Sickle cell disease acute chest syndrome (ACS) clinical features and management implications in children and adults

	Children	Adults
Causes	Acute infection (viral or bacterial bronchiolitis, pneumonia) accounts for most episodes	Typically occurs as a complication of a vaso-occlusive pain episode Bone marrow and fat emboli are more frequent in adults than children, although these are challenging to diagnose Pulmonary embolism or thrombosis
Risk factors	Hb SS or Hb S-beta⁰-thalasssemia genotype Prior ACS or vaso-occlusive episodes Winter months Asthma Exposure to environmental smoke	Hb SS or Hb S-beta⁰-thalasssemia genotype Prior ACS or vaso-occlusive episodes Recurrent wheezing^[1,2] High WBC, hemoglobin Smoking
Incidence*	32 per 100 patient-years (patients with asthma)^[3] 16 per 100 patient-years (patients without asthma)^[3]	9 per 100 patient-years^[4]
Evaluation	Examination including temperature, chest and abdomen, and any sites of pain Laboratory testing as appropriate^¶ Chest imaging^¶ Pulse oximetry^¶ Consider other diagnoses (asthma, other infections)	Examination including temperature, chest and abdomen, and any sites of pain Laboratory testing as appropriate^¶ Chest imaging^¶ Pulse oximetry^¶ Consider other diagnoses (pulmonary embolism, vaso-occlusive pain, acute coronary syndrome/myocardial infarction)
Management	Pain control and active management of acute vaso-occlusive pain Respiratory support including supplemental oxygen if needed and incentive spirometry Antibiotics Transfusion for moderate to severe cases (simple or exchange) Supportive care (bronchodilators for wheezing or history of asthma) Post-discharge assessment for pulmonary function and assessment of aeroallergens	Pain control and active management of acute vaso-occlusive pain Respiratory support including supplemental oxygen if needed and incentive spirometry; intubation may be required Antibiotics Transfusion for most cases (simple or exchange) Supportive care (fluids, bronchodilators for wheezing or history of asthma)
Prognosis	Death is rare in children with SCD who have an ACS episode^[5]	Patients with rapidly progressive ACS: mortality 6%^[5] Patients without rapidly progressive ACS: mortality 0%^[5]
Prevention	Education Vaccinations Prophylactic penicillin Asthma treatment and control of environmental allergens Hydroxyurea Chronic transfusion if hydroxyurea is ineffective	Education Vaccinations Asthma treatment Hydroxyurea Chronic transfusion if hydroxyurea is ineffective

All patients with suspected ACS should have chest imaging regardless of age. Causes, evaluation, and management are similar among children and adults, but the relative frequency differs. As examples, adults are more likely to have ACS as a complication of a vaso-occlusive pain episode while children are more likely to have a viral or bacterial infection or asthma. Refer to UpToDate topics for additional information and supporting evidence.

Hb: hemoglobin; ACS: acute chest syndrome; WBC: white blood count; SCD: sickle cell disease; CBC: complete blood count; COVID-19: coronavirus disease 2019; CTPA: computed tomography with pulmonary angiography.

* Rates of ACS are highest in individuals with Hb SS or Hb S-beta⁰-thalassemia (approximately 3-fold higher than other genotypes), but individuals with any genotype can develop ACS, and ACS cannot be excluded based on genotype.

¶ Laboratory testing includes:

CBC
Type and crossmatch
Hb S percentage
Metabolic panel
Infectious disease testing (cultures, COVID-19 test, influenza test)

Chest radiography is the standard imaging modality for diagnosing ACS. In some cases, other modalities such as chest ultrasound may be used if there are additional indications or local expertise. CTPA may be appropriate if pulmonary embolism or pulmonary thrombosis is suspected. Pulse oximetry is important, but arterial blood gas is rarely needed unless there is impending respiratory collapse or need for intubation.

References:

Cohen RT, Madadi A, Blinder MA, et al. Recurrent, severe wheezing is associated with morbidity and mortality in adults with sickle cell disease. Am J Hematol 2011; 86:756.
Jakubowiak AJ, Richardson PG, Zimmerman T, et al. Perifosine plus lenalidomide and dexamethasone in relapsed and relapsed/refractory multiple myeloma: a Phase I Multiple Myeloma Research Consortium study. Br J Haematol 2012; 158:472.
Willen SM, Rodeghier M, DeBaun MR. Asthma in children with sickle cell disease. Curr Opin Pediatr 2019; 31:349.
Castro O, Brambilla DJ, Thorington B, et al. The acute chest syndrome in sickle cell disease: incidence and risk factors. The Cooperative Study of Sickle Cell Disease. Blood 1994; 84:643.
Chaturvedi S, Ghafuri DL, Glassberg J, et al. Rapidly progressive acute chest syndrome in individuals with sickle cell anemia: a distinct acute chest syndrome phenotype. Am J Hematol 2016; 91:1185.

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Sickle cell disease acute chest syndrome (ACS) clinical features and management implications in children and adults