Sickle cell disease (acute chest syndrome)
Citation, DOI and article data
Acute chest syndrome (ACS) 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 opacity on chest x-ray 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 to acute chest syndrome but rather a variety of infectious and noninfectious causes, including 5:
- pulmonary infarction
- fat embolism
- rib or sternal infarct causing atelectasis (from splinting)
Point-of-care lung ultrasonography in the 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
May show a mosaic perfusion pattern that could be associated with a pleural effusion. The radiographic signs above may also be seen on CT.
History and etymology
The term was first proposed in patients with sickle cell disease by Charache et al in 1979 as a description for “the combination of chest pain, fever, increased leukocytosis, and appearance of a new shadow on a chest radiograph.
General imaging differential considerations include:
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