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Celiac disease – sprue

Sprue; Nontropical sprue; Gluten intolerance; Gluten-sensitive enteropathy; Celiac sprue

Celiac disease is an autoimmune condition that damages the lining of the small intestine. This damage comes from a reaction to eating gluten. This is a substance that is found in wheat, rye, barley, and possibly oats. It is also found in food made from these ingredients.

The damaged intestine is not able to fully absorb nutrients from food.

Causes

The exact cause of celiac disease is not known. The lining of the intestines have small areas called villi which project outward into the opening of the intestine. These structures help absorb nutrients.

When people with celiac disease eat foods with gluten, their immune system reacts by damaging the villi. Because of the damage, the villi are unable to properly absorb iron, vitamins, and other nutrients. This may cause a number of symptoms and other health problems.

The disease can develop at any point in life, from infancy to late adulthood.

People who have a family member with celiac disease are at greater risk of developing the disease. The disorder is most common in Europeans.

People with celiac disease are more likely to have:

Symptoms

The symptoms of celiac disease can be different from person to person. This can make diagnosis difficult. For example, one person may have constipation, a second may have diarrhea, and a third may have no problem with stools.

Gastrointestinal symptoms include:

Other problems that can develop over time because the intestines do not absorb key nutrients include:

  • Easy bruising
  • Depression or anxiety
  • Fatigue
  • Growth delay in children
  • Hair loss
  • Itchy skin with a rash (dermatitis herpetiformis)
  • Missed menstrual periods
  • Mouth ulcers
  • Muscle cramps and joint pain
  • Nosebleeds
  • Seizures
  • Tingling or numbness in the hands or feet
  • Unexplained short height

Children with celiac disease may have:

  • Defects in the tooth enamel and changes in tooth color
  • Delayed puberty
  • Muscle wasting
  • Diarrhea, constipation, fatty or foul-smelling stools, nausea, or vomiting
  • Irritable and fussy behavior
  • Poor weight gain
  • Slowed growth and shorter than normal height for their age

Exams and Tests

The following tests may be performed:

  • Bone density
  • Cholesterol (may be low)
  • Complete blood count (CBC - test for anemia)
  • Comprehensive metabolic panel
  • Folate level (serum)
  • Iron level (serum)
  • Ferritin level (serum)
  • Prothrombin time
  • Vitamin B12 level (serum)
  • Vitamin D level (serum)

Blood tests can detect antibodies, called antitissue transglutaminase antibodies (tTGA) or anti-endomysial antibodies (EMA) which may help detect the condition. The health care provider will order these antibody tests if celiac disease is suspected.

If the tests are positive, upper endoscopy is often performed to sample a piece of tissue (biopsy) from the first part of the small intestine (duodenum). The biopsy may show a flattening or loss of the villi in the parts of the intestine affected in the duodenum.

Genetic testing of the blood can also be done to help detect who may be at risk for celiac disease. Those who test negative for the common genetic variant are unlikely to have celiac disease.

A follow-up biopsy or blood test may be ordered several months after the diagnosis and treatment. These tests assess how well treatment is working. Normal results mean that you have responded to treatment. This confirms the diagnosis. However, this does not mean that the disease has been cured.

Treatment

Celiac disease cannot be cured. Your symptoms will go away and the villi in the intestines will heal if you follow a lifelong gluten-free diet. Do not eat foods, drink beverages, or take medicines that contain wheat, barley, rye, and possibly oats.

You must read food and drug labels carefully to look for ingredients that may include these grains. It may be hard to stick to a gluten-free diet because wheat and barley grains are common in the American diet. Over time, most people are able to adapt and get better. You should NOT begin the gluten-free diet before you are diagnosed. Starting the diet will affect testing for the disease.

In most cases, following a well-balanced, gluten-free diet is the only treatment you need to stay well. Your provider may need to prescribe vitamin and mineral supplements.

Sometimes, short-term use of corticosteroids (such as prednisone) may be needed if celiac disease does not respond to treatment.

When you are diagnosed, get help from a registered dietitian who specializes in celiac disease and the gluten-free diet. A support group may also help you cope with the disease and diet.

Support Groups

You can ease the stress of illness by joining a support group. More information and support for people with celiac disease and their families can be found at the National Celiac Association.

Outlook (Prognosis)

Following a gluten-free diet heals the damage to the intestines and prevents further damage. This healing most often occurs within 3 to 6 months in children. Recovery may take 2 to 3 years in adults.

Rarely, long-term damage will be done to the lining of the intestines before the diagnosis is made.

Some problems caused by celiac disease may not improve, such as a short height and damage to the teeth.

Possible Complications

You must carefully continue to follow the gluten-free diet. When untreated, the disease can cause fatal complications.

Delaying diagnosis or not following the diet puts you at risk for related conditions such as:

When to Contact a Medical Professional

Contact your provider if you have symptoms of celiac disease.

Prevention

Because the exact cause is not known, there is no way to prevent the development of celiac disease. However, you should be aware of the risk factors such as family history. This may increase your chances of early diagnosis and treatment.

Open References

References

Lebwohl B, Green PHR. Celiac disease. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger and Fordtran's Gastrointestinal and Liver Disease. 11th ed. Philadelphia, PA: Elsevier; 2021:chap 107.

Lin JC, Benz EJ Jr. Approach to anemia in the adult and child. In: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Basic Principles and Practice. 8th ed. Philadelphia, PA: Elsevier; 2023:chap 35.

Rubio-Tapia A, Hill ID, Semrad C, et al. American College of Gastroenterology guidelines update: diagnosis and management of celiac disease. Am J Gastroenterol. 2023;118(1):59-76. PMID: 36602836 pubmed.ncbi.nlm.nih.gov/36602836/.

Schiller LR. Malabsorption. In: Kellerman RD, Rakel DP, Heidelbaugh JJ, Lee EM, eds. Conn's Current Therapy 2024. Philadelphia, PA: Elsevier; 2024:273-279.

Semrad CE. Approach to the patient with diarrhea and malabsorption. In: Goldman L, Cooney KA, eds. Goldman-Cecil Medicine. 27th ed. Philadelphia, PA: Elsevier; 2024:chap 126.

  • Celiac disease

    Celiac disease - illustration

    The intestinal lining has small hair-like projections called villi, which help absorb nutrients. When people with celiac disease eat foods with gluten, it triggers an immune response. Over time, the inflammation caused by the immune response damages the villi. Because of the damage, the villi are unable to properly absorb iron, vitamins, and other nutrients. This may cause a number of symptoms, including diarrhea, fatigue, and weight loss, as well as a number of other health problems.

    Celiac disease

    illustration

  • Digestive system

    Digestive system - illustration

    The esophagus, stomach, large and small intestine, aided by the liver, gallbladder and pancreas convert the nutritive components of food into energy and break down the non-nutritive components into waste to be excreted.

    Digestive system

    illustration

  • Celiac sprue - foods to avoid

    Celiac sprue - foods to avoid - illustration

    Celiac disease causes inflammation in the small intestine and damage in the lining. This prevents the body from properly absorbing the nutrients in food. The damage to the lining of the intestine comes from a reaction to eating gluten, which is found in wheat, rye, barley, and possibly oats, and in food made from these ingredients. The inability to absorb nutrients can lead to weight loss, fatigue, malnourishment, and other health problems. Gluten may be found in many foods, especially processed foods and baked goods. Breads, cakes, desserts, alcoholic beverages (except wine), cereals, and pastas may all contain gluten. Eating a gluten-free diet heals the intestines and prevents further damage.

    Celiac sprue - foods to avoid

    illustration

  • Dermatitis - herpetiformis on the knee

    Dermatitis - herpetiformis on the knee - illustration

    This picture shows the knee of a person with a chronic inflammatory disease known as dermatitis herpetiformis. It produces red, raised (papular), small or large blisters (vesicles or bullae) that burn and itch intensely. Dermatitis herpetiformis develops suddenly, lasts for weeks to months, and may be associated with digestive diseases (such as Celiac disease).

    Dermatitis - herpetiformis on the knee

    illustration

  • Dermatitis - herpetiformis on the arm and legs

    Dermatitis - herpetiformis on the arm and legs - illustration

    This picture shows a chronic inflammatory disease (dermatitis herpetiformis) that produces red (erythematous), raised (papular), small or large blisters (vesicles or bullae) that burn and itch intensely. Dermatitis herpetiformis develops suddenly, lasts for weeks to months, and may be associated with digestive diseases (such as Celiac disease).

    Dermatitis - herpetiformis on the arm and legs

    illustration

  • Digestive system organs

    Digestive system organs - illustration

    The digestive system organs in the abdominal cavity include the liver, gallbladder, stomach, small intestine and large intestine.

    Digestive system organs

    illustration

    • Celiac disease

      Celiac disease - illustration

      The intestinal lining has small hair-like projections called villi, which help absorb nutrients. When people with celiac disease eat foods with gluten, it triggers an immune response. Over time, the inflammation caused by the immune response damages the villi. Because of the damage, the villi are unable to properly absorb iron, vitamins, and other nutrients. This may cause a number of symptoms, including diarrhea, fatigue, and weight loss, as well as a number of other health problems.

      Celiac disease

      illustration

    • Digestive system

      Digestive system - illustration

      The esophagus, stomach, large and small intestine, aided by the liver, gallbladder and pancreas convert the nutritive components of food into energy and break down the non-nutritive components into waste to be excreted.

      Digestive system

      illustration

    • Celiac sprue - foods to avoid

      Celiac sprue - foods to avoid - illustration

      Celiac disease causes inflammation in the small intestine and damage in the lining. This prevents the body from properly absorbing the nutrients in food. The damage to the lining of the intestine comes from a reaction to eating gluten, which is found in wheat, rye, barley, and possibly oats, and in food made from these ingredients. The inability to absorb nutrients can lead to weight loss, fatigue, malnourishment, and other health problems. Gluten may be found in many foods, especially processed foods and baked goods. Breads, cakes, desserts, alcoholic beverages (except wine), cereals, and pastas may all contain gluten. Eating a gluten-free diet heals the intestines and prevents further damage.

      Celiac sprue - foods to avoid

      illustration

    • Dermatitis - herpetiformis on the knee

      Dermatitis - herpetiformis on the knee - illustration

      This picture shows the knee of a person with a chronic inflammatory disease known as dermatitis herpetiformis. It produces red, raised (papular), small or large blisters (vesicles or bullae) that burn and itch intensely. Dermatitis herpetiformis develops suddenly, lasts for weeks to months, and may be associated with digestive diseases (such as Celiac disease).

      Dermatitis - herpetiformis on the knee

      illustration

    • Dermatitis - herpetiformis on the arm and legs

      Dermatitis - herpetiformis on the arm and legs - illustration

      This picture shows a chronic inflammatory disease (dermatitis herpetiformis) that produces red (erythematous), raised (papular), small or large blisters (vesicles or bullae) that burn and itch intensely. Dermatitis herpetiformis develops suddenly, lasts for weeks to months, and may be associated with digestive diseases (such as Celiac disease).

      Dermatitis - herpetiformis on the arm and legs

      illustration

    • Digestive system organs

      Digestive system organs - illustration

      The digestive system organs in the abdominal cavity include the liver, gallbladder, stomach, small intestine and large intestine.

      Digestive system organs

      illustration

    Tests for Celiac disease – sprue

     
     

    Review Date: 3/31/2024

    Reviewed By: Jenifer K. Lehrer, MD, Department of Gastroenterology, Aria - Jefferson Health Torresdale, Jefferson Digestive Diseases Network, Philadelphia, PA. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

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