• Clinical science
  • Clinician

Acute chest syndrome

Summary

Acute chest syndrome (ACS) is a potentially fatal complication of sickle cell anemia caused by vaso-occlusion of the pulmonary vasculature. Symptoms may include chest pain, shortness of breath, and fever. Diagnosis is based on clinical symptoms and chest imaging findings of new pulmonary infiltrate. Management consists of antibiotics, supportive care with IV fluids and oxygen, and possibly a blood transfusion.

See also sickle cell anemia.

Definition

  • Vaso-occlusion of the pulmonary vasculature
  • Triggers include infection, asthma, surgery/general anesthesia
  • Common cause of death in patients with sickle cell anemia [1]

Clinical features

Diagnostics

ACS is a clinical diagnosis supported by characteristic clinical features and the presence of new pulmonary infiltrate on imaging. [1]

Diagnostic criteria for acute chest syndrome [2][3][4]

Laboratory studies

Imaging

  • Chest x-ray
    • Indication: all patients suspected of having ACS
    • Supportive findings:
      • New pulmonary infiltrate
      • Segmental, lobar, or multilobular consolidation with or without the presence of pleural effusion
      • If CXR is normal, it should be repeated in 24–48 hours if there is ongoing clinical suspicion for ACS. [6]
  • CT pulmonary angiography: if there is a concern for pulmonary embolism (PE) [8]

Other

Management

Management consists of supportive care, antibiotics, evaluation for blood transfusion, and hospital admission with close monitoring and hematology consult. Critically ill or rapidly progressing patients should also receive respiratory and hemodynamic support, evaluation for urgent exchange transfusion, and be admitted to the ICU. [1][4][9]

Supportive care

Avoid overhydration in patients with acute chest syndrome because of the risk of pulmonary edema.

Antibiotic therapy [7]

Blood transfusion

Monitoring and disposition

  • Monitoring
    • Continuous or frequent (e.g., every 4 hours) pulse oximetry monitoring
    • Consider continuous cardiac monitoring.
    • Consider frequent clinical assessments of patients with features of impending severe ACS. [4]
    • Monitor for progression to multisystem organ failure.
    • Monitor closely for anemia and bronchospasm.
  • Disposition
    • Admit all patients to the hospital. [4]
    • Admit/transfer to the ICU if the patient is at risk of progression to respiratory failure, is intubated, and/or requires an exchange transfusion.

Complications

We list the most important complications. The selection is not exhaustive.

Acute management checklist

  • 1. Howard J, Hart N, Roberts-Harewood M, et al. Guideline on the management of acute chest syndrome in sickle cell disease. Br J Haematol. 2015; 169(4): pp. 492–505. doi: 10.1111/bjh.13348.
  • 2. Ballas SK, Lieff S, Benjamin LJ, et al. Definitions of the phenotypic manifestations of sickle cell disease. Am J Hematol. 2009: pp. NA–NA. doi: 10.1002/ajh.21550.
  • 3. Vichinsky EP, Neumayr LD, Earles AN, et al. Causes and Outcomes of the Acute Chest Syndrome in Sickle Cell Disease. N Engl J Med. 2000; 342(25): pp. 1855–1865. doi: 10.1056/nejm200006223422502.
  • 4. National Heart, Lung, and Blood Institute (NHLBI). Evidence-Based Management of Sickle Cell Disease: Expert Panel Report, 2014. https://www.nhlbi.nih.gov/sites/default/files/media/docs/sickle-cell-disease-report%20020816_0.pdf. Updated September 1, 2014. Accessed November 27, 2019.
  • 5. Chaturvedi S, Ghafuri DL, Glassberg J, Kassim AA, Rodeghier M, DeBaun MR. Rapidly progressive acute chest syndrome in individuals with sickle cell anemia: a distinct acute chest syndrome phenotype. Am J Hematol. 2016; 91(12): pp. 1185–1190. doi: 10.1002/ajh.24539.
  • 6. Al-Salem A. The Acute Chest Syndrome in Sickle Cell Anemia. Springer; 2015.
  • 7. National Heart, Lung, and Blood Institute (NHLBI). The management of sickle cell disease. https://www.nhlbi.nih.gov/files/docs/guidelines/sc_mngt.pdf. Updated January 1, 2002. Accessed February 26, 2020.
  • 8. Dessap AM, Deux J-F, Abidi N, et al. Pulmonary Artery Thrombosis during Acute Chest Syndrome in Sickle Cell Disease. Am J Respir Crit Care Med. 2011; 184(9): pp. 1022–1029. doi: 10.1164/rccm.201105-0783oc.
  • 9. Yawn et al. Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members. JAMA. 2014; 312(10): pp. 1033–48. doi: 10.1001/jama.2014.10517.
  • 10. Okomo U, Meremikwu MM. Fluid replacement therapy for acute episodes of pain in people with sickle cell disease. Cochrane Database of Systematic Reviews. 2017. doi: 10.1002/14651858.cd005406.pub5.
  • 11. Otrock ZK, Thibodeaux SR, Jackups R. Vascular access for red blood cell exchange. Transfusion. 2018; 58(S1): pp. 569–579. doi: 10.1111/trf.14495.
last updated 06/11/2020
{{uncollapseSections(['Y51nih0', 'zm1rRh0', '-m1DRh0', 'Z51Zih0', '051eih0', 'a51Qih0', 'b51Hih0'])}}