Nishiura Kazuto, Nakazato Kazuhiko, Yokokawa Tetsuro, Suzuki Yoshinori, Kurosawa Yuta, Wada Kento, Shimizu Takeshi, Oikawa Masayoshi, Kobayashi Atsushi, Sugimoto Koichi, Shakespear Norshalena, Hashimoto Yuko, Takeishi Yasuchika
Department of Cardiovascular Medicine, Fukushima Medical University, Fukushima 960-1295, Japan.
Department of Diagnostic Pathology, Fukushima Medical University, Fukushima 960-1295, Japan.
J Clin Med. 2023 Oct 4;12(19):6359. doi: 10.3390/jcm12196359.
Idiopathic pulmonary arterial hypertension is a progressive and life-threatening disease with pulmonary vasculature remodeling, leading to right-sided heart failure. Epoprostenol (prostaglandin I) is highly recommended for patients with severe pulmonary arterial hypertension (PAH) categorized by the World Health Organization as functional class III or IV. It has been reported that prostaglandin I analogs can cause thyroid gland swelling and abnormal thyroid function. A 34-year-old woman was diagnosed with idiopathic pulmonary arterial hypertension and started receiving continuous intravenous epoprostenol. Three years after starting epoprostenol, she began complaining of neck swelling and was diagnosed with Graves' disease. The patient's thyroid function was controlled by thiamazole and levothyroxine; nevertheless, her thyroid gland enlargement worsened as the epoprostenol dose was titrated. After 20 years, she developed respiratory failure with a giant goiter leading to airway stenosis, and she passed away. The pathological autopsy confirmed a massive goiter associated with hyperthyroidism and airway stenosis. We experienced a case of idiopathic pulmonary hypertension with a giant goiter and airway stenosis after long-term intravenous epoprostenol therapy.
特发性肺动脉高压是一种进行性且危及生命的疾病，伴有肺血管重塑，可导致右心衰竭。依前列醇（前列腺素I）强烈推荐用于世界卫生组织分类为功能Ⅲ级或Ⅳ级的重度肺动脉高压（PAH）患者。据报道，前列腺素I类似物可导致甲状腺肿大和甲状腺功能异常。一名34岁女性被诊断为特发性肺动脉高压，并开始接受依前列醇持续静脉输注治疗。开始使用依前列醇三年后，她开始抱怨颈部肿胀，并被诊断为格雷夫斯病。患者的甲状腺功能通过甲巯咪唑和左甲状腺素控制；然而，随着依前列醇剂量的滴定，她的甲状腺肿大加剧。20年后，她因巨大甲状腺肿导致气道狭窄而出现呼吸衰竭，并去世。病理尸检证实存在与甲状腺功能亢进和气道狭窄相关的巨大甲状腺肿。我们遇到了一例长期静脉输注依前列醇治疗后出现巨大甲状腺肿和气道狭窄的特发性肺动脉高压病例。
