Systemic scleroderma is a rare, incurable autoimmune disease associated with progressive hardening of the skin and fibrosis of internal organs. An international team of authors (first author: Lenny van Bon, Boston School of Medicine) has now published a contribution to research into the origin and development of this rare disease in the renowned New England Journal of Medicine. While investigating the basics of the incurable autoimmune disease, a new aspect of its development could also be described.
The scientists were able to show that the chemokine CXCL4, the predominant signaling protein in patients with systemic scleroderma, is produced by certain cells of the immune system (plasma cytoid dendritic cells). The higher the concentration of chemokine in blood serum, the more severe the patient's disease was with regard to skin and pulmonary fibrosis and pulmonary hypertension.
The chemokine CXCL4 can be used as a biomarker for the disease, according to the authors. However, the development of scleroderma is still not fully investigated and therefore requires further studies. (Original publication: New England Journal of Medicine, 2014: 30; 433-43.)(Source and further information: www.uniklinik-duesseldorf.de
