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PubMedDate not supplied

Cutaneous vasculitis: diagnosis and management.

Vasculitis is histologically defined as inflammatory cell infiltration and destruction of blood vessels. Vasculitis is classified as primary (idiopathic, eg, cutaneous leukocytoclastic angiitis, Wegener's granulomatosis) or secondary, a manifestation of connective tissue diseases, infections, adverse drug eruptions, or a paraneoplastic phenomenon. Cutaneous vasculitis, manifested as urticaria, purpura, hemorrhagic vesicles, ulcers, nodules, livedo, infarcts, or digital gangrene, is a frequent and often significant component of many systemic vasculitic syndromes such as lupus or rheumatoid vasculitis and antineutrophil cytoplasmic antibody-associated primary vasculitic syndromes such as Churg-Strauss syndrome. In most instances, cutaneous vasculitis represents a self-limited, single-episode phenomenon, the treatment of which consists of general measures such as leg elevation, warming, avoidance of standing, cold temperatures and tight fitting clothing, and therapy with antihistamines, aspirin, or nonsteroidal anti-inflammatory drugs. More extensive therapy is indicated for symptomatic, recurrent, extensive, and persistent skin disease or coexistence of systemic disease. For mild recurrent or persistent disease, colchicine and dapsone are first-choice agents. Severe cutaneous and systemic disease requires more potent immunosuppression (prednisone plus azathioprine, methotrexate, cyclophosphamide, cyclosporine, or mycophenolate mofetil). In cases of refractory vasculitis, plasmapheresis and intravenous immunoglobulin are viable considerations. The new biologic therapies that work via cytokine blockade or lymphocyte depletion such as tumor alpha inhibitor infliximab and the anti-B-cell antibody rituximab, respectively, are showing benefit in certain settings such as Wegener's granulomatosis, antineutrophil cytoplasmic antibody-associated vasculitis, Behçet's disease, and cryoglobulinemic vasculitis.

Anti-Inflammatory Agents, Non-Steroidal↗

PubMed1995

Cocaine-associated cerebral vasculitis.

Cocaine use is associated with a variety of serious neurological complications, including cerebral infarction, intracerebral and subarachnoid hemorrhage, transient ischemic attacks, migraines, and seizures. We report two cases of intracerebral hemorrhage with biopsy-proven cerebral vasculitis associated with the use of cocaine. The first case involved a 32-year-old man who presented with headache, left-sided hemiparesis, and severe hypertension and who was found to have a large right putaminal hemorrhage on cranial tomographic (CT) scan. Cerebral angiography did not show vasculitic changes, but brain tissue obtained during hematoma evacuation revealed a nonnecrotizing leukocytoclastic angiitis of the small vessels. The second case involved a 20-year-old man who presented with headache, agitation, and speech difficulty that progressed to disorientation and dysphasia. He had a large left temporoparietal hematoma seen on CT scan. Cerebral angiography was consistent with vasculitis, and brain tissue obtained during hematoma evacuation revealed a small vessel vasculitis. In both cases, thorough clinical and laboratory investigations found no evidence of systemic vasculitis or an etiologic agent other than cocaine. We also critically reviewed the previously reported cases of cocaine-associated cerebral vasculitis and the relevant medical literature to discuss the "cocaine-associated vasculitis syndrome" in the context of more established vasculitidies, including hypersensitivity vasculitis. In addition, we outline a diagnostic and therapeutic approach to patients with possible cocaine-associated vasculitis.

Adult↗

PubMed1996

Chronic hepatitis C, cryoglobulinemia, and cutaneous necrotizing vasculitis. Clinical, pathologic, and immunopathologic study of twelve patients.

BACKGROUND: Several patients with chronic hepatitis C infection and cutaneous vasculitis have been described. OBJECTIVE: The objective of this study was to define better the features of necrotizing vasculitis and mixed cryoglobulinemia in patients with hepatitis C infection. METHODS: A retrospective review of 611 patients with hepatitis C antibodies was conducted. Patients with clinical and histopathologic findings of cutaneous necrotizing vasculitis were identified. Clinical, histologic, and laboratory data were recorded. RESULTS: Twelve patients with necrotizing vasculitis and chronic hepatitis C infection were identified. Palpable purpura was the most common clinical presentation. Onset of skin lesions was usually more than 10 years after infection. The lower extremities were affected in all patients. Cryoglobulinemia of the mixed type II was present in 10 of 11 patients. Liver function tests were evaluated at the time of vasculitis in most of the patients. Rheumatoid factor was elevated in all nine patients tested. Total complement was decreased in seven of nine patients, and C4 was decreased in six of seven patients. CONCLUSION: Cutaneous vasculitis associated with cryoglobulinemia and hypocomplementemia is not uncommon in the course of chronic active hepatitis C infection. The triad of necrotizing vasculitis, chronic hepatitis C infection, and cryoglobulinemia occurs late after initial infection with hepatitis C. Antibodies to hepatitis C virus should be determined in a patient with necrotizing vasculitis, especially if liver function tests are elevated.

Adult↗

PubMed1996

Eosinophilic vasculitis in connective tissue disease.

BACKGROUND: Neutrophilic and lymphocytic vascular inflammation is common in vasculitis associated with connective tissue disease (CTD). We recently identified eight patients with CTD and eosinophilic vasculitis. OBJECTIVE: The purpose of this study was to characterize a variant form of vasculitis in CTD with eosinophilic infiltration. METHODS: Of 98 CTD patients with cutaneous necrotizing vasculitis, eight were found with predominantly eosinophilic vascular infiltration. Nine CTD patients with cutaneous neutrophilic vasculitis were identified for comparison. Clinical and laboratory findings were reviewed and compared. Indirect immunofluorescence for eosinophil granule major basic protein (MBP), neutrophil elastase, and mast cell tryptase was performed on lesional tissue. MBP levels and eosinophil survival enhancing activity were assayed in sera from three patients. RESULTS: The patients with eosinophilic vasculitis had depressed serum complement levels and peripheral blood eosinophilia; MBP levels were elevated in serum and eosinophil survival was prolonged. Immunofluorescence of tissue showed marked angiocentric eosinophil MBP staining with peripheral neutrophil elastase staining; mast cell tryptase staining was notably absent. The patients with neutrophilic vasculitis were variably hypocomplementemic and did not have peripheral blood eosinophilia. Immunofluorescence showed marked angiocentric neutrophil elastase staining with scattered eosinophil MBP staining; mast cell tryptase staining showed normal mast cell numbers. CONCLUSION: Patients with eosinophilic vasculitis, CTD, and hypocomplementemia show vessel wall destruction in association with vessel wall deposition of cytotoxic eosinophil granule MBP, which suggests that eosinophils mediate vascular damage in this disease process. In addition, perivascular mast cells appear diminished, thereby suggesting that mast cell degranulation occurs.

Adult↗

PubMed1997

Cutaneous vasculitis in Behçet's disease: a clinical and histopathologic study of 20 patients.

BACKGROUND: Despite the frequency of small vessel vasculitis in skin lesions of Behçet's disease (BD), BD is classified as a neutrophilic dermatosis. OBJECTIVE: Our goal was to determine whether the various cutaneous manifestations of BD are secondary to cutaneous vasculitis. METHODS: Twenty-three specimens with histologically proven necrotizing vasculitis from 20 of 42 patients with BD were investigated. RESULTS: The cutaneous vasculitic manifestations were characterized as erythema nodosum-like eruptions, palpable purpura, hemorrhagic blisters, infiltrated erythema, Sweet's syndrome-like eruptions, papulopustular lesions, and extragenital ulcerations. Combinations of various skin lesions were commonly seen in the same patient. Venous vessels in the entire dermis to the subcutis were affected with sparing of arterial vessels from middermis to subcutis. Histologic features included leukocytoclastic vasculitis in seven and lymphocytic vasculitis in 13 patients with extensive to focal localized fibrinoid necrosis of vessel walls. CONCLUSION: Cutaneous vasculitis in BD is predominantly venulitis or phlebitis. Approximately half (48%; 20 of 42) of BD patients with cutaneous lesions had either lymphocytic (31%; 13 of 42) or leukocytoclastic vasculitis (17%; 7 of 42). We suggest that BD be considered as a vasculitis-associated disease separate from the neutrophilic dermatoses.

Adult↗

PubMed1997

Classification and histopathologic spectrum of central nervous system vasculitis.

There are many different and diverse causes of central nervous system (CNS) vasculitis, and many nonvasculitic disorders that often mimic CNS vasculitis. CNS vasculitis is usually suspected clinically with compatible or suggestive angiographic findings, but a definitive diagnosis is not possible without biopsy confirmation, especially with CNS vasculitis mimickers. Primary CNS vasculitis, although relatively uncommon, is most important because of its overall unfavorable prognosis. Secondary CNS vasculitis occurs in association with a long list of systemic vasculitic and nonvasculitic disorders with variable brain biopsy findings. Because of the focal and segmental distribution of CNS vasculitis, a positive biopsy is diagnostic for the disease demonstrated, but a single isolated negative biopsy does not necessarily exclude primary or secondary CNS vasculitis.

Brain Diseases↗

PubMed2002

Platelet involvement in cutaneous small vessel vasculitis.

BACKGROUND: Secretory products of platelets serve important functions in inflammation and thrombosis. Participation of platelets in the tissue reaction associated with cutaneous small vessel vasculitis has not yet been evaluated, so we systematically investigated the presence of platelet aggregates in inflamed microvessels. METHODS: Thirty-six biopsies containing vasculitis and 18 biopsies with perivascular or interface type dermatitis were reviewed and adjacent sections were immunohistochemically stained with anti-CD61 antibody recognizing GPIIbIIIa receptors on platelets and with anti-von Willebrand factor (anti-vWF) antibody. RESULTS: Platelet aggregates were observed in 27 (75%) of the vasculitis biopsies and three (16.7%) of the perivascular dermatitis biopsies, of which two (11%) had traumatic vessel damage. In all vasculitis cases, platelet clumps were associated with diffuse immunostaining of the perivascular stroma with the initiator of platelet aggregation anti-vWF. In the non-vasculitis type of dermatitis anti-vWF staining remained strictly limited to the cytoplasm of endothelial cells in 10 cases, and in eight cases there was also slight diffuse perivascular staining, albeit less extensively than in vasculitis cases. CONCLUSION: Formation of platelet aggregates appears to play a thus far unrecognized role in cutaneous small vasculitis. Secretory products of platelets will likely contribute to the inflammatory response and tissue damage in vasculitis. Moreover, platelet immunohistochemistry may be helpful in the diagnosis of microvascular damage in paraffin sections.

Adolescent↗

PubMed1999

A comparison of the characteristics of circulating anti-myeloperoxidase autoantibodies in vasculitis with those in non-vasculitic conditions.

Although circulating anti-neutrophil cytoplasmic antibodies (ANCA) specific for myeloperoxidase (MPO) are strongly associated with the presence of vasculitis, they have been described in sera from patients with other conditions. High levels of anti-MPO antibodies can also persist in sera from patients with vasculitis despite the achievement of clinical remission. One possible interpretation is that a potentially pathogenic subset of anti-MPO antibodies exists, which is only present in patients with active vasculitis. We therefore compared the characteristics of anti-MPO antibodies in sera from patients with active vasculitis (n = 18) with those present in remission (n = 9) and in a disease control group (n = 10) without clinical evidence of vasculitis. The class, subclass and ability of anti-MPO antibodies from the three groups of patients to recognize three different conformational epitopes were analysed using ELISA-based techniques. The expression of an idiotope, designated 9G4, was also examined. Epitope recognition by anti-MPO antibodies from all patients tested was found to be similar. Sera from patients with active vasculitis showed an over-representation of IgG4 subclass anti-MPO antibodies and a more frequent presence of IgM class anti-MPO antibodies. In disease controls, IgG1 anti-MPO antibodies were predominant. In vitro, neutrophil activation by ANCA has been shown to be dependent on engagement of neutrophil FcgammaRIIa receptors following binding of these autoantibodies to surface-expressed ANCA antigens. We found that active vasculitis may be associated with the presence of circulating anti-MPO antibodies which do not significantly bind this receptor, suggesting that mechanisms other than those dependent on FcgammaRIIa binding should be explored. In addition, the expression of the 9G4 idiotope on anti-MPO antibodies in 60% (12/18) of patients with active vasculitis and 20% (2/10) of disease control patients may indicate a common origin for anti-MPO antibodies in different individuals.

Adult↗

PubMed2000

Antiproteinase 3- and antimyeloperoxidase-associated vasculitis.

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 between patients with anti-PR3 and those with anti-MPO-associated vasculitis. This review focuses on the clinical, histopathologic, and possibly pathophysiologic differences between anti-PR3- and anti-MPO-associated vasculitis. Although there is considerable overlap, the anti-PR3- and anti-MPO-associated vasculitides are each characterized by particular clinical and histopathological findings. Extrarenal organ manifestations and respiratory tract granulomas occur more frequently in patients with anti-PR3 than in those with anti-MPO. Anti-PR3-positive patients with NCGN generally have a more dramatic deterioration of their renal function compared with anti-MPO-positive patients. The term "ANCA-associated vasculitis" is considered as a useful concept in the presence of systemic vasculitis. Likewise, in the presence of vasculitis, the terms "anti-PR3-associated vasculitis" and "anti-MPO-associated vasculitis" are useful concepts.

Antibodies, Antineutrophil Cytoplasmic↗

PubMed2002

Skeletal muscle vasculitis exclusive of inflammatory myopathic conditions: a clinicopathologic study of 40 patients.

Vasculitis is an infrequently encountered pathology in skeletal muscle biopsy specimens, the diagnosis of which has significant therapeutic implications. This study retrospectively reviewed the clinicopathologic features of 40 patients with vasculitis (infiltration of vessel walls by inflammatory cells) diagnosed via skeletal muscle biopsy during a 27-year period of time. Cases of vasculitis associated with inflammatory myopathic conditions, such as polymyositis or dermatomyositis, were excluded from study. Forty patients, including 21 women and 19 men ranging in age from 19 to 83 years (mean of 52 years), formed the study group. The most common clinical presentations included muscle pain (n = 22, 55%), paresthesias (n = 16, 40%), and weight loss (n = 15, 38%). Westergren erythrocyte sedimentation rate was known in 31 patients and ranged from 12 to >150 mm/h (mean, 61 mm/h). Necrotizing vasculitis was identified in 35 patients (88%). The vasculitic process involved multiple vessels in 30 cases (75%). Vessel wall scarring and/or fibrosis were identified in 11 patients (28%), thrombi in 3 patients (8%), eosinophils in 2 patients (5%), and granulomas in 1 patient (3%). Neurogenic changes were observed in the majority of biopsy specimens as follows: angular atrophic esterase-positive muscle fibers (n = 35, 88%), grouped atrophy (n = 14, 35%), fiber type grouping (n = 17, 43%), and target fibers (n = 6, 15%). Other commonly identified pathologies included scattered degenerating muscle fibers (n = 15, 38%), regenerating muscle fibers (n = 17, 43%), and type II muscle fiber atrophy (n = 10, 25%). Thirty-three patients also underwent sural nerve biopsy. Vasculitis was identified in the nerve in 26 of 33 of these patients (79%). Peripheral nerve vasculitis was classified as necrotizing in 15 cases (57.7%) and as nonnecrotizing in 11 cases (42.5%). Seventeen of 33 patients (52%) with known follow-up showed clinical improvement with steroid/immunosuppressive therapy. The following 2 conclusions were made: the majority of vasculitis cases arising outside the setting of inflammatory myopathy in skeletal muscle are necrotizing, and the predominant muscle pathology in the setting of vasculitis is neurogenic atrophy, likely caused by concomitant involvement of the peripheral nervous system.

Adult↗

PubMed1987

Vasculitis in primary Sjögren's syndrome. Histologic classification and clinical presentation.

Nine out of 70 patients with primary Sjögren's syndrome had vasculitis of small and/or medium-sized vessels. The small vessel vasculitis was of the hypersensitivity type: leukocytoclastic and lymphocytic. The medium vessel vasculitis was acute necrotizing and simulated polyarteritis nodosa histologically but lacked the characteristic aneurysmal formation. A healing stage of vasculitis, presenting as endarteritis obliterans, was seen in patients with Sjögren's syndrome and a long-standing history of vasculitis. The hypersensitivity vasculitis was localized mainly in the skin and provided mild clinical symptoms. The polyarteritis nodosa-like vasculitis was localized predominantly in internal organs and was associated with life-threatening symptoms. Endarteritis obliterans was mainly seen in acral sites of the extremities and presented with recurrent symptoms of obstruction. One patient died of vasculitis. The remaining patients are alive. The presence of anemia, cryoglobulinemia, and hypocomplementemia suggested an immune-complex origin of the vasculitic process.

Adult↗

PubMed1999

A clinical and histologic study of 37 cases of immunoglobulin A-associated vasculitis.

Immunoglobulin (Ig) A-associated vasculitis is commonly equated with the multiorgan systemic vasculitic syndrome Henoch-Schonlein purpura (HSP), which occurs predominantly in the pediatric age group. By natural language search of the databases of two outpatient dermatopathology practices, the authors selected for review 37 cases of IgA-associated vasculitis, 23 of which were associated with antecedent infection, most commonly of the upper respiratory tract. Criteria for a diagnosis of HSP were met in 15 cases, 13 of which were in the setting of prior infection. Lower extremity skin involvement was ubiquitous. A more widespread form of vasculitis was also seen, particularly in the setting of previous infection. Several of the patients with previous infection had underlying medical illnesses including rheumatoid arthritis, atopy, renal failure, lupus erythematosus, insulin dependent diabetes mellitus, autoimmune thyroid disease, and Wegener's granulomatosis. In those patients lacking an apparent microbial trigger, Sjogren's disease with anti-Ro antibodies and hypergammaglobulinemia, lupus erythematosus, inflammatory bowel disease, IgA paraproteinemia, bronchogenic and prostatic carcinoma, cryoglobulinemia, and lymphoma were uncovered. Regardless of whether an infectious stimulus was implicated, certain cofactors with the potential to enhance vascular injury were uncovered; these included anti-Ro antibodies, antineutrophil cytoplasmic antibody, diabetic microangiopathy, and a hyperviscosity state. In the infective group, a pustular vasculitis, defined as a neutrophilic vascular reaction in concert with epithelial pustulation, was seen in 81% of cases versus 33% in the noninfectious group (p = 0.02). The prototypic histomorphology in the noninfective group was one of a mild cell poor leukocytoclastic vasculitis; Vasculitis was of greater severity in patients with antecedent infection (p = 0.026). An infectious trigger, typically of mucosal origin, can frequently be identified in patients with cutaneous IgA-associated vasculitis, especially those with the symptom complex of HSP. The light microscopy appears to distinguish patients who have an infectious trigger from those who do not. IgA-associated vasculitis may be a clue to the presence of certain underlying disorders where there is immune dysregulation or enhanced susceptibility to immune complex entrapment.

Adolescent↗

PubMed2000

Histopathologic features of erythema nodosum--like lesions in Behçet disease: a comparison with erythema nodosum focusing on the role of vasculitis.

Many patients with Behçet disease (BD) develop lesions that clinically resemble those of erythema nodosum (EN) but differ from that condition with regard to their microscopic features. We examined 11 sections of EN-like lesions in BD and compared them with 9 sections of classic EN using routine histopathology and immunohistochemistry so as to form a comprehensive picture of the pathologic findings in BD and to determine the role of vasculitis in the formation of lesions. Erythema nodosum-like lesions of BD are characterized by panniculitis, usually lobular or mixed septal and lobular in pattern, with variable numbers of neutrophils, lymphocytes, and histiocytes as well as variable numbers of necrotic adipocytes. Vasculitis was noted in most EN-like lesions in BD. Scattered vessels showing lymphocytic vasculitis were evident in 6 sections, and foci of leukocytoclastic vasculitis were obvious in 4 sections, sometimes with phlebitis or arteriolitis. In specimens with classic EN, we did not observe vasculitis. Only the percentages of CD3+ lymphocytes and chloroacetate esterase-positive neutrophils in the infiltrating cells showed statistically significant differences (P < 0.05) between EN-like lesions in BD and EN through immunohistochemical and enzyme cytochemical studies. Because vasculitis in the EN-like lesions in BD was extensive and not limited to areas of severe inflammation, we believe that it is primary vasculitis. We suggest that vasculitis is an important pathologic event in EN-like lesions in BD but cannot determine the extent to which other pathologic changes such as septal or lobular panniculitis, fat necrosis, neutrophilic infiltration, or microabscess formation are secondary features.

Adult↗

PubMed2004

Central nervous system vasculitis in children.

PURPOSE OF THE REVIEW: To summarize the current literature on central nervous system vasculitis in childhood because this condition remains a diagnostic and therapeutic challenge. RECENT FINDINGS: Central nervous system vasculitis in childhood may be primary or secondary to a variety of conditions including infections, collagen vascular diseases, systemic vasculitides, and malignancies. Conditions that result in vasospasm or are associated with noninflammatory vasculopathies may mimic the features of central nervous system vasculitis. Recent studies have described the clinical spectrum of CNS vasculitis in childhood. The most common presenting features are headaches and focal neurologic deficits. The diagnosis of central nervous system vasculitis remains particularly difficult because the available investigative modalities have limited sensitivities and specificities. The most helpful diagnostic tests include cerebrospinal fluid analysis, MRI of the brain, and angiography. However, brain biopsy may be required to diagnose small vessel vasculitis. SUMMARY: This review summarizes recent data on primary central nervous system vasculitis and some of the secondary CNS vasculitides in children. Awareness of the presenting clinical features of CNS vasculitis should lead to consideration of the diagnosis. Awareness of the sensitivity and specificity of the various diagnostic tests should lead to the more prudent use of invasive diagnostic tests including angiography and brain biopsy.

Brain↗

PubMed2000

Systemic vasculitis presenting as a tumorlike lesion. Four case reports and an analysis of 79 reported cases.

Systemic vasculitis might present as a tumorlike lesion that initially could misdirect the correct diagnosis and the appropriate medical treatment. The aim of the present study is to summarize all reported cases of tumorlike presentation in systemic vasculitides, in order to have comprehensive data on the characteristics of this unusual phenomenon. We report 4 cases of systemic vasculitis presenting as a tumorlike lesion. In addition, we performed a MEDLINE search of all English-language papers published from 1966 to 1999, looking for vasculitis presenting as tumorlike lesion. Details were included concerning vasculitis classification, specific characteristics, location of the "tumor," the presence or absence of systemic involvement, and whether surgery was performed before diagnosis. Seventy-nine cases of vasculitis presenting as a tumorlike lesion were found in the literature, in addition to the 4 new cases described. The average age of the reported cases was 50.5 +/- 15.8 years, and 51% were female. In 82% of the cases the "tumor" was associated with constitutional symptoms and elevated erythrocyte sedimentation rate (ESR). The most common vasculitis categories with tumorlike presentation were Wegener granulomatosis (WG; 28 cases) and giant cell arteritis (GCA; 17 cases). In almost half the patients, surgery was performed before diagnosis. All patients with GCA presented with either a breast or an ovarian tumor. The most common location of a tumorlike lesion was the breast (22%), followed by central nervous system lesions (16%). Other frequent locations were the ovary (10%), caused exclusively by GCA and polyarteritis nodosa (PAN), and the male genitourinary system, almost all caused by PAN. Including vasculitis in the differential diagnosis of a tumorlike lesion might lead to an earlier diagnosis and consequently to prompt and appropriate treatment, avoiding needless operations. Constitutional symptoms and elevated ESR should alert clinicians to the possible diagnosis of vasculitis rather than a tumor. The association of GCA with ovarian pseudotumor is distinct and has not been emphasized before. We therefore suggest that GCA should be included in the list of differential diagnosis of an ovarian or breast tumor in an elderly woman, particularly when systemic symptoms and parameters of inflammation are present.

Adult↗

PubMed2006

Cryoglobulinemic vasculitis.

PURPOSE OF REVIEW: Cryoglobulinemic vasculitis is an immune-complex-mediated systemic vasculitis involving small-medium-sized vessels. A causative role of hepatitis C virus in over 80% patients has been definitively established, with heterogeneous geographical distribution. This review focuses on recent etiopathogenetic, clinico-diagnostic, and therapeutical studies. RECENT FINDINGS: Hepatitis C virus cannot be integrated into the host genome; it may exert a chronic stimulus to the immune system. The interaction between hepatitis C virus envelope protein E2 with B-cell CD 81 receptor may increase the frequency of VDJ rearrangement in antigen-reactive B lymphocytes. One consequence is the activation of various protooncogenes, including anti-apoptotic Bcl-2. The extended B-cell survival is responsible for autoantibody and immune-complex production, including mixed cryoglobulins; some malignancies, mainly B-cell lymphomas, may complicate cryoglobulinemic vasculitis. Environmental or viral/host genetic cofactors should be relevant in the pathogenesis of hepatitis C virus-related diseases. Cryoglobulinemic vasculitis may overlap with other diseases (systemic vasculitides, Sjögren's syndrome, autoimmune hepatitis, lymphoma), which should be carefully considered for a correct diagnosis and treatment. Cumulative survival of cryoglobulinemic vasculitis is significantly lower compared with the general population. Therapeutic strategies for cryoglobulinemic vasculitis include etiologic (antiviral), pathogenetic (cyclophosfamide, rituximab), or symptomatic (steroids, plasmapheresis) treatments, which should be tailored to the individual patient according to the severity/activity of clinical symptoms. SUMMARY: Cryoglobulinemic vasculitis represents a crossroads among autoimmune and lymphoproliferative disorders; as hepatitis C virus infection is the major causative factor, cryoglobulinemic vasculitis is an important model for etiopathogenetic studies of virus-related diseases.

Cryoglobulinemia↗

PubMed2005

Cutaneous vasculitis update: diagnostic criteria, classification, epidemiology, etiology, pathogenesis, evaluation and prognosis.

Vasculitis, inflammation of the vessel wall, can result in mural destruction with hemorrhage, aneurysm formation, and infarction, or intimal-medial hyperplasia and subsequent stenosis leading to tissue ischemia. The skin, in part due to its large vascular bed, exposure to cold temperatures, and frequent presence of stasis, is involved in many distinct as well as un-named vasculitic syndromes that vary from localized and self-limited to generalized and life-threatening with multi-organ disease. To exclude mimics of vasculitis, diagnosis of cutaneous vasculitis requires biopsy confirmation where its acute signs (fibrinoid necrosis), chronic signs (endarteritis obliterans), or past signs (acellular scar of healed arteritis) must be recognized and presence of extravascular findings such as patterned fibrosis or collagenolytic granulomas noted. Although vasculitis can be classified by etiology, many cases have no identifiable cause, and a single etiologic agent can elicit several distinct clinicopathologic expressions of vasculitis. Therefore, the classification of cutaneous vasculitis is best approached morphologically by determining vessel size and principal inflammatory response. These histologic patterns roughly correlate with pathogenic mechanisms that, when coupled with direct immunofluorescent examination, anti-neutrophil cytoplasmic antibody (ANCA) status, and findings from work-up for systemic disease, allow for specific diagnosis, and ultimately, more effective therapy. Herein, we review cutaneous vasculitis focusing on diagnostic criteria, classification, epidemiology, etiology, pathogenesis, and evaluation of the cutaneous vasculitis patient.

Humans↗

PubMed2005

Titre and affinity of propylthiouracil-induced anti-myeloperoxidase antibodies are closely associated with the development of clinical vasculitis.

Substantial evidences suggested that propylthiouracil (PTU) could induced anti-myeloperoxidase (MPO) antibodies in sera from patients with hyperthyroidism, however, only a subgroup of the PTU-induced anti-MPO antibody positive patients developed clinical evident vasculitis. The aim of this study is to compare the titres and affinities of PTU induced anti-MPO antibodies in sera from patients with hyperthyroidism with and without clinical vasculitis. Anti-MPO antibody positive sera from patients diagnosed hyperthyroidism with (n = 13) and without (n = 14) clinical evident vasculitis were collected. The titre was determined by MPO-ELISA and expressed as logarithm value (lgT). The affinity constant (aK) of anti-MPO IgG was measured by antigen inhibition assay. The titre and aK values were compared between patients with and without vasculitis. In patients with vasculitis, the mean lgT of anti-MPO antibodies was 3.62 +/- 0.66; the median aK was 4.47 x 10(7)M(-1). In patients without vasculitis, the mean lgT was 2.54 +/- 0.29; the median aK was 0.14 x 10(7)M(-1), and both were significant lower than those in patients with vasculitis (t = 5.464; P = 0.000 & z = -4.373; P = 0.000, respectively). We concluded that the titre and affinity of anti-MPO antibodies might be associated with the development of clinical vasculitis in patients with PTU-induced ANCA.

Adolescent↗