IgG4相关性腹膜后纤维化一例及IgG4相关性疾病文献复习

Report on one case of IgG4-related retroperitoneal fibrosis and literatures review for IgG4-related disease

目的探讨IgG4相关性腹膜后纤维化（IgG4-RRPF）和IgG4相关性疾病（IgG4．RD）的临床表现、诊断和治疗方法。方法对1例IgG4-RRPF患者的临床资料进行回顾性分析，复习相关文献并介绍一个新的临床病种IgG4-RD。结果患者，52岁，男性，以左下腹痛和左。肾积液为表现，影像学显示左侧腰大肌内侧异常软组织影，PET—CT显示该软组织影脱氧葡萄糖代谢摄取增高，经肿块切取活检术，病理诊断符合IgG4-RD，诊断为IgG4-RRPF，予泼尼松治疗后症状缓解，肿块缩小。复习最新文献发现IgG4-RRPF即以往所称的特发性腹膜后纤维化（IRF）。结论IgG4-RRPF是IgG4-RD谱中的一种罕见类型，以腹膜后组织慢性炎症和显著纤维化为特征，常包绕输尿管或其他腹部脏器，血液学检查显示血清IgG4浓度升高（≥1．35g／L），组织病理学检查显示明显淋巴细胞和IgG4＋浆细胞浸润伴纤维化。一线治疗给予糖皮质激素。

Objective To explore the clinical manifestation, diagnosis and treatment of IgG4-related retroperitoneal fibrosis （IgG4-RRPF） and IgG4-related disease （IgG4-RD）. Methods The clinical data of a case of IgG4-RRPF was analyzed retrospectively, the related literatures were reviewed, and a novel clinical entity IgG4-RD was introduced. Results The patient was a 52-year-old man with the complaint of left lower abdominal pain due to left hydronephrosis. CT and MRI revealed a soft tissue mass at the left psoas major muscle. PET-CT showed increased FDG uptake. After biopsy, the pathological diagnosis was consistent with IgG4-RD. The patient was diagnosed as IgG4-RRPF and treated with prednisone. The symptoms improved and retroperitoneal mass reduced. According to review the latest literatures, it is found that IgG4-RRPF was so- called idiopathic retroperitoncal fibrosis （IRF） formerly. Conclusion IgG4-RRPF is a rare disease in IgG4- RD spectrum, characterised by the presence of a retroperitoneal tissue, consisting of chronic inflammation and marked fibrosis, which often entraps the ureters or other abdominal organs. Hematological examination shows elevated serum IgG4 concentration （≥1.35 g/L）. Histopathological examination shows marked tissue infiltration by lymphocytes and IgG4-plasma cells with fibrosis. Glucocorticoids are the first line of therapy.