Clinical characteristics and prognostic risk factors for interstitial lung disease in patients with systemic sclerosis

Objective: Investigate the clinical characteristics, treatment effects, and prognostic factors for interstitial lung disease (ILD) in patients with systemic sclerosis (SSc). Methods: This retrospective study examined the records of 320 patients with SSc. Presence of ILD, extent of skin involvement, effect of treatment (based on high-resolution computed tomography), and prognostic risk factors were examined. Results: One hundred and sixty-nine patients (52.8%) had ILD. Patients with SSc-ILD had greater average age (51.30 +/- 10.86 vs. 48.58 +/- 12.52 years); longer (9.5 vs. 6.5 months); higher prevalence of cough, exertional dyspnea, chest tightness and pain, and pulmonary hypertension; higher levels of erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP); and anti-Scl-70 antibody positivity. In males, ILD is more common in patients with dcSSc than in patients with lcSSc. All patients received immunosuppressants and/or glucocorticoids for at least 12 months. Follow-up data were available for 71 SSc-ILD patients. Based on HRCT scores, 34 (47.9%) patients experienced deterioration, 18 (25.3%) remained stable, and 19 (26.8%) improved. Those who deteriorated were older, had a higher prevalence of exertional dyspnea, and higher ESR. Multivariate logistic regression showed that disease duration was independently associated with ILD. Age and exertional dyspnea were independent risk factors for SSc-ILD deterioration. After immunosuppressive therapy, disease progression occurred in 47.9% of patients. Conclusion: ILD in SSc correlated with prolonged disease duration, presence of cough and pulmonary hypertension, active inflammation, and anti-Scl70 antibody positivity. Age and exertional dyspnea were associated with deterioration of SSc-ILD.