Proteome-wide Analysis and CXCL4 as a Biomarker in Systemic Sclerosis

In this study, the level of the chemokine CXCL4, which is secreted by plasmacytoid dendritic cells, was elevated in patients with systemic sclerosis and correlated with the presence and progression of complications. Systemic sclerosis (also called scleroderma) is a complex heterogeneous fibrosing autoimmune disorder with an unknown pathogenesis. The way in which its three major pathologic hallmarks — extensive fibrosis, vasculopathy, and immune dysfunction — are interconnected is unknown. Mechanistic understanding is limited, in part, by a lack of animal models and by clinically heterogeneous patient populations. 1 This disorder is classified into two major subtypes on the basis of the extent of cutaneous fibrosis: limited cutaneous and diffuse cutaneous systemic sclerosis. 2 Pulmonary fibrosis and pulmonary arterial hypertension are the two most serious complications — currently the major causes of death among patients . . .