Official reprint from UpToDate®
www.uptodate.com ©2017 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

Interstitial lung disease in dermatomyositis and polymyositis: Clinical manifestations and diagnosis

Paul F Dellaripa, MD
Marc L Miller, MD
Section Editors
Ira N Targoff, MD
Kevin R Flaherty, MD, MS
Deputy Editors
Helen Hollingsworth, MD
Monica Ramirez Curtis, MD, MPH


Dermatomyositis (DM) and polymyositis (PM) are classified as idiopathic inflammatory myopathies. Among patients with DM or PM, interstitial lung disease (ILD) is a major cause of morbidity and mortality. A general approach to the evaluation of ILD is provided separately. (See "Approach to the adult with interstitial lung disease: Clinical evaluation" and "Approach to the adult with interstitial lung disease: Diagnostic testing".)

In patients with DM or PM, ILD must be distinguished from other causes of pulmonary disease:


Drug-induced pneumonitis


To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:

Subscribers log in here

Literature review current through: Sep 2017. | This topic last updated: Dec 19, 2016.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2017 UpToDate, Inc.
  1. Marie I, Hachulla E, Chérin P, et al. Interstitial lung disease in polymyositis and dermatomyositis. Arthritis Rheum 2002; 47:614.
  2. Love LA, Leff RL, Fraser DD, et al. A new approach to the classification of idiopathic inflammatory myopathy: myositis-specific autoantibodies define useful homogeneous patient groups. Medicine (Baltimore) 1991; 70:360.
  3. Fathi M, Vikgren J, Boijsen M, et al. Interstitial lung disease in polymyositis and dermatomyositis: longitudinal evaluation by pulmonary function and radiology. Arthritis Rheum 2008; 59:677.
  4. Connors GR, Christopher-Stine L, Oddis CV, Danoff SK. Interstitial lung disease associated with the idiopathic inflammatory myopathies: what progress has been made in the past 35 years? Chest 2010; 138:1464.
  5. Dickey BF, Myers AR. Pulmonary disease in polymyositis/dermatomyositis. Semin Arthritis Rheum 1984; 14:60.
  6. Bohan A, Peter JB, Bowman RL, Pearson CM. Computer-assisted analysis of 153 patients with polymyositis and dermatomyositis. Medicine (Baltimore) 1977; 56:255.
  7. Frazier AR, Miller RD. Interstitial pneumonitis in association with polymyositis and dermatomyositis. Chest 1974; 65:403.
  8. Salmeron G, Greenberg SD, Lidsky MD. Polymyositis and diffuse interstitial lung disease. A review of the pulmonary histopathologic findings. Arch Intern Med 1981; 141:1005.
  9. Hochberg MC, Feldman D, Stevens MB. Adult onset polymyositis/dermatomyositis: an analysis of clinical and laboratory features and survival in 76 patients with a review of the literature. Semin Arthritis Rheum 1986; 15:168.
  10. Tymms KE, Webb J. Dermatopolymyositis and other connective tissue diseases: a review of 105 cases. J Rheumatol 1985; 12:1140.
  11. Nambu Y, Mouri M, Toga H, et al. Gender and underlying diseases affect the frequency of the concurrence of adult polymyositis/dermatomyositis and interstitial pneumonia. Chest 1994; 106:1931.
  12. Ikeda S, Arita M, Misaki K, et al. Incidence and impact of interstitial lung disease and malignancy in patients with polymyositis, dermatomyositis, and clinically amyopathic dermatomyositis: a retrospective cohort study. Springerplus 2015; 4:240.
  13. Richards TJ, Eggebeen A, Gibson K, et al. Characterization and peripheral blood biomarker assessment of anti-Jo-1 antibody-positive interstitial lung disease. Arthritis Rheum 2009; 60:2183.
  14. Chen YJ, Wu CY, Shen JL. Predicting factors of malignancy in dermatomyositis and polymyositis: a case-control study. Br J Dermatol 2001; 144:825.
  15. Douglas WW, Tazelaar HD, Hartman TE, et al. Polymyositis-dermatomyositis-associated interstitial lung disease. Am J Respir Crit Care Med 2001; 164:1182.
  16. Tazelaar HD, Viggiano RW, Pickersgill J, Colby TV. Interstitial lung disease in polymyositis and dermatomyositis. Clinical features and prognosis as correlated with histologic findings. Am Rev Respir Dis 1990; 141:727.
  17. Bradley B, Branley HM, Egan JJ, et al. Interstitial lung disease guideline: the British Thoracic Society in collaboration with the Thoracic Society of Australia and New Zealand and the Irish Thoracic Society. Thorax 2008; 63 Suppl 5:v1.
  18. Cottin V, Thivolet-Béjui F, Reynaud-Gaubert M, et al. Interstitial lung disease in amyopathic dermatomyositis, dermatomyositis and polymyositis. Eur Respir J 2003; 22:245.
  19. Koreeda Y, Higashimoto I, Yamamoto M, et al. Clinical and pathological findings of interstitial lung disease patients with anti-aminoacyl-tRNA synthetase autoantibodies. Intern Med 2010; 49:361.
  20. Tillie-Leblond I, Wislez M, Valeyre D, et al. Interstitial lung disease and anti-Jo-1 antibodies: difference between acute and gradual onset. Thorax 2008; 63:53.
  21. Fathi M, Dastmalchi M, Rasmussen E, et al. Interstitial lung disease, a common manifestation of newly diagnosed polymyositis and dermatomyositis. Ann Rheum Dis 2004; 63:297.
  22. Sato S, Hoshino K, Satoh T, et al. RNA helicase encoded by melanoma differentiation-associated gene 5 is a major autoantigen in patients with clinically amyopathic dermatomyositis: Association with rapidly progressive interstitial lung disease. Arthritis Rheum 2009; 60:2193.
  23. Schwarz MI, Matthay RA, Sahn SA, et al. Interstitial lung disease in polymyositis and dermatomyositis: analysis of six cases and review of the literature. Medicine (Baltimore) 1976; 55:89.
  24. Arsura EL, Greenberg AS. Adverse impact of interstitial pulmonary fibrosis on prognosis in polymyositis and dermatomyositis. Semin Arthritis Rheum 1988; 18:29.
  25. Mukae H, Ishimoto H, Sakamoto N, et al. Clinical differences between interstitial lung disease associated with clinically amyopathic dermatomyositis and classic dermatomyositis. Chest 2009; 136:1341.
  26. Lakhanpal S, Lie JT, Conn DL, Martin WJ 2nd. Pulmonary disease in polymyositis/dermatomyositis: a clinicopathological analysis of 65 autopsy cases. Ann Rheum Dis 1987; 46:23.
  27. Padley SP, Hansell DM, Flower CD, Jennings P. Comparative accuracy of high resolution computed tomography and chest radiography in the diagnosis of chronic diffuse infiltrative lung disease. Clin Radiol 1991; 44:222.
  28. Kalluri M, Sahn SA, Oddis CV, et al. Clinical profile of anti-PL-12 autoantibody. Cohort study and review of the literature. Chest 2009; 135:1550.
  29. Gil B, Merav L, Pnina L, Chagai G. Diagnosis and treatment of clinically amyopathic dermatomyositis (CADM): a case series and literature review. Clin Rheumatol 2016; 35:2125.
  30. Chen Z, Cao M, Plana MN, et al. Utility of anti-melanoma differentiation-associated gene 5 antibody measurement in identifying patients with dermatomyositis and a high risk for developing rapidly progressive interstitial lung disease: a review of the literature and a meta-analysis. Arthritis Care Res (Hoboken) 2013; 65:1316.
  31. Ceribelli A, Fredi M, Taraborelli M, et al. Prevalence and clinical significance of anti-MDA5 antibodies in European patients with polymyositis/dermatomyositis. Clin Exp Rheumatol 2014; 32:891.
  32. Moghadam-Kia S, Oddis CV, Sato S, et al. Anti-Melanoma Differentiation-Associated Gene 5 Is Associated With Rapidly Progressive Lung Disease and Poor Survival in US Patients With Amyopathic and Myopathic Dermatomyositis. Arthritis Care Res (Hoboken) 2016; 68:689.
  33. Dalakas MC. Inflammatory muscle diseases. N Engl J Med 2015; 372:1734.
  34. Ghirardello A, Borella E, Beggio M, et al. Myositis autoantibodies and clinical phenotypes. Auto Immun Highlights 2014; 5:69.
  35. Tomonaga M, Sakamoto N, Ishimatsu Y, et al. Comparison of pulmonary involvement between patients expressing anti-PL-7 and anti-Jo-1 antibodies. Lung 2015; 193:79.
  36. Marie I, Josse S, Decaux O, et al. Comparison of long-term outcome between anti-Jo1- and anti-PL7/PL12 positive patients with antisynthetase syndrome. Autoimmun Rev 2012; 11:739.
  37. Labirua-Iturburu A, Selva-O'Callaghan A, Vincze M, et al. Anti-PL-7 (anti-threonyl-tRNA synthetase) antisynthetase syndrome: clinical manifestations in a series of patients from a European multicenter study (EUMYONET) and review of the literature. Medicine (Baltimore) 2012; 91:206.
  38. Marie I, Josse S, Hatron PY, et al. Interstitial lung disease in anti-Jo-1 patients with antisynthetase syndrome. Arthritis Care Res (Hoboken) 2013; 65:800.
  39. Hall JC, Casciola-Rosen L, Samedy LA, et al. Anti-melanoma differentiation-associated protein 5-associated dermatomyositis: expanding the clinical spectrum. Arthritis Care Res (Hoboken) 2013; 65:1307.
  40. Nakajima H, Harigai M, Hara M, et al. KL-6 as a novel serum marker for interstitial pneumonia associated with collagen diseases. J Rheumatol 2000; 27:1164.
  41. Chinoy H, Salway F, Fertig N, et al. In adult onset myositis, the presence of interstitial lung disease and myositis specific/associated antibodies are governed by HLA class II haplotype, rather than by myositis subtype. Arthritis Res Ther 2006; 8:R13.
  42. Kumánovics G, Minier T, Radics J, et al. Comprehensive investigation of novel serum markers of pulmonary fibrosis associated with systemic sclerosis and dermato/polymyositis. Clin Exp Rheumatol 2008; 26:414.
  43. Gono T, Kawaguchi Y, Hara M, et al. Increased ferritin predicts development and severity of acute interstitial lung disease as a complication of dermatomyositis. Rheumatology (Oxford) 2010; 49:1354.
  44. Wells AU, du Bois RM. Bronchiolitis in association with connective tissue disorders. Clin Chest Med 1993; 14:655.
  45. Mino M, Noma S, Taguchi Y, et al. Pulmonary involvement in polymyositis and dermatomyositis: sequential evaluation with CT. AJR Am J Roentgenol 1997; 169:83.
  46. Akira M, Hara H, Sakatani M. Interstitial lung disease in association with polymyositis-dermatomyositis: long-term follow-up CT evaluation in seven patients. Radiology 1999; 210:333.
  47. Arakawa H, Yamada H, Kurihara Y, et al. Nonspecific interstitial pneumonia associated with polymyositis and dermatomyositis: serial high-resolution CT findings and functional correlation. Chest 2003; 123:1096.
  48. Morganroth PA, Kreider ME, Okawa J, et al. Interstitial lung disease in classic and skin-predominant dermatomyositis: a retrospective study with screening recommendations. Arch Dermatol 2010; 146:729.
  49. Braun NM, Arora NS, Rochester DF. Respiratory muscle and pulmonary function in polymyositis and other proximal myopathies. Thorax 1983; 38:616.
  50. Astudillo LM, Carreiro M, Sailler L, et al. Hypercapnic coma due to diaphragmatic involvement in a patient with dermatomyositis. Clin Exp Rheumatol 2001; 19:456.
  51. Oh TH, Brumfield KA, Hoskin TL, et al. Dysphagia in inflammatory myopathy: clinical characteristics, treatment strategies, and outcome in 62 patients. Mayo Clin Proc 2007; 82:441.
  52. Viguier M, Fouéré S, de la Salmonière P, et al. Peripheral blood lymphocyte subset counts in patients with dermatomyositis: clinical correlations and changes following therapy. Medicine (Baltimore) 2003; 82:82.
  53. Sostman HD, Matthay RA, Putman CE, Smith GJ. Methotrexate-induced pneumonitis. Medicine (Baltimore) 1976; 55:371.
  54. Marie I, Hachulla E, Chérin P, et al. Opportunistic infections in polymyositis and dermatomyositis. Arthritis Rheum 2005; 53:155.
  55. Kadoya A, Okada J, Iikuni Y, Kondo H. Risk factors for Pneumocystis carinii pneumonia in patients with polymyositis/dermatomyositis or systemic lupus erythematosus. J Rheumatol 1996; 23:1186.
  56. Do-Pham G, Pagès C, Picard C, et al. A first case report of a patient with paraneoplastic dermatomyositis developing diffuse alveolar haemorrhage. Br J Dermatol 2010; 163:227.
  57. Schwarz MI, Sutarik JM, Nick JA, et al. Pulmonary capillaritis and diffuse alveolar hemorrhage. A primary manifestation of polymyositis. Am J Respir Crit Care Med 1995; 151:2037.
  58. Sato K, Morozumi S, Takeuchi Y, et al. [Case of dermatomyositis with anti-CADM-140 antibody and alveolar hemorrhage]. Rinsho Shinkeigaku 2014; 54:408.
  59. Le Goff B, Chérin P, Cantagrel A, et al. Pneumomediastinum in interstitial lung disease associated with dermatomyositis and polymyositis. Arthritis Rheum 2009; 61:108.
  60. Kono H, Inokuma S, Nakayama H, Suzuki M. Pneumomediastinum in dermatomyositis: association with cutaneous vasculopathy. Ann Rheum Dis 2000; 59:372.
  61. Caceres M, Ali SZ, Braud R, et al. Spontaneous pneumomediastinum: a comparative study and review of the literature. Ann Thorac Surg 2008; 86:962.
  62. Teixeira A, Cherin P, Demoule A, et al. Diaphragmatic dysfunction in patients with idiopathic inflammatory myopathies. Neuromuscul Disord 2005; 15:32.
  63. Selva-O'Callaghan A, Labrador-Horrillo M, Muñoz-Gall X, et al. Polymyositis/dermatomyositis-associated lung disease: analysis of a series of 81 patients. Lupus 2005; 14:534.
  64. Carruthers EC, Choi HK, Sayre EC, Aviña-Zubieta JA. Risk of deep venous thrombosis and pulmonary embolism in individuals with polymyositis and dermatomyositis: a general population-based study. Ann Rheum Dis 2016; 75:110.
  65. Chung WS, Lin CL, Sung FC, et al. Increased risk of venous thromboembolism in patients with dermatomyositis/polymyositis: a nationwide cohort study. Thromb Res 2014; 134:622.