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PubMed2002

Serum sFAS levels are elevated in ANCA-positive vasculitis compared with other autoimmune diseases.

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, 17 in first remission while on therapy, and 12 in quiescence. For comparison, 10 patients with Sjogren's syndrome (SS), 14 patients with systemic lupus erythematosus (SLE), 29 patients with rheumatoid arthritis (RA), 7 patients on dialysis (DP), and 26 healthy controls (HC) were studied. In addition, Fas expression in mononuclear cells was examined at the mRNA level using reverse transcriptase (RT)-PCR in 6 vasculitis patients at onset and in first remission. The expression of CD95 on the surface of leukocytes was determined by flow cytometry in 6 vasculitis patients at onset of the disease, in 6 patients in clinical remission, and in 6 HC. Expression of Fas and FasL in renal biopsy specimens was studied using immunohistochemistry. Patients with vasculitis had high sFas levels irrespective of disease phase. Both vasculitis patients and patients with RA and SLE had significantly increased sFas levels compared with healthy controls. All patient groups had sFas levels, which correlated with raised serum creatinine values. However, the sFas levels in vasculitis patients in first remission and in quiescence were increased despite a lower serum creatinine compared with onset. Some of the vasculitis patients showed an increased mRNA expression of Fas in mononuclear cells after treatment, suggesting that Fas production fluctuates with the intensity of the disease. The expression of CD95 on leukocytes was slightly decreased in vasculitis patients compared to healthy controls. No alterations of Fas and FasL expression were seen in renal biopsy specimens. These results show that ANCA-positive vasculitis patients have high sFas levels and that the levels remain elevated even in clinical remission. The findings indicate that perturbations in the Fas/Fas ligand system may play a role in the disease process in ANCA vasculitis.

Adult↗

PubMed2001

Systemic medium-sized vessel vasculitis associated with chronic myelomonocytic leukemia.

OBJECTIVE: To determine the clinical aspects of systemic vasculitis associated with chronic myelomonocytic leukemia (CMML). METHODS: In this retrospective study, 8 patients suffering from systemic vasculitis associated with CMML are described. The French and English literature on systemic vasculitis associated with myelodysplasia was reviewed. RESULTS: All 8 patients had a systemic medium-sized vessel vasculitis which fulfilled the American College of Rheumatology criteria for polyarteritis nodosa in the setting of active CMML. Antineutrophil cytoplasmic antibodies (ANCA) were negative in 7 patients. One patient had cytoplasmic ANCA by indirect immunofluorescence without antiproteinase 3 or antimyeloperoxydase antibodies on the enzyme-linked immunosorbent assay. At presentation, 6 patients had fever of unknown origin, 5 had polymyalgia rheumatica, 3 had sensory hearing loss, and 4 had eosinophilia. None had viral infection or drug-associated vasculitis. Diagnostic procedures included renal or hepatic angiography in 6 patients which showed microaneurysms in 4, skin and temporal artery biopsy in 2 which showed vasculitis, and 1 postmortem examination which showed gastroduodenal arteritis. All patients were treated with corticosteroids, and 7 received immunosuppressive drugs. Death was attributable to vasculitis in 2 cases, infection in 3, and other vasculitis-related causes in 2. In a review of the French-English literature, we found 11 similar cases of ANCA-negative systemic vasculitis, generally associated with refractory anemia, with or without blast excess. CONCLUSIONS: Systemic ANCA-negative polyarteritis nodosa-type vasculitis seems closely associated to CMML. Clinical presentation is nonspecific, and systemic vasculitis should be suspected when a patient with myelodysplasia develops atypical manifestations. Renal, gastrointestinal, or hepatic angiography are useful diagnostic procedures when more invasive biopsies should be avoided because of low platelet count. The prognosis of CMML-associated systemic vasculitis is poor.

Aged↗

PubMed1993

Abnormal plasma fibrinolysis in patients with rheumatoid arthritis and impaired endothelial fibrinolytic response in those complicated by vasculitis.

OBJECTIVES: (a) To assess plasma fibrinolytic parameters in patients with rheumatoid arthritis (RA) and to determine whether there are differences between patients with RA alone and those with RA complicated by vasculitis. (b) To determine if patients with RA respond differently to venous occlusion compared with normal subjects and to assess whether such a response differs in patients with RA alone and those with rheumatoid vasculitis. (c) To determine the extent of vascular damage in patients with rheumatoid vasculitis and if this correlates with the levels of plasma fibrinolytic parameters. METHODS: Sixty three patients with RA (38 had RA only and 25 had evidence of rheumatoid vasculitis) were recruited. Plasma levels of tissue plasminogen activator antigen (t-PA Ag), plasminogen activator inhibitor (PAI) activity, and factor VIII von Willebrand factor (vWF) were measured before and 10 minutes after venous occlusion. RESULTS: Patients with RA, with or without rheumatoid vasculitis, had higher baseline PAI levels than control subjects. The difference was statistically significant for patients with RA alone but was not statistically significant for patients with rheumatoid vasculitis. After venous occlusion, t-PA Ag levels increased significantly in normal subjects and patients with RA alone, but not in patients with rheumatoid vasculitis. Plasma levels of vWF were significantly higher in patients with rheumatoid vasculitis than in normal subjects and those with RA alone. In patients with RA alone, baseline vWF correlated positively with t-PA Ag levels, whereas a negative correlation was found between these two parameters in patients with rheumatoid vasculitis. A negative correlation between vWF and t-PA Ag levels after venous occlusion was also found in patients with rheumatoid vasculitis. CONCLUSIONS: Patients with rheumatoid vasculitis showed evidence of vascular damage with increased levels of vWF and impaired t-PA Ag release after venous occlusion, a useful measurement of endothelial reserve to remove fibrin. This may be of pathophysiological importance in the development of vasculitis in these patients.

Adult↗

PubMed2000

Defining lymphocytic vasculitis.

Vasculitis can be defined as vascular inflammation which is usually immune mediated, leading to structural and functional damage to the vessel wall. Our concept of vasculitis has been built around the events surrounding immune-complex-triggered leukocytoclastic vasculitis, while the role of lymphocyte mediated vasculitis remains largely undefined. This may be due to maintaining a stereotypic image of lymphocytic vasculitis as one associated with vascular necrosis, fibrin deposition and haemorrhage and merely substituting lymphocytes for neutrophils. Our understanding of lymphocytic vasculitis may be advanced by identifying the clinical settings in which such reactions may occur, such as autoimmune skin diseases sharing features with graft vs host disease, as well as recognizing that lymphocyte mediated inflammation may lead to a morphologically distinct group of vasculitides, apart from those associated with angiodestruction. Lymphocytic endovasculitis, lymphocytic lichenoid vasculitis and granulomatous vasculitis are potential examples of lymphocytic vasculitis that differ from the histological pattern seen in association with immune complex leukocytoclastic vasculitis. Ultimately, the proof that these vasculitides are examples of lymphocytic vasculitis will rest on new techniques emerging in molecular pathology that have the capacity of analysing the immunological events beyond the current limitations posed by routine histopathology.

Connective Tissue Diseases↗

PubMed1998

Central nervous system vasculitis.

Vasculitis is inflammation of blood vessel walls, which produces dysfunction in both the peripheral and central nervous system (CNS). Cerebral ischemia is the major cause for neurological manifestations of CNS vasculitis. Unfortunately, a universally accepted classification of vasculitis has not emerged. Vasculitis affecting the CNS alone is referred to as primary angiitis of the CNS; secondary vasculitis occurs in association with a variety of conditions, including infections, drug abuse, lymphoproliferative disease and connective tissue diseases. The pathogenesis of vasculitis includes different immunological mechanisms. Recently, anti-neutrophil cytoplasmatic antibody (ANCA) has been demonstrated to play an active role in the immunopathogenesis of the vasculitis. Diagnosis of vasculitis depends on a combination of clinical, radiographic and pathologic features. A wide spectrum of clinical features may occur. The most typical clinical picture of CNS vasculitis is troke, encephalopathy or seizures. Assays for ANCA, serum cytokines, antibodies to endothelial cell antigens have been reported to be useful in diagnosing or monitoring the disease activity. The gold standard in diagnosis is confirmation of vasculitis in a biopsy specimen. Angiography may suggest the diagnosis but no abnormalities are pathognomonic. Ideally, the therapy of each vasculitis would focus on the specific immunologic mechanism causing the disease. Such specific interventions are not yet available. In general the most important approaches induce global immunosuppression. The goal of therapy, however, is to prevent recurrence of disease.

Central Nervous System Diseases↗

PubMed2003

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.

Adult↗

PubMed2004

[Vasculitis associated with viral infections].

VIRUSES, THE CAUSE OF VASCULITIS: Although the majority of systemic vasculitis are of unknown causes, the responsibility of a viral infection has been formally demonstrated in some of them and specific treatment can permanently cure them. Each virus incriminated accounts for a particular type of vasculitis. HEPATITIS B VIRAL INFECTION (HBV): Is the cause of polyarteritis nodosa in 36 to 50% of cases. The onset of the symptomatology is acute, usually within a few months following the infection; it is comparable to that observed in the absence of HBV infection. CRYOGLOBULINEMIA RELATED TO THE HEPATITIS C VIRUS (HCV): The clinical manifestations are those of systemic vasculitis with particular tropism for the skin (involvement generally inaugural and almost constant), peripheral nerves and the glomerula. They occur fairly late during the infection. VASCULITIS ASSOCIATED WITH HIV INFECTION: There is strong tropism for the peripheral (multi-neuritis) and central nervous system. During acute parvovirus B19 infection Vasculitis lesions have occasionally been reported following the viremic phase, generally limited to one or several flares of vascular purpura predominating on the lower limbs. FOLLOWING VARICELLA-HERPES ZOSTER INFECTION: Vasculitis occasionally develops in the form of a central neurological deficiency (locomotor deficiency with or without aphasia around one month after an ophthalmologic herpes zoster) or involving the retina or, more rarely, the skin or the kidneys. VASCULITIS ASSOCIATED WITH CYTOMEGALOVIRAL INFECTION: Predominantly observed in immunodepressed patients, vasculitis after CMV infection is diffuse and basically involving the digestive tube, notably the colon, the central nervous system and the skin. A RARE COMPLICATION OF AN HTLV1 INFECTION: Vasculitis of the retina often in the form of necrotic retinitis is often associated with spasmodic paraparessia. THERAPEUTIC STRATEGY: For many vasculitis of viral origin, corticosteroid and immunosuppressive treatments are only indicated in second intention following failure with antiviral agents and the combination of antivirals and plasma exchanges.

Adrenal Cortex Hormones↗

PubMed1999

Histological evidence of concomitant intramyocardial and epicardial vasculitis in necropsied heart allografts: a possible relationship with graft coronary arteriosclerosis.

BACKGROUND: The significance of medial lymphocytic vasculitis in intramural coronary vessels in heart transplantation is very poorly understood. This study was designed to identify histological evidence of an association between the presence of epicardial coronary lesions and the occurrence of intramyocardial vasculitis and/or myocardial ischemia. METHODS: We analyzed the frequency of medial vasculitis and other myocardial histological alterations in a retrospective study of 24 human cardiac allografts from patients who died of ischemic heart disease and/or myocardial rejection. RESULTS: Medial lymphocytic vasculitis in the myocardium was associated with vasculitis in the vasa vasorum of the epicardial coronary arteries and the presence of microfoci of acute myocardial infarction but was independent of the occurrence of myocardial fiber rejection. Chronic graft epicardial arteriopathy revealed two patterns of lesions. One pattern was similar to that of usual atherosclerosis, compromising mainly the proximal segments of the coronary artery, and was not associated with intramural vasculitis. The other pattern demonstrated diffuse involvement of the epicardial artery associated with vasculitis of its vasa vasorum and lymphocytic vasculitis of the intramural vessels. This second type of epicardial coronary lesion seemed to evolve to fibrotic arteries with thinned walls, frequently demonstrating aneurysmal dilatation with severe fibrosis of the adventitia and poor vasa vasorum. CONCLUSION: Medial vasculitis affecting intramyocardial vessels is associated with adventitial epicardial coronary vasculitis in the transplanted heart. The process of vasculitis may be involved in the development of chronic graft arteriosclerosis and is associated with ischemic myocardial lesions, but seems independent of myocardial fiber rejection.

Acute Disease↗

PubMed1994

Comparison of cell adhesion molecule expression in cutaneous leucocytoclastic and lymphocytic vasculitis.

AIMS: To compare the expression of the cell adhesion molecules intercellular adhesion molecule-1 (ICAM-1), ELAM-1 (E-selectin), and vascular cell adhesion molecule-1 (VCAM-1) in cutaneous leucocytoclastic and lymphocytic vasculitis. METHODS: Immunohistochemical analysis was performed on early lesional skin biopsy specimens of leucocytoclastic vasculitis (n = 14), lymphocytic vasculitis (n = 10), non-lesional skin (n = 12), and normal skin (n = 5). A standard immunoperoxidase technique was used to detect expression of ICAM-1, E-selectin, VCAM-1, and the cell markers CD11a, CD11b, CD11c, von Willebrand factor, CD3, CD68, and neutrophil elastase (NP57). RESULTS: Basal keratinocyte intercellular adhesion molecule-1 was expressed in eight (80%) cases of lymphocytic and in only one (7%) case of leucocytoclastic vasculitis, and not in non-lesional skin or control biopsy specimens from normal subjects. E-selectin was expressed on vascular endothelium in eight (57%) cases of leucocytoclastic and in seven (70%) cases of lymphocytic vasculitis. Endothelial vascular cell adhesion molecule-1 expression was seen in three (21%) biopsy specimens of leucocytoclastic and five (50%) of lymphocytic vasculitis. There were increased numbers of cells in the dermal infiltrate stained for NP57, CD11b, and CD11c in leucocytoclastic compared with lymphocytic vasculitis (p < 0.001, p = 0.013, p = 0.009, respectively); immunoreactive positive cells for CD3 and CD11a were increased in lymphocytic compared with leucocytoclastic vasculitis (p < 0.001, p = 0.011, respectively). CONCLUSIONS: These observations indicate that upregulation of adhesion molecule expression occurs in both leucocytoclastic and lymphocytic vasculitis. The different patterns of adhesion molecule expression in the two groups of vasculitis may reflect differences in the local release of cytokines. In particular, detection of intercellular adhesion molecule-1 expression by keratinocytes in lymphocytic vasculitis is consistent with an active role for mediators derived from T lymphocytes in the pathogenesis of the lesion.

Cell Adhesion Molecules↗

PubMed1989

[Classification and index of necrotizing vasculitis].

The diagnosis of necrotizing vasculitis rests on histology which shows parietal fibrinoid necrosis, vascular and perivascular infiltrates and intraluminal thrombosis in varying degrees of association. However, histopathological classifications of necrotizing vasculitis according to the size of the diseased vessels or the appearance of vascular infiltrates are not satisfactory, since microvasculitis may successively to simultaneously involve vessels of different calibers and result (generally in succession) in leukoclastic or lymphocytic vasculitis. For this reason we give preference to an aetiological classification which includes five major entities, some of which were described over a century ago (periarteritis nodosa, Churg-Strauss syndrome, Wegener's disease, rheumatoid purpura and Mac Duffie syndrome); drug-induced vasculitis, infective vasculitis, vasculitis associated with blood diseases; vasculitis of connective tissue diseases; vasculitis that may accompany some system diseases (e.g. Kawasaki disease and Cogan's syndrome); and vasculitis that may complicate cryoglobulinaemia or congenital deficiencies in complement fraction or in alpha-1 antitrypsin. It can be seen that this is not the ideal classification, since the same causative agent may be found in several chapters (e.g. HB virus in periarteritis nodosa, infective vasculitis and cryoglobulinaemia). We describe in detail only those types of vasculitis which are not treated in other in this issue of the Revue du Praticien, notably Wegener's disease, Mac Duffie syndrome and Kawasaki disease.

Humans↗

PubMed1994

Diagnosing intracranial vasculitis: the roles of MR and angiography.

PURPOSE: To describe our experience with MR and angiography in diagnosing intracranial vasculitis and to test the hypothesis that MR can be used to screen for patients unlikely to have vasculitis. METHODS: Ninety-two patients who had angiography with "exclude vasculitis" as the indication or who had angiography and a clinical diagnosis of vasculitis were identified. Angiograms of all 92 patients and the MRs of the 70 patients who had both studies were reviewed. RESULTS: Eleven patients had intracranial vasculitis. Angiography showed characteristic changes in 8. MR, performed in 9 of 11 vasculitis cases, was significantly abnormal in all 9. Among 70 patients who had both studies, 19 had MR that was completely normal or showed only incidental findings. None of these 19 was diagnosed with vasculitis. The diagnostic yield of angiography performed to exclude vasculitis was only 6%. CONCLUSIONS: Evaluation for intracranial vasculitis should include MR. A negative MR excludes intracranial vasculitis more definitively than does a negative angiogram and makes the likelihood of finding vasculitis with angiography negligible.

Adolescent↗

PubMed1996

[Vasculitis in rheumatoid arthritis].

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

Adolescent↗

PubMed1999

Systemic vasculitis in adults in northwestern Spain, 1988-1997. Clinical and epidemiologic aspects.

The vasculitides constitute a heterogeneous group of diseases characterized by blood vessel inflammation and necrosis with different but frequently overlapping clinical and pathologic manifestations. The incidence of these conditions is frequently controversial. To further investigate the incidence and clinical manifestations of vasculitides, we reviewed the spectrum of these diseases in an unselected population of adults (age > 20 years) from northwestern Spain during a 10-year period. From January 1988 through December 1997, 267 adults were diagnosed as having vasculitis. The overall average annual incidence rate of vasculitis in the region of Lugo, Spain, between 1988 and 1997 for the population older than 20 years was 141.54/million. Primary vasculitis (115.04/million for the population older than 20 years; 81.3%), especially giant cell arteritis (GCA) was the most common group. Small vessel primary vasculitis (hypersensitivity vasculitis and Henoch-Schönlein purpura) was the second most common group. Both GCA and small vessel primary vasculitis had a good outcome. However, although less common, patients with medium and small vessel primary vasculitis, in particular those with polyarteritis nodosa, had a high mortality related to the systemic manifestations of the disease or to the immunosuppressive therapy. Among the group of adults with secondary vasculitis (26.51/million; 18.7%), rheumatic diseases and specifically those occurring in the context of rheumatoid arthritis were the most common group. Patients with secondary vasculitis had clinical or laboratory data that may suggest the presence of an underlying disease. In summary, systemic vasculitides are somewhat more common than previously considered. As in other western countries, GCA constitutes the most common type of vasculitis in northwestern Spain. Better physician awareness may contribute to the progressive increase in the recognition of these conditions.

Adult↗

PubMed2002

Acute vasculitis after endovascular brachytherapy.

PURPOSE: Angioplasty effectively relieves coronary artery stenosis but is often followed by restenosis. Endovascular radiation (beta or gamma) at the time of angioplasty prevents restenosis in a large proportion of vessels in swine (short term) and humans (short and long term). Little information is available about the effects of this radiation exposure beyond the wall of the coronary arteries. METHODS AND MATERIALS: Samples were obtained from 76 minipigs in the course of several experiments designed to evaluate endovascular brachytherapy: 76 of 114 coronary arteries and 6 of 12 iliac arteries were exposed to endovascular radiation from 32P sources (35 Gy at 0.5 mm from the intima). Two-thirds of the vessels had angioplasty or stenting. The vessels were systematically examined either at 28 days or at 6 months after radiation. RESULTS: We found an unexpected lesion: acute necrotizing vasculitis in arterioles located < or =2.05 mm from the target artery. It was characterized by fibrinoid necrosis of the wall, often associated with lymphocytic exudates or thrombosis. Based on the review of perpendicular sections of tissue samples, the arterioles had received between 6 and 40 Gy. This arteriolar vasculitis occurred at 28 days in samples from 51% of irradiated coronary arteries and 100% of irradiated iliac arteries. By 6 months, the incidence of acute vasculitis decreased to 24% around the coronary arteries. However, at that time, healing vasculitis was evident, often with luminal narrowing, in 46% of samples. Vasculitis was not seen in any of 44 samples from unirradiated vessels (0%) and had no relation to angioplasty, stenting, or their sequelae. This radiation-associated vasculitis in the swine resembles the localized lymphocytic vasculitis that we have reported in tissues of humans exposed to external radiation. On the other hand, it is quite different from the various types of systemic vasculitis that occur in nonirradiated humans. CONCLUSION: Endoarterial brachytherapy using 32P results in vascular effects beyond the adventitia of the target vessel. This necrotizing vasculitis is causally related to radiation, but its mechanism is unclear and a dose effect is not evident. Quite possibly, local upregulation of inflammatory cytokines contributes to this radiation-associated vasculitis, which only involved some of the arterioles in each sample. It is likely that radiation-associated vasculitis also occurs around human coronary arteries and may result in foci of ischemia. To our knowledge, this lesion has not been previously recognized, either in experimental models or in human specimens examined after angioplasty/brachytherapy.

Acute Disease↗

PubMed2003

Temporal arteritis associated with systemic necrotizing vasculitis.

OBJECTIVE: To evaluate the clinical and laboratory characteristics of patients with systemic vasculitis associated with temporal artery involvement. METHODS: From a cohort of 120 patients fulfilling American College of Rheumatology criteria for temporal arteritis, we retrospectively identified 7 patients with systemic necrotizing vasculitis associated with histological temporal arteritis. RESULTS: Among the 7 patients, 2 had classic polyarteritis nodosa, one had unclassified systemic vasculitis, one had Wegener's granulomatosis (WG), and 3 had microscopic polyangiitis. The mean age of the patients was 70.2 years, and cranial symptoms revealed the disease in all but one patient. Temporal arteritis was generally associated with extracephalic manifestations suggestive of systemic vasculitis. Antineutrophilic cytoplasmic antibodies were positive in 3 of the 4 patients with small vessel vasculitis. Pathologically, the main temporal artery was involved in all but one patient, with inflammatory infiltrate of vasa vasorum and adventitia associated in 5 with small tributary involvement. Fibrinoid necrosis was rare, observed in 2 specimens; 2 patients with unclassified systemic vasculitis and WG had a classic giant cell arteritis (GCA) histologic pattern. Only one patient had exclusive involvement of small vessels, surrounding the spared main temporal artery. Muscle biopsies showed histopathological evidence of vasculitis in 2 patients, skin biopsy in one, and vein biopsy in the other. CONCLUSION: Temporal artery involvement in systemic necrotizing vasculitis was generally associated with extracranial clinical features suggestive of systemic vasculitis. Temporal artery biopsy is a simple tool for diagnosis of vasculitis, but the histopathological findings do not always discriminate between necrotizing vasculitis and classic GCA.

Aged↗

PubMed1998

Limitations of the 1990 American College of Rheumatology classification criteria in the diagnosis of vasculitis.

BACKGROUND: The American College of Rheumatology (ACR) established criteria to discriminate among patients with seven types of vasculitis. Although designated as "classification criteria" for research, these criteria are often used for diagnosis. OBJECTIVE: To examine the operating characteristics of the 1990 ACR classification criteria in the diagnosis of Wegener granulomatosis, giant-cell arteritis, polyarteritis nodosa, and hypersensitivity vasculitis. DESIGN: Prospective cohort study. SETTING: University medical center and Veterans Affairs medical center. PATIENTS: 198 consecutive patients referred to rheumatologists for evaluation of possible vasculitis. MEASUREMENTS: Blinded chart audits were done to classify patients according to the 1990 ACR classification criteria for Wegener granulomatosis, polyarteritis nodosa, giant-cell arteritis, and hypersensitivity vasculitis on the basis of the patients' initial presentation. Chart audits done 2 to 8 months after baseline provided the patients' final diagnoses, which were considered the gold standard, as in the development of the ACR criteria. Test operating characteristics of the ACR classification criteria were calculated according to 2 x 2 tables for the entire cohort and for only the patients with a final diagnosis of vasculitis. RESULTS: Vasculitis was diagnosed in 51 (26%) patients. Thirty-eight (75%) of 51 patients with vasculitis and 31 (21%) of 147 patients without vasculitis met ACR criteria for one or more types of vasculitis. The positive predictive values for the four vasculitides according to ACR criteria were 17% to 29% for the entire cohort and 29% to 75% for only the patients with a final diagnosis of vasculitis. CONCLUSION: The 1990 ACR classification criteria function poorly in the diagnosis of specific vasculitides.

Diagnosis, Differential↗

PubMed2005

Anti-myeloperoxidase IgG subclass distribution and avidity in sera from patients with propylthiouracil-induced antineutrophil cytoplasmic antibodies associated vasculitis.

OBJECTIVE: Propylthiouracil (PTU) could induce MPO-ANCA-positive vasculitis. The aim of this study was to compare the IgG subclass distribution and avidity of MPO-ANCA in sera from patients with primary ANCA-associated vasculitis (AASV) and PTU-induced vasculitis. METHODS: Nineteen patients with primary AASV with MPO-ANCA and thirteen patients with PTU-induced vasculitis were enrolled in the current study. Sera in both active phase and remission were collected. Anti-MPO IgG subclasses were detected by antigen specific ELISAs using specific monoclonal antibodies as second antibodies, and MPO-ANCA avidity was assessed by antigen-inhibition ELISAs. RESULTS: In primary AASV, all four anti-MPO IgG subclasses could be detected in active phase with IgG1 (100%), IgG2 (73.7%), IgG3 (63.2%) and IgG4 (94.7%), and in remission, IgG1 and IgG4 subclasses in most patients remained positive. However, in PTU-induced vasculitis, anti-MPO IgG3 subclass could not be detected, the anti-MPO IgG subclasses in active phase were IgG1 (100%), IgG2 (61.5%) and IgG4 (46.2%). Furthermore, five out of the six patients (88.8%) with PTU-induced vasculitis with positive IgG4 subclass in active phase turned to negative in remission, however, only eight out of the fourteen patients (57.1%) with primary AASV turned to negative. The median avidity constant of MPO-ANCA was 56 (8.96 to >140) x 10(7) mol/l for patients with primary AASV and 0.7 (<0.28 to >140) x 10(7) mol/l for patients with PTU-induced vasculitis respectively. Furthermore, the relative levels of MPO-ANCA avidity were associated with elevation of ESR in primary AASV and were associated with BVAS scores in patients with PTU-induced vasculitis, respectively. CONCLUSION: MPO-ANCA IgG subclass distribution and avidity were different between patients with primary AASV and PTU-induced vasculitis. It was suggested that the mechanism of ANCA production in PTU-induced vasculitis was different from that in primary AASV, and the avidity of MPO-ANCA might be associated with disease activity.

Adult↗

PubMed2006

Localized vasculitis of the gastrointestinal tract.

BACKGROUND AND OBJECTIVES: Isolated vasculitis of the gastrointestinal (GI) tract is a rare entity. Endoscopic biopsies have low sensitivity to diagnose intestinal vasculitis, even though the endoscopic findings may be suggestive of this condition. Our aims were to describe a case of biopsy-proven colonic leukocytoclastic vasculitis and review the literature. METHODS: A patient with biopsy-proven colonic leukocytoclastic vasculitis is described. A Medline database search of cases with localized GI vasculitis between January 1985 and September 2005 was conducted. RESULTS: A 32-year-old man was admitted to the hospital because of abdominal pain and diarrhea. A colonic biopsy showed leukocytoclastic vasculitis. There are very few articles on leukocytoclastic GI vasculitis as a separate disease, and most of them emphasize the difficulty in classification. Unlike our case, in former cases of localized vasculitis a diagnosis was made after surgery. Although our patient had steroid-refractory biopsy-proven isolated intestinal vasculitis, treatment with intravenous cyclophosphamide resulted in rapid resolution of symptoms and surgery was not required. CONCLUSIONS: In patients with abdominal pain a diagnosis of intestinal vasculitis should be considered. Immunosuppressive therapy allowed our patient to avoid surgery and may be similarly beneficial in other similar cases.

Adult↗