Rheumatology: Current Research
Open Access

Abstract

Progress in Understanding the Pathogenesis of Systemic Sclerosis with Lung Involvement: The Contribution of Proteomic Studies

Cinzia Scambi, Lucia De Franceschi, Silvia Bosello, Paola Caramaschi, Gianfranco Ferraccioli and Domenico Biasi

Systemic sclerosis (SSc) is a chronic inflammatory disease involving the skin and various internal organs. SSc is characterized by microvascular dysfunction, activation of the immune system and tissue fibrosis. Endothelial cell damage seems to be the initiating factor, but the precise triggering events that underlie the development of the disease remain unclear.

Lung involvement is a frequent complication, which causes increased morbidity and mortality in patients with SSc. In particular, pulmonary arterial hypertension and interstitial lung disease are the two major clinical diseases that affect SSc patients, but the current treatments appear to have no satisfying effects on these pulmonary complications, until yet.

Recently researches using innovative technologies have highlighted several peptides that might contribute to better understand the underlying pathogenic mechanisms of pulmonary injury and could promote more effective therapeutic strategies for SSc patients. Here, we focus on the major proteomic studies on biological fluid from patients with SSc.