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Pulmonary manifestations of sickle cell disease

¹ Division of Pulmonary and Critical Care Medicine, Department of Medicine, Long Island Jewish Medical Center, New Hyde Park, the Long Island Campus for the Albert Einstein College of Medicine, Bronx, New York
² Division of Hematology and Oncology

Correspondence to:
Correspondence to:
Dr Shahid Ahmed, Division of Hematology-Oncology, Department of Medicine, Long Island Jewish Medical Center, 270–05, 76th Ave, New Hyde Park, NY, 11040, USA;
shahidahmed00{at}yahoo.com

Pulmonary complications account for significant morbidity and mortality in patients with sickle cell disease. Clinical lung involvement manifests in two major forms: the acute chest syndrome and sickle cell chronic lung disease. Acute chest syndrome is characterised by fever, chest pain, and appearance of a new infiltrate on chest radiograph. Sickle cell chronic lung disease, on the other hand, manifests as radiographic interstitial abnormalities, impaired pulmonary function, and, in its most severe form, by the evidence of pulmonary hypertension. Progress has been made in understanding the pathophysiology and management of these complications. In this review the current knowledge of the mechanism, diagnosis, and treatment of pulmonary complications of sickle cell disease are discussed.

Keywords: sickle cell disease; pulmonary complications; acute chest syndrome

Abbreviations: ACS, acute chest syndrome; MACSS, multicentre acute chest syndrome study; NO, nitric oxide; NYHA, New York Heart Association; SCCLD, sickle cell chronic lung disease; SCD, sickle cell disease; VCAM-1, vascular cell adhesion molecule-1

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