Idiopathic hypereosinophilic syndrome presenting with hepatitis and achalasia

Research paper by Amanda C. Cheung, Christine Y. Hachem, Jinping Lai

Indexed on: 14 Jun '16Published on: 13 Jun '16Published in: Clinical Journal of Gastroenterology


Idiopathic hypereosinophilic syndrome (HES) is a rare diagnosis defined by the World Health Organization as a persistent eosinophilia for 6 months and resulting in end-organ dysfunction. While many patients present with nonspecific symptoms, others will present with symptoms of the affected organs, most commonly those involving the heart, skin, or nervous system. Gastrointestinal or liver involvement is estimated to affect up to one-third of patients with HES, although patients with clinically significant disease are limited to case reports. This is the first report of a patient presenting with hepatitis and achalasia related to idiopathic HES.