Significance of Systemic Scleroderma-Specific Autoantibodies in Idiopathic Interstitial Pneumonia

Objective Patients with idiopathic interstitial pneumonia (IIP) often test positive for systemic scleroderma-specific autoantibodies (SSc-Ab), even if they do not meet the diagnostic criteria for systemic scleroderma (SSc). However, the significance of SSc-Ab in IIP is unknown. Methods We retrospectively studied the medical records of all patients suspected of interstitial lung disease (ILD) who visited our center between January 2016 and December 2021. We evaluated the association between SSc-Ab subtypes and clinical characteristics, prognosis, and incidence of acute exacerbation (AE) of IIP. Among 571 patients suspected of having IIP and SSc-Ab measured, we excluded cases with clear causes of ILD or those diagnosed with other diseases and analyzed 386 cases diagnosed as IIP. Results Among 386 IIP patients, 48 were SSc-Ab positive (platelet-derived growth factor receptor (PDGFR) in 0, Th/To in 10, anti-nucleolar organizer region 90 antibodies <i>(</i>NOR90) in 12, fibrillarin in five, RP155 in 14, RP11 in three, CENP A in seven, CENP B in 10, and Scl-70 in six). There was no significant difference in survival rate or incidence of AE between patients with or without SSc-Ab. Multivariate logistic regression analysis showed that age and malignancy were significant risk factors for death, whereas age, male sex, and anti-fibrillarin antibodies were significant risk factors for AE of IIP. Conclusion None of the SSc-Abs were associated with the risk of mortality, and anti-fibrillarin antibodies, along with age and male sex may contribute to the risk of AE of IIP, predicting severe lung involvement and warranting multidisciplinary treatment and careful follow-up.