Sickle cell disease (acute chest syndrome)

Last revised by Travis Fahrenhorst-Jones on 24 May 2023

Acute chest syndrome in sickle cell disease is a leading thoracic complication - as well as leading cause of mortality - in those affected by sickle cell disease. The diagnosis is made on the combination of new pulmonary opacities on chest radiograph with at least one new clinical symptom or sign. 

For a general discussion of sickle cell disease, please refer to sickle cell disease.

Patients may present with acute fever, cough, wheezing, tachypnea and/or chest pain on a background of established sickle cell disease. 

There is no single underlying etiology in acute chest syndrome but rather a variety of infectious and non-infectious causes including 5:

Chest radiograph typically shows segmental or subsegmental atelectasis/consolidation with a lower lobe predilection, and/or pleural effusion

A chest radiograph may also show other sequelae from sickle cell disease such as bone infarcts, rib enlargement, and cardiomegaly (from anemia).  

Point-of-care lung ultrasonography in acute chest syndrome may reveal one of the following patterns;

  • alveolar consolidation

    • the most common abnormality found, with a posterobasal regional predilection

    • air bronchograms may be visualized

  • anterior subpleural consolidations

  • lung rockets

    • three or more B-lines per sonographic field, typically 3 cm apart (B3 lines): defines the sonographic interstitial syndrome

    • bilateral diffuse anterolateral interstitial syndrome may be observed 7

  • pleural effusions 9

CT may show a mosaic perfusion pattern that may be associated with a pleural effusion. The radiographic signs above may also be seen on CT.

Broad-spectrum antibiotics, bronchodilators, and oxygen form the basis of treatment 12. Although severity is variable, 13% of patients with acute chest syndrome require ventilation and 3% do not survive 12.

The term was first proposed in patients with sickle cell disease by Charache et al. in 1979 to describe the combination of chest pain, fever, leukocytosis, and the appearance of a new opacity on a chest radiograph.

General imaging differential considerations include:

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Cases and figures

  • Case 1: plain radiograph
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  • Case 1: CT chest
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  • Case 2
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