Acute Chest Syndrome

Acute chest syndrome is a dangerous complication of sickle cell disease characterized by a new radiodensity on chest imaging accompanied by respiratory symptoms and possibly fever. Acute chest syndrome occurs when various factors in individuals with sickle cell disease trigger vaso-occlusion within the pulmonary vasculature. While acute chest syndrome can occur in any sickle cell disease phenotype, it is most prevalent in HbSS.This syndrome can progress quickly and represents the leading cause of death in patients with sickle cell disease. Therefore, early diagnosis and prompt initiation of treatment are crucial to improve patient outcomes.