An incidental finding of occult HIV infection.
PH is a rare systemic manifestation of HIV infection, first reported by Kim et al in 1987 in an end-stage AIDS patient who presented with dyspnea and renal insufficiency. (1) Subsequent series have clearly documented an association between HIV and PH, which can lead to profound right-sided heart failure and a universally poor prognosis. (2,3) The current case is unique because to our knowledge it is the first to describe bilateral leg swelling as the presenting symptom of HIV and associated PH. With improved survival and a more prolonged natural history of disease owing to the widespread use of HAART, it is anticipated that more clinicians will be presented with the important task of managing the late and often systemic manifestations of HIV, such as PH.
PH occurs when mean pulmonary arterial pressure exceeds 25 mm Hg at rest or 30 mm Hg with exercise. Unlike the current case, most patients with PH initially present with fatigue, lethargy, and decreased exercise tolerance. Leg edema can occur but usually exclusively during the late stages of disease when there has been definite progression to right ventricular failure.
The gold standard for the diagnosis of PH is right heart catheterization, but it is often possible to reliably establish the diagnosis with less invasive methods, such as the use of echocardiography to indirectly estimate pulmonary artery pressure. (4) Additional investigations should be performed to rule out more common secondary causes of PH, including collagen vascular conditions, chronic pulmonary emboli, and restrictive lung diseases. The specific diagnosis of HIV-associated PH is often more challenging as the clinical and histopathologic features of this entity do not differ drastically from those of primary or idiopathic PH. Therefore, as in this case, a high index of suspicion is warranted and the diagnosis is frequently confirmed once other causes have been carefully excluded. In this patient, HIV seropositivity was the only laboratory abnormality found in conjunction with pulmonary hypertension.
The underlying etiology and pathophysiology of HIV-associated PH remains unclear. Genetic susceptibility, environmental factors, and viral characteristics are believed to play important roles. The fact that this patient developed HIV-associated PH despite a CD4 lymphocyte count of approximately 500 [mm.sup.3] suggests that the degree of immunodeficiency is a poor predictor of whether an individual will develop PH.
Typically, a combination of supplemental oxygen, bosentan, ACE inhibitors, and calcium channel blockers are used to provide patients with some symptomatic relief. Currently, the role of HAART in the treatment of HIV-associated PH remains uncertain since most studies have been retrospective in nature or have involved only small numbers of patients. (5) More importantly, it is unclear whether HAART influences the progression or prognosis of HIV-associated PH. The widespread use of HAART has altered the natural history of HIV such that more patients are living long enough to display some of the late and systemic manifestations of the infection, such as PH. Although the etiology of PH is quite broad, this case highlights the importance of considering causes like HIV in the differential diagnosis. The presence of bilateral leg swelling in a HIV patient should also raise the clinical suspicion for possible PH and prompt appropriate clinical evaluation.
Winson Y. Cheung, MD
Department of Internal Medicine
University of Manitoba
Winnipeg, Manitoba, Canada
1. Kim KK, Factor SM. Membranoproliferative glomerulonephritis and plexogenic pulmonary arteriopathy in a homosexual man with acquired immunodeficiency syndrome. Hum Pathol 1987;18:1293-1296.
2. Mehta NJ, Khan IA, Mehta RN, et al. HIV-related pulmonary hypertension: analytic review of 131 cases. Chest 2000;118:1133-1141.
3. Petitpretz P, Brenot F, Azarian R, et al. Pulmonary hypertension in patients with human immunodeficiency virus infection. Comparison with primary pulmonary hypertension. Circulation 1994;89:2722-2727.
4. McGoon M, Gutterman D, Steen V, et al. Screening, early detection, and diagnosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest 2004;126(1 suppl):14S-34S.
5. Zuber JP, Calmy A, Evison JM, et al. Pulmonary arterial hypertension related to HIV infection: improved hemodynamics and survival associated with antiretroviral therapy. Clin Infect Dis 2004;38:1178-1185.
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|Author:||Cheung, Winson Y.|
|Publication:||Southern Medical Journal|
|Article Type:||Letter to the editor|
|Date:||Nov 1, 2006|
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