摘要
Background: The transforming growth factor-β(TGF-β) system plays a critical role both in systemic sclerosis (SSc) and hereditary hemorrhagic telangiectasia (HHT). Endoglin, known as a gene responsible for HHT, is a TGF-βreceptor preferentially expressed on endothelial cells. The role of endoglin in SSc is potentially intriguing since limited cutaneous SSc (IcSSc)-and HHT share several symptoms, including telangiectasia. Objective: To determine serum levels of soluble endoglin (sEndoglin) and clinical associations in patients with SSc. Methods: Serums Endoglin levels were examined by ELISA in 70 patients with SSc, 20 patients with systemic lupus erythematosus and 20 healthy individuals. Results: Serum sEndoglin levels were significantly elevated in patients with IcSSc compared with diffuse cutaneous SSc and systemic lupus erythematosus patients as well as normal controls. Patients with elevated sEndoglin levels had telangiectasia more frequently than those with normal sEndoglin levels. Furthermore, pulmonary artery pressure was positively correlated with sEndoglin levels in patients with IcSSc. Conclusion: Abnormal expression/function of endoglin may be linked to IcSSc-specific manifestations.
Background: The transforming growth factor-β(TGF-β) system plays a critical role both in systemic sclerosis (SSc) and hereditary hemorrhagic telangiectasia (HHT). Endoglin, known as a gene responsible for HHT, is a TGF-βreceptor preferentially expressed on endothelial cells. The role of endoglin in SSc is potentially intriguing since limited cutaneous SSc (IcSSc)-and HHT share several symptoms, including telangiectasia. Objective: To determine serum levels of soluble endoglin (sEndoglin) and clinical associations in patients with SSc. Methods: Serums Endoglin levels were examined by ELISA in 70 patients with SSc, 20 patients with systemic lupus erythematosus and 20 healthy individuals. Results: Serum sEndoglin levels were significantly elevated in patients with IcSSc compared with diffuse cutaneous SSc and systemic lupus erythematosus patients as well as normal controls. Patients with elevated sEndoglin levels had telangiectasia more frequently than those with normal sEndoglin levels. Furthermore, pulmonary artery pressure was positively correlated with sEndoglin levels in patients with IcSSc. Conclusion: Abnormal expression/function of endoglin may be linked to IcSSc-specific manifestations.
