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目的提高对结缔组织病(CTD)合并肺动脉高压(PAH)患儿的认识,力求达到早期发现及治疗。方法 2007年1月至2012年12月中国医科大学附属盛京医院共收治60例CTD患儿,重点回顾分析其中合并PAH的15例患儿临床资料。总结其临床表现、超声心动图检测等实验室检查特点、治疗及预后等,并与原发病相同但未合并PAH者进行对比。结果 15例合并PAH的CTD患儿中系统性红斑狼疮(SLE)10例,类风湿关节炎(RA)2例,皮肌炎(DM)2例,混合性结缔组织病(MCTD)1例。临床表现以雷诺现象和皮疹、发热最常见(均为11例,73.3%)。而有呼吸系统症状者不多(4例,26.7%)。抗核抗体检测示ANA阳性率最高(14/15,93.3%),其次为u1-RNP(9/15,60.0%)。类风湿因子(RF)阳性也较常见(11/15,73.3%)。均予糖皮质激素、环磷酰胺联合其他免疫抑制剂治疗,其中有呼吸系统症状的4例患儿予甲基泼尼松龙冲击治疗,有2例使用西地那非治疗。15例中3例死亡,均为有呼吸系统症状者,余患儿预后较好。结论 PAH是CTD较为严重的并发症之一,也是影响CTD患儿预后的重要因素;临床上对有雷诺现象且u1-RNP阳性的CTD患儿,要警惕早期PAH的发生;早期发现及积极治疗可改善其预后。
Objective To improve the understanding of children with connective tissue disease (CTD) complicated with pulmonary hypertension (PAH) and to achieve early detection and treatment. Methods From January 2007 to December 2012, 60 cases of children with CTD were admitted to Shengjing Hospital affiliated to China Medical University. The clinical data of 15 children with PAH were retrospectively analyzed. To summarize its clinical manifestations, laboratory features such as echocardiography, treatment and prognosis, and compared with the same primary disease but without PAH. Results Fifteen cases of systemic lupus erythematosus (SLE), two cases of rheumatoid arthritis (RA), two cases of dermatomyositis (DM) and one case of mixed connective tissue disease (MCTD) were detected in 15 children with CTD complicated with PAH. Renault phenomenon and rash clinical manifestations, the most common fever (11 cases, 73.3%). Respiratory symptoms were rare (4 patients, 26.7%). Anti-nuclear antibody test showed the highest positive rate of ANA (14 / 15,93.3%), followed by u1-RNP (9/15, 60.0%). Rheumatoid factor (RF) positive is also more common (11/15, 73.3%). All patients were treated with glucocorticoid and cyclophosphamide combined with other immunosuppressive agents. Among them, 4 patients with respiratory symptoms were treated with methylprednisolone and 2 with sildenafil. Three of 15 patients died of respiratory symptoms, and the remaining children had better prognosis. Conclusions PAH is one of the more serious complication of CTD. It is also an important factor affecting the prognosis of children with CTD. Clinical manifestations of early onset of PAH should be vigilant for CTD children with Renault phenomenon and u1-RNP positive. Can improve its prognosis.