Celiac disease is an immune mediated enteropathy characterized by malabsorption and villous atrophy triggered by gluten proteins.13
Currently, in most adult cases, even untreated celiac disease has an indolent course with gastrointestinal symptoms and nutritional abnormalities, but does not result in severe or life-threatening illness. This is in stark contrast to the past when celiac disease was known as a severe disease of childhood. While data do suggest that celiac disease is becoming more common overall14, 15
it is unclear whether the dramatic change in clinical spectrum is due to early recognition and treatment, improved diagnosis of milder cases or an actual change in the nature of celiac disease over time.
The term “celiac crisis” has been used since the 1950s to describe the acute, fulminant form of celiac disease.3
Clinically it is characterized by severe diarrhea, dehydration and metabolic disturbances including hypokalemia, hyponatremia, hypocalcemia, hypomagnesemia, and hypoproteinemia. Traditionally, celiac crisis was associated with a high mortality rate, however, medical care has progressed greatly over the last half century and no recent deaths from celiac crisis have been reported in the literature since Lloyd-Still described the successful treatment of three cases of celiac crisis in children with corticosteroids in 1972.16
The reason why some individuals present with celiac crisis whereas the vast majority of patients with celiac disease run a much more mild course is unclear, however there is likely a combination of severe mucosal inflammation, immune activation and disruption of normal patterns of motility. Like celiac disease in general, celiac crisis in this series appears often to be precipitated by a general immune stimulus such as surgery, infection or pregnancy as has been previously described.17–19
However, it is unclear if celiac crisis in adults occurs at disease onset or if celiac disease has present but undiagnosed until a trigger leads to disease exacerbation. It is notable that in 5 of the 12 patients, symptom onset clearly occurred immediately after surgery.12
It is possible that the combination of celiac disease with a second intestestinal insult (Whipple resection) could result in more severe symptoms.
It is notable that all 11 patients in whom initial labs are available, had either high titer IgA tTG or IgA deficiency suggesting that standard diagnostic testing is adequate for initial evaluation of celiac crisis in acutely ill individuals. Additionally, in all patients, small intestinal biopsy revealed marked villous atrophy, and given the prolonged time to recovery of many of these patients, data from the initial biopsy was clinically valuable. As with all celiac disease, gluten withdrawal with nutritional support is the treatment of choice, and 50% of patients responded quickly to these interventions alone. For individuals not responding promptly to gluten restriction, treatment with prednisone or budesonide were efficacious, and all patients were able to wean off of steroids completely within 8 months (mean 5.3, Range 4 to 7) with eventual good response to a gluten free diet alone.
In summary, we present data on 12 adult individuals presenting with celiac crisis over the past eight years. This series provides new information regarding the spectrum of celiac crisis and celiac disease in general. Additionally, the diagnostic criteria developed for this project may be of benefit in helping clinicians to more promptly diagnose and treat celiac crisis in adult patients. We believe that celiac disease should be considered in the differential diagnosis of all patients presenting with an acute onset of severe diarrhea with metabolic disturbances once common infectious etiologies have been ruled out. Any patient found to have an elevated IgA tTG or IgA deficiency in this setting should be placed on a gluten free diet and small intestinal biopsy performed as soon as possible. Corticosteroids should be considered in cases of celiac crisis when a gluten-free diet, in conjunction with fluid and electrolyte repletion, does not result in rapid improvement.