Interstitial lung disease pathology in systemic sclerosis

Abstract

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.

High-Resolution Computed Tomography: Lights and Shadows in Improving Care for SSc-ILD Patients

“…Considering the heterogeneity of SSc-ILD, the potential absence of symptoms in early or mild disease, lung function tests and chest imaging can help to identify who has SScassociated ILD and predict progression [1][2][3]. Furthermore, patients may be diagnosed earlier and more efficiently through multidisciplinary evaluation and collaboration, an essential element to optimize and improve clinical outcomes [1,2,[106][107][108][109]. Several studies have suggested that all SSc patients should undergo a baseline pulmonary function test and lung HRCT screening to allow for an early diagnosis of ILD and tailor further management.…”

Advancements in Dermatological Imaging Modalities

“…NSIP presents with features like ground glass opacities, reticular opacities, and traction bronchiectasis, with characteristic subpleural sparing, while UIP is characterized by honeycombing. [ 82 ] Vascular anomalies and malformations: CT scans serve as an excellent modality to assess the depth and size of vascular anomalies, playing a crucial role in determining the appropriate treatment modality. They also assist in evaluating treatment response and internal organ involvement.…”

“…NSIP presents with features like ground glass opacities, reticular opacities, and traction bronchiectasis, with characteristic subpleural sparing, while UIP is characterized by honeycombing. [ 82 ]…”

Alveolar epithelial-to-mesenchymal transition in scleroderma interstitial lung disease: Technical challenges, available evidence and therapeutic perspectives

“…Nevertheless, crucial differences between NSIP and UIP in SSc have not been conclusively recognised in terms of epidemiology, progression rate, treatment response or related mortality. 4 Multiple sources have been demonstrated or postulated for the hyperactive fibroblast pool involved in SSc-ILD ECM deposition, including activation of quiescent resident tissue fibroblasts, recruitment of circulating fibrocytes or transformation of pericytes, endothelial cells and alveolar epithelial cells (AECs). 5 The epithelial-to-mesenchymal transition (EMT) is a process of gradual transformation of fully differentiated epithelial cells towards mesenchymal-like cells through functional and morphological changes.…”

“…Nevertheless, crucial differences between NSIP and UIP in SSc have not been conclusively recognised in terms of epidemiology, progression rate, treatment response or related mortality. 4 …”