elevated hepatic enzymes or hepatitis. Similarly, cerebral thromboembolism and cerebral vasculitis would be unexpected (by virtue of greater specificity)...



21 CFR 310.305 - Records and reports concerning adverse drug experiences on marketed prescription drugs for human...  

Code of Federal Regulations, 2013 CFR

...necrosis would be unexpected (by virtue of greater severity) if the labeling only referred to elevated hepatic enzymes or hepatitis. Similarly, cerebral thromboembolism and cerebral vasculitis would be unexpected (by virtue of greater specificity) if...



Undifferentiated Connective Tissue Disease  


... vessels. Examples of connective tissue diseases include lupus , scleroderma , rheumatoid arthritis , Sjögren's syndrome , myositis , and vasculitis . There ... connective tissue diseases, such as lupus, Sjögren's or scleroderma. More UCTD Information Overview Causes Symptoms Diagnosis Treatment ...


Giant Cell Arteritis  


... prevented by prompt diagnosis and treatment. What is giant cell arteritis? GCA is a type of vasculitis ... pain right away to their doctors. What causes giant cell arteritis? As with PMR, the cause of ...


21 CFR 866.5700 - Whole human plasma or serum immunological test system.  

Code of Federal Regulations, 2010 CFR

...serum aid in the diagnosis of any disease concerned with abnormal levels of plasma or serum proteins, e.g., agammaglobulinemia, allergies, multiple myeloma, rheumatoid vasculitis, or hereditary angioneurotic edema. (b)...



21 CFR 866.5700 - Whole human plasma or serum immunological test system.  

Code of Federal Regulations, 2010 CFR

...serum aid in the diagnosis of any disease concerned with abnormal levels of plasma or serum proteins, e.g., agammaglobulinemia, allergies, multiple myeloma, rheumatoid vasculitis, or hereditary angioneurotic edema. (b)...



A case of Degos disease successfully treated with corticosteroid combined with cyclophosphamide.  


Degos disease is a rare disorder characterized by systemic vasculitis involving various organs. There is no established, effective treatment for the disorder, and its prognosis is still poor. Combination therapy with corticosteroid and cyclophosphamide is considered effective for vasculitides involving the small arteries such as ANCA-associated vasculitis. We present here a 42-year-old man who developed Degos disease over several months, and was successfully treated using combined treatment with corticosteroid and cyclophosphamide. PMID:20352224

Kameda, Tomohiro; Dobashi, Hiroaki; Yoneda, Kozo; Susaki, Kentaro; Kuno, Toshiya; Murao, Koji; Ishida, Toshihiko



Henoch Schönlein Purpura Presenting as Duodenal Ulcer and Gastric Outlet Obstruction.  


Henoch-Schönlein purpura (HSP) is an acute small vessel leucocytoclastic vasculitis. It is the commonest vasculitis in children, with an incidence of about 10 cases per 100, 000 a year. Gastrointestinal manifestations are commonly encountered, however hematemesis and gastric outlet obstruction are rarely reported. The authors present the case of a 5-y-old boy having hematemesis, gastric outlet obstruction and multiple duodenal ulcers. He improved with steroids and conservative management. PMID:23564516

Rathore, Mukesh; Shrivastava, Rimjhim; Goyal, Ravinder; Radotra, B D; Thapa, B R




Microsoft Academic Search

An apparently novel adenovirus was associated with an epizootic of hemorrhagic disease that is believed to have killed thousands of mule deer (Odocoileus heinionus) in California (USA) during 1993-1994. A systemic vasculitis with pulmonary edema and hemorrhagic enter- opathy or a localized vasculitis associated with necrotizing stomatitis\\/pharyngitis\\/glossitis or osteo- myelitis of the jaw were common necropsy findings in animals that

Leslie W. Woods; Richard S. Manley; Philip H. W. Chiu; Matthew Burd; Robert W. Nordhausen; Michelle H. Stillian; Pamela K. Swift


Livedoid Vasculopathy and Mononeuritis Multiplex, with a Fulminant Hepatic Failure which was caused by Herpes Simplex Hepatitis: A Case Report  

PubMed Central

Livedoid vasculopathy with mononeuritis multiplex is a rare association. We are presenting a case of an unusual association of livedoid vasculopathy with mononeuritis multiplex, who developed fulminant hepatic failure which was secondary to Herpes simplex virus (HSV) hepatitis, while she was on treatment with immunosuppressants. Her skin biopsy and immunofluorescence studies showed the features of vasculitis. A biopsy from the sural nerve showed the features of chronic vasculitis.

Pai B, Sathish; Pai, Kanthilatha



Popliteal artery thrombosis in a patient with Cogan syndrome: Treatment with thrombolysis and percutaneous transluminal angioplasty  

SciTech Connect

A 31-year-old woman with Cogan syndrome (a rare form of systemic vasculitis) was evaluated for a cold, painful left foot with diminished pulses. Arteriography demonstrated thrombosis of the left popliteal artery with evidence of vasculitis. Thrombolytic therapy was begun with initial success but eventual rethrombosis. After reinitiating thrombolytic therapy combined with intraarterial vasodilator therapy, successful angioplasty was performed with sustained results, at 6-month follow-up.

Bastug, Demir E.; Dominic, Anthony; Ortiz, Orlando [Robert C. Byrd Health Sciences Center, Department of Radiology, West Virginia University (United States); DiBartolomeo, Anthony G. [Robert C. Byrd Health Sciences Center, Department of Medicine, Rheumatology Section, West Virginia University (United States); Kotzan, Jeffrey M. [Robert C. Byrd Health Sciences Center, Department of Radiology, West Virginia University (United States); Abraham, F. Matthew [Robert C. Byrd Health Sciences Center, Department of Medicine, West Virginia University (United States)



IL4 gene expression up-regulated by mercury in rat mast cells: a role of oxidant stress in IL4 transcription  

Microsoft Academic Search

In the Brown Norway (BN) rat, chemical compounds (mercuric chloride (HgCl2), D-penicillamine or gold salts) induce a Th2-dominated autoimmune syndrome with tissue injury in the form of a vasculitis and arthritis. An early phase of vasculitis in the model occurs within 24 h of an injection of HgCl2 ,i s?? T cell independent and involves the mast cell. In addition,

Zhonglin Wu; David R. Turner; David B. G. Oliveira



Recurrent pancreatic arteritis and vasculogenic relapsing pancreatitis in rheumatoid arthritis - a retrospective clinicopathologic and immunohistochemical study of 161 autopsy patients.  


The aim of this study was to determine: the prevalence, and histological characteristics of vasculitis in the pancreas, and to follow the formal pathogenesis of multifocal pancreatitis due to arteritis and/or arteriolitis (multifocal vasculogenic pancreatitis). A randomized autopsy population of 161 in-patients with rheumatoid arthritis (RA) was studied. Systemic vasculitis (SV) complicated RA in 36 (22.36%) of 161 cases; tissue samples of pancreas were available for histologic evaluation in 28 patients. Pancreatitis and vasculitis were characterized histologically and immunohistochemically. Vasculogenic, multifocal pancreatitis was not recognized clinically. Vasculitis of the pancreatic arterioles and small arteries (branches of splenic artery, upper and lower gastroduodenal arteries) can lead to local ischaemia and to regressive changes in the pancreas. This vasculogenic process is more or less widespread and multifocal, depending on the number of involved vessels and is followed by reactive inflammation, depending on the stages of the pathological process. Because of the recurrent nature of vasculitis with time these regressive changes accumulate within the pancreas and may contribute to an unexpected circulatory failure and sudden death of the patient. Vasculogenic microinfarcts in the pancreas may be clinically characterized by unexplained recurrent abdominal symptoms and spontaneous remissions which insidiously may lead to metabolic failure resistant to therapy. PMID:18975138

Bély, Miklós; Apáthy, Agnes



[Biologic therapies in the systemic vasculitides].  


Several biological therapies have been evaluated in systemic vasculitis. Anti TNF-? agents may have a role in the treatment of Takayasu's arteritis and probably in giant cell arteritis. In Kawasaki's disease, infliximab is an option in subjects with intravenous immunoglobulin-resistant disease. Anti TNF-? cannot be recommended to treat ANCA-associated vasculitis. Anti-T lymphocyte globulin and alemtuzumab could have a role in the treatment of ANCA associated vasculitis, although current information about these two biological treatments comes from conventional resistant treatment cases, so the high incidence of complications and relapses observed with these treatment may be intrinsic to the severity of the disease and not related to the biological agents. PMID:22152288

Díaz-Orta, Mariano Arturo; Rojas-Serrano, Jorge



Direct percutaneous coronary intervention for NSTEMI in a patient with seropositive Wegener's granulomatosis.  


A case of a 49-year-old man who presented with symptoms of generalized classical Wegener's granulomatosis with the exceptional complication of acute non-ST-elevation myocardial infarction (NSTEMI) is reported. Coronary arteriography revealed an extensive arteritis with multi-local stenosis of the left anterior descending coronary artery. The culprit lesion was treated by stent implantation with success while immunosuppressive treatment with cyclophosphamide and prednisolone was continued. Using arteriography, 4 months later we found normal coronary arteries without manifestation of vasculitis. Within 8 months of medical treatment complete remission was achieved and therapy was changed to low-dose methotrexate once a week. Meanwhile medical treatment has been stopped entirely. The patient is in good clinical condition. This case indicates that the adverse event of coronary vasculitis in any patient suffering from primary or secondary vasculitis can occur. Clinically significant myocardial ischemia can occur and can even lead to myocardial infarction. PMID:16142518

Schmidt, G; Gareis, R; Störk, T



Is this an exacerbation of asthma? A cautionary tale.  


Asthma exacerbations are a fairly common presentation to emergency departments. However, a careful history, clinical examination and interpretation of investigations may uncover a more serious pathology. We present an interesting case of a 44-year-old man with symptoms suggestive of an asthma exacerbation, who was found to have Churg-Strauss vasculitis/eosinophilic granulomatosis with polyangitis. He had clinical features of a vasculitis, ECG changes and a troponin T level of 7562 ng/L. Renal biopsy confirmed Churg-Strauss vasculitis. Cardiac involvement in Churg-Strauss Syndrome (CSS) is one of the leading causes of mortality. We describe the successful diagnosis and management of a patient with CSS. PMID:24072836

Sidhu, Bharat; Nanda, Uttam; Abbas, Shahab



Eritema elevatum diutinum as a differential diagnosis of rheumatic diseases: case report.  


Erythema elevatum diutinum is a chronic and rare cutaneous leukocytoclastic vasculitis, characterized by red, purple and yellow papules, plaques and nodules, distributed symmetrically on the extensor surfaces of the limbs. It is associated with several autoimmune, neoplastic and infectious processes, mainly hematological malignancies in about 30% of the cases. Joint pain and arthritis are frequent symptoms, affecting approximately 40% of the patients, indicating the need for its inclusion in the differential diagnosis of rheumatic diseases, chiefly the other presentations of leukocytoclastic vasculitis, which are characterized by the combination of rheumatic manifestations and peculiar cutaneous lesions. We report the case of an 18-year-old female patient who developed erythema elevatum diutinum and whose diagnosis was based on the morphologic characteristics, the distribution pattern of the cutaneous lesions and the histopathological findings of leukocytoclastic vasculitis. The major systemic symptom was severe arthritis. PMID:22460417

Rosa, Dário Júnior de Freitas; Machado, Ronaldo Figueiredo; Fraga, Rafael de Oliveira; Scotton, Antônio Scafuto; de Souza, Viviane Angelina; Fernandes, Bruno


Benign angiopathy of the central nervous system or reversible cerebral vasoconstriction syndrome.  


Benign angiopathy of the central nervous system is a subset of primary angiitis of the central nervous system characterized by "benign" course. It means that changes of cerebral vessels are reversible after treatment with corticosteroids and calcium channel blockers, so these abnormalities are believed to reflect vasospasm rather than true vasculitis. The diagnosis is made on the basis of clinical presentation, brain magnetic resonance imaging and cerebral angiography. We present a young man with acute onset of headache and neurologic impairment secondary to ischemic stroke with intracerebral and subarachnoid hemorrhage. Cerebral angiography showed characteristic findings of diffuse vasculitis but good response to treatment with corticosteroids and calcium channel blockers distinguish this benign angiopathy from the more aggressive form of the central nervous system vasculitis. PMID:22263392

Ivankovi?, Mira; Bogoje-Raspopovi?, Andrijana; Drobac, Marina; Mami?-Martinovi?, Drazana; Vodopi?, Maro



[Behcet's disease with pulmonary vessel involvement].  


The case of systemic vasculitis with involvement of pulmonary vessels was described. 36-years white woman with cerebral vasculitis and recurrent uveitis 5 and 3 years ago, now was admitted to hospital because of the mouth ulceration and lesions in the chest x-ray. After lung cancer exclusion, aneurysm of pulmonary artery branch was confirmed by dynamic tomocomputer examination All mentioned above manifestations were diagnosed as Behcet disease. Patient was treated with prednison, cyclophosphamide and cyclosporine. Clinical effect was observed after corticotherapy, but no improvement in chest X-ray picture was obtained also after immunosuppression. Patient died because of pulmonary haemorrhage 7 years after first symptoms of vasculitis and 2 years after first massive haemorrhage. PMID:9760800

Wiatr, E; Chmielewska, B; Jotowicz, E; Pawlicka, L; Remiszewski, P; Rowi?ska-Zakrzewska, E



Cutaneous changes: an initial manifestation of pulmonary Wegener's granulomatosis.  


Cutaneous vasculitis can occur as an isolated dermatologic disorder or as manifestation of a potentially life-threatening systemic vasculitis such as Wegener's granulomatosis (WG). The aim of the study was to characterize cutaneous lesions in 66 WG patients (30 female, 36 male) and to assess the viability of skin biopsy the diagnosis of WG. Skin involvement was observed in 21 (32%) WG patients; in 14 (21%) patients as an initial manifestation and in other seven during the diagnosis establishment. Cutaneous lesions included palpable purpura(n=10), subcutaneous nodules(=4), hemorrhagic bullae (n=3), ulcers (n=2), pustules (n=1), pyoderma gangrenosum (n =1). The patient with pulmonary WG can present initially with cutaneous symptoms and positive PR3-ANCA serologic test results. Leukocytoclastic vasculitis (LCV) was the predominant histopathologic pattern. PMID:22826081

Zycinska, Katarzyna; Wardyn, Kazimierz; Zielonka, Tadeusz M; Nitsch-Osuch, Aneta; Smolarczyk, Roman



The safety and tolerability of an HIV-1 DNA Prime-Protein Boost Vaccine (DP6-001) in healthy adult volunteers  

PubMed Central

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 reactionswere 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.

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



Effect of penicillamine treatment on immune complexes in two cases of seropositive juvenile rheumatoid arthritis  

PubMed Central

Bresnihan, F. P., and Ansell, B. M. (1976).Annals of the Rheumatic Diseases,35, 463-465. Effect of penicillamine treatment on immune complexes in two cases of seropositive juvenile rheumatoid arthritis. A correlation has previously been observed between the presence of enhancing complexes and cutaneous vasculitis in rheumatoid arthritis. Two patients with seropositive juvenile rheumatoid arthritis are described in whom enhancing complexes were detected before the appearance of cutaneous vasculitis. Their contrasting response to penicillamine is discussed in relation to the role of rheumatoid factor and antinuclear antibodies.

Bresnihan, F. P.; Ansell, Barbara M.



A case of Henoch-Schönlein purpura in the elderly: not just a 'second childhood'.  


Henoch-Schönlein Purpura (HSP) is a small vessel-vasculitis that usually affects children and adolescents; its onset in adults is uncommon.We describe a case of HSP complicated with nephritis and extensive deep vein thrombosis in an 81-year-old Caucasian woman, successfully treated with oral corticosteroids. Even at the extremes of age, HSP should be considered in the differential diagnosis of leukocytoclastic vasculitis, with a particular attention to renal involvement, because of its potential morbidity and mortality in the elderly; in addition, ruling out an occult thrombotic event in course of HSP is mandatory, especially in the presence of additional thrombotic risk factors. PMID:22732439

Soriano, Alessandra; Galati, Giovanni; Vespasiani-Gentilucci, Umberto; Gallo, Paolo; de Vincentis, Antonio; Picardi, Antonio; Afeltra, Antonella



[Pulmonary-renal syndrome].  


The pulmonary-renal syndrome is defined as a combination of diffuse alveolar hemorrhage and glomerulonephritis. The coexistence of these two clinical conditions is due to diseases with different pathogenic mechanisms. Primary systemic vasculitis and Goodpasture syndrome are the most frequent etiologies. Systemic lupus erythematosus, connective tissue diseases, negative anti neutrophil cytoplasmic antibody vasculitis and those secondary to drugs are far less common causes. An early diagnosis based on clinical, radiologic, laboratory and histologic criteria enables early treatment, thus diminishing its high morbidity-mortality rate. Therapy is based on high doses of corticosteroids, immunosuppressants, tumor necrosis factor inhibitors and plasmapheresis. PMID:20053612

Risso, Jorge A; Mazzocchi, Octavio; De All, Jorge; Gnocchi, César A



Churg-Strauss syndrome presenting with eosinophilic myocarditis: A diagnostic challenge.  


Churg-Strauss syndrome (CSS) is an unusual disease that presents as systemic vasculitis and peripheral eosinophilia in patients with an atopic constitution. Cardiac involvement is unusual and often not prominent on initial presentation, but is an important cause of morbidity and mortality in patients with CSS. We report the case of a young woman with severe acute myocarditis. Coronary arteriography demonstrated extensive focal vasculopathy, consistent with coronary vasculitis, and myocardial biopsy showed eosinophilic myocarditis. This presentation led to an initial diagnosis of CSS in this patient and appropriate therapy resulted in a spectacular remission of disease activity. PMID:23890465

Correia, Ana Sofia; Gonçalves, Alexandra; Araújo, Vítor; Almeida E Silva, Joăo; Pereira, José Manuel; Rodrigues Pereira, Pedro; Pizarro, Manuel; Silva, Joăo Carlos; Maciel, Maria Júlia



Livedoid vasculiltis associated with a double heterozygous Factor V Leiden and prothrombin G20210A gene mutations.  


Livedoid vasculitis (LV) may be an isolated condition or associated with an underlying systemic disease, including coagulation disorders, periarteritis nodosa and systemic autoimmune diseases. The classic histological features of LV (fibrin deposition on dermal vessels and the absence of important perivascular infiltrate or leucocytoclasia) argue against a primary vasculitis and is more in keeping with a thrombotic process. Factor V Leiden mutations have rarely been reported in association with LV. We report a patient with LV, who had doubly inherited thrombophilia, with heterozygous mutations in the Factor V Leiden (G1691A) and prothrombin (G20210A) genes. PMID:19793098

Khenifer, S; Thomas, L; Balme, B; Dalle, S



Superficial femoral artery aneurysm: a rare complication of wegener granulomatosis.  


Wegener granulomatosis (WG) is a systemic disease of unknown etiology characterized by necrotizing granulomatous inflammation, tissue necrosis, and variable degrees of vasculitis, typically in small and medium-sized blood vessels. The classic clinical pattern is a triad involving the upper airways, lungs, and kidneys. However, large vessel aneurysm is an extremely rare finding in WG. We describe a 67-year-old Caucasian male with formerly proven WG who presented with a progressively growing superficial femoral artery aneurysm. Histologic findings revealed necrotizing granulomatous vasculitis involving this artery. PMID:19698302

Luebke, Thomas; Aleksic, Marko; Brunkwall, Jan


Takayasu arteritis associated with hepatic sinusoidal dilatation.  


Hepatic sinusoidal dilatation (HSD) is pathological entity that is characterized by peliosis hepatis (PH) like lesions, with vascular lesions that consist of multiple cyst-like, blood-filled cavities within the liver. To the best of our knowledge, neither PH nor HSD have been associated with systemic vasculitis. We describe herein two cases of idiopathic HSD associated with Takayasu arteritis (TA), diagnosed at an early stage of vasculitis. The same endothelial target in HSD and TA, and the favorable outcome of HSD with treatment of TA, suggest a pathogenic link between the two diseases. PMID:21911899

Durant, Cécile; Martin, Jérome; Hervier, Baptiste; Gournay, Jérome; Hamidou, Mohamed


A hidden giant: Wallenberg syndrome and aortal wall thickening as an atypical presentation of a giant cell arteritis.  


We report a case of a 73-year-old woman with a brainstem stroke presenting as Wallenberg syndrome. By transoesophageal echocardiography and combined 18F-fluordeoxyglucose positron emission and CT (18F-FDG PET/CT), the diagnosis of large artery vasculitis owing to giant cell arteritis was confirmed. In the absence of classical clinical signs, the examination of the large extracranial vessels by ultrasound and 18F-FDG PET/CT played the key role in detecting a widespread vasculitis. PMID:23456154

Stengl, Katharina Luisa; Buchert, Ralph; Bauknecht, Hans; Sobesky, Jan



Prevalence of rheumatic manifestations and antineutrophil cytoplasmic antibodies in haematological malignancies. A prospective study  

Microsoft Academic Search

Objective. To evaluate the prevalence of antineutrophil cytoplasmic antibodies (ANCA) and rheumatic manifestations associated with chronic haematological malignancies. Methods. Two groups of patients were prospectively studied (group I: 60 patients with myelodysplastic syndromes and group II: 140 patients with lymphoid malignancies) for clinical 'immune' manifestations and ANCA. Results. In the myelodysplastic group, six patients had ANCA-negative systemic medium- size vasculitis,

M. A. Hamidou; S. Derenne; M. A. P. Audrain; J. M. Berthelot; A. Boumalassa; J. Y. Grolleau



Sciatic Neuropathies in Childhood: A Report of Ten Cases and Review of the Literature  

Microsoft Academic Search

Primary lesions affecting the sciatic nerve are uncommon, especially in children. Isolated sciatic nerve involvement was found in ten patients during an 81\\/2-year period at a metropolitan children's hospital. Etiologic mechanisms included three with compression and one each with stretch injury after operation using the lithotomy position, stretch injury after closed reduction of hip dislocation, puncture wound, lymphoma, hypersensitivity vasculitis

H. Royden Jones; Laurie E. Gianturco; Paul T. Gross; Jeffrey Buchhalter



Therapy Insight: the changing spectrum of rheumatic disease in HIV infection  

Microsoft Academic Search

HIV infection and AIDS have protean and multisystem manifestations throughout the various stages of infection. Progression from HIV infection to AIDS is associated with a gradual loss of immunocompetence and the occurrence of opportunistic infections and malignancies; it is also associated with immune dysregulation and persistent, prolonged immune activation that leads to autoimmune phenomena such as vasculitis and serological abnormalities.

Rashmi M Maganti; Frances M Williams; John D Reveille



CASE REPORT: Intestinal Behçet's Disease Presenting as a Massive Acute Lower Gastrointestinal Bleed  

Microsoft Academic Search

Behcet's disease was originally described in 1937 as a syndrome involving oral and genital ulceration and ocular inflammation (1). Since this time Behcet's disease has been recognized as a systemic vasculitis that can affect multiple organs of the body concur- rently or at variable times. Although it may have an acute presentation, its coarse is usually indolent and chronic. Its

Jeffrey A. Smith; Dabir Siddiqui



Rift Valley fever ocular manifestations: observations during the 1977 epidemic in Egypt  

Microsoft Academic Search

Ocular manifestations resulting from Rift Valley fever (RVF) virus infection were studied during an extensive RVF epidemic in Egypt during 1977. Colour photography and fluorescein angiography of 7 serologically diagnosed patients showed the commonest manifestations to be macular, paramacular, and\\/or extramacular retinal lesions, often occurring bilaterally. Haemorrhage and oedema were frequently associated with the lesions, and vasculitis, retinitis, and vascular

A L Siam; J M Meegan; K F Gharbawi



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


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

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



Acute Tubulo-interstitial Nephritis with Positive Anti-Neutrophil Cytoplasmic Antibodies.  


Introduction: Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis involving the kidney usually comprises pauci-immune, necrotizing glomerulonephritis with crescents. Mononuclear tubulo-interstitial infiltrates are common in ANCA associated vasculitis, but these have usually been described in conjunction with glomerulitis. Acute tubulo-interstitial nephritis (ATIN) is a common cause of acute kidney injury that is most frequently induced by drugs or infections. Idiopathic ATIN has rarely been reported in association with the presence of a positive ANCA. These two entities seem to share a common immunological basis. Case report: We report a 75 years-old male patient who presented with acute kidney injury and his serum tested positive for p-ANCA by indirect immunofluoresence with a titer of 1/320. Testing by ELISA demonstrated anti-myeloperoxidase (MPO) specificity with a level of 28.8 IU/mL. His kidney biopsy showed features of ATIN with no glomerular involvement. Treatment with corticosteroids led to improvement of his kidney function and serology for ANCA became negative. In this case report ATIN seems to be associated with ANCA positivity, in the absence of other obvious causes for the acute tubulo-interstitial insult. Conclusion: ATIN can be associated with positive ANCA without features of renal-limited vasculitis or systemic vasculitis. This can occur in the absence of drug exposure. The outcome in our case was favorable with corticosteroid therapy. Key words: ANCA; Acute Tubulo-interstitial Nephritis; Pathogenesis. PMID:24053745

Hassani, Kawtar; Hamzi, Amine Mohamed; Hassani, Mohamed; Benyahia, Mohamed



A Genome-Wide Association Study Identifies Novel and Functionally Related Susceptibility Loci for Kawasaki Disease  

Microsoft Academic Search

Kawasaki disease (KD) is a pediatric vasculitis that damages the coronary arteries in 25% of untreated and approximately 5% of treated children. Epidemiologic data suggest that KD is triggered by unidentified infection(s) in genetically susceptible children. To investigate genetic determinants of KD susceptibility, we performed a genome-wide association study (GWAS) in 119 Caucasian KD cases and 135 matched controls with

David Burgner; Sonia Davila; Willemijn B. Breunis; Sarah B. Ng; Yi Li; Carine Bonnard; Ling Ling; Victoria J. Wright; Anbupalam Thalamuthu; Miranda Odam; Chisato Shimizu; Jane C. Burns; Michael Levin; Taco W. Kuijpers; Martin L. Hibberd



A genome-wide association study identifies novel and functionally related susceptibility loci for Kawasaki Disease  

Microsoft Academic Search

Kawasaki disease (KD) is a pediatric vasculitis that damages the coronary arteries in 25% of untreated and approximately 5% of treated children. Epidemiologic data suggest that KD is triggered by unidentified infection(s) in genetically susceptible children. To investigate genetic determinants of KD susceptibility, we performed a genome-wide association study (GWAS) in 119 Caucasian KD cases and 135 matched controls with

David Burgner; Sonia Davila; Willemijn B. Breunis; Sarah B. Ng; Yi Li; Carine Bonnard; Ling Ling; Victoria J. Wright; Anbupalam Thalamuthu; Miranda Odam; Chisato Shimizu; Jane C. Burns; Michael Levin; Taco W. Kuijpers; Martin L. Hibberd



Manifestations dermatologiques du syndrome des antiphospholipides  

Microsoft Academic Search

A wide variety of dermatologic manifestations has been described in the antiphospholipid syndrome (APS). The most frequent skin lesion is livedo reticularis, present not only on the limbs but also on the trunk, with a fine irregular pattern. It belongs to the arterial subset of APS. Circumscribed ulcerations, resembling livedoid vasculitis, may be the first manifestation of APS. Ulcerations may

C. Francčs; S. Barete; A. Soria


The relationship of asthma therapy and Churg-Strauss syndrome: NIH workshop summary report  

Microsoft Academic Search

The Churg-Strauss syndrome (CSS) is a distinct form of vasculitis that is notable for its eosinophilia and frequent associations with asthma and sinusitis. Because there has been an increasing recognition that CSS can develop in patients with asthma and that CSS might be associated with specific asthma treatments, the National Heart, Lung, and Blood Institute, the National Institute of Allergy

Peter F. Weller; Marshall Plaut; Virginia Taggart; Anne Trontell



Intravenous immunoglobulin therapy in rheumatic diseases  

Microsoft Academic Search

Prepared from the collective plasma of several thousand people, therapeutic intravenous immunoglobulin (IVIg) consists mostly of human polyspecific IgG. In addition to its use in primary and secondary immune deficiencies, IVIg is used in the treatment of several rheumatic conditions, including Kawasaki disease, dermatomyositis and antineutrophil cytoplasmic antibody (ANCA)-positive vasculitis. In these diseases, IVIg therapy generally involves the use of

Jagadeesh Bayry; Vir Singh Negi; Srini V. Kaveri




Microsoft Academic Search

Background and Design.- Malassezia is a dimorphic fungus which forms part of the normal skin flora, it may exist as budding yeasts in stratum corneum in patients with several cutaneous disease and can cause iatrogenic systemic infections such as fungemia, pulmonary vasculitis, peritonitis in predisposed neonates, children and adults. Malassezia may induce humoral and cellular immunity via activating the classical

A. Serda; Ayhan YÜCEL


Herbal cardiotonic pills prevent gut ischemia\\/reperfusion-induced hepatic microvascular dysfunction in rats fed ethanol chronically  

Microsoft Academic Search

AIM: Cardiotonic Pill (CP), an oral herbal medicine that includes Danshen (Salviae Miltiorrhizae), Panax notoginseny and Dyroblanops aromatica gaertn, has been clinically used for vascular diseases such as occlusive vasculitis, coronary diseases, atherosclerosis, and cerebral infarction. The main component, Salviae Miltiorrhizae, has been reported to prevent cerebral and intestinal reperfusion injury. However, little is known about the effect of CP

Yoshinori Horie; Jing-Yan Han; Shuka Mori; Masahiro Konishi; Mikio Kajihara; Takehiko Kaneko; Yoshiyuki Yamagishi; Shinzo Kato; Hiromasa Ishii; Toshifumi Hibi



Intracardiac thrombus in Behcet’s disease: A rare case in the United States  

Microsoft Academic Search

Behcet’s disease is a rare systemic disorder which is associated with an underlying vasculitis. Most cases of BD occur in Asia and North American cases are rare. Patients often present with aphthous ulcers and have a number of other clinical findings including genital lesions, skin lesions (folliculitis, erythema nodosum, and acne), and uveitis. Hemoptysis and cough in BD may be

Swapna Gopathi; Ryan T. Hurt; Juan Guardiola


Behçet's disease with a large intracardiac thrombus: a case report  

Microsoft Academic Search

Behçet's disease is recognised as a chronic multisystem disorder with vasculitis as its underlying pathological process. Cardiac involvement is rare and often associated with poor prognosis. A case of a 33 year old man with Behçet's disease, presenting with a large right ventricle and right atrial thrombus, is reported. Two dimensional (cross sectional), colour Doppler, and transoesophageal echocardiography, angiography, computed

M Baykan; S? Çelik; C Erdöl; E C Baykan; S Bahadir; H Erdöl; C Örem; H Çakirbay



Endovascular treatment of aortic and primitive iliac artery aneurysms associated with Behçet disease.  


Behçet disease is a systemic vasculitis that can cause vascular complications. We describe a 42-year-old woman with an aortic aneurysm and common right iliac aneurysm, both saccular and complicating Behçet disease. The patient was successfully treated by an endovascular method, which currently seems to be the best therapeutic choice given the frequent anastomotic complications of conventional surgical treatment. PMID:23541779

Ducos, Caroline; de Lambert, Alberic; Pirvu, Augustin; Cochet, Emmanuel; Sessa, Carmine; Magne, Jean-Luc



Mucormycosis associated with carotid cavernous fistula and cavernous carotid mycotic aneurysm.  


Mucormycosis has not been described previously as a cause of carotid-cavernous sinus fistula or mycotic aneurysm, certainly not coexisting in the same patient. CT and angiographic findings are presented in a patient who had a fistula, mycotic aneurysm, and associated cerebral vasculitis. PMID:2809556

Saff, G; Frau, M; Murtagh, F R; Silbiger, M L



Vascular diseases of the spinal cord.  


Vascular disease affecting the spinal can cause substantial neurologic morbidity. Several vascular spinal cord ailments present as neurologic emergencies, and should thus be recognizable to the practicing neurologist. We review the epidemiology, presentation, management strategies, and prognosis of various pathologies, including infarction, dural arteriovenous fistula, arteriovenous malformation, cavernous malformation, compressive epidural hematoma, vasculitis, and genetic abnormalities. PMID:23186899

Rubin, Mark N; Rabinstein, Alejandro A



Peripheral nerve involvement in Churg-Strauss syndrome  

Microsoft Academic Search

Peripheral neuropathy associated with bronchial asthma, multisystem organ dysfunction and idiopathic hypereosinophilia may be found in Churg-Strauss syndrome, hypereosinophilic syndrome and polyarteritis nodosa. Some authors have diagnosed their patients according to the presence in tissue biopsies of the three histological criteria of Churg and Strauss (necrotizing vasculitis, tissue eosinophilic infiltration, extravascular granulomas). We have observed three patients with a common

Raffaella Marazzi; Davide Pareyson; Amerigo Boiardi; Massimo Corbo; Vidmer Scaioli; Angelo Sghirlanzoni



Procalcitonin for discrimination between activity of systemic autoimmune disease and systemic bacterial infection  

Microsoft Academic Search

Objective: To investigate whether serum procalcitonin (PCT) levels could be useful to differentiate between systemic infection and the activity of the underlying disease in autoimmune disease.¶Methods: In 18 patients with systemic lupus erythematodes (SLE) and 35 patients with systemic antineutrophil cytoplasmic antibody-associated vasculitis (AAV) clinical disease activity was assessed by score systems. Infection was defined by clinical and microbiological means.

R. Brunkhorst; O. K. Eberhardt; M. Haubitz; F. M. Brunkhorst



Polyarteritis nodosa in patients with Familial Mediterranean Fever (FMF): A concomitant disease or a feature of FMF?  

Microsoft Academic Search

Background: Familial Mediterranean Fever (FMF) is caused by mutations in the gene encoding pyrin and is characterized by self-limited, recurrent attacks of fever and serositis. Vasculitis has been increasingly reported in FMF. A study evaluating the prognosis in FMF and polyarteritis nodosa (PAN) patients has not been reported previously. Objectives: To determine the special characteristics and the prognosis of PAN

Seza Ozen; Eldad Ben-Chetrit; Aysin Bakkaloglu; Hanan Gur; Keriman Tinaztepe; Meral Calguneri; Cetin Turgan; Aydin Turkmen; Ilkser Akpolat; Murat Danaci; Nesrin Besbas; Tekin Akpolat



Thrombotic Microangiopathy Associated with Parvovirus B19 Infection after Renal Transplantation  

Microsoft Academic Search

Human parvovirus B19 is considered an etiologic agent of aplastic anemia in immunosuppressed patients. Mi- croscopic vasculitis, with or without renal involvement, has recently been attributed to this viral infection in immunocom- petent patients. This study describes four cases of thrombotic renal graft microangiopathy presumably secondary to B19 infection. Twelve to 50 days after transplantation, four patients presented a renal





Microsoft Academic Search

Periodontal disease is the major cause of tooth loss in man. The initial histological picture of the inflamed gingiva is characteristic of local inflammatory reaction involving polymorphonuclear leukocytes, vasculitis and localized tissue loss. Subsequent clinical stages of periodontal disease (mild gingivitis) show histological evidence of the involvement of the immune response with initial accumulation of macrophages, and lymphocytes devoid of




Benzylthiouracil-induced glomerulonephritis.  


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

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



Hepatitis C Virus, Autoimmunity and Lymphoproliferation  

Microsoft Academic Search

We summarize clinical, laboratory and pathologic details regarding a patient who presented with extrahepatic disease manifesta- tions of hepatitis C virus (HCV) infection, including cryoglobulinemic leg ulcers due to cutaneous vasculitis, peripheral senso ri- motor neuropathy, and recurrent pulmonary infiltrates. The patient had evidence for B-cell lymphoproliferation, diagnosed as extranodal lymphoma on initial (though not subsequent) bone marrow examination, retroperitoneal




Primary angiitis of the central nervous system presenting with subacute and fatal course of disease: a case report  

Microsoft Academic Search

BACKGROUND: Primary angiitis of the central nervous system is an idiopathic disorder characterized by vasculitis within the dural confines. The clinical presentation shows a wide variation and the course and the duration of disease are heterogeneous. This rare but treatable disease provides a diagnostic challenge owing to the lack of pathognomonic tests and the necessity of a histological confirmation. CASE

Carsten Lukas; Kathy Keyvani; Christian Börnke



Superantigens and their Association with Dermatological Inflammatory Diseases: Facts and Hypotheses  

Microsoft Academic Search

Superantigens have been suggested to play an important role in the pathogenesis of several inammatory skin diseases as well as systemic diseases such as atopic dermatitis, psoriasis, vasculitis, T-cell lymphoma and autoimmune diseases. Infections often precede the onset and relapse of these diseases, and antibiotic treatment with or without additional glucocorticosteroids and immunoglobulins is occasionally successful. Superantigens are microbial proteins

Uta Jappe



Current Concepts in the Etiology and Treatment of Behçet Disease  

Microsoft Academic Search

Behçet disease is an idiopathic, multisystem, chronic, and recurrent disease characterized by exacerbations alternating with phases of quiescence, episodic panuveitis, and aggressive non-granulomatous occlusive vasculitis of the arteries and veins of any size with explosive ocular inflammatory attacks that primarily affect the retinal and anterior segment vasculature of the eye. The disease is characterized by endothelial dysfunction and may produce

Cem Evereklioglu



Behcet's disease: evaluation of a new instrument to measure clinical activity  

Microsoft Academic Search

Objective. Behcet's disease (BD) is a rare multisystem disorder characterized by vasculitis. At present, there are no laboratory markers that correlate well with the clinical activity in BD. This has led to the development of an instrument (BD Current Activity Form) to measure activity. Scoring is based on the history of new clinical features present over the preceding 4 weeks

B. B. Bhakta; P. Brennan; T. E. James; M. A. Chamberlain; B. A. Noble; A. J. Silman



Localized scleroderma and hemiatrophy in association with antibodies to double-stranded DNA.  

PubMed Central

Localized scleroderma involves primarily skin but also muscle, bone and synovium. Further associations and transitional forms have been reported. We report here two cases showing associations between localized scleroderma and vasculitis, mononeuritis multiplex, juvenile chronic arthritis and hemiatrophy. In particular our cases both possess antibodies to double-stranded DNA, a finding not previously reported. Images Figure 1 Figure 2

Adebajo, A. O.; Crisp, A. J.; Nicholls, A.; Hazleman, B. L.



Infection of Human Endothelial Cells with Spotted Fever Group Rickettsiae Stimulates Cyclooxygenase 2 Expression and Release of Vasoactive Prostaglandins  

Microsoft Academic Search

Rickettsiae, a diverse group of obligately intracellular gram-negative bacteria, include etiologic agents of the spotted fever and typhus groups of diseases. Rocky Mountain spotted fever and boutonneuse fever, due to Rickettsia rickettsii and R. conorii, respectively, are characterized by widespread infection of the vascular endothelium, microvascular injury, and vasculitis. Cultured human endothelial cells (EC) are highly suscep- tible to infection

Elena Rydkina; Abha Sahni; Raymond B. Baggs; David J. Silverman; Sanjeev K. Sahni



Scurvy: historical review and current diagnostic approach  

Microsoft Academic Search

Scurvy, a deficiency of vitamin C, now most often occurs in disadvantaged groups seen frequently in EDs: alcoholics with poor nutrition, the isolated elderly, and the institutionalized. Its prominent clinical features are lethargy; purpuric lesions, especially affecting the legs; myalgia; and, in advancing disease, bleeding from the gums with little provocation. Common misdiagnoses are vasculitis, blood dyscrasias, and ulcerative gingivitis.

Laura Pimentel



First report case of cryoglobulinemia and cryofibrinogenemia in Thailand.  


A 43-year-old monk had generalized purpura, arthritis of both shoulders, erythrocyanosis of lip and oral mucous membrane, Raynaud's phenomenon and uveitis. Platelets were normal. Cryoglobulin and cryofibrinogen were positive. Biopsy revealed vasculitis. No underlying infection, collagen vascular disease, lymphoproliferative, myeloproliferative and malignancy were found. He was diagnosed as having essential cryoglobulinemia and cryofibrinogenemia. PMID:2809461

Vithayasai, P; Rungruangtanakit, K; Vithayasai, V



[Atypical Cogan's syndrome. Based on a case].  


Cogan's syndrome is a systemic inflammatory disease that associates typical (interstitial keratitis) and atypical (such as anterior uveitis) ocular manifestations to vestibulo-auditory dysfunction. It has also a systemic vascular association of vasculitis type. We report a case of a 64 years old woman who presented an atypical form with anterior uveitis. PMID:15682920

Nandu, A; Salu, P; Caspers, L; Gordts, F; Sennesael, J