Abstract

BackgroundInterstitial lung disease (SSc-ILD) and pulmonary hypertension are the leading causes of death in patients with systemic sclerosis (SSc). Identifying SSc-ILD development and initiating treatment is essential to optimize therapeutic benefit.ObjectivesWe aimed to identify predictors of SSc-ILD and compared early (<5 years from diagnosis) versus late (>5 years from diagnosis) onset.MethodsWe conducted a retrospective cohort study by including patients diagnosed with SSc from 1980 to 2020 followed in our unit and compared the clinical profile of patients with SSc-ILD to control SSc-non-ILD patients. Demographic features, clinical and immunological characteristics, baseline pulmonary function and capillaroscopy data were retrieved. Logistic regression modelling was run to identify factors associated with SSc-ILD development. Factors associated with ILD were then determined as factors associated with early or late onset using multivariate analysis. Bonferroni correction was used to limit Type I errors.ResultsWe have included 103 patients from our patient registry from 1980 to 2021 (42% with SSc-ILD). Logistic regression identified risk factors associated with increased or decreased odds ratio for developing ILD is summarized in Table 1. Smoking history, male sex, the presence of myositis, anti-Scl70 and anti-Ro52 positivity, baseline pulmonary function including FVC and DLCO, mMRC (Modified Medical Research Council) dyspnea scale>2, mMSS (Modified Rodnan Skin Score), and late pattern in capillaroscopy were identified as SSc-ILD predictors. Older age at SSc diagnosis, the presence of telangiectasias and smoking status were correlated with of SSc-ILD onset before 5 years, while male gender, the presence of myositis and antiphospholid antibodies were correlated with late-onset SSc-ILD.Table 1.Significant logistic regressions for predictors for SSc-ILDPredictorOR (95% CI)P valueMale2.8 (1.16-6.8)0.02Smoking history2.15 (1.33-3.46)0.001Diffuse cutaneous involvement10.4 (2.2-48.3)0.003Raynaud duration0.89 (0.79-1.0)0.04Myositis3.45 (1.09-10.9)0.03Anti-Scl705.45 (2.02-14.7)0.001Anti-Ro523.37 (1.49-9.45)0.002FVC0.97 (0.91-0.98)0.03DLCO0.96 (0.93-0.98)0.003mMSS3.73 (1.64-8.5)0.01mMRC>22.01 (1.52-4.2)0.04Late capilaroscopy pattern2.3 (1.02-5.27)0.04Early onset SSc-ILDβ valueP valueOlder age at onset0.470.0017Telangiectasias0.290.04Smoking stauts0.370.02Late onset SSc-ILDβ valueP valueMale gender0.570.001Myositis0.340.02Antiphospholipid antibodies0.310.04ConclusionWe identified 10 factors significantly associated with risk of developing SSc-ILD: smoking, male sex, diffuse cutaneous involvement, the presence of myositis, shorter Raynaud duration to SSc diagnosis, anti-Scl70 and anti-Ro52 positivity and baseline pulmonary function (lower baseline DLCO and FVC increasing risk) and late capillaroscopy pattern and identified predictors for early and late-onset SSc-ILD.

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