Anti-MDA-5 antibody-positive clinically amyopathic dermatomyositis with rapidly progressive interstitial lung disease treated with therapeutic plasma exchange: A case series.
We present six cases of antimelanoma differentiation-associated gene 5 antibody (anti-MDA5-Ab)-positive clinically amyopathic dermatomyositis (CADM) with rapidly progressive interstitial lung disease (RP-ILD), which is known to have a poor prognosis. The outcomes of these cases are described after treatment with therapeutic plasma exchange (TPE). Clinical and therapeutic data for patients with CADM with RP-ILD were collected retrospectively from medical records. All six patients received early intensive care including high-dose corticosteroids, intravenous cyclophosphamide, and a calcineurin inhibitor, but lung disease and hypoxia became more severe. TPE was performed over a median of 9.5 sessions (range 3-14) per patient, and the median duration from admission to TPE was 23 days. Three patients received combined direct hemoperfusion using a polymyxin B-immobilized fiber column (PMX-DHP) therapy on successive days to manage acute respiratory failure. Four patients survived and two died due to respiratory failure. In the survival cases, ferritin decreased, and ferritin and KL-6 were lower at diagnosis. The patients who died had a higher alveolar-arterial oxygen difference and more severe lung lesions at the time of initiation of TPE. These findings indicate that a combination of conventional therapy and TPE may be useful for improvement of the prognosis of CADM with RP-ILD at the early stage of onset.
['Saito|Tomohiro|T|https://orcid.org/0000-0003-2303-5401', 'Mizobuchi|Masahide|M|', 'Miwa|Yusuke|Y|', 'Sugiyama|Motonori|M|', 'Mima|Yuuki|Y|', 'Iida|Ayana|A|', 'Kanazawa|Nobuhiro|N|https://orcid.org/0000-0001-6111-8027', 'Morikawa|Tomoki|T|', 'Hayashi|Junichi|J|', 'Fukuda|Kei|K|', 'Shikida|Yasuto|Y|', 'Suzuki|Taihei|T|', 'Honda|Hirokazu|H|']
D000368:Aged D001323:Autoantibodies D003882:Dermatomyositis D005260:Female D006801:Humans D000072640:Interferon-Induced Helicase, IFIH1 D017563:Lung Diseases, Interstitial D008297:Male D008875:Middle Aged D010951:Plasma Exchange
clinically amyopathic dermatomyositis interstitial lung disease therapeutic plasma exchange anti MDA-5 antibody