Interstitial lung disease pathology in systemic sclerosis

Interstitial lung disease is a relatively frequent manifestation of systemic sclerosis with approximately one-third of patients developing clinical restrictive lung disease. Fibrotic nonspecific interstitial pneumonia is the most common cause of diffuse parenchymal lung disease in patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD), followed by usual interstitial pneumonia (UIP). Radiographic pleuroparenchymal fibroelastosis-like changes may accompany other forms of interstitial lung disease, most commonly UIP. In an appropriate clinical setting with supportive high-resolution computed tomography findings, lung biopsy is not needed to confirm the presence of interstitial lung disease and surgical lung biopsies are often reserved for atypical presentations. In this review, we discuss the histological findings that define the most common patterns of SSc-ILD and outline other findings sometimes encountered in lung biopsies obtained from systemic sclerosis patients, including pulmonary vascular changes, aspiration, chronic pleuritis, and diffuse alveolar damage.

Automated summary

Interstitial lung disease is a common manifestation of systemic sclerosis, with fibrotic nonspecific interstitial pneumonia being the most common form followed by usual interstitial pneumonia. Lung biopsy may not be necessary for confirming the presence of interstitial lung disease in the right clinical setting, with surgical lung biopsies often reserved for atypical presentations. This review discusses histological findings in SSc-ILD and other changes sometimes seen in lung biopsies from systemic sclerosis patients.