Sarcoidosis-Associated Pulmonary Hypertension
Sarcoidosis is a granulomatous disorder of unknown etiology characterized by multisystem non-caseating granulomas. Pulmonary hypertension (PH) is a well-known complication of sarcoidosis and is associated with increased morbidity and mortality. The actual epidemiology of sarcoidosis-associated PH (S...
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MDPI AG
2025-02-01
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| Series: | Medicina |
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| Online Access: | https://www.mdpi.com/1648-9144/61/2/342 |
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| author | Yoshitaka Morimatsu Nobuhiro Tahara Masaki Okamoto Munehisa Bekki Atsuko Tahara Yoshiko Eto Tadahiro Kugai Yuki Koga Shoko Maeda-Ogata Akihiro Honda Sachiyo Igata Yoshiaki Zaizen Shuichi Tanoue Tomoaki Hoshino Tatsuya Ishitake Yoshihiro Fukumoto |
| author_facet | Yoshitaka Morimatsu Nobuhiro Tahara Masaki Okamoto Munehisa Bekki Atsuko Tahara Yoshiko Eto Tadahiro Kugai Yuki Koga Shoko Maeda-Ogata Akihiro Honda Sachiyo Igata Yoshiaki Zaizen Shuichi Tanoue Tomoaki Hoshino Tatsuya Ishitake Yoshihiro Fukumoto |
| author_sort | Yoshitaka Morimatsu |
| collection | DOAJ |
| description | Sarcoidosis is a granulomatous disorder of unknown etiology characterized by multisystem non-caseating granulomas. Pulmonary hypertension (PH) is a well-known complication of sarcoidosis and is associated with increased morbidity and mortality. The actual epidemiology of sarcoidosis-associated PH (SAPH) remains unknown, and its pathogenesis has not been fully elucidated. SAPH is classified under the miscellaneous category (group 5 of the PH classification). The clinical presentation of SAPH is variable and not always proportional to the severity of sarcoidosis. Appropriate management for SAPH by an experienced physician is important; however, no treatment algorithm for SAPH has been established. Lung transplantation should be considered in refractory cases. Pulmonary arterial hypertension-specific vasodilators targeting the endothelin pathway, nitric oxide pathway, and prostacyclin pathway have improved the clinical functions and hemodynamics in some patients with SAPH. |
| format | Article |
| id | doaj-art-a4d947d32a0f47cbb1e209ac6c81fd94 |
| institution | OA Journals |
| issn | 1010-660X 1648-9144 |
| language | English |
| publishDate | 2025-02-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Medicina |
| spelling | doaj-art-a4d947d32a0f47cbb1e209ac6c81fd942025-08-20T02:03:40ZengMDPI AGMedicina1010-660X1648-91442025-02-0161234210.3390/medicina61020342Sarcoidosis-Associated Pulmonary HypertensionYoshitaka Morimatsu0Nobuhiro Tahara1Masaki Okamoto2Munehisa Bekki3Atsuko Tahara4Yoshiko Eto5Tadahiro Kugai6Yuki Koga7Shoko Maeda-Ogata8Akihiro Honda9Sachiyo Igata10Yoshiaki Zaizen11Shuichi Tanoue12Tomoaki Hoshino13Tatsuya Ishitake14Yoshihiro Fukumoto15Department of Environmental Medicine, Kurume University School of Medicine, Kurume 830-0011, JapanDivision of Cardiovascular Medicine, Department of Medicine, Kurume University School of Medicine, Kurume 830-0011, JapanDepartment of Respirology, NHO Kyushu Medical Center, Fukuoka 810-0065, JapanDivision of Cardiovascular Medicine, Department of Medicine, Kurume University School of Medicine, Kurume 830-0011, JapanDivision of Cardiovascular Medicine, Department of Medicine, Kurume University School of Medicine, Kurume 830-0011, JapanDivision of Cardiovascular Medicine, Department of Medicine, Kurume University School of Medicine, Kurume 830-0011, JapanDivision of Cardiovascular Medicine, Department of Medicine, Kurume University School of Medicine, Kurume 830-0011, JapanDivision of Cardiovascular Medicine, Department of Medicine, Kurume University School of Medicine, Kurume 830-0011, JapanDivision of Cardiovascular Medicine, Department of Medicine, Kurume University School of Medicine, Kurume 830-0011, JapanDivision of Cardiovascular Medicine, Department of Medicine, Kurume University School of Medicine, Kurume 830-0011, JapanDivision of Cardiovascular Medicine, Department of Medicine, Kurume University School of Medicine, Kurume 830-0011, JapanDivision of Respirology, Neurology and Rheumatology, Department of Internal Medicine, Kurume University School of Medicine, Kurume 830-0011, JapanDepartment of Radiology, Kurume University School of Medicine, Kurume 830-0011, JapanDivision of Respirology, Neurology and Rheumatology, Department of Internal Medicine, Kurume University School of Medicine, Kurume 830-0011, JapanDepartment of Environmental Medicine, Kurume University School of Medicine, Kurume 830-0011, JapanDivision of Cardiovascular Medicine, Department of Medicine, Kurume University School of Medicine, Kurume 830-0011, JapanSarcoidosis is a granulomatous disorder of unknown etiology characterized by multisystem non-caseating granulomas. Pulmonary hypertension (PH) is a well-known complication of sarcoidosis and is associated with increased morbidity and mortality. The actual epidemiology of sarcoidosis-associated PH (SAPH) remains unknown, and its pathogenesis has not been fully elucidated. SAPH is classified under the miscellaneous category (group 5 of the PH classification). The clinical presentation of SAPH is variable and not always proportional to the severity of sarcoidosis. Appropriate management for SAPH by an experienced physician is important; however, no treatment algorithm for SAPH has been established. Lung transplantation should be considered in refractory cases. Pulmonary arterial hypertension-specific vasodilators targeting the endothelin pathway, nitric oxide pathway, and prostacyclin pathway have improved the clinical functions and hemodynamics in some patients with SAPH.https://www.mdpi.com/1648-9144/61/2/342sarcoidosissarcoidosis-associated pulmonary hypertensionepidemiologypathogenesistreatment |
| spellingShingle | Yoshitaka Morimatsu Nobuhiro Tahara Masaki Okamoto Munehisa Bekki Atsuko Tahara Yoshiko Eto Tadahiro Kugai Yuki Koga Shoko Maeda-Ogata Akihiro Honda Sachiyo Igata Yoshiaki Zaizen Shuichi Tanoue Tomoaki Hoshino Tatsuya Ishitake Yoshihiro Fukumoto Sarcoidosis-Associated Pulmonary Hypertension Medicina sarcoidosis sarcoidosis-associated pulmonary hypertension epidemiology pathogenesis treatment |
| title | Sarcoidosis-Associated Pulmonary Hypertension |
| title_full | Sarcoidosis-Associated Pulmonary Hypertension |
| title_fullStr | Sarcoidosis-Associated Pulmonary Hypertension |
| title_full_unstemmed | Sarcoidosis-Associated Pulmonary Hypertension |
| title_short | Sarcoidosis-Associated Pulmonary Hypertension |
| title_sort | sarcoidosis associated pulmonary hypertension |
| topic | sarcoidosis sarcoidosis-associated pulmonary hypertension epidemiology pathogenesis treatment |
| url | https://www.mdpi.com/1648-9144/61/2/342 |
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