Current Rheumatology Reports

, Volume 14, Issue 3, pp 264–274

Interstitial Lung Disease in Myositis: Clinical Subsets, Biomarkers, and Treatment


DOI: 10.1007/s11926-012-0246-6

Cite this article as:
Mimori, T., Nakashima, R. & Hosono, Y. Curr Rheumatol Rep (2012) 14: 264. doi:10.1007/s11926-012-0246-6


Interstitial lung disease (ILD) is the most frequent organ involvement (found in nearly half) of myositis patients, but it reveals various clinical courses and therapeutic responsiveness according to clinical and serological subsets. Autoantibodies, as well as imaging and histopathological studies, are useful for the classification of ILD in myositis and provide useful information for predicting prognosis and determining treatment. Antisynthetase antibodies are correlated with chronic and recurrent ILD, whereas anti-CADM-140 (MDA5/IFIH1) antibodies are a marker of acute progressive ILD in clinically amyopathic dermatomyositis. Serum KL-6, SP-D, and ferritin are useful biomarkers for monitoring the activity and severity of ILD. Regarding treatment, glucocorticoids are the first-line drug, but additional immunomodulating drugs are also used in refractory patients. Cyclophosphamide and calcineurin inhibitors (cyclosporine and tacrolimus) appear to be the key drugs in the treatment of refractory myositis–ILD. Rituximab may become another candidate if these drugs are not effective.


Idiopathic inflammatory myopathyMyositisBiomarkersPolymyositisDermatomyositisClinically amyopathic dermatomyositisInterstitial lung diseaseInterstitial pneumoniaNSIPAntisynthetase antibodyAnti-CADM-140 antibodyAminoacyl-transfer RNA synthetaseMDA5IFIH1HRCTKL-6SP-DFerritinGlucocorticoidsCyclophosphamideCyclosporineTacrolimusRituximabTreatment

Copyright information

© Springer Science+Business Media, LLC 2012

Authors and Affiliations

  1. 1.Department of Rheumatology and Clinical Immunology, Graduate School of MedicineKyoto UniversityKyotoJapan