A clue for telangiectasis in systemic sclerosis: Elevated serum soluble endoglin levels in patients with the limited cutaneous form of the disease

被引：35

作者：

Fujimoto, M.

Hasegawa, M.

Hamaguchi, Y.

Komura, K.

Matsushita, T.

Yanaba, K.

Kodera, M.

Takehara, K.

Sato, S.

机构：

[1] Kanazawa Univ, Grad Sch Med Sci, Dept Dermatol, Kanazawa, Ishikawa 9208641, Japan

[2] Nagasaki Univ, Grad Sch Biomed Sci, Dept Dermatol, Nagasaki 852, Japan

来源：

DERMATOLOGY | 2006年 / 213卷 / 02期

关键词：

anticentromere antibody; pulmonary hypertension; hereditary hemorrhagic telangiectasia;

D O I：

10.1159/000093846

中图分类号：

R75 [皮肤病学与性病学];

学科分类号：

100206 ;

摘要：

Background: The transforming growth factor-beta (TGF-beta) 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-beta receptor preferentially expressed on enclothelial 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: Serum sEndoglin 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. Copyright (c) 2006 S. Karger AG, Basel

引用

页码：88 / 92

页数：5

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