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Serum IgA htTG in concert with small intestinal histology are a powerful diagnostic duo,and when both are performed correctly and are positive, provide a near absolute diagnosis ofuntreated or incompletely treated celiac disease. As a result, other diagnostic criteria such asclinical response to a gluten-free diet, histological response to a gluten-free diet, and glutenchallenge now play lesser roles in diagnosis.The remarkably high positive predictive value of IgA EMA or high-titer IgA htTGserology has led to debate as to whether endoscopy with biopsy is required for diagnosis—especially in instances where the pretest probability of celiac disease was already high. Eachpatient must be the final and individual arbiter of this debate, however, most expert celiacclinicians advocate strongly for histological confirmation of a diagnosis of celiac disease for thefollowing reasons: (1) celiac disease is defined by an enteropathy, not by the presence of a serumautoantibody; (2) positive serology in the absence of significant enteropathy does occur and doesnot predicate lifelong treatment with a gluten-free diet; (3) endoscopy with biopsy is readilyavailable in the United States and is relatively simple and safe; (4) it is difficult to either confirmor refute the diagnosis after dietary treatment is initiated; and (5) the cost of a false-positivediagnosis is enormous in that a patient may needlessly and fruitlessly adhere to an inconvenientand expensive gluten-free diet for decades while the actual etiology of their initial symptoms andsigns remains undiagnosed.A suggested clinical algorithm for diagnosis of celiac disease is shown in figure 1. Thefirst and essential step is to consider the diagnosis. The next is to determine—on the basis of thepatient’s clinical presentation, medical and family history, and available laboratory data—whether a diagnosis of celiac disease is highly likely (e.g., greater than 10 percent pretestprobability) or less likely. For those with a lower pretest probability, IgA htTG serology is thecurrent test of choice. A positive IgA htTG serology indicates the need for small bowel biopsy todiagnose celiac disease prior to lifelong treatment with a gluten-free diet. For those who arenegative by IgA htTG, a serum IgA level may be checked to exclude IgA deficiency; however,but this additional test has a relatively low diagnostic impact. Antigliadin serology is largelycounterproductive especially in individuals older than 2 years because of the plethora offalse-positive results in low-risk populations. For high-risk populations (e.g., greater than10 percent pretest probability), the likelihood of a false-negative IgA htTG serology may beunacceptably high; therefore, both serology and endoscopy with biopsy should be performed atthe outset. Additional diagnostic steps are needed in instances where IgA htTG serology ispositive but biopsy histology is reportedly normal, or where IgA htTG serology is negative butthe small intestinal morphology and inflammatory infiltrate are consistent with a celiac lesion.There is no place for a trial of a gluten-free diet prior to diagnostic testing for celiac disease.Unfortunately, in some instances, patients present on treatment with a gluten-free diet in theabsence of diagnostic pretreatment serology or histopathology. In such circumstances, glutenchallenge followed by serology and biopsy is needed to establish a secure diagnosis.When celiac disease is diagnosed, there should be an evaluation for deficiencies innutrients such as iron, folic acid, vitamin B12 and vitamin D. The patient should be educated onthe cause of intestinal injury and malabsorption in celiac disease, encouraged to adhere to alifelong gluten-free diet, and provided with access to expert nutritional counseling. The clinicaland serologic response to treatment should be monitored and reversal of any identifiednutritional deficiencies confirmed. With accurate diagnosis and appropriate treatment thesubstantial morbidity associated with unrecognized symptomatic celiac disease can be avoidedcompletely.34
Considera diagnosisof celiac diseaseSerum IgA htTG 2Determinethe pretestprobability>10% 1Serum IgA htTGandSI biopsy histology
Page 1 and 2: NIH Consensus Development Conferenc
Page 3 and 4: III. What Are the Manifestations an
Page 5 and 6: • What is the management of celia
Page 7 and 8: Monday, June 28, 2004 (continued)I.
Page 9 and 10: Monday, June 28, 2004 (continued)II
Page 11 and 12: Wednesday, June 30, 2004 (continued
Page 13 and 14: Lisa H. RichardsonConsumer Represen
Page 15 and 16: Ciaran P. Kelly, M.D.Director, Celi
Page 17 and 18: Van S. Hubbard, M.D., Ph.D.Director
Page 19 and 20: AbstractsThe following are abstract
Page 21 and 22: susceptibility (e.g., DR17 homozygo
Page 23 and 24: The Pathology of Celiac DiseaseDavi
Page 25 and 26: In this regard, the transport pathw
Page 27 and 28: for the IgG-based test, while speci
Page 29 and 30: 15. de Lecea A, Ribes-Koninckx C, P
Page 31: Clinical Algorithm in Celiac Diseas
Page 35 and 36: There are populations at particular
Page 37 and 38: Serological Testing for Celiac Dise
Page 39 and 40: Estimates of the sensitivity of the
Page 41 and 42: the risk and severity of CD may als
Page 43 and 44: What Are the Prevalence and Inciden
Page 45 and 46: ascribed to excess menstrual loss.
Page 47 and 48: ReferencesFamily History of Celiac
Page 49 and 50: Carroccio A, Iannitto E, Cavataio F
Page 51 and 52: identified by surveys or through so
Page 53 and 54: Clinical Presentation of Celiac Dis
Page 55 and 56: een widely reported. The question r
Page 57 and 58: The Many Faces of Celiac Disease: C
Page 59 and 60: References1. Green PH, Jabri B. Coe
Page 61 and 62: Association of Celiac Disease and G
Page 63 and 64: Does the Gluten-Free Diet Protect F
Page 65 and 66: Skin Manifestations of Celiac Disea
Page 67 and 68: allow a better understanding of the
Page 69 and 70: 4. Henriksson KG, Hallert C, Walan
Page 71 and 72: characterized, clinically-identifie
Page 73 and 74: 10. Hoffenberg EJ, Emery LM, Barrig
Page 75 and 76: ataxia, epilepsy with posterior cer
Page 77 and 78: Consequences of Testing for Celiac
Page 79 and 80: Osteoporosis/FracturesThere were 11
Page 81 and 82: Dietary Guidelines for Celiac Disea
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21. Thompson T. Thiamin, riboflavin
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In order to effectively counsel ind
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9. Hallert C, Granno C, Hulten S, M
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adhered to the GFD after more than
Page 94 and 95:
Patient education, close supervisio
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26. Mustalahti K, Lohiniemi S, Laip
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