摘要

目的：明确干燥综合征(SS)相关的间质性膀胱炎(IC)的特点。

方法：报告了三例新患者，均符合美国和欧洲共识标准的干燥综合征和欧洲间质性膀胱炎研究协会标准，并存在间质性膀胱炎阳性组织学检查结果。

结果：文献共报告了13例女性SS合并IC，其中包括3例我们的报告，平均年龄54岁。 SS在77%(n = 10)患者中作为首发症状出现，平均在IC出现前6.6年。 IC症状包括尿频(n = 11)，下腹痛(n = 8)，尿急(n = 5)，排尿痛(n = 6)，血尿(n = 3)和排尿困难(n = 3) 。尿路扩张3例，导致2例急性肾功能衰竭。所有(n = 13)患者均通过膀胱活检提示的膀胱炎症的解剖证据证实了IC的诊断。9例患者的治疗有报道，其中7例(78%)接受皮质类固醇治疗，其中6例部分或全部有效。3例患者接受了免疫抑制治疗(环孢素，n = 2;硫唑嘌呤，n = 1;环磷酰胺，n = 1)。部分患者接受了局部膀胱治疗，其中5例进行了液压扩张治疗，1例接受了DMSO滴注治疗。两例急性梗阻性肾衰竭患者接受了导尿治疗。

结论：医师发现无感染的尿路症状应考虑SS合并IC的诊断。可能发生尿路扩张，导致急性梗阻性肾衰竭。皮质类固醇治疗可能是有效的，并且已有局部治疗的尝试。

附原文

AbstractOBJECTIVES: To characterize the interstitial cystitis (IC) associated with Sjögren's syndrome (SS).METHODS: Report of three new cases. Only cases fulfilling the American-European consensus criteria for SS and the European Society for the Study of Interstitial Cystitis criteria with positive histological findings for IC were included.RESULTS: Thirteen cases of SS and IC have been reported in women, including the three reported here, with a mean age of 54 years. SS appeared first in 77% (n=10) of cases, a mean of 6.6 years before IC. The symptoms of IC included pollakiuria (n=11), lower abdominal pain (n=8), urinary urgency (n=5), painful micturition (n=6), hematuria (n=3) and dysuria (n=3). Urinary dilatation occurred in three cases, leading to acute renal failure in two patients. The diagnosis of IC was confirmed by anatomical evidence of cystitis inflammation on bladder biopsy in all (n=13) patients. Treatment was reported for nine patients, seven of whom (78%) received corticosteroid treatment, which was partially or completely effective in six cases. Immunosuppressive treatment was added in three cases (cyclosporine, n=2; azathioprine, n=1; cyclophosphamide, n=1). Local bladder treatments were performed, with hydraulic distension in five cases and DMSO instillation in one patient. A urinary catheter was inserted in the two cases of acute obstructive renal failure.CONCLUSIONS: Urinary symptoms without infection should lead the physician to consider a diagnosis of IC in SS patients. Urinary dilatation may occur, leading to acute obstructive renal failure. Corticosteroid treatment may be effective and local treatments have been tried

引自

Darrieutort-Laffite C1, André V2, Hayem G3, Saraux A4, Le Guern V5, Le Jeunne C5, Puéchal X6. Sjögren's syndrome complicated by interstitial cystitis: A case series and literature review.Joint Bone Spine. 2015 Jul;82(4):245-50. doi: 10.1016/j.jbspin.2014.12.007. Epub 2015 Feb 10.