How is low cortisol related to APS?

The adrenal glands produce cortisol, a hormone essential for stress response and metabolism. In APS, the immune system attacks the adrenal cortex, reducing cortisol production. Low cortisol levels are often the first sign of APS and can cause fatigue, weight loss, and dangerous drops in blood pressure. Regular cortisol testing helps catch adrenal insufficiency early.

Autoimmune Polyglandular Syndromes (APS Type 1 and 2)

What is Autoimmune Polyglandular Syndromes (APS Type 1 and 2)?

Autoimmune Polyglandular Syndromes are rare conditions where your immune system attacks multiple hormone producing glands. These glands normally release hormones that control metabolism, stress response, blood sugar, and other vital functions. When the immune system mistakenly targets them, gland function declines over time.

There are two main types. APS Type 1 usually appears in childhood and affects the parathyroid glands, adrenal glands, and often the ovaries or testes. APS Type 2 typically develops in adults between ages 20 and 60. It most commonly involves the adrenal glands, thyroid gland, and sometimes the pancreas. Both types can cause a cascade of hormone imbalances that require lifelong monitoring.

The term polyglandular means many glands are affected. Each person experiences a different combination of gland problems. Some develop symptoms slowly over years, while others notice changes within months. Early detection through blood testing helps catch hormone deficiencies before they cause serious complications.

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Symptoms

Extreme fatigue that does not improve with rest
Unexplained weight loss despite normal eating
Darkening of skin, especially in folds and scars
Salt cravings and low blood pressure
Dizziness when standing up quickly
Muscle weakness and joint pain
Nausea, vomiting, or stomach pain
Low blood sugar episodes
White patches on skin, called vitiligo
Chronic yeast infections or fungal infections in Type 1
Hair loss or changes in hair texture
Menstrual irregularities or fertility problems

Some people have no obvious symptoms in the early stages. Hormone levels may decline gradually over months or years. Regular blood testing can catch deficiencies before symptoms become severe or life threatening.

Causes and risk factors

Autoimmune Polyglandular Syndromes happen when the immune system produces antibodies that attack your own glands. In APS Type 1, mutations in the AIRE gene cause the immune system to fail at recognizing body tissues as safe. This genetic form typically runs in families and appears in childhood. APS Type 2 has a more complex genetic pattern involving multiple genes that regulate immune function. It often occurs alongside other autoimmune conditions like celiac disease or rheumatoid arthritis.

Risk factors include family history of autoimmune disease, being female for Type 2, and having other autoimmune conditions already. Environmental triggers like infections or severe stress may activate the disease in people who carry genetic risk. The adrenal glands are often affected first, leading to primary adrenal insufficiency. Once one gland is attacked, others frequently follow within months or years. Early intervention can slow progression and prevent dangerous hormone crises.

How it's diagnosed

Diagnosis starts with blood tests that measure hormone levels from different glands. Cortisol testing is essential because adrenal insufficiency is common in both APS types. Low cortisol levels suggest the adrenal glands are not working properly. Your doctor may also test thyroid hormones, blood sugar, calcium, and other markers depending on your symptoms. Antibody tests can identify which glands the immune system is attacking.

Rite Aid offers cortisol testing as part of our core panel to screen for adrenal problems. Getting tested twice per year helps catch hormone changes early. If APS is suspected, your doctor will order additional specialized tests to confirm which glands are affected. Genetic testing may be recommended, especially for Type 1. Early diagnosis prevents life threatening adrenal crises and helps you start hormone replacement before damage becomes severe.

Treatment options

Hormone replacement therapy for deficient glands, such as hydrocortisone for adrenal insufficiency
Thyroid hormone medication if thyroid function is low
Calcium and vitamin D supplements for parathyroid problems
Insulin or other medications for diabetes if pancreas is affected
Regular blood tests every 3 to 6 months to monitor hormone levels
Stress dose steroids during illness, injury, or surgery to prevent adrenal crisis
Medical alert bracelet indicating adrenal insufficiency
Nutrition focused on stable blood sugar and mineral balance
Working with an endocrinologist who specializes in hormone disorders
Screening family members if Type 1 is diagnosed

Frequently asked questions

APS Type 1 usually starts in childhood and is caused by a single gene mutation. It most often affects the parathyroid glands, adrenal glands, and causes chronic fungal infections. APS Type 2 develops in adulthood and involves multiple genes. It typically affects the adrenal glands, thyroid, and sometimes the pancreas, but not the parathyroids.

There is no cure for APS because the immune system continues to attack glands throughout life. However, hormone replacement therapy can manage symptoms very effectively. Most people with APS live normal lifespans when they take their medications consistently and monitor their hormone levels regularly. Treatment focuses on replacing the hormones your body can no longer produce.

Adrenal crisis happens when cortisol levels drop dangerously low during physical stress. Infections, injuries, surgery, or severe illness increase your body's cortisol needs. If you have adrenal insufficiency from APS and do not take extra medication during stress, you can develop shock, confusion, and life threatening complications. This is a medical emergency requiring immediate treatment.

Most doctors recommend testing hormone levels every 3 to 6 months once you are stable on treatment. More frequent testing may be needed when adjusting medications or if new symptoms appear. Cortisol, thyroid hormones, blood sugar, and calcium are typically monitored. Regular testing ensures your replacement doses remain appropriate as your condition changes over time.

Yes, APS Type 1 is inherited in an autosomal recessive pattern. This means both parents must carry the mutated AIRE gene for a child to develop the condition. If both parents are carriers, each child has a 25 percent chance of having APS Type 1. Genetic testing and counseling are recommended for families affected by this condition.

Yes, physical or emotional stress increases your body's demand for cortisol. If your adrenal glands cannot produce enough cortisol due to APS, you may experience worsening fatigue, nausea, and low blood pressure during stressful periods. Many patients need to increase their cortisol replacement dose during illness or major life events to prevent adrenal crisis.

APS Type 2 often appears alongside celiac disease, type 1 diabetes, vitiligo, and pernicious anemia. Some people also develop rheumatoid arthritis or autoimmune liver disease. Having one autoimmune condition increases your risk for others. Your doctor should screen for these conditions if you are diagnosed with APS Type 2.

Yes, children diagnosed with APS Type 1 need lifelong hormone replacement therapy. The condition does not go away with age. As children grow, their medication doses need regular adjustment. They also require ongoing monitoring because new gland problems can develop years after the initial diagnosis.

While diet cannot cure APS, proper nutrition supports hormone balance and overall health. Eating regular meals helps prevent low blood sugar episodes. Getting enough salt supports blood pressure if you have adrenal insufficiency. Some people with APS also have celiac disease and must avoid gluten. Work with your doctor or a nutritionist to create a meal plan that supports your specific hormone needs.