title: Comprehensive assessment of myositis-specific autoantibodies in polymyositis/dermatomyositis-associated interstitial lung disease.
abstract: Myositis-specific autoantibodies (MSAs) are associated with clinical phenotypes in polymyositis/dermatomyositis (PM/DM). No study has investigated the clinical features based on comprehensive MSA assessment in PM/DM-associated interstitial lung disease (ILD). We aimed to determine the practical significance of MSAs in PM/DM-ILD.
  
  
  
  Sixty consecutive PM/DM-ILD patients were retrospectively analysed. Serum MSAs were comprehensively measured using immunoprecipitation assay. Clinical features and prognosis were compared among MSA subgroups.
  
  
  
  Twenty-six (43.3%) PM/DM-ILD patients were anti-aminoacyl tRNA-synthetase antibody-positive (anti-ARS-positive), 15 (25.0%) were anti-melanoma differentiation-associated gene 5 antibody-positive (anti-MDA5-positive), 3 (5%) were anti-signal recognition particle antibody-positive, 1 (1.7%) was anti-transcriptional intermediary factor 1-gamma antibody-positive, and 15 (25%) were MSA-negative. There were significant differences in clinical features, including ILD form, serum ferritin and surfactant protein-D levels at ILD diagnosis, and high-resolution CT pattern among the anti-ARS-positive, anti-MDA5-positive and MSA-negative groups. The anti-MDA5-positive group showed the lowest 90-day survival rate (66.7%, anti-MDA5-positive; 100%, anti-ARS-positive; 100%, MSA-negative; P < 0.01). The anti-ARS-positive group had the highest 5-year survival rate (96%, anti-ARS-positive; 66.7%, anti-MDA5-positive; 68.3%, MSA-negative, P = 0.02). Univariate analysis revealed that anti-ARS antibody was associated with better prognosis (HR = 0.45; 95% CI, 0.18-0.89; P = 0.02), whereas anti-MDA5 antibody was associated with poorer prognosis (HR = 1.90; 95% CI, 1.02-3.39; P = 0.04).
  
  
  
  The comprehensive MSA assessment demonstrated that anti-ARS and anti-MDA5 antibodies were two major MSAs, and the clinical features differed depending on MSA status in PM/DM-ILD. Assessment of anti-ARS and anti-MDA5 antibodies is practically useful for predicting clinical course and prognosis in PM/DM-ILD patients.
authors.affiliation: Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama Higashiku, Hamamatsu, Shizuoka 431-3192, Japan. Electronic address: jubilooreoresagi@ybb.ne.jp.
authors.forename: Hironao
authors.initials: H
authors.lastname: Hozumi
authors.affiliation: Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama Higashiku, Hamamatsu, Shizuoka 431-3192, Japan.
authors.forename: Tomoyuki
authors.initials: T
authors.lastname: Fujisawa
authors.affiliation: Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, 54 Kawaharacho, Shogoin, Sakyo-ku, Kyoto 606-8507, Japan.
authors.forename: Ran
authors.initials: R
authors.lastname: Nakashima
authors.affiliation: Department of Radiology, Kinki Central Hospital of Mutual Aid Association of Public School Teachers, 3-1 Kurumazuka, Itami, Hyogo 664-8533, Japan.
authors.forename: Takeshi
authors.initials: T
authors.lastname: Johkoh
authors.affiliation: Department of Diagnostic Radiology, Osaka Medical Center for Cancer and Cardiovascular Diseases, 1 Chome-3-3 Nakamichi, Higashinari-ku, Osaka 537-0025, Japan.
authors.forename: Hiromitsu
authors.initials: H
authors.lastname: Sumikawa
authors.affiliation: Department of IVD Development, Medical & Biological Laboratories Co., Ltd., 1063-103 Terasawaoka, Ina, Nagano 396-0002, Japan.
authors.forename: Akihiro
authors.initials: A
authors.lastname: Murakami
authors.affiliation: Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama Higashiku, Hamamatsu, Shizuoka 431-3192, Japan.
authors.forename: Noriyuki
authors.initials: N
authors.lastname: Enomoto
authors.affiliation: Department of Clinical Pharmacology and Therapeutics, Hamamatsu University School of Medicine, 1-20-1 Handayama Higashiku, Hamamatsu, Shizuoka 431-3192, Japan.
authors.forename: Naoki
authors.initials: N
authors.lastname: Inui
authors.affiliation: Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama Higashiku, Hamamatsu, Shizuoka 431-3192, Japan.
authors.forename: Yutaro
authors.initials: Y
authors.lastname: Nakamura
authors.affiliation: Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, 54 Kawaharacho, Shogoin, Sakyo-ku, Kyoto 606-8507, Japan.
authors.forename: Yuji
authors.initials: Y
authors.lastname: Hosono
authors.affiliation: Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, 54 Kawaharacho, Shogoin, Sakyo-ku, Kyoto 606-8507, Japan.
authors.forename: Yoshitaka
authors.initials: Y
authors.lastname: Imura
authors.affiliation: Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, 54 Kawaharacho, Shogoin, Sakyo-ku, Kyoto 606-8507, Japan.
authors.forename: Tsuneyo
authors.initials: T
authors.lastname: Mimori
authors.affiliation: Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama Higashiku, Hamamatsu, Shizuoka 431-3192, Japan.
authors.forename: Takafumi
authors.initials: T
authors.lastname: Suda
mesh_terms: D000328:Adult; D000368:Aged; D000604:Amino Acyl-tRNA Synthetases / Q000276:immunology; D001323:Autoantibodies / Q000097:blood*; D015415:Biomarkers / Q000097:blood; D003882:Dermatomyositis / Q000150:complications / Q000276:immunology; D005260:Female; D006801:Humans; D000072640:Interferon-Induced Helicase, IFIH1 / Q000276:immunology; D017563:Lung Diseases, Interstitial / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000209:etiology / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D017285:Polymyositis / Q000150:complications / Q000276:immunology*; D011379:Prognosis; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed
chemical_list: D001323:Autoantibodies; D015415:Biomarkers; C452815:IFIH1 protein, human; D000072640:Interferon-Induced Helicase, IFIH1; D000604:Amino Acyl-tRNA Synthetases
keywords: ARS; Dermatomyositis; Interstitial pneumonia; MDA5; Myositis-specific autoantibody; Polymyositis