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Endomysial antibody-negative coeliac disease: clinical characteristics and intestinal autoantibody deposits.
Gut 2006; 55(12):1746-53Gut

Abstract

BACKGROUND

Some patients with untreated coeliac disease are negative for serum endomysial autoantibodies (EmA) targeted against transglutaminase 2 (TG2).

AIMS

To evaluate the clinical and histological features of EmA-negative coeliac disease, and to examine whether EmA-equivalent autoantibodies against TG2 can be seen in the small-bowel mucosa when absent in serum.

PATIENTS

Serum EmA was studied in 177 biopsy-proved specimens from adult patients with coeliac disease. 20 patients with intestinal diseases served as non-coeliac controls; three had autoimmune enteropathy with villous atrophy.

METHODS

Clinical manifestations, small-bowel mucosal morphology, intraepithelial inflammation and TG2-specific extracellular immunoglobulin A (IgA) deposits were investigated in both serum EmA-negative and EmA-positive patients.

RESULTS

22 patients with IgA-competent coeliac disease were negative for serum EmA. Three of these had small-bowel lymphoma. Patients with EmA-negative coeliac disease were older, had abdominal symptoms more often, and the density of gammadelta+ intraepithelial lymphocytes in their intestinal mucosa was lower than in EmA-positive patients; otherwise the histology was similar. All serum EmA-negative patients with coeliac disease, but none of the disease controls, had gluten-dependent mucosal IgA deposits alongside TG2 in the small-bowel mucosal specimens. In vivo deposited IgA was shown to be TG2-specific by its ability to bind recombinant TG2.

CONCLUSIONS

Negative serum EmA might be associated with advanced coeliac disease. TG2-targeted autoantibodies were deposited in the small-bowel mucosa even when absent in serum. This finding can be used in the diagnosis of seronegative coeliac disease when the histology is equivocal. It may also be helpful in the differential diagnosis between autoimmune enteropathy and coeliac disease.

Authors+Show Affiliations

Medical School, University of Tampere, Finland 33014. katri.kaukinen@uta.fiNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

16571636

Citation

Salmi, T T., et al. "Endomysial Antibody-negative Coeliac Disease: Clinical Characteristics and Intestinal Autoantibody Deposits." Gut, vol. 55, no. 12, 2006, pp. 1746-53.
Salmi TT, Collin P, Korponay-Szabó IR, et al. Endomysial antibody-negative coeliac disease: clinical characteristics and intestinal autoantibody deposits. Gut. 2006;55(12):1746-53.
Salmi, T. T., Collin, P., Korponay-Szabó, I. R., Laurila, K., Partanen, J., Huhtala, H., ... Kaukinen, K. (2006). Endomysial antibody-negative coeliac disease: clinical characteristics and intestinal autoantibody deposits. Gut, 55(12), pp. 1746-53.
Salmi TT, et al. Endomysial Antibody-negative Coeliac Disease: Clinical Characteristics and Intestinal Autoantibody Deposits. Gut. 2006;55(12):1746-53. PubMed PMID: 16571636.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Endomysial antibody-negative coeliac disease: clinical characteristics and intestinal autoantibody deposits. AU - Salmi,T T, AU - Collin,P, AU - Korponay-Szabó,I R, AU - Laurila,K, AU - Partanen,J, AU - Huhtala,H, AU - Király,R, AU - Lorand,L, AU - Reunala,T, AU - Mäki,M, AU - Kaukinen,K, Y1 - 2006/03/29/ PY - 2006/3/31/pubmed PY - 2007/1/4/medline PY - 2006/3/31/entrez SP - 1746 EP - 53 JF - Gut JO - Gut VL - 55 IS - 12 N2 - BACKGROUND: Some patients with untreated coeliac disease are negative for serum endomysial autoantibodies (EmA) targeted against transglutaminase 2 (TG2). AIMS: To evaluate the clinical and histological features of EmA-negative coeliac disease, and to examine whether EmA-equivalent autoantibodies against TG2 can be seen in the small-bowel mucosa when absent in serum. PATIENTS: Serum EmA was studied in 177 biopsy-proved specimens from adult patients with coeliac disease. 20 patients with intestinal diseases served as non-coeliac controls; three had autoimmune enteropathy with villous atrophy. METHODS: Clinical manifestations, small-bowel mucosal morphology, intraepithelial inflammation and TG2-specific extracellular immunoglobulin A (IgA) deposits were investigated in both serum EmA-negative and EmA-positive patients. RESULTS: 22 patients with IgA-competent coeliac disease were negative for serum EmA. Three of these had small-bowel lymphoma. Patients with EmA-negative coeliac disease were older, had abdominal symptoms more often, and the density of gammadelta+ intraepithelial lymphocytes in their intestinal mucosa was lower than in EmA-positive patients; otherwise the histology was similar. All serum EmA-negative patients with coeliac disease, but none of the disease controls, had gluten-dependent mucosal IgA deposits alongside TG2 in the small-bowel mucosal specimens. In vivo deposited IgA was shown to be TG2-specific by its ability to bind recombinant TG2. CONCLUSIONS: Negative serum EmA might be associated with advanced coeliac disease. TG2-targeted autoantibodies were deposited in the small-bowel mucosa even when absent in serum. This finding can be used in the diagnosis of seronegative coeliac disease when the histology is equivocal. It may also be helpful in the differential diagnosis between autoimmune enteropathy and coeliac disease. SN - 0017-5749 UR - https://www.unboundmedicine.com/medline/citation/16571636/Endomysial_antibody_negative_coeliac_disease:_clinical_characteristics_and_intestinal_autoantibody_deposits_ L2 - http://gut.bmj.com/cgi/pmidlookup?view=long&pmid=16571636 DB - PRIME DP - Unbound Medicine ER -