This is based on study findings that CXCL4 levels are raised in patients with systemic sclerosis and high plasma levels correlate with the presence and progression of complications such as lung fibrosis and pulmonary arterial hypertension.
In a proteome-wide analysis, CXCL4 levels in patients with systemic sclerosis were significantly higher than in controls for patients with rheumatoid arthritis, ankylosing spondylitis and systemic lupus erythematosus.
CXCL4 was the main protein secreted by plasmacytoid dendritic cells from the circulation and skin in those patients with systemic sclerosis.
These results suggest that CXCL4 may be central to the pathogenesis of systemic sclerosis, and therapy aimed at decreasing its activity may prove helpful in managing the disorder.
Van Bon L, Affandi AJ, Broen J et al. N Engl J Med 2014; 370(5): 433-43