Luo Ri-qiang, Lei Yun-xia, Zhang Xiao, Liang Fei
Department of Rheumatology, Guangdong Provincial People's Hospital, Guangzhou 510080, China.
Nan Fang Yi Ke Da Xue Xue Bao. 2008 Oct;28(10):1860-3.
To investigate the clinical manifestations, diagnosis and interventions of pulmonary hypertension (PAH) in patients with systemic lupus erythematosus (SLE).
From January 2001 to December 2007, 798 SLE patients without prior diagnosis were admitted in our hospital, among whom 39 were identified to have concomitant PAH defined by echocardiography. The clinical data of the 39 cases were analyzed retrospectively.
The incidence of PAH was 4.9% in these cohort of SLE patients. The 39 SLE patients with concomitant PAH included 5 men and 34 women with a mean age of 34-/+12 years. Positive correlations were found between the occurrence of PAH and the Raynaud phenomenon, fingertip vasculitis, anti-u1RNP antibody positivity, antiphospholipid antibody positivity, pericardial effusion, and interstitial pneumonia (P < 0.05). Patients with higher scores for SLE Disease Activity Index were liable to PAH. The presence of Raynaud phenomenon, fingertip vasculitis, anti-u1RNP antibody positivity, antiphospholipid antibody positivity, pericardial effusion, and interstitial pneumonia is correlated to greater severity PAH with poor prognosis.
PAH is not a rare concomitant disease in SLE patients. The presence of Raynaud phenomenon, fingertip vasculitis, anti-u1RNP antibody positivity, antiphospholipid antibody positivity, pericardial effusion, and interstitial pneumonia all suggest the likeliness of PAH in SLE patients, and echocardiographic examination may help derive an early diagnosis.
探讨系统性红斑狼疮(SLE)患者肺动脉高压(PAH)的临床表现、诊断及干预措施。
2001年1月至2007年12月,我院收治798例既往未诊断的SLE患者,其中39例经超声心动图确诊合并PAH。对这39例患者的临床资料进行回顾性分析。
该队列SLE患者中PAH发病率为4.9%。39例合并PAH的SLE患者中,男性5例,女性34例,平均年龄34±12岁。PAH的发生与雷诺现象、指尖血管炎、抗U1RNP抗体阳性、抗磷脂抗体阳性、心包积液及间质性肺炎呈正相关(P<0.05)。SLE疾病活动指数评分较高的患者易患PAH。雷诺现象、指尖血管炎、抗U1RNP抗体阳性、抗磷脂抗体阳性、心包积液及间质性肺炎的存在与PAH病情严重程度及预后不良相关。
PAH在SLE患者中并非罕见的合并症。雷诺现象、指尖血管炎、抗U1RNP抗体阳性、抗磷脂抗体阳性、心包积液及间质性肺炎的存在均提示SLE患者可能患有PAH,超声心动图检查有助于早期诊断。