Prognostic values of anti-Ro52 antibodies in anti-MDA5-positive clinically amyopathic dermatomyositis associated with interstitial lung disease
Prognostic values of anti-Ro52 antibodies in anti-MDA5-positive clinically amyopathic dermatomyositis associated with interstitial lung disease
复制标题
抗Ro52抗体在抗MDA5阳性临床无肌病性皮肌炎与间质性肺病相关的预后价值
DOI：
10.1093/rheumatology/keaa786
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发表时间：
2021-07-01
期刊：
影响因子：
5.5
通讯作者：
Bao, Chunde
中科院分区：
文献类型：
--
作者：
Xu, Antao;Ye, Yan;Bao, Chunde
目的：在抗合成酶综合征中，抗Ro52抗体常与抗Jo1抗体并存，且与临床表型越侵袭性越强，预后越差。抗Ro52抗体与抗黑色素瘤分化相关蛋白-5(抗MDA5)抗体在幼年性肌炎中有很强的相关性。本研究的目的是评估抗MDA5阳性的成人临床肌肉性皮肌炎合并间质性肺疾病(CADM-ILD)患者中抗Ro52抗体的临床意义。免疫印迹法检测抗MDA5抗体和抗Ro52抗体，并用光密度法进行半定量分析。结果：在抗MDA5阳性的CADM-ILD患者中，抗Ro52抗体的阳性率为74.7%，与快速进展性间质性肺疾病(54.8%vs23.8%；P=0.014)和皮肤溃疡(27.4%vs4.8%；P=0.033)相关。抗Ro52抗体阳性患者的累计24个月生存率低于阴性患者(59.9%vs85.7%；P=0.051)。抗Ro52抗体状态和抗MDA5抗体水平的结合进一步对患者的生存率进行分层，抗MDA5抗体和抗Ro52抗体双阳性患者的生存率显著低于单抗弱阳性患者(59.9%vs100%；P=0.019)。结论：抗Ro52抗体在抗MDA5阳性的慢性糖尿病-ILD患者中高度流行，其共存与更具侵袭性表型的患者亚群相关。联合检测抗MDA5抗体水平和抗Ro52抗体状态有助于预测患者预后，指导基于风险的治疗。
Objective: 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).Methods: 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.Results: 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 24month 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).Conclusion: 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.