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Sickle cell disease (SCD) is one of the most common hematologic genetic disorders affecting millions of people worldwide. SCD affects virtually any organ in the body; however, its effect is particularly pronounced in the lungs. The pulmonary complications can be acute in the form of the acute chest syndrome that is the leading cause for admission to the hospital, and/or in the form of chronic lung disease that consists of permanent changes in lung function and most importantly of the development of pulmonary hypertension that is the leading cause of death among adult patients with SCD. Despite extensive research, the actual pathogenic mechanisms of the pulmonary complications of SCD remain rather elusive. This study focuses on issues that are controversial or remain unclear such as (a) the association of asthma and airway reactivity with sickle cell lung disease, (b) the effect of SCD on lung function, (c) the use of systemic corticosteroids for the treatment of acute chest syndrome, and (d) the measurement and significance of oxyhemoglobin saturation in SCD.