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Case Reports
A Case of Anti-synthetase Syndrome that Relapsed with Pulmonary Arterial Hypertension and Malignancy.
- Hideki Oka, Shuji Sumitomo, Hayato Shimizu, Maki Kanamori, Shiori Murata, Daisuke Yamashita, Taiji Okada, Hiroaki Nishioka, and Koichiro Ohmura.
- Department of Rheumatology, Kobe City Medical Center General Hospital, Japan.
- Intern. Med. 2023 Sep 15; 62 (18): 274727512747-2751.
AbstractA 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.
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