Hypergammaglobulinemia (HC) is a genetic disorder characterized by excessive production of antibodies against gliadin, a protein found in wheat, rye and barley. Gluten sensitivity is not the only cause of HC; however it may play a role. People with HC are sensitive to gluten, which is a protein found in grains such as wheat, rye and barley. They have an autoimmune reaction when they eat these foods. The immune system mistakenly attacks the small intestine lining where there is a high concentration of gliadin. These individuals develop symptoms such as abdominal pain, diarrhea or constipation and may even experience life threatening complications like malnutrition or death if left untreated.
The most common form of HC is celiac disease (CD). CD is a genetically inherited condition in which the body’s immune system recognizes gluten as foreign and initiates an attack on the small intestine. There are two forms of CD: non-celiac gluten sensitivity (NCGS) and celiac disease (CD).
NCGS occurs when someone does not have celiac disease but develops symptoms after eating gluten containing products. Celiac disease is a chronic inflammatory bowel disorder that causes damage to the small intestine.
TESTING IN HYPER GAMMAGLOBULINEMIA
No formal testing is required as celiac disease is a clinical condition and can be detected by a small bowel biopsy. A doctor may also order a series of lab tests to rule out other conditions that may cause similar symptoms. The most important test is the IgA anti-gliadin antibody test.
Your doctor may also order an anti-endomysial antibody test, which can detect a specific antibody for CD. In some cases the doctor may perform an ileocolonoscopy to directly view the small intestine and take a biopsy to confirm the diagnosis.
WHAT IS THE TREATMENT FOR HYPER GAMMAGLOBULINEMIA?
The treatment of celiac disease is a strict gluten-free diet. When gluten is removed from the diet, symptoms improve and damage to the small intestine is reversed. A gluten free diet is essential to prevent recurrence of celiac disease and associated conditions such as hypergammaglobulinemia. Gluten is a protein found in grains such as wheat, barley and rye. Oats do not have gluten but may be contaminated with other grains during processing and should also be avoided. Gluten can be found in foods with flavoring such as malt, natural flavors or vegetable broth. Gluten can also be found in additives such as modified food starch or soy sauce. Alcohol and distilled beverages such as whiskey, gin and vodka are sometimes distilled with gluten grains so these should be avoided as well.
This condition is life-long and requires careful attention to diet to avoid ingestion of gluten. It is important for people with this condition to be very careful about their diet as long as they wish to stay healthy. A person who has been diagnosed with celiac disease should always inform any physicians or medical professionals that they have been diagnosed with celiac disease.
FREQUENTLY ASKED QUESTIONS
What is the difference between Celiac Disease and Gluten Sensitivity?
Celiac disease and gluten sensitivity are two different conditions that present with similar symptoms. Both conditions cause damage to the small intestine and can be diagnosed with a biopsy. Gluten sensitivity is not caused by a problem with gluten itself but by a problem with another component of wheat or other gluten containing grains.
How common is hypergammaglobulinemia?
The frequency of hypergammaglobulinemia has not been determined exactly, but it is believed to be rare although it seems to be on the rise. In the United States it is thought to be as common as one in a thousand people.
What are some other names for hypergammaglobulinemia?
Some other names for this condition include Increased Immunoglobulins, Gammopathy, Myeloma Disorder and Plasmacytoma.
How does hypergammaglobulinemia affect the body?
This condition is marked by an excessive increase in the amount of immunoglobulin (antibody) in the blood. When these immunoglobulins are present in high numbers in the blood they can cause damage to various organs such as the kidneys, heart and peripheral nerves.
Is hypergammaglobulinemia inherited?
The exact manner in which this condition is inherited is not known. In most cases family members of people with this condition also have the condition, but this may be due to environmental factors or a combination of genetic and environmental factors.
Can hypergammaglobulinemia be cured?
There is no known cure for this condition. The only treatment is the removal of the cause which can only be done through special diets. In most cases it is due to food allergies. Once the offending food has been identified and removed from the diet, symptoms and progression of the disease should improve.
Can hypergammaglobulinemia cause other conditions?
Yes, this condition can cause other related medical problems such as hypogammaglobulinemia, myeloma and plasmacytoma.
What are the types of hypergammaglobulinemia?
There are five major types of hypergammaglobulinemia and they are categorized by the type of antibody that is in excess.
IgG – This type is also known as Anderson’s Syndrome.
IgM – This type has not been linked to an increased risk of disease.
IgA – This type is also known as Shwartzman Syndrome.
IgD – This type has not been linked to an increased risk of disease.
IgE – This type is also known as Hyper-IgE Syndrome.
What are the symptoms of hypergammaglobulinemia?
The symptoms of this condition vary depending on which type of antibodies are in excess and the organs that are affected by the damage. These conditions can cause damage to various organs such as the liver, heart, and peripheral nerves.
How is hypergammaglobulinemia diagnosed?
The diagnosis of this condition is one of elimination. Doctors will first look for other causes such as autoimmune disorders, lymphoma and diet before arriving at a diagnosis of hypergammaglobulinemia.
What causes hypergammaglobulinemia?
The exact cause of this condition is unknown; however, doctors believe that a combination of genetic and environmental factors are involved in most cases. It is known that in some cases this condition is an inherited condition.
Can hypergammaglobulinemia be treated?
Yes, there are treatments available for this condition. The type of treatment varies depending on the exact cause in each case. The most common type of treatment is the diet treatment. In addition, immunoglobulin levels may also be monitored to make sure they do not get too low. Other types of treatment may include the use of steroids and other medications.
Other therapies that may be recommended include bone marrow or stem cell transplants.
How is hypergammaglobulinemia inherited?
This condition is considered to be an inherited autosomal dominant disorder. Autosomal refers to any trait that is not located on the X or Y chromosomes. Dominant traits are determined by the presence of only one copy of the mutant allele. In other words, if one copy of the allele is responsible for the disease, then the person will have the disease to some degree. In some cases, an affected person inherits the mutation from an affected parent.
What is the prognosis of hypergammaglobulinemia?
The prognosis of this condition depends on which type is present and the extent of damage it has caused. Most types cause varying degrees of damage to the body’s organs.
A few types have a good prognosis if they are caught early and treatment is started, otherwise the outlook can be fatal.
How can hypergammaglobulinemia be prevented?
There is no method of preventing this condition at this time. Doctors do recommend that those who have a family history of this condition should consult with a genetic counselor to see if they should undergo screening.
Glossary
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