Heavy Chain Deposition Disease (HCDD) is rare, and presents with renal failure, proteinuria, hematuria, hypertension, and often hypocomplementemia. HCDD may mimic the pathology of immune complex, crescentic glomerulonephritis, with isolated heavy chain deposits along mesangial, glomerular, and tubular Basement Membranes (BMs), without associated light chains. The predominant heavy chain subtype is gamma. Rare cases of alpha and mu subtypes have also been reported. Immunofluorescence for heavy chains alone is diagnostic. Renal pathology includes mesangial matrix expansion, hypercellularity, and nodular sclerosis resembling diabetic glomerulosclerosis. Fewer than 40 cases have been reported. We report a case of HCDD with the novel complication of acquired angioedema.
No comments:
Post a Comment