Pulmonary Hypertension in Pulmonary Langerhans Cell Granulomatosis
Introduction. Pulmonary Langerhans cell granulomatosis is a rare disease with a variable course. In pulmonary Langerhans cell granulomatosis pulmonary hypertension is frequent and has an independent prognostic impact. A vasculopathy which ist not related to ventilatory disturbance and fibrosis has b...
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| Format: | Article |
| Language: | English |
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Wiley
2012-01-01
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| Series: | Case Reports in Medicine |
| Online Access: | http://dx.doi.org/10.1155/2012/378467 |
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| author | Matthias Held Philipp Schnabel Arne Warth Berthold Jany |
| author_facet | Matthias Held Philipp Schnabel Arne Warth Berthold Jany |
| author_sort | Matthias Held |
| collection | DOAJ |
| description | Introduction. Pulmonary Langerhans cell granulomatosis is a rare disease with a variable course. In pulmonary Langerhans cell granulomatosis pulmonary hypertension is frequent and has an independent prognostic impact. A vasculopathy which ist not related to ventilatory disturbance and fibrosis has been identified. An arteriopathy and even a venulopathy have been described. Due to this possible venulopathy vasodilators carry a significant risk for pulmonary congestion and edema. No drugs have been approved until now. Case Presentation. One female with PLCG developed severe PH four years after primary diagnosis of pulmonary Langerhans cell granulomatosis. Retrospective analysis of lung biopsies revealed an arterial vasculopathy at the time of primary diagnosis without clinical signs of PH at this time. Sildenafil led to a sustained improvement of hemodynamic features and exercise capacity. Conclusion. This paper underlines that patients with PLCG with an arterial vasculopathy-related PH might improve under sildenafil. Further trials addressing treatment of PH and vasculopathy are needed. |
| format | Article |
| id | doaj-art-c64776885bf2413e99861e60f4cc50c1 |
| institution | Kabale University |
| issn | 1687-9627 1687-9635 |
| language | English |
| publishDate | 2012-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Medicine |
| spelling | doaj-art-c64776885bf2413e99861e60f4cc50c12025-02-03T01:30:27ZengWileyCase Reports in Medicine1687-96271687-96352012-01-01201210.1155/2012/378467378467Pulmonary Hypertension in Pulmonary Langerhans Cell GranulomatosisMatthias Held0Philipp Schnabel1Arne Warth2Berthold Jany3Medical Mission Hospital and Department of Internal Medicine, Academic Teaching Hospital, University of Würzburg, Salvatorstrasse 7, 97064 Würzburg, GermanyInstitute of Pathology, Heidelberg University, Im Neuenheimer Feld 220, 69120 Heidelberg, GermanyInstitute of Pathology, Heidelberg University, Im Neuenheimer Feld 220, 69120 Heidelberg, GermanyMedical Mission Hospital and Department of Internal Medicine, Academic Teaching Hospital, University of Würzburg, Salvatorstrasse 7, 97064 Würzburg, GermanyIntroduction. Pulmonary Langerhans cell granulomatosis is a rare disease with a variable course. In pulmonary Langerhans cell granulomatosis pulmonary hypertension is frequent and has an independent prognostic impact. A vasculopathy which ist not related to ventilatory disturbance and fibrosis has been identified. An arteriopathy and even a venulopathy have been described. Due to this possible venulopathy vasodilators carry a significant risk for pulmonary congestion and edema. No drugs have been approved until now. Case Presentation. One female with PLCG developed severe PH four years after primary diagnosis of pulmonary Langerhans cell granulomatosis. Retrospective analysis of lung biopsies revealed an arterial vasculopathy at the time of primary diagnosis without clinical signs of PH at this time. Sildenafil led to a sustained improvement of hemodynamic features and exercise capacity. Conclusion. This paper underlines that patients with PLCG with an arterial vasculopathy-related PH might improve under sildenafil. Further trials addressing treatment of PH and vasculopathy are needed.http://dx.doi.org/10.1155/2012/378467 |
| spellingShingle | Matthias Held Philipp Schnabel Arne Warth Berthold Jany Pulmonary Hypertension in Pulmonary Langerhans Cell Granulomatosis Case Reports in Medicine |
| title | Pulmonary Hypertension in Pulmonary Langerhans Cell Granulomatosis |
| title_full | Pulmonary Hypertension in Pulmonary Langerhans Cell Granulomatosis |
| title_fullStr | Pulmonary Hypertension in Pulmonary Langerhans Cell Granulomatosis |
| title_full_unstemmed | Pulmonary Hypertension in Pulmonary Langerhans Cell Granulomatosis |
| title_short | Pulmonary Hypertension in Pulmonary Langerhans Cell Granulomatosis |
| title_sort | pulmonary hypertension in pulmonary langerhans cell granulomatosis |
| url | http://dx.doi.org/10.1155/2012/378467 |
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