Medication Summary

Glucocorticoids may be needed for refractory celiac disease (a condition, however, not reported convincingly in pediatric patients).

More interesting is the active research on promising new drugs, especially those aimed at substantially decreasing the amount of toxic gliadin peptides delivered to the small intestine after gluten ingestion, such as latiglutenase.^{[67, 68]}

Class Summary

Corticosteroids can rapidly control severe symptoms of celiac disease (CD). They may also have a role in rare cases in which the patient has no response to diet; this condition is known as refractory celiac disease and occurs exclusively in adults (1-3% of total).

For celiac disease in children, steroids are almost never needed.

Hydrocortisone (A-Hydrocort, Solu-Cortef)

View full drug information

Some cases of refractory celiac disease (with all other forms of colitis and enteritis excluded) respond to parenteral corticosteroids, for reasons unknown. Exclude other etiologies of failure to thrive, especially in children, because systemic steroids can pose risk to growth. Sodium succinate salt formulation may be administered IV or IM.

Questions

Guandalini S, Discepolo V. Celiac Disease. Guandalini S, Dhawan A. Textbook of Pediatric Gastroenterology, Hepatology and Nutrition. 2nd. Springer, Cham.; 2022. 525-548. [Full Text].
Husby S, Koletzko S, Korponay-Szabo IR, et al. European Society for Pediatric Gastroenterology, Hepatology, and Nutrition guidelines for diagnosing coeliac disease 2020. J Pediatr Gastroenterol Nutr. 2020. 70:141-157. [QxMD MEDLINE Link].
Brown NK, Guandalini S, Semrad C, Kupfer SS. A Clinician's Guide to Celiac Disease HLA Genetics. Am J Gastroenterol. 2019 Oct. 114 (10):1587-92. [QxMD MEDLINE Link].
Rubio-Tapia A, Kyle RA, Kaplan EL, et al. Increased prevalence and mortality in undiagnosed celiac disease. Gastroenterology. 2009 Jul. 137(1):88-93. [QxMD MEDLINE Link]. [Full Text].
Tang F, Chen Z, Ciszewski C, et al. Cytosolic PLA2 is required for CTL-mediated immunopathology of celiac disease via NKG2D and IL-15. J Exp Med. 2009 Mar 16. 206(3):707-19. [QxMD MEDLINE Link].
Barone MV, Troncone R, Auricchio S. Gliadin peptides as triggers of the proliferative and stress/innate immune response of the celiac small intestinal mucosa. Int J Mol Sci. 2014 Nov 7. 15(11):20518-37. [QxMD MEDLINE Link].
Marsh MN, Hinde J. Inflammatory component of celiac sprue mucosa. I. Mast cells, basophils, and eosinophils. Gastroenterology. 1985 Jul. 89(1):92-101. [QxMD MEDLINE Link].
Mårild K, Stephansson O, Montgomery S, Murray JA, Ludvigsson JF. Pregnancy outcome and risk of celiac disease in offspring: a nationwide case-control study. Gastroenterology. 2012 Jan. 142(1):39-45.e3. [QxMD MEDLINE Link]. [Full Text].
Akobeng AK, Ramanan AV, Buchan I, Heller RF. Effect of breast feeding on risk of coeliac disease: a systematic review and meta-analysis of observational studies. Arch Dis Child. 2006 Jan. 91(1):39-43. [QxMD MEDLINE Link].
Olsson C, Hernell O, Hornell A, Lonnberg G, Ivarsson A. Difference in celiac disease risk between Swedish birth cohorts suggests an opportunity for primary prevention. Pediatrics. 2008 Sep. 122(3):528-34. [QxMD MEDLINE Link].
Troncone R, Auricchio S. Rotavirus and celiac disease: clues to the pathogenesis and perspectives on prevention. J Pediatr Gastroenterol Nutr. 2007 May. 44(5):527-8. [QxMD MEDLINE Link].
Myleus A, Hernell O, Gothefors L, et al. Early infections are associated with increased risk for celiac disease: an incident case-referent study. BMC Pediatr. 2012 Dec 19. 12:194. [QxMD MEDLINE Link]. [Full Text].
Myleus A, Stenlund H, Hernell O, et al. Early vaccinations are not risk factors for celiac disease. Pediatrics. 2012 Jul. 130(1):e63-70. [QxMD MEDLINE Link].
Catassi C, Kryszak D, Louis-Jacques O, et al. Detection of celiac disease in primary care: a multicenter case-finding study in North America. Am J Gastroenterol. 2007 Jul. 102(7):1454-60. [QxMD MEDLINE Link].
Riddle MS, Murray JA, Porter CK. The incidence and risk of celiac disease in a healthy US adult population. Am J Gastroenterol. 2012 Aug. 107(8):1248-55. [QxMD MEDLINE Link].
Catassi C, Kryszak D, Bhatti B, et al. Natural history of celiac disease autoimmunity in a USA cohort followed since 1974. Ann Med. 2010 Oct. 42(7):530-8. [QxMD MEDLINE Link].
Catassi C, Cobellis G. Coeliac disease epidemiology is alive and kicking, especially in the developing world. Dig Liver Dis. 2007 Oct. 39(10):908-10. [QxMD MEDLINE Link].
Dydensborg S, Toftedal P, Biaggi M, Lillevang ST, Hansen DG, Husby S. Increasing prevalence of coeliac disease in Denmark: a linkage study combining national registries. Acta Paediatr. 2012 Feb. 101(2):179-84. [QxMD MEDLINE Link].
Lohi S, Mustalahti K, Kaukinen K, et al. Increasing prevalence of coeliac disease over time. Aliment Pharmacol Ther. 2007 Nov 1. 26(9):1217-25. [QxMD MEDLINE Link].
Walker MM, Murray JA, Ronkainen J, et al. Detection of celiac disease and lymphocytic enteropathy by parallel serology and histopathology in a population-based study. Gastroenterology. 2010 Jul. 139(1):112-9. [QxMD MEDLINE Link].
Bach JF. The effect of infections on susceptibility to autoimmune and allergic diseases. N Engl J Med. 2002 Sep 19. 347(12):911-20. [QxMD MEDLINE Link].
Marild K, Stephansson O, Montgomery S, Murray JA, Ludvigsson JF. Pregnancy outcome and risk of celiac disease in offspring: a nationwide case-control study. Gastroenterology. 2012 Jan. 142(1):39-45.e3. [QxMD MEDLINE Link].
Myleus A, Ivarsson A, Webb C, et al. Celiac disease revealed in 3% of Swedish 12-year-olds born during an epidemic. J Pediatr Gastroenterol Nutr. 2009 Aug. 49(2):170-6. [QxMD MEDLINE Link].
Zingone F, West J, Crooks CJ, et al. Socioeconomic variation in the incidence of childhood coeliac disease in the UK. Arch Dis Child. 2015 May. 100(5):466-73. [QxMD MEDLINE Link].
Lewis R. Pediatric celiac disease diagnoses tripled in 20 years. Medscape Medical News. January 23, 2015. Available at http://www.medscape.com/viewarticle/838622. Accessed: September 30, 2015.
Silano M, Volta U, Vincenzi AD, Dessi M, Vincenzi MD. Effect of a gluten-free diet on the risk of enteropathy-associated T-cell lymphoma in celiac disease. Dig Dis Sci. 2008 Apr. 53(4):972-6. [QxMD MEDLINE Link].
Sharaiha RZ, Lebwohl B, Reimers L, Bhagat G, Green PH, Neugut AI. Increasing incidence of enteropathy-associated T-cell lymphoma in the United States, 1973-2008. Cancer. 2012 Aug 1. 118(15):3786-92. [QxMD MEDLINE Link].
Metzger MH, Heier M, Maki M, et al. Mortality excess in individuals with elevated IgA anti-transglutaminase antibodies: the KORA/MONICA Augsburg cohort study 1989-1998. Eur J Epidemiol. 2006. 21(5):359-65. [QxMD MEDLINE Link].
Akobeng AK, Thomas AG. Systematic review: tolerable amount of gluten for people with coeliac disease. Aliment Pharmacol Ther. 2008 Jun 1. 27(11):1044-52. [QxMD MEDLINE Link].
Canova C, Pitter G, Zanier L, Simonato L, Michaelsson K, Ludvigsson JF. Risk of fractures in youths with celiac disease: a population-based study. J Pediatr. 2018 Jul. 198:117-120. [QxMD MEDLINE Link].
Sansotta N, Amirikian K, Guandalini S, Jericho H. Celiac Disease Symptom Resolution: Effectiveness of the Gluten-free Diet. J Pediatr Gastroenterol Nutr. 2018. 66:48-52. [QxMD MEDLINE Link].
Jericho H, Sansotta N, Guandalini S. Extraintestinal Manifestations of Celiac Disease: Effectiveness of the Gluten-Free Diet. J Pediatr Gastroenterol Nutr. 2017. 65:75-79. [QxMD MEDLINE Link].
Veeraraghavan G, Therrien A, Degroote M, McKeown A, Mitchell PD, Silvester JA, et al. Non-responsive celiac disease in children on a gluten free diet. World J Gastroenterol. 2021 Apr 7. 27 (13):1311-20. [QxMD MEDLINE Link]. [Full Text].
Therrien A, Silvester JA, Leonard MM, Leffler DA, Fasano A, Kelly CP. Enteric-Release Budesonide May Be Useful in the Management of Non-Responsive Celiac Disease. Dig Dis Sci. 2021 Jun. 66 (6):1989-97. [QxMD MEDLINE Link]. [Full Text].
Ludvigsson JF, Agreus L, Ciacci C, et al. Transition from childhood to adulthood in coeliac disease: the Prague consensus report. Gut. 2016 Aug. 65(8):1242-51. [QxMD MEDLINE Link].
[Guideline] Leonard MM, Lebwohl B, Rubio-Tapia A, Biagi F. AGA Clinical Practice Update on the Evaluation and Management of Seronegative Enteropathies: Expert Review. Gastroenterology. 2021 Jan. 160 (1):437-44. [QxMD MEDLINE Link].
Marild K, Stordal K, Bulik CM, et al. Celiac disease and anorexia nervosa: a nationwide study. Pediatrics. 2017 May. 139(5):[QxMD MEDLINE Link]. [Full Text].
Lewis R. Celiac disease and anorexia nervosa linked, study shows. Medscape Medical News. April 03, 2017. Available at http://www.medscape.com/viewarticle/878092. Accessed: April 05, 2017.
Campisi G, Di Liberto C, Carroccio A, et al. Coeliac disease: oral ulcer prevalence, assessment of risk and association with gluten-free diet in children. Dig Liver Dis. 2008 Feb. 40(2):104-7. [QxMD MEDLINE Link].
Baydoun A, Maakaron JE, Halawi H, Abou Rahal J, Taher AT. Hematological manifestations of celiac disease. Scand J Gastroenterol. 2012 Dec. 47(12):1401-11. [QxMD MEDLINE Link].
Vajro P, Paolella G, Maggiore G, Giordano G. Pediatric celiac disease, cryptogenic hypertransaminasemia, and autoimmune hepatitis. J Pediatr Gastroenterol Nutr. 2013 Jun. 56(6):663-70. [QxMD MEDLINE Link].
Tunio NA, Mansoor E, Sheriff MZ, Cooper GS, Sclair SN, Cohen SM. Epidemiology of Autoimmune Hepatitis (AIH) in the United States Between 2014 and 2019: A Population-based National Study. J Clin Gastroenterol. 2021. 55:903-910. [QxMD MEDLINE Link].
Di Biase AR, Colecchia A, Scaioli E, et al. Autoimmune liver diseases in a paediatric population with coeliac disease - a 10-year single-centre experience. Aliment Pharmacol Ther. 2010 Jan 15. 31 (2):253-60. [QxMD MEDLINE Link].
Caprai S, Vajro P, Ventura A, Sciveres M, Maggiore G, SIGENP Study Group for Autoimmune Liver Disorders in Celiac Disease. Autoimmune liver disease associated with celiac disease in childhood: a multicenter study. Clin Gastroenterol Hepatol. 2008 Jul. 6 (7):803-6. [QxMD MEDLINE Link].
Lionetti E, Francavilla R, Pavone P, et al. The neurology of coeliac disease in childhood: what is the evidence? A systematic review and meta-analysis. Dev Med Child Neurol. 2010 Aug. 52(8):700-7. [QxMD MEDLINE Link].
Lebwohl B, Haggård L, Emilsson L, et al. Psychiatric Disorders in Patients With a Diagnosis of Celiac Disease During Childhood From 1973 to 2016. Clin Gastroenterol Hepatol. 2021 Oct. 19 (10):2093-2101.e13. [QxMD MEDLINE Link].
Pope R, Sheiner E. Celiac disease during pregnancy: to screen or not to screen?. Arch Gynecol Obstet. 2009 Jan. 279(1):1-3. [QxMD MEDLINE Link].
Marild K, Stordal K, Hagman A, Ludvigsson JF. Turner syndrome and celiac disease: a case-control study. Pediatrics. 2016 Feb. 137(2):e20152232. [QxMD MEDLINE Link].
Osterweil N. Celiac disease more common in patients with Turner syndrome. Medscape Medical News. January 14, 2016. Available at http://www.medscape.com/viewarticle/857225. Accessed: June 27, 2016.
Doyle JB, Lebwohl B, Askling J, et al. Risk of Juvenile Idiopathic Arthritis and Rheumatoid Arthritis in Patients With Celiac Disease: A Population-Based Cohort Study. Am J Gastroenterol. 2022 Dec 1. 117 (12):1971-81. [QxMD MEDLINE Link].
Crist C. Celiac Disease Linked to Higher Risk for Rheumatoid Arthritis, Juvenile Idiopathic Arthritis. Medscape Medical News. 2022 Nov 17. Available at https://www.medscape.com/viewarticle/984209.
Paez MA, Gramelspacher AM, Sinacore J, Winterfield L, Venu M. Delay in Diagnosis of Celiac Disease in Patients Without Gastrointestinal Complaints. Am J Med. 2017 Nov. 130 (11):1318-23. [QxMD MEDLINE Link].
Badizadegan K, Vanlandingham DM, Hampton W, Thompson KM. Value of biopsy in a cohort of children with high-titer celiac serologies: observation of dynamic policy differences between Europe and North America. BMC Health Serv Res. 2020 Oct 20. 20 (1):962. [QxMD MEDLINE Link]. [Full Text].
Cristofori, F., F. S. D'Abramo, V. Rutigliano, V. N. Dargenio, S. Castellaneta, et al. Esophageal Eosinophilia and Eosinophilic Esophagitis in Celiac Children: A Ten Year Prospective Observational Study. Nutrients. 2021. 13:3755. [QxMD MEDLINE Link].
Marsh MN. Gluten, major histocompatibility complex, and the small intestine. A molecular and immunobiologic approach to the spectrum of gluten sensitivity ('celiac sprue'). Gastroenterology. 1992 Jan. 102 (1):330-54. [QxMD MEDLINE Link].
Kurppa K, Collin P, Viljamaa M, et al. Diagnosing mild enteropathy celiac disease: a randomized, controlled clinical study. Gastroenterology. 2009 Mar. 136(3):816-23. [QxMD MEDLINE Link].
[Guideline] Mearin ML, Agardh D, Antunes H, et al. ESPGHAN Position Paper on Management and Follow-up of Children and Adolescents With Celiac Disease. J Pediatr Gastroenterol Nutr. 2022 Sep 1. 75 (3):369-86. [QxMD MEDLINE Link].
Sansotta N, Guandalini S, Romano S, et al. The Gluten Free Diet's Impact on Growth in Children with Celiac Disease in Two Different Countries. Nutrients. 2020 May 26. 12 (6):[QxMD MEDLINE Link]. [Full Text].
Rubio-Tapia A, Rahim MW, See JA, Lahr BD, Wu TT, Murray JA. Mucosal recovery and mortality in adults with celiac disease after treatment with a gluten-free diet. Am J Gastroenterol. 2010 Jun. 105(6):1412-20. [QxMD MEDLINE Link].
Diamanti A, Ferretti F, Guglielmi R, et al. Thyroid autoimmunity in children with coeliac disease: a prospective survey. Arch Dis Child. 2011 Nov. 96(11):1038-41. [QxMD MEDLINE Link].
Auricchio R, Troncone R. Can Celiac Disease Be Prevented?. Front Immunol. 2021, May 14. 12:672148. [QxMD MEDLINE Link].
Leffler DA, Dennis M, Edwards George JB, et al. A simple validated gluten-free diet adherence survey for adults with celiac disease. Clin Gastroenterol Hepatol. 2009 May. 7(5):530-6, 536.e1-2. [QxMD MEDLINE Link].
[Guideline] Rubio-Tapia A, Hill ID, Semrad C, Kelly CP, Lebwohl B. American College of Gastroenterology Guidelines Update: Diagnosis and Management of Celiac Disease. Am J Gastroenterol. 2023 Jan 1. 118 (1):59-76. [QxMD MEDLINE Link]. [Full Text].
[Guideline] Husby S, Koletzko S, Korponay-Szabo I, et al. European Society Paediatric Gastroenterology, Hepatology and Nutrition guidelines for diagnosing coeliac disease 2020. J Pediatr Gastroenterol Nutr. 2019 Oct 17. [QxMD MEDLINE Link].
[Guideline] Rubio-Tapia A, Murray JA. Updated guidelines by the European Society for the Study of Coeliac Disease. United European Gastroenterol J. 2019 Jun. 7(5):581-2. [QxMD MEDLINE Link]. [Full Text].
[Guideline] Al-Toma A, Volta U, Auricchio R, et al. European Society for the Study of Coeliac Disease (ESsCD) guideline for coeliac disease and other gluten-related disorders. United European Gastroenterol J. 2019 Jun. 7(5):583-613. [QxMD MEDLINE Link]. [Full Text].
Kulkarni A, Patel S, Khanna D, Parmar MS. Current pharmacological approaches and potential future therapies for Celiac disease. Eur J Pharmacol. 2021 Aug 18. 909:174434. [QxMD MEDLINE Link].
Murray JA, Syage JA, Wu TT, Dickason MA, Ramos AG, Van Dyke C, et al. Latiglutenase Protects the Mucosa and Attenuates Symptom Severity in Patients With Celiac Disease Exposed to a Gluten Challenge. Gastroenterology. 2022. 163(6):1510-1521. [QxMD MEDLINE Link].

Media Gallery

Potbelly and muscle wasting in a child with celiac disease.
The celiac iceberg.
Presentations of celiac disease.
Extraintestinal manifestations of celiac disease.
GI signs and symptoms of celiac disease.
Approximate prevalence of celiac disease in other autoimmune disorders.

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Author

Stefano Guandalini, MD, AGAF Founder and Director Emeritus, Celiac Disease Center, University of Chicago; Former Chief, Section of Pediatric Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, University of Chicago Medicine; Professor Emeritus, The University of Chicago Pritzker School of Medicine

Stefano Guandalini, MD, AGAF is a member of the following medical societies: American Gastroenterological Association, European Society for Paediatric Gastroenterology, Hepatology and Nutrition, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, North American Society for the Study of Celiac Disease

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Imaware.health.

Coauthor(s)

Phyllis A Vallee, MD Associate Program Director, Department of Emergency Medicine, Henry Ford Hospital

Phyllis A Vallee, MD is a member of the following medical societies: American Academy of Emergency Medicine, American Medical Association, Michigan State Medical Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Chief Editor

Carmen Cuffari, MD Associate Professor, Department of Pediatrics, Division of Gastroenterology/Nutrition, Johns Hopkins University School of Medicine

Carmen Cuffari, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, Royal College of Physicians and Surgeons of Canada

Disclosure: Received honoraria from Prometheus Laboratories for speaking and teaching; Received honoraria from Abbott Nutritionals for speaking and teaching. for: Abbott Nutritional, Abbvie, speakers' bureau.

Additional Contributors

Jorge H Vargas, MD Professor of Pediatrics and Clinical Professor of Pediatric Gastroenterology, University of California, Los Angeles, David Geffen School of Medicine; Consulting Physician, Department of Pediatrics, University of California at Los Angeles Health System

Jorge H Vargas, MD is a member of the following medical societies: American Liver Foundation, Latin American Society of Pediatric Gastroenterology, Hepatology & Nutrition, American Society for Gastrointestinal Endoscopy, American Society for Parenteral and Enteral Nutrition, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author Ginette V Busschots, MD, to the writing and development of this article.