An Autopsy Case of Anti-melanoma Differentiation-associated Gene-5 Antibody-positive Clinical Amyopathic Dermatomyositis Complicated by 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.
Department of Medicine, Division of Respirology, Neurology, and Rheumatology, Kurume University School of Medicine, Japan.
Naomi
N
Yoshida
Shinjiro
S
Kaieda
Kumi
K
Tomozoe
Morihiro
M
Tajiri
Daisuke
D
Wakasugi
Masaki
M
Okamoto
Masaki
M
Tominaga
Hiroaki
H
Ida
Tomoaki
T
Hoshino
D000888:Antibodies, Anti-Idiotypic / Q000097:blood*; D001344:Autopsy; D003882:Dermatomyositis / Q000150:complications* / Q000473:pathology*; D006801:Humans; D007564:Japan; D017563:Lung Diseases, Interstitial / Q000150:complications* / Q000473:pathology*; D008297:Male; D008545:Melanoma / Q000150:complications* / Q000473:pathology*; D008875:Middle Aged
D000888:Antibodies, Anti-Idiotypic