AA-amyloidosis in patient, suffering from granulomatosis with polyangiitis (Wegener)

Despite the fact that ANCA-associated vasculitis are pronounced inflammatory diseases, there are only few reports of cases of diagnosis of AA-amyloidosis in microscopic polyangiitis and the eosinophilic granulomatosis with polyangiitis (Churg-Strauss) in foreign literature. In this article we for the first time present evidence for the development of AA-amyloidosis in an elderly man, suffering from granulomatosis with polyangiitis (Wegener). Renal disease was characterized by the development of crescent pauci-immune glomerulonephritis with rapidly progressive course. After a course of induction therapy complete clinical and immunological remission was achieved, which persisted on the background of maintenance therapy. Rare manifestations of a weak activity of vasculitis almost did not affect the renal function. Death has developed as a result of multiple organ failure on the background of the septic complications of acute pneumonia. Clinical manifestations of systemic amyloidosis were absent. AA-amyloidosis was diagnosed by post-mortem morphological study and with high probability was a result of the underlying disease.

АА-амилоидоз, ANCA-ассоциированные васкулиты, гранулематоз с полиангиитом (Вегенера), AA-amyloidosis, the ANCA-associated vasculitis, granulomatosis with polyangiitis (Wegener&apos, s)

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