Severe deficiency of alpha 1-antitrypsin associated with cutaneous vasculitis, rapidly progressive glomerulonephritis, and colitis.
The association of vasculitis with severe deficiency of alpha 1-antitrypsin is rare. This report describes a 44-year-old man with severe deficiency of alpha 1-antitrypsin associated with diffuse vasculitis involving skin, kidney (rapidly progressive glomerulonephritis), and colon (colitis). Colitis has not previously been reported in association with deficiency of alpha 1-antitrypsin. Other reported cases are reviewed and the possible immunologic mechanisms underlying the association are discussed.
D000328:Adult; D003092:Colitis / Q000150:complications*; D003106:Colon / Q000473:pathology; D005921:Glomerulonephritis / Q000150:complications* / Q000473:pathology; D006801:Humans; D007678:Kidney Glomerulus / Q000473:pathology; D008099:Liver / Q000473:pathology; D008297:Male; D010641:Phenotype; D012867:Skin / Q000473:pathology; D014657:Vasculitis / Q000150:complications* / Q000473:pathology; D019896:alpha 1-Antitrypsin Deficiency