Estimated Prevalence of Cronkhite-Canada Syndrome, Chronic Enteropathy Associated With SLCO2A1 Gene, and Intestinal Behçet's Disease in Japan in 2017: A Nationwide Survey.

Mari S Oba,Yoshitaka Murakami,Yosikazu Nakamura,Keiko Asakura,Yuji Nishiwaki,Wakaba Fukushima,Yasuo Suzuki,Satoko Ohfuji

doi:10.2188/jea.je20190349

Mari S Oba, Yoshitaka Murakami + Show 6 more

Open Access

https://doi.org/10.2188/jea.je20190349

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Abstract

BackgroundCronkhite-Canada syndrome (CCS), chronic enteropathy associated with SLCO2A1 gene (CEAS), and intestinal Behçet’s disease (BD) are classified as intractable intestinal disorders in Japan. However, the national prevalence of these diseases remains unknown. We performed a nationwide survey to estimate the patient numbers and prevalence rates of these diseases throughout Japan in 2017.MethodsWe conducted a mail-based survey targeting hospitals across Japan to estimate the annual numbers of patients with CCS, CEAS, and intestinal BD in 2017. Using a stratified random sampling method, we selected 2,979 hospital departments and asked them to report the number of patients who met specific diagnostic criteria. The total number of patients for each disease was estimated by multiplying the reported numbers by the reciprocal of the sampling rate and response rate. The corresponding prevalence rates per 1,000,000 population were calculated based on the mid-year population of Japan in 2017.ResultsThe overall survey response rate was 68.1% (2,029 departments). The estimated numbers of patients with CCS, CEAS, and intestinal BD were 473 (95% confidence interval [CI], 357–589), 388 (95% CI, 289–486), and 3,139 (95% CI, 2,749–3,529), respectively; the prevalence rates per 1,000,000 population were 3.7 (male: 4.0; female: 3.5), 3.1 (male: 3.0; female: 3.1), and 24.8 (male: 24.5; female: 25.0), respectively. The male-to-female ratios were 1.10, 0.94, and 0.93 for patients with CCS, CEAS, and intestinal BD, respectively.ConclusionsEstimates of the national prevalence of CCS, CEAS, and intestinal BD in Japan were generated and found to be higher than those previously reported.

Highlights

Cronkhite-Canada syndrome (CCS), chronic enteropathy associated with SLCO2A1 gene (CEAS), and intestinal Behçet’s disease (BD) are rare intestinal disorders classified as intractable diseases in Japan
The prevalence and total numbers of patients with ulcerative colitis and Crohn’s disease in Japan have been reported in a nationwide survey,[1] CCS, CEAS, and intestinal BD have different diagnostic criteria and must be evaluated separately from these diseases
From a total of 12,326 departments in 8,445 hospitals, 2,979 departments in 1,586 hospitals were sampled through stratified random sampling

Summary

Introduction

Cronkhite-Canada syndrome (CCS), chronic enteropathy associated with SLCO2A1 gene (CEAS), and intestinal Behçet’s disease (BD) are rare intestinal disorders classified as intractable diseases in Japan. The prevalence and total numbers of patients with ulcerative colitis and Crohn’s disease (which are classified as intractable diseases) in Japan have been reported in a nationwide survey,[1] CCS, CEAS, and intestinal BD have different diagnostic criteria and must be evaluated separately from these diseases. CCS is characterized by gastrointestinal polyposis, chronic diarrhea, skin hyperpigmentation, hair loss, and nail atrophy.[2,3,4] Numerous case series have been reported in Asian countries.[5,6,7,8,9] In. Cronkhite-Canada syndrome (CCS), chronic enteropathy associated with SLCO2A1 gene (CEAS), and intestinal Behçet’s disease (BD) are classified as intractable intestinal disorders in Japan. We performed a nationwide survey to estimate the patient numbers and prevalence rates of these diseases throughout Japan in 2017

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