Darrieutort-Laffite Christelle, André Vincent, Hayem Gilles, Saraux Alain, Le Guern Véronique, Le Jeunne Claire, Puéchal Xavier
National Referral Center for Rare Systemic Autoimmune Diseases, Department of Internal Medicine, hôpital Cochin, université Paris Descartes, Assistance publique-Hôpitaux de Paris, 27, rue du Faubourg Saint-Jacques, 75014 Paris, France; Department of Rheumatology, Nantes University Hospital, 44000 Nantes, France.
Department of Rheumatology, Le Mans General Hospital, 72000 Le Mans, France.
Joint Bone Spine. 2015 Jul;82(4):245-50. doi: 10.1016/j.jbspin.2014.12.007. Epub 2015 Feb 10.
To characterize the interstitial cystitis (IC) associated with Sjögren's syndrome (SS).
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.
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.
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.
描述与干燥综合征（SS）相关的间质性膀胱炎（IC）。
报告3例新病例。仅纳入符合美国-欧洲干燥综合征共识标准以及欧洲间质性膀胱炎研究学会标准且IC组织学检查结果为阳性的病例。
已报道13例女性SS合并IC病例，包括此处报告的3例，平均年龄54岁。77%（n = 10）的病例中SS先出现，平均比IC早6.6年。IC的症状包括尿频（n = 11）、下腹痛（n = 8）、尿急（n = 5）、尿痛（n = 6）、血尿（n = 3）和排尿困难（n = 3）。3例发生尿路扩张，导致2例患者出现急性肾衰竭。所有（n = 13）患者经膀胱活检的膀胱炎炎症解剖学证据确诊为IC。报告了9例患者的治疗情况，其中7例（78%）接受了皮质类固醇治疗，6例部分或完全有效。3例加用免疫抑制治疗（环孢素，n = 2；硫唑嘌呤，n = 1；环磷酰胺，n = 1）。进行了局部膀胱治疗，5例进行了水囊扩张，1例患者进行了二甲基亚砜灌注。2例急性梗阻性肾衰竭患者插入了导尿管。
无感染的泌尿系统症状应使医生考虑SS患者患有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有效，且已尝试局部治疗。
