Recurrence in long-term survivor of anti-MDA5 antibody-positive clinically amyopathic dermatomyositis: case series and literature review

• Published in: Modern rheumatology case reports Vol. 5; no. 2; pp. 1 - 316
• Main Authors: Suzuki, Satoshi, Ikeda, Keigo, Yamaji, Ken, Tamura, Naoto, Morimoto, Shinji
• Format: Journal Article
• Language: English
• Published: England 03.04.2021
• Subjects: Anti-MDA5 antibody; cyclophosphamide; dermatomyositis; interstitial lung disease; apheresis
• DOI: 10.1080/24725625.2021.1886666
• ISSN: 2472-5625

Anti-MDA5 antibody-positive clinically amyopathic dermatomyositis (CADM) is often complicated by rapidly progressive interstitial lung disease and is associated with poor prognosis. However, even though recurrence is reported to be infrequent if successful medical treatment is administered, the long-term prognosis remains unclear. In this case report, we examined the clinical features and treatment details of three patients with anti-MDA5 antibody-positive CADM with multiple recurrences during long-term survival at Juntendo University Urayasu Hospital. Of the three patients, two failed to convert to an anti-MDA5 antibody-negative status, and one patient died. One of the remaining patients experienced two relapses but eventually tested negative for anti-MDA5 antibodies and showed a relatively stable clinical course. Although cases of recurring anti-MDA5 antibody-positive CADM rarely occur, they may occasionally be fatal. The prognosis for anti-MDA5 antibody-positive CADM has improved over time owing to its establishment as a disease. However, further information and research is necessary to ascertain its long-term prognosis.