系统性红斑狼疮相关的肺动脉高压不同分型的临床特点分析

Clinical Features Analysis of Patients with Systemic Lupus Erythematosus Concomitant Pulmonary Arterial Hypertension in Different Classifications

目的系统性红斑狼疮(SLE)相关的肺动脉高压(PAH)在亚洲人群中发生率较其他结缔组织病高,且不同患者预后相差较大,本研究拟从SLE的不同临床分型的临床特点探讨对预后的影响。方法84例SLE相关PAH患者根据PAH严重程度、SLE疾病活动度、治疗可逆性、PAH出现的病程进行分型,对其临床特点进行回顾性分析,评价不同分型方法对预后判断的影响。结果SLE相关PAH的危险因素为雷诺现象、指端血管炎、心包积液、肺间质病变、抗U1RNP阳性和ACA-IgG阳性。轻中度PAH与重度PAH危险因素的比较发现,抗U1RNP阳性差异有统计学意义(P<0.05);SLEDAI<8分和SLEDAI≥8分危险因素的比较发现,指端血管炎和抗U1RNP阳性差异有统计学意义(P<0.05);治疗缓解、稳定和恶化危险因素的比较发现,指端血管炎和心包积液比较差异有统计学意义(P<0.05);PAH出现的病程<1年和病程≥1年危险因素的比较发现,心包积液差异有统计学意义(P<0.05)。结论重度SLE-PAH患者中抗U1RNP的阳性率较轻中度SLE-PAH患者中更高;SLE-PAH患者中疾病活动度较高的患者出现指端血管炎的阳性率高于疾病活动度低的患者,而疾病活动度低的患者抗U1RNP阳性率高于疾病活动度较高的患者;指端血管炎在缓解组SLE-PAH患者中阳性率最高,心包积液在恶化组阳性率最高;PAH出现超过1年的患者心包积液的阳性率较<1年的患者高;抗U1RNP是SLE-PAH患者的预后不良因素,出现指端血管炎的患者预后可能较好,合并心包积液的患者可能预后不佳。

Objective The incidence of systemic lupus erythematosus(SLE)concomitant pulmonary arterial hypertension(PAH)in Asia is higher than other connective tissue disease,and different patients have different prognosis.We intend to discuss the effect of clinical features in different classifications to the prognosis of patients.Methods The 84 patients with SLE-PAH were randomly divided into different types based on the severity of PAH,systemic lupus erythematosus disease activity index(SLEDAI),reversible of treatment and process of PAH.We analysis the clinical features of patients and evaluate the effect of judgment to the prognosis of patients by different classifications.Results The risk factors of SLE-PAH were raynaud phenomenon,fingertip vasculitis,pericardial effusion,interstitial lung disease,anti-U1RNP antibody positivity and antiphospholipid antibody positivity.Anti-U1RNP antibody positivity in mild or moderate PAH patients compared with severe PAH patients had statistical significance(P<0.05).Fingertip vasculitis and anti-u1RNP antibody positivity in SLEDAI<8 patients compared with SLEDAI≥8 patients had statistical significance(P<0.05).Fingertip vasculitis and pericardial effusion in relieved,stable and deteriorated patients had statistical significance(P<0.05).Pericardial effusion in PAH course more than 1 years patients compared with PAH course less than 1 years patients had statistical significance(P<0.05).Conclusion Anti-U1RNP antibody positivity in severe PAH patients was higher than mild or moderate PAH patients.Fingertip vasculitis in SLEDAI≥8 patients was higher than SLEDAI<8 patients.Anti-U1RNP antibody positivity in SLEDAI<8 patients was higher than SLEDAI≥8 patients.SLE-PAH patients with fingertip vasculitis had higher reversible of treatment.SLE-PAH patients with pericardial effusion was difficult to treat.Positive rate of ericardial effusion in PAH course more than 1 years patients was more than PAH course less than 1 years patients.SLE-PAH patients had two pathology which were vascular inflammation and vascular disease.The severe PAH,SLEDAI<8,deteriorated and PAH course more than 1 years patients′pathological changes were related to vascular disease,especially the anti-U1RNP antibody positivity patients.