Rheumatology Department, Bach Mai Hospital, Hanoi, Vietnam.
Internal Medicine Department, Hanoi Medical University, Hanoi, Vietnam.
Int J Rheum Dis. 2021 May;24(5):663-670. doi: 10.1111/1756-185X.14105. Epub 2021 Mar 31.
To assess clinical phenotypes of anti-aminoacyl-transfer RNA synthetases (aaRS) autoantibodies in Vietnamese patients of Kinh ethnicity with idiopathic inflammatory myopathies (IIM).
In a cross-sectional study 23 patients with anti-aaRS autoantibodies were compared to 36 patients with other myositis-specific antibodies and to 69 seronegative patients with IIM. Assessments included muscle performance, extra-muscular involvement, and disease activity according to the International Myositis Assessment and Clinical Studies (IMACS). Sera were tested by a line immunoassay (Euroline Myositis Profile 4).
The frequency of anti-Jo-1 antibodies was 56.5%, anti-EJ antibodies 26.1%, and anti-PL-7 antibodies 17.4%, while anti-PL-12 and anti-OJ antibodies were not present in any case. All patients with anti-aaRS autoantibodies had signs of myositis. At time of investigation 22/23 patients had muscle weakness, 52.2% arthritis, 34.8% Raynaud's phenomenon, 73.9% fever, 14.3% mechanic's hands and 56.5% dysphagia. Interstitial lung disease was present in 52.2%, and pulmonary hypertension in 56.5%. The anti-aaRS autoantibody positive group had higher disease activity in the domains of skin and pulmonary disease compared to the seronegative group and had lower disease activity in skeletal disease compared to the anti-melanoma differentiation-associated protein 5-positive patients. The clinical presentation of antisynthetase syndrome was similar between the aaRS autoantibody specificities with the exception of more frequent pulmonary hypertension in anti-Jo-1 positive patients.
Different aaRS autoantibody specificities may vary between different ethnic populations for reasons that still need to be clarified. Furthermore, the high frequency of pulmonary hypertension is noteworthy but otherwise clinical manifestations associated with aaRS autoantibodies did not differ from other ethnic populations.
评估越南京族特发性炎性肌病（IIM）患者抗氨酰基-tRNA 合成酶（aaRS）自身抗体的临床表型。
在一项横断面研究中，将 23 例抗 aaRS 自身抗体阳性患者与 36 例其他肌炎特异性抗体阳性患者和 69 例肌炎抗体阴性患者进行比较。评估内容包括肌肉功能、肌肉外表现和疾病活动度，采用国际肌炎评估和临床研究（IMACS）标准进行评估。血清通过线免疫分析法（Euroline Myositis Profile 4）进行检测。
抗-Jo-1 抗体的频率为 56.5%，抗-EJ 抗体为 26.1%，抗-PL-7 抗体为 17.4%，而抗-PL-12 和抗-OJ 抗体在任何情况下均未出现。所有抗 aaRS 自身抗体阳性患者均有肌炎表现。研究时，22/23 例患者有肌肉无力，52.2%有关节炎，34.8%有雷诺现象，73.9%有发热，14.3%有技工手，56.5%有吞咽困难。52.2%有间质性肺病，56.5%有肺动脉高压。与肌炎抗体阴性组相比，抗 aaRS 自身抗体阳性组在皮肤和肺部疾病领域的疾病活动度更高，与抗黑色素瘤分化相关蛋白 5 阳性患者相比，在骨骼疾病领域的疾病活动度更低。除了抗-Jo-1 阳性患者更常见肺动脉高压外，不同抗合成酶综合征的临床特征在不同 aaRS 自身抗体特异性之间相似。
由于尚需阐明原因，不同 aaRS 自身抗体特异性在不同种族人群中可能存在差异。此外，肺动脉高压的高频率值得关注，但与 aaRS 自身抗体相关的其他临床表现与其他种族人群并无不同。
