Recurrent idiopathic pulmonary hemosiderosis after long-term remission presented with Sjogren's syndrome: Idiopathic no more?. (2018)

• Record Type: Journal Article
• Authors: Yanagihara, Toyoshi; Yamamoto, Yuzo; Hamada, Naoki; Suzuki, Kunihiro; Ogata-Suetsugu, Saiko; Harada, Eiji; Tagawa, Tetsuzo; Fujiwara, Minako; Hashisako, Mikiko; Fukuoka, Junya; Nakanishi, Yoichi
• Is Part Of: Respiratory medicine case reports. Volume 25(2018)
• Page Start: 68
• Page End: 72
• Publication Date: 2018
• Subjects: Idiopathic pulmonary hemosiderosis -- Sjögren's syndrome; IPH Idiopathic pulmonary hemosiderosis -- CT computed tomography -- BALF bronchoalveolar lavage fluid -- KL-6 Krebs von den Lungen-6 -- MPO-ANCA myeloperoxidase anti-neutrophil cytoplasmic antibody -- 18F-FDG PET Positron emission tomography with 2-deoxy-2-[fluorine-18]fluoro - d-glucose -- VATS video-assisted thoracic surgery -- NSIP nonspecific interstitial pneumonitis; Respiratory organs -- Diseases -- Periodicals; Chest -- Diseases -- Periodicals; Respiratory Tract Diseases -- Periodicals; Respiratory System -- Periodicals; Chest -- Diseases; Respiratory organs -- Diseases; Electronic journals; Periodicals; 616.2
• DOI: 10.1016/j.rmcr.2018.06.013
• Languages: English
• ISSNs: 2213-0071

Abstract

Abstract: We report a case of recurrent idiopathic pulmonary hemosiderosis after a long-term remission presented with Sjögren's syndrome. The patient was diagnosed with IPH due to repeated pneumonia and blood sputum in his childhood. He was admitted to our hospital due to exertional dyspnea and dry cough with bilateral ground-glass opacity in chest computed tomography at the age of 32. Video-assisted thoracoscopic surgery was performed and the specimens showed nonspecific interstitial pneumonia pattern with diffuse, chronic alveolar hemorrhage, suggesting recurrence of IPH. He was also diagnosed with Sjögren's syndrome. Further immunological studies will reveal the pathogenesis of IPH.