IgG4相关性腹膜后纤维化CT诊断

摘要

Objective: To study and summarize the clinical features and the imaging manifestations of IgG4-related retroperitoneal fibrosis and to improve the understanding of IgG4-related sclerosing disease. Methods: A case of IgG4-related retroperitoneal fibrosis was reported,including clinical features,laboratory reports,imaging manifestations and pathological results,and relative literatures were reviewed. Results: The disease had no distinctive clinical features. Symptoms include vague pain in the lower back or abdomen,anorexia,fatigue,fever and weight loss. The imaging manifestation was soft tissue mass. The level of serum IgG (especially IgG4) was strikingly high. The histological manifestation in the involved organs showed diffuse infiltration of lymphoplasma cells and fibrosis. Abundant IgG4-positive plasma cells could be found and the treatment with glucocorticoid was effective. Conclusion:IgG4-related retroperitoneal fibrosis lacks specifity. It is essential to improve its understanding to avoid misdiagnosis.%目的:总结IgG4相关腹膜后纤维化的临床表现及影像特点,提高时IgG4相关的硬化性疾病的认识.方法:报告1例IgC4相关腹膜后纤维化的临床表现、实验室检查、影像学资料、病理结果等,并复习相关文献.结果:该病临床症状无明显特异性,可有腹痛、背痛、疲乏、食欲下降等,影像表现为腹膜后肿块,该病与自身免疫性胰腺炎关系密切,血清IgG尤其是IgG4显著升高;受累脏器组织学表现为弥漫性淋巴浆细胞漫润和纤维化,大量IgG4阳性浆细胞;糖皮质激素治疗有效.结论:IgG4相关腹膜后纤维化临床症状缺乏特异性,极易误诊或漏诊,需提高对该病的认识.