IgG4相关腹膜后纤维化致急性肾损伤和硬化性胆管炎1例及文献复习

Acute Kidney Injury and Sclerosing Cholangitis due to IgG4-Related Retroperitoneal Fibrosis: A Case Report and Literature Review

摘要: 目的：通过1随访长达8年的IgG4相关疾病(IgG4-related disease, IgG4-RD)患者诊治过程探讨该病的临床特征和诊治进展。方法：对1例IgG4-RD患者的诊治过程进行描述和分析，结合文献和最新进展进行文献复习。结果：老年男性患者，于2011年6月因“左下肢水肿4月余，双下肢水肿并加重10天”第1次入院，CT显示后腹膜增厚和双侧肾盂积水，诊断为特发性腹膜后纤维化、急性梗阻性肾病和急性肾衰，给予皮质激素治疗后迅速好转。2013年5月因“腹痛1月，全身皮肤及巩膜黄染5天”第2次入院，影像学检查发现近端肝内狭窄伴远端胆管扩张，肝门软组织占位，诊断为胆管癌。但在长期随访中发现血清IgG4水平异常升高，病程良好，而且激素治疗有效，最终诊断为IgG4相关硬化性胆管炎。结论：IgG4-RD可在不同时间先后以IgG4相关腹膜后纤维化和IgG4相关硬化性胆管炎形式发病，前者可表现独特的影像学特点，后者可高度模拟胆管癌的影像学改变，容易误诊。

Abstract: Objective: We discuss the clinical characteristics and progress in diagnosis and treatment of IgG4-related disease (IgG4-RD) by following up a patient with this disease for up to 8 years. Methods: The diagnosis and treatment of a case with IgG4-RD were described and analyzed. Meanwhile, a literature review was carried out by focusing on IgG4 related retroperitoneal fibrosis (IgG4-RPF) and IgG4-related sclerosing cholangitis (IgG4-SC). Methods: The elderly male patient was admitted to our hospital for the first time in June 2011 due to “left lower limb edema for more than 4 months, then progress to bilateral lower limb edema for 10 days”. CT revealed retroperitoneal membrane thickening and bilateral hydronephrosis. The patient was diagnosed as idiopathic retroperitoneal fibrosis, acute obstructive nephropathy, and acute renal failure. He improved rapidly by corticosteroid therapy. In May 2013, he was readmitted because of “abdominal pain for 1 month, yellow staining of skin and sclera for 5 days”. The imaging examination revealed proximal intrahepatic stenosis with distal bile duct dilation and soft tissue occupying lesions at hilar region, which might imply a diagnosis of cholangiocarcinoma. However, in the long-term follow-up, the course of the disease was good, and corticosteroid therapy was effective. Combining the evidence of abnormal increased serum IgG4 levels, the patient was finally diagnosed as IgG4-SC. Conclusions: IgG4-RD can occur at different times in the form of IgG4-RPF and IgG4-SC successively. The former may present unique imaging characteristics, while the latter may highly mimick cholangiocarcinoma and is easy to be misdiagnosed.