title: Cyclophosphamide therapy for Weber-Christian disease associated with alpha 1-antitrypsin deficiency.
abstract: Weber-Christian disease is an uncommon disorder of unclear etiology whose unifying feature is fatty tissue inflammation. Its association with alpha 1-antitrypsin deficiency produces severe clinical manifestations. Although many agents have been used to treat Weber-Christian disease, current therapy is based on the poorly documented use of corticosteroids. We have reported the case of a young man with life-threatening Weber-Christian disease associated with alpha 1-antitrypsin deficiency unresponsive to corticosteroids who responded to cyclophosphamide.
mesh_terms: D000328:Adult; D003520:Cyclophosphamide / Q000627:therapeutic use*; D006801:Humans; D008297:Male; D010201:Panniculitis, Nodular Nonsuppurative / Q000150:complications / Q000188:drug therapy*; D019896:alpha 1-Antitrypsin Deficiency
chemical_list: D003520:Cyclophosphamide