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CASE REPORT
Pulmonary Langerhans cell histiocytosis-associated pulmonary hypertension Report of two cases
1
Pulmonologist-Intensivist, 2nd Department of
Critical Care, National and Kapodistrian University of
Athens Medical School, Attikon Hospital,
Athens, Greece
2
Radiologist, 2nd Department of Radiology, National and Kapodistrian University of
Athens Medical School, Attikon Hospital,
Athens, Greece
3
Professor of Critical Care, 2nd Department of
Critical Care, Clinical Co-director of Pulmonary
Hypertension Clinic,National and Kapodistrian University of
Athens Medical School, Attikon Hospital,
Athens, Greece
4
Cardiologist, 2nd Department of Cardiology,
National and Kapodistrian University of
Athens Medical School, Attikon Hospital,
Athens, Greece
5
Cardiologist-Intensivist, 2nd Department of
Critical Care,National and Kapodistrian University of
Athens Medical School, Attikon Hospital,
Athens, Greece
6
Clinical Nurse, 2nd Department of Critical Care
and 2nd Department of Cardiology,
National and Kapodistrian University of
Athens Medical School, Attikon Hospital,
Athens, Greece
7
Pulmonologist-Intensivist, Professor of
Critical Care & Director of 2nd Department of
Critical Care and Pulmonary Hypertension
Clinic,
National and Kapodistrian University of
Athens Medical School, Attikon Hospital,
Athens, Greece
Corresponding author
Aikaterini Flevari
2nd Department of Critical Care, Attikon Hospital, 1 Rimini street, Haidari, 12462 Athens, Greece
2nd Department of Critical Care, Attikon Hospital, 1 Rimini street, Haidari, 12462 Athens, Greece
Pneumon 2019;32(1-2):43–48
KEYWORDS
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.
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