title: When "high" is not very high: high altitude pulmonary edema as first manifestation of sarcoidosis-related pulmonary hypertension.
abstract: High altitude pulmonary edema (HAPE) is a life-threatening complication of high altitude stay, which may occur above altitudes of 2500 m. This is a case report of a healthy 41-year-old man, presenting with recurrent HAPE at moderate altitude. Medical work-up revealed an idiopathic pulmonary artery hypertension (PAH), and specific vasoactive treatment was started. Despite treatment with an endothelin receptor antagonist, the patient deteriorated clinically. Subsequent medical reevaluation showed a significant progress of mediastinal lymphadenopathy. Due to the histological proof of sarcoidosis, the initial diagnosis of PAH had to be changed to sarcoidosis-related pulmonary hypertension. Initiation of immunosuppressive therapy with corticosteroids led to significant and clinically relevant decrease in pulmonary artery pressure, even allowing episodes of asymptomatic re-exposure to moderate altitude. This case describes HAPE as first manifestation of a sarcoidosis-related pulmonary hypertension with a very unusual and early presentation of the underlying disease in an apparently healthy mountaineer.
authors.affiliation: Department of Pulmonary Medicine, University Hospital and University of Bern, Bern, Switzerland.
authors.forename: Anne-Kathrin
authors.initials: AK
authors.lastname: Brill
authors.forename: Alexander
authors.initials: A
authors.lastname: Kunz
authors.forename: Sebastian R
authors.initials: SR
authors.lastname: Ott
authors.forename: Thomas
authors.initials: T
authors.lastname: Geiser
authors.forename: Jacqueline Pichler
authors.initials: JP
authors.lastname: Hefti
mesh_terms: D000328:Adult; D000532:Altitude Sickness / Q000175:diagnosis / Q000209:etiology*; D017809:Fatal Outcome; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000209:etiology*; D008297:Male; D017565:Sarcoidosis, Pulmonary / Q000150:complications / Q000175:diagnosis*