Systematic Reviews
Copyright ©The Author(s) 2019. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Feb 6, 2019; 7(3): 311-319
Published online Feb 6, 2019. doi: 10.12998/wjcc.v7.i3.311
Celiac crisis, a rare occurrence in adult celiac disease: A systematic review
Daniel Vasile Balaban, Alina Dima, Ciprian Jurcut, Alina Popp, Mariana Jinga
Daniel Vasile Balaban, Alina Dima, Mariana Jinga, Department of Internal Medicine and Gastroenterology, Carol Davila University of Medicine and Pharmacy, Bucharest 020021, Romania
Ciprian Jurcut, Department of Internal Medicine, Dr. Carol Davila Central Military Emergency University Hospital, Bucharest 010825, Romania
Alina Popp, Department of Pediatrics, Carol Davila University of Medicine and Pharmacy, Bucharest 020021, Romania
Alina Popp, Alessandrescu-Rusescu Institute for Mother and Child Health, Bucharest 020395, Romania
Alina Popp, Center for Child Health Research, University of Tampere and Tampere University Hospital, Tampere 33521, Finland
Author contributions: Balaban DV proposed the research idea; Balaban DV, Dima A, Jurcut C, Popp A, and Jinga M conceived and design the general research structure; Balaban DV and Dima A searched the literature data; Balaban DV and Dima A drafted the manuscript; Jurcut C, Popp A, and Jinga M critically reviewed the manuscript; All authors approved the final version of the manuscript.
Conflict-of-interest statement: All the authors declare that they have no competing interests.
PRISMA 2009 Checklist statement: PRISMA checklist was assessed for this article.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Corresponding author: Alina Dima, MD, PhD, Assistant Professor, Department of Internal Medicine and Gastroenterology, Carol Davila University of Medicine and Pharmacy, 37 Dionisie Lupu, Bucharest 020021, Romania.
Telephone: +40-72-9024568 Fax: +40-21-3180719
Received: October 30, 2018
Peer-review started: November 1, 2018
First decision: November 22, 2018
Revised: December 10, 2018
Accepted: December 12, 2018
Article in press: December 12, 2018
Published online: February 6, 2019

Celiac crisis (CC), a potentially life-threatening condition, is one of the rare clinical presentations of celiac disease (CD). Several cases have been documented in the literature, mostly in children.


To perform a review of CC cases reported in adult CD patients.


A systematic search of the literature was conducted in two databases, PubMed/MEDLINE and EMBASE, using the term “celiac crisis" and its variant "coeliac crisis", from January 1970 onwards. Altogether, 29 articles reporting 42 biopsy-proven cases were found in the search. Here, we summarized the demographic, clinical characteristics, laboratory and diagnostic work-ups, and therapeutic management in these patients.


Among the 42 CD cases, the median age was 50 years (range 23-83), with a 2:1 female to male ratio. The majority of patients (88.1%) developed CC prior to CD diagnosis, while the remaining were previously diagnosed CD cases reporting low adherence to a gluten-free diet (GFD). Clinically, patients presented with severe diarrhea (all cases), weight loss (about two thirds) and, in particular situations, with neurologic (6 cases) or cardiovascular (1 case) manifestations or bleeding diathesis (4 cases). One in four patients had a precipitating factor that could have triggered the CC (e.g. trauma, surgery, infections). Laboratory workup of patients revealed a severe malabsorptive state with metabolic acidosis, dehydration, hypoalbuminemia and anemia. The evolution of GFD was favorable in all cases except one, in whom death was reported due to refeeding syndrome.


Celiac crisis is a rare but severe and potentially fatal clinical feature of CD. A high index of suspicion is needed to recognize this clinical entity and to deliver proper therapy consisting of supportive care and, subsequently, GFD.

Keywords: Celiac disease, Celiac crisis, Malabsorption, Malnutrition, Diarrhea

Core tip: Celiac disease is well recognized as having a broad spectrum of presentations. Among them, celiac crisis is a rare but potentially life-threatening clinical feature, which has been reported in both pediatric and adult celiac disease cases. Several case reports have been published so far in adults, and our aim was to summarize the current literature in order to better delineate the patients’ profile with respect to possible triggers, clinical characteristics, laboratory workups and management of celiac crisis.