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Autoimmune enteropathy is a rare disease that affects people’s small intestine. It’s more common in babies than in adults. But both babies and adults have the same symptom: long-term and severe watery diarrhea. The disease typically affects people with autoimmune disorders. Treatments are corticosteroids and immunosuppressants.
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Autoimmune enteropathy (AW-toh-ih-MYOON en-teh-RAH-puh-thee) is a rare autoimmune disease that typically affects babies but may affect adults. Babies with the disease have very watery diarrhea that doesn’t go away within two or three days. The diarrhea typically starts within two to four weeks after birth.
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In autoimmune enteropathy, your baby’s immune system attacks and damages their intestine. That damage means your baby’s body can’t absorb nutrients from breast milk or formula. Instead, nutrients flow out of your baby’s body in the form of diarrhea. Your baby may become dehydrated. They may not gain weight at the expected rate.
Your baby’s pediatrician may notice any issues with your baby’s development and overall health during well-baby care visits. But don’t hesitate to contact your baby’s pediatrician any time you notice a change in your baby’s poop.
Treatment for autoimmune enteropathy includes drugs that slow down or stop immune system attacks on your baby’s intestines.
Autoimmune enteropathy is rare. Experts estimate it affects 1 in 100,000 babies worldwide. Adults may have the disease, but much less frequently than babies.
The main symptom is a steady flow of watery diarrhea instead of the soft poop that most newborns and babies produce. Not peeing (urinating) is another symptom. A newborn should have at least five to six wet diapers a day. Fewer wet diapers may be a sign your baby is becoming dehydrated.
Experts don’t know the exact cause. Often, babies with autoimmune enteropathy have a type of primary immunodeficiency that damages villi in their small intestines. Villi are microscopic, hair-like projections lining the walls of your baby’s small intestine. They absorb nutrients from food (breast milk or formula) and shuttle the nutrients to your baby’s bloodstream.
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Autoimmune enteropathy can cause complications, including:
A pediatrician will ask about your baby’s symptoms and do a physical examination. They may order the following tests:
They may refer you to a gastroenterologist for additional tests like a small intestine biopsy. Your baby’s pediatrician may also refer you to a specialist in autoimmune disorders.
If your baby has this disease, they’ll probably receive fluids and nutrition intravenously (IV, through a vein). They may receive drugs that limit immune system attacks on their small intestine. Specific drugs may include:
Every baby is unique, so what you can expect will depend on their situation. In some cases, drugs that protect your baby’s small intestine from immune system attacks will help their body to absorb nutrients. But babies with this disease have an underlying issue with their immune systems.
If that’s the case with your baby, they may need different kinds of treatment and long-term medical support. For example, your baby’s healthcare provider may recommend your baby have a stem cell transplant.
If tests show your baby has autoimmune enteropathy, you should contact their pediatrician any time you see changes in how often your baby pees and poops — like your baby producing fewer wet diapers.
Autoimmune enteropathy is a rare disease. You may not know much about it. Here are some questions you may want to ask:
Autoimmune enteropathy in adults is very rare, but it’s hard to estimate the exact number. Healthcare providers in one hospital report making an autoimmune enteropathy diagnosis just 13 times in 16 years. All 13 people had some type of autoimmune disease.
If you have a young baby, you may feel anxious if one day, your baby’s poop is looser than usual. You may worry about their wet diaper production.
The occasional loose poop or dry diaper isn’t cause for alarm. Chances are that your baby’s digestive issues aren’t signs of a serious illness like autoimmune enteropathy. But changes in your baby’s poop or pee that don’t go away may be a reason to call their pediatrician — and give you peace of mind.
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Last reviewed on 09/16/2024.
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