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Systemic sclerosis (SSc), also called scleroderma, is an autoimmune fibrosing disorder involving multiple organs, including skin; fatal complications, such as pulmonary fibrosis, are possible. Plasmacytoid dendritic cells (pDC), so critically important for anti-viral immunity, are suspected culprits, but how their involvement drives disease has been unknown until now.
van Bon and colleagues isolated pDC from patients with SSc, and using a proteome-wide screen, identified CXCL4 as highly expressed by pDC in early diffuse disease. CXCL4 expression was more than threefold lower in late diffuse SSc or localized SSc than in early diffuse SSc, and also lower in normal control samples. Markedly elevated CXCL4 levels were also found in serum in earl…