title: Successful polymyxin B hemoperfusion treatment associated with serial reduction of serum anti-CADM-140/MDA5 antibody levels in rapidly progressive interstitial lung disease with amyopathic dermatomyositis.
abstract: Clinically amyopathic dermatomyositis (CADM), a subtype of dermatomyositis with subtle or no muscle involvement, is occasionally accompanied by fatal, rapidly progressive interstitial lung disease (RP-ILD) that is resistant to aggressive immunosuppressive therapy. The presence of anti-CADM-140/MDA5 antibodies is diagnostic for patients with dermatomyositis (particularly CADM) and is known to be strongly associated with the pathogenesis, disease activity, and mortality of RP-ILD. Polymyxin-B direct hemoperfusion (PMX-DHP), originally developed for the removal of endotoxin, has been demonstrated to be effective for treating various types of acute respiratory failure. We describe a patient with amyopathic dermatomyositis who developed RP-ILD characterized by elevated anti-CADM-140/MDA5 autoantibodies, was resistant to combined steroid and immunosuppressant therapy, and was treated successfully with PMX-DHP. To our knowledge, this is the first case to indicate a serial reduction of anti-CADM-140/MDA5 autoantibodies, associated with clinical improvement, following PMX-DHP. Early intervention using PMX-DHP may improve the prognosis of RP-ILD accompanied by CADM.
authors.affiliation: Division of Respiratory Medicine, Saiseikai Kumamoto Hospital, Chikami, Kumamoto, Japan. Electronic address: tanaka.aoi10003@gmail.com.
authors.forename: Aoi
authors.initials: A
authors.lastname: Teruya
authors.affiliation: Division of Respiratory Medicine, Saiseikai Kumamoto Hospital, Chikami, Kumamoto, Japan.
authors.forename: Kodai
authors.initials: K
authors.lastname: Kawamura
authors.affiliation: Division of Respiratory Medicine, Saiseikai Kumamoto Hospital, Chikami, Kumamoto, Japan.
authors.forename: Kazuya
authors.initials: K
authors.lastname: Ichikado
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, Tokai University School of Medicine, Isehara, Kanagawa, Japan.
authors.forename: Shinji
authors.initials: S
authors.lastname: Sato
authors.affiliation: Division of Respiratory Medicine, Saiseikai Kumamoto Hospital, Chikami, Kumamoto, Japan.
authors.forename: Yuko
authors.initials: Y
authors.lastname: Yasuda
authors.affiliation: Division of Respiratory Medicine, Saiseikai Kumamoto Hospital, Chikami, Kumamoto, Japan.
authors.forename: Masakazu
authors.initials: M
authors.lastname: Yoshioka
mesh_terms: D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D000906:Antibodies / Q000097:blood* / Q000276:immunology; D015897:Comorbidity; D053487:DEAD-box RNA Helicases / Q000276:immunology*; D003882:Dermatomyositis / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy*; D018450:Disease Progression; D006464:Hemoperfusion / Q000379:methods*; D006801:Humans; D036341:Intercellular Signaling Peptides and Proteins; D000072640:Interferon-Induced Helicase, IFIH1; D017563:Lung Diseases, Interstitial / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D010455:Peptides / Q000276:immunology*; D011112:Polymyxin B / Q000627:therapeutic use*; D011379:Prognosis; D016896:Treatment Outcome
chemical_list: D000900:Anti-Bacterial Agents; D000906:Antibodies; C501597:CADM-140 peptide, human; D036341:Intercellular Signaling Peptides and Proteins; D010455:Peptides; C452815:IFIH1 protein, human; D053487:DEAD-box RNA Helicases; D000072640:Interferon-Induced Helicase, IFIH1; D011112:Polymyxin B