医学
皮肌炎
间质性肺病
肌炎
内科学
皮疹
胃肠病学
高分辨率计算机断层扫描
自身抗体
抗体
结缔组织病
DLCO公司
多发性肌炎
肺
病理
自身免疫性疾病
免疫学
疾病
扩散能力
肺功能
作者
Fang Chen,Yu Zuo,Shanshan Li,Jingli Shi,Guochun Wang,Xi Shu
标识
DOI:10.1016/j.rmed.2019.07.020
摘要
AimTo describe and expand the phenotype of isolated anti-Ro-52-associated rapid progressive interstitial lung disease (RP-ILD) in Dermatomyositis(DM) in Chinese patients.Methods491 patients with PM/DM-ILD hospitalized in the China-Japan Friendship Hospital from 2000 to 2017 were screened retrospectively. All proven cases of isolated anti-Ro-52-associated RP-ILD were selected for inclusion. The clinical features in this group were recorded.ResultsIsolated Ro-52 antibodies existed in 20 PM/DM-ILD patients. Among them 5 patients developed RP-ILD. The 5 patients had typical rashes including Gottron's sign (80%), Helitrope rash (80%) and mechanic's hands (100%), but only few patients (20%) had arthralgia and muscle weakness. All patients had elevated levels of serum ferritin and decreased counts of CD3+ T cells. The estimated high-resolution computed tomography (HRCT) patterns of the five patients showed organizing pneumonia (OP) while RP-ILD patients without Ro-52 antibodies and non-RP-ILD patients with isolated Ro-52 antibodies mainly showed non-specific interstitial pneumonia (NSIP) patterns(P < 0.05). Although one patient died of infection after one month, 80%(4/5) of patients had good response to glucocorticoid treatment and these four patients survived were all alive at the end of follow-up. The survival rate in this group was the highest than those in RP-ILD patients with other myositis specific autoantibodies though the difference had no statistically significance.ConclusionsA small group of patients with isolated anti-Ro-52 antibody in DM could develop RP-ILD, which mainly presented OP on HRCT. Patients with isolated anti-Ro-52 antibody associated RP-ILD responded well to therapy and had good prognosis in DM.
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