Horimoto Alex Magno Coelho, Silveira Aida Freitas do Carmo, Costa Izaias Pereira da
Universidade Federal de Mato Grosso do Sul (UFMS), Campo Grande, MS, Brasil; Serviço de Reumatologia, Hospital Regional de Mato Grosso do Sul, Campo Grande, MS, Brasil; Programa de Residência Médica em Reumatologia, Universidade Federal de Mato Grosso do Sul (UFMS), Campo Grande, MS, Brasil.
Programa de Residência Médica em Reumatologia, Universidade Federal de Mato Grosso do Sul (UFMS), Campo Grande, MS, Brasil.
Rev Bras Reumatol. 2016 Jan 13. doi: 10.1016/j.rbr.2015.09.002.
Systemic sclerosis (SSc) is a connective tissue disease of unknown etiology, characterized by a triad of vascular injury, autoimmunity and tissue fibrosis. It is known that a positive family history is the greatest risk factor already identified for the development of SSc in a given individual. Preliminary observation of a high prevalence of poliautoimmunity and of familial autoimmunity in SSc patients support the idea that different autoimmune phenotypes may share common susceptibility variants.
To describe the frequency of familial autoimmunity and poliautoimmunity in 60 SSc patients in the Midwest region of Brazil, as well as to report the main autoimmune diseases observed in this association of comorbidities.
A cross-sectional study with recruitment of 60 consecutive patients selected at the Rheumatology Department, University Hospital, Medicine School, Federal University of Mato Grosso do Sul (FMUFMS), as well as interviews of their relatives during the period from February 2013 to March 2014.
A frequency of 43.3% of poliautoimmunity and of 51.7% of familial autoimmunity in SSc patients was found. Patients with presence of poliautoimmunity and familial autoimmunity presented primarily the diffuse form of SSc, but this indicator did not reach statistical significance. The autoimmune diseases most frequently observed in poliautoimmunity patients were: Hashimoto's thyroiditis (53.8%), Sjögren's syndrome (38.5%), and inflammatory myopathy (11.5%). The main autoimmune diseases observed in SSc patients' relatives were: Hashimoto's thyroiditis (32.3%), rheumatoid arthritis (22.6%), and SLE (22.6%). The presence of more than one autoimmune disease in SSc patients did not correlate with disease severity or activity.
From the high prevalence of coexisting autoimmune diseases found in SSc patients, we stress the importance of the concept of shared autoimmunity, in order to promote a continued vigilance and promptly diagnose other possible autoimmune disease in patients, or in their kin.
系统性硬化症（SSc）是一种病因不明的结缔组织疾病，其特征为血管损伤、自身免疫和组织纤维化三联征。已知阳性家族史是已确定的特定个体发生SSc的最大风险因素。对SSc患者中多自身免疫和家族性自身免疫高患病率的初步观察支持了不同自身免疫表型可能共享常见易感性变异的观点。
描述巴西中西部地区60例SSc患者中家族性自身免疫和多自身免疫的频率，并报告在此合并症关联中观察到的主要自身免疫性疾病。
进行一项横断面研究，于2013年2月至2014年3月期间，在南马托格罗索联邦大学医学院大学医院风湿病科连续招募60例患者，并对其亲属进行访谈。
发现SSc患者中多自身免疫的频率为43.3%，家族性自身免疫的频率为51.7%。存在多自身免疫和家族性自身免疫的患者主要表现为弥漫型SSc，但该指标未达到统计学显著性。多自身免疫患者中最常观察到的自身免疫性疾病为：桥本甲状腺炎（53.8%）、干燥综合征（38.5%）和炎性肌病（11.5%）。在SSc患者亲属中观察到的主要自身免疫性疾病为：桥本甲状腺炎（32.3%）、类风湿关节炎（22.6%）和系统性红斑狼疮（22.6%）。SSc患者中存在一种以上自身免疫性疾病与疾病严重程度或活动度无关。
鉴于在SSc患者中发现共存自身免疫性疾病的高患病率，我们强调共享自身免疫概念的重要性，以便提高持续警惕性，并及时诊断患者及其亲属中其他可能的自身免疫性疾病。
