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At least 19 recordsLinked to original sources
PubMed1993

Serum soluble interleukin 2 receptor levels in anti-neutrophil cytoplasmic autoantibodies--positive systemic vasculitis.

Systemic vasculitis is characterized by the presence of autoantibodies to neutrophil cytoplasmic antigens (ANCA). The role of T-lymphocytes in systemic vasculitis remains uncertain. In the present study, we attempted to explore the role of T-lymphocytes in systemic vasculitis by measuring the serum soluble interleukin 2 receptor (sIL2R) levels in seven vasculitic patients and comparing the sequential measurements with the titres of ANCA which satisfactorily reflect the disease activity. The serum levels of both ANCA and sIL2R were elevated at clinical presentation. Contrary to ANCA, the serum sIL2R remained elevated in most patients despite clinical remission following immunosuppressive therapy. These findings suggest that T-lymphocytes may be activated in the acute phase of the disease. The finding of elevated serum sIL2R levels in most patients during clinical remission indicates that it is not a good measure of the disease activity and tends to argue against the role of T-lymphocytes as a major effector mediating inflammatory injuries in systemic vasculitis.

Aged↗
PubMed1999

An approach to diagnosis and initial management of systemic vasculitis.

Systemic vasculitis occurs in a heterogeneous group of primary disorders or can be a manifestation of infection, an adverse drug reaction, malignancy or a connective tissue disease. A vasculitic process should be suspected in patients with unexplained ischemia or multiple organ involvement, especially when such features as polymyalgia rheumatica, inflammatory arthritis, palpable purpura, glomerulonephritis or multiple mononeuropathy are also present. The clinical features of systemic vasculitis depend on the organs involved and, in turn, organ involvement is largely influenced by the size of the affected blood vessels. The diagnostic work-up should be tailored to the clinical situation and geared toward a tissue or angiographic diagnosis, bearing in mind that the findings from these studies are not always pathognomonic. Emphasis should also be placed on exclusion of a secondary process. The diagnosis of the specific type of vasculitis may be made on the basis of the clinical features and the histopathologic or angiographic findings. Initial therapy for most types of systemic vasculitis consists of high-dose corticosteroids, with the addition of immunosuppressive therapy in certain patients.

Churg-Strauss Syndrome↗
PubMed1983

Gastrointestinal manifestations of systemic vasculitis.

Systemic vasculitis is known to affect the gastrointestinal tract but the nature of the complication is poorly characterized. Out of 65 patients with systemic vasculitis, the majority of whom had renal disease, the intestine was found to be affected in 18. These comprised four of eight patients with polyarteritis nodosa, nine of seventeen with microscopic polyarteritis, four of thirty-six with Wegener's granulomatosis and one of four with Churg-Strauss syndrome. The features included abdominal pain (85 per cent), diarrhoea (50 per cent), gut haemorrhage (44 per cent) and abnormal liver function tests (50 per cent). Manifestations of gastrointestinal disease were evident at presentation in half the patients and led to a fetal outcome in five. Ileus, mucosal abnormalities, perforation and slow transit were evident radiographically, and selective visceral angiography showed aneurysms or organ infarcts in five patients. Histological assessment of gut biopsies (chiefly rectal) revealed non-specific inflammation or ulceration in nine patients and intramucosal haemorrhage in two. Focal areas of necrosis and ulceration in colonoscopic biopsies were highly suggestive of vasculitis whereas arteritis was only found in one full thickness biopsy. Hence the diagnosis of gastrointestinal complications depends largely on clinical evidence. In patients who survived, the gastrointestinal features remitted as the systemic illness improved following treatment with steroids, cyclophosphamide or plasma exchange.

Abdomen↗
PubMed2000

[Systemic vasculitis].

Systemic vasculitis is a general term designating a wide range of sometimes overlapping vascular conditions with marked tropism for peripheral constituting a complex nosological structure. Angiologists must be aware of the different telltale clinical signs including purpura, polyarthritis, glomerulonephritis, polyneuritis and unexplained inflammatory syndrome. Medium-sized and small-sized vessels are involved in a wide range of visceral damage. Several typical clinical presentations have led to the individualization of recognized syndromes such as rheumatoid type purpura, Wegener disease, Churg disease, Strauss disease, or peri-arteritis nodosa. Anti-nuclear serologies (pANCA and cANCA) contribute to diagnosis and have been helpful in individualizing a new entity: microscopic polyangeitis. In angiological practice, macroscopic expression of systemic vasculitis can lead to Takayasu type granulomatous angeitis in yong women and giant-cell arteritis in the elderly. These different arteriopathies should be treated by corticosteroid therapy with regular laboratory tests to follow inflammation and angiograhic exploration in case of occlusive arterial complications. Although these conditions are quite rare, a better understanding of their pathogenesis and their clinical course should help the angiologist make the initial diagnosis and the therapist to monitor treatment efficacy.

Aged↗
PubMed1976

Approach to systemic vasculitis.

Systemic vasculitis is a confusing subject which has been difficult to classify and understand. Improvements in therapy increase the importance of early diagnosis. This paper reviews the clinical and pathological differences of the systemic vasculitides, and a working approach to the differential diagnosis of vasculitis is formulated based on the size of the vessel involved. Vessel size and histopathology determine to what group of diseases a particular syndrome belongs, thereby allowing subclassification and a more rational approach to management.

Arteritis↗
PubMed2001

Primary systemic vasculitis.

Systemic vasculitides are multisystem diseases characterised pathologically by necrotising inflammation of blood vessels. The clinical presentation of vasculitis depends on the vessels involved. Classification of such diseases is now by the size of the affected vessel. This review focuses predominantly on the small vessel vasculitides shown to be associated with the presence of anti-neutrophil cytoplasmic antibodies (ANCA), that is Wegener's granulomatosis, microscopic polyangiitis and Churg-Strauss syndrome. It will examine the clinical characteristics of these diseases, the use of ANCA in diagnosis and monitoring of disease along with current and novel treatment strategies. The immunopathology of the ANCA-associated vasculitides will also be explored reviewing the roles of ANCA, neutrophils, T cells and apoptosis in the production of disease.

Adult↗
PubMedDate not supplied

[Anti-GM1 and anti-sulphatide antibodies in systemic idiopathic vasculitis, systemic lupus erythematosus and mixed cryoglobulinaemia: Serum detection and clinical and electrophysiologic correlations].

BACKGROUND: In the last two decades increasing interest has been focused on the association between autoimmune polyneuropathies and high titers of anti-nervous serum autoantibodies. High titer of IgG anti-GM1 antibody could be detected in Guillain Barre' syndrome and in chronic painful axonal sensory immune-mediated polyneuropathy. The possible occurrence of anti-nervous autoantibodies in autoimmune diseases not limited to the nervous system is still under study. METHODS: We evaluated 29 patients with systemic lupus erythematosus (SLE), 19 with biopsy-proven renal involvement, 28 patients with mixed IgM-K/IgG polyclonal cryoglobulinaemia (18 with glomerulonephritis) and 19 with small-sized vessel ANCA-associated vasculitis (12 with renal involvement) by using a sensitive immunoenzyme method of autoantibody detection. RESULTS: Compared to controls (1/176+/-1/205; 1:204+/-1:103), we found a significant increase in plasma IgM and IgG anti-GM1 titers (1:643+/-1:531; 1:444+/-1:309) in SLE patients (p<0.0001). We also found IgM (1:3032+/-1:2844) and IgG (1:1560) anti-sulphatide titers to be higher than the control group (p<0.0001). Mean plasma IgM and IgG anti-GM1 titers of the cryoglobulinaemic patients were 1:524+/-1:403 and 1:501+/-1:415, respectively, once again higher than the controls (p<0.0001). Mean plasma IgM and IgG anti-sulphatide titers in this group were 1:1864+/-1:1189 and 1:1350 (p<0.0001). We found idiopathic systemic vasculitis patients to have significantly increased levels of anti-sulphatides IgG class autoantibodies (1:1400; p<0.0001). We found no correlation with the serologic markers for vasculitis or the clinical or histologic extent of renal involvement. Electrophysiologic studies revealed that in 38% of SLE patients (p<0.005), 61% of cryoglobulinaemic patients (p<0.01) and 42% of ANCA-related vasculitic patients (p<0.01) the abnormal titers of antineuronal antibodies were associated with clinical or subclinical evidence of neuropathy. CONCLUSIONS: In patients with systemic idiopathic or secondary vasculitis anti-GM1 and anti-sulphatide antibodies can frequently be found. Their presence should prompt an accurate neurological examination because these serologic abnormalities are significantly associated with neurologic, often subclinical, involvement. Antineuronal reactivity might be the epiphenomenon of primary phylogistic damage, which exposes normally segregated neuronal epitopes or be directly involved in triggering neurological injury.

Antibodies↗
PubMed1999

Anti-endothelial cell antibodies in systemic vasculitis and systemic lupus erythematosus (SLE): effects of heat inactivation on binding and specificity.

Heating sera is used to inactivate complement but may affect the binding characteristics of autoantibodies. We studied the effect of heating sera from patients with systemic vasculitides and SLE on antibody binding to cultured human umbilical vein endothelial cells. Sera from 32 patients with systemic vasculitides, eight with SLE and 10 healthy controls were studied for anti-endothelial cell antibodies (AECA) using an ELISA before and after heating sera to 56 degrees C for 30 min. The median (range) AECA binding index in the patient group increased from 20% (0-153%) to 71.5% (10-259%) (P < 0.0001). The AECA binding index in the control group also increased from 14% (0-52%) to 90% (42-154%) (P < 0.0001). The increased binding was unaffected by the addition of fresh complement or removal of immune complexes and the increased binding after heating persisted even after cooling to 4 degrees C. Specificity experiments showed that after heating, the binding specificity of sera was lost. Removal of immunoglobulin with Protein A abolished the increased binding seen after heating. Heating sera increases AECA binding in both patient and control sera. The mechanism is probably non-specific damage to the immunoglobulin molecule, and heating sera should thus be avoided.

Antibody Specificity↗
PubMed1997

Treatment of antineutrophil cytoplasm autoantibody-associated systemic vasculitis: initiatives of the European Community Systemic Vasculitis Clinical Trials Study Group.

The diagnosis, treatment, and monitoring of the primary systemic vasculitides associated with circulating antineutrophil cytoplasm autoantibodies (ANCA) have formed the focus of a multicenter collaborative study. Consensus has been reached on criteria for classification, clinical subgroupings by extent and severity of disease, and "standard" and "best alternative" therapeutic regimens. Two series of randomized controlled trials have been designed; their aims are (1) to harmonize current approaches to treatment and (2) to test the value of newer therapeutic agents. In support of these trials, semiobjective scoring systems have been created and validated, and previous standardization of ANCA serologic and histologic analysis has been adopted. The systems of classification and clinical management described herein represent the recommendations of a multidisciplinary study group that hopes to improve the outcome of patients with primary systemic vasculitis by wide dissemination of the collective experience from interested centers.

Adrenal Cortex Hormones↗
PubMed2000

Sarcoidosis and systemic vasculitis.

BACKGROUND: Systemic vasculitis is an unusual complication of sarcoidosis. Over a 10-year period, the authors have provided care for six patients who had features of both sarcoidosis and vasculitis. Vasculitis could not be attributed to other causes. OBJECTIVES: To report six patients (five children) who had sarcoidosis and systemic vasculitis and compare our experience with previous literature. To better delineate the clinical spectrum of sarcoid vasculitis and its response to therapy. METHODS: Retrospective analysis and a Medline literature review of sarcoid and concurrent vasculitis from 1966. RESULTS: Our six patients had systemic illnesses that included fever, peripheral adenopathy, hilar adenopathy, rash, pulmonary parenchymal disease, musculoskeletal symptoms, and scleritis or iridocyclitis. Biopsies revealed features compatible with the diagnosis of sarcoidosis or necrotizing sarcoid granulomata in either skin, lymph node, lung, synovium, bone, bone marrow, liver, trachea, or sclera. Arteriography showed features of large vessel vasculitis in three patients, all of whom were African American, whereas patients with small vessel vasculitis were white. Prior reports of sarcoid and vasculitis included 14 adults, of whom half had predominantly small vessel disease, and half had medium- or large-sized vessel disease. Eight previously reported children included seven with primarily large vessel sarcoid vasculitis. Racial background was noted in 15 reported cases and included whites (6), African Americans (5), and Asians (4). Among the authors' six patients, four improved when treated with prednisone alone. However, relapses occurred when the drug was tapered or withdrawn. CONCLUSIONS: Sarcoidosis may be complicated by systemic vasculitis that can affect small- to large-caliber vessels. Sarcoid vasculitis can mimic hypersensitivity vasculitis, polyarteritis nodosa, microscopic polyangiitis, or Takayasu's arteritis. African American and Asian patients are disproportionately represented among cases with large vessel involvement. Corticosteroid and cytotoxic therapy is palliative for all forms of sarcoid vasculitis. However, relapses and morbidity from disease and treatment is common.

Adolescent↗
PubMed1992

A retrospective study of radiolabeled granulocyte kinetics in patients with systemic vasculitis.

Patients with systemic vasculitis, including Wegener's granulomatosis (WG) and microscopic polyarteritis (MP), may undergo white cell scanning for the investigation of infective complications and/or occult fever. In a retrospective study of 12 patients with systemic vasculitis (six each of WG and MP), all with renal disease, we observed increase diffuse lung radioactivity soon after the injection of 111In-labeled granulocytes or 99mTc-HMPAO-labeled leukocytes in all patients with WG and in three with MP. Lung activity was quantified by comparison with the liver or spleen. The lung:liver count rate ratio per pixel, 1-1.5 hr after injection, in patients with systemic vasculitis was 0.87 (s.d. 0.25), significantly higher (p less than 0.001) than the ratio 0.38 (0.13) in patient controls who had normal white cell scans. The majority of patients with systemic vasculitis had scintigraphic evidence of abnormal splenic function. Two had focal splenic defects, while 7 had increased labeled cell uptake. Nine of the patients with vasculitis showed cell migration into the gut, presumably as a result of vasculitis, and in 6 it was prominent. Focal nasal uptake was found in 5/7 patients with systemic vasculitis who had their heads imaged, and may be specific for WG. Although all patients had renal disease, there was scintigraphic evidence of diffuse parenchymal renal uptake of 111In-labeled granulocytes in only one (with MP). The presence of anti-neutrophil cytoplasmic antibodies did not correlate with any abnormality or with lung uptake. Systemic vasculitis is associated with abnormalities of granulocyte kinetics, particularly involving the lung and spleen.

Granulocytes↗
PubMed2001

Tissue targeting and disease patterns in systemic vasculitis.

The systemic vasculitides include a group of diseases with highly heterogeneous organ distribution and disease expression patterns. The mechanisms mediating tissue targeting in systemic vasculitis are largely unknown. Mechanical forces may contribute to the distribution of lesions in immune complex-mediated vasculitis. The site at which the antigen is encountered may be crucial in determining the location of inflammatory infiltrates in some vasculitides. Co-existence of different immunopathogenic mechanisms with variable dominance may generate diversity in disease presentation patterns. Heterogeneous and incompletely understood triggering mechanisms attract inflammatory cells to the site of interest through sophisticated molecular mechanisms: interplay between leukocyte receptors and endothelial ligands, and interactions between chemokines and chemokine receptors. Even with a similar distribution of lesions, patients with vasculitis may display highly variable clinical manifestations. Variations in genes involved in immune response might determine the severity of disease, the intensity of the systemic inflammatory response, the degree of vessel occlusion and the response to therapy.

Adult↗
PubMed1994

Circulating myeloperoxidase may cause false negative findings in the analysis of myeloperoxidase antibodies in systemic vasculitis.

In systemic vasculitis reliable detection of myeloperoxidase antibodies (MPO-Abs) is of great clinical importance in the diagnosis and follow-up of patients. We have studied whether circulating myeloperoxidase (MPO) could have an effect on MPO-Ab findings. Serum MPO and MPO-Abs were measured in 50 healthy individuals, 35 patients and in the follow-up samples from two patients with Wegener's granulomatosis. Heating the sera at 56 degrees C for 30 min reduced the concentration of immunoreactive MPO both in control and patient sera. In 71% of the patient sera heating made initially negative MPO-Abs detectable. In a few cases with severe vasculitis the antibody findings remained totally negative. These results, together with the data from the follow-up samples from two patients with Wegener's granulomatosis, revealed that the serological diagnosis of vasculitis may be considerably delayed if only native samples are analysed for MPO-Abs. These findings are of considerable clinical significance for the interpretation of MPO-Ab results. Circulating myeloperoxidase affects MPO-Ab measurements, causing false negative findings in MPO-Ab assays. Therefore, it is recommended to denaturate circulating MPO by heating the sera before the analysis of MPO-Abs and to re-evaluate the cut off-values.

Adult↗
PubMed2001

Management of systemic vasculitis.

The systemic vasculitides are a wide-ranging group of diseases that are characterized by the presence of blood vessel inflammation. Despite this common feature, each type of vasculitis has a unique variety of clinical manifestations that influences its degree of disease severity and ultimately its management. Immunosuppressive therapy forms the foundation of treatment for almost all forms of systemic vasculitis. Because of this, treatment can be associated with its own risk of morbidity, or even mortality, related to specific medication side-effects or infections which occur as a result of impaired host defences. This chapter seeks to review the approach to management in selected forms of systemic vasculitis. Questions examined include the following. When should one treat systemic vasculitis? How does the nature of the disease and its severity affect treatment decisions? What are the data regarding the effectiveness of individual therapeutic regimens?

Azathioprine↗
PubMed1996

Classification, pathogenesis, and treatment of systemic vasculitis.

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

Animals↗
PubMed2003

Echocardiographic and clinical characteristics of aortic regurgitation because of systemic vasculitis.

BACKGROUND: Although systemic vasculitis has been described as one cause of aortic regurgitation (AR), clinical features of this disease entity have not been fully characterized. METHODS: The clinical data of 153 consecutive patients (103 male), who underwent open-heart operation to correct predominant AR from January 1990 to December 2000 were retrospectively analyzed. RESULTS: A total of 16 patients (10%) were found to have AR associated with systemic vasculitis; there were 9 patients with Takayasu's arteritis (TA) and 7 with Behçet's disease (BD). All patients with TA, but only 3 with BD, were female (P <.05). Echocardiography showed redundant motion of the elongated aortic cusp with the frequent periaortic echo-free space in BD, and characteristic motion limitation of thickened cusp with dilated and thickened aorta in TA. All patients with TA, but only 1 patient with BD, were given a diagnosis before operation (P <.05); the others underwent emergency operation to control acute AR of unknown etiology, and were given a correct diagnosis after detection of paravalvular leakage. In TA, paravalvular leakage developed in 1 patient (11%) and another died suddenly with chest pain. In BD, paravalvular leakage developed in 6 patients (86%) who needed repeated operation, and 2 died even after the third operation. The 2-year survival without clinical events (paravalvular leakage, repeated operation, or death) was 76 +/- 15% in TA and 14 +/- 13% in BD (P <.05). CONCLUSIONS: TA and BD are 2 important causes of AR, and show different clinical features. A high rate of paravalvular leakage, especially in BD, warrants cautious systemic evaluations for the cause of AR, and characteristic echocardiographic findings seem to be helpful in the differential diagnosis.

Adult↗
PubMed2003

Central nervous system vasculitis.

Vasculitis is an inflammation of the vessel wall. It may be either primary or secondary. Primary vasculitis includes systemic vasculitis (large, medium, and small-vessel vasculitis) and localized vasculitis (isolated angiitis of the central nervous system and non-systemic vasculitic neuropathy). Secondary vasculitis may be present in connective tissue disorders or may be caused by infections, neoplasms, and substance abuse. Patients presenting with symptoms suggestive of vasculitis require brain neuroimaging, lumbar puncture, and angiography, but only biopsy allows a definite diagnosis.

Cerebral Angiography↗