Published online Feb 6, 2019. doi: 10.12998/wjcc.v7.i3.311
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
Processing time: 92 Days and 7.9 Hours
Celiac disease (CD) is well-recognized as a clinical chameleon, which is responsible for the high under-diagnosis rates of this common digestive condition. One of its presenting features is celiac crisis (CC), a potentially life-threatening acute malabsorptive state accompanied by metabolic and electrolyte disturbances and hemodynamic instability, usually requiring hospitalization and intensive care. Only a handful of case reports so far have been published in the literature, both in adults and children, and current available guidelines do not make any mention of this condition.
We aimed to perform a systematic review focused on CC in order to better delineate the possible triggers, clinical features, diagnostic work-ups and treatment measures. Only case reports or case series were published so far in the literature. Therefore, another objective was raising awareness about this condition as a possible heraldic feature of CD.
The main objective was to get a clear characterization of CC by summarizing all case reports with regard to clinical manifestations, laboratory data, diagnostic and therapeutic approaches. Also, we intended to gain some insight about the possible trigger factors of CC.
For this purpose, we performed a systematic review of published case reports by searching the literature in two major databases, PubMed and EMBASE. All abstracts meeting the inclusion criteria were processed, and relevant articles were read in their full text forms and further analyzed for the specific objectives.
Our review summarizes the available literature on the occurrence of CC in adults, which included 42 cases reported in 29 articles. Common clinical features (profuse diarrhea, hemodynamic instability, weight loss, renal impairment) and particular ones (neurologic, cardiovascular or hematologic) were described, along with possible triggers (infections, surgery, pregnancy). Besides the laboratory alterations secondary to the acute malabsorptive state, the diagnostic approach included CD serology testing and histology. Management and outcome, referring to intensive care support, the need for steroids, and evolution under a gluten free diet were also analyzed.
To the best of our knowledge, this review is the first to summarize clinical, diagnostic and therapeutic approaches of a rare but potentially life-threatening presenting feature of CD using an extensive analysis of currently available data. In addition to a clear definition and description of the characteristics of CC, the gaps in our current understanding of this condition were also outlined.
This research presents the main characteristics of CC occurrence in adult CD patients and also emphasizes there is a need to increase awareness of this condition. Further research is warranted to elucidate the pathological mechanism that triggers CC.