Angiopoietin-1, Endoglin與紅斑性狼瘡肺高壓的相關性研究

Angiopoietin-1 and endoglin are not associated with pulmonary arterial hypertension in patients with systemic lupus erythematous

目的：Angiopoietin（Ang）-1為血管新生因子，可調節病變平滑肌細胞的增生，而endoglin（Eng）則在心臟血管生成、血管重塑、及活化血管新生扮演重要角色。兩者均證實與硬皮症引起的肺高壓相關。本研究主要探討Ang-1及Eng是否與紅斑性狼瘡併發的肺高壓相關。方法：共收集80位紅斑性狼瘡未合併肺高壓病患與22位紅斑性狼瘡合併肺高壓病患，藉由酵素免疫分析法測定病患血清Ang-1與Eng濃度，並評估與臨床疾病活性（C3、C4與anti-dsDNA）之相關性。結果：紅斑性狼瘡病患的Ang-1血清濃度明顯低於正常人（分別為19.37 ± 8.02及32.00 ± 9.89 ng/mL，p<0.001），而在紅斑性狼瘡的族群中，未合併肺高壓的Ang-1濃度雖低於合併肺高壓病患，但未達顯著差異（分別為18.75 ± 7.90及21.60 ± 8.24 ng/mL，p=0.394）。而Eng濃度在無論是紅斑性狼瘡病患與正常人（分別為4.62 ± 1.60 及4.78 ± 0.99 ng/mL，p=0.400）或是紅斑性狼瘡病患有無合併肺高壓（分別為4.37 ± 1.20及4.69 ± 1.69 ng/mL，p=0.441）之間，皆無顯著差異。此外，紅斑性狼瘡病患的Ang-1與Eng血清濃度與狼瘡活性指標C3、C4及anti-dsDNA Ab均未呈現顯著相關性。結論：Ang-1與Eng無法作為紅斑性狼瘡病人是否併發肺高壓之生物指標。

Objective: Angiopoietin (Ang)-1, an angiogenic factor, has been demonstrated to regulate pathologic smooth muscle cell hyperplasia. Endoglin (Eng) plays a role in cardiovascular development, vascular remodeling and active angiogenesis. Both were reported to be associated with the development of pulmonary artery hypertension (PAH) in scleroderma. Therefore, we hypothesized that serum levels of Ang-1 and Eng would be associated with PAH in patients with systemic lupus erythematous (SLE). Methods: Serum Ang-1 and Eng levels were measured by enzyme-linked immunoassay in 22 SLE patients with PAH, 80 patients without PAH and 16 healthy controls, and were correlated with disease activities. Results: Ang-1 levels were significantly lower in SLE patients (19.37 ± 8.02 ng/mL) compared with healthy controls (32.00 ± 9.89 ng/mL, p<0.001). Among SLE patients, Ang-1 levels were lower in patients without PAH (18.75 ± 7.90 ng/mL) compared with those with PAH (21.60 ± 8.24 ng/mL, p=0.394). However, the difference did not reach a statistical significance. There was no difference of Eng levels between SLE patients (4.62 ± 1.60 ng/mL) and healthy controls (4.78 ± 0.99 ng/mL, p=0.400) and between SLE patients without PAH (4.69 ± 1.69 ng/mL) and with PAH (4.37 ± 1.20 ng/mL, p=0.441). In addition, Ang-1 and Eng levels did not correlate with C3, C4 and anti-dsDNA Ab in patients with SLE. Conclusion: Serum Ang-1 and Eng levels were not good biomarkers in predicting PAH in patients with SLE.