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Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pulmonary arterial hypertension (PAH) is a progressive arteriopathy of the pulmonary circulation that can affect a wide group of patients. Presentation and natural history of PAH are intimately linked to progressive right ventricular failure. Historically, survival in PAH has been poor, especially for patients with PAH associated with progressive systemic sclerosis and human immunodeficiency virus. Oral, subcutaneous, and intravenous therapies that have emerged during the last decade can improve symptoms and hemodynamics. This review explores the impact of these therapies on prognosis in PAH, which ultimately rests on the ability to reverse the pulmonary arteriopathy and preserve right ventricular function.
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PMID:Changing the prognosis of pulmonary arterial hypertension: impact of medical therapy. 1612 18

Pulmonary arterial hypertension (PAH) is a pathological condition of the small pulmonary arteries. PAH is characterized histopathologically by vasoconstriction, vascular proliferation, in situ thrombosis, and remodeling of all 3 levels of the vascular walls. These pathologic changes result in progressive increases in the mean pulmonary-artery pressure and pulmonary vascular resistance, which, if untreated, leads to right-ventricular failure and death. PAH can be associated with multiple conditions or risk factors (eg, collagen vascular diseases, liver disease, human immunodeficiency virus, congenital heart disease, or ingestion of certain medications or toxins) or it can be idiopathic. Up to 10% of the idiopathic cases are familial. Regardless of the etiology, the clinical presentation, histopathologic lesions, and response to therapy are all similar. Early in the disease process, the signs and symptoms of PAH are often subtle and nonspecific, making diagnosis challenging. Patients most often present with progressively worsening dyspnea and fatigue. An extensive evaluation is indicated to diagnose PAH, decipher its etiology, and determine long-term treatment goals. Transthoracic echocardiogram is an excellent screening tool to evaluate PAH, but every patient requires a right-side heart catheterization to help stage the disease and guide therapy. Prior to a decade ago, clinicians were only able to offer symptomatic therapy to this challenging group of patients. Earlier diagnosis, rapidly advancing understanding of the pathogenesis, and an increasing number of treatment options have changed the course of PAH, which was once thought to be invariably fatal.
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PMID:Diagnosis and management of pulmonary arterial hypertension: Implications for respiratory care. 1656 91

Pulmonary hypertension (PH), defined as a mean pulmonary arterial (PA) pressure of >25 mmHg at rest or >30 mmHg during exercise, is characterized by a progressive and sustained increase in pulmonary vascular resistance that eventually leads to right ventricular failure. Clinically, PH may result from a variety of underlying diseases (Table 1 and Refs. 50, 113, 124). Pulmonary arterial hypertension (PAH) may be familial (FPAH) or sporadic (idiopathic, IPAH), formerly known as primary pulmonary hypertension, i.e., for which there is no demonstrable cause. More often, PAH is due to a variety of identifiable diseases including scleroderma and other collagen disorders, liver disease, human immunodeficiency virus, and the intake of appetite-suppressant drugs such as phentermine and fenfluramine (72). Other, more common, causes of PAH include left ventricular failure (perhaps the most common cause), valvular lesions, chronic pulmonary diseases, sleep-disordered breathing, and prolonged residence at high altitude. This classification, now widely accepted, was first proposed at a meeting in Evian, France, in 1998, and modified in Venice, Italy, in 2003 (124).
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PMID:Mediators and modulators of pulmonary arterial hypertension. 1669 50

Pulmonary arterial hypertension (PAH) affects approximately 0.5% of human immunodeficiency virus (HIV)-infected adults with poor prognosis. The effectiveness of highly active antiretroviral therapy for treatment of HIV-related PAH (HIV-PAH) remains controversial. Little is known about the incidence, clinical course, and therapy options for PAH in HIV-1-infected pediatric patients. Here, we report the case of a preterm infant with HIV-related life-threatening PAH, which resolved after initiation of highly active antiretroviral therapy.
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PMID:Resolution of human immunodeficiency virus type 1 infection-related severe pulmonary hypertension in a very low-birth-weight infant. 1844 67

Pulmonary arterial hypertension [PAH] is a rare but well-known cardiovascular condition potentially associated with human immunodeficiency virus [HIV] infection and is currently recognized to be one of the most ominous noninfectious HIV complications. Although there is no clear evidence supporting the use of any medication for the treatment of HIV-related PAH, many of the currently available agents have been shown to exert some clinical benefits HIV-PAH patients. To date, no data are available regarding the potential effects of sitaxsentan, a selective endothelin type-A receptor antagonist, in this peculiar patient population. We report the case of a successful switch to sitaxsentan in a HIV-infected patient with PAH initially receiving bosentan who developed a late treatment-related side-effect.
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PMID:Successful switch to sitaxsentan in a patient with HIV-related pulmonary arterial hypertension and late intolerance to nonselective endothelin receptor blockade. 1929 98

Pulmonary arterial hypertension (PAH) is a distinct subgroup of pulmonary hypertension that comprises idiopathic PAH, familial/heritable forms, and PAH associated with connective tissue disease, congenital heart disease, portal hypertension, human immunodeficiency virus (HIV) infection, and some other conditions. The hemodynamic definition of PAH was recently revised: PAH is now defined by a mean pulmonary artery pressure at rest > or =25 mm Hg in the presence of a pulmonary capillary wedge pressure < or =15 mm Hg. The exercise criterion (mean pulmonary artery pressure > or =30 mm Hg during exercise) that was used in the old definition of PAH has been removed because there are no robust data that would allow defining an upper limit of normal for the pulmonary pressure during exercise. The revised classification of pulmonary hypertension still consists of five major groups: (1) PAH, (2) pulmonary hypertension due to left heart disease, (3) pulmonary hypertension due to chronic lung disease and/or hypoxia, (4) chronic thromboembolic pulmonary hypertension, and (5) miscellaneous forms. Modifications have been made in some of these groups, such as the addition of schistosomiasis-related pulmonary hypertension and pulmonary hypertension in patients with chronic hemolytic anemia to group 1.
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PMID:Definition, classification, and epidemiology of pulmonary arterial hypertension. 1963 76

Pulmonary arterial hypertension (PAH) is a rare but life-threatening complication of human immunodeficiency virus (HIV) infection. PAH can complicate the course of HIV infection regardless of the route of HIV transmission, the stage of HIV infection, and the degree of immunosuppression. The clinical presentation and underlying pathology of PAH associated with HIV infection (PAH-HIV) are similar to those encountered in other forms of PAH, although there are data suggesting a greater inflammatory component in the HIV-related form. Given the good long-term prognosis of HIV patients with highly active antiretroviral treatments and the severity of PAH in HIV-infected patients, screening for pulmonary hypertension according to a precise algorithm is warranted in HIV-infected patients presenting with dyspnea not explained by another cause. In all cases, right heart catheterization must be performed to establish the diagnosis of PAH. Beneficial effects of antiretroviral treatments on PAH-HIV still remain to be proven. Patients with PAH-HIV appear to respond well to treatment with the prostacyclin epoprostenol, although continuous intravenous infusion is associated with a range of potential complications. Treatment with the oral dual endothelin receptor antagonist bosentan has been shown to benefit patients with PAH-HIV without adversely affecting the control of HIV infection, and resulted in functional and hemodynamic normalization in approximately 20% of patients. Other PAH therapies, including prostacyclin analogs, type 5 phosphodiesterase inhibitors, and single endothelin receptor antagonists, have yet to be evaluated in PAH-HIV.
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PMID:Pulmonary arterial hypertension and HIV infection. 1963 83

Pulmonary arterial hypertension (PAH) is a life-threatening complication of HIV infection. The prevalence of HIV-associated PAH (HIV-PAH) seems not to be changed over time, regardless of the introduction of highly active antiretroviral therapy (HAART). HIV-PAH treatment is similar to that for all PAH conditions and includes lifestyle modifications, general treatments, and disease-specific treatments. We reviewed the cases of HIV-PAH reported in the Literature in order to evaluate the role of HAART and specific PAH therapy in the prognosis and outcome of HIV-PAH. The research was performed through the PubMed database, by using the following key words: human immunodeficiency virus, AIDS, pulmonary hypertension, antiretroviral, and treatment. The outcome was reported as survival at the end of the observation period of each study. We found 509 patients with HIV-PAH described in the literature to date. At the end of follow-up period, survival rates were 55% and 22% among patients treated or not with antiretroviral therapy (ART), respectively (p = 0.02). Moreover, survival rates at the end of follow-up were 76% and 32% among patients treated or not with specific therapy for PAH (PAH-ST), respectively (p<0.0000001). Survival rates were 69% and 38% among patients treated or not with ART and PAH-ST, respectively (p = 0.02). Specific therapy for PAH should be strongly recommended in patients with HIV-PAH. The role of the HAART in influencing the outcome of HIV-PAH is controversial, even if some evidences seem to indicate a beneficial effect in the clinical course of the disease.
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PMID:Treatment and outcome of pulmonary arterial hypertension in HIV-infected patients: a review of the literature. 1992 93

Pulmonary arterial hypertension (PAH), which can be a complication of human immunodeficiency virus (HIV) infection, is characterized by increased pulmonary arterial pressure and peripheral vascular resistance, subsequently leading to right heart failure. In HIV-infected patients, the management of PAH is challenging given the potential drug interactions between PAH-specific vasodilators and antiretroviral drugs. We describe a 51-year-old female with acquired immunodeficiency syndrome (AIDS) and HIV-associated PAH. She was treated with the oral endothelin receptor antagonist bosentan while taking a nevirapine (a nonnucleoside reverse transcriptase inhibitor)-based antiretroviral regimen. Due to concerns about potential drug interactions with the antiretroviral therapy, her nevirapine plasma concentration, as well as CD4(+) cell count and viral load, were continuously monitored. We observed no interaction between bosentan and nevirapine during a 4-year period. To our knowledge, this report is the first to demonstrate successful, long-term coadministration of bosentan and a nonnucleoside reverse transcriptase inhibitor.
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PMID:Successful bosentan and nonnucleoside reverse transcriptase inhibitor-based therapy in a patient with acquired immunodeficiency syndrome and pulmonary arterial hypertension. 2033 65

Extract: Pulmonary hypertension (PH) was previously termed primary (idiopathic or of unknown origin, i.e., spontaneous) or secondary (as a result of another disorder) pulmonary hypertension. It is now clear, however, that many of the entities labeled as secondary pulmonary hypertension resemble primary pulmonary hypertension in both their histopathological features and their response to treatment. For this reason, the World Health Organization (WHO) has recently classified PH into five groups on the basis of their proposed underlying mechanism. Group I in this classification, designated pulmonary arterial hypertension, is the focus of this overview. Pulmonary arterial hypertension (PAH) is defined as a sustained elevation of the pulmonary arterial pressure to greater than 25 mmHg at rest or greater than 30 mmHg following exercise, with a mean pulmonary-capillary wedge pressure (an indirect measure of left atrial pressure) of less than 15 mmHg. The entities within the category of PAH include idiopathic pulmonary arterial hypertension (IPAH; formerly, primary pulmonary hypertension), PAH associated with collagen vascular disease (e.g., in limited systemic sclerosis), portal hypertension (high pressure in the vessel that carries blood from the intestines to the liver), congenital left-to-right intra-cardiac shunts, infection with the human immunodeficiency virus (HIV), and persistent pulmonary hypertension of the newborn. The histological appearance of lung vessels in each of these conditions is similar: intimal fibrosis (formation of fibrous material in the inner lining of the blood vessel), increased medial thickness, pulmonary arteriolar occlusion (block of arterioles [the vessels that join the arteries and capillaries] in the lung), and plexiform (web-like) lesions predominate.
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PMID:Mechanism of disease: Pulmonary hypertension. 2070 29


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