抗 C1q 自身抗体在伴有肺纤维化的系统性硬化症患者中经常被检测到。

Anti-C1q autoantibodies are frequently detected in patients with systemic sclerosis associated with pulmonary fibrosis.

机构信息

Department of Rheumatology and Clinical Immunology, University of Thessaly, Larissa, 40500, Greece.

Inova Diagnostics Inc., San Diego, CA, U.S.A.

出版信息

Br J Dermatol. 2019 Jul;181(1):138-146. doi: 10.1111/bjd.17886. Epub 2019 May 6.

DOI:10.1111/bjd.17886

PMID:30875084

Abstract

BACKGROUND

Anti-C1q autoantibodies (autoAbs) are associated with systemic lupus erythematosus (SLE), but their presence in other rheumatic diseases has not been adequately investigated.

OBJECTIVES

We aimed to assess anti-C1q autoAbs and circulating immune complexes (CICs) in systemic sclerosis (SSc).

METHODS

In total 124 patients with SSc were studied; 106 were female and the median age was 59·4 years (range 25-81·4). Overall 75 (60·5%) had limited cutaneous SSc and 49 (39·5%) had diffuse cutaneous SSc. Also included were 25 patients with Sjögren syndrome (SjS), 29 with rheumatoid arthritis (RA), 38 with SLE and 53 healthy controls. Enzyme-linked immunosorbent assays with high- and low-salt buffers were used to measure anti-C1q antibodies and CICs. The former allows only anti-C1q antibody binding to C1q and the latter also allows IgG Fc to bind to C1q.

RESULTS

Anti-C1q antibodies were present in 20 of 124 (16·1%) patients with SSc: five had high levels (> 80 RU mL ) and 10 (50%) had moderate levels (40-80 RU mL ). Anti-C1q antibodies were also present in one of 25 (4%) patients with SjS, one of 29 (3%) with RA (P < 0·05 for both) and three of 53 (6%) healthy controls (P < 0·01). Anti-C1q antibodies were detected in 13 of 38 (34%) patients with SLEs. Anti-C1q antibodies were more frequent in male than female patients with SSc (P = 0·005); this association remained after multivariate regression analysis. Anti-C1q antibody level was the most important factor in predicting the presence of pulmonary fibrosis, and the second most important in predicting pulmonary arterial hypertension. Fourteen patients with SSc (11·3%) had CICs.

CONCLUSIONS

Anti-C1q autoAbs were frequently detected in patients with SSc, and their high levels predict the co-occurrence of pulmonary fibrosis or pulmonary arterial hypertension.

摘要

背景

抗 C1q 自身抗体（autoAbs）与系统性红斑狼疮（SLE）相关，但它们在其他风湿性疾病中的存在尚未得到充分研究。

目的

我们旨在评估系统性硬化症（SSc）中的抗 C1q 自身抗体和循环免疫复合物（CICs）。

方法

共研究了 124 例 SSc 患者；106 例为女性，中位年龄为 59.4 岁（范围 25-81.4）。共有 75 例（60.5%）为局限性皮肤 SSc，49 例（39.5%）为弥漫性皮肤 SSc。还纳入了 25 例干燥综合征（SjS）患者、29 例类风湿关节炎（RA）患者、38 例系统性红斑狼疮（SLE）患者和 53 例健康对照者。采用高盐和低盐缓冲液酶联免疫吸附试验（ELISA）检测抗 C1q 抗体和 CICs。前者仅允许抗 C1q 抗体与 C1q 结合，后者还允许 IgG Fc 与 C1q 结合。

结果

124 例 SSc 患者中有 20 例（16.1%）存在抗 C1q 抗体：5 例（25%）为高水平（>80 RU/ml），10 例（50%）为中水平（40-80 RU/ml）。在 25 例 SjS 患者中有 1 例（4%）、29 例 RA 患者中有 1 例（3%）（两者均 P<0.05）和 53 例健康对照者中有 3 例（6%）（P<0.01）存在抗 C1q 抗体。在 38 例 SLE 患者中有 13 例（34%）检测到抗 C1q 抗体。男性 SSc 患者中抗 C1q 抗体的检出率高于女性（P=0.005）；经多变量回归分析后仍存在这种关联。抗 C1q 抗体水平是预测肺纤维化存在的最重要因素，也是预测肺动脉高压的第二重要因素。14 例 SSc 患者（11.3%）存在 CICs。