![](/images/graphics-bg.png)
Pulmonary Hypertension in Pulmonary Langerhans Cell Granulomatosis
Joint Authors
Schnabel, Philipp
Jany, Berthold
Held, Matthias
Warth, Arne
Source
Issue
Vol. 2012, Issue 2012 (31 Dec. 2012), pp.1-5, 5 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2012-03-13
Country of Publication
Egypt
No. of Pages
5
Main Subjects
Abstract EN
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.
American Psychological Association (APA)
Held, Matthias& Schnabel, Philipp& Warth, Arne& Jany, Berthold. 2012. Pulmonary Hypertension in Pulmonary Langerhans Cell Granulomatosis. Case Reports in Medicine،Vol. 2012, no. 2012, pp.1-5.
https://search.emarefa.net/detail/BIM-467322
Modern Language Association (MLA)
Held, Matthias…[et al.]. Pulmonary Hypertension in Pulmonary Langerhans Cell Granulomatosis. Case Reports in Medicine No. 2012 (2012), pp.1-5.
https://search.emarefa.net/detail/BIM-467322
American Medical Association (AMA)
Held, Matthias& Schnabel, Philipp& Warth, Arne& Jany, Berthold. Pulmonary Hypertension in Pulmonary Langerhans Cell Granulomatosis. Case Reports in Medicine. 2012. Vol. 2012, no. 2012, pp.1-5.
https://search.emarefa.net/detail/BIM-467322
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-467322