Lung transplant to manage end-stage lung disease due to idiopathic pulmonary hemosiderosis: A review of the literature |
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| Affiliation: | 1. Division of Pulmonary and Critical Care Medicine, Ozarks Medical Center, 1100 N Kentucky Avenue, West Plains, MO, 65775, USA;2. Division of Pulmonary and Critical Care Medicine, Albany Medical College, 43 New Scotland Avenue, Albany, NY, 12208, USA;1. Department of Respiratory Medicine, Nagasaki University Hospital, Nagasaki, Japan;2. Department of Infectious Diseases, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan;3. Department of Internal Medicine, Michinoo Hospital, Nagasaki, Japan;4. Clinical Research Center, Nagasaki University Hospital, Nagasaki, Japan;5. Department of Laboratory Medicine, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan;1. Department of Respiratory Medicine, St. Luke''s International Hospital, Tokyo, Japan;2. Department of Thoracic Surgery, Thoracic Center, St. Luke''s International Hospital, Tokyo, Japan;1. Department of Pulmonary Medicine, Fukushima Medical University, Fukushima, Japan;2. Department of Cardiology, Pulmonology, and Nephrology, Yamagata University Faculty of Medicine, Yamagata, Japan;1. Department of Respiratory Medicine, Graduate School of Medicine, Kyoto University, 54 Kawahara-cho, Shogoin, Sakyo-ku, Kyoto 606-8507, Japan;2. Department of Diagnostic Imaging and Nuclear Medicine, Graduate School of Medicine, 54 Kawahara-cho, Shogoin, Sakyo-ku, Kyoto 606-8507, Japan;3. Division of Clinical Radiology Service, Kyoto University Hospital, 54 Kawahara-cho, Shogoin, Sakyo-ku, Kyoto 606-8507, Japan;1. Department of Respiratory Medicine, Tohoku University Graduate School of Medicine, Sendai, 980 8574, Japan;2. Department of Thoracic Surgery, Institute of Development, Aging and Cancer, Tohoku University, 980 8575, Japan;3. Academic Center of Osaki Citizen Hospital, Miyagi, Japan |
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| Abstract: | Idiopathic pulmonary hemosiderosis (IPH) is a rare immunological disease with a genetic predisposition. It is characterized by recurrent episodes of diffuse alveolar hemorrhage (DAH). Timely use of immunosuppressive medications has significantly improved overall outcomes, including mortality. Still, uncontrolled and frequent episodes of DAH can eventually cause pulmonary fibrosis, leading to end-stage lung disease (ESLD). The objective of the present project was to scrutinize the literature and summarize the demographic, clinical, radiological, and histopathological features, as well as the overall outcomes, in this patient population following lung transplant.The Medline database was searched using the PubMed platform. Articles published in English between 1960 and 2020 were included in the search. Different search terms were used to identify all patients who underwent lung transplantation to manage ESLD due to IPH.Only four cases of lung transplantation have been reported in the literature in patients with IPH. All but one of these underwent deceased donor lung transplant; recurrence was reported in two of these patients and suspected in the third. One patient received living donor lung transplant and had no recurrence during a five-year follow-up. Patients with IPH should not be excluded from lung transplantation because the disease may not recur in all patients, and even when it does recur it can be promptly treated by increasing immunosuppression. |
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| Keywords: | Hemoptysis Pulmonary hemorrhage Idiopathic pulmonary hemosiderosis End-stage lung disease Lung transplantation |
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