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[系统性硬化症与类风湿关节炎的重叠:一种独特的临床实体?]

[Overlap between systemic sclerosis and rheumatoid arthritis: a distinct clinical entity?].

作者信息

Horimoto Alex Magno Coelho, Costa Izaias Pereira da

机构信息

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.

出版信息

Rev Bras Reumatol. 2015 Mar 4. doi: 10.1016/j.rbr.2014.12.011.

Abstract

INTRODUCTION

Systemic sclerosis (SSc) is an autoimmune disease of the connective tissue characterized by the triad of vascular injury, autoimmunity (cellular and humoral) and tissue fibrosis. It is estimated that musculoskeletal pain is a common complaint of patients with SSc, ranging from 40 to 80%, and mainly in patients with early diffuse disease. Arthritis, clinically observed, may be a feature seen in the presentation of SSc, often leading to early diagnostic errors with rheumatoid arthritis (RA). In the course of the disease, arthritis is observed in 24 to 97% of patients with SSc.

OBJECTIVES

To correlate the occurrence or nonoccurrence of arthritis in patients with SSc of the Midwest region of Brazil with possible distinct clinical and laboratory manifestations observed in three groups of patients. To report the frequency of true association between systemic sclerosis and rheumatoid arthritis in patients with clinically and radiologically observed synovitis.

METHODS

Sixty-one SSc patients were subsequently assessed every 3 months within 1 year, in order to clinically observe the occurrence of synovitis and its patterns of progression. Patients were divided into 3 groups: 41 patients with SSc without arthritis, 16 SSc patients with arthritis and 4 patients with overlap of SSc and RA. All patients underwent a radiological examination of the hands at the end of the study.

RESULTS

Among all patients evaluated, we found a female predominance (98.7%), mean age of 50.94 years, white color (49.2%), limited form of the disease (47.6%), time of diagnosis between 5 to 10 years (47.6%) and duration of the disease of 8.30 years. Among all patients, 14 (22.9%) had positive rheumatoid factor (RF), while among those with positive RF, only 10 patients had arthritis during one-year follow-up. The antibody anticitrulline (anti-CCP) test was performed in 24 patients, being positive in 4 of them (16.7%), with positivity being observed only in patients with SSc/RA overlap. Comparing the clinical manifestations among the groups of patients, there was a higher incidence of gastritis and cardiac valvulopathy in patients with SSc and arthritis, but not in the others. In the group of patients with SSc/RA overlap and in patients with SSc and arthritis a significant reduction in quality of life was observed, measured by HAQ index, especially in patients with arthritis present during clinical evaluation. We found radiographic changes in 42.6% of patients with SSc. However, in patients with synovitis, radiological changes consistent with rheumatoid arthritis were found in 50% of patients.

CONCLUSIONS

While the frequency of clinical arthritis observed in patients with systemic sclerosis was 32.8%, the true overlap between of SSc and RA was 6.6% in this study. We also observed the frequency of positive anti-CCP in 20% of patients with arthritis versus no patients with SSc without arthritis.

摘要

引言

系统性硬化症(SSc)是一种结缔组织自身免疫性疾病,其特征为血管损伤、自身免疫(细胞免疫和体液免疫)和组织纤维化三联征。据估计,肌肉骨骼疼痛是SSc患者的常见主诉,发生率在40%至80%之间,主要见于早期弥漫性疾病患者。临床上观察到的关节炎可能是SSc表现中的一个特征,常导致早期误诊为类风湿关节炎(RA)。在疾病过程中,24%至97%的SSc患者会出现关节炎。

目的

将巴西中西部地区SSc患者关节炎的发生与否与三组患者中观察到的可能不同的临床和实验室表现相关联。报告临床和放射学观察到滑膜炎的患者中系统性硬化症与类风湿关节炎真正关联的频率。

方法

61例SSc患者在1年内每3个月进行一次评估,以便临床观察滑膜炎的发生及其进展模式。患者分为3组:41例无关节炎的SSc患者、16例有关节炎的SSc患者和4例SSc与RA重叠患者。研究结束时,所有患者均接受了手部放射学检查。

结果

在所有评估患者中,女性占主导(98.7%),平均年龄50.94岁,白人(49.2%),疾病为局限性(47.6%),诊断时间在5至10年之间(47.6%),病程8.30年。所有患者中,14例(22.9%)类风湿因子(RF)呈阳性,而在RF阳性患者中,只有10例在1年随访期间有关节炎。对24例患者进行了抗瓜氨酸抗体(抗CCP)检测,其中4例(16.7%)呈阳性,仅在SSc/RA重叠患者中观察到阳性。比较各组患者的临床表现,SSc合并关节炎患者胃炎和心脏瓣膜病的发生率较高,其他组则不然。通过健康评估问卷(HAQ)指数测量,在SSc/RA重叠组和SSc合并关节炎组患者中观察到生活质量显著下降,尤其是在临床评估时有关节炎的患者中。我们发现42.6%的SSc患者有放射学改变。然而,在滑膜炎患者中,50%的患者有与类风湿关节炎一致的放射学改变。

结论

本研究中,虽然系统性硬化症患者临床关节炎的发生率为32.8%,但SSc与RA的真正重叠率为6.6%。我们还观察到20%有关节炎的患者抗CCP呈阳性,而无关节炎的SSc患者中无此情况。

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