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PubMed1995

Epidemiology of systemic vasculitis: changing incidence or definition?

The epidemiology of the systemic vasculitides is poorly documented. Many studies have been conducted from tertiary referral centers, with resulting problems of referral bias and uncertainty of denominator population, or have involved small populations. We have estimated the incidence of the major forms of systemic vasculitis in a stable, ethnically homogeneous population of 414,000 adults from 1988 to 1994. The overall annual incidence of systemic vasculitis (excluding giant cell arteritis) is 39/million (95% confidence intervals; ranging from 31 to 47). The annual incidence of Wegener's granulomatosis is 8.5/million (range, 5.2 to 12.9), Churg-Strauss syndrome 2.4/million (0.9 to 5.3), microscopic polyangiitis 2.4/million (0.9 to 5.3), adult Henoch-Schonlein purpura 1.2/million (0.3 to 3.5), and systemic rheumatoid vasculitis 12.5/million (8.5 to 17.7). These data suggest that the overall incidence of systemic vasculitis is greater than previously thought (10/million) with Wegener's granulomatosis and systemic rheumatoid vasculitis being the most common. Whether this represents a genuine increase in incidence or increased physician awareness is uncertain.

Churg-Strauss Syndrome↗
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↗
PubMedDate not supplied

Diagnosis and assessment of systemic vasculitis.

The diagnosis of systemic vasculitis requires clinical evidence with appropriate symptoms and physical signs, supported by histological or radiological confirmation. Earlier recognition of these diseases has been facilitated by a greater awareness of their incidence, and also by the more widespread introduction of the anti-neutrophil cytoplasmic antibody (ANCA) test. Early diagnosis provides a greater potential for effective intervention in the course of disease and this may limit subsequent damage. However, an early diagnosis poses the more difficult challenge in the classification of the vasculitides, since traditional classification systems have depended on the presence of well-established manifestations of the disease. The accurate assessment of disease activity and damage in vasculitis has become necessary as a result of significant improvements in survival with the use of chemotherapy. The disease course however is frequently characterised by relapse as well as the scars of irreversible organ damage from disease and drug toxicity. Clinical methods of assessment are simple to apply, reliable and often more effective than any current laboratory test in evaluating the effects of therapy and determining changes in therapy. The increasing use of surrogate clinical measures of disease should provide a greater opportunity to establish the effectiveness of existing and novel therapies in the management of these complex diseases.

Antibodies, Antineutrophil Cytoplasmic↗
PubMed1994

New developments in the treatment of systemic vasculitis.

Although precise diagnosis of the systemic vasculitides can provide general prognostic information and help to guide initial therapy, recent studies on the long-term clinical course have revealed considerable variation in clinical severity. Therefore, anatomic distribution of involvement and speed of progression should be the principle determinants of the intensity of immunosuppressive therapy. In progressive pulmonary or renal disease, eg, Wegener's granulomatosis, aggressive "standard" therapy is obligatory, eg, daily cyclophosphamide and glucocorticoids. Such regimens, however, should be applied with caution in chronic or indolent and abortive forms of systemic vasculitis, because follow-up studies (eg, in Wegener's granulomatosis) have revealed treatment-associated morbidity rates of up to 42%, disease-related morbidity, and a high incidence of relapse under treatment. Moreover, less toxic therapeutic strategies are being pursued with remarkable success: low-dose weekly methotrexate, monthly intravenous or oral pulses of cyclophosphamide plus glucocorticoids, and high-dose intravenous immunoglobulin. Long-term remission of intractable (non-antineutrophil cytoplasmic antibody-associated) systemic vasculitis has been achieved using humanized monoclonal antibodies (ie, anti-CD4/anti-CDw52); and amelioration of glomerulonephritis in immune complex diseases (eg, systemic lupus erythematosus) has been achieved with nafamostat mesilate, an inhibitor of complement serine proteases. In addition, leukocytoclastic vasculitis has been effectively controlled with pentoxifylline, presumably by neutralizing proinflammatory cytokines, and hepatitis C virus-associated mixed cryoglobulinemia has been successfully treated with interferon alfa.

Anti-Infective Agents↗
PubMed1996

Microangiopathic haemolytic anaemia and systemic vasculitis.

Two cases of systemic vasculitis complicated by microangiopathic haemolytic anaemia (MAHA) are described: this association has not previously been reported. Both patients had atypical presentations of their primary disease, one with parotitis and one with a Guillain-Barré syndrome. Other causes of MAHA were excluded and a possible link with macromolecular von Willebrands factor is speculated upon.

Aged↗
PubMed1997

A comparison of two nomenclature systems for primary systemic vasculitis.

Recently, two new systems have been proposed for the nomenclature of primary vasculitides: the 1990 American College of Rheumatology (ACR) classification criteria and the 1992 Chapel Hill Consensus Conference (CHCC) definitions. We compared these two systems in the same cohort of patients with primary systemic vasculitis. Twenty-four patients were studied and, applying the 1990 ACR criteria, the diagnoses were Wegener's granulomatosis (WG) (15), Churg Strauss syndrome (CSS) (4), polyarteritis nodosa (PAN) (2) and unclassified (3). Using the CHCC definitions, the diagnoses were WG (5), microscopic polyangiitis (MPA) (8), possible MPA (1), PAN (1) and undefined (9). There was concordance in only five patients (all WG). Significant discordance exists between these two criteria sets. Since the ACR criteria set does not include MPA, WG tends to be overdiagnosed. The Chapel Hill definitions are biopsy dependent and surrogate features for the defining histology are required to allow their practical application.

Adult↗
PubMed1986

Combined cyclophosphamide and corticosteroid-induced remission in severe glomerulopathy associated with systemic vasculitis.

Three patients with systemic vasculitis and severe renal disease as major manifestations are reported. In 2 cases, rapidly progressive glomerulonephritis presented as oliguric renal failure. In the third case, the clinical picture was severe nephrotic syndrome with decreased renal function. Combined cyclophosphamide and corticosteroid treatment resulted in dramatic improvement of renal function and remission of nephrotic syndrome. In 2 cases, histological improvement was documented by repeated kidney biopsy. The optimal duration of cyclophosphamide therapy has to be determined.

Adult↗
PubMed1997

Radiographic features of central nervous system vasculitis.

Central nervous system (CNS) vasculitis refers to primary and secondary disorders of the CNS vasculature. Most authorities agree that CNS vasculitis is a potentially serious disorder; therefore, prompt diagnosis and initiation of therapy are high priorities in treatment. Remarkable progress has been made in the diagnosis, evaluation, and treatment of this disorder. This article examines many aspects of the radiographic evaluation of CNS vasculitis.

Adult↗
PubMed1991

Wegener's granulomatosis, systemic vasculitis, and antineutrophil cytoplasmic autoantibodies.

Systemic vasculitis is often difficult to diagnose. The recent discovery of a new class of autoantibodies [antineutrophil cytoplasmic autoantibodies (ANCA)] provides clinicians with a serological test that strongly supports the diagnosis of the most common forms of systemic necrotizing vasculitis. The spectrum of patients with this form of vascular inflammation ranges from those with renal-limited glomerulonephritis to patients with multisystem disease, including Wegener's granulomatosis and polyarteritis nodosa.

Autoantibodies↗
PubMed2002

Antineutrophilic cytoplasmic antibody-positive systemic vasculitis associated with propylthiouracil therapy: report of 2 children with Graves' disease.

Systemic vasculitis is a rare complication of therapy with antithyroid medication. Antineutrophilic cytoplasmic antibody (ANCA)-associated vasculitis has been described in patients treated with propylthiouracil (PTU) and methimazole (MMI). The majority of cases have underlying Graves' disease. The authors report 2 children who developed ANCA-associated systemic vasculitis during PTU therapy of Graves' disease. One patient, after PTU treatment for 3 years, developed severe systemic vasculitis. After 3 weeks of arthritis, she abruptly presented with hematuria, proteinuria and edema concomitant with anemia. Her serum creatinine was elevated, to 6 mg/dl. Renal biopsy revealed crescentic glomerulonephritis. After admission, she developed intracerebral hemorrhage and pulmonary hemorrhage. She had positive perinuclear-ANCA (p-ANCA) with a titer of 1:160. Despite intensive therapy with immunosuppressive agents and plasmapheresis, as well as discontinuation of PTU, she died of the complications of severe systemic vasculitis. The other patient developed fever, arthralgia and leukocytoclastic vasculitis of the skin during treatment with PTU for about 2 years. Her symptoms and skin lesions disappeared after discontinuation of PTU. However, she has had a persistently high titer of p-ANCA 1:320 through 17 months follow-up time. Thus, patients who are treated with PTU can develop ANCA-positive vasculitis in a mild or severe form. Therefore, they should be carefully followed and monitored, not only for their thyroid status but also the serious complications of PTU.

Antibodies, Antineutrophil Cytoplasmic↗
PubMed2004

[Anti-endothelial cell antibodies in systemic vasculitis: detection and correlation with disease activity].

OBJECTIVE: To investigate the prevalence of anti-endothelial cell antibodies (AECA) in systemic vasculitis and to assess the correlation between AECA and disease activity, and try to discuss the classification of AECA. METHODS: Cyto-ELISA with EA.hy926 and HMEC-1, two immortalized cell lines, as substrates, was applied to detect AECA in 122 cases of systemic vasculitis, including 43 cases of Behcet disease, 19 cases of Takayasu arteritis, 19 cases of Wegener's granulomatosis, 11 cases of microscopic polyangiitis, 9 cases of polyarteritis nodosa, 3 cases of Churg-Strause syndrome, 2 cases of giant cell arteritis and other 16 cases of which could not be classified clearly. Patients with SLE, RA, and fever of unknown origin, and normal persons were used as control groups. Then the associations of AECA to laboratory findings and clinical disease activity (scored by BVAS) were analyzed. Furthermore, the AECA value from the two different substrate cells were analyzed for correlation and difference. RESULTS: With either EA.hy926 or HMEC-1 as substrates, the AECA prevalence rates of systemic vasculitis (respectively 33.61% and 37.70%) were significantly higher than those of the normal and RA groups (less than 10%), and the prevalence of AECA in SLE (61.75%) was higher than that of systemic vasculitis (37.70%) when using HMEC-1 as substrates. AECA was found to correlate very well with ESR in 122 cases of systemic vasculitis and with BVAS in 40 cases of small systemic vasuculitides, including Wegener's disease, microscopic polyangiitis, Churg-Strause syndrome and so on. The pair AECA values with EA.hy926 and HMEC-1 as substrate cells were found to be correlated significantly, and the matching rate was 92.62%. CONCLUSION: Prevalence of AECA in systemic vasculitis is high. AECA indicates the clinical disease activity. As to the assumption of classification of AECA into antibodies against microvascular and macrovascular endothelial cells, further study need to be done to prove or disprove it.

Adult↗
PubMed1997

Damage occurs early in systemic vasculitis and is an index of outcome.

Because death after acute systemic vasculitis is now uncommon, alternative measures of outcome are required. A significant component of patient morbidity is disease-related damage, which can be quantified by the Vasculitis Damage Index (64 items in 11 organ-based systems). We investigated serially the time-course of damage in 120 patients with systemic vasculitis, to determine the earliest indicators of outcome. High damage scores at 2 years after presentation were characteristic of fatal disease (OR 8.1-12.4). Significant damage occurred within 6 months of presentation, and was a feature of fatal disease. More damage occurred after presentation than after relapse. Lung and multi-system damage were early indicators of poor outcome in severe non-fatal disease. Damage occurs early in systemic vasculitis, and is an indicator of poor outcome. This novel observation, together with evidence of persistent subclinical disease activity and the high frequency of relapse, suggests a need for new treatment strategies. Analogy with the management of acute leukaemia suggests a strategy of early diagnosis and intensive induction of remission, with early escalation of treatment for resistant disease.

Acute Disease↗
PubMed1997

Enhanced endothelium-dependent vasodilator responses in patients with systemic vasculitis.

The aim of the study was to investigate endothelium-dependent vasodilator responses in the forearm vasculature of patients with systemic vasculitis. We studied 10 patients with systemic vasculitis and 16 healthy control subjects using forearm venous occlusion plethysmography to assess changes in forearm blood flow in response to acetylcholine (ACh). NG-monomethyl-L-arginine (L-NMMA), was also used to assess the contribution of endothelium derived relaxing factor/nitric oxide (EDRF/NO) to the vasodilator responses to acetylcholine. A significantly greater vasodilator response to ACh was seen in the patient group at all doses infused (p < 0.01). After pre-infusion of L-NMMA, the differences in ACh dilator responses were no longer significant. There was a greater magnitude of inhibition of ACh responses by L-NMMA in the patient group. The enhanced vasodilator response to ACh observed was in part abolished by L-NMMA, suggesting that EDRF/NO is produced in excess in systemic vasculitis. The precise role played by EDRF/NO in the pathogenesis of systemic vasculitis requires further study.

Acetylcholine↗
PubMed2000

Is it possible to offer evidence-based treatment for systemic vasculitis?

The evidence base for treatment of systemic vasculitis is reviewed in this article. Accurate diagnosis and evaluation of disease status is crucial to the appropriate management of these cases and disease status should be employed as a means of staging so that appropriate therapy can be offered tailored to the individual requirements of each case. Large vessel vasculitides are almost exclusively managed with corticosteroids. Small vessel vasculitis with internal organ involvement usually requires more aggressive immuno-suppression; by contrast, small vessel vasculitis without internal organ involvement rarely requires intervention. Despite our current efforts, the long-term morbidity of patients with systemic vasculitis remains high. We need better therapeutic strategies in order to limit or control disease rapidly and thereby prevent the accumulation of subsequent damage. Large-scale randomised trials comparing different therapeutic strategies offer the only hope of establishing a firmer evidence base for the optimal management of these diseases.

Diagnosis, Differential↗
PubMedDate not supplied

Spectrum of autoimmune responses in systemic vasculitis.

Growing evidence supports the inclusion of primary forms of systemic vasculitis within the category of autoimmune diseases. Thus their association with a family of autoantibodies, related to their specificity or neutrophil cytoplasm antigens, has been identified and the role these autoantibodies play in pathogenesis is now being explored. Different members of the autoantibody family have now been characterised in terms of their molecular specificity and the isotype of immunoglobulin of which they are composed. These properties may be a useful adjunct in the classification of systemic vasculitis (see table I). It is the purpose of this review to put a perspective on the specificity and pathogenicity of autoantibodies found in patients with systemic vasculitis and to outline new strategies for the treatment of these conditions, based on the involvement of autoimmune mechanisms in the development of these disorders.

Antibodies, Antineutrophil Cytoplasmic↗
PubMed1991

Anti-idiotypes against anti-neutrophil cytoplasmic antigen autoantibodies in normal human polyspecific IgG for therapeutic use and in the remission sera of patients with systemic vasculitis.

Anti-neutrophil cytoplasmic antigen (ANCA) activity was inhibited in 15 out of 21 sera from patients with acute systemic vasculitis following incubation with normal polyspecific IgG for therapeutic use (IVIg). ANCA antibodies reacted with IVIg through idiotypic-anti-idiotypic interactions, as shown in competitive binding assays using F(ab')2 fragments from IVIg and affinity chromatography of ANCA IgG on Sepharose-bound F(ab')2 fragments from IVIg. Co-incubation of sera from patients with acute systemic vasculitis with paired autologous remission stage sera also resulted in inhibition of ANCA activity in acute sera. Remission sera contain IgM and IgG capable of interacting with beta and or alpha idiotypes of ANCA IgG from acute sera. Anti-idiotypic IgM may account for the lack of expression of ANCA activity in whole serum from patients in remission from systemic vasculitis, which were found to contain high titres of ANCA IgG. These observations suggest that remission of systemic vasculitis is associated with the generation of anti-idiotypes against autoantibodies rather than the suppression of production of ANCA autoantibodies. IVIg may modulate the activity of systemic vasculitis in vivo.

Antibodies, Anti-Idiotypic↗
PubMed2006

Primary systemic vasculitis: treatment of difficult cases.

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

Antibodies, Antineutrophil Cytoplasmic↗
PubMed1989

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

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

Biopsy↗