Department of Pediatrics, Toho University Omori Medical Center, Tokyo, Japan.
Circ J. 2010 Feb;74(2):371-4. doi: 10.1253/circj.cj-09-0369. Epub 2009 Dec 15.
Previous studies have reported a high prevalence of autoimmune thyroid disease (AITD) in adult patients with pulmonary arterial hypertension (PAH). The aim of this retrospective study was to determine the prevalence of AITD in children and adolescents with idiopathic PAH (IPAH).
The study group included 16 patients who had been diagnosed as having idiopathic PAH when they were younger than 15 years old; all were younger than 20 years of age. Thyroid function and antithyroid antibody levels were examined regularly at 6-12-month intervals and when there were clinical signs of thyroid dysfunction. In total, 7 patients (44%) had AITD; 2 patients developed Graves' disease, 2 developed silent thyroiditis, and 3 had antithyroid antibodies with euthyroidism. The duration after PAH onset and the prostacyclin (PGI(2)) treatment period were significantly longer in patients with AITD (7.6+/-2.1 and 7.4+/-2.3 years, respectively) than in patients without AITD (5.0+/-1.1 and 4.8+/-1.2 years, respectively; P<0.01 and P<0.05).
The prevalence of AITD is high in children and adolescents with IPAH, so evaluation of thyroid function is important to prevent deterioration of right heart failure.
既往研究报道成人肺动脉高压（PAH）患者中自身免疫性甲状腺疾病（AITD）的患病率较高。本回顾性研究旨在确定特发性PAH（IPAH）患儿中 AITD 的患病率。
研究组纳入 16 名年龄小于 15 岁时被诊断为特发性 PAH 的患者，所有患者均小于 20 岁。甲状腺功能和抗甲状腺抗体水平每 6-12 个月定期检查，并在出现甲状腺功能障碍的临床症状时检查。共计 7 名（44%）患者患有 AITD；2 名患者发生格雷夫斯病，2 名患者发生静默性甲状腺炎，3 名患者甲状腺功能正常但存在抗甲状腺抗体。AITD 患者的 PAH 发病后时间和前列环素（PGI（2））治疗时间显著长于无 AITD 患者（分别为 7.6+/-2.1 和 7.4+/-2.3 年，P<0.01 和 P<0.05）。
特发性 PAH 患儿 AITD 的患病率较高，因此评估甲状腺功能对于预防右心衰竭恶化很重要。
