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Rheumatology (Oxford). 2018 Mar 27. doi: 10.1093/rheumatology/key060. [Epub ahead of print]

Initial predictors of poor survival in myositis-associated interstitial lung disease: a multicentre cohort of 497 patients.

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Division of Rheumatology, Department of Internal Medicine, Tokai University School of Medicine, Kanagawa.
Department of Anaesthesiology, National Defence Medical College School of Medicine, Saitama.
Department of Anesthesiology, Showa University School of Medicine.
Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine.
Institute of Rheumatology, Tokyo Women's Medical University, Tokyo.
Unit of Translational Medicine, Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki.
Department of Internal Medicine and Clinical Immunology, Yokohama City University Graduate School of Medicine, Yokohama.
Department of Allergy and Clinical Immunology, Chiba University Hospital, Chiba.
Department of Rheumatic Diseases, Tokyo Metropolitan Tama Medical Centre, Tokyo.
Department of Pulmonary Medicine, Fukushima Medical University School of Medicine, Fukushima.
Division of Respiratory Medicine, Niigata University Medical and Dental Hospital, Niigata.
Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo.
Department of Rheumatology, Saitama Medical Centre, Jichi Medical University, Saitama.
Division of Rheumatology, Department of Internal Medicine, The Jikei University School of Medicine, Tokyo.
Department of Medicine, Division of Respirology, Neurology, and Rheumatology, Kurume University School of Medicine, Fukuoka.
Division of Respiratory Medicine, Allergy and Rheumatology, Nagoya City University School of Medicine, Aichi, Japan.



To identify initial predictors of poor survival in patients with PM/DM-associated interstitial lung disease (ILD).


We established a multicentre retrospective cohort of incident cases of PM/DM-associated ILD from 44 institutions across Japan (Multicentre Retrospective Cohort of Japanese Patients with Myositis-associated ILD, JAMI). Inclusion criteria were an onset age ⩾16 years; PM/DM or clinically amyopathic DM according to the published criteria; imaging evidence of ILD; and availability of serum samples for assays of autoantibodies such as anti-melanoma differentiation-associated gene 5 and anti-aminoacyl tRNA synthetase. We collected demographic data and clinical characteristics recorded at the time of diagnosis, as well as follow-up survival data. Predictors of ILD-related mortality were identified by univariate and multivariate analyses.


JAMI enrolled a cohort of 497 patients with PM (15%), classic DM (32%) and clinically amyopathic DM (53%). During the observation period (median 20 months), 76 died of respiratory insufficiency directly related to ILD. Univariate analysis revealed several initial parameters associated with ILD mortality, including demographic, clinical, laboratory, imaging and autoantibody variables. We used multivariate analysis with a stepwise selection of parameters to generate an appropriate predictive model, and identified the following independent risk factors for ILD mortality: age at onset ⩾60 years [hazard ratio (HR) = 4.3, 95% CI: 2.4, 7.5], CRP ⩾1 mg/dl (HR = 2.6, 95% CI: 1.5, 4.8), peripheral capillary oxygen saturation <95% (HR = 2.0, 95% CI: 1.2, 3.4) and anti-melanoma differentiation-associated gene 5 antibody (HR = 7.5, 95% CI: 2.8, 20.2).


We established a large cohort of incident cases of PM/DM-associated ILD, and successfully identified independent predictors of short-term ILD mortality.

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