Prevalence and clinical associations of myositis antibodies in a large cohort of interstitial lung diseases

Background Serologic testing for autoantibodies is recommended in interstitial lung diseases (ILDs), as connective tissue diseases (CTDs) are an important secondary cause. Myositis antibodies are associated with CTD-ILD, but clinical associations with other ILDs are unclear. In this study, associations of myositis antibodies in various ILDs were evaluated. Methods 1463 ILD patients and 116 healthy subjects were screened for myositis antibodies with a line-blot assay on serum available at time of diagnosis. Additionally, bronchoalveolar lavage fluid (BALf) was analysed. Results A total of 394 patients demonstrated reactivity to at least one antibody, including anti-Ro52 (36.0%), anti-Mi-2 beta (17.3%) and anti-Jo-1 (10.9%). Anti-Jo-1 (OR 6.4; p<0.100) and anti-Ro52 (OR 6.0; p<0.001) were associated with CTD-ILD. Interestingly, anti-Mi-2 beta was associated with idiopathic pulmonary fibrosis (IPF; OR 5.3; p = 0.001) and hypersensitivity pneumonitis (HP; OR 5.9; p<0.001). Furthermore, anti-Mi-2 beta was strongly associated with a histological usual interstitial pneumonia (UIP) pattern (OR 6.5; p < 0.001). Moreover, anti-Mi-2 beta reactivity was identified in BALf and correlated with serum anti-Mi-2 beta (r = 0.64; p = 0.002). No differences were found in survival rates between ILD patients with and without serum Mi-2 beta reactivity (hazard ratio 0.835; 95% CI 0.442-1.575; p = 0.577). Conclusion In conclusion, novel associations of antibody Mi-2 beta with fibrotic ILD were found. Furthermore, serum anti-Mi-2 beta was associated with a histological UIP pattern and presence of anti-Mi-2 beta in BALf. Possibly, anti-Mi-2 beta could be implemented as a future diagnostic biomarker for fibrotic ILD.