Clinical features and radiological findings of pulmonary fibrosis associated with systemic sclerosis and idiopathic pulmonary fibrosis

Systemic sclerosis-associated interstitial lung disease (SSc-ILD) often has progressive course and poor prognosis. Prognosis depends on the extent of pulmonary parenchyma damage, functional impairment, and ILD pattern.

The aim. To analyze clinical, functional, and hemodynamic parameters in patients with various radiological patterns of SSc-ILD compared to idiopathic pulmonary fibrosis (IPF).

Methods. This retrospective longitudinal study included a total of 176 patients (103 with SSc and 73 with IPF). The clinical data, functional indices, patterns on high-resolution computed tomography (HRCT) of the lungs and echocardiography were analyzed. Descriptive statistics (frequencies, median, interquartile range) and comparisons between groups (Mann – Whitney U-test for continuous variables and chi-square test for categorical variables) were performed. Factors associated with the presence of pulmonary fibrosis and emphysema were determined using regression and ROC analysis.

Results. The results demonstrate a high prevalence of pulmonary fibrosis in SSc-ILD patients – 53.4%. The typical interstitial pneumonia pattern was detected in 8.7% of these cases. Compared with IPF, patients with fibrotic SSc-ILD had longer disease duration, better GAP (Gender, Age, Physiology) scores, and fewer comorbidities. Patients with IPF were characterized by lower FVC and a greater prevalence of pulmonary hypertension, though there were no significant differences in DLCO. The main predictors of the presence of pulmonary fibrosis in patients with SSc-ILD were disease duration of more than 8 years, decreased FVC less than 80%pred., DLCO less than 45%pred., and increased pulmonary artery systolic pressure greater than 35 mmHg. Patients with fibrotic SSc-ILD had lower FVC and DL CO than those with non-fibrotic SSc-ILD and were more likely to develop pulmonary hypertension. Pulmonary emphysema was detected in 21.4% of patients with SSc-ILD, the main predictors of which were the presence of pulmonary fibrosis, a disease duration of more than 12 years, a decrease in DLCO of less than 35%pred., and an increase in FVC/DLCO of more than 1.9.

Conclusion. This study revealed differences in clinical and functional parameters between patients with fibrotic SSc-ILD and IPF, as well as between fibrotic and non-fibrotic SSc-ILD. Additionally, this study identified factors associated with the presence of pulmonary fibrosis and emphysema in SSc.

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