风湿

羟氯喹治疗原发性干燥综合征有效

作者:翻译:北医三院 金银姬 来源:中国风湿病公众论坛 日期:2017-12-08
导读

         在过去几年,很多研究评估了羟氯喹在原发性干燥综合征(pSS)的治疗价值,但其在sicca综合征及腺体外表现(EGM)的疗效结果不一。

        ☆ 目的

        本研究的目的为比较使用和未使用羟氯喹的干燥综合征患者EGM的发生率。

        ☆ 方法

        我们进行了一项多中心回顾性研究,纳入符合欧洲分类标准的干燥综合征患者,随访至少1年。排除合并纤维肌痛、自身免疫性肝炎、原发性胆汁性肝硬化和原发性硬化性胆管炎的患者。对干燥综合征人口统计学及特征进行记录。根据ESSDAI评分定义EGM。根据是否使用HCQ,将患者分为两组。我们评估了HCQ的使用与EGM的关系。HCQ治疗被定义为持续使用HCQ至少3个月。

        ☆ 结果

        我们通过χ2检验或Fisher精确检验比较了两组之间EGM发生率。本研究共纳入221名患者,其中97.1%为女性,平均年龄为55.7岁(SD 14)。诊断干燥综合征时平均年龄为48.8岁(SD 15)。平均病程60个月(IQR 35-84)。170例患者(77%)接受HCQ治疗。约一半的患者在发病过程中至少有一个EGM,其中20%患者在sicca综合征发病前发展为EGM,26%患者同时合并干燥症状。总的来说,EGM在用HCQ治疗的患者中少见(36.5% vs 63.5%,p<0.001)。考虑到每个EGM的个体表现,以下临床表现在未用HCQ组中更常见:关节炎(P<0.001),乏力 (P<0.001), 紫癜(P=0.01),雷诺现象(P=0.003)及高丙种球蛋白血症 (P=0.006)。28例患者有使用免疫抑制剂的指征,其中13例同时接受了HCQ。治疗的首要原因为12/28个患者存在关节炎(42.8%),上述患者均使用甲氨蝶呤。仅有3名患者因存在肾小球肾炎、血管炎及间质性肺病,使用环磷酰胺治疗,没有患者用生物制剂。

        ☆ 结论

        使用HCQ治疗的患者中EGM发生率低,表明HCQ对原发性干燥综合征治疗有效。然而,需要进一步的大规模前瞻性研究来证实该发现。

        原 文

        Abstract

        Theuse of hydroxychloroquine (HCQ) in Primary Sjögren's Syndrome (pSS) has beenassessed in different studies over the last years, with conflicting resultsregarding its efficacy in sicca syndrome and extraglandular manifestations(EGM).

        The goal of this study was to compare the incidence rate of EGM in pSSpatients with and without HCQ therapy.

        We performed a multicenter retrospectivestudy, including patients with pSS (European classification criteria) with atleast 1year of follow-up. Subjects with concomitant fibromyalgia,autoimmune hepatitis, primary biliary cirrhosis, and primary sclerosingcholangitis were excluded. Demographics and pSS characteristics were recorded.The EGM were defined by EULAR-SS disease activity index (ESSDAI). Patients weredivided into two groups according to their use or not of HCQ therapy. Weevaluated the use of HCQ and its relationship to EGM. HCQ therapy was definedas the continuous use of the drug for at least 3months. A descriptiveanalysis of demographics and pSS characteristics was performed.

        We compared theincidence of EGM between groups defined by HCQ therapy using chi2test or Fisher's exact test. A total of 221 patients were included (97.3%women), mean age, 55.7years (SD 14). Mean age at diagnosis,48.8years (SD 15); median disease duration, 60months (IQR 35-84).One hundred and seventy patients (77%) received HCQ. About half of the patientshad at least one EGM during the course of the disease, 20% of them developed anEGM before the onset of the sicca syndrome and 26% simultaneously with drynesssymptom. Overall, EGM were less frequent in those on HCQ therapy (36.5% vs63.5%, p<0.001). Considering each EGM individually, the followingmanifestations were more frequent in the non-treated group: arthritis(p<0.001), fatigue (p<0.001), purpura(p=0.01), Raynaud phenomenon (p=0.003), andhypergammaglobulinemia (p=0.006). Immunosuppressive treatment wasindicated on 28 patients (12.7%), 13 of which were receiving also HCQ. Thefirst reason for those treatments was the presence of arthritis in 12/28patients (42.8%), and the drug used in all the cases was methotrexate. Only three patients required immunosuppressive therapy with cyclophosphamide, due tothe presence of glomerulonephritis, vasculitis, and interstitial lung disease.None of the patients received biologic therapy.

        The lower incidence of EGM wasobserved in patients on HCQ therapy supports its efficacy in pSS. However,further large scale prospective studies are needed to confirm these findings.

        引自:Demarchi J,Papasidero S,Medina MA,et al.Primary Sjögren's syndrome: Extraglandularmanifestations and hydroxychloroquine therapy.ClinRheumatol.2017 Sep 14. doi: 10.1007/s10067-017-3822-3. [Epubahead of print]

        转自:中国风湿病公众论坛

        翻译:北医三院 金银姬

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