WCN25-3856 BIZARRE CLINICAL PRESENTATIONS OF DISORDERS OF COMPLEMENT DYSREGULATION – CASE SERIES

There are 3 pathways of complement activation-Classical, Lectin and Alternative pathway The alternative pathway is persistently active at a low level . Complement regulators are present to prevent its uncontrolled activation . The major complement factor regulators are Factor H(CFH) , Factor I (CFI), MCP (CD 46).Dysregulation of alternative pathway can lead on to Atypical HUS (TMA) and C3 glomerulopathy.They have varied clinical and pathological presentations .Genetic study is essential for diagnosis and in further management especially with regard to renal transplantation .Aim of this case series is to analyse the varied clinical presentations of disorders of complement dysregulation and to highlight the importance of genetic testing in diagnosis and treatment.