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ANCA-ASSOCIATED VASCULITIS, DIAGNOSIS, PATHOPHYSIOLOGY AND TREATMENT
The idiopathic small vessel vasculitides have been recognized as autoimmune disorders characterized by the presence of anti-neutrophil cytoplasmic antibodies (ANCA) directed to proteinase-3 (Pr3) and myeloperoxidase (MPO). Anti-Pr3/anti-MPO are both sensitive and specific markers for the disorders, and rises in their levels proceed relapses in about 78% of cases although these rises are not highly specific for ensuing relapses. In vitro and in vivo experimental data suggest that the autoimmune response contributes to the immunopathology of the lesions but additional factors seem to be required. These supposedly exogenous factors may include chronic nasal carriage of Staphylococcus aureus, silica exposure, and others. ANCA-testing has contributed to the (early) diagnosis of the associated diseases as well as to the insight into their pathophysiology, but treatment, generally, still consists of steroids and cyclophosphamide. However, new and less toxic treatment modalities are currently being tested by the European Vasculitis Working Party. It has already been shown that azathioprine is as effective and less toxic than cyclophosphamide for maintenance of remission. Other studies will be discussed.
VASCULITIS IN CHILDHOOD
The childhood vasculitides can be considered under a number of headings but to formally classify them has proved difficult. A classification of sorts that has had some practical value divides the childhood vasculitides into the following groups: polyarteritis (macroscopic, microscopic, cutaneous), Kawasaki disease, granulomatous vasculitis (Wegener's, Churg-Strauss, primary cerebral angiitis), leucocytoclastic vasculitis (Henoch-Schonlein, hypersensitivity angiitis, hypocomplementaemic urticarial vasculitis), “Rheumatic” vasculitis (SLE, dermatomyositis, juvenile chronic arthritis, mixed connective tissue disease), giant cell arteritis (Takayasu) and miscellaneous (Cogan's etc). This is far from perfect and criteria for inclusion into the various categories have not been fully established. This is compounded by the considerable overlap that occurs between various vasculitic syndromes causing most clinicians who see a lot of vasculitis to become “lumpers” rather than “splitters” in terms of categorization. ACR criteria …