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- Masami Yamazoe, Kazuya Takeda, Yutaro Nagano, Kanami Nagano, Koji Kato, Takashi Inoue, Kazuhiro Horiuchi, and Kazuro Kamada.
- Department of Respiratory Medicine, Hakodate Municipal Hospital, Japan.
- Intern. Med. 2024 Sep 15; 63 (18): 257125782571-2578.
AbstractA 36-year-old man with inverse Gottron's sign was admitted for clinically amyopathic dermatomyositis (CADM) with rapidly progressive interstitial lung disease (RP-ILD). Early addition of plasma exchange (PE) to triple therapy improved severe respiratory failure and transiently decreased serum ferritin levels and anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5 Ab) titers. Furthermore, switching from tacrolimus to tofacitinib resulted in disease remission. Recognition of the inverse Gottron's sign may allow for the earlier diagnosis of anti-MDA5 Ab-positive dermatomyositis, and early addition of PE to triple therapy and administration of tofacitinib in refractory cases may be effective for anti-MDA5 Ab-positive CADM with RP-ILD under life-threatening conditions.
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