Scleroderma-associated interstitial lung disease – new therapeutic approach

Scleroderma-related interstitial lung disease

Scleroderma-related interstitial lung disease (SSc-ILD) is a pulmonary fibrosing disorder characterized by systemic inflammation and progressive scarring of the lungs that leads to respiratory failure. Although certain immunosuppressive therapies may slow disease progression, current treatment strategies are not curative; consequently, SSc-ILD continues to be a major cause of morbidity and mort...

Interstitial lung disease in scleroderma

Pulmonary involvement is quite frequent in the development of progressive systemic sclerosis (PSS), a disease that affects the skin and internal organs and may present in various forms, especially as interstitial lung disease (ILD) or pulmonary hypertension (PH).(1) The incidence of pulmonary involvement varies depending on the population and on the methodology used for the diagnosis, reaching ...

The Assessment of Health-Related Quality of Life in Scleroderma-Interstitial Lung Disease

Introduction: Pulmonary involvement is the most common cause of mortality and disability in patients with systemic sclerosis and it significantly affects the quality of life in these patients. Therefore, early diagnosis and treatment of pulmonary involvement seems necessary in patients with SSc. In this study, we aimed to assess the health-related quality of life (HRQoL) in patients with Sclero...

A New Era in the Treatment of Scleroderma-associated Interstitial Lung Disease?

Chitinase 1 is a biomarker for and therapeutic target in scleroderma-associated interstitial lung disease that augments TGF-β1 signaling.

Interstitial lung disease (ILD) with pulmonary fibrosis is an important manifestation in systemic sclerosis (SSc, scleroderma) where it portends a poor prognosis. However, biomarkers that predict the development and or severity of SSc-ILD have not been validated, and the pathogenetic mechanisms that engender this pulmonary response are poorly understood. In this study, we demonstrate in two dif...