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Original research
New entity of adult ultra-short coeliac disease: the first international cohort and case–control study
  1. Suneil A Raju1,2,
  2. Emily A Greenaway1,2,
  3. Annalisa Schiepatti3,4,
  4. Giovanni Arpa5,6,
  5. Nicoletta Vecchione7,
  6. Chao LA Jian8,
  7. Charlotte Grobler9,
  8. Margherita Maregatti10,
  9. Olivia Green1,2,
  10. Freya J Bowker-Howell1,2,
  11. Mohamed G Shiha1,2,
  12. Hugo A Penny1,2,
  13. Simon S Cross2,
  14. Carolina Ciacci7,
  15. Kamran Rostami11,
  16. Shokoufeh Ahmadipour12,
  17. Afshin Moradi13,
  18. Mohammad Rostami-Nejad14,
  19. Federico Biagi3,4,
  20. Umberto Volta15,
  21. Michelangelo Fiorentino15,
  22. Benjamin Lebwohl16,
  23. Peter HR Green16,
  24. Suzanne Lewis16,
  25. Javier Molina-Infante17,18,
  26. Pilar Mata-Romero18,
  27. Valentina Vaira19,20,
  28. Luca Elli10,
  29. Irfan Soykan21,
  30. Arzu Ensari22,
  31. David S Sanders1,2
  1. 1 Academic Unit of Gastroenterology, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK
  2. 2 Division of Clinical Medicine, Faculty of Medicine and Population Health, The University of Sheffield Medical School, Sheffield, UK
  3. 3 Gastroenterology Unit of Pavia Institute, Istituti Clinici Scientifici Maugeri IRCCS, Pavia, Italy
  4. 4 Department of Internal Medicine and Medical Therapy, University of Pavia, Pavia, Italy
  5. 5 Department of Molecular Medicine, Fondazione IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy
  6. 6 Anatomical Pathology Unit of Pavia Institute, Istituti Clinici Scientifici Maugeri IRCCS, Pavia, Italy
  7. 7 Department of Medicine, Surgery, Dentistry, University of Salerno, Fisciano, Italy
  8. 8 Gastroenterology and Hepatology, MidCentral District Health Board, Palmerston North, New Zealand
  9. 9 Medlab Central Limited, Palmerston North, New Zealand
  10. 10 Center for Prevention and Diagnosis of Celiac Disease, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy
  11. 11 Department of Gastroenterology, MidCentral District Health Board, Palmerston North, New Zealand
  12. 12 Hepatitis ResearcH Center, Lorestan University of Medical Sciences, Khoram-Abad, Iran (the Islamic Republic of)
  13. 13 School of Medicine, Department of Pathology, Shahid Beheshti University of Medical Sciences, Tehran, Iran (the Islamic Republic of)
  14. 14 Celiac Disease and Gluten Related Disorders Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran (the Islamic Republic of)
  15. 15 Department of Medical and Surgical Sciences, University of Bologna, Bologna, Italy
  16. 16 Celiac Disease Center, Columbia University Medical Center, New York, New York, USA
  17. 17 Department of Gastroenterology, Centro de Investigación Biomédica en Red, Madrid, Spain
  18. 18 Department of Gastroenterology, Hospital Universitario de Caceres, Caceres, Spain
  19. 19 Department of Pathophysiology and Transplantation, University of Milan, Milano, Italy
  20. 20 Division of Pathology, Fondazione IRCCS Ca’ Granda-Ospedale Maggiore Policlinico, Milano, Italy
  21. 21 Department of Gastroenterology, Ankara University Faculty of Medicine, Ankara, Turkey
  22. 22 Department of Pathology, Ankara University Faculty of Medicine, Ankara, Turkey
  1. Correspondence to Dr Suneil A Raju, Academic Unit of Gastroenterology, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield S10 2JF, UK; suneilraju{at}gmail.com

Abstract

Background Ultra-short coeliac disease (USCD) is defined as villous atrophy only present in the duodenal bulb (D1) with concurrent positive coeliac serology. We present the first, multicentre, international study of patients with USCD.

Methods Patients with USCD were identified from 10 tertiary hospitals (6 from Europe, 2 from Asia, 1 from North America and 1 from Australasia) and compared with age-matched and sex-matched patients with conventional coeliac disease.

Findings Patients with USCD (n=137, median age 27 years, IQR 21–43 years; 73% female) were younger than those with conventional coeliac disease (27 vs 38 years, respectively, p<0.001). Immunoglobulin A-tissue transglutaminase (IgA-tTG) titres at index gastroscopy were lower in patients with USCD versus conventional coeliac disease (1.8×upper limit of normal (ULN) (IQR 1.1–5.9) vs 12.6×ULN (IQR 3.3–18.3), p<0.001).

Patients with USCD had the same number of symptoms overall (median 3 (IQR 2–4) vs 3 (IQR 1–4), p=0.875). Patients with USCD experienced less iron deficiency (41.8% vs 22.4%, p=0.006).

Both USCD and conventional coeliac disease had the same intraepithelial lymphocytes immunophenotype staining pattern; positive for CD3 and CD8, but not CD4.

At follow-up having commenced a gluten-free diet (GFD) (median of 1181 days IQR: 440–2160 days) both USCD and the age-matched and sex-matched controls experienced a similar reduction in IgA-tTG titres (0.5 ULN (IQR 0.2–1.4) vs 0.7 ULN (IQR 0.2–2.6), p=0.312). 95.7% of patients with USCD reported a clinical improvement in their symptoms.

Interpretation Patients with USCD are younger, have a similar symptomatic burden and benefit from a GFD. This study endorses the recommendation of D1 sampling as part of the endoscopic coeliac disease diagnostic workup.

  • coeliac disease
  • gluten free diet
  • gluten sensitive enteropathy
  • gluten
  • celiac disease

Data availability statement

Data are available on reasonable request.

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Data availability statement

Data are available on reasonable request.

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Footnotes

  • X @DrSunnyR, @Mo_Shiha

  • Contributors SAR, EAG and DSS conceptualised and designed the study with comments from AS, GA, NV, CLAJ, CG, MM, OG, FJB-H, MGH, HAP, SSC, CC, KR, SA, AM, MR-N, FB, UV, MF, BL, PHRG, SL, JM-I, PMR, VV, LE, IS and AE. Data were collected by all authors and collated by SAR. Data analysis was completed by SAR and interpreted by all authors. Writing of the manuscript was completed by SAR and edited initially by DSS and then all authors. DSS is the guarantor. The final version was approved by all authors.

  • Funding DSS has previously received an educational grant from Dr Schaer (a gluten‐free food manufacturer). Dr Schaer has no involvement in this study. HAP funded by a Clinical Lecturers grant (CL-2021-04-002) from the NIHR.

  • Competing interests None declared.

  • Patient and public involvement Patients and/or the public were not involved in the design, or conduct, or reporting, or dissemination plans of this research.

  • Provenance and peer review Not commissioned; externally peer reviewed.