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Case Reports in Medicine
Volume 2012, Article ID 378467, 5 pages
http://dx.doi.org/10.1155/2012/378467
Case Report

Pulmonary Hypertension in Pulmonary Langerhans Cell Granulomatosis

1Medical Mission Hospital and Department of Internal Medicine, Academic Teaching Hospital, University of Würzburg, Salvatorstrasse 7, 97064 Würzburg, Germany
2Institute of Pathology, Heidelberg University, Im Neuenheimer Feld 220, 69120 Heidelberg, Germany

Received 5 December 2011; Revised 16 December 2011; Accepted 16 December 2011

Academic Editor: Stephen P. Peters

Copyright © 2012 Matthias Held et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

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.