eMedicine Specialties > Radiology > Gastrointestinal

Sprue

Author: J Kevin Smith, MD, PhD, Professor of Abdominal Imaging, Vice Chair for Veterans Affairs, Department of Radiology, University of Alabama at Birmingham; Chief of Service, Department of Radiology, Birmingham Veterans Affairs Medical Center
Coauthor(s): Carolyn R Bray Hebson, MD, Staff Physician, Department of Ophthalmology, Emory University School of Medicine
Contributor Information and Disclosures

Updated: Jun 2, 2009

Introduction

Background

Celiac disease is an autoimmune disorder of the small intestine caused by gluten intolerance in genetically predisposed individuals. It is a chronic disease characterized by mucosal lesions of the small bowel that impair nutrient absorption.¹

Studies show that a genetic predisposition in certain individuals can cause an immune response, which damages the small intestine mucosa. Celiac disease is strongly associated with HLA class II D-region genes in the major histocompatability complex on chromosome 6. Approximately 90-95% of patients have either DR3-DQ2 or DR5/7DQ2 haplotypes.^3,4

Radiographic findings of celiac sprue are present in virtually all patients in the clinically active phase of the disease. However, these symptoms may also present in patients with a wide variety of other malabsorption disorders. Common findings in celiac sprue include dilatation of the small intestine lumen, intussusception, hypersecretion, segmentation, a mosaic pattern, and jejunoileal fold-pattern reversal.⁵

The definitive diagnosis of celiac sprue is based on a small-bowel biopsy and clinical improvement after the introduction of a gluten-free diet.² Nevertheless, an accurate radiologic examination is important for recognition of small-bowel abnormalities and documentation of normal morphology before biopsy. Radiologic examination can also exclude complicating lesions, such as intussusception, jejunal ulceration with stricture, lymphoma, or GI carcinoma.⁶

For excellent patient education resources, visit eMedicine's Esophagus, Stomach, and Intestine Center and Cancer and Tumors Center. Also, see eMedicine's patient education articles Celiac Sprue, Cancer of the Small Intestine, and Lymphoma.

Pathophysiology

The mucosal abnormalities associated with celiac sprue are more pronounced in the jejunum than in the ileum. There is flattening, broadening, and coalescence, sometimes with complete loss of the small-bowel villi. The epithelial cells of the villi are also flattened and cuboidal, and the nuclei of these cells, instead of maintaining a regular basal orientation, appear at irregular levels. The crypts of Lieberkuhn become elongated, so the overall thickness of the mucosa may not be much different from normal. The lamina propria is infiltrated with lymphocytes, plasma cells, and eosinophils (see Images 1-2).⁵

Frequency

United States

Adult celiac disease occurs largely in whites. A definitive frequency of celiac sprue in the United States is unknown, and estimates vary from a prevalence of 1:4700-6000. Additional sources predict a US frequency of celiac disease similar to that of Europeans, approximately 1:300. This inconsistency might be due to underdiagnosis of the disease due to asymptomatic patients and/or an unfamiliarity with celiac sprue in US laboratories.

International

European prevalence ratios range from a high of 1:300 in western Ireland to 1:1000-2000 in other regions. The true prevalence is predicted to be higher because a substantial number of individuals report mild symptoms or none at all.² Celiac disease is rare in people of African or Asian descent.⁶ See also Celiac Sprue.

Mortality/Morbidity

The primary morbidity associated with celiac disease is malabsorption, which results from intestinal mucosa damage in the affected portion of small bowel. See Complications in Clinical details below.

In a Mayo Clinic study by Rubio-Tapia et al, undiagnosed celiac disease was found to be associated with an approximately fourfold increased risk of death over 45 years of follow-up. In addition, the study findings suggested a dramatic increase (4- to 4.5-fold) in undiagnosed celiac disease in the United States over the past 50 years.⁷

Race

Celiac disease is thought to be more prevalent in white Europeans than Americans and distinctly uncommon in Asians, Africans, and African Americans.

Sex

Some studies indicate that celiac disease is more common in females than in males. Other reports show that celiac disease affects males and females equally. An overall affected female-to-male ratio of 2.4:1 has led researchers to believe that undiagnosed adult celiac sprue patients were predominantly male, which is supported by the 1:1 female-to-male ratio of children in the Coeliac Society of the United Kingdom.

Age

In children, the onset of celiac disease is usually within the first through third years of life, with a classic presentation of chronic diarrhea, failure to thrive, and abdominal distention. Clinical symptoms often diminish or disappear during adolescence. Adult patients may present at any age, although there are noted peaks among women in their 30s and 40s and men in their 40s and 50s. The clinical presentation can be variable and appears to depend largely on the length of the affected small intestine.³

Anatomy

Celiac disease is a malabsorptive disorder of the small bowel. Studies indicate the severity of the disease—and thus its radiographic manifestations—proceeds in a proximal-to-distal fashion, with more pronounced lesions in the jejunum than in the ileum. Jejunal atrophy may be compensated for by ileal hypertrophy, resulting in a distinct pattern of jejunoileal fold pattern reversal characteristic of celiac sprue.⁸

Presentation

Symptoms

The symptoms are wide-ranging, show significant variation among patients, and depend largely on the length and severity of damage to the small bowel. The classic clinical presentation is malabsorption and includes diarrhea, steatorrhea, flatulence, weight loss, and fatigue.

Laboratory findings

Associated laboratory abnormalities may include iron or folate deficiency anemia, hypocholesterolemia, hypocalcemia, hypoalbuminemia, elevated alkaline phosphatase and liver enzymes, and a prolonged prothrombin time.²

Risk factors

Risk factors for celiac disease include dermatitis herpetiformis, type I diabetes mellitus, autoimmune thyroid disease, selective IgA deficiency, connective-tissue diseases, Down syndrome, collagenous or lymphocytic colitis, neurological and neuromuscular disorders, and a family history of celiac disease.

The primary morbidity associated with celiac disease is malabsorption, which results from intestinal mucosa damage in the affected portion of small bowel. Malabsorption can cause symptoms such as diarrhea, steatorrhea, weight loss, and fatigue. It also leads to a nutrient imbalance, which can cause macrocytic and microcytic anemia, hypocalcemia, hypomagnesemia, and hypoalbuminemia.

Malabsorption of fat-solute vitamins A, D, E, and K can result in rickets and osteomalacia, reduced visual acuity, and hypoprothrombinemia. Neurologic symptoms such as seizures, paresthesia, and ataxia, as well as hormonal disorders such amenorrhea, delayed menarche, and infertility are also associated with malabsorption.^9,10 See also Celiac Sprue.

Almost 100 diseases have been described as occurring concurrently with celiac disease. Endocrine disorders have been found in 11.9% of celiac patients, and insulin-dependent diabetes mellitus has occurred in 5.4%. Connective tissue disorders, autoimmune thyroid disease, and Sjögren syndrome have been found in 7.2%, 5.4%, and 3.3% of celiac patients, respectively.¹¹

Celiac disease alone is a common cause of splenic atrophy, and 30-50% of patients are affected. The correlation between celiac disease and hyposplenism is unknown. Arterial hypotension is the most relevant and common hemodynamic abnormality observed in patients with untreated celiac disease, with roughly 70% affected.¹²

Celiac disease is considered a premalignant condition for the development of lymphoma or GI carcinoma, with patients at a 40- to 100–fold increased risk.^11,13 Patients with celiac disease and malignancy have a poor prognosis, with survival averaging only 9 months, as compared with an almost 50% 5-year survival for those with primary intestinal lymphoma.¹³ The development of lymphoma should be suspected whenever unexplained clinical deterioration occurs in a celiac patient who was previously stable on a gluten-free diet or whenever symptoms develop in a patient who has villous atrophy with or without celiac disease.³

Ulcerative jejunoileitis and cavitary mesenteric lymph node syndrome are rare but usually fatal complications of celiac disease. Ulcerative jejunoileitis is characterized by fever, acute abdominal pain, distention, and weight loss, symptoms similar to those of celiac disease complicated by a malignant neoplasm. Complications of ulcerative jejunoileitis include hemorrhage, perforation, and obstruction. Radiologically, ulcerative jejunoileitis can be seen as an irregular, diffuse thickening of the bowel wall. Cavitary mesenteric lymph node syndrome is characterized by mesenteric lymph node cavitation, splenic atrophy, and villous atrophy of the small intestine mucosa, with clinical features of diarrhea, malabsorption, abdominal mass, and small-bowel obstruction.^1,14

Preferred Examination

Small-bowel series and enteroclysis are the preferred radiographic examinations for the diagnosis of celiac sprue. CT shows many of the findings well and is often performed when patients have nonspecific findings and when malignancy must be excluded.

Limitations of Techniques

When multiple findings of sprue are present on small-bowel follow-though or enteroclysis, the diagnosis is fairly specific. However, up to 25% of patients may not have enough findings for a confident diagnosis, and a small percentage of patients may have multiple findings that are radiographically suggestive of celiac sprue with negative biopsy results. Duodenal biopsy is performed to confirm the diagnosis in many cases and is considered the criterion standard for diagnosing celiac disease.

Serologic testing of celiac patients is a less common means of diagnosis, whereby patients are screened for the presence of immunoglobulin G and immunoglobulin A (IgA) antigliadin antibodies (AGAs) and/or IgA antiendomysial antibodies (EMAs). The sensitivity of AGA tests varies because AGAs are also found in autoimmune disorders other than celiac disease. In addition, AGAs become undetectable after the institution of a gluten-free diet.

IgA EMA screening is considered the most reliable serologic test for the detection of celiac disease, with a specificity of nearly 100%. Nevertheless, EMA testing has its drawbacks, with poor sensitivity in children younger than 2 years and with false-negative results in 2-3% of patients who have selective IgA deficiency.

When all 3 antibodies are screened simultaneously, the positive or negative predictive value is 99%. Therefore, these serologic tests may obviate biopsy, especially if the condition responds to a gluten-free diet.³

Differential Diagnoses

• Search for CME/CE on This Topic »

• Celiac Sprue (Gastroenterology)
• Sprue, Tropical (Gastroenterology)
• Celiac Disease (Pediatrics: General Medicine)
• Vitamin A Deficiency (Endocrinology)

• Esophagus, Stomach, and Intestine Center
• Celiac Sprue Overview
• Celiac Sprue Causes
• Celiac Sprue Signs and Symptoms
• Celiac Sprue Treatment
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• Cancer of the Small Intestine Introduction
• Lymphoma Overview

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