摘要
33岁男性患者,胰、胆管、胆囊、肺和肾脏等多系统受累,哮喘病史多年。消化系统有梗阻性黄疸,伴腹部疼痛,CT示胰腺炎,胰腺弥漫性肿大,胰腺病理示少量浸润细胞,但可见纤维化;胆囊活检病理示Ig G4阳性浆细胞>50个/HP。肾脏病以肾病综合征起病,肾功能正常,肾活检病理示肾小球膜性病变。血清Ig G4水平明显升高,最终诊断为Ig G4相关性疾病。
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.
出处
《肾脏病与透析肾移植杂志》
CAS
CSCD
北大核心
2014年第5期496-500,共5页
Chinese Journal of Nephrology,Dialysis & Transplantation
