Acute and chronic pulmonary complications are a very important cause of morbidity and mortality in sickle cell disease. The Acute Chest Syndrome (ACS) is the most common form of acute pulmonary disease and will be reviewed in this article. Early recognition and aggressive treatment of ACS are important for successful management. The development of pulmonary hypertension is a very important cause of chronic pulmonary disease and contributes to mortality. Because of the increased numbers of sickle cell patients living to adulthood, it is important for primary care providers to become familiar with these complications.

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