From the Guidelines
Acute chest syndrome is diagnosed and treated based on the presence of a new pulmonary infiltrate on chest X-ray, accompanied by fever, respiratory symptoms, and/or hypoxemia, with treatment involving supplemental oxygen, broad-spectrum antibiotics, pain management, and blood transfusions as needed. The diagnostic criteria for acute chest syndrome include a new segmental infiltrate identified on a chest radiograph, accompanied by lower respiratory tract symptoms, chest pain, and/or hypoxemia 1. According to the American Society of Hematology 2020 guidelines, early recognition and treatment of acute chest syndrome are crucial, as it is one of the leading causes of death in patients with sickle cell disease 1.
Diagnostic Evaluation
The diagnosis of acute chest syndrome requires careful evaluation, including:
- Complete blood count
- Reticulocyte count
- Blood cultures
- Chest imaging
Treatment Options
Treatment of acute chest syndrome involves a comprehensive approach, including:
- Supplemental oxygen to maintain saturation above 95%
- Intravenous hydration with maintenance fluids
- Broad-spectrum antibiotics, such as a third-generation cephalosporin like ceftriaxone 1-2g IV every 24 hours, plus a macrolide such as azithromycin 500mg daily
- Pain management with opioids as needed
- Incentive spirometry every 2 hours while awake
- Blood transfusions, with simple transfusion indicated for moderate hypoxemia and exchange transfusion recommended for severe cases with respiratory failure or multi-organ dysfunction 1
Additional Considerations
- Bronchodilators should be used if wheezing is present
- Corticosteroids may be considered, but remain controversial due to rebound pain concerns
- Early intervention is critical, as acute chest syndrome can rapidly progress to respiratory failure
- Prevention strategies include hydroxyurea therapy, pneumococcal and influenza vaccinations, and prompt treatment of respiratory infections in sickle cell patients 1
From the Research
Diagnostic Criteria for Acute Chest Syndrome
- Acute chest syndrome (ACS) is defined by respiratory symptoms and/or fever and a new radiodensity on chest imaging in a patient with sickle cell disease (SCD) 2
- Diagnostic criteria include a new infiltrate on pulmonary imaging combined with any of the following: fever > 38.5°C (101.3°F), cough, wheezing, hypoxemia (PaO2 < 60 mm Hg), tachypnea, or chest pain 3
- Patients typically present with or develop respiratory symptoms including fever, cough, chest pain, and shortness of breath, which can progress to respiratory failure requiring mechanical ventilation in 20% of adult patients 2
Treatment Options for Acute Chest Syndrome
- Management focuses on analgesia, oxygen supplementation, incentive spirometry, bronchodilators, rehydration, antibiotics, consideration for transfusion, and specialist consultation 2
- Empiric antibiotics that cover atypical pathogens are necessary along with measures to increase oxygen-carrying capacity in those with hypoxemia such as simple transfusion or exchange transfusion 2
- Treatment with hydroxyurea should be considered for prevention of recurrent episodes 4
- Hydration with IV crystalloid solutions, antibiotics, judicious analgesia, oxygen, and, in severe cases, transfusion are also recommended 3
Causes and Outcomes of Acute Chest Syndrome
- The acute chest syndrome is the leading cause of death among patients with sickle cell disease 5
- A specific cause of the acute chest syndrome was identified in 38 percent of all episodes and 70 percent of episodes with complete data, including pulmonary fat embolism and 27 different infectious pathogens 5
- Infection was a contributing factor in 56 percent of the deaths, and among older patients and those with neurologic symptoms, the syndrome often progresses to respiratory failure 5