Abstract:

ABSTRACT A 33-year-old man with multiple organ systems involvement, including the pancreas、cholecyst、cholangitis、lung and kidney , were admitted to our hospital. The disorders developed in kidney were nephrotic syndrome with normal glomerular filtration, the renal biopsy showed membranous nephropathy. The disorders developed in digestive system were obstructive jaundice an refractory upper abdominal pain. Cross-sectional imaging of abdomen showed diffuse pancreatic enlargement, pathological manifestations were few infiltrated lymphocytes with obvious fibrosis. The cholecyst biopsy showed that IgG4- positive plasma cells were over 50 every high power field. Bronchial asthma was last for many years. Evidently elevated serum IgG4 concentrations were detected. Hence, combining the clinical manifestation、elevated serum IgG4 concentrations and the histopathologic features, a diagnosis of IgG4-related disease was reached.

Key words: IgG4-related disease, membranous nephropathy, renal biospy