Intapiboon Porntip, Siripaitoon Boonjing
Allergy and Rheumatology Unit, Division of Internal Medicine, Faculty of Medicine, Prince of Songkla University, HatYai, Thailand.
Respir Med Case Rep. 2020 Nov 19;31:101299. doi: 10.1016/j.rmcr.2020.101299. eCollection 2020.
INTRODUCTION/OBJECTIVES: The anti-melanoma differentiation-associated gene 5 (Anti-MDA5) antibody is associated with rapidly progressive interstitial lung disease (RP-ILD) and clinically amyopathic dermatomyositis (CADM). The predictors of treatment response would help in classifying the subgroups and decision-making. Here, we aimed to report an observational skin lesion that might be associated with a grave prognosis in six patients with anti-MDA5 antibody-positive ILD.
This case series included 6 anti-MDA5 antibody-positive ILD patients, who were admitted to Songklanagarind Hospital between January 2018 and June 2020. Their medical records were reviewed for clinical phenotypes, laboratory results, imaging studies, treatment, and outcomes. The cutaneous manifestations associated with fatal outcomes were observed and reported.
Among 6 patients with anti-MDA5 antibody-positive ILD, 5 patients had CADM and one patient had no skin involvement. Four patients manifested as RP-ILD within a few months. Three deaths occurred despite highly intensive immunosuppressive treatment. All the patients in the dead group exhibited erythematous papules on their auricles and a presence of pulmonary consolidation at lower lung fields was additionally observed.
Erythematous auricular papules may be a hallmark of grave prognosis in anti-MDA5 positive CADM with ILD.
引言/目的：抗黑色素瘤分化相关基因5（Anti-MDA5）抗体与快速进展性间质性肺病（RP-ILD）及临床无肌病性皮肌炎（CADM）相关。治疗反应的预测因素有助于对亚组进行分类及决策。在此，我们旨在报告一种观察到的皮肤病变，其可能与6例抗MDA5抗体阳性的间质性肺病患者的不良预后相关。
本病例系列纳入了6例抗MDA5抗体阳性的间质性肺病患者，他们于2018年1月至2020年6月期间入住宋卡纳加拉医院。对他们的病历进行回顾，以了解临床表型、实验室检查结果、影像学研究、治疗及结局。观察并报告与致命结局相关的皮肤表现。
在6例抗MDA5抗体阳性的间质性肺病患者中，5例患有CADM，1例无皮肤受累。4例在数月内表现为RP-ILD。尽管进行了高强度免疫抑制治疗，仍有3例死亡。死亡组的所有患者耳廓均出现红斑丘疹，且在下肺野还观察到肺部实变。
耳部红斑丘疹可能是抗MDA5阳性CADM合并间质性肺病不良预后的标志。
