Acute chest syndrome (ACS), defined as the presence of a new lung infiltrates in a patient with acute onset of lower respiratory symptoms such as cough and shortness of breath, is less common than VOC (vascular occlusion syndrome) but potentially life-threatening. In children, it often presents with fever and signs of middle lobe lung involvement, whereas adults are often afebrile and have multilobe infiltrates. ACS can present on its own or as a complication of a VOC; it requires prompt evaluation and, once diagnosed, early intervention with antibiotic therapy and hospitalization. During hospitalization for a VOC, incentive spirometry can reduce the risk of ACS. When evaluating a new lung infiltrate in a patient with SCD, physicians should consider ACS before assuming the infiltrate represents community-acquired pneumonia.

Fever higher than 101°F (38.3°C), even in the absence of other signs of infection, should be evaluated carefully. Because of absent or reduced splenic function, persons with SCD have a high risk of overwhelming bacterial infections or sepsis. Fevers with possible infections should be treated empirically until culture results are available.

From: Management of Sickle Cell Disease: Recommendations from the 2014 Expert Panel Report