ANCA associated systemic necrotising vasculitides are The revised wording was reviewed and ANCA associated systemic Description life-threatening immune-mediated inflammatory agreed by Specialist Working Group necrotising vasculitides are and diseases comprising one of four clinical syndromes: consensus. immune-mediated Diagnostic 1. Granulomatosis with polyangiitis (Wegener inflammatory diseases comprising Criteria Granulomatosis); one of four clinical syndromes: 2. Microscopic polyangiitis; 3. Eosinophilic granulomatosis with polyangiitis 1. Granulomatosis with (Churg-Strauss Syndrome); polyangiitis (Wegener 4. ANCA (PR3 or MPO)-positive idiopathic rapidly Granulomatosis); progressive glomerulonephritis. 2. Microscopic polyangiitis; In cases the ANCA specificity is directed against the neutrophil cytoplasmic antigens PR3 and MPO. ANCA 3. Eosinophilic that lack MPO or PR3 specificity tend to be non-specific. granulomatosis with Biopsy of affected tissue is required to establish the polyangiitis (Churg-Strauss diagnosis. Syndrome); Standard combinations of corticosteroids and cytotoxic 4. ANCA (PR3 or immunosuppression are generally effective at controlling idiopathic rapidly disease, but relapses are common. 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.
Description and Diagnostic Criteria ANCA associated systemic necrotising vasculitides are immune-mediated inflammatory diseases comprising one of four clinical syndromes: 1. Granulomatosis with polyangiitis (Wegener Granulomatosis); 2. Microscopic polyangiitis; 3. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss Syndrome); 4. ANCA (PR3 or 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. ANCA associated systemic necrotising vasculitides are life-threatening immune-mediated inflammatory diseases comprising one of four clinical syndromes: 1. Granulomatosis with polyangiitis (Wegener Granulomatosis); 2. Microscopic polyangiitis; 3. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss Syndrome); 4. ANCA (PR3 or MPO)-positive idiopathic rapidly progressive glomerulonephritis. In 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. The revised wording was reviewed and agreed by Specialist Working Group consensus.