Celiac disease is an autoimmune disorder. People with one autoimmune disorder are prone to getting other autoimmune disorders. For people with celiac disease, the later the age of diagnosis, the greater the chance of developing another autoimmune disorder. Other serious conditions and some cancers are also associated with celiac disease, though following a strict, gluten-free diet may decrease cancer risk.
|Age of Diagnosis||Chance of Developing Another Autoimmune Disorder|
|Autoimmune Disorder||Increased Risk|
|Idiopathic dilated cardiomyopathy||5.7%|
|IgA nephropathy (Berger’s disease)||3.6%|
|Multiple Sclerosis (MS)||11%|
|Type 1 Diabetes Mellitus||2.4-16.4%|
University of Chicago Medicine Celiac Disease Center, February 2013.
Autoimmune Disorders and Other Conditions
There are a number of autoimmune disorders and other serious conditions associated with celiac disease. These include:
Arthritis/Juvenile Idiopathic Arthritis
Arthritis is the informal name used to identify over 100 diseases of the joints; common symptoms are pain, inflammation and stiffness. Some types of arthritis are autoimmune, like celiac disease, and may respond to treatment with a gluten-free diet, though a strong correlation between the two conditions has yet to be established.
Addison’s Disease is a disorder of the adrenal glands, causing them to produce insufficient amounts of cortisol and aldosterone. Addison’s Disease occurs across all age groups and both sexes and can be life threatening; between 5-12% of individuals with Addison’s Disease also have celiac disease, and vice versa. Treatment generally consists of hormone replacement therapy to compensate for adrenal insufficiency.
Autoimmune hepatitis occurs when the body’s immune system attacks the liver, causing damage. It is common for autoimmune conditions to co-exist; approximately 6% of autoimmune hepatitis patients also have celiac disease. There is some indication that a gluten-free diet can reverse the liver damage in these patients, though a gluten-free diet has yet to be shown to be effective with autoimmune hepatitis generally.
Hashimoto’s Thyroiditis (Autoimmune Thyroid Disease)
A decrease in the thyroid gland’s hormone production leads to a condition called hypothyroidism; the most common cause for this is an autoimmune condition called Hashimoto’s Thyroiditis, in which the immune system attacks and damages the thyroid gland. This condition is most common in middle-aged women but can occur across all ages and both sexes; about 3.5% of patients with Hashimoto’s also have celiac disease.
Crohn’s Disease; Inflammatory Bowel Disease
Crohn’s Disease and celiac disease share many common symptoms, though the underlying causes are different. In Crohn’s disease, the immune system could be causing disruption anywhere along the gastrointestinal tract, and more diagnostic testing is needed than to establish a celiac diagnosis. Treatment consists of changes to diet and possible surgery. A substantial overlap (up to 10%) of patients with these two conditions suggests there may be a genetic connection between the two, and research is continuing in that area.
Chronic pancreatitis is an inflammation of the pancreas that worsens over time, ultimately leading to an inability to digest food and to produce pancreatic hormones. Celiac patients have long been known to be at increased risk for pancreatitis, a risk up to three times greater than in the general population.
Between 5-16% of people with Down Syndrome also have celiac disease; all individuals with DS should be tested for celiac between ages 2 and 3. Down Syndrome, also known as Trisomy-21, is a congenital disorder arising from a chromosomal defect. It has many hallmark physical characteristics, including broad facial features, short stature, and disorders of the gastrointestinal system.
Idiopathic Dilated Cardiomyopathy
Dilated cardiomyopathy is a condition in which the chambers of the heart have become enlarged, or dilated, causing thinning of the muscle and difficulty pumping blood. This can lead to a variety of serious conditions, including heart failure, heart valve problems, arrhythmias and blood clots. A nationwide study published through the American Heart Association found a moderate but not statistically significant prevalence of dilated cardiomyopathy in patients with biopsy-confirmed celiac disease.
IgA Nephropathy (Berger’s Disease)
IgA nephropathy occurs when the antibody immunoglobulin A lodges in the kidneys, impeding their ability to filter wastes from the blood. The exact causes of IgAN are not known, though genetic factors, infections, and celiac disease are all possible sources of predisposition – approximately 2-3% of patients with celiac disease also have IgAN.
Irritable Bowel Syndrome (IBS)
IBS is a disruption of the gastrointestinal system, specifically the colon, that can cause a constellation of symptoms including diarrhea, constipation or both. It is quite widespread, affecting about 10-15% of the worldwide population. As many as 10% of those diagnosed with IBS may actually have celiac disease instead of, or in addition to, IBS.
Like celiac disease, lupus is an autoimmune disorder; it affects an estimate 5 million people worldwide, of whom 90% are women. Lupus can affect any part of the body, and is characterized by flares and remissions. Though there is certainly overlap between lupus and celiac disease, no specific causation or correlation has been established.
Multiple sclerosis is a chronic, long-term, degenerative disease in which the immune system destroys myelin, the protective sheath that covers nerve cells. Because of its involvement with the central nervous system, this devastating disease can affect all manner of functions, including vision, speech, cognition and motor skills. There is an increased chance of co-occurrence of MS and celiac disease, and it is recommended that patients with either condition be routinely tested for the other.
Primary Biliary Cirrhosis
PBC is a rare autoimmune disease in which the bile ducts are slowly destroyed. This can cause buildup of harmful substances in the liver, leading to scar tissue (cirrhosis) and liver failure. There is a strong association between PBC and celiac disease; reciprocal screening is crucial.
Primary Sclerosing Cholangitis
PSC presents similarly to PBC, but is a result of different antibody production affecting the bile ducts. This condition is more commonly associated with ulcerative colitis, and is more prevalent in men, while PBC is more prevalent in women. While there is some discrepancy in the literature regarding the overall comorbidity of PSC and celiac disease, routine celiac screening is recommended for patients with cholestatic liver diseases, as early adoption of the gluten-free diet can limit additional complications for these patients.
Psoriasis is a skin condition identified by rough, red, scaly patches, and is believed to be an immune condition. As such, it is certain that there is some correlation between celiac disease and psoriasis, though the rate of comorbidity is currently unknown. Though in many case psoriasis symptoms may be relieved through treatment with a gluten-free diet, this does not necessarily mean that celiac disease is present.
RA is a systemic, autoimmune type of arthritis that causes inflammation, specifically in the joints of the hands, feet, knees, wrists, elbows and ankles. Unchecked, it can cause irreversible damage and deformation of the joints. While there is no documented correlation between RA and celiac disease, the development of effective drug treatments for RA has given researchers hope that similar drugs may be able to be developed for other autoimmune conditions, like celiac disease.
Another autoimmune condition, scleroderma, causes a hardening and tightening of the skin and connective tissue. It is chronic and without cure, and though it is likely genetically linked, it does not seem to be hereditary. Celiac disease is a known trigger for scleroderma, and celiac patients should be routinely monitored for development of skin and rheumatoid symptoms.
Sjogren’s syndrome is an autoimmune condition which manifests as dryness of the lachrymal and salivary glands, causing dry eyes and dry mouth. The rate of celiac disease in Sjogren’s patients may be as high as 15%, and may often present as silent (asymptomatic) celiac. Individuals with Sjogren’s syndrome may wish to consider screening for celiac disease, as adherence to a gluten-free diet may help alleviate symptoms when both conditions are present.
Turner Syndrome is a chromosomal abnormality in which females are born with only a single X chromosome. Affected individuals experience a variety of physical and reproductive consequences of the condition, including short stature, delayed or absent puberty, and infertility. A large percentage (33-50%) of affected women also experience significant, even life-threatening, heart defects. Prevalence of celiac disease is estimated at about 4-8% of the Turner Syndrome population, compared to 1-2% in the general populace, and routine screening after age 3 is recommended.
Type 1 Diabetes
Type 1 Diabetes, formerly known as Juvenile Diabetes, is an autoimmune condition in which the body attacks the insulin-producing cells of the pancreas, preventing the body’s cells from absorbing and using glucose. Treatment for this condition is the external provision of insulin, usually through daily injections. The relationship between type 1 diabetes and celiac is well-established, with approximately 6% overlap between the patient populations. Reciprocal testing is recommended, though it is common for T1D to be diagnosed first.
Ulcerative Colitis; Inflammatory Bowel Disease
Ulcerative Colitis causes inflammation and ulceration throughout the large intestine, resulting in pain and gastrointestinal distress. Unlike Crohn’s disease, which can affect any part of the GI tract at any tissue depth, ulcerative colitis is limited to the lining of the colon. While both ulcerative colitis/IBD and celiac disease both cause affect the gut and cause gastrointestinal symptomology, correlation between the two diseases is no stronger than with other autoimmune conditions.
Williams Syndrome is a genetic condition affecting an estimated 1 in 10,000 people worldwide. It is identified by characteristic facial features, a cheerful and endearing personality, learning and developmental delays and a variety of cardiovascular and musculoskeletal issues. An estimated 8% of those with Williams Syndrome develop celiac disease; routine screening is recommended beginning at age 3.
There is an increased risk of developing certain kinds of cancers in patients with celiac disease. This risk appears to lessen for some cancers after five years on a strict, gluten-free diet.
Non-Hodgkin lymphoma (intestinal and extra-intestinal, T- and B-cell types)
Non-Hodgkin lymphoma is any of a number of cancers that originate in the body’s immune supportive lymph system. Different types of lymphoma spread and are treated in different ways; an accurate diagnosis is critical for proper provision of medical treatment. Risk of lymphoma is higher for celiac disease patients with persistent villous atrophy, underscoring the importance of adhering to a gluten-free diet to reduce not only present symptoms, but long-term health risks.
Small intestinal adenocarcinoma
Adenocarcinomas account for about 30-40% of cancers in the small intestine. Tumors originate in the cells that line the intestine and can grow and block the passage of material. Though occurrence of this cancer (like lymphoma) may be slightly higher in the celiac disease population, studies suggest that healing the intestine through adherence to a gluten-free diet may reduce risk back to the level seen in the general population.
As the name suggests, esophageal carcinoma is cancer that occurs in the esophagus, the pipe through which food travels from the mouth to the stomach. In patients with untreated celiac disease, occurrence of esophageal carcinoma can by eight times that of the general population; strict following of a gluten-free diet can substantially decrease that risk.
Papillary thyroid cancer
Papillary thyroid carcinoma accounts for 80-90% of all thyroid cancers and is highly treatable. In about 50% of cases, cancer spreads to the lymph nodes, causing a higher recurrence rate but not a higher mortality rate. As with most cancers, patients with untreated celiac disease are at about a threefold higher risk of occurrence, though in this case dietary changes do not seem as effective, suggesting an alternate interaction between celiac and thyroid malignancy.
Melanoma is a malignant tumor of the melanin cells present in skin and, as such, can appear anywhere on the body. Though some initial studies suggested a correlation between celiac disease and melanoma, it has since been determined that that correlation was largely a product of referral bias. Current studies indicate no elevated risk of melanoma for patients with celiac disease.