How is Granulomatosis with polyangiitis diagnosed

How is Granulomatosis with polyangiitis diagnosed?

GPA can present in many different ways, and some manifestations may share similarity to infectious and neoplastic etiologies. A thorough history and examination should be performed to determine the extent of organ involvement (see Questions 7–11), identify recent new medications or illicit drug exposure, and highlight clinical features that may be atypical for AAV (hard palate ulceration, single organ involvement, rapid onset without prodromal features). Laboratory and imaging studies should be conducted to assess the severity of organ involvement, to determine if ANCA antibodies are present, and to rule out other etiologies. Ideally tissue can be obtained from the lung, sinuses, skin, or kidney to identify supporting features of GPA (namely vasculitis, extravascular granulomatous inflammation, and necrosis). Biopsy of these sites may ultimately fail to identify features specific to GPA, but often can be helpful nonetheless to rule out competing diagnoses on the differential, especially in cases of limited GPA where classic organ involvement may be absent and ANCA positivity may be as low as 60%. In classic presentations of generalized GPA, the sensitivity of ANCA testing is high (90%) and the presence of concurrent c-ANCA and PR-3 antibodies is highly specific (98%); in such settings, biopsy may not be necessary.

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