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(Circulation. 1998;97:1111-1113.)
© 1998 American Heart Association, Inc.

Editorials

High-Altitude Pulmonary Edema

An Immunogenetically Mediated Disease?

Frank C. Arnett, MD

From the Division of Rheumatology (Elizabeth Bidgood Chair in Rheumatology) and Clinical Immunogenetics, Department of Internal Medicine, and Department of Pathology and Laboratory Medicine, University of Texas–Houston Medical School.

Correspondence to Frank C. Arnett, MD, Division of Rheumatology, University of Texas–Houston Medical School, 6431 Fannin, Room 5.268 MSB, Houston, TX 77030.

Key Words: Editorials • genetics • hypertension, pulmonary • immune system • risk factors

High-altitude pulmonary edema (HAPE), a potentially life-threatening complication of acute mountain sickness, is postulated to be a noncardiogenic permeability edema caused by acute pulmonary arteriolar vasoconstriction and resultant pulmonary hypertension in response to the hypoxia of rapid ascent to high altitudes.^{1 2 3 4} HAPE typically occurs unexpectedly in young, otherwise healthy mountaineers. A constitutional susceptibility has been noted for some time^{5 6} ; the disease tends to recur in the same individuals on reexposure to high altitude, whereas others appear not to be susceptible at all. The basis for this predisposition to HAPE, whether genetic or environmental, and its underlying pathophysiology remain poorly understood.

In this issue of Circulation, Hanaoka and colleagues⁷ present interesting new evidence that certain human leukocyte antigens (HLA) are increased in Japanese patients with HAPE, especially those with recurrent disease. HLA-DR6 and/or HLA-DQ4 were found to each occur significantly more often in Japanese patients with HAPE than in a large number of normal Japanese control subjects. Moreover, the HLA-DR6–positive patients with HAPE had significantly higher pulmonary arterial pressures than did their HLA-DR6–negative counterparts with HAPE. The authors speculate that at least some cases of HAPE are immunogenetically mediated, perhaps through an inherent HLA-associated susceptibility to pulmonary hypertension. They cite several recent studies reporting HLA associations, specifically HLA-DR6, with pulmonary hypertension complicating other diseases, namely scleroderma and human immunodeficiency virus (HIV) infection.^{8 9}

What is implied by the finding of an HLA association with a disease? First, HLA antigens are cell surface molecules encoded by a cluster of highly polymorphic . . . [Full Text of this Article]