累及肾盂的IgG4相关性疾病1例报告并文献复习

Renal pelvis involved IgG4-related disease(A case report and literature review)

目的:分析1例累及肾盂的IgG4相关性疾病的临床资料并进行文献复习,探讨该病的诊断和治疗经验,以提高对IgG4相关性肾盂疾病的认识及诊治水平。方法:分析北京协和医院收治的1例IgG4相关性肾盂疾病患者的临床表现、实验室检查、影像学及组织病理学资料。以"IgG4相关性疾病"和"肾盂"为关键词,自Pubmed共计检索到累及肾盂的IgG4相关性疾病4例,对临床症状、肾盂外器官受累情况、血清IgG4水平、影像学表现和组织学以及治疗等进行分析。结果:患者女性,53岁,B超发现右肾盂占位,无临床不适,体重近半年减重3kg。尿路CT成像示右肾盂及中下部肾盏团块样软组织密度影。逆行造影未见右肾盂内明确充盈缺损。PET/CT示右肾盂软组织密度影放射性摄取增高。行腹腔镜右肾输尿管全长切除术,病理符合IgG4相关性疾病。血清IgG4明显升高(3 240mg/L),给以泼尼松口服治疗,3个月后复查IgG4水平降至1 890mg/L。文献报道IgG4相关性肾盂疾病共4例,合并其他器官受累3例。2例行患侧肾输尿管全长切除,1例行肾切除。应用糖皮质激素治疗3例,疗效良好,1例因合并严重消化道出血死亡。结论:IgG4相关性疾病是一少见疾病,累及肾盂罕见,临床易误诊为肾盂癌而行不必要的手术切除,可同时累及其他器官,诊断有赖于血清IgG4水平测定及组织病理学检查,糖皮质激素治疗有效,预后良好。

Objective： To analyze the clinical data of one case of IgG4-related renal pelvis disease and review the literature so as to improve the diagnosis and treatment of the disease. Method： The clinical symptoms, laboratory tests, radiographic patterns, histopathological features and therapeutic management of a patient with IgG4-re- lated renal pelvis disease were described and the literature was reviewed. Result： A 53-year-old female without sig- nificant symptoms was admitted to our hospital because of the tumor of right renal pelvis, with weight loss about 3 kg in half a year. CTU revealed soft tissue in the right renal pelvis, while no obvious filling defect was found with the retrograde urethrography. PET/CT demonstrated a soft tissue in right renal pelvis, with elevated 18F- FDG uptake. Surgical laparoscopic resection of the tissue from the right kidney to whole right ureter was per- formed. Histological examination disclosed IgG4-related disease of the renal pelvis. Her postoperative serum level of IgG4 elevated （3 240 mg/L）. Systemic steroid therapy was performed, then three months later her serum level of IgG4 decreased （t 890 mg/L）. Literature retrieval to four cases of IgG4-related renal pelvis disease, three pa- tients showed other organs involvement. Ureteronephrectomy was performed in two patients, while nephrectomy was performed in a patient. Systemic steroid therapy was performed in three patients, and curative effect was sig- nificant. However, only one patient died of severe gastrointestinal bleeding. Conclusion： IgG4-related renal pelvis clisease is rare. It may be misdiagnosed easily as carcinoma of the renal pelvis, and followed by unnecessary sur- geries. This kind of disease can involve other organs. The diagnosis depends on serum level of IgG4 and his- topathological examination. Systemic steroid therapy is effective, so the prognosis is good.