Screening for Myositis Antibodies in Idiopathic Interstitial Lung Disease



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.

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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


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

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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.

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Correspondence to Lee Fidler.

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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.

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Fidler, L., Doubelt, I., Kandel, S. et al. Screening for Myositis Antibodies in Idiopathic Interstitial Lung Disease. Lung 197, 277–284 (2019).

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  • Connective tissue disease
  • Idiopathic inflammatory myositis
  • Interstitial lung disease