原发性干燥综合征合并肺动脉高压33例分析

An analysis of 33 cases with primary Sjgren's syndrome combined with pulmonary arterial hypertension

目的分析原发性干燥综合征(pSS)合并肺动脉高压(PAH)的临床特征、治疗及预后。方法回顾性分析33例pSS合并PAH的患者(PAH组)和30例未合并PAH的pSS患者(非PAH组)的临床资料。结果 PAH组雷诺现象、发热、咳嗽咳痰、胸闷、进展性呼吸困难和肺间质病变的发生率均高于非PAH组(P<0.05或P<0.01)。PAH组外周血WBC、C反应蛋白、总胆红素、直接胆红素、间接胆红素和抗SSA抗体阳性率均高于非PAH组,PAH组补体C3、白蛋白、TG低于非PAH组(P<0.05或P<0.01)。PAH组患者给予激素、免疫抑制剂或降压治疗,2例患者死亡,其余患者病情得到缓解和控制。结论 PAH是pSS罕见且严重的并发症,临床症状缺乏特异性,经常出现延迟诊断,且治疗和预后不佳,因此早期诊断及早期治疗尤为重要。

Objective To analyze the clinical features,treatment and prognosis of primary Sjgren＇s syndrome（pSS）patients combined with pulmonary arterial hypertension（PAH）.Methods The clinical data of 33 pSS patients combined with PAH（group PAH）and 30 pSS patients without PAH（group NPAH）were analyzed restrospectively.Results The incidences of Raynaud＇s phenomenon,fever,cough and sputum,chest distress,progressive dyspnea and interstitial lung disease were all significantly higher in group PAH than those in group NPAH（P〈0.05 or P〈0.01）.Serum levels of WBC,C-reactive protein,total bilirubin,direct bilirubin,indirect bilirubin and anti-SSA antibody positive rate were higher,but those of complement C3,albumin and triglyceride were lower in group PAH than those in group NPAH（P〈0.05 or P〈0.01）.Group PAH was given glucocorticoid,immunosuppressant or antihypertensive therapy,of whom two cases died and others were with attenuated and controled illness state.Conclusion PAH is a rare but severe complication of pSS.The clinical symptoms of pSS combined with PAH lack of specificity,the diagnosis is often delayed and the treatment and prognosis are poor.Thus early diagnosis and treatment are very important in pSS patients with PAH.