Acute chest syndrome of sickle cell disease: radiographic and clinical analysis of 70 cases.

BACKGROUND

Acute chest syndrome (ACS) is a pulmonary illness with fever, chest pain, leukocytosis and new pulmonary opacity in a patient with sickle cell disease. It is a common reason for hospitalization in sickle cell patients, and a significant cause of mortality. The etiology of ACS is unclear. Lung or bone infarction and infection, among other possible causes, have been proposed.

OBJECTIVE

We reviewed the chest radiographs and medical records of 41 patients with 70 episodes of ACS and correlated the clinical and radiographic courses in an attempt to better characterize and understand the syndrome.

RESULTS

In 87 % of episodes, no identifiable etiology of ACS was found. This group of patients had a median age of 14 years and showed dramatic clinical and radiographic improvement within 24 h of therapy. In the remainder of episodes (13 %), an identifiable etiology was found, usually bacterial pneumonia. These patients were younger than the group without an identifiable etiology (median age 2 years) and had a prolonged radiographic course of illness.

CONCLUSION

The chest radiographs of children with ACS without an identifiable etiology have an extremely typical appearance and evolution. Only in cases which do not have this typical pattern should infection be suspected as the underlying cause.