Acute Chest Syndrome in Patients with Sickle Cell Disease in Nigeria: Clinical Features and Management

Abstract

Acute chest syndrome (ACS) is one of the most serious pulmonary complications of sickle cell disease and remains an important cause of hospitalization and death among affected patients. This issue is particularly relevant in Nigeria, where sickle cell disease is common and access to early diagnosis, blood transfusion, respiratory support, and specialist care may differ between medical settings. ACS does not usually result from a single cause. Pulmonary vaso-occlusion, infection, fat embolism, hypoventilation, and tissue hypoxia may occur together and lead to rapid deterioration. Patients may initially present with fever, cough, chest pain, tachypnea, or mild hypoxemia, while severe cases can progress to multilobar pulmonary involvement, marked oxygen desaturation, respiratory failure, and other systemic complications. The clinical picture may also differ between children and adults, which makes early recognition especially important. Diagnosis is mainly based on respiratory symptoms combined with a new pulmonary infiltrate on chest imaging, supported by laboratory findings and assessment of oxygenation. Clinical management requires more than treatment of the pulmonary lesion alone. Oxygen therapy, antibiotics, appropriate pain control, cautious fluid therapy, simple blood transfusion, exchange transfusion, and respiratory support may all be needed depending on disease severity. In Nigeria, delayed presentation, limited access to blood products, uneven availability of exchange transfusion, and restricted diagnostic resources may further complicate care. Better recognition of high-risk patients, earlier treatment, standardized hospital management, and improved long-term sickle cell disease care are therefore important for reducing severe ACS-related outcomes.