title: Chronic sickle cell lung disease: new insights into the diagnosis, pathogenesis and treatment of pulmonary hypertension.
abstract: Pulmonary hypertension is a common complication of sickle cell disease (SCD). In spite of the mild elevations in pulmonary artery pressures in these patients, the associated morbidity and mortality is high. In fact, in adult patients with SCD, pulmonary hypertension is emerging as the major independent risk factor for death. The aetiology of pulmonary hypertension is probably multifactorial, including haemolysis, impaired nitric oxide bioavailability, chronic hypoxaemia, thromboembolism, parenchymal and vascular injury because of sequestration of sickle erythrocytes, chronic liver disease and asplenia. Interestingly, pulmonary hypertension is emerging as a common, and probably, invariant sequella of lifelong haemolytic anaemia in other hereditary and acquired haemolytic diseases, such as thalassaemia, stomatocytosis and spherocytosis. There are currently limited specific data on the effects of any treatment modality for pulmonary hypertension in patients with SCD. It is likely that maximization of SCD therapy, in all patients, and treatment with selective pulmonary vasodilators and antiproliferative agents, in patients with severe disease, would be beneficial. A large trial evaluating the effects of therapy for pulmonary hypertension in the SCD population is clearly indicated.
authors.affiliation: Vascular Therapeutics Section, Cardiovascular Branch, National Heart Lung and Blood Institute and Critical Care Medicine Department, Clinical Center, National Institutes of Health, 9000 Rockville Pike, Bethesda, MD 20892, USA. robertom@nhlbi.nih.gov
authors.forename: Roberto F
authors.initials: RF
authors.lastname: Machado
authors.forename: Mark T
authors.initials: MT
authors.lastname: Gladwin
mesh_terms: D000755:Anemia, Sickle Cell / Q000150:complications* / Q000401:mortality; D006328:Cardiac Catheterization; D002908:Chronic Disease; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D008168:Lung / Q000000981:diagnostic imaging / Q000503:physiopathology; D020030:Nitric Oxide Donors / Q000627:therapeutic use; D011453:Prostaglandins / Q000627:therapeutic use; D011877:Radionuclide Imaging; D012129:Respiratory Function Tests; D015996:Survival Rate; D014057:Tomography, X-Ray Computed; D014692:Ventilation-Perfusion Ratio
chemical_list: D020030:Nitric Oxide Donors; D011453:Prostaglandins