Hara Yuki, Morita Takayoshi, Tanaka Katsunao, Sera Fusako, Sakata Yasushi, Nishide Masayuki, Maeda Yuichi, Narazaki Masashi, Kumanogoh Atsushi
Department of Respiratory Medicine and Clinical Immunology, Graduate School of Medicine Osaka University Osaka Japan.
Department of Cardiovascular Medicine, Graduate School of Medicine Osaka University Osaka Japan.
Respirol Case Rep. 2021 Apr 28;9(5):e00746. doi: 10.1002/rcr2.746. eCollection 2021 May.
Adult-onset Still's disease (AOSD) is a rare inflammatory autoimmune disorder characterized by fever, skin rash, and arthralgia. Pulmonary artery hypertension (PAH) rarely occurs with AOSD and has not been reported in the absence of typical symptoms of AOSD. A 33-year-old woman was admitted to our hospital with dyspnoea on exertion. Although she had not had symptoms of AOSD for 18 months before her admission, she presented with gradually progressing PAH. Because she had no typical symptoms of AOSD, she was treated with pulmonary vasodilators. However, her PAH did not improve. At one month after vasodilator treatment, she developed a high fever with elevation of ferritin. We determined that her AOSD had relapsed. Immunosuppressants were started and both her AOSD and PAH quickly improved. PAH may develop in the absence of typical symptoms of AOSD and immunosuppressants may be effective in such a case.
成人斯蒂尔病（AOSD）是一种罕见的炎症性自身免疫性疾病，其特征为发热、皮疹和关节痛。肺动脉高压（PAH）在AOSD中很少见，且在无AOSD典型症状的情况下尚未见报道。一名33岁女性因劳力性呼吸困难入院。尽管入院前18个月她没有AOSD症状，但她出现了逐渐进展的PAH。由于她没有AOSD的典型症状，她接受了肺血管扩张剂治疗。然而，她的PAH并未改善。在血管扩张剂治疗一个月后，她出现高热且铁蛋白升高。我们确定她的AOSD复发了。开始使用免疫抑制剂后，她的AOSD和PAH均迅速改善。PAH可能在无AOSD典型症状的情况下发生，在这种情况下免疫抑制剂可能有效。
