Budd-Chiari syndrome and renal failure in Behcet disease. Report of a case and review of the literature.
A case of Behcet syndrome associated with three rare complications is presented. The patient initially presented with asymmetric polyarticular arthritis, subcutaneous nodules, conjunctivitis, and episcleritis. Necrotizing vasculitis and renal failure subsequently evolved. Despite immunosuppressive therapy, Budd-Chiari syndrome with high grade hepatic obstruction developed. A Denver shunt procedure failed to alter the terminal course. At postmortem examination, skin lesions, carditis, hepatic vein thrombosis, gastric ulcerations, and focal proliferative glomerulonephritis were found. The immunoglobulins and complement present in skin and renal tissue suggest an immune complex pathogenesis.
D000328:Adult; D000936:Antigen-Antibody Complex / Q000032:analysis; D001528:Behcet Syndrome / Q000150:complications* / Q000473:pathology; D006502:Budd-Chiari Syndrome / Q000150:complications* / Q000473:pathology; D005921:Glomerulonephritis / Q000209:etiology / Q000276:immunology / Q000473:pathology; D006503:Hepatic Veins / Q000473:pathology; D006801:Humans; D007676:Kidney Failure, Chronic / Q000150:complications*; D007678:Kidney Glomerulus / Q000473:pathology; D008297:Male; D008854:Microscopy, Electron; D009206:Myocardium / Q000473:pathology; D012867:Skin / Q000473:pathology; D014457:Ulna / Q000473:pathology
D000936:Antigen-Antibody Complex