Department of Rheumatology, Renji Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China.
Rheumatology (Oxford). 2021 Jul 1;60(7):3343-3351. doi: 10.1093/rheumatology/keaa786.
Anti-Ro52 antibody often co-occurs with anti-Jo1 antibody in antisynthetase syndrome and their co-occurrence correlates with a more aggressive clinical phenotype and poorer prognosis. The strong association of anti-Ro52 antibody with anti-melanoma differentiation-associated protein-5 (anti-MDA5) antibody has been indicated in juvenile myositis. The aim of this study was to assess the clinical significance of anti-Ro52 antibody in a cohort of adult patients with anti-MDA5-positive clinically amyopathic dermatomyositis with interstitial lung disease (CADM-ILD).
We assessed a cohort of 83 consecutive patients with anti-MDA5-positive CADM-ILD. Anti-MDA5 antibodies and anti-Ro52 antibodies were detected in immunoblotting and semi-quantitatively analysed by densitometry. Clinical features and the 24 month survival were compared between anti-MDA5-positive patients with and without anti-Ro52 antibodies.
Anti-Ro52 antibodies were found in 74.7% of anti-MDA5-positive CADM-ILD patients and were associated with an increased frequency of rapidly progressive interstitial lung disease (RP-ILD; 54.8% vs 23.8%; P = 0.014) and cutaneous ulcerations (27.4% vs 4.8%; P = 0.033). The cumulative 24 month survival rate tended to be lower in patients with anti-Ro52 antibodies than patients without (59.9% vs 85.7%; P = 0.051). The combination of anti-Ro52 antibody status and anti-MDA5 antibody levels further stratified patients' survival rates, showing that the survival rate of patients who were dual positive for anti-MDA5 antibody and anti-Ro52 antibody was significantly lower than patients with mild positive anti-MDA5 antibody alone (59.9% vs 100%; P = 0.019).
Anti-Ro52 antibody is highly prevalent in anti-MDA5-positive CADM-ILD patients and their coexistence correlates with a subgroup of patients with more aggressive phenotypes. The combination of anti-MDA5 antibody levels and anti-Ro52 antibody status could help to predict patients' prognosis and guide risk-based therapy.
抗 Ro52 抗体常与抗 Jo1 抗体共同出现在抗合成酶综合征中，且其共同出现与更具侵袭性的临床表型和更差的预后相关。在青少年皮肌炎中，抗 Ro52 抗体与抗黑色素瘤分化相关蛋白 5（抗 MDA5）抗体的强相关性已被证实。本研究旨在评估抗 Ro52 抗体在一组抗 MDA5 阳性的、伴间质性肺病的临床无肌病性皮肌炎（CADM-ILD）成年患者中的临床意义。
我们评估了 83 例连续的抗 MDA5 阳性 CADM-ILD 患者。采用免疫印迹法检测抗 MDA5 抗体和抗 Ro52 抗体，并通过密度法进行半定量分析。比较抗 MDA5 阳性患者中有无抗 Ro52 抗体的临床特征和 24 个月生存率。
抗 Ro52 抗体在 74.7%的抗 MDA5 阳性 CADM-ILD 患者中被发现，与快速进展性间质性肺病（RP-ILD；54.8% vs. 23.8%；P=0.014）和皮肤溃疡（27.4% vs. 4.8%；P=0.033）的发生率增加相关。有抗 Ro52 抗体的患者 24 个月累积生存率低于无抗 Ro52 抗体的患者（59.9% vs. 85.7%；P=0.051）。抗 Ro52 抗体状态与抗 MDA5 抗体水平的联合进一步分层了患者的生存率，结果显示抗 MDA5 抗体和抗 Ro52 抗体双阳性患者的生存率明显低于单纯抗 MDA5 抗体轻度阳性患者（59.9% vs. 100%；P=0.019）。
抗 Ro52 抗体在抗 MDA5 阳性 CADM-ILD 患者中高度流行，其共存与具有更侵袭性表型的亚组患者相关。抗 MDA5 抗体水平与抗 Ro52 抗体状态的联合可帮助预测患者的预后并指导基于风险的治疗。
