Acute Chest Syndrome in Sickle Cell - Quick Consult
Last Updated / Reviewed: June 2024

Definition
Pathophysiology
Key History
Key Physical Exam
Diagnostic Testing
Treatment
Complications

Definition

Acute chest syndrome (ACS) is a type of sickle cell crisis with pulmonary symptoms and a new pulmonary infiltrate found on radiograph. The patient may have pleuritic chest pain, shortness of breath, cough, fever, hypoxia and/or tachypnea.

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Pathophysiology

The exact etiology of the chest syndrome is unclear, but infection, infarction (ribs and/or lung), and pulmonary fat embolism (from ischemic marrow fat necrosis) all have been implicated.

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Key History

  • Pleuritic chest pain
  • Shortness of breath
  • Cough, which may contain blood
  • Fever
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Key Physical Exam

  • Hypoxia
  • May have bronchospasm
  • Tachypnea
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Diagnostic Testing

Current recommendations include:
  • Complete blood count (CBC) with white blood cell differential to assess for infection and the degree of anemia.
  • Reticulocyte count to evaluate bone marrow response.
  • Blood cultures to identify potential bacterial pathogens.
  • Sputum cultures and nasopharyngeal aspirate for immunofluorescence or polymerase chain reaction (PCR) for viruses, especially if there are respiratory symptoms.
  • Serology for atypical respiratory organisms and urine for Pneumococcal and Legionella antigen to cover common and atypical pathogens.
  • Anteroposterior and lateral chest radiograph to identify new pulmonary infiltrates, which are a hallmark of ACS.
  • Arterial blood gas (ABG) measurement on room air, if oxygen saturation (SpO2) is ≤ 94% on room air, to assess for hypoxemia.
  • Electrocardiogram (ECG) and troponin test, particularly in older adolescents and adults presenting with chest pain and shortness of breath, to evaluate for acute myocardial infarction (AMI) or myocardial ischemia.
These recommendations are in line with the guidelines from the British Society for Haematology and the American Heart Association/American College of Cardiology.

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Treatment

Current recommendations include:
  • Supplemental oxygen to maintain oxygen saturation >95%.
  • Analgesia to manage chest pain, with opioids when appropriate, titrated to the patient's pain level.
  • Antibiotics to cover typical and atypical pathogens, including macrolide antibiotics for atypical coverage.
  • Incentive spirometry to prevent atelectasis and improve lung function.
  • Blood transfusion, either simple or exchange, to reduce the proportion of hemoglobin S and improve oxygen-carrying capacity. The decision to transfuse and the choice of transfusion method should be based on the severity of ACS and the patient's hemoglobin level.
  • Corticosteroids may be considered, although their use requires careful consideration due to potential side effects and lack of consensus on optimal dosing strategies.
The British Committee for Standards in Haematology provides guidelines for the management of acute complications in sickle cell disease, including ACS, and recommends the use of incentive spirometry and transfusions when indicated. The Expert Panel report from the National Heart, Lung, and Blood Institute (NHLBI) also provides recommendations for the management of sickle cell disease, including the use of hydroxyurea and transfusion therapy.

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Complications

  • Lung injury can proceed to ARDS-like picture.
  • ACS is a leading cause of death in sickle cell disease.
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  1. Azar S, Wong TE. Sickle cell disease: A brief update. Clin North Am. 2017;101(2):375-393.
  2. Farooq S, Abu Omar M, Salzman GA. Acute chest syndrome in sickle cell disease. Hosp Pract. 2018;46(3):144-151.
  3. Jain S, Bakshi N, Krishnamurti L. Acute chest syndrome in children with sickle cell disease. Pediatr Allergy Immunol Pulmonol. 2017;30(4):191-201.
  4. Paul RN, Castro OL, Aggarwal A, Oneal PA. Acute chest syndrome: Sickle cell disease. Eur J Haematol. 2011;87(3):191-207.
  5. Piel FB, Steinberg HM, Rees DC. Sickle cell disease. N Engl J Med. 2017;376(16):1561-1573.
  6. Subramaniam S, Chao JH. Managing acute complications of sickle cell disease in pediatric patients. Pediatr Emerg Med Pract. 2016;13(11):1-28.
  7. Ware RE, de Montalembert M, Tshilolo L, Abboud MR. Sickle cell disease. Lancet. 2017;390(10091):311-323.
  8. Williams-Johnson J. Sickle Cell Disease and Hereditary Hemolytic Anemias. In: Tintinalli’s Emergency Medicine: A Comprehensive Study Guide. 8th ed. New York, NY: McGraw-Hill Education; 2016.
  9. Writing Committee Members; Gulati M, Levy PD, Mukherjee D, Amsterdam E, Bhatt DL, Birtcher KK, Blankstein R, Boyd J, Bullock-Palmer RP, Conejo T, Diercks DB, Gentile F, Greenwood JP, Hess EP, Hollenberg SM, Jaber WA, Jneid H, Joglar JA, Morrow DA, O'Connor RE, Ross MA, Shaw LJ. 2021 AHA/ACC/ASE/CHEST/SAEM/SCCT/SCMR Guideline for the Evaluation and Diagnosis of Chest Pain: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. J Am Coll Cardiol. 2021 Nov 30;78(22):e187-e285. doi: 10.1016/j.jacc.2021.07.053. Epub 2021 Oct 28. PMID: 34756653.

This is intended solely as reference material and is not a recommendation for any specific patient. The practitioner must rely upon his or her own professional judgment and medical decision-making to determine whether it is relevant in a particular case. Materials are derived from medical and nursing texts, medical literature and national guidelines and should not be considered complete or authoritative. Users must rely on specific patient presentation, experience and judgment when utilizing any of the information contained herein relative to an actual patient.
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