A clue for telangiectasis in systemic sclerosis: elevated serum soluble endoglin levels in patients with the limited cutaneous form of the disease.
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 endothelial cells. The role of endoglin in SSc is potentially intriguing since limited cutaneous SSc (lcSSc) 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 lcSSc 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 lcSSc.


CONCLUSIONS
Abnormal expression/function of endoglin may be linked to lcSSc-specific manifestations.
Fujimoto
M
M
Department of Dermatology, Kanazawa University Graduate School of Medical Science, Japan. fujimoto-m@umin.ac.jp
Hasegawa
M
M
Hamaguchi
Y
Y
Komura
K
K
Matsushita
T
T
Yanaba
K
K
Kodera
M
M
Takehara
K
K
Sato
S
S
D000293:Adolescent; D000328:Adult; D000368:Aged; D015703:Antigens, CD / Q000097:blood*; D015415:Biomarkers / Q000097:blood; D002648:Child; D000071063:Endoglin; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D011956:Receptors, Cell Surface / Q000097:blood*; D012595:Scleroderma, Systemic / Q000097:blood* / Q000150:complications; D012720:Severity of Illness Index; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000097:blood* / Q000150:complications
D015703:Antigens, CD; D015415:Biomarkers; C064206:ENG protein, human; D000071063:Endoglin; D011956:Receptors, Cell Surface