Description and ANCA associated systemic necrotising ANCA associated systemic necrotising Diagnostic criteria have clinical syndrome names Diagnostic Criteria vasculitides are life-threatening vasculitides are life-threatening revised. inflammatory diseases comprising one inflammatory diseases comprising of four clinical syndromes: one of four clinical syndromes: Wegener granulomatosis; Granulomatosis with polyangiitis (Wegener Granulomatosis); microscopic polyangiitis; Microscopic polyangiitis; syndrome; and Eosinophilic granulomatosis with ANCA (PR3 or MPO)-positive idiopathic polyangiitis (Churg-Strauss Syndrome); rapidly progressive glomerulonephritis. ANCA (PR3 or MPO)-positive idiopathic In these cases the ANCA specificity is directed rapidly progressive glomerulonephritis. against the neutrophil cytoplasmic antigens PR3 In these cases the ANCA specificity is directed and MPO. ANCA that lack MPO or PR3 specificity against the neutrophil cytoplasmic antigens tend to be non-specific. Biopsy of affected tissue PR3 and MPO. ANCA that lack MPO or PR3 is required to establish the diagnosis. specificity tend to be non-specific. Biopsy of affected tissue is required to establish the Standard combinations of corticosteroids and diagnosis. cytotoxic immunosuppression are generally effective at controlling disease, but relapses are Standard combinations of corticosteroids and common. IVIg has a limited role as one of several cytotoxic immunosuppression are generally therapeutic options in relapsing disease. effective at controlling disease, but relapses are common. IVIg has a limited role as one of several therapeutic options in relapsing disease Diagnosis is Yes Rheumatologist Treating specialists have been definded (A) required Speciality Immunologist Diagnosis must be No Which verified Specialty Exclusion Criteria Initial therapy First line or initial treatment for ANCA Wording revised (A)
Description and Diagnostic Criteria ANCA associated systemic necrotising vasculitides are life-threatening inflammatory diseases comprising one of four clinical syndromes: Wegener granulomatosis; microscopic polyangiitis; syndrome; and ANCA (PR3 or MPO)-positive idiopathic rapidly progressive glomerulonephritis. In these cases the ANCA specificity is directed against the neutrophil cytoplasmic antigens PR3 and MPO. ANCA that lack MPO or PR3 specificity tend to be non-specific. Biopsy of affected tissue is required to establish the diagnosis. Standard combinations of corticosteroids and cytotoxic immunosuppression are generally effective at controlling disease, but relapses are common. IVIg has a limited role as one of several therapeutic options in relapsing disease. ANCA associated systemic necrotising vasculitides are life-threatening inflammatory diseases comprising one of four clinical syndromes: Granulomatosis with polyangiitis (Wegener Granulomatosis); Microscopic polyangiitis; Eosinophilic granulomatosis with polyangiitis (Churg-Strauss Syndrome); ANCA (PR3 or MPO)-positive idiopathic rapidly progressive glomerulonephritis. In these cases the ANCA specificity is directed against the neutrophil cytoplasmic antigens PR3 and MPO. ANCA that lack MPO or PR3 specificity tend to be non-specific. Biopsy of affected tissue is required to establish the diagnosis. Standard combinations of corticosteroids and cytotoxic immunosuppression are generally effective at controlling disease, but relapses are common. IVIg has a limited role as one of several therapeutic options in relapsing disease Diagnostic criteria have clinical syndrome names revised. Diagnosis is required Yes Speciality Rheumatologist Immunologist Treating specialists have been definded (A) Diagnosis must be verified No Which Specialty Exclusion Criteria Initial therapy First line or initial treatment for ANCA Wording revised (A)
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