An Autopsy Case of Anti-melanoma Differentiation-associated Gene-5 Antibody-positive Clinical Amyopathic Dermatomyositis Complicated by Rapidly Progressive Interstitial Lung Disease

作者：Yoshida Naomi; Kaieda Shinjiro^*; Tomozoe Kumi; Tajiri Morihiro; Wakasugi Daisuke; Okamoto Masaki; Tominaga Masaki; Ida Hiroaki; Hoshino Tomoaki

来源：Internal Medicine, 2016, 55(12): 1653-1659.

DOI：10.2169/internalmedicine.55.6055

• anti-MDA-5 antibody
• clinical amyopathic dermatomyositis
• palmar papules
• rapidly progressive interstitial lung disease

A 62-year-old man presented with heliotrope rash, Gottron's sign, and mild muscle weakness. Both of his lung fields showed interstitial changes that worsened rapidly. He was diagnosed with clinical amyopathic dermatomyositis with rapidly progressive interstitial lung disease. The patient died of respiratory failure, despite the administration of immunosuppressive therapy. Autopsy revealed diffuse alveolar damage. An antibody analysis, which was performed postmortem, detected the presence of anti-melanoma differentiation-associated gene (MDA)-5 antibodies. Clinicians should note the clinical, radiologic, and serologic findings to predict anti-MDA-5 antibody-associated rapidly progressive interstitial lung disease.

• 出版日期2016