, Volume 197, Issue 3, pp 277–284 | Cite as

Screening for Myositis Antibodies in Idiopathic Interstitial Lung Disease

  • Lee FidlerEmail author
  • Irena Doubelt
  • Sonja Kandel
  • Jolene H. Fisher
  • Shikha Mittoo
  • Shane Shapera



International guidelines recommend screening for connective tissue disease (CTD) with autoantibodies when evaluating patients with idiopathic interstitial lung disease (ILD). Idiopathic inflammatory myositis comprises of a subgroup of CTD diagnosed with myositis antibodies (MA), often presenting with ILD. Our aim was to evaluate the utility of MA screening in patients with idiopathic ILD.


A retrospective analysis was conducted on patients referred with idiopathic ILD to a tertiary centre ILD clinic who were screened for MA. Patients with known or suspected CTD were excluded. Descriptive statistics, univariate analysis and multivariable logistic regression were used to detect associations between MA and patient characteristics.


Of 360 patients, 165 met inclusion criteria and 44 (26.7%) were identified to have MA. Fourteen patients (8.5%) had a change in diagnosis as a result of MA screening. Multivariable logistic regression identified the presence of MA to be associated with current smoking [OR 6.87 (1.65–28.64), p = 0.008] and a diffusing capacity of < 70% predicted [OR 2.55 (1.09–5.97), p = 0.03]. In patients with a change in diagnosis due to MA screening, 3 (1.8%) underwent a surgical lung biopsy and 2 (1.2%) were previously treated with antifibrotic therapy.


Screening for MA in patients with idiopathic ILD can contribute to a change in patient diagnosis, and may prevent invasive testing and unproven use of antifibrotic therapy. These results support the addition of MA to CTD screening panels during the initial evaluation of idiopathic ILD.


Connective tissue disease Idiopathic inflammatory myositis Interstitial lung disease 



Antinuclear antibodies


Aminoacyl-tRNA synthetase antibodies


Antisynthetase syndrome


Anti-cyclic citrullinated peptide


Computed tomography


Connective tissue disease


Diffusing capacity of the lung for carbon monoxide


Forced vital capacity


Gastroesophageal reflux


Idiopathic inflammatory myositis


Interstitial lung disease


Idiopathic pulmonary fibrosis


Myositis-associated antibodies


Myositis antibodies


Myositis-specific antibodies


Non-specific interstitial pneumonia


Pulmonary function testing


Rheumatoid factor


Surgical lung biopsy


Anti-signal recognition particle


Usual interstitial pneumonia


Author Contributions

LF is the guarantor of this research, and takes responsibility for the integrity of this work. All authors contributed to manuscript revisions, and provided final approval of the version for publication. LF, SS and SM contributed to the study concept and design, statistical analysis, drafting of initial manuscript and study supervision. Other contributions: none.


Funding for this project was obtained from the Canadian Pulmonary Fibrosis Foundation.

Compliance with Ethical Standards

Conflict of interest

SS has participated in speaking engagements and advisory boards for Hoffman-LaRoche Canada, Boehringer-Ingelheim Canada and AstraZeneca Canada. He has participated in clinical trials research for Hoffman-LeRoche Canada, Boehringer-Ingelheim Canada, Prometic Pharmaceuticals, Gilead Pharmaceuticals and Sanofi-Aventis Canada. All others have no conflicts of interest to declare.

Ethical Approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed Consent

For this type of study, formal consent is not required.


  1. 1.
    Mittoo S, Gelber AC, Christopher-Stine L, Horton MR, Lechtzin N, Danoff SK (2009) Ascertainment of collagen vascular disease in patients presenting with interstitial lung disease. Respir Med 103:1152–1158CrossRefPubMedGoogle Scholar
  2. 2.
    Raghu G, Collard HR, Egan JJ, Martinez FJ, Behr J, Brown KK et al (2011) An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med 183:788–824CrossRefPubMedPubMedCentralGoogle Scholar
  3. 3.
    Raghu G, Remy-Jardin M, Myers JL, Richeldi L, Ryerson CJ, Lederer DJ et al (2018) Diagnosis of idiopathic pulmonary fibrosis. An official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med 198:e44–e68CrossRefPubMedGoogle Scholar
  4. 4.
    Idiopathic Pulmonary Fibrosis Clinical Research N, Raghu G, Anstrom KJ, King TE Jr, Lasky JA, Martinez FJ (2012) Prednisone, azathioprine, and N-acetylcysteine for pulmonary fibrosis. N Engl J Med 366: 1968–1977CrossRefGoogle Scholar
  5. 5.
    Watanabe K, Handa T, Tanizawa K, Hosono Y, Taguchi Y, Noma S et al (2011) Detection of antisynthetase syndrome in patients with idiopathic interstitial pneumonias. Respir Med 105:1238–1247CrossRefPubMedGoogle Scholar
  6. 6.
    Ghirardello A, Borella E, Beggio M, Franceschini F, Fredi M, Doria A (2014) Myositis autoantibodies and clinical phenotypyes. Auto Immun Highlights 5:69–75CrossRefPubMedPubMedCentralGoogle Scholar
  7. 7.
    Vrancken A, De Langhe E, Westhovens R, Yserbyt J, Cottin V, Wuyts W (2015) Difficulties in diagnosis of systemic sclerosis-related interstitial lung disease. Respirol Case Rep 2015 3:99–101PubMedGoogle Scholar
  8. 8.
    Hallowell RW, Ascherman DP, Danoff SK (2014) Pulmonary manifestations of polymyositis/dermatomyositis. Semin Respir Crit Care Med 35:239–248CrossRefPubMedGoogle Scholar
  9. 9.
    Stone KB, Oddis CV, Fertig N, Katsumata Y, Lucas M, Vogt M et al (2007) Anti-Jo-1 antibody levels correlate with disease activity in idiopathic inflammatory myopathy. Arthritis Rheum 56:3125–3131CrossRefPubMedGoogle Scholar
  10. 10.
    Hunninghake GW, Lynch DA, Galvin JR, Gross BH, Muller N, Schwartz DA et al (2003) Radiologic findings are strongly associated with a pathologic diagnosis of usual interstitial pneumonia. Chest 124:1215–1223CrossRefPubMedGoogle Scholar
  11. 11.
    Hwang JH, Misumi S, Sahin H, Brown KK, Newell JD, Lynch DA (2009) Computed tomographic features of idiopathic fibrosing interstitial pneumonia: comparison with pulmonary fibrosis related to collagen vascular disease. J Comput Assist Tomogr 33:410–415CrossRefPubMedGoogle Scholar
  12. 12.
    Akira M (2017) Radiographic differentiation of advanced fibrocystic lung diseases. Ann Am Thorac Soc 14:432–440CrossRefPubMedGoogle Scholar
  13. 13.
    Aggarwal R, Dhillon N, Fertig N, Koontz D, Qi Z, Oddis CV (2017) A negative antinuclear antibody does not indicate autoantibody negativity in myositis: role of anticytoplasmic antibody as a screening test for antisynthetase syndrome. J Rheumatol 44:223–229CrossRefPubMedGoogle Scholar
  14. 14.
    Antoniou KM, Margaritopoulos GA, Goh NS, Karagiannis K, Desai SR, Nicholson AG et al (2016) Combined pulmonary fibrosis and emphysema in scleroderma-related lung disease has a major confounding effect on lung physiology and screening for pulmonary hypertension. Arthritis Rheumatol 68:1004–1012CrossRefPubMedGoogle Scholar
  15. 15.
    van Wesemael TJ, Ajeganova S, Humphreys J, Terao C, Muhammad A, Symmons DP et al (2016) Smoking is associated with the concurrent presence of multiple autoantibodies in rheumatoid arthritis rather than with anti-citrullinated protein antibodies per se: a multicenter cohort study. Arthritis Res Ther 18:285CrossRefPubMedPubMedCentralGoogle Scholar
  16. 16.
    Klareskog L, Stolt P, Lundberg K, Kallberg H, Bengtsson C, Grunewald J et al (2006) A new model for an etiology of rheumatoid arthritis: smoking may trigger HLA-DR (shared epitope)-restricted immune reactions to autoantigens modified by citrullination. Arthritis Rheum 54:38–46CrossRefGoogle Scholar
  17. 17.
    Chinoy H, Adimulam S, Marriage F, New P, Vincze M, Zilahi E et al (2012) Interaction of HLA-DRB1*03 and smoking for the development of anti-Jo-1 antibodies in adult idiopathic inflammatory myopathies: a European-wide case study. Ann Rheum Dis 71:961–965CrossRefPubMedGoogle Scholar
  18. 18.
    Lilleker JB, Vencovsky J, Wang G, Wedderburn LR, Diederichsen LP, Schmidt J et al (2018) The EuroMyositis registry: an international collaborative tool to facilitate myositis research. Ann Rheum Dis 77:30–39CrossRefPubMedGoogle Scholar
  19. 19.
    Hutchinson JP, Fogarty AW, McKeever TM, Hubbard RB (2016) In-hospital mortality after surgical lung biopsy for interstitial lung disease in the United States. 2000 to 2011. Am J Respir Crit Care Med 193:1161–1167CrossRefPubMedGoogle Scholar
  20. 20.
    De Sadeleer LJ, De Langhe E, Bodart N, Vigneron A, Bossuyt X, Wuyts WA (2018) Prevalence of myositis-specific antibodies in idiopathic interstitial pneumonias. Lung 196:329–333CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of Medicine, Division of Respirology, University Health NetworkUniversity of TorontoTorontoCanada
  2. 2.Department of MedicineUniversity of TorontoTorontoCanada
  3. 3.Department of Medicine, Department of RadiologyUniversity Health NetworkTorontoCanada
  4. 4.Division of RheumatologyUniversity Health NetworkTorontoCanada

Personalised recommendations