The acute chest syndrome of sickle cell disease

Acute chest syndrome (ACS), a leading cause of morbidity and mortality in sickle cell disease (SCD), is an acute illness characterized by fever and/or respiratory symptoms, accompanied by a new pulmonary infiltrate on a chest X-ray. There is increasing knowledge regarding the etiology and pathogenesis of ACS in SCD. A high index of suspicion is required for the diagnosis of ACS. Treatment of ACS involves the judicious use of intravenous fluids and analgesics, aggressive incentive spirometer and pulmonary toileting, antibiotics and transfusion therapy.

This review evaluates the epidemiology, clinical and laboratory presentation, etiology and pathogenesis of ACS. It also reviews the standard treatments as well as experimental treatments in ACS.

Despite an increased understanding of its etiology and pathogenesis, ACS remains a leading cause of morbidity and mortality in SCD. In patients admitted with a painful crisis, there is need for a high index of suspicion, as pain episodes may be a prodrome for the development of ACS. Patients with a diagnosis of ACS should be aggressively managed to prevent clinical deterioration. Clinical trials using novel drugs for the treatment of ACS are greatly warranted.