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)
Indication for use Control of vasculitic activity in rare of systemic necrotising vasculitis failing to respond to corticosteroids and cytotoxic immunosuppression. ANCA positive systemic necrotising vasculitis failing to respond to corticosteroids and cytotoxic immunosuppression Relapse in ANCA positive systemic necrotising vasculitis resistant following response to Ig therapy Two indications have been used. All qualifying patients will only receive 6 months treatment. A second indication supports the further treatment of responders who relapse following cessation of Ig therapy. Different qualifying and review criteria will (A) trial
Qualifying Criteria MPO or PR3 ANCA-positive systemic necrotising vasculitis with both of the following: Current (or within the previous six months) standard cytotoxic immunosuppressive ANCA-vasculitis regimens; AND Persistent active disease. ANCA positive systemic necrotising vasculitis failing to respond to corticosteroids and cytotoxic immunosuppression Evidence of active MPO or PR3 positive vasculitis confirmed by serology and an ANCA level above the normal reference range unless negative ANCA level with active vasculitis AND At least two reactive indicators when assessed by Birmingham Vasculitis Activity Score (BVAS) version 3 (v3) (score > 5), Erythrocyte Sedimentation Rate (ESR) (score >5) and protein (CRP) (score >6). AND Persistent disease despite standard Corticosteroid therapy for 6 months unless steroid therapy is contraindicated AND A of Rituximab has failed to demonstrate a response unless Rituximab is inaccessible or contraindicated AND At least two other immunosuppressant agents have been trialled in addition to steroids and Qualifying criteria have been defined to expand diagnostic requirements and gather data to be used as a comparison (to ensure response to Ig) should patients relapse and require further treatment in the future. (A) Requirements consistent with the original criteria have been used and revised to include rituximab treatment (if available) and steroid treatment. The Immunosuppressant therapy options are : Cyclophosphamide Azathioprine Mycophenolate Methotrexate