[巴西中西部地区系统性硬化症患者的自身抗体及其临床相关性]

Coelho Horimoto Alex Magno, da Costa Izaias Pereira

Universidade Federal de Mato Grosso do Sul, Campo Grande, MS, Brasil.

Universidade Federal de Mato Grosso do Sul, Campo Grande, MS, Brasil; Universidade de São Paulo, São Paulo, SP, Brasil.

Rev Bras Reumatol. 2015 May-Jun;55(3):229-39. doi: 10.1016/j.rbr.2014.09.007. Epub 2014 Nov 7.

INTRODUCTION

Systemic sclerosis (SSc) is a connective tissue disease of autoimmune nature characterized by the triad of vascular injury, autoimmunity (cellular and humoral) and tissue fibrosis. Autoantibodies do not seem to be simply epiphenomena, but are involved in disease pathogenesis. It is believed that the SSc-specific autoantibodies are responsible both for amplifying immune response and targeting cell types that are relevant in the pathophysiology of SSc.

OBJECTIVES

To correlate the profile of the following specific autoantibodies: anti-centromere (ACA), anti-topoisomerase I (topo I) and anti-RNA polymerase III (RNAP III) with clinical and laboratory manifestations observed in 46 patients with SSc in the Midwest region of Brazil.

METHODS

The occurrence of specific autoantibodies in 46 patients with SSc was investigated, correlating the type of autoantibody with clinical and laboratory manifestations found.

RESULTS

Among all patients evaluated, we found a predominance of females (97.8%), mean age 50.21 years old, Caucasian (50%), limited cutaneous SSc (47.8%), time of diagnosis between 5-10 years (50%), and disease duration of 9.38 years. According to the specific autoantibody profile, 24 patients were ACA-positive (52.2%), 15 were positive for anti-topo I (32.6%), and 7 showed positive anti-RNAP III (15.2%). The anti-topo I autoantibody correlated with diffuse scleroderma, with greater disease severity and activity, with worse quality of life measured by the SHAQ index, with a higher prevalence of objective Raynaud's phenomenon and digital pitting scars of fingertips. The ACA correlated with limited scleroderma, with earlier onset of disease, as well as higher prevalence of telangiectasias. The anti- RNAP III correlated with diffuse scleroderma, with a higher occurrence of subjective Raynaud's phenomenon and muscle atrophy. There was no association between the positivity for anti-topo I, ACA and anti-RNAP III antibodies and other variables related to laboratory abnormalities, as well as Rodnan skin score and skin, vascular, musculoskeletal, gastrointestinal, cardiopulmonary and renal manifestations.

CONCLUSIONS

The clinical subtype of the disease and some clinical manifestations in SSc may correlate positively with the presence of specific autoantibodies.

引言

系统性硬化症（SSc）是一种自身免疫性结缔组织疾病，其特征为血管损伤、自身免疫（细胞免疫和体液免疫）及组织纤维化三联征。自身抗体似乎并非仅仅是附带现象，而是参与了疾病的发病机制。据信，SSc特异性自身抗体既负责放大免疫反应，又靶向与SSc病理生理学相关的细胞类型。

目的

将以下特异性自身抗体的谱型：抗着丝点抗体（ACA）、抗拓扑异构酶I（topo I）和抗RNA聚合酶III（RNAP III）与巴西中西部地区46例SSc患者的临床和实验室表现进行关联分析。

方法

调查46例SSc患者中特异性自身抗体的出现情况，将自身抗体类型与所发现的临床和实验室表现进行关联分析。

结果

在所有评估患者中，女性占主导（97.8%），平均年龄50.21岁，白种人（50%），局限性皮肤型SSc（47.8%），诊断时间在5至10年之间（50%），病程9.38年。根据特异性自身抗体谱型，24例患者ACA阳性（52.2%），15例抗topo I阳性（32.6%），7例抗RNAP III阳性（15.2%）。抗topo I自身抗体与弥漫性硬皮病相关，疾病严重程度和活动度更高，通过SHAQ指数测量的生活质量更差，客观雷诺现象和指尖凹陷性瘢痕的患病率更高。ACA与局限性硬皮病相关，发病更早，毛细血管扩张的患病率更高。抗RNAP III与弥漫性硬皮病相关，主观雷诺现象和肌肉萎缩的发生率更高。抗topo I、ACA和抗RNAP III抗体阳性与其他实验室异常相关变量以及罗德南皮肤评分和皮肤、血管、肌肉骨骼、胃肠道、心肺和肾脏表现之间无关联。