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Seronegative celiac disease: where is the specific setting?

Ierardi E, Losurdo G, Piscitelli D, Giorgio F, Sorrentino C, Principi M, Montenegro L, Amoruso A, Di Leo A - Gastroenterol Hepatol Bed Bench (2015)

Bottom Line: Another reason for seronegativity may be found in an incomplete maturation of plasma cells with a consequent failure of antibodies production.The choice of GFD administration could be linked to an investigation able to diagnose SNCD in no doubt even if a reliable test is not currently available.On these bases, a test helping the diagnosis of SNCD is justifiable and desirable.

View Article: PubMed Central - PubMed

Affiliation: Section of Gastroenterology, Department of Emergency and Organ Transplantation, University of Bari, Italy.

ABSTRACT
The diagnosis of Celiac Disease (CD) relies on the concordance of pathological, serological, genetic and clinical features. For this reason, the diagnosis of CD is often a challenge. Seronegative celiac disease (SNCD) is defined by the negativity of anti-tissue transglutaminase antibodies in the presence of a positive histology on duodenal biopsy samples, i.e. inflammatory infiltrate of intra-epithelial lymphocytes (IELs > 25/100 enterocytes), mild villous atrophy and uneven brush border associated to human leukocyte antigen (HLA) haplotype DQ2 and/or DQ8. SNCD is characterized by mucosal deposits of tissue transglutaminase (tTG)/anti-tTG immuno-complexes. These may counteract the passage of anti-tTG into the bloodstream, thus explaining seronegativity. Another reason for seronegativity may be found in an incomplete maturation of plasma cells with a consequent failure of antibodies production. This condition often characterizes immunoglobulin deficiencies, and, indeed, SNCD is common in subjects with immunoglobulin deficiencies. The management of SNCD still remains debated. The treatment option for SNCD may be represented by gluten free diet (GFD), but the usefulness and appropriateness of prescribing GFD are controversial. Some evidences support its use only in SNCD subjects showing CD clear clinical picture and compatible HLA status. The choice of GFD administration could be linked to an investigation able to diagnose SNCD in no doubt even if a reliable test is not currently available. On these bases, a test helping the diagnosis of SNCD is justifiable and desirable.

No MeSH data available.


Related in: MedlinePlus

A multi-step classification of gluten related disorders according to the entity of adaptive immune system involvement. T1D: type-1 diabetes. Adapted and modified from Troncone et al. ref 32.
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Figure 2: A multi-step classification of gluten related disorders according to the entity of adaptive immune system involvement. T1D: type-1 diabetes. Adapted and modified from Troncone et al. ref 32.

Mentions: The spectrum of gluten-related disorders is wide, and encloses several conditions. For its singular pathogenesis, SNCD may be considered as an “immature” CD, where the global expression of autoantibodies is lacking. In a multi-step classification of gluten-related conditions (32), it has been proposed to place CD one step under the evident CD, due to a weaker involvement of adaptive immunity and a minor activation of tTG, as displayed in figure 2.


Seronegative celiac disease: where is the specific setting?

Ierardi E, Losurdo G, Piscitelli D, Giorgio F, Sorrentino C, Principi M, Montenegro L, Amoruso A, Di Leo A - Gastroenterol Hepatol Bed Bench (2015)

A multi-step classification of gluten related disorders according to the entity of adaptive immune system involvement. T1D: type-1 diabetes. Adapted and modified from Troncone et al. ref 32.
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4403023&req=5

Figure 2: A multi-step classification of gluten related disorders according to the entity of adaptive immune system involvement. T1D: type-1 diabetes. Adapted and modified from Troncone et al. ref 32.
Mentions: The spectrum of gluten-related disorders is wide, and encloses several conditions. For its singular pathogenesis, SNCD may be considered as an “immature” CD, where the global expression of autoantibodies is lacking. In a multi-step classification of gluten-related conditions (32), it has been proposed to place CD one step under the evident CD, due to a weaker involvement of adaptive immunity and a minor activation of tTG, as displayed in figure 2.

Bottom Line: Another reason for seronegativity may be found in an incomplete maturation of plasma cells with a consequent failure of antibodies production.The choice of GFD administration could be linked to an investigation able to diagnose SNCD in no doubt even if a reliable test is not currently available.On these bases, a test helping the diagnosis of SNCD is justifiable and desirable.

View Article: PubMed Central - PubMed

Affiliation: Section of Gastroenterology, Department of Emergency and Organ Transplantation, University of Bari, Italy.

ABSTRACT
The diagnosis of Celiac Disease (CD) relies on the concordance of pathological, serological, genetic and clinical features. For this reason, the diagnosis of CD is often a challenge. Seronegative celiac disease (SNCD) is defined by the negativity of anti-tissue transglutaminase antibodies in the presence of a positive histology on duodenal biopsy samples, i.e. inflammatory infiltrate of intra-epithelial lymphocytes (IELs > 25/100 enterocytes), mild villous atrophy and uneven brush border associated to human leukocyte antigen (HLA) haplotype DQ2 and/or DQ8. SNCD is characterized by mucosal deposits of tissue transglutaminase (tTG)/anti-tTG immuno-complexes. These may counteract the passage of anti-tTG into the bloodstream, thus explaining seronegativity. Another reason for seronegativity may be found in an incomplete maturation of plasma cells with a consequent failure of antibodies production. This condition often characterizes immunoglobulin deficiencies, and, indeed, SNCD is common in subjects with immunoglobulin deficiencies. The management of SNCD still remains debated. The treatment option for SNCD may be represented by gluten free diet (GFD), but the usefulness and appropriateness of prescribing GFD are controversial. Some evidences support its use only in SNCD subjects showing CD clear clinical picture and compatible HLA status. The choice of GFD administration could be linked to an investigation able to diagnose SNCD in no doubt even if a reliable test is not currently available. On these bases, a test helping the diagnosis of SNCD is justifiable and desirable.

No MeSH data available.


Related in: MedlinePlus