Pulmonary involvement in systemic sclerosis

- Pulmonary complications are the main cause of death of SSc patients.
- Multiple cellular and humoral mechanisms interact in interstitial lung disease (ILD)-SSc pathophysiology.
- Further research in predictive factors and biomarkers for ILD-SSc is warranted.
- Diverse therapeutic regimens have been studied in ILD-SSc patients, with incomplete efficacy.
- Longitudinal studies are required to determine the best therapeutic approach for ILD-SSc.

Systemic sclerosis (SSc) is a multi-systemic autoimmune disease that mainly affects the skin, lungs, gastrointestinal tract, heart and kidneys. Pulmonary disease in patients with SSc is strongly associated with mortality. The mechanisms involved into its pathophysiology include the activation of autoimmune cells and hyperplasia of fibroblasts with an increased capacity to produce collagen and diminished collagen breakdown. Although pulmonary biopsy is the gold standard for the diagnosis of interstitial lung disease in SSc, the most commonly used method is high-resolution computed tomography due to its high sensitivity and specificity. Herein, a comprehensive review on the pulmonary involvement in SSc is presented highlighting the radiologic-pathologic correlations.