Refractory celiac disease is continued damage to the small intestine after adhering to a gluten free diet for an extended period (about a year). To fully explain refractory celiac disease, we need to first examine regular celiac disease to clearly distinguish between the two.
Celiac disease is an inflammatory disease of the small intestine brought about by the ingestion of gluten. If you’re extremely sensitive, it may also be triggered by inhalation (when you breathe through your mouth and nose, particles of flour can be trapped in your saliva and mucus and then be swallowed. Then, of course, it enters your digestive system).
Diagnosis is determined with genetic testing, blood testing for specific antibodies, and an intestinal biopsy.
When you remove gluten from your diet, inflammation in your small intestine typically begins to ease within a few weeks or months, though complete healing can take years.
About 10% of people who are diagnosed don’t get better, or get better for a short time but then all their symptoms come back. While a variety of things can cause this, the most frequent cause is that the individual continues to eat gluten (whether on purpose or because of cross-contamination).
GLUTEN FREE DIET
The only treatment for celiac disease is to eat gluten free for the rest of your life. Unfortunately many people don’t understand the seriousness of their condition, and aren’t very earnest in their efforts to avoid gluten.
I know several people with celiac disease who don’t pay attention to trace amounts of gluten (they basically just avoid bread items), regularly eat out at restaurants that aren’t safe, and regularly “cheat” because eating gluten free is hard and they miss regular food.
This is dangerous because it continues damaging your intestine, causes a laundry list of other health issues, and can eventually contribute to a dire health situation that leads to death.
Unintentional ingestion of gluten is the primary reason people fail to heal on a gluten free diet. Some people try to be very diligent but are lacking information and education on the subject. And gluten can hide in sources you wouldn’t anticipate.
If you’re not healing, a good first course of action is to consult with a dietitian (though many aren’t knowledgeable on refractory celiac disease) or get guidance from someone very experienced with celiac disease.
OTHER POTENTIAL CAUSES
Another common factor for a return of symptoms is lactose or fructose intolerance as many people increase the amount of dairy or fruit they eat when they cut wheat out of their diet. Other things to consider include:
- Autoimmune enteropathy (when your body makes antibodies against your small intestinal lining cells—very rare)
- Bacterial overgrowth of the small intestine
- Collagenous sprue (collagen deposits beneath the intestinal lining)
- Drug induced damage to the small intestine
- Exocrine pancreatic insufficiency (reduced pancreatic digestive enzymes which makes it difficult to digest properly)
- Food allergy
- Hypogammaglobulinemic sprue (say that 3 times fast!)
- Irritable Bowel Syndrome
- Microscopic colitis (inflammation under the intestinal lining)
- Tropical Sprue
In case you’re wondering, sprue is defined as a disorder in which nutrients are poorly absorbed by the body from the intestine.
If you’re having ongoing issues, go back to your doctor to try and resolve the issue. Or perhaps find a different doctor for a re-look at your diagnosis. Make sure you had proper blood testing and a biopsy.
REFRACTORY CELIAC DISEASE / REFRACTORY SPRUE
When all other explanations for failure to recover have been ruled out, refractory celiac disease should be considered. Some people never respond to a gluten free diet while others get better at first but then their symptoms and intestinal damage come back.
Only about 1.5% of celiacs will develop refractory celiac disease, and these individuals are usually over 50 years old.
Symptoms are similar to untreated celiac disease but they’re generally more severe, often disabling. Common symptoms include abdominal pain, anemia, diarrhea, fatigue, malnutrition, and weight loss.
When you have celiac disease, T-cells (a type of white blood cell) that recognize gluten proteins are activated and multiply. When you go gluten free, these T-cells become dormant and intestinal damage heals.
With refractory celiac disease, these T-cells are activated without gluten and intestinal trauma continues despite a gluten free diet. Refractory celiac disease is split into two types.
Type I is characterized by normal T-cell population along the intestinal lining and is the most common form–approximately 85% of people with refractory celiac disease have Type I. It typically responds to aggressive nutritional support, a strict gluten free diet, and steroid treatment.
Type II is characterized by abnormal T-cell population along the intestinal lining and more severe symptoms. Individuals with this often need intravenous nutrition, don’t respond well to steroids, and can experience complications such as ulcerative jejunitis (ulceration of the middle portion of the small intestine) and T-cell lymphoma (a type of cancer).
Type II is very serious and has a poor prognosis, but for either type you should be referred to and treated by an expert. Please be mindful that non-responsive celiac disease is rarely due to refractory celiac disease. Other causes are much more probable.