Villanacci V, et al. - In this retrospective analysis, researchers studied patients with autoimmune enteropathy (AIE), focusing on clinical and histological findings. Celiac disease-like pattern was identified to be the prevalent histological presentation, independently of age. With regard to the histological presentation of AIE, a significant difference was observed between patients with and without primary immunodeficiencies, for the first time in this study. The main presentation identified in patients with primary immunodeficiencies (24/40) and in those without primary immunodeficiencies was celiac disease pattern and a mixed histological pattern, respectively. Patients with this rare disorder may be more precisely and timely diagnosed and managed with the aid of these findings.