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Acute chest syndrome in sickle cell

Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

Authoring team

Sickle cell crises in the lung - otherwise referred to as acute chest syndrome (ACS) - are characterised by pleuritic chest pains, fever, abnormal chest examinations and presence of new infiltrations in the chest X ray (1).

  • is the second most common cause of hospital admission in SCD patients (2)
  • study evidence showed that 50% of patients with sickle cell crises in the lung, had a pain event in the two weeks preceding the event (3)
  • around half of all SCD patients will experience at least one episode of ACS during their lives (4)
  • in some areas, the acute chest syndrome is the commonest cause of death in sickle cell anaemia.

They are of mixed pathology; caused by a combination of infection, fat embolism and vaso occlusion of the pulmonary vasculature (2)

  • mostly seen during early childhood (usually presents with clinical features that are more typical for pneumonia) (2)
  • is a combination of pulmonary infarction, infection, and fat embolism (3)
  • caused by sickling and ischaemia in deep tissues of the chest wall (3)
  • a review describes typical features as fever, acute respiratory signs and symptoms, such as cough, wheezing, dyspnoea, crepitations or chest pain, and consolidation on chest radiograph (3)
  • in later childhood and adulthood, it is more frequent during a painful crises or after anaesthesia (1)

A combination of clinical signs or symptoms of chest consolidation and worsening hypoxia is sufficient for the diagnosis of ACS (since X ray changes often lag behind clinical signs) (4)

Local protocols should be prepared for management of ACS and should include:

  • analgesia
  • monitoring arterial blood gas measurement
  • oxygen and respiratory support - persistent, profound hypoxia is an indication for ventilation.
  • incentive spirometry – useful in SCD patients presenting with chest or back pain above the diaphragm to prevent pulmonary complications
  • fluid management
  • bronchodilator therapy – for patients with evidence of wheeze or reversible airways disease, or a history of asthma
  • antibiotic treatment may be indicated
  • blood transfusion (4)

Longterm treatment with hydroxycarbamide (former British Approved Name, hydroxyurea) has led to a reduction by 50% in acute chest syndrome in some studies.


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