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Clin Immunol. 2019 Jul 4;207:10-17. doi: 10.1016/j.clim.2019.07.001. [Epub ahead of print]

Clinical manifestations and gastrointestinal pathology in 40 patients with autoimmune enteropathy.

Author information

1
Institute of Pathology, Spedali Civili Brescia, Italy.
2
Pediatrics Clinic and Institute for Molecular Medicine A. Nocivelli, Department of Clinical and Experimental Sciences, University of Brescia and ASST-Spedali Civili of Brescia, Italy. Electronic address: vlougarisbs@yahoo.com.
3
Gastroenterology and GI Endoscopy Unit, University Department of Pediatrics, Children's Hospital, Brescia, Italy.
4
Gastroenterology and Endoscopy Department, Maggiore Hospital, ASST Crema, Crema, Italy.
5
Pathology Institute, Azienda Ospedaliera Universitaria, Ospedali Riuniti di Trieste, Italy.
6
Meyer Children Hospital, University of Firenze, Italy.
7
Marenal and Infantile Department of Pediatrics, Ospedale Ca' Foncello, Treviso, Italy.
8
Maternal and Infantile Department of Pediatrics ASST Grande Ospedale Metropolitano Niguarda Milano, Italy.
9
Pediatric Surgery, Maternal and Infantile Department ASST Grande Ospedale Metropolitano Niguarda Milano, Italy.
10
Pediatric Surgery Department, Children's Hospital G. Di Cristina, ARNAS Civico-Di Cristina-Benfratelli, Palermo, Italy.
11
Gastroenterology and Hepatology Section, Department of Medicine, University of Perugia Medical School, Perugia, Italy.

Abstract

Autoimmune enteropathy (AIE) is a rare condition that may affect pediatric and adult patients, frequently associated with primary immunodeficiencies. We performed a retrospective study on clinical and histological findings from 40 AIE patients. Histological presentation showed a prevalent celiac disease pattern (50%), followed by the mixed pattern (35%), independently of age, chronic active duodenitis (10%), and GVHD-like pattern (5%). Patients with primary immunodeficiencies (24/40) presented mainly with the celiac disease pattern (72.2% versus 22.2%; p < .0001), while patients without primary immunodeficiencies presented with a mixed histological pattern (61.1% versus 13.6%; p < .0001). Our study shows that the prevalent histological presentation is the celiac disease-like pattern, independently of age, and, for the first time, that the histological presentation of AIE differs significantly between patients with and without primary immunodeficiencies. These findings may be helpful for more precise and timely diagnosis and management of this rare disorder.

KEYWORDS:

Autoimmune enteropathy (AIE); Celiac disease; Duodenum; Primary immunodeficiencies (PIDs)

PMID:
31279857
DOI:
10.1016/j.clim.2019.07.001

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