Current guidelines for the management of celiac disease: A systematic review with comparative analysis

Abstract

Background: Wheat and other gluten-containing grains are widely consumed, providing approximately 50% of the caloric intake in both industrialised and developing countries. The widespread diffusion of gluten-containing diets has rapidly led to a sharp increase in celiac disease prevalence. This condition was thought to be very rare outside Europe and relatively ignored by health professionals and the global media. However, in recent years, the discovery of important diagnostic and pathogenic milestones has led to the emergence of celiac disease (CD) from obscurity to global prominence. These modifications have prompted experts worldwide to identify effective strategies for the diagnosis and follow-up of CD. Different scientific societies, mainly from Europe and America, have proposed guidelines based on CD's most recent evidence.

Aim: To identify the most recent scientific guidelines on CD, aiming to find and critically analyse the main differences.

Methods: We performed a database search on PubMed selecting papers published between January 2010 and January 2021 in the English language. PubMed was lastly accessed on 1 March 2021.

Results: We distinguished guidelines from 7 different scientific societies whose reputation is worldwide recognized and representative of the clinical practice in different geographical regions. Differences were noted in the possibility of a no-biopsy diagnosis, HLA testing, follow-up protocols, and procedures.

Conclusion: We found a relatively high concordance between the guidelines for CD. Important modifications have occurred in the last years, especially about the possibility of a no-biopsy diagnosis in children. Other modifications are expected in the next future and will probably involve the extension of the non-invasive diagnosis to the adult population and the follow-up modalities.

Keywords: Celiac disease; Clinical guidelines; Genetics; Gluten; Gluten sensitivity; Gluten-free diet; Histopathological findings; Non-invasive diagnosis; Serological markers.

Figure 1

PRISMA flow diagram.

Figure 2

Recommendations about case finding.

Figure 3

Worldwide adapted decision-making process for diagnosing celiac disease. Highly suspicious celiac disease (CD) comprises “classical presentation” (i.e., classical symptoms in children include failure to thrive, weight loss, growth failure, vomiting, chronic diarrhea, bloating, Iron-deficiency anemia, muscle wasting, oedema due to hypoproteinemia, irritability and unhappiness; in adults, classical symptoms include chronic diarrhea, weight loss, iron-deficiency anemia, malaise and fatigue, oedema due to hypoproteinemia, and osteoporosis), frequent “non-classical presentation” (i.e., iron deficiency and hypertransaminasemia) and “non-classical presentation” but high risk group (i.e., CD first-degree relatives, autoimmune conditions such as type 1 Diabetes Mellitus, and thyroid disease, genetic conditions such as IgA deficiency, Down syndrome, Turner syndrome and Williams-Beuren syndrome).

Figure 4

Recommendations about serology. IgA: Immunoglobulin A; IgG: Immunoglobulin G; DGP: Deamidated gliadin peptides; EMA: Anti-endomysium antibodies.

Figure 5

Recommendations about serology.

Figure 6

Recommendations about Human Leukocyte Antigen testing.

Figure 7

Recommendations about the possibility of a no-biopsy diagnosis. TGA: Anti-transglutaminase antibodies; IgA: Immunoglobulin A; EMA: Anti-endomysium antibodies; HLA: Human leukocytes antigen; CD: Celiac disease.

Figure 8

Recommendations about potential, silent, and seronegative celiac disease. GFD: Gluten-free diet; HLA: Human leukocytes antigen.

Figure 9

Recommendations about refractory and complicated celiac disease. GFD: Gluten-free diet; TCR: T-cell receptor.

Figure 10

Recommendations about follow-up of celiac disease. TGA: Anti-transglutaminase antibodies; GFD: Gluten-free diet.

Figure 11

Recommendations about the gluten-free diet for celiac disease.