Centre Hospitalier Universitaire de Bordeaux, FHU ACRONIM, Place Amélie Raba Léon, 33076 Bordeaux, France; Centre national de référence des maladies auto-immunes et systémiques rares Est/Sud-Ouest (RESO), France; CNRS-UMR 5164 Immuno ConcEpT, 146 rue Léo Saignat, 33076 Bordeaux, France.
Centre national de référence des maladies auto-immunes et systémiques rares Est/Sud-Ouest (RESO), France; Service de rhumatologie, Centre Hospitalier Universitaire de Strasbourg, 1 avenue Molière, 67098 Strasbourg, France.
Semin Arthritis Rheum. 2021 Feb;51(1):36-42. doi: 10.1016/j.semarthrit.2020.10.009. Epub 2020 Dec 18.
To screen for concomitant autoimmune disease in patients with systemic sclerosis (overlap SSc) and to describe their clinical characteristics and prognosis.
This was a two-center retrospective observational study. Patients diagnosed with SSc according to the 2013 ACR-EULAR scleroderma classification criteria were screened for concomitant rheumatoid arthritis (RA), Sjögren syndrome (SgS) and systemic lupus erythematosus (SLE). Patient characteristics were retrieved from the medical records and were compared to those of a non-overlap SSc cohort.
Among the 534 SSc patients studied, thirty-four (6.4%) were identified as having overlap SSc. There were 21 (3.9%) patients with RA, 14 (2.6%) with SgS and 4 (0.7%) with SLE (5 patients had 2 AISD) . The disease phenotype of overlap SSc was similar to that of non-overlap SSc in terms of cutaneous phenotype, prevalence of pulmonary arterial hypertension, interstitial lung disease, digital ulcers and mortality. Using a multivariate Cox model, age (HR = 1.04, 95% CI [1.02-1.07]), the modified Rodnan skin score (HR = 1.08 per point, 95% CI [1.05-1.11]), and the presence of concomitant SgS (HR = 3.79, 95% CI [1.38-10.40]) were significantly associated with mortality. Overlap SSc were more likely to receive corticosteroids (85.3% vs. 45%, p < 0.001), immunosuppressive drugs (82.4% vs. 49.2%, p < 0.001) and biologics (52.9% vs. 3.8%, p < ZZ0.001).
While overlap and non-overlap SSc shared common characteristics, patients with SgS/SSc had a higher risk of mortality, and those with RA/SSc received more corticosteroids, methotrexate and biologics. Screening for an associated AISD should be promoted since their co-occurrence with SSc may affect prognosis and treatments.
筛查系统性硬化症（重叠型 SSc）患者合并自身免疫性疾病，并描述其临床特征和预后。
这是一项两中心回顾性观察研究。根据 2013 年 ACR-EULAR 硬皮病分类标准诊断为 SSc 的患者，筛查合并类风湿关节炎（RA）、干燥综合征（SgS）和系统性红斑狼疮（SLE）的情况。从病历中提取患者特征，并与非重叠型 SSc 队列进行比较。
在研究的 534 例 SSc 患者中，有 34 例（6.4%）被诊断为重叠型 SSc。其中 21 例（3.9%）合并 RA，14 例（2.6%）合并 SgS，4 例（0.7%）合并 SLE（5 例合并 2 种 AISD）。重叠型 SSc 的疾病表型在皮肤表型、肺动脉高压、间质性肺病、指端溃疡和死亡率方面与非重叠型 SSc 相似。使用多变量 Cox 模型，年龄（HR=1.04，95%CI[1.02-1.07]）、改良 Rodnan 皮肤评分（HR=每点 1.08，95%CI[1.05-1.11]）和合并 SgS（HR=3.79，95%CI[1.38-10.40]）与死亡率显著相关。重叠型 SSc 更可能接受皮质类固醇（85.3% vs. 45%，p<0.001）、免疫抑制剂（82.4% vs. 49.2%，p<0.001）和生物制剂（52.9% vs. 3.8%，p<0.001）。
尽管重叠型和非重叠型 SSc 具有共同特征，但合并 SgS/SSc 的患者死亡风险更高，而合并 RA/SSc 的患者接受皮质类固醇、甲氨蝶呤和生物制剂的治疗更多。应提倡筛查相关的 AISD，因为它们与 SSc 的共存可能影响预后和治疗。
