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Therapeutic plasma exchange for clinically amyopathic dermatomyositis ( CADM ) associated with rapidly progressive interstitial pneumonia
Author(s) -
Yamagata Akira,
Arita Machiko,
Tanaka Ayaka,
Tokioka Fumiaki,
Yoshida Tomohiro,
Nishimura Keisuke,
Ishida Tadashi
Publication year - 2020
Publication title -
journal of clinical apheresis
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.697
H-Index - 46
eISSN - 1098-1101
pISSN - 0733-2459
DOI - 10.1002/jca.21824
Subject(s) - medicine , dermatomyositis , refractory (planetary science) , interstitial pneumonia , therapeutic effect , gastroenterology , lung , physics , astrobiology
Background Patients with clinically amyopathic dermatomyositis (CADM) with anti‐melanoma differentiation‐associated gene 5 antibody (anti‐MDA5 Ab) frequently develop rapidly progressive interstitial pneumonia (RPIP), often with fatal outcomes. Therapeutic plasma exchange (TPE) has been reported as effective against CADM‐RPIP refractory to conventional immunosuppressive therapy. However, the detailed mechanisms by which TPE improves disease activity of CADM‐RPIP remain unclear. Aim To elucidate the clinical and demographic characteristics of patients with anti‐MDA5 Ab‐positive CADM‐RPIP treated with TPE and to analyze changes in laboratory findings before, during, and after TPE. Materials & Methods Patients hospitalized for CADM‐RPIP and treated with TPE in 2017 and 2018 were analyzed retrospectively. Results Three patients were successfully treated with TPE, with good tolerance. Anti‐MDA5 Ab titers decreased significantly over the course of TPE. Conclusion We emphasize that TPE could represent an effective treatment option for CADM‐RPIP refractory to traditional therapy. Removal of anti‐MDA5 Ab and other pathogenic factors may facilitate favorable outcomes.