Sarcoidosis-Associated Fibrosing Mediastinitis with Resultant Pulmonary Hypertension: A Case Report and Review of the Literature
Pulmonary hypertension, a common manifestation of advanced sarcoidosis, is thought to result from fibrosis with chronic hypoxia and destruction of small vessels, extrinsic compression of pulmonary arteries, or granulomatous vasculitis. We report a case of sarcoidosis-associated pulmonary hypertensio...
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| Veröffentlicht in: | Respiration 2010-01, Vol.79 (4), p.341-345 |
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| creator | Toonkel, Rebecca L. Borczuk, Alain C. Pearson, Gregory D. Horn, Evelyn M. Thomashow, Byron M. |
| description | Pulmonary hypertension, a common manifestation of advanced sarcoidosis, is thought to result from fibrosis with chronic hypoxia and destruction of small vessels, extrinsic compression of pulmonary arteries, or granulomatous vasculitis. We report a case of sarcoidosis-associated pulmonary hypertension due to fibrosing mediastinitis. Our patient presented with cough and dyspnea on exertion and was found to have pulmonary artery enlargement, pulmonary venous compression, and mediastinal soft tissue enhancement on magnetic resonance imaging. Pulmonary hypertension was confirmed by right heart catheterization and sarcoidosis was diagnosed by histologic examination of tissue obtained at mediastinoscopy. Treatment with steroids resulted in decreased pulmonary artery pressures as well as symptomatic improvement. While pulmonary hypertension is a common complication of sarcoidosis, fibrosing mediastinitis is an unusual etiology that should be considered by clinicians. |
| doi_str_mv | 10.1159/000243786 |
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We report a case of sarcoidosis-associated pulmonary hypertension due to fibrosing mediastinitis. Our patient presented with cough and dyspnea on exertion and was found to have pulmonary artery enlargement, pulmonary venous compression, and mediastinal soft tissue enhancement on magnetic resonance imaging. Pulmonary hypertension was confirmed by right heart catheterization and sarcoidosis was diagnosed by histologic examination of tissue obtained at mediastinoscopy. Treatment with steroids resulted in decreased pulmonary artery pressures as well as symptomatic improvement. While pulmonary hypertension is a common complication of sarcoidosis, fibrosing mediastinitis is an unusual etiology that should be considered by clinicians.</description><identifier>ISSN: 0025-7931</identifier><identifier>EISSN: 1423-0356</identifier><identifier>DOI: 10.1159/000243786</identifier><identifier>PMID: 19797887</identifier><identifier>CODEN: RESPBD</identifier><language>eng</language><publisher>Basel, Switzerland: Karger</publisher><subject>Arterial hypertension. Arterial hypotension ; Biological and medical sciences ; Blood and lymphatic vessels ; Cardiology. Vascular system ; Humans ; Hypertension ; Hypertension, Pulmonary - etiology ; Inflammatory diseases ; Male ; Mediastinitis - complications ; Medical sciences ; Middle Aged ; Novel Insights from Clinical Practice ; Pneumology ; Pulmonary arteries ; Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases ; Sarcoidosis ; Sarcoidosis, Pulmonary - complications ; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis ; Steroids</subject><ispartof>Respiration, 2010-01, Vol.79 (4), p.341-345</ispartof><rights>2009 S. Karger AG, Basel</rights><rights>2015 INIST-CNRS</rights><rights>Copyright 2009 S. Karger AG, Basel.</rights><rights>Copyright (c) 2010 S. 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We report a case of sarcoidosis-associated pulmonary hypertension due to fibrosing mediastinitis. Our patient presented with cough and dyspnea on exertion and was found to have pulmonary artery enlargement, pulmonary venous compression, and mediastinal soft tissue enhancement on magnetic resonance imaging. Pulmonary hypertension was confirmed by right heart catheterization and sarcoidosis was diagnosed by histologic examination of tissue obtained at mediastinoscopy. Treatment with steroids resulted in decreased pulmonary artery pressures as well as symptomatic improvement. While pulmonary hypertension is a common complication of sarcoidosis, fibrosing mediastinitis is an unusual etiology that should be considered by clinicians.</description><subject>Arterial hypertension. Arterial hypotension</subject><subject>Biological and medical sciences</subject><subject>Blood and lymphatic vessels</subject><subject>Cardiology. Vascular system</subject><subject>Humans</subject><subject>Hypertension</subject><subject>Hypertension, Pulmonary - etiology</subject><subject>Inflammatory diseases</subject><subject>Male</subject><subject>Mediastinitis - complications</subject><subject>Medical sciences</subject><subject>Middle Aged</subject><subject>Novel Insights from Clinical Practice</subject><subject>Pneumology</subject><subject>Pulmonary arteries</subject><subject>Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases</subject><subject>Sarcoidosis</subject><subject>Sarcoidosis, Pulmonary - complications</subject><subject>Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. 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| ispartof | Respiration, 2010-01, Vol.79 (4), p.341-345 |
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| source | MEDLINE; Karger Journals; Alma/SFX Local Collection |
| subjects | Arterial hypertension. Arterial hypotension Biological and medical sciences Blood and lymphatic vessels Cardiology. Vascular system Humans Hypertension Hypertension, Pulmonary - etiology Inflammatory diseases Male Mediastinitis - complications Medical sciences Middle Aged Novel Insights from Clinical Practice Pneumology Pulmonary arteries Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases Sarcoidosis Sarcoidosis, Pulmonary - complications Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis Steroids |
| title | Sarcoidosis-Associated Fibrosing Mediastinitis with Resultant Pulmonary Hypertension: A Case Report and Review of the Literature |
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