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PubMed This is a summary of 17 peer-reviewed journal articles Updated
Endocrinology · Autoimmune Polyglandular Syndrome Type 2

Life with APS-2: Staying Prepared and Protecting Your Family

At a Glance

Managing Autoimmune Polyglandular Syndrome Type 2 (APS-2) requires lifelong monitoring and strict emergency preparedness. Patients must practice "stress dosing" steroids during illness, carry an injectable hydrocortisone kit for emergencies, and wear a medical alert ID at all times.

Living well with Autoimmune Polyglandular Syndrome Type 2 (APS-2) is a lifelong journey of awareness and preparation. Because your immune system has a tendency to target different glands over time, the goal of long-term care is to stay “one step ahead” of the condition through regular monitoring and a robust emergency plan [1][2].

Your Lifelong Surveillance Schedule

Even if you only have one component of APS-2 today, your care team will perform “dynamic monitoring” to catch new issues before they become symptomatic [1].

  • Annual Blood Work: Most guidelines recommend yearly checks of your TSH (thyroid function) and HbA1c (blood sugar levels) [3][4].
  • Symptom Check: At every visit, your doctor will look for signs of new autoimmune attacks, such as skin hyperpigmentation (adrenal) or unexplained weight changes (thyroid/pancreas) [5][6].
  • Diabetes Watch: If you already have Type 1 Diabetes, pay close attention to any unexplained, frequent “lows” (hypoglycemia). This is often a first warning sign that the adrenal glands are becoming involved [7][8].

Emergency Preparedness: The “Safety Net”

Because your body cannot produce extra cortisol during times of physical stress, you must manually provide it [9]. This is known as stress dosing.

  1. Sick Day Rules: During minor illnesses like a fever, flu, or infection (including COVID-19), you must double or triple your usual oral steroid dose [10][9]. Crucially, contact your endocrinologist if your fever lasts more than 48-72 hours or if you are unsure how long to maintain the higher dose.
  2. The Emergency Kit: If you are vomiting or have severe diarrhea and cannot absorb your pills, you are at immediate risk of an adrenal crisis [2]. You must have an injectable hydrocortisone kit (such as Solu-Cortef). Note: This is usually an intramuscular injection that requires mixing a vial and drawing it into a syringe—it is not an auto-injector like an EpiPen. Ensure you and a family member practice this process [11][12].
  3. Medical Alert ID: You should wear a medical alert bracelet or necklace at all times. In an emergency where you are unconscious or confused, this tells medical responders that you require life-saving steroids immediately [13].
  4. Major Stress/Surgery: If you are having surgery or a major procedure, you will require high-dose steroids given through an IV to keep your body stable [14].

Protecting Your Family

APS-2 has a strong genetic component. While the condition itself is not passed down directly, the “susceptibility” to autoimmune issues is [15].

First-degree relatives (parents, siblings, and children) have an increased risk of developing endocrine autoimmunity [16]. Rather than widespread autoantibody testing—which can cause unnecessary anxiety if the antibodies never lead to disease—it is generally recommended that your healthy family members undergo periodic screening with functional labs (like checking TSH for thyroid or HbA1c for blood sugar) [4]. You should encourage them to discuss specific autoantibody testing options with their own primary care doctor or an endocrinologist [15].

Daily Management Checklist

  • [ ] Medication: Take your hormones at the same time every day to mimic natural rhythms.
  • [ ] ID: Wear your medical alert jewelry.
  • [ ] Kit: Check the expiration date on your emergency injection kit every 6 months.
  • [ ] Awareness: Keep a “Sick Day” protocol on your fridge or in your wallet.
  • [ ] Family: Ensure your emergency contacts know how to recognize the signs of an adrenal crisis (confusion, extreme weakness, vomiting) [17].

Common questions in this guide

Why do I need to increase my steroid dose when I am sick with APS-2?
During illness or physical stress, a healthy body naturally produces extra cortisol. Because your adrenal glands cannot do this, you must manually increase your oral steroid dose to prevent a life-threatening adrenal crisis.
When should I use my emergency injectable hydrocortisone kit?
You should use your emergency injectable hydrocortisone kit if you are vomiting, have severe diarrhea, or cannot keep your oral steroid pills down. This requires immediate administration to prevent an adrenal crisis.
Should my family members be tested for APS-2?
First-degree relatives have an increased risk for autoimmune conditions. Doctors generally recommend periodic screening with functional labs, such as checking TSH for thyroid function and HbA1c for blood sugar, rather than widespread autoantibody testing.
How does having Type 1 Diabetes affect my APS-2 monitoring?
If you have Type 1 Diabetes, unexplained or frequent low blood sugars can be an early warning sign that your adrenal glands are failing. You should report these frequent lows to your endocrinologist immediately.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Can you help me create a written 'Sick Day Protocol' tailored to my specific medications and lifestyle?
  2. 2.At what point during an illness (e.g., fever duration) should I contact your office for guidance?
  3. 3.Can we schedule a time for me (and my family or partner) to practice mixing and using my emergency Solu-Cortef injection kit?
  4. 4.Since I have both diabetes and Addison's, how should I manage my insulin when I'm increasing my steroid dose during illness?

Questions For You

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References

References (17)
  1. 1

    The "polyglandular crisis" behind recurrent hyponatremia: misdiagnosis of a case of autoimmune polyglandular syndrome type 2 and clinical lessons learned.

    Yan M, Wu H, Deng J, et al.

    Frontiers in immunology 2026; (17()):1744295 doi:10.3389/fimmu.2026.1744295.

    PMID: 41659856
  2. 2

    ICPis-Induced Autoimmune Polyendocrine Syndrome Type 2: A Review of the Literature and a Protocol for Optimal Management.

    Shi Y, Shen M, Zheng X, et al.

    The Journal of clinical endocrinology and metabolism 2020; (105(12)) doi:10.1210/clinem/dgaa553.

    PMID: 32905579
  3. 3

    Adrenocortical Crisis Triggered by Levothyroxine in an Unrecognized Autoimmune Polyglandular Syndrome Type-2: A Case Report with Review of the Literature.

    Patel DM, Gurumikhani JK, Patel MV, et al.

    Current drug safety 2021; (16(1)):101-106 doi:10.2174/1574886315666200826095842.

    PMID: 32851966
  4. 4

    Adrenal crisis in a 14-year-old boy 12 years after hematopoietic stem cell transplantation.

    Penger T, Albrecht A, Marx M, et al.

    Endocrinology, diabetes & metabolism case reports 2018; (2018()).

    PMID: 29899990
  5. 5

    Autoimmune Polyglandular Syndrome Type 2 With Hurthle Cell Adenoma: A Rare Association.

    Pandya SS, Shah K, Chavda H, Shah K

    Cureus 2025; (17(9)):e92931 doi:10.7759/cureus.92931.

    PMID: 41133083
  6. 6

    Adolescent onset of autoimmune polyglandular syndrome type 2.

    Murphy SA, Mohd Din FH, O'Grady MJ

    BMJ case reports 2022; (15(5)) doi:10.1136/bcr-2022-249839.

    PMID: 35606028
  7. 7

    MANAGEMENT OF ENDOCRINE DISEASE Disease burden and treatment challenges in patients with both Addison's disease and type 1 diabetes mellitus.

    Chantzichristos D, Eliasson B, Johannsson G

    European journal of endocrinology 2020; (183(1)):R1-R11.

    PMID: 32299062
  8. 8

    Novel presentation of autoimmune polyglandular syndrome II in a child with simultaneous Addison's disease, type 1 diabetes, and Hashimoto's thyroiditis: A case report.

    Staub K, Abrams P

    Clinical case reports 2021; (9(8)):e04453 doi:10.1002/ccr3.4453.

    PMID: 34457274
  9. 9

    Daily adjustment of glucocorticoids by patients with adrenal insufficiency.

    Schöfl C, Mayr B, Maison N, et al.

    Clinical endocrinology 2019; (91(2)):256-262 doi:10.1111/cen.14004.

    PMID: 31050815
  10. 10

    Adrenal crisis in a patient with APS2 due to COVID-19: A case report.

    Suryadevara V, Merugu C, Perumal NL, et al.

    Journal of family medicine and primary care 2022; (11(5)):2228-2230 doi:10.4103/jfmpc.jfmpc_1405_21.

    PMID: 35800560
  11. 11

    Adrenal Insufficiency in Adults: A Review.

    Vaidya A, Findling J, Bancos I

    JAMA 2025; (334(8)):714-725 doi:10.1001/jama.2025.5485.

    PMID: 40522647
  12. 12

    Prehospital Management of Acute Addison Disease: Audit of Patients Attending a Referral Hospital in a Regional Area.

    Goubar T, Torpy DJ, McGrath S, Rushworth RL

    Journal of the Endocrine Society 2019; (3(12)):2194-2203 doi:10.1210/js.2019-00263.

    PMID: 31723718
  13. 13

    People With Adrenal Insufficiency Who Are in Adrenal Crisis Are Frequently Unable to Self-Administer Rescue Injections.

    Hover WJ, Krein AD, Kallet J, et al.

    Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists 2025; (31(5)):625-630 doi:10.1016/j.eprac.2025.02.017.

    PMID: 40043845
  14. 14

    Prevention of Adrenal Crisis: Cortisol Responses to Major Stress Compared to Stress Dose Hydrocortisone Delivery.

    Prete A, Taylor AE, Bancos I, et al.

    The Journal of clinical endocrinology and metabolism 2020; (105(7)) doi:10.1210/clinem/dgaa133.

    PMID: 32170323
  15. 15

    Polyglandular syndrome type 2 in a Mexican family and its association with human leukocyte antigen.

    Bermeo-Cabrera J, Reza-Albarrán AA, Granados J, et al.

    Clinical case reports 2019; (7(1)):79-82 doi:10.1002/ccr3.1919.

    PMID: 30656013
  16. 16

    Increased risk of endocrine autoimmunity in first-degree relatives of patients with autoimmune Addison's disease.

    Fichna M, Małecki PP, Młodzikowska M, et al.

    European journal of endocrinology 2020; (183(1)):73-81.

    PMID: 32487775
  17. 17

    Management of adrenal emergencies in educated patients with adrenal insufficiency-A prospective study.

    Burger-Stritt S, Kardonski P, Pulzer A, et al.

    Clinical endocrinology 2018; (89(1)):22-29 doi:10.1111/cen.13608.

    PMID: 29617051

This page provides general information on managing APS-2 and emergency preparedness for educational purposes. Always consult your endocrinologist to develop a personalized sick day protocol and emergency care plan.

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