FAQ: Conditions Associated with Celiac Disease
*Words that are starred (*) have definitions at the bottom.
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More detailed answers are available on www.celiacnow.org.
Are there other diseases related to celiac disease?
A number of autoimmune* diseases are associated with celiac disease. These include:
- Autoimmune hepatitis
- Autoimmune thyroid disease
- Biliary autoimmune liver disease (PBC, PSC)
- Cerebellar ataxia* (can be autoimmune)
- Dermatitis Herpetiformis (described below)
- IgA nephropathy*
- Addison’s disease (damage by the immune system to the adrenal glands that produce critical hormones)
- Insulin-dependent diabetes (Type 1)
- Neuromuscular disorders (can be autoimmune)
- Rheumatoid arthritis
- Sjögren's syndrome
- Systemic lupus erythematous*
Others diseases include:
- Down's syndrome
- IgA deficiency*
- Iron deficiency anemia
- Microscopic or lymphocytic colitis
- A variety of cancers
What is Dermatitis Herpetiformis?
Dermatitis Herpetiformis (DH) is an intensely itchy blistering skin rash- that is associated with celiac disease. The rash most commonly affects the elbows, knees, buttocks, scalp, and back. Men with DH may have oral or genital lesions. DH is seen in about 5-10% of people with celiac disease and may sometimes be the sole initial manifestation. It usually starts as small bumps that become blisters and then get scratched off. It appears in several areas, and is formed as a result of ingesting gluten. DH is caused by skin deposits of IgA antibodies (protein that reacts against the body’s own cells or tissue) due to immune reaction in skin after ingestion of gluten. It appears as a blistering rash. This skin reaction is an external presentation of celiac disease that manifests as an abnormal immune response to gluten ingestion. DH is diagnosed by a small skin biopsy done by a dermatologist in the office. The rash usually improves or even disappears on a gluten-free diet, but may not go away completely. There is a medication called Dapsone that will help treat the rash. However, Dapsone is only a temporary remedy for DH, and a gluten-free diet is necessary to treat DH, as well as intestinal damage. Most individuals with DH do not have obvious gastrointestinal symptoms, but almost all have some intestinal damage seen under the microscope.
Can Dermatitis Herpetiformis (DH) be misdiagnosed as any other skin condition?
Though DH may appear similar to other skin conditions, a biopsy of the lesions will confirm the diagnosis of DH. While the findings of DH are very specific, it is important that the dermatologist considers this diagnosis so that he/she can take a sample of the correct area and order the correct tests.
People with type 1 diabetes (T1D) are routinely checked for celiac disease because 1 in 4 people with TID develops celiac disease (CD). Is the inverse true? Do people with CD have to be regularly checked for T1D? Are they more likely to get T1D than the general population?
CD and Type 1 diabetes mellitus (T1D) are autoimmune conditions that are closely associated with each other compared to the general population. The prevalence of CD in patients with T1D ranges from 2% to 5% in adults and from 3% to 8% in children. Doctors caring for patients with T1D should be aware of this association and consider testing for CD if symptoms occur. Moreover, duodenal biopsies should be considered if patients with T1D present for upper endoscopy.
Similarly the prevalence of T1D is higher in those with CD compared to the general population (about 5% versus 0.5%). The average age to develop CD is 40 years old. People diagnosed with T1D are usually less than 20 years old. However, there are no specific guidelines for screening of T1D in those with CD or even in the general population unless there is a clinical suspicion.
If a person with diabetes goes on a gluten-free diet (GFD), will the diabetes improve?
Unfortunately, there is no evidence showing that diabetes will improve on a GFD; however, it is important for people with celiac disease (CD) and diabetes to follow the GFD like all people with CD. When starting a GFD you should monitor your sugars carefully, as there are often changes in weight and response to insulin. A dietitian skilled in nutrition for both diseases should be consulted.
What are some of the symptoms of celiac disease (CD) outside the gastrointestinal tract?
People with CD do not always show common gastrointestinal symptoms of the disease, such as diarrhea, cramping and bloating. In fact, they may have extra-intestinal symptoms, or problems outside of the intestine, that can involve many of the body's different organs and systems. In children, the symptoms may include failure to thrive (an inability to grow and put on weight), irritability, an inability to concentrate, diarrhea and bloating.
Extra-intestinal Symptoms (not all inclusive):
- Improper absorption of vitamin K (a fat soluble vitamin) can result in bleeding problems
- Anemia due to poor absorption of folate and iron in the small intestine, as well as chronic mild blood loss; in severe cases of celiac disease, B12 deficiency can also lead to anemia.
- Protein-calorie malnutrition
- Osteoporosis / Osteopenia (decrease in the amount of bone tissue): These conditions result from poor absorption of calcium and vitamin D by the damaged intestinal villi, as well as due to inflammatory processes directly targeting bone.
- Joint Pain/Bone Pain
- Neurological symptoms include muscle weakness, numbness, and ataxia (poor muscle coordination), and peripheral neuropathy (tingling and numbness in extremities)
- Seizures may develop with or without cerebral calcifications*. This is rare and has only been shown in limited studies.
- Chronic fatigue syndrome (extreme tiredness that may last for months, along with sore throat, myalgias,* and cognitive dysfunction)
Reproductive and Endocrine System
- Recurrent Abortions
- Delayed puberty
- Short stature or growth failure
- Hormonal problems that include amenorrhea (lack of a menstrual cycle), delayed menstruation, and infertility in women. Impotence and infertility in men has been seen as well.
- Skin disorders including Dermatitis Herpetiformis have been seen in patients with celiac disease. See the "What is Dermatitis Herpetiformis?" section above.
- Dental enamel problems ( reported in 20% of patients)
- Canker sores
- Increased risk of developing several different types of cancer has been seen in patients with celiac disease. These cancers include small bowel lymphoma/enteropathy associated T-cell lymphoma (EATL), small bowel adenocarcinoma, large bowel carcinoma, Hodgkins lymphoma*, oropharyngeal* cancer and primary liver cancers. See the "Do I have an increased risk of getting cancer if I have celiac disease?" section below.
Do I have an increased risk of getting cancer if I have celiac disease (CD)?
If you do not treat your CD, you will have a slightly greater risk of developing certain types of gastrointestinal cancer as well as non-Hodgkin's lymphoma. However, after remaining on a gluten-free diet for five years, these higher risks will return to that of the general American population.
People with CD will continue to have an increased risk of developing enteropathy-associated T-cell lymphoma (EATL), which is a type of cancer. However, this cancer is extremely rare, even among patients with CD.
What is ataxia? What are the symptoms? Can you have celiac disease (CD) and ataxia? How do you test for ataxia?
Ataxia describes difficulty coordinating movements. This often appears as a wide based gait (“drunken walk”). This symptom is often related to problems in the function of the cerebellum (a region of the brain that is located in the lower part of the brain). Although rare, ataxia is one of the several neuropsychiatric symptoms that are reported frequently by patients with active CD. Along with other symptoms of CD, ataxia usually stabilizes or gradually decreases over time if the patient maintains a strict gluten-free diet.
A condition called Gluten Ataxia has been described in patients with ataxia, positive anti-gliadin antibodies and no other evident cause. Though still a controversial disease, some researchers believe it is linked to the presence of gluten-like molecules on the surface of cells in the cerebellum. Nonetheless, only one third of patients with gluten ataxia will show intestinal damage in their biopsies and only one tenth of them will have digestive symptoms. The diagnosis is based on a neurologic exam and the anti-gliadin antibodies. The IgA-tTG test has not been shown to be of use in this case. The response of this condition to a gluten-free diet has still not been well established but it may be worth the try.
Have you noticed an increased prevalence of lyme disease in people with celiac disease (CD)?
There are no controlled studies that report a strong association between Lyme disease and CD. However, Lyme disease is an inflammatory disease that affects multiple body organs including the gastrointestinal system. And subjects with lyme can be more sensitive to several foods such as gluten-containing dairy and high-fructose products. This sensitivity has been reported by patients recovering from a recent gastrointestinal infection, those with inflammatory bowel disease, and others.
What is the association between celiac disease (CD) and skin rashes after following a gluten-free diet (GFD)?
Example: flaking scalp issues using gluten-free products, seasonal psoriasis on hands and feet. Which professional should I see?
CD is related to a skin condition called Dermatitis Herpetiformis which responds well to the gluten-free diet. A separate condition called gluten/wheat allergy can be associated with eczema that responds to avoiding wheat.
However, the skin/scalp issues or seasonal psoriasis may or may not be directly linked to CD or the gluten-free diet. One reason could be the nutritional deficiencies due to malabsorption either from untreated CD or dietary restrictions from not following the gluten-free diet in a healthy way. A visit with an allergist or dermatologist may be helpful to uncover the reason for the rash. Meeting with a dietitian skilled in CD can help to balance the diet.
* Key Words:
Autoimmune disease: diseases in which the immune system attacks healthy parts of the body; examples are celiac disease, thyroid disease, vitiligo, rheumatoid arthritis, and type 1 diabetes
Cerebellar ataxia: poor or no coordination of voluntary movements that occur as a manifestation of a disease of the brain
Cerebral calcification: a process by which organic tissue becomes hardened by the physiologic deposit of calcium salts
Hodgkins lymphoma: a malignant disease with progressive enlargement of the lymph nodes, spleen, and general lymphoid tissue
IgA deficiency: a deficiency of the antibody that protects the mucous membranes
IgA nephropathy: deposits of IgA antibodies in the kidney
Myalgias: muscular pain or tenderness
Oropharyngeal: upper part of the throat
Psoriasis: a skin condition that causes scaly patches to develop on the body
Systemic lupus erythematous: a chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue
Vitiligo: a chronic condition that results in the loss of skin pigment
Revision Date: 3-17-16
Authors: Clinicians of the Celiac Center, Dharmesh H Kaswala MD, Javier Villafuerte MD, Rohini Vanga MD, with assistance from Annie Peer
Editors: Melinda Dennis, MS, RD, LDN and Daniel Leffler MD, MS, Rupa Mukherjee MD