Sickle cell disease (acute chest syndrome)

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, tachypnoea and/or chest pain on a background of established sickle cell disease. 

There is no single underlying aetiology to ACS but rather a variety of infectious and noninfectious causes, including 5:

Typically seen as 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 anaemia).  

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

General imaging differential considerations include:

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Article information

rID: 7343
System: Chest
Synonyms or Alternate Spellings:
  • Acute chest syndrome in sickle cell disease
  • Acute chest syndrome in sickle cell anemia

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