Department of Rheumatology, Immunology and Allergology, University Hospital and University of Bern, Freiburgstrasse 4, 3010, Bern, Switzerland.
German Rheumatism Research Center, A Leibniz Institute, Berlin, Germany.
Arthritis Res Ther. 2018 Jan 30;20(1):17. doi: 10.1186/s13075-018-1517-z.
BACKGROUND: Interstitial lung disease in systemic sclerosis (SSc-ILD) is a major cause of SSc-related death. Imunosuppressive treatment (IS) is used in patients with SSc for various organ manifestations mainly to ameliorate progression of SSc-ILD. Data on everyday IS prescription patterns and clinical courses of lung function during and after therapy are scarce. METHODS: We analysed patients fulfilling American College of Rheumatology (ACR)/European League against Rheumatism (EULAR) 2013 criteria for SSc-ILD and at least one report of IS. Types of IS, pulmonary function tests (PFT) and PFT courses during IS treatment were evaluated. RESULTS: EUSTAR contains 3778/11,496 patients with SSc-ILD (33%), with IS in 2681/3,778 (71%). Glucocorticoid (GC) monotherapy was prescribed in 30.6% patients with GC combinations plus cyclophosphamide (CYC) (11.9%), azathioprine (AZA) (9.2%), methotrexate (MTX) (8.7%), or mycophenolate mofetil (MMF) (7.3%). Intensive IS (MMF + GC, CYC or CYC + GC) was started in patients with the worst PFTs and ground glass opacifications on imaging. Patients without IS showed slightly less worsening in forced vital capacity (FVC) when starting with FVC 50-75% or >75%. GC showed negative trends when starting with FVC <50%. Regarding diffusing capacity for carbon monoxide (DLCO), negative DLCO trends were found in patients with MMF. CONCLUSIONS: IS is broadly prescribed in SSc-ILD. Clusters of clinical and functional characteristics guide individualised treatment. Data favour distinguished decision-making, pointing to either watchful waiting and close monitoring in the early stages or start of immunosuppressive treatment in moderately impaired lung function. Advantages of specific IS are difficult to depict due to confounding by indication. Data do not support liberal use of GC in SSc-ILD.
背景:系统性硬化症(SSc)相关的间质性肺疾病(SSc-ILD)是 SSc 相关死亡的主要原因。免疫抑制治疗(IS)用于治疗 SSc 的各种器官表现,主要是为了改善 SSc-ILD 的进展。关于治疗期间和治疗后日常 IS 处方模式和肺功能临床病程的数据很少。
方法:我们分析了符合美国风湿病学会(ACR)/欧洲抗风湿病联盟(EULAR)2013 年 SSc-ILD 标准且至少有一次 IS 报告的患者。评估了 IS 类型、肺功能检查(PFT)和 IS 治疗期间的 PFT 过程。
结果:EUSTAR 包含 3778/11496 例 SSc-ILD(33%)患者,其中 2681/3778(71%)例接受 IS。30.6%的患者接受了糖皮质激素(GC)单药治疗,GC 联合环磷酰胺(CYC)(11.9%)、硫唑嘌呤(AZA)(9.2%)、甲氨蝶呤(MTX)(8.7%)或吗替麦考酚酯(MMF)(7.3%)。在影像学上出现严重的 PFT 下降和磨玻璃样混浊的患者开始接受强化 IS(MMF+GC、CYC 或 CYC+GC)治疗。无 IS 治疗的患者,在开始治疗时 FVC 为 50-75%或>75%时,FVC 的恶化程度略低。当开始 FVC<50%时,GC 呈负趋势。对于一氧化碳弥散量(DLCO),在 MMF 治疗的患者中发现 DLCO 呈负趋势。
结论:IS 在 SSc-ILD 中广泛应用。临床和功能特征的聚类指导个体化治疗。数据支持在疾病早期进行观察和密切监测,或在中度肺功能受损时开始免疫抑制治疗,从而做出明智的决策,这与负面趋势相矛盾。由于混杂因素的影响,很难描述特定 IS 的优势。数据不支持在 SSc-ILD 中自由使用 GC。
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