The rare condition known as “Autoimmune Enteropathy” (AIE) is brought on when the intestinal lining becomes inflamed or irritated as a result of the body’s immune system attacking itself. It is characterized by intractable diarrhea brought on by gut autoantibodies often to the point of needing intravenous (IV) fluids.
Autoimmune enteropathy in adults is rarely seen. Histologically, it resembles focal diseases like celiac disease, where patients face serious life-threatening complications. Immunosuppressive therapies are used to treat this condition.
There are different kinds of autoimmune enteropathy. Several different tests are needed to figure out which kind of autoimmune enteropathy a person may have. In the blog, we are going to discuss what is autoimmune enteropathy, its symptoms, diagnosis, treatment options available, and autoimmune survival rate.
What is an Autoimmune Enteropathy?
Autoimmune enteropathy is a condition in which the small intestine sustains ongoing damage, irritation, and swelling due to an immune-mediated response from the body. It is believed to be an autoimmune disorder in which the body attacks itself instead of a foreign invader such as a bacterium or virus. In some cases, a genetic cause of the condition can be the reason, but what causes this erroneous immune response still needs to be confirmed.
A faulty immune system causes immune cells to attack the lining of the small intestine, but it can also affect the stomach and large intestine. This causes cell damage, causing the lining to become inflamed and preventing nutrients from being absorbed. Ultimately resulting in inadequate weight gain and growth as seen in autoimmune enteropathy in adults.
What causes this Condition?
The blood cells in the body’s immune system help protect the body from harmful substances like bacteria, viruses, toxins, cancer cells, and blood and tissue from outside the body. When you have an autoimmune disorder, your immune system does not distinguish between healthy tissue and potentially harmful antigens. As a result, the body initiates a chain reaction that destroys normal tissues.
The exact pathogenesis of autoimmune disorders is unknown. Certain microorganisms (such as bacteria or viruses) or drugs may cause changes that confuse the immune system. This may occur more frequently in people who have genes that make them more susceptible to autoimmune diseases. An autoimmune disorder can cause:
- The amputation of body tissue
- An organ’s abnormal growth
- Organ function changes
- One or more organs or tissues to be potentially affected
A person can have multiple autoimmune disorders at once.
What are the common Symptoms?
AIE symptoms, which include both intestinal and extraintestinal manifestations, can be quite debilitating. Among the autoimmune enteropathy symptoms are:
- Constant diarrhea/loose watery stools
- Inadequate weight gain and loss
- Urine output has been reduced
- Bloody stool or occasional skin rash
The main symptom is severe diarrhea, which usually appears within the first six months of life. Diarrhea develops regardless of how healthy the diet is and can quickly lead to dehydration. This condition in adults and children manifests as malabsorption of nutrients from food leading to poor weight gain and growth problems over time. A blood test is usually performed to see if children with autoimmune enteropathy are also affected by other autoimmune disorders.
What are the Characteristics in Adults?
Autoimmune enteropathy is a rare but severe immune-mediated disorder. It’s a rare cause of chronic diarrhea in adults. It was believed that autoimmune, which has a strong male predominance, co-occurring autoimmune-related disorders, and positive serologies for gut autoantibodies, occurs in children before the age of 6 months. However, research has shown that male and female cohort members were equally affected.
Additionally, autoimmune enteropathy can affect people of any age, including newborns and the elderly. Endocrine, renal, pulmonary, liver, hematologic, and musculoskeletal system involvement are just a few of the wide range of associated autoimmune conditions.
How is Autoimmune Enteropathy Diagnosed?
To rule out more common causes of diarrhea, the physician will usually begin by taking a clinical history of the autoimmune enteropathy symptoms present and when they first appeared. Several tests and procedures will be required to diagnose this autoimmune disorder:
- Tests for antinuclear antibodies (ANA)
- Tests for autoantibodies
- Complete blood count (CBC) with differential white blood cell count (CBC with WBC differential)
- A complete metabolic panel
- C-reactive protein (CRP) (CRP)
- The rate of erythrocyte sedimentation (ESR)
Small tissue samples (biopsies) will also be taken from various locations in the intestines. Biopsies will typically reveal villi destruction and areas of sore tissue. Blood tests will also be required to assess the immune system and nutritional status. Stool samples will be tested in a laboratory to exclude other causes of diarrhea.
How to Treat Autoimmune Enteropathy?
AIE is a rare autoimmune disorder that causes intractable watery diarrhea in both children and adult patients. To ensure optimal health, people with autoimmune enteropathy require nutritional support as well as adequate hydration. Total parenteral nutrition (TPN) may be required long-term in some severe cases of autoimmune enteropathy in adults. Immunosuppression therapy has proven effective, but in more severe cases, a bone marrow transplant may be required.
Because the disease responds differently to different immunosuppressive regimens such as corticosteroids, azathioprine, cyclophosphamide, 6-mercaptopurine, tacrolimus, cyclosporine-A, infliximab, vedolizumab, and abatacept, autoimmune symptoms management is not specified.
What is the Estimated Autoimmune Enteropathy Survival Rate?
Currently, therapeutic options are limited. Medication for autoimmune enteropathy symptoms responses has been poor or short-lived. Some patients with chronic antibiotic use have achieved and maintained remission. Diarrhea associated with AIE is unrelenting in the absence of treatment, and the mortality rate has been reported to be as high as 30%.
Autoimmune enteropathy is a heterogeneous disease that affects both children and adults. It is characterized by persistent, severe diarrhea, intestinal damage caused by the immune system, and other autoimmune-related conditions. Immunodeficiency disorders frequently occur in conjunction with autoimmune enteropathy.
This has a wide range of histologic findings that can resemble other immune-related diseases. Therefore, making the correct diagnosis and starting the proper treatment as soon as possible depends on the recognition of both the clinical and pathologic features of this entity.