Acute chest syndrome (ACS) is a potentially life-threatening complication of sickle cell disease characterized by lung infiltrates, fever and respiratory symptoms, including cough, tachypnea, wheeze, increased work of breathing / shortness of breath, and reduced oxygen saturations. ACS is the second most common reason for hospitalization in children and adults with sickle cell disease and is the leading cause of mortality. Management of ACS in children and adults is similar, although the disease is often more severe in adults and associated with bone marrow and fat emboli. ACS can be triggered by an underlying infection or vaso-occlusive crisis, although in the majority of cases, an identifiable trigger is not determined.
Sickle cell disease is the primary risk factor for ACS. Within the population with sickle cell disease, the following are predictors for increased risk of developing ACS: young age, low fetal hemoglobin, leukocytosis, genotypes HbSS (sickle cell anemia) and HbSβ0, asthma, tobacco exposure, recent surgery, or 3 or more severe veno-occlusive crises in the past year.
Management requires hospitalization for hydration, oxygenation, intravenous (IV) antibiotics, pain control, and blood transfusions and, in some cases, exchange transfusion.