Lazzaroni Maria-Grazia, Marasco Emiliano, Campochiaro Corrado, DeVries-Bouwstra Jeska, Gonzalez-Perez Montserrat-Ixchel, Rojas-Serrano Jorge, Hachulla Eric, Zanatta Elisabetta, Barsotti Simone, Furini Federica, Triantafyllias Konstantinos, Abignano Giuseppina, Truchetet Marie-Elise, De Luca Giacomo, De Langhe Ellen, Hesselstrand Roger, Ingegnoli Francesca, Bertoldo Eugenia, Smith Vanessa, Bellando-Randone Silvia, Poormoghim Hadi, Colombo Enrico, Ceribelli Angela, Furloni Alessio, Zingarelli Stefania, Cavazzana Ilaria, Franceschini Franco, Del Galdo Francesco, Denton Christopher P, Cavagna Lorenzo, Distler Oliver, Allanore Yannick, Airò Paolo
Rheumatology and Clinical Immunology Unit, ASST Spedali Civili of Brescia.
Department of Clinical and Experimental Sciences, University of Brescia, Brescia.
Rheumatology (Oxford). 2021 Nov 3;60(11):5028-5041. doi: 10.1093/rheumatology/keab152.
To evaluate clinical associations of anti-PM/Scl antibodies in patients with SSc in a multicentre international cohort, with particular focus on unresolved issues, including scleroderma renal crisis (RC), malignancies, and functional outcome of interstitial lung disease (ILD).
(1) Analysis of SSc patients from the EUSTAR database: 144 anti-PM/Scl+ without SSc-specific autoantibodies were compared with 7202 anti-PM/Scl-, and then to 155 anti-Pm/Scl+ with SSc-specific antibodies. (2) Case-control study: additional data were collected for 165 anti-PM/Scl+ SSc patients (85 from the EUSTAR registry) and compared with 257 anti-PM/Scl- SSc controls, matched for sex, cutaneous subset, disease duration and age at SSc onset.
Patients with isolated anti-PM/Scl+, as compared with anti-Pm/Scl-, had higher frequency of muscle involvement, ILD, calcinosis and cutaneous signs of DM, but similar frequency of SRC and malignancies (either synchronous with SSc onset or not). The presence of muscle involvement was associated with a more severe disease phenotype. Although very frequent, ILD had a better functional outcome in cases than in controls. In patients with both anti-PM/Scl and SSc-specific antibodies, a higher frequency of typical SSc features than in those with isolated anti-PM/Scl was observed.
The analysis of the largest series of anti-PM/Scl+ SSc patients so far reported helps to delineate a specific clinical subset with muscle involvement, cutaneous DM, calcinosis and ILD characterized by a good functional outcome. SRC and malignancies do not seem to be part of this syndrome.
在一个多中心国际队列中评估系统性硬化症（SSc）患者抗PM/Scl抗体的临床关联，特别关注未解决的问题，包括硬皮病肾危象（RC）、恶性肿瘤以及间质性肺病（ILD）的功能转归。
（1）对EUSTAR数据库中的SSc患者进行分析：将144例无SSc特异性自身抗体的抗PM/Scl阳性患者与7202例抗PM/Scl阴性患者进行比较，然后再与155例有SSc特异性抗体的抗PM/Scl阳性患者进行比较。（2）病例对照研究：收集了165例抗PM/Scl阳性SSc患者（85例来自EUSTAR登记处）的额外数据，并与257例抗PM/Scl阴性SSc对照进行比较，两组在性别、皮肤亚型、病程和SSc发病年龄方面相匹配。
与抗PM/Scl阴性患者相比，单纯抗PM/Scl阳性患者肌肉受累、ILD、钙质沉着和皮肌炎皮肤表现的发生率更高，但SRC和恶性肿瘤的发生率相似（与SSc发病同时或不同时）。肌肉受累与更严重的疾病表型相关。尽管ILD非常常见，但病例组的功能转归优于对照组。在同时有抗PM/Scl和SSc特异性抗体的患者中，典型SSc特征的发生率高于单纯抗PM/Scl阳性患者。
对迄今为止报道的最大系列抗PM/Scl阳性SSc患者的分析有助于明确一个特定的临床亚组，其特征为肌肉受累、皮肌炎、钙质沉着和ILD，功能转归良好。SRC和恶性肿瘤似乎不是该综合征的一部分。
