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Acute chest syndrome in adults with sickle cell disease

Joshua J Field, MD
Michael R DeBaun, MD, MPH
Section Editor
Stanley L Schrier, MD
Deputy Editor
Jennifer S Tirnauer, MD


Acute chest syndrome (ACS) is a leading cause of death for patients with sickle cell disease (SCD) [1,2]. Defined as a new radiodensity on chest radiograph accompanied by fever and/or respiratory symptoms, ACS in adults with SCD requires prompt management to prevent clinical deterioration and death [3].

ACS in adults with SCD will be discussed here. ACS in children and adolescents with SCD is discussed separately, although management is largely the same in both settings. (See "The acute chest syndrome in children and adolescents with sickle cell disease".)


The severity of ACS events is categorized as mild, moderate and severe [3]; the definition of ACS and the severity categories apply equally to children and adults. (See 'Diagnostic testing' below.)

A key difference in the clinical presentation of ACS between children and adults with SCD is the greater disease severity and a higher mortality rate in adults, largely due to a higher incidence of bone marrow and fat emboli in adults [4,5]. In adults with SCD, up to 77 percent of ACS episodes are the result of bone marrow or fat emboli [6]; in children, the proportion is much lower [5,7].

The differing etiologies and clinical course of ACS episodes underscore the notion that the definition of ACS (a new radiodensity on chest radiograph with respiratory symptoms) captures a non-specific clinical endpoint that includes different underlying pathogenic processes. As examples, the broad definition of ACS does not differentiate between a patient with SCD who presents with fever, increased respiratory effort, and a new radiodensity due to bronchiolitis requiring a brief inpatient admission (a more common scenario in children) from ACS with intra-pulmonary vasoocclusion and respiratory failure due to fat emboli requiring management in the intensive care unit (a more common scenario in adults).


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Literature review current through: Jan 2017. | This topic last updated: Tue Feb 07 00:00:00 GMT 2017.
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