Wang Gang, Yan Dong, Weng Chenghua, Xue Leixi, Liu Zhichun
Department of Rheumatology and Immunology, The Second Affiliated Hospital of Soochow University, Suzhou, Jiangsu, China.
Clin Rheumatol. 2025 Feb;44(2):767-774. doi: 10.1007/s10067-024-07298-4. Epub 2025 Jan 3.
This study aimed to evaluate the incidence and clinical significance of coexistence of anti-aminoacyl-tRNA synthetase (anti-ARS) antibody in patients with anti-melanoma differentiation-associated gene 5-positive dermatomyositis (anti-MDA5 + DM).
We assessed a cohort of 246 consecutive patients with anti-MDA5 + DM. Clinical characteristics and survival rates were compared between patients with and without anti-ARS antibodies.
Of these 246 patients, 15 (15/246, 6.1%) were positive for anti-ARS antibodies. The anti-ARS-positive group had a higher proportion of mechanic's hands (53.3% vs 25.5%, P = 0.019), and the positivity rates for antinuclear antibody (ANA) (80.0% vs 50.6%, P = 0.033) and anti-Ro52 antibodies (93.3% vs 62.3%, P = 0.013) were also higher. However, the CRP levels were comparatively low [4.0 (0.5, 7.8) vs 6.0 (3.1, 12.2), P = 0.019]. In the overall population, no difference in survival rates was observed between anti-MDA5 + DM patients with or without anti-ARS antibodies (log-rank P = 0.339). The multivariate Cox regression analysis revealed that elevated lactate dehydrogenase (LDH) levels and the presence of rapidly progressive interstitial lung disease (RPILD) were associated with poor prognosis [hazard ratios of 1.002 (95% CI 1.001, 1.002, P < 0.001) and 11.096 (95% CI 5.006, 24.598, P < 0.001), respectively].
Among the 246 patients with anti-MDA5 + DM, only 15 patients (6.1%) tested positive for anti-ARS antibodies. The presence of anti-ARS antibodies is associated with the occurrence of mechanic's hands, as well as an increased positive rate of ANA and anti-Ro52 antibodies. Elevated LDH levels and the presence of RPILD are poor prognostic risk factors for patients with anti-MDA5 + DM. Key Points • The presence of anti-ARS antibodies is associated with the occurrence of mechanic's hands, as well as an increased positive rate of ANA and anti-Ro52 antibodies. • Elevated LDH levels and the presence of RPILD are poor prognostic risk factors for patients with anti-MDA5 + DM. • Understanding the clinical characteristics of these patients will help clinicians to develop individualised treatment plans for each patient.
本研究旨在评估抗黑色素瘤分化相关基因5阳性皮肌炎（抗MDA5+DM）患者中抗氨酰tRNA合成酶（抗ARS）抗体共存的发生率及其临床意义。
我们评估了连续246例抗MDA5+DM患者的队列。比较了有和没有抗ARS抗体患者的临床特征和生存率。
在这246例患者中，15例（15/246，6.1%）抗ARS抗体呈阳性。抗ARS阳性组中技工手的比例更高（53.3%对25.5%，P=0.019），抗核抗体（ANA）（80.0%对50.6%，P=0.033）和抗Ro52抗体的阳性率（93.3%对62.3%，P=0.013）也更高。然而，CRP水平相对较低[4.0（0.5，7.8）对6.0（3.1，12.2），P=0.019]。在总体人群中，有或没有抗ARS抗体的抗MDA5+DM患者的生存率没有差异（对数秩检验P=0.339）。多因素Cox回归分析显示，乳酸脱氢酶（LDH）水平升高和快速进展性间质性肺病（RPILD）的存在与预后不良相关[风险比分别为1.002（95%CI 1.001，1.002，P<0.001）和11.096（95%CI 5.006，24.598，P<0.001）]。
在246例抗MDA5+DM患者中，只有15例（6.1%）抗ARS抗体检测呈阳性。抗ARS抗体的存在与技工手的发生以及ANA和抗Ro52抗体阳性率增加有关。LDH水平升高和RPILD的存在是抗MDA5+DM患者预后不良的危险因素。要点•抗ARS抗体的存在与技工手的发生以及ANA和抗Ro52抗体阳性率增加有关。•LDH水平升高和RPILD的存在是抗MDA5+DM患者预后不良的危险因素。•了解这些患者的临床特征将有助于临床医生为每位患者制定个体化的治疗方案。
