PMID- 33908742 OWN - NLM STAT- MEDLINE DCOM- 20210709 LR - 20230822 IS - 1536-4801 (Electronic) IS - 0277-2116 (Linking) VI - 72 IP - 6 DP - 2021 Jun 1 TI - Low-Risk Human Leukocyte Antigen Genes and Mild Villous Atrophy Typify Celiac Disease With Immunoglobulin A Deficiency. PG - 889-893 LID - 10.1097/MPG.0000000000003129 [doi] AB - OBJECTIVES: We aimed to establish if in celiac disease (CD) with immunoglobulin A deficiency (IgAD) duodenal histopathology is influenced by human leukocyte antigen (HLA)-DQB1 *02 alleles dosage. Clinical differences between patients with CD and patients with CD and IgAD (CD-IgAD) were also evaluated. METHODS: Five hundred and sixteen CD and 16 patients with CD-IgAD, enrolled over the time of 8 years, took part in this study. The severity of duodenal histopathology and frequency of CD at-risk HLA class II genes were compared in patients with CD versus patients with CD-IgAD. HLA class II genotypes were subdivided into two categories of genetic risk: high: HLA-DR3/DR7, -DR3/DR3, -DR4/DR4 -DR3/DR4 and low: HLA-DR5/DR7, -DR3/X, -DR4/X and X/X, where X means neither -DR3 nor -DR4. Then, they were compared with two types of duodenal histopathology: 0, 1, 2 and 3a of mild villous atrophy (MVA) and 3b and 3c of severe villous atrophy (SVA) according to the Marsh-Oberhuber classification. Clinical data concerning gender, number of esophagogastroduodenoscopies (EGDs) and association with other autoimmune diseases were obtained from medical records. RESULTS: In comparison with CD, CD-IgAD showed an increased frequency of MVA (P < 0.0001). Furthermore, CD-IgAD with MVA showed an increase of HLA low-risk genotypes (P = 0.036) and half HLA-DQ2 heterodimers (P = 0.0443). Interestingly, CD-IgAD demanded an increased number of EGDs to reach the diagnosis of CD (P = 0.0104) and autoimmune liver diseases were more frequent compared to CD (P = 0.0049). CONCLUSIONS: CD-IgAD is associated with MVA, low-risk HLA class II genes, an increased number of EGDs and autoimmune liver diseases. CI - Copyright (c) 2021 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition. FAU - Schirru, Enrico AU - Schirru E AD - Centro Servizi di Ateneo per gli Stabulari (CeSASt), Universita degli Studi di Cagliari, Cittadella Universitaria, Monserrato, Cagliari. FAU - Jores, Rita Desiree AU - Jores RD AD - ATS Sardegna, Poliambulatorio di Quartu Sant'Elena, Cagliari. FAU - Rossino, Rossano AU - Rossino R AD - Dipartimento di Scienze Mediche e Sanita Pubblica, Universita degli studi di Cagliari, Cittadella Universitaria, Monserrato, Cagliari. FAU - Corpino, Mara AU - Corpino M AD - Gastroenterologia Pediatrica, Clinica Pediatrica e Malattie Rare, Ospedale Pediatrico Microcitemico A. Cao, ARNAS Brotzu, Cagliari. FAU - Cucca, Francesco AU - Cucca F AD - Dipartimento di Scienze Biomediche, Universita degli Studi di Sassari, Sassari. AD - Istituto di Ricerca Genetica e Biomedica, Consiglio Nazionale delle Ricerche (CNR), Monserrato, Cagliari, Italy. FAU - Congia, Mauro AU - Congia M AD - Gastroenterologia Pediatrica, Clinica Pediatrica e Malattie Rare, Ospedale Pediatrico Microcitemico A. Cao, ARNAS Brotzu, Cagliari. AD - Istituto di Ricerca Genetica e Biomedica, Consiglio Nazionale delle Ricerche (CNR), Monserrato, Cagliari, Italy. LA - eng PT - Journal Article PL - United States TA - J Pediatr Gastroenterol Nutr JT - Journal of pediatric gastroenterology and nutrition JID - 8211545 RN - 0 (HLA Antigens) RN - 0 (HLA-DR3 Antigen) RN - 0 (Immunoglobulin A) SB - IM MH - Atrophy MH - *Celiac Disease/complications/genetics MH - HLA Antigens MH - HLA-DR3 Antigen/genetics MH - Haplotypes MH - Humans MH - Immunoglobulin A COIS- The authors report no conflicts of interest. EDAT- 2021/04/29 06:00 MHDA- 2021/07/10 06:00 CRDT- 2021/04/28 10:24 PHST- 2021/04/29 06:00 [pubmed] PHST- 2021/07/10 06:00 [medline] PHST- 2021/04/28 10:24 [entrez] AID - 00005176-202106000-00021 [pii] AID - 10.1097/MPG.0000000000003129 [doi] PST - ppublish SO - J Pediatr Gastroenterol Nutr. 2021 Jun 1;72(6):889-893. doi: 10.1097/MPG.0000000000003129.