Review
The imaging of coeliac disease and its complications

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Abstract

Coeliac disease is a malabsorption syndrome in which dietary gluten damages the small bowel mucosa. Gluten contains gliadin, the primary toxic component that is primarily found in wheat, barley and rye products. The initial diagnosis of coeliac disease is usually made by endoscopic biopsy of the jejunum although sometimes imaging features can suggest the diagnosis. Once a diagnosis is made, patients need to be diet compliant and monitored for potential complications. Many complications are more common when dietary compliance is poor. Complications include intussusception (usually intermittent), ulcerative jejunitis, osteomalacia, cavitating lymph node syndrome and an increased risk of malignancies such as lymphoma, adenocarcinoma and squamous cell carcinoma. Radiological evaluation is central in the evaluation of these complications. Imaging may assist both in the diagnosis and staging of complications as well as enabling radiological guided percutaneous biopsy for complications of coeliac disease such as lymphoma. As coeliac disease is a relatively common disorder, it is likely that most radiologists will encounter the disease and its potential complications. The aim of this review article is to discuss and illustrate the role of modern radiology in evaluating the many presentations of this complex disease.

Introduction

The prevalence of coeliac disease in Western civilisation is as high as 1/100–1/500 people [1], [2], [3]. There is a recognised genetic predisposition with HLA B8 identified in 80% of coeliac sufferers compared with 20% of the general population [4], [5]. Coeliac disease also has a slightly increased prevalence within Northern Europe [5]. It can sometimes present with non-specific symptoms and non-specific radiological findings and the differential diagnosis for the clinical and radiological presentation of suspected coeliac disease includes viral enteritis, Giardiasis, Crohn's disease, AIDS and Whipples disease. The gold standard diagnostic test for coeliac disease is jejunal biopsy demonstrating villous atrophy that improves after a period of gluten free diet. Antibody testing is now employed and the combination of positivity for anti-endomyseal antibody and anti-transgluaminase antibody is considered highly predictive for the condition [6]. Despite these useful diagnostic tests, there is often difficulty in diagnosing the condition and radiological findings can be helpful in suggesting coeliac disease as part of a differential diagnosis. Traditionally, barium studies (in particular small bowel barium studies) were the radiological investigation of choice and the features of coeliac disease have been well described with this modality. However, patients often present late and are initially evaluated with cross sectional imaging in a work up for weight loss or non-specific gastrointestinal symptoms. Imaging in these cases may not only suggest a diagnosis of coeliac disease but more importantly demonstrate and evaluate potential complications. In this review article, we review both the well-known radiological features of coeliac disease as well as discuss with examples the range of complications that may occur in this disorder.

Section snippets

Clinical presentation

Although previously considered a paediatric condition that is detected in early childhood when infants fail to thrive, two later peak incidences have now been observed. These occur in the forth decade and in the sixth and seventh decades [7]. In fact, 25% of patients are now diagnosed at a mean of 60 years of age [8]. In addition, the median age of diagnosis is presently increasing [9]. Presentation is varied. Sometimes only vague symptoms such as fatigue or malaise may be the only presenting

Pathophysiology

Gluten that is contained in wheat, barley and rye contains gliaden a protein that appears to trigger an autoimmune reaction in certain individuals. An immune mediated mechanism is postulated with evidence that CD4+ T lymphocyte cells may infiltrate the small bowel mucosa leading to crypt hyperplasia and villous atrophy [11]. The entire small bowel is sensitive to the offending protein in affected individuals but the proximal small bowel is usually worst affected [12]. The terminal ileum may be

Uncomplicated coeliac disease

Traditionally barium studies have been considered a good screening tool for the diagnosis of coeliac disease. Findings seen on barium studies include small bowel dilatation, particularly affecting the jejunum, which is felt to occur secondary to intestinal hypomotility (Fig. 2). Failure to absorb fluid results in excess fluid within the small bowel [13], [14], [15]. The fluid excess induces segmentation, fragmentation and flocculation of ingested barium (Fig. 3). Segmentation of barium is more

Intussusception

Transient non-obstructing intussusceptions are seen in 20% of patients with coeliac disease [21]. They may be asymptomatic or may be the initial presenting feature of coeliac disease. The intussusception is typically found in the proximal jejunum and does not involve a long segment of small bowel. There may be no identifiable lead point and the mechanism may be related to abnormalities in small bowel muscle tone secondary to gluten [22]. They have a characteristic ‘coiled spring’ appearance on

Cavitating lymph node syndrome

Cavitating lymph node syndrome is a rare but severe complication of coeliac disease. Patients present with weight loss, diarrhea and anorexia. The mortality from this complication is as high as 50% and is usually related to sepsis or haemorrhage [24]. It is commonly associated with hyposplenism and Howell–Jolly bodies may be detected on blood film analysis [25]. In the past the diagnosis of cavitatory lymph node syndrome was made at laparotomy or even post mortem when biopsy of the lymph nodes

Coeliac disease and malignancy

The development of malignancy is the most serious complication that can occur in patients with coeliac disease. When compared to the general population, patients with coeliac disease are at increased risk of lymphoma, adenocarcinoma and squamous cell carcinomas [27], [28]. Patients with coeliac disease who present with new onset symptoms of weight loss or altered bowel habits should be investigated for the potential presence of lymphoma. The increased relative risk of developing a cancer is

Conclusion

Coeliac disease is a relatively common condition but its clinical and radiological features are non-specific and making a diagnosis can be difficult. The condition is no longer considered purely a paediatric disease. Many patients are now presenting later in life with the complications of the gluten sensitive enteropathy such as malignancy. Frequently, cross sectional imaging is employed as a first line imaging modality in patients that present with non-specific weight loss and gastrointestinal

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