Triple a Syndrome: Causes, Symptoms, and Treatment
Medically reviewed by Harsha Moole, MD, Internal Medicine. Last reviewed: October 2, 2026.
What kind of doctor treats Triple a Syndrome?
Several specialists may be involved. A gastroenterologist treats the esophagus problems, an endocrinologist manages the adrenal hormones, and an eye doctor treats dry eyes. A primary care doctor often coordinates care. A direct primary care or concierge doctor can be a first stop for an evaluation and can refer you to a specialist when needed.
What it is
Triple a syndrome is a rare condition people are born with. The name comes from its three main features: achalasia, alacrima, and Addison disease. Achalasia means the esophagus has trouble pushing food down to the stomach. Alacrima means the body does not make enough tears. Addison disease means the adrenal glands do not make enough of certain hormones. Many people also have nerve damage that causes muscle weakness and slow development.
Symptoms
Signs vary from person to person. Trouble swallowing is one of the most common problems; food may feel stuck in the chest, and children may eat slowly or vomit undigested food. Dry eyes come from the lack of tears. Low adrenal hormones can cause tiredness, low blood sugar, low blood pressure, and dark skin patches. Some children have muscle weakness or learn to walk and talk later than expected.
Causes and who gets it
Triple a syndrome is caused by changes in a gene called AAAS, inherited from both parents. Parents carrying only one changed gene usually have no symptoms. It was first described in people of North African and Middle Eastern background but can occur in anyone.
How doctors diagnose it
Doctors look for the main features: trouble swallowing, dry eyes, and low adrenal hormones. Tests may include an esophagus study, an eye test measuring tear production, and blood tests for adrenal hormones. Genetic testing can confirm the diagnosis.
Treatment
There is no cure, but each feature can be treated. Achalasia may be managed with medicines, stretching of the esophagus, or surgery. Dry eyes are treated with artificial tears and protective drops. Addison disease is treated with hormone replacement pills, with extra medicine needed during illness or injury.
When to see a doctor
See a doctor if a child has long-lasting trouble swallowing, vomiting of undigested food, very dry eyes, or unusual tiredness and dark skin patches. Seek urgent care for severe vomiting, dehydration, confusion, or a sudden worsening during illness. These can signal an adrenal crisis, which needs fast treatment.
How a direct primary care or concierge doctor fits in
A direct primary care doctor charges a flat monthly fee instead of billing insurance. A concierge doctor usually charges more and often works alongside insurance. Either can spend extra time coordinating this complex condition and referring the patient to specialists.
Common questions
Is triple a syndrome the same as Allgrove syndrome?
Yes. It is also called Allgrove syndrome, named after one of the doctors who first described it.
Can triple a syndrome be prevented?
No. Because it is genetic and present from birth, it cannot be prevented. Families with a history may talk to a genetic counselor.
How serious is the adrenal problem?
Low adrenal hormones can be dangerous if untreated, especially during illness or injury. With hormone replacement and a sick-day plan, many people manage this well.
Does everyone with triple a syndrome have all three main features?
Not always. Some people have only one or two features, and signs can appear at different ages.
Sources
- MedlinePlus Genetics - Triple A syndrome
- NIH Genetic and Rare Diseases Information Center - Glucocorticoid deficiency with achalasia
- MedlinePlus - Addison Disease
Medical Disclaimer
This content is for general educational and informational purposes only and is not medical advice. It is not a substitute for professional medical advice, diagnosis, or treatment, and reading it does not create a doctor-patient relationship. Always seek the advice of your physician or another qualified health provider with any questions about a medical condition, and never disregard or delay seeking professional advice because of something you read here. If you think you may have a medical emergency, call 911 (or your local emergency number) immediately.
