PMID- 29324472 OWN - NLM STAT- MEDLINE DCOM- 20190226 LR - 20200130 IS - 1532-0979 (Electronic) IS - 0147-5185 (Linking) VI - 42 IP - 4 DP - 2018 Apr TI - Collagenous Enteritis is Unlikely a Form of Aggressive Celiac Disease Despite Sharing HLA-DQ2/DQ8 Genotypes. PG - 545-552 LID - 10.1097/PAS.0000000000001022 [doi] AB - Collagenous enteritis is an uncommon small intestinal injury pattern with unclear pathogenesis. While it has been speculated that collagenous enteritis represents a form of refractory celiac disease, recent clinical studies suggest a potential link to exposure to the antihypertensive medication olmesartan. Here we hypothesized that the pathogenesis of collagenous enteritis involves both genetic and environmental factors. All subjects with biopsy-proven collagenous enteritis diagnosed between 2002 and 2015 were identified from 2 tertiary care medical centers. Human leukocyte antigen (HLA)-DQ genotyping was performed by polymerase chain reaction on archived tissue. Celiac disease serology, past medical history, medications, smoking history, demographics, histology, clinical management, and follow-up were recorded. A total of 32 subjects were included. In contrast to celiac disease, subjects with collagenous enteritis were mostly elderly (median age at diagnosis, 69 y; range, 33 to 84 y). Seventy percent of collagenous enteritis subjects harbored celiac disease susceptibility alleles HLA-DQ2/DQ8; however, only 1 subject had elevated serum levels of celiac disease-associated autoantibodies while on a gluten-containing diet. Furthermore, 56% of subjects were taking nonsteroidal anti-inflammatory drugs, 36% proton-pump inhibitors, 28% statins, and 32% olmesartan at the time of diagnosis. Discontinuation of olmesartan and treatments with steroids and/or gluten-free diet resulted in symptomatic and histologic improvement. Neither lymphoma nor collagenous enteritis-related death was seen in this cohort. Therefore, while collagenous enteritis shares similar HLA genotypes with celiac disease, the difference in demographics, the lack of celiac disease-associated autoantibodies, and potential link to medications as environmental triggers suggest the 2 entities are likely distinct in pathogenesis. FAU - Kung, Vanderlene Liu AU - Kung VL AD - Department of Pathology and Immunology, Washington University School of Medicine, Saint Louis, MO. FAU - Liu, Ta-Chiang AU - Liu TC AD - Department of Pathology and Immunology, Washington University School of Medicine, Saint Louis, MO. FAU - Ma, Changqing AU - Ma C AD - Department of Pathology, School of Medicine, University of Pittsburgh, Pittsburgh, PA. LA - eng PT - Journal Article PT - Multicenter Study PL - United States TA - Am J Surg Pathol JT - The American journal of surgical pathology JID - 7707904 RN - 0 (Angiotensin II Type 1 Receptor Blockers) RN - 0 (HLA-DQ Antigens) RN - 0 (HLA-DQ2 antigen) RN - 0 (HLA-DQ8 antigen) RN - 0 (Steroids) RN - 9007-34-5 (Collagen) SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Angiotensin II Type 1 Receptor Blockers/adverse effects MH - Biopsy MH - Celiac Disease/classification/*genetics/immunology/pathology MH - Collagen/*analysis MH - Collagenous Sprue/classification/*genetics/metabolism/therapy MH - Diet, Gluten-Free MH - Enteritis/classification/*genetics/metabolism/therapy MH - Female MH - Genetic Predisposition to Disease MH - HLA-DQ Antigens/*genetics/immunology MH - Humans MH - Intestine, Small/*chemistry/drug effects/pathology MH - Male MH - Middle Aged MH - Missouri MH - Pennsylvania MH - Phenotype MH - Retrospective Studies MH - Risk Factors MH - Steroids/therapeutic use MH - Treatment Outcome EDAT- 2018/01/13 06:00 MHDA- 2019/02/27 06:00 CRDT- 2018/01/12 06:00 PHST- 2018/01/13 06:00 [pubmed] PHST- 2019/02/27 06:00 [medline] PHST- 2018/01/12 06:00 [entrez] AID - 10.1097/PAS.0000000000001022 [doi] PST - ppublish SO - Am J Surg Pathol. 2018 Apr;42(4):545-552. doi: 10.1097/PAS.0000000000001022.