系统性红斑狼疮合并肺动脉高压的风险因素筛查以及p-ANCA检测的临床价值

Risk factor screening for systemic lupus erythematosus combined with pulmonary hypertension and the clinical value of p-ANCA testing

目的探讨系统性红斑狼疮合并肺动脉高压的风险因素以及p-ANCA检测的临床价值。方法筛选河北医科大学第二医院系统性红斑狼疮(systemic lupus erythematosus,SLE)合并肺动脉高压(pulmonary hypertension,PAH)患者64例作为SLE-PAH组,其中核周型抗中性粒细胞胞浆抗体(perinuclear-ANCA,p-ANCA)阳性44例,p-ANCA阴性20例;按照1∶3比例匹配同期住院的不合并PAH的SLE患者192例作为SLE-nonPAH组。记录患者基本信息、SLE活动相关临床资料、实验室化验结果以及经胸心脏超声结果。分析SLE-PAH患者临床特征以及PAH患者中p-ANCA阳性和p-ANCA阴性患者的临床和实验室特点,探讨SLE-PAH的风险因素。结果与SLE-nonPAH组相比,SLE-PAH组年龄较大、中重度病情活动度占比较高,2组差异有统计学意义(P<0.05)。更多患者出现雷诺现象、白细胞计数和中性粒细胞绝对值增高、狼疮抗凝物标准化SCT比值升高、抗心磷脂抗体阳性以及肺动脉内径、右心房内径、室间隔厚度异常;SLE-PAH患者中,p-ANCA阳性组与P-ANCA阴性肺动脉高压严重程度存在差异,2组差异有统计学意义(P<0.05)。多因素Logistic回归分析提示雷诺现象、抗心磷脂抗体、中性粒细胞数量增高(OR=0.802)为SLE患者合并肺动脉高压的独立危险因素(P<0.05)。结论SLE患者出现中重度疾病活动度,外周血白细胞、中性粒细胞、狼疮抗凝物标准化SCT比值升高和抗心磷脂抗体阳性时警惕PAH发生。p-ANCA阳性可能与肺动脉高压严重程度有关,需密切随访。

Objective To investigate the risk factors of systemic lupus erythematosus(SLE)combined with pulmonary arterial hypertension(PAH)and the clinical value of perinuclear-ANCA(p-ANCA)test.Methods In total,64 patients with SLE combined with PAH treated in the Second Hospital of Hebei Medical University were screened as the SLE-PAH group,of which 44 patients were positive for p-ANCA and 20 patients were negative for p-ANCA.Then according to the ratio of 1∶3 we matched 192 SLE patients without PAH hospitalized during the same period as SLE-nonPAH group.The patients′basic information,clinical data related to SLE activities,laboratory test results and transthoracic echocardiography(TTE)results were recorded.Clinical characteristics of SLE-PAH patients as well as clinical and laboratory features of p-ANCA-positive and p-ANCA-negative patients with PAH were analyzed to explore the risk factors of SLE-PAH.Results Compared with the SLE-nonPAH group,patients in the SLE-PAH group had olderer age and a higher percentage of moderate-to-severe disease activity,showing significant difference between the two groups(P<0.05).More patients showed Raynaud′s phenomenon,increased white blood cell count and absolute neutrophil count,elevated standardized SCT ratio of lupus anticoagulant,positive anticardiolipin antibodies,and abnormalities of pulmonary artery diameter,right atrial diameter,and interventricular septal thickness.There was a significant difference in the severity of PAH between the p-ANCA positive group and the p-ANCA negative group in SLE-PAH patients(P<0.05).Multivariate Logistic regression analysis suggested that Raynaud′s phenomenon,anticardiolipin antibodies,and increased neutrophil count(OR=0.802)were independent risk factors for combined PAH in patients with SLE(P<0.05).Conclusion SLE patients presenting with moderate to severe disease activity,elevated peripheral blood leukocytes,neutrophils,standardized SCT ratios,and positive anticardiolipin antibodies are alerted to the development of PAH.p-ANCA positivity may be associated with the severity of pulmonary hypertension and requires close follow up.