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What is
Celiac Sprue?

Celiac sprue should be distinguished from gluten sensitivity. Gluten sensitivity implies that a persons immune system is intolerant of gluten in the diet and is forming antibodies or displaying some other evidence of an inflammatory reaction. When these reactions cause intestinal damage visible on a biopsy, the syndrome has been called celiac sprue, celiac disease, or gluten sensitive enteropathy. (Nontropical sprue and idiopathic steatorrhea are other terms that have been used for this disorder in the past.) It should be noted that most of this content pertains to patients with enough damage of the intestine to be classified as having celiac sprue. Patients with gluten sensitivity without intestinal damage may not necessarily experience the signs or symptoms discussed in this section.

Thus, the clinical definition of celiac sprue is a small intestinal histopathologic (abnormal under the microscope) lesion, symptoms or signs due to malabsorption of fluid, electrolytes or nutrients, and clinical improvement following a gluten-free diet. New developments in diagnosis of celiac sprue include the anti-gliadin and anti-endomysial antibody blood tests and HLA genetic tests.

Populations thought to be at greatest risk for celiac sprue are mostly northern Europeans, and southern Europeans (England, Ireland, Spain, Italy). It is more frequent in women, although that may reflect more female patients seeing doctors and being diagnosed, and recent research is suggesting that the disease may not have any gender predilection. We have little information on the prevalence of celiac sprue in the Near East, Middle East, Latin America although my recent research suggests Mexican_Americans have a high prevalence; it has been thought not to occur in Africa or the Far East but this may not hold true for descendants from these lands that have immigrated to the U.S.

Celiac Sprue: Cause, Symptoms, Signs, and Treatment

The cause of celiac sprue is consumption of gluten contained in wheat, barley, rye and oats. The toxic subfraction of gluten is gliadin from wheat; from other grains they are avenins, secalins, and hordeins. Because wheat is the most common grain ingested in this country, we hear mostly about gliadin.

There is a genetic predisposition to the disease; there is increased prevalence in first degree relatives, approximately ten percent; and the disease is associated with certain HLA genes (primarily HLA DQ2, and less often DQ8). HLA genes are in charge of the body's immune system. There may be environmental factors; a theory that a virus may set off the system to react to gluten has not been confirmed yet. I have recently reported that Hepatitis C seems to trigger celiac sprue. Probably there are other unknown factors relating to acquired infections or bacteria in the intestine, etc. Researchers are still working on trying to determine the trigger factors but other inflammatory disorders including microscopic colitis may be a triggering cause.

Common symptoms include intestinal symptoms of weight loss, flatulence (gas), diarrhea, constipation, abdominal distention, bloating or vomiting. Other symptoms not associated with the gut include weakness, peripheral neuropathy (numbness or tingling in hands or feet), muscle spasms, bone pain, night blindness and cessation of menses. Some patients do not have any symptoms but Celiac sprue was detected by their doctors in a routine examination of blood work and have iron deficiency anemia and other nutritional deficiencies.

Signs that can be measured by doctors include steatorrhea (fat in the stool), anemia (and a variety of other nutrient deficiencies), osteopenia, peripheral neuropathy, hyposplenism (decreased function of the spleen); decreased calcium, magnesium, zinc, phosphorus, albumin, cholesterol and carotene in the blood stream.

Villi serve to increase the surface area of the small intestine. In a celiac patient, the genes tell your intestinal system to attack the gliadin protein, and in the process, the small intestine becomes damaged. This causes diarrhea due to interference of normal absorption of fluid, electrolytes, and nutrients. Under the microscope, villous atrophy of varying degrees is observed.

The only treatment for celiac sprue is a strict gluten free diet. The necessary degree of gluten restriction for patients to remain asymptomatic varies. Even though you may be less sensitive than your neighbor, that doesn't necessarily mean you're not doing any kind of immunologic damage to your intestine.

Initially you should probably avoid milk sugar (lactose) because your intestine needs to rebuild the ability to digest lactose. Once that happens, you can resume it, probably without symptoms, although remember that a lot of normal people cannot tolerate lactose. Folic acid or folate is a vitamin that is necessary for regeneration of cells, and almost all sprue patients will be deficient in this vitamin. Now there is evidence that folic acid is good for other things, like preventing heart attacks. If deficiencies in other nutrients such as iron, calcium, or magnesium were present before diagnosis, supplementation should be prescribed.

If a patient does not respond right away on a gluten-free diet, doctors may prescribe corticosteriods (like prednisone). This seems to happen more in older people, and probably has to do with the fact that sprue may have been present for a long time and the body needs assistance in decreasing inflammation. Regeneration of villi occurs quicker in children, but regrowth/healing should be accomplished by one year in most celiacs of all ages.

After resolution of symptoms on a gluten-free diet, patients may be tempted to reintroduce gluten containing foods to see the response. At first no symptoms may appear. However, in most if not all patients, symptoms will reappear after some latency period. If this is done, the symptoms may come back with greater severity and the process may then become refractory.

Refractory Sprue

Most patients and doctors do not need to worry about refractory sprue, as doctors do at specialty medical centers because most people do respond to the gluten-free diet. Refractory sprue is basically a disease identical to celiac sprue, but there is no response to a gluten-free diet, or a relapse following the initial response.

You might never respond if you actually inadvertently continue to ingest gluten. Unfortunately, this is so common as gluten occurs in processed foods and hidden in different forms. There have been cases where there was a sprue-like syndrome due to other things, like fish proteins or egg proteins, but those have been rare (or never discovered). There can be a co-existent intestinal lymphoma to explain the refractoriness of this complicated condition. Or alternatively, your diagnosis may not be sprue but it may be something that looks like celiac sprue but has a different cause and may requires different treatment, i.e., Crohn's disease, etc. If it's not due to gluten, of course, it's not going to respond to a gluten-free diet.

Sometimes a refractory sprue condition may be treated with certain immunosuppressant agents. Immunosuppressants are drugs that suppress the immune system's response to stimuli. The prognosis of refractory sprue is variable, but usually poor in its true form.

Associated Disorders

Some of the associated disorders that occur with celiac sprue in addition to microscopic colitis include microscopic colitis, pancreatic insufficiency, dermatitis herpetiformis (skin syndrome), autoimmune-related diabetes type 1, hypothyroidism, Sjogren's syndrome (causing dry mouth and eyes), asthma, rheumatoid arthritis, autoimmune hepatitis, primary biliary cirrhosis, hepatitis C infection, psoriasis, and other "autoimmune" syndromes.

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The Health and Nutritional information contained in this Website is based on scientific facts, medical research, and the personal and professional experiences of Dr. Kenneth D. Fine. It is provided as a free public service. It does not constitute medical diagnosis or treatment, and should not be construed or used as such.

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