Department of Rheumatology, AP-HP, Paris-Sud University Hospitals, Le Kremlin-Bicêtre Hospital, Le Kremlin-Bicêtre, France.
INSERM U1184, Center for Immunology of Viral Infections and Autoimmune Diseases, Paris-Sud University, Le Kremlin-Bicêtre, France.
RMD Open. 2019 Oct 1;5(2):e001033. doi: 10.1136/rmdopen-2019-001033. eCollection 2019.
To describe and compare the clinical and biological characteristics of subjects with primary Sjögren's syndrome (pSS) with and without anti-RNP antibodies.
Patients fulfilling the American College of Rheumatology (ACR)/EULAR 2016 criteria for pSS and having anti-RNP antibodies, without other connective tissue disease diagnosed and no anti-dsDNA antibodies were retrieved from the database from our French National Reference Center. These patients were compared with all other patients with pSS with negative anti-Sm, anti-RNP and anti-dsDNA antibodies.
Overall, 21 patients with pSS positive for anti-RNP antibodies and 446 negative for anti-RNP antibodies were retrieved. Anti-RNP-positive patients had a lower median age at onset of pSS symptoms (41.0 vs 50.0 years, p=0.01), a higher median EULAR Sjögren's syndrome disease activity index at inclusion (8.0 vs 3.0, p<0.01), more frequently constitutional symptoms (14.3% vs 0.01%, p<0.01), myositis (19.0% vs 2.3%, p<0.01) and pulmonary (19.0% vs 5.7%, p=0.04) involvement. Moreover, anti-RNP-positive patients had higher median gammaglobulin levels (22.5 vs 13 g/L, p<0.01), more frequently anti-SSA antibodies (90.5% vs 67.1%, p=0.03), but less frequent lymphocytic sialadenitis with a focus score ≥1 (66.7% vs 85.5%, p=0.03). If the analysis is restricted to anti-SSA-positive patients, anti-RNP positivity is associated with the same clinicobiologic features except the pulmonary involvement.
Patients with pSS with anti-RNP antibodies displayed a more active systemic disease, with more frequent muscular and pulmonary involvement, and increased gammaglobulin level, compared with anti-RNP-negative patients.
描述并比较原发性干燥综合征(pSS)患者中存在和不存在抗 RNP 抗体的临床和生物学特征。
从我们的法国国家参考中心的数据库中检索出符合美国风湿病学会(ACR)/欧洲抗风湿病联盟(EULAR)2016 年 pSS 标准且存在抗 RNP 抗体、无其他结缔组织病诊断且无抗 dsDNA 抗体的患者。将这些患者与所有其他抗 Sm、抗 RNP 和抗 dsDNA 抗体均为阴性的 pSS 患者进行比较。
共检索到 21 例抗 RNP 抗体阳性的 pSS 患者和 446 例抗 RNP 抗体阴性的 pSS 患者。抗 RNP 阳性患者的 pSS 症状发病年龄中位数较低(41.0 岁 vs 50.0 岁,p=0.01),纳入时 EULAR 干燥综合征疾病活动指数中位数较高(8.0 分 vs 3.0 分,p<0.01),更常出现全身症状(14.3% vs 0.01%,p<0.01)、肌炎(19.0% vs 2.3%,p<0.01)和肺部受累(19.0% vs 5.7%,p=0.04)。此外,抗 RNP 阳性患者的γ球蛋白水平中位数较高(22.5 克/升 vs 13 克/升,p<0.01),更常出现抗 SSA 抗体(90.5% vs 67.1%,p=0.03),但较少出现焦点评分≥1 的淋巴细胞性涎腺炎(66.7% vs 85.5%,p=0.03)。如果分析仅限于抗 SSA 阳性患者,抗 RNP 阳性与除肺部受累以外的相同临床和生物学特征相关。
与抗 RNP 阴性患者相比,抗 RNP 抗体阳性的 pSS 患者表现出更活跃的系统性疾病,更常出现肌肉和肺部受累,并伴有更高的γ球蛋白水平。