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Understanding Refractory Celiac Disease

Medically reviewed on Sept 20, 2023 by Jordan Stachel, M.S., RDN, CPT. To give you technically accurate, evidence-based information, content published on the Everlywell blog is reviewed by credentialed professionals with expertise in medical and bioscience fields.

Table of contents

Celiac disease is a lifelong, autoimmune disorder that diminishes a person’s ability to eat gluten safely. Healthcare providers will typically prescribe gluten-free celiac diet plans to people with celiac disease, but for a small group of people, limiting or eliminating gluten ingestion does not help their digestive system recover. [1] This may point to a rare type of celiac disease called refractory celiac disease (RCD). [2]

Cases of RCD are extremely scarce: only 1.5% of people diagnosed with celiac disease have it. [3] However, if refractory celiac disease develops, it can be hard to live with and treat. Educating yourself about the risks and types of celiac disease can be a life-saving part of your treatment plan if you’re diagnosed.

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What Causes Refractory Celiac Disease?

All types of celiac disease have the potential to damage the small intestine.4 When people with celiac disease ingest gluten, their immune systems respond by trying to dispel gluten from the small intestine. In the long term, this can result in villous atrophy, which occurs when the villi of the intestines (small, finger-like structures) degrade. [3]

People with refractory celiac disease have sustained significant villous atrophy, making it difficult to absorb nutrients and essential fluids through food. [1] But, because cases of RCD are so scarce, the factors and causes responsible can be unique to each person. [1]

Refractory celiac disease may develop due to:

  • Untreated celiac disease – Many experts believe celiac disease is underreported due to poor healthcare education and escalating rates of gluten sensitivities.5 So, a person could develop RCD if they’ve lived with undiagnosed celiac disease for a long time but haven’t been properly diagnosed or treated. [1]

    Alternatively, some people don’t follow a strict gluten-free diet even if they have been diagnosed. Even then, RCD can take decades to develop. [6]

  • Gluten exposure – Even strict gluten-free diets can admit small amounts of gluten into the digestive system. Gluten ingestion may be more likely in people who regularly eat highly processed foods, which may contain some gluten due to manufacturing processes and exposure to glutenous ingredients. [7] If a person is extremely gluten-sensitive, even trace amounts of gluten can damage the small intestine.

Risks of Refractory Celiac Disease

All forms of celiac disease are considered severe, chronic autoimmune conditions, but RCD can have especially serious health consequences. If managed improperly, refractory celiac disease may result in [2]:

  • Malnutrition – The most common complication of RCD is malnutrition. If the small intestine is sufficiently damaged, it can be difficult for the digestive system to retrieve nutrients normally (through food).
  • Certain cancers – RCD has been linked to a rare form of cancer known as enteropathy-associated T-cell lymphoma. [8] Lymphomas are a type of cancer originating in cells belonging to the lymphatic system, the network of the body that plays a key role in eliminating waste. Adhering to a strict gluten-free diet from childhood on can significantly lower one’s risk of developing lymphoma. [9]

Refractory celiac disease primarily affects older adults. If you’re diagnosed with RCD, your healthcare provider will categorize your condition into one of two types [6]:

  • Type I – Affecting 1 in 100 people with celiac disease
  • Type II – Affecting 1 in 200 people with celiac disease

Type II refractory celiac disease is more severe, as patients have a greater than 50% likelihood of developing enteropathy-associated T-cell lymphoma [6]

How is Refractory Celiac Disease Diagnosed?

Because refractory celiac disease is rare and difficult to treat, healthcare providers only issue a diagnosis after ruling out other possible causes. Persistent symptoms associated with refractory celiac disease may also be caused by [3]:

  • Intestinal bacterial overgrowth
  • Crohn’s disease
  • Intestinal lymphoma
  • Hypogammaglobulinemia

Moreover, individuals are only evaluated for RCD if they have not begun to heal after 1 year of adhering to the diet prescribed by a healthcare provider. [3] So, if you’ve been on a gluten-free diet for several months and are still experiencing ongoing symptoms, your small intestine may just need some time to recover.

Refractory Celiac Disease Treatment and Management

So, can celiac disease go away over time? Refractory celiac disease requires special treatment by a celiac specialist. [1] Some people who have it may need to meet their nutritional needs intravenously.

In other cases, you can manage refractory celiac disease with [10]:

  • A liquid diet – Your healthcare provider will run a thorough nutritional assessment to determine any micro- or macronutrient deficiencies. Then, they’ll prescribe a liquid diet that can fulfill your nutritional requirements without putting undue stress on your digestive system.
  • Immunosuppressant medication – To healthcare providers, RCD can indicate that it’s the immune system that requires correction, rather than the presence of gluten. In this case, your healthcare provider may prescribe drugs that quell your immune response to certain nutrients.

Whatever treatment protocol you undergo, managing refractory celiac disease requires regular check-ins with healthcare providers. Taking control of your well-being, no matter your diagnosis, is critical to ensuring you get the highest quality of life possible.

Screen for Celiac Disease with Everlywell

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All Everlywell tests are privacy-protected and reviewed by CLIA-certified labs to get results you can rely on. If your results indicate a need for further treatment, Everlywell will even loop you into a vetted provider network that is able to counsel you on next steps that best suit your needs.

Find out your celiac disease status and take charge of your well-being by visiting Everlywell today.

Buy Celiac Screening Test

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References

  1. National Institute of Diabetes and Digestive and Kidney Diseases. Definition & Facts for Celiac Disease | NIDDK. National Institute of Diabetes and Digestive and Kidney Diseases. Published October 17, 2019. URL. Accessed Sept 15, 2023.
  2. NIDDK. Treatment for Celiac Disease | NIDDK. National Institute of Diabetes and Digestive and Kidney Diseases. Published April 5, 2019. URL. Accessed Sept 15, 2023.
  3. Refractory Celiac Disease. NORD (National Organization for Rare Disorders). URL. Accessed Sept 15, 2023.
  4. Mayo Clinic. Celiac Disease - Symptoms and Causes. Mayo Clinic. Published August 10, 2021. URL. Accessed Sept 15, 2023.
  5. Caio G, Volta U, Sapone A, et al. Celiac disease: a comprehensive current review. BMC Medicine. 2019;17(1). doi:https://doi.org/10.1186/s12916-019-1380-zURL. Accessed Sept 15, 2023.
  6. Non-Responsive and Refractory Celiac Disease | BeyondCeliac.org. Beyond Celiac. URL. Accessed Sept 15, 2023.
  7. El Khoury D, Balfour-Ducharme S, Joye IJ. A Review on the Gluten-Free Diet: Technological and Nutritional Challenges. Nutrients. 2018;10(10):1410. doi:https://doi.org/10.3390/nu10101410URL. Accessed Sept 15, 2023.
  8. National Cancer Institute. Lymphoma. National Cancer Institute. Published 2019. URL. Accessed Sept 15, 2023.
  9. Adult Non-Hodgkin Lymphoma Treatment. National Cancer Institute. Published 2019. URL. Accessed Sept 15, 2023.
  10. Green PHR, Paski S, Ko CW, Rubio-Tapia A. AGA Clinical Practice Update on Management of Refractory Celiac Disease: Expert Review. Gastroenterology. Published online September 2022. doi:https://doi.org/10.1053/j.gastro.2022.07.086URL. Accessed Sept 15, 2023.
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