Spontaneous jejunoileal perforation as a manifestation of celiac disease—a rare entity: a case report

Abstract

Celiac disease or gluten-sensitive enteropathy is characterized by an autoimmune response in the small intestine triggered by the ingestion of gluten in the diet. It has a prevalence of 0.62% worldwide with considerable variation in incidence among countries. The clinical manifestations of celiac disease differ according to type: the classical type presents with intestinal symptoms, the non-classical type with intestinal or extraintestinal symptoms and the silent type is asymptomatic. Human leukocyte antigens (HLA)-DQ2 (DQA1_05/DQB1_02) are expressed in >90% of patients with celiac disease, and the presence of HLA-DQ2 or HLA-DQ8 (DQA1_ 0301/DQB1_0302) is necessary for its development. One complication of this disease is ulcerative jejunoileitis, a rare condition characterized by chronic idiopathic ulceration affecting the small intestine that can cause intestinal perforation resulting in high morbidity.