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PubMed1994

[Diagnosis and therapy in systemic vasculitis with renal involvement].

The diagnosis of Wegener's granulomatosis, microscopic polyarteritis and idiopathic rapidly progressive glomerulonephritis as the main cause of renal involvement in systemic necrotizing vasculitis was revolutionized following the introduction of the ANCA test. The different clinical manifestations of these diseases are described. By means of the ANCA test it could be demonstrated that the pulmonary-renal syndrome was mainly caused by these vasculitides and not by Goodpasture's syndrome. Cyclophosphamide and steroids remain the drugs of choice in the treatment. They can be administered in different ways depending upon the clinical manifestation of the diseases. As therapeutic alternatives cyclosporin, trimethoprim/sulfamethoxazole, intravenous immunglobulins and monoclonal antibodies are discussed.

Anti-Glomerular Basement Membrane Disease↗
PubMed1993

Animal models of systemic vasculitis.

Necrotizing leucocytoclastic vasculitis is the histopathological hallmark of the small vessel systemic vasculitides (SV), a group of human diseases commonly associated with anti-neutrophil cytoplasm autoantibodies (ANCA). Necrotizing vasculitis is seen in a number of experimental systems, but none of these provide an ideal animal model for human SV. Vasculitis occurs in serum sickness reactions; in murine models of systemic lupus erythematosus; in association with infection, particularly chronic viral infections; and after treatment with certain drugs or inflammatory mediators. 'Spontaneous' vasculitis has been reported in specific mouse strains, especially with ageing, and in some larger species. The size of vessel involved and the type of inflammatory cells predominating are variable in these experimental situations, and none of these models feature antibodies analogous to ANCA. We have recently reported that Brown Norway rats treated with mercuric chloride (HgCl2) develop necrotizing leucocytoclastic vasculitis, especially in the gut, and also develop antibodies to myeloperoxidase (MPO) which recognize similar determinants on MPO to those bound by a subset of ANCA. Transfer of serum from HgCl2-treated rats to naive animals does not induce tissue injury. Preliminary experiments using pooled immunoglobulin or an anti-CD4 monoclonal antibody did not show useful therapeutic benefit from these treatments. HgCl2-induced vasculitis has weaknesses as an animal model of human SV, but is the only experimental model in which anti-MPO autoantibodies have so far been demonstrated, and therefore may be of particular relevance to ANCA-associated SV.

Animals↗
PubMed2003

Therapy of neurological disorders in systemic vasculitis.

Significant progress has been made in our understanding of the underlying cellular and molecular mechanisms associated with vasculitis. Simultaneous involvement of the nervous system and systemic organs points to shared antigenic targets or immune mechanisms operative at both sites. Although specific immunotherapy is not available, the old and new immunomodulatory therapies have been instrumental in providing effective therapy in most of these chronic autoimmune disorders. The treatment of giant cell arteritis, neurosarcoidosis, Wegener's granulomatosis, systemic lupus erythematosus, polyarteritis, Sjögren's syndrome, and Behcet disease will be reviewed.

Adjuvants, Immunologic↗
PubMedDate not supplied

[Systemic necrotizing vasculitis].

Systemic necrotic angiites as well as Vegenera granulomatosis were shown according to literature data and the experience of the authors to be polysymptomatic. The fact makes diagnostics difficult. Multi-biopsies is recommended to conduct a histological verification of the disease.

Adult↗
PubMed2002

Central nervous system vasculitis in the intensive care unit.

Intensivists are sometimes faced with unexplained neurologic defects in ICU patients. A subacute presentation over weeks or months characterized by headache and mental status change with focal deficits in the absence of evidence of secondary vasculitis or other diseases mentioned in the differential diagnosis should arouse suspicion of PACNS. Delay in diagnosis of this rare condition may lead to additional morbidity and prolong ICU stay. There is also a risk of permanent cognitive dysfunction with untreated PACNS. A reactive CSF picture, ischemic changes on MR imaging, and alterations in vessel caliber on cerebral angiography are not diagnostic but strengthen the evidence for PACNS. A brain biopsy may be required to confirm the diagnosis. High-dose steroid therapy with a prolonged course and gradual taper controls the disease in most cases. Additional immunosuppressive therapy is needed in some patients.

Communicable Diseases↗
PubMed2006

Stroke associated with central nervous system vasculitis after West Nile virus infection.

We report the association of West Nile virus infection, isolated vasculitis, and stroke in a 9-year-old girl. West Nile virus is of growing epidemiologic importance and should be considered in the differential diagnosis of stroke etiologies, especially during late summer and in patients with a history of exposure in areas where West Nile virus transmission is present.

Child↗
PubMed2000

A case of central nervous system vasculitis related to an episode of Guillain-Barrè syndrome.

The authors report their knowledge about an uncommon case of isolated vasculitis, restricted to the left sylvian artery during an auto-immune Guillain-Barrè syndrome (GBS), sustained by cytomegalovirus (CMV). An acute cardiopulmonary failure requiring a ventilator and vasopressor support manifested, notwithstanding plasma exchanging and immune-modulating therapy. An IgM-enriched formula administration coincided with a rapid amelioration of GBS and vasculitis to a complete recovery the next month after her discharge to a rehabilitation centre.

Adjuvants, Immunologic↗
PubMed2004

Branch retinal artery occlusions as the presenting feature of primary central nervous system vasculitis.

A 39-year-old woman presented with multiple branch retinal artery occlusions almost three years before developing a mass lesion containing calcium in the left frontal lobe. Brain biopsy revealed a small vessel vasculitis and ischemic necrosis of brain with dystrophic calcification. We believe this to be the first case of primary CNS vasculitis with branch retinal artery occlusions and brain calcification.

Adult↗
PubMed1998

Optimisation of cyclophosphamide therapy in systemic vasculitis.

There is no doubt that the prognosis for systemic vasculitides has been considerably improved by the use of immunosuppressive agents, chiefly cyclophosphamide. Increasingly, we are becoming aware of the enormous burden of chronic 'grumbling' disease, the high incidence of relapse and the longer term effects of toxic therapy in these patients. The general approach is more intense therapy (with intermittent high dose 'pulses' or lower dose continuous cyclophosphamide) in the initial phase of therapy to induce remission, followed by a less toxic therapy in the maintenance phase (either longer intervals between pulses or a switch to a less toxic drug, such as azathioprine). The pathogenetic mechanisms in vasculitis, which are becoming more precisely defined, are diverse, but cyclophosphamide remains the drug of choice. A number of different cyclophosphamide regimens are in use, which reflects the current dilemma of trying to balance effectiveness with toxicity in diseases where the quality of long term survival remains unsatisfactory. Evidence from controlled trials does not support major differences in immediate outcome between different regimens of cyclophosphamide. Future studies need to address the use of agents designed to interfere precisely with the underlying pathogenetic mechanisms. Alternative approaches should also be considered, for example the use of sublethal doses of cyclophosphamide, with autologous bone marrow rescue, which may achieve long lasting remission or even cure.

Controlled Clinical Trials as Topic↗
PubMed1996

Primary retinal vasculitis. Systemic associations and diagnostic evaluation.

BACKGROUND: Inflammation of the retinal vasculature without an infectious etiology, systemic disease association, or concomitant ocular disease is termed primary retinal vasculitis, and can result in severe and permanent vision loss. Patients with primary retinal vasculitis usually are subjected to an extensive but unrewarding diagnostic evaluation. This study was undertaken to determine the value of such a diagnostic workup, and to determine whether systemic diseases develop in these patients during the course of their illness. METHODS: The clinical records of 25 patients seen between 1984 and 1994 with the referring diagnosis of primary retinal vasculitis were reviewed retrospectively. Recorded data included patient age, sex, race, medical history, medications, visual acuity, extent of retinal disease, and the results of their diagnostic evaluations. RESULTS: On presentation, none of the patients had an underlying systemic disease attributable as the cause of their retinal vasculitis. Review of systems was suggestive of an underlying systemic disease in 1 of 25 patients. Diagnostic evaluation in this patient showed a positive antinuclear antibody value and a double-stranded DNA, suggestive of systemic lupus erythematosus. Subsequently, systemic lupus erythematosus was diagnosed based on the development of other diagnostic criteria. The review of systems was not suggestive of an underlying systemic disease in 24 of 25 patients. False-positive diagnostic test results were obtained in 5 (20.8%) of these 24 patients. No underlying systemic disease associated with the patients' retinal vasculitis subsequently developed in any of these five patients (mean follow-up, 4 years). CONCLUSION: Few diagnostic tests should be ordered in patients with retinal vasculitis in the absence of a medical history suggestive of underlying systemic disease.

Adolescent↗
PubMed2003

[Treatment of primary systemic vasculitis with TNF alpha-antagonists].

After the introduction of the TNF alpha blocking drugs Etanercept and Infliximab for the standard therapy of rheumatoid arthritis these effective substances have also been used successfully in many patients with primary systemic vasculitides, who were unresponsive to standard therapy. From pathophysiologic findings their use is justified by the prominent role of TNF alpha in the inflammation of small and large vessels. So far only open studies with a maximum of 20 patients and case reports are published. In summary there are reports of the successful treatment of 7 patients with rheumatoid vasculitis, 39 patients with Wegener's granulomatosis, 3 patients each with microscopic Polyangiitis, and 5 patients each with temporal Arteritis or Takayasu disease with Etanercept as well as with Infliximab. In addition, Infliximab was also used with a good response in severe Polymyalgia rheumatica in 4 cases and in one case of a cryoglobulinemic vasculitis. More than 60 patients with Panuveitis and other manifestations of Behçet's disease were treated with Infliximab or Etanercept according to preliminary reports. Because of the overall positive reports with TNF alpha collected in this review controlled investigations for their use in primary systemic vasculitis are necessary.

Antibodies, Monoclonal↗