Rheumatology A Department, Paris Descartes University, Cochin Hospital, Paris, France.
J Rheumatol. 2010 Mar;37(3):608-14. doi: 10.3899/jrheum.090815. Epub 2010 Jan 28.
To assess the prevalence and potential associations with the systemic sclerosis (SSc) phenotype of additional autoimmune diseases (AID).
A multicenter study was performed in France and Italy to recruit consecutive European Caucasian patients with SSc systematically assessed for the coexistence of predefined AID known to occur with connective tissue diseases.
We recruited 585 French and 547 Italian patients with SSc. Specific AID were found in 114/585 (19%) French and 179/547 (33%) Italians with SSc (p < 0.0001). Sjögren's syndrome and thyroiditis were the predominant AID in both cohorts (12% for Sjögren's syndrome and 6% for thyroiditis in the combined populations). The frequency of myositis, primary biliary cirrhosis, rheumatoid arthritis, and systemic lupus erythematosus was low (< 4%) and similar in both cohorts. The coexistence of at least 1 of the AID in the whole cohort was associated in multivariate analysis with the limited cutaneous subtype, the presence of antinuclear antibodies, and a lower prevalence of digital ulcers.
Our study shows that 21% of this large series of European Caucasian patients with SSc have developed at least 1 AID. This latter condition identified a subset of patients with milder disease. Thus, associations of AID and autoimmune background in SSc have to be considered for further therapeutic and biological investigations in SSc.
评估系统性硬化症（SSc）表型患者伴发其他自身免疫性疾病（AID）的患病率及潜在相关性。
法国和意大利进行了一项多中心研究，对连续入组的欧洲白种人 SSc 患者进行系统性评估，以明确已知与结缔组织病相关的预定义 AID。
我们共招募了 585 例法国和 547 例意大利 SSc 患者。在法国患者（114/585，19%）和意大利患者（179/547，33%）中发现了特定的 AID（p<0.0001）。干燥综合征和甲状腺炎是这两个队列中的主要 AID（合并人群中干燥综合征为 12%，甲状腺炎为 6%）。多发性肌炎、原发性胆汁性肝硬化、类风湿关节炎和系统性红斑狼疮的发生率较低（<4%），且在两个队列中相似。在多变量分析中，整个队列中至少伴发 1 种 AID 与局限性皮肤亚型、抗核抗体存在及较少发生指端溃疡有关。
我们的研究表明，在这一大系列欧洲白种人 SSc 患者中，有 21%至少伴发 1 种 AID。后者可识别出疾病较轻的患者亚群。因此，在 SSc 中，应考虑 AID 与自身免疫背景的相关性，以进一步进行治疗和生物学研究。
