Refractory coeliac disease in a country with a high prevalence of clinically-diagnosed coeliac disease
Article first published online: 6 JAN 2014
© 2014 John Wiley & Sons Ltd
Alimentary Pharmacology & Therapeutics
Volume 39, Issue 4, pages 418–425, February 2014
How to Cite
Ilus, T., Kaukinen, K., Virta, L. J., Huhtala, H., Mäki, M., Kurppa, K., Heikkinen, M., Heikura, M., Hirsi, E., Jantunen, K., Moilanen, V., Nielsen, C., Puhto, M., Pölkki, H., Vihriälä, I. and Collin, P. (2014), Refractory coeliac disease in a country with a high prevalence of clinically-diagnosed coeliac disease. Alimentary Pharmacology & Therapeutics, 39: 418–425. doi: 10.1111/apt.12606
- Issue published online: 22 JAN 2014
- Article first published online: 6 JAN 2014
- Manuscript Accepted: 13 DEC 2013
- Manuscript Revised: 12 DEC 2013
- Manuscript Revised: 29 SEP 2013
- Manuscript Received: 17 SEP 2013
- Competitive State Research Financing of the Expert Responsibility Area of Tampere University Hospital. Grant Numbers: 9P008, 9P020, 9P033
- Academy of Finland
- Sigrid Juselius Foundation
- Research Fund of the Finnish Coeliac Society
- Elna Kaarina Savolainen′s fund
Refractory coeliac disease (RCD) is thought to be a rare disorder, but the accurate prevalence is unknown.
We aimed to identify the prevalence of and the risk factors for developing RCD in a Finnish population where the clinical detection rate of coeliac disease is high.
The study involved 11 hospital districts in Finland where the number of treated RCD patients (n = 44), clinically diagnosed coeliac disease patients (n = 12 243) and adult inhabitants (n = 1.7 million) was known. Clinical characteristics at diagnosis of coeliac disease between the RCD patients and patients with uncomplicated disease were compared.
The prevalence of RCD was 0.31% among diagnosed coeliac disease patients and 0.002% in the general population. Of the enrolled 44 RCD patients, 68% had type I and 23% type II; in 9% the type was undetermined. Comparing 886 patients with uncomplicated coeliac disease with these 44 patients that developed RCD later in life, the latter were significantly older (median 56 vs 44 years, P < 0.001), more often males (41% vs. 24%, P = 0.012) and seronegative (30% vs. 5%, P < 0.001) at the diagnosis of coeliac disease. Patients with evolving RCD had more severe symptoms at the diagnosis of coeliac disease, including weight loss in 36% (vs. 16%, P = 0.001) and diarrhoea in 54% (vs. 38%, P = 0.050).
Refractory coeliac disease is very rare in the general population. Patients of male gender, older age, severe symptoms or seronegativity at the diagnosis of coeliac disease are at risk of future refractory coeliac disease and should be followed up carefully.