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January - June 2019: 
Volume 32, Issue 1-2

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Pneumon 2019, 32(1-2):43-48, Case Report
Pulmonary Langerhans cell histiocytosis-associated pulmonary hypertension Report of two cases
Authors Information
1Pulmonologist-Intensivist, 2nd Department of Critical Care,  
2Radiologist, 2nd Department of Radiology,  
3Professor of Critical Care, 2nd Department of Critical Care, Clinical Co-director of Pulmonary Hypertension Clinic, 
4Cardiologist, 2nd Department of Cardiology,  
5Cardiologist-Intensivist, 2nd Department of Critical Care,  
6Clinical Nurse, 2nd Department of Critical Care and 2nd Department of Cardiology,  
7Pulmonologist-Intensivist, Professor of Critical Care & Director of 2nd
Abstract

Langerhans cell histiocytosis (LCH) is a multisystemic disease affecting mainly the skeleton and the lungs. It is an uncommon interstitial lung disease whose radiological findings are characterized by centrilobular nodules and cysts of varying sizes of mid to upper lung distribution. Pulmonary LCH can be associated with pulmonary hypertension (PH) which is often severe. We report two cases with Pulmonary Langerhans cell histiocytocis who were referred to our pulmonary hypertension clinic because of echocardiographic signs of severe PH. Right heart catheterization confirmed the presence of precapillary PH in both patients; however in one patient the severity of PH was disproportional to lung disease, as revealed from pulmonary function tests and highresolution computed Tomography chest findings, suggesting pulmonary vascular involvement. We would like to emphasize the wide spectrum of Pulmonary LCH - associated PH and the rationale to treat some patients with specific PAH medication.