Methods: Sixty-five Japanese patients recruited by a nationwide survey of CEAS during 2012–2016 were enrolled in this present study. We reviewed the clinical information of the genetically confirmed CEAS patients. [biosimilartoday.com]

The median age at disease onset was 16.5 years, and parental consanguinity was present in 13 patients (28%). Anemia was present in 45 patients (98%), while a single patient experienced gross hematochezia. [pubmed.ncbi.nlm.nih.gov]

However, it is often difficult to distinguish CEAS from CD, since both diseases affect the small bowel with intractable chronic ulcers.9,10 Recently, we evaluated SLCO2A1 protein expression in the intestinal tissues of patients with CEAS.11 In the present [ncbi.nlm.nih.gov]

• Intestinal Disease

  […] resected intestinal tissues was confirmed in two of three patients with CEAS, while the expression of the protein was positive in all 22 patients with CD or intestinal Behçet’s disease.11 In the present study, we further confirmed similar findings in [ncbi.nlm.nih.gov]

• Hematochezia

  Anemia was present in 45 patients (98%), while a single patient experienced gross hematochezia. All patients showed relatively low inflammatory markers in blood tests (median CRP 0.20 mg/dl). [pubmed.ncbi.nlm.nih.gov]

• Hepatosplenomegaly

  Hepatosplenomegaly and increased bone density of spine and pelvis suggested the diagnosis of myelofibrosis. X-ray films showed the cortical thickening of tibiofibula. [pubmed.ncbi.nlm.nih.gov]

• Suggestibility

  Hepatosplenomegaly and increased bone density of spine and pelvis suggested the diagnosis of myelofibrosis. X-ray films showed the cortical thickening of tibiofibula. [pubmed.ncbi.nlm.nih.gov]

  The ulcers occasionally fuse, thus showing a geographic configuration.1–5 In 2015, we identified the solute carrier organic anion transporter family member 2A1 gene (SLCO2A1) as the causative gene for CNSU, and we suggested a more appropriate nomenclature [ncbi.nlm.nih.gov]

• Increased Bone Density

  Hepatosplenomegaly and increased bone density of spine and pelvis suggested the diagnosis of myelofibrosis. X-ray films showed the cortical thickening of tibiofibula. [pubmed.ncbi.nlm.nih.gov]

• Multiple Ulcerations

  Chronic non-specific multiple ulcer of the small intestine segregates in offspring from consanguinity. [pubmed.ncbi.nlm.nih.gov]

  The ulcers are multiple (usually >20) and each lesion manifests a shallow and flat ulcer bed surrounded by a discrete margin. The configuration of each ulcer is usually linear or a tall triangle, and the ulcer is aligned circularly or obliquely. [ncbi.nlm.nih.gov]

• Colitis

  J Crohns Colitis. 2017 Oct 1;11(10):1277-1281. doi: 10.1093/ecco-jcc/jjx068. J Crohns Colitis. 2017. PMID: 28510689 Clinical features of chronic enteropathy associated with SLCO2A1 gene: a new entity clinically distinct from Crohn's disease. [pubmed.ncbi.nlm.nih.gov]

  J Crohns Colitis. 2011;5:559–565. doi: 10.1016/j.crohns.2011.05.008. [PubMed] [CrossRef] [Google Scholar] 6. Umeno J, Hisamatsu T, Esaki M, et al. [ncbi.nlm.nih.gov]

NSAID enteropathy: appearance at CT and MR enterography in the age of multi-modality imaging and treatment. Frye JM, Hansel SL, Dolan SG, Fidler JL, Song LM, Barlow JM, Smyrk TC, Flicek KT, Hara AK, Bruining DH, Fletcher JG. Frye JM, et al. [pubmed.ncbi.nlm.nih.gov]

Treatment with biologics (infliximab or adalimumab) was carried out in 25 of 29 patients (86%) with CD, while none of the four patients with CEAS (p=0.001) were undergoing treatment with biologics. [ncbi.nlm.nih.gov]