免疫球蛋白第四型相關後腹腔纖維化罕見病例討論併文獻探討
吳翊豪 陳文祥 林政鋒 劉冠麟 林承家 吳俊德 陳鴻毅
基隆長庚紀念醫院 外科部 泌尿科
IgG4-related retroperitoneal fibrosis: a rare disease case report and literature review
Yi-Hao Wu, Wen-hsiang Chen, Cheng-Feng Lin, KL Liu, Cheng-Chia Lin, Chun-Te Wu, Hong-Yi Chen
Department of urology, Chang Gung Memorial Hospital, Keelung, Taiwan
Background:
Retroperitoneal fibrosis (RPF) is a rare disease characterized by chronic, nonspecific inflammatory and sclerotic or fibrotic tissue in the periaortic or periiliac retroperitoneum that encases adjacent structures. There will be a series of clinical manifestations once the proliferated fibrous tissues encase the abdominal aorta, iliac arteries and urinary duct. RPF is generally divided into two types: idiopathic retroperitoneal fibrosis (IRPF) without identified pathogenesis, making up about two-thirds of cases, and secondary retroperitoneal fibrosis. Recent studies on Immunoglobulin G4-related disease (IgG4-RD) reveal that abundant infiltration of IgG4 positive plasma cells is found in biopsies on the mass of RPF of some IRPF patients, which is identified as one spectrum of IgG4-RD and is named IgG4-related RPF.
Case summary: A 66 year-old male with underlying disease of hypertension suffered from oligouria for two days. Abdominal CT showed retroperitoneal mass encase aorta, bilateral common iliac vessel, bilateral ureter, which lead to bilateral hydronephrosis. Therefore, PCN was placed for emergent urine diversion. CT guided biopsy showed IgG4 related disease even no high serum IgG4 was noticed. After methylprednisolone treatment, retroperitoneal fibrosis was regressed and hydronephrosis was subsided.
Discussion:
Because IgG4-related RPF is a rare and relatively newly characterized disease, there is no precise report of its morbidity rate. IgG4-related RPF has no specific symptoms at the early stage. However, fibrosclerotic retroperitoneal tissues may encase and compress visceral organs at a later stage, resulting in damage of different visceral organs. Elevated serum IgG4 concentration is an indicator of IgG4-RD. However, studies show that between 3% and 30% of IgG4-RD patients have normal serum IgG4 concentrations. Histopathology and immunohistochemistry examinations of these patients meet the diagnostic criteria of IgG4-RD. Glucocorticoid is commonly regarded as the first-line agent for treatment. We report IgG4 related retroperitoneal fibrosis case and literature review.