Department of Rheumatology, Kobe City Medical Center General Hospital, Japan.
Department of General Internal Medicine, Kobe City Medical Center General Hospital, Japan.
Intern Med. 2023 Sep 15;62(18):2747-2751. doi: 10.2169/internalmedicine.1275-22. Epub 2023 Feb 8.
A 69-year-old man with a history of anti-synthetase antibody-positive polymyositis and interstitial lung disease (ILD) stable for more than 20 years suddenly developed pulmonary artery hypertension (PAH) with a mean PA pressure of 46 mmHg. At this stage, ILD was mild, but it became acutely exacerbated later, and high-dose corticosteroid and intravenous cyclophosphamide ameliorated both PAH and ILD. The tricuspid regurgitation pressure gradient decreased from 80 to 49 mmHg and ILD recovered almost completely. During a systemic examination, bone metastatic cancer of unknown origin was found. We herein report the relationship between anti-synthetase syndrome (ASS) and PAH as well as ASS and malignancy.
一位 69 岁男性，有抗合成酶抗体阳性多发性肌炎和间质性肺病（ILD）病史 20 余年，病情稳定。患者突然出现肺动脉高压（PAH），平均肺动脉压为 46mmHg。此时ILD 处于轻度阶段，但后来急剧恶化，高剂量皮质类固醇和静脉环磷酰胺改善了 PAH 和ILD。三尖瓣反流压力梯度从 80mmHg 降至 49mmHg，ILD 几乎完全恢复。在全身检查中，发现不明来源的骨转移性癌。我们在此报告抗合成酶综合征（ASS）与 PAH 以及 ASS 与恶性肿瘤之间的关系。
