Management of celiac disease in adults

INTRODUCTION

Celiac disease can be defined as a condition in which there is an abnormal small intestinal mucosa that improves morphologically when treated with a gluten-free diet and relapses when gluten is reintroduced. The disorder is commonly referred to as "celiac sprue" or "gluten-sensitive enteropathy" in the United States. It was first described by Samuel Gee in 1887, although a similar description of a chronic malabsorptive disorder was recorded as far back as the second century AD [1].

As a general rule, there are six key elements in the management of patients with celiac disease, which can be summarized with the following acronym [2]:

  • Consultation with a skilled dietitian
  • Education about the disease
  • Lifelong adherence to a gluten-free diet
  • Identification and treatment of nutritional deficiencies
  • Access to an advocacy group
  • Continuous long-term follow-up by a multidisciplinary team

The recommendations made in this topic are generally consistent with the guidelines on the recognition and assessment of celiac disease in children and adults from the British National Institute for Health and Clinical Excellence in 2009 and the American Gastroenterological Association (AGA) 2006 guidelines for the diagnosis and management of celiac disease [3]. The discussion below also reflects a 2004 consensus statement issued by the National Institutes of Health [2].

The management of celiac disease and its complications will be reviewed here. Its pathogenesis, clinical manifestations, and diagnosis are discussed separately. (See "Pathogenesis, epidemiology, and clinical manifestations of celiac disease in adults" and "Diagnosis of celiac disease".)

                      

Subscribers log in here

To continue reading this article you must have access through your hospital or your group practice, log in to your personal subscription, or purchase a personal subscription. For more information, click below.
Literature review current through: 20.6: May 2012
This topic last updated: Dec 6, 2011
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use (click here) ©2012 UpToDate, Inc.
References
Top
  1. Adams F. The extant works of Aretaeus the Cappadocian, London Sydenham Society, 1856.
  2. National Institutes of Health Consensus Development Conference Statement. Celiac Disease 2004. Available at: http://consensus.nih.gov/ (Accessed on March 11, 2011).
  3. AGA Institute. AGA Institute Medical Position Statement on the Diagnosis and Management of Celiac Disease. Gastroenterology 2006; 131:1977.
  4. Ravelli A, Bolognini S, Gambarotti M, Villanacci V. Variability of histologic lesions in relation to biopsy site in gluten-sensitive enteropathy. Am J Gastroenterol 2005; 100:177.
  5. Janatuinen EK, Pikkarainen PH, Kemppainen TA, et al. A comparison of diets with and without oats in adults with celiac disease. N Engl J Med 1995; 333:1033.
  6. Srinivasan U, Leonard N, Jones E, et al. Absence of oats toxicity in adult coeliac disease. BMJ 1996; 313:1300.
  7. Hardman CM, Garioch JJ, Leonard JN, et al. Absence of toxicity of oats in patients with dermatitis herpetiformis. N Engl J Med 1997; 337:1884.
  8. Janatuinen EK, Kemppainen TA, Julkunen RJ, et al. No harm from five year ingestion of oats in coeliac disease. Gut 2002; 50:332.
  9. Högberg L, Laurin P, Fälth-Magnusson K, et al. Oats to children with newly diagnosed coeliac disease: a randomised double blind study. Gut 2004; 53:649.
  10. Peräaho M, Kaukinen K, Mustalahti K, et al. Effect of an oats-containing gluten-free diet on symptoms and quality of life in coeliac disease. A randomized study. Scand J Gastroenterol 2004; 39:27.
  11. Janatuinen EK, Kemppainen TA, Pikkarainen PH, et al. Lack of cellular and humoral immunological responses to oats in adults with coeliac disease. Gut 2000; 46:327.
  12. Sturgess R, Day P, Ellis HJ, et al. Wheat peptide challenge in coeliac disease. Lancet 1994; 343:758.
  13. Shidrawi RG, Day P, Przemioslo R, et al. In vitro toxicity of gluten peptides in coeliac disease assessed by organ culture. Scand J Gastroenterol 1995; 30:758.
  14. Schmitz J. Lack of oats toxicity in coeliac disease. BMJ 1997; 314:159.
  15. Comino I, Real A, de Lorenzo L, et al. Diversity in oat potential immunogenicity: basis for the selection of oat varieties with no toxicity in coeliac disease. Gut 2011; 60:915.
  16. Lundin KE, Nilsen EM, Scott HG, et al. Oats induced villous atrophy in coeliac disease. Gut 2003; 52:1649.
  17. Thompson T. Gluten contamination of commercial oat products in the United States. N Engl J Med 2004; 351:2021.
  18. Hall NJ, Rubin G, Charnock A. Systematic review: adherence to a gluten-free diet in adult patients with coeliac disease. Aliment Pharmacol Ther 2009; 30:315.
  19. Shaker JL, Brickner RC, Findling JW, et al. Hypocalcemia and skeletal disease as presenting features of celiac disease. Arch Intern Med 1997; 157:1013.
  20. West J, Logan RF, Smith CJ, et al. Malignancy and mortality in people with coeliac disease: population based cohort study. BMJ 2004; 329:716.
  21. Green PH, Fleischauer AT, Bhagat G, et al. Risk of malignancy in patients with celiac disease. Am J Med 2003; 115:191.
  22. Holmes GK, Prior P, Lane MR, et al. Malignancy in coeliac disease--effect of a gluten free diet. Gut 1989; 30:333.
  23. Corrao G, Corazza GR, Bagnardi V, et al. Mortality in patients with coeliac disease and their relatives: a cohort study. Lancet 2001; 358:356.
  24. Askling J, Linet M, Gridley G, et al. Cancer incidence in a population-based cohort of individuals hospitalized with celiac disease or dermatitis herpetiformis. Gastroenterology 2002; 123:1428.
  25. Collin P, Reunala T, Pukkala E, et al. Coeliac disease--associated disorders and survival. Gut 1994; 35:1215.
  26. Ventura A, Magazzù G, Greco L. Duration of exposure to gluten and risk for autoimmune disorders in patients with celiac disease. SIGEP Study Group for Autoimmune Disorders in Celiac Disease. Gastroenterology 1999; 117:297.
  27. Cosnes J, Cellier C, Viola S, et al. Incidence of autoimmune diseases in celiac disease: protective effect of the gluten-free diet. Clin Gastroenterol Hepatol 2008; 6:753.
  28. Sategna Guidetti C, Solerio E, Scaglione N, et al. Duration of gluten exposure in adult coeliac disease does not correlate with the risk for autoimmune disorders. Gut 2001; 49:502.
  29. Nørgård B, Fonager K, Sørensen HT, Olsen J. Birth outcomes of women with celiac disease: a nationwide historical cohort study. Am J Gastroenterol 1999; 94:2435.
  30. Ludvigsson JF, Montgomery SM, Ekbom A. Celiac disease and risk of adverse fetal outcome: a population-based cohort study. Gastroenterology 2005; 129:454.
  31. Collin P, Thorell L, Kaukinen K, Mäki M. The safe threshold for gluten contamination in gluten-free products. Can trace amounts be accepted in the treatment of coeliac disease? Aliment Pharmacol Ther 2004; 19:1277.
  32. Pink IJ, Creamer B. Response to a gluten-free diet of patients with the coeliac syndrome. Lancet 1967; 1:300.
  33. MACDONALD WC, BRANDBORG LL, FLICK AL, et al. STUDIES OF CELIAC SPRUE. IV. THE RESPONSE OF THE WHOLE LENGTH OF THE SMALL BOWEL TO A GLUTEN-FREE DIET. Gastroenterology 1964; 47:573.
  34. Bürgin-Wolff A, Gaze H, Hadziselimovic F, et al. Antigliadin and antiendomysium antibody determination for coeliac disease. Arch Dis Child 1991; 66:941.
  35. Kelly CP, Feighery CF, Gallagher RB, et al. Mucosal and systemic IgA anti-gliadin antibody in celiac disease. Contrasting patterns of response in serum, saliva, and intestinal secretions. Dig Dis Sci 1991; 36:743.
  36. Kilander AF, Nilsson LA, Gillberg R. Serum antibodies to gliadin in coeliac disease after gluten withdrawal. Scand J Gastroenterol 1987; 22:29.
  37. Hopper AD, Hadjivassiliou M, Hurlstone DP, et al. What is the role of serologic testing in celiac disease? A prospective, biopsy-confirmed study with economic analysis. Clin Gastroenterol Hepatol 2008; 6:314.
  38. Kelly CP. Coeliac disease: Non-invasive tests to screen for gluten sensitive enteropathy and to monitor response to dietary therapy, Dublin University, Trinity College, Dublin 1995.
  39. Leffler DA, Edwards George JB, Dennis M, et al. A prospective comparative study of five measures of gluten-free diet adherence in adults with coeliac disease. Aliment Pharmacol Ther 2007; 26:1227.
  40. Vahedi K, Mascart F, Mary JY, et al. Reliability of antitransglutaminase antibodies as predictors of gluten-free diet compliance in adult celiac disease. Am J Gastroenterol 2003; 98:1079.
  41. Kapuscinska A, Zalewski T, Chorzelski TP, et al. Disease specificity and dynamics of changes in IgA class anti-endomysial antibodies in celiac disease. J Pediatr Gastroenterol Nutr 1987; 6:529.
  42. Kumar V, Lerner A, Valeski JE, et al. Endomysial antibodies in the diagnosis of celiac disease and the effect of gluten on antibody titers. Immunol Invest 1989; 18:533.
  43. Lee SK, Lo W, Memeo L, et al. Duodenal histology in patients with celiac disease after treatment with a gluten-free diet. Gastrointest Endosc 2003; 57:187.
  44. Selby WS, Painter D, Collins A, et al. Persistent mucosal abnormalities in coeliac disease are not related to the ingestion of trace amounts of gluten. Scand J Gastroenterol 1999; 34:909.
  45. Revised criteria for diagnosis of coeliac disease. Report of Working Group of European Society of Paediatric Gastroenterology and Nutrition. Arch Dis Child 1990; 65:909.
  46. KRAINICK HG, DEBATIN F, GAUTIER E, et al. [Additional research on the injurious effect of wheat flour in celiac disease.I. Acute gliadin reaction (gliadin shock)]. Helv Paediatr Acta 1958; 13:432.
  47. Selby PL, Davies M, Adams JE, Mawer EB. Bone loss in celiac disease is related to secondary hyperparathyroidism. J Bone Miner Res 1999; 14:652.
  48. Abdulkarim AS, Burgart LJ, See J, Murray JA. Etiology of nonresponsive celiac disease: results of a systematic approach. Am J Gastroenterol 2002; 97:2016.
  49. Leffler DA, Dennis M, Hyett B, et al. Etiologies and predictors of diagnosis in nonresponsive celiac disease. Clin Gastroenterol Hepatol 2007; 5:445.
  50. 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; 7:530.
  51. Shah VH, Rotterdam H, Kotler DP, et al. All that scallops is not celiac disease. Gastrointest Endosc 2000; 51:717.
  52. Fine KD, Meyer RL, Lee EL. The prevalence and causes of chronic diarrhea in patients with celiac sprue treated with a gluten-free diet. Gastroenterology 1997; 112:1830.
  53. Rubio-Tapia A, Barton SH, Rosenblatt JE, Murray JA. Prevalence of small intestine bacterial overgrowth diagnosed by quantitative culture of intestinal aspirate in celiac disease. J Clin Gastroenterol 2009; 43:157.
  54. Tursi A, Brandimarte G, Giorgetti G. High prevalence of small intestinal bacterial overgrowth in celiac patients with persistence of gastrointestinal symptoms after gluten withdrawal. Am J Gastroenterol 2003; 98:839.
  55. Carroccio A, Iacono G, Lerro P, et al. Role of pancreatic impairment in growth recovery during gluten-free diet in childhood celiac disease. Gastroenterology 1997; 112:1839.
  56. Green PH, Yang J, Cheng J, et al. An association between microscopic colitis and celiac disease. Clin Gastroenterol Hepatol 2009; 7:1210.
  57. Trier JS, Falchuk ZM, Carey MC, Schreiber DS. Celiac sprue and refractory sprue. Gastroenterology 1978; 75:307.
  58. Ryan BM, Kelleher D. Refractory celiac disease. Gastroenterology 2000; 119:243.
  59. Mulder CJ, Wahab PJ, Moshaver B, Meijer JW. Refractory coeliac disease: a window between coeliac disease and enteropathy associated T cell lymphoma. Scand J Gastroenterol Suppl 2000; :32.
  60. Cellier C, Delabesse E, Helmer C, et al. Refractory sprue, coeliac disease, and enteropathy-associated T-cell lymphoma. French Coeliac Disease Study Group. Lancet 2000; 356:203.
  61. Cellier C, Patey N, Mauvieux L, et al. Abnormal intestinal intraepithelial lymphocytes in refractory sprue. Gastroenterology 1998; 114:471.
  62. Bagdi E, Diss TC, Munson P, Isaacson PG. Mucosal intra-epithelial lymphocytes in enteropathy-associated T-cell lymphoma, ulcerative jejunitis, and refractory celiac disease constitute a neoplastic population. Blood 1999; 94:260.
  63. Olaussen RW, Løvik A, Tollefsen S, et al. Effect of elemental diet on mucosal immunopathology and clinical symptoms in type 1 refractory celiac disease. Clin Gastroenterol Hepatol 2005; 3:875.
  64. Hadithi M, Mallant M, Oudejans J, et al. 18F-FDG PET versus CT for the detection of enteropathy-associated T-cell lymphoma in refractory celiac disease. J Nucl Med 2006; 47:1622.
  65. Van Weyenberg SJ, Meijerink MR, Jacobs MA, et al. MR enteroclysis in refractory celiac disease: proposal and validation of a severity scoring system. Radiology 2011; 259:151.
  66. Al-Toma A, Verbeek WH, Hadithi M, et al. Survival in refractory coeliac disease and enteropathy-associated T-cell lymphoma: retrospective evaluation of single-centre experience. Gut 2007; 56:1373.
  67. Malamut G, Afchain P, Verkarre V, et al. Presentation and long-term follow-up of refractory celiac disease: comparison of type I with type II. Gastroenterology 2009; 136:81.
  68. Gao Y, Kristinsson SY, Goldin LR, et al. Increased risk for non-Hodgkin lymphoma in individuals with celiac disease and a potential familial association. Gastroenterology 2009; 136:91.
  69. Rubio-Tapia A, Kelly DG, Lahr BD, et al. Clinical staging and survival in refractory celiac disease: a single center experience. Gastroenterology 2009; 136:99.
  70. McCashland TM, Donovan JP, Strobach RS, et al. Collagenous enterocolitis: a manifestation of gluten-sensitive enteropathy. J Clin Gastroenterol 1992; 15:45.
  71. Baker AL, Rosenberg IH. Refractory sprue: recovery after removal of nongluten dietary proteins. Ann Intern Med 1978; 89:505.
  72. Rolny P, Sigurjonsdottir HA, Remotti H, et al. Role of immunosuppressive therapy in refractory sprue-like disease. Am J Gastroenterol 1999; 94:219.
  73. Vaidya A, Bolanos J, Berkelhammer C. Azathioprine in refractory sprue. Am J Gastroenterol 1999; 94:1967.
  74. Mauriño E, Niveloni S, Cherñavsky A, et al. Azathioprine in refractory sprue: results from a prospective, open-label study. Am J Gastroenterol 2002; 97:2595.
  75. Daum S, Ipczynski R, Heine B, et al. Therapy with budesonide in patients with refractory sprue. Digestion 2006; 73:60.
  76. Brar P, Lee S, Lewis S, et al. Budesonide in the treatment of refractory celiac disease. Am J Gastroenterol 2007; 102:2265.
  77. Jamma S, Leffler DA, Dennis M, et al. Small intestinal release mesalamine for the treatment of refractory celiac disease type I. J Clin Gastroenterol 2011; 45:30.
  78. Vivas S, Ruiz de Morales JM, Ramos F, Suárez-Vilela D. Alemtuzumab for refractory celiac disease in a patient at risk for enteropathy-associated T-cell lymphoma. N Engl J Med 2006; 354:2514.
  79. Verbeek WH, Mulder CJ, Zweegman S. Alemtuzumab for refractory celiac disease. N Engl J Med 2006; 355:1396.
  80. Al-Toma A, Goerres MS, Meijer JW, et al. Cladribine therapy in refractory celiac disease with aberrant T cells. Clin Gastroenterol Hepatol 2006; 4:1322.
  81. Ashton-Key M, Diss TC, Pan L, et al. Molecular analysis of T-cell clonality in ulcerative jejunitis and enteropathy-associated T-cell lymphoma. Am J Pathol 1997; 151:493.
  82. Bayless TM, Kapelowitz RF, Shelley WM, et al. Intestinal ulceration--a complication of celiac disease. N Engl J Med 1967; 276:996.
  83. Isaacson PG. Intestinal lymphoma and enteropathy. J Pathol 1995; 177:111.
  84. Egan LJ, Walsh SV, Stevens FM, et al. Celiac-associated lymphoma. A single institution experience of 30 cases in the combination chemotherapy era. J Clin Gastroenterol 1995; 21:123.
  85. Walters JR, Banks LM, Butcher GP, Fowler CR. Detection of low bone mineral density by dual energy x ray absorptiometry in unsuspected suboptimally treated coeliac disease. Gut 1995; 37:220.
  86. Mustalahti K, Collin P, Sievänen H, et al. Osteopenia in patients with clinically silent coeliac disease warrants screening. Lancet 1999; 354:744.
  87. Meyer D, Stavropolous S, Diamond B, et al. Osteoporosis in a north american adult population with celiac disease. Am J Gastroenterol 2001; 96:112.
  88. Cooke WT, Holmes GK. Clinical presentation. In: Coeliac Disease, Cooke WT, Holmes GK (Eds), Churchill Livingston, London 1984. p.90.
  89. Scott EM, Gaywood I, Scott BB. Guidelines for osteoporosis in coeliac disease and inflammatory bowel disease. British Society of Gastroenterology. Gut 2000; 46 Suppl 1:i1.
  90. Fry L. Dermatitis herpetiformis. Baillieres Clin Gastroenterol 1995; 9:371.
  91. Garioch JJ, Lewis HM, Sargent SA, et al. 25 years' experience of a gluten-free diet in the treatment of dermatitis herpetiformis. Br J Dermatol 1994; 131:541.
  92. Book L, Zone JJ, Neuhausen SL. Prevalence of celiac disease among relatives of sib pairs with celiac disease in U.S. families. Am J Gastroenterol 2003; 98:377.
  93. Gudjónsdóttir AH, Nilsson S, Ek J, et al. The risk of celiac disease in 107 families with at least two affected siblings. J Pediatr Gastroenterol Nutr 2004; 38:338.
  94. Greco L, Romino R, Coto I, et al. The first large population based twin study of coeliac disease. Gut 2002; 50:624.
  95. Rubio-Tapia A, Van Dyke CT, Lahr BD, et al. Predictors of family risk for celiac disease: a population-based study. Clin Gastroenterol Hepatol 2008; 6:983.
  96. Fasano A, Berti I, Gerarduzzi T, et al. Prevalence of celiac disease in at-risk and not-at-risk groups in the United States: a large multicenter study. Arch Intern Med 2003; 163:286.