This document discusses autoimmune polyglandular syndrome (AIPGS), which involves the dysfunction of two or more endocrine glands due to autoimmunity. It is classified into two main types. Type I occurs in childhood and involves adrenal insufficiency, hypoparathyroidism, and mucocutaneous candidiasis. Type II occurs in adults, predominantly in females, and involves primary adrenal insufficiency in 100% of cases and autoimmune thyroid disease in 70% of cases. The document provides details on the clinical features, causes, investigations, treatment, and prognosis of the different conditions involved in AIPGS.

1. Dr.K.Jeyapalan M.D., D.Diab.,
Prof of Medicine - M II Unit,
M.G.M.C & R.I –Pudhucherry.

2. Auto Immune Poly Glandular
Syndrome (AIPGS)
Definition:
Two or more endocrine glands are simultaneously
hypo or hyperfunctional as the result of auto immune
dysfunction.
Classified – 02 Main Types – Type I & Type II

3. AIPGS – Type I
 Childhood – Equal M: F, autosomal recessive, AIRE –
gene on Chr 21
1. Mucocutaneous Candidiasis
2. Hypoparathyroidism – 89%
3. Adrenal insufficiency – 60%
Hypogonadism, Alopecia, Hypothyroidism, Dental
Enamel hypoplasmia, Malabsoption, Chronic active
hypatitis, Vitiligo, Pernicious anemia, DMT1 (1-4%)
 Clinically apparent by age of 15yrs.

4. AIPGS – Type II
 Schmidt Syndrome / Adult – female predominent
 Familial, autosomal dominent, HLA – DR3 & 4
1. Primary Adrenal Insufficiency – 100%
2. Auto Immune Thyroid disease – 70%
3. Diabetes Mellitus – Type 1, upto 50%
 Hypogonadism, Hypophysitis, Myasthenia gravis,
Vitiligo, Alopecia, Pernicious anemia, Celiac disease.
 No mucocutaneous candidiasis (differ - AIPGS T1)
 AIPGS T1 & T2 – both – non endocrine organ
dysfunction primarily – GIT & Skin diseases.

5. Endocrine – Pancreas & G.I.T
Hormone Effects Tumour
Insulin Recurrent Hypoglycemia Insulinoma
Glucogan D.M Necrolytic migratory Erythema Glucagonoma
Somatostatin DM & Steatorrhoea Somatostatinoma
Gastrin P.U, Steatorrhoea, Z.E Syndrome Gastrinoma
VIP (Vasoactive
Intestinal
Peptide)
Watery diarrhoea & Hypokalemia VIPoma

6. Clinical Features – AIPGS - TII
 Adrenaline insufficiency – 100%
- Addison’s disease
 Auto Immune Thyroiditis / Hypothyroidism 70%
 Diabetic mellitis – 50%

7. Primary Adrenal insufficiency –
Addison’s disease – Causes.
 I. Primary ACH – Addison’s disease
 II. Secondary ACTH
* Withdrawal of suppresive glucocorticoid treatment
* Hypothalamic or Pituitary disease
 III. Corticosteroid biosynthetic Enzyme defect
Congenital adrenal hyperplasias
Drugs – Metyrapone, Ketoconazole, etomidate.
Common cause Rare Cause
Auto immune (AIPGS) Lymphoma, Intra ad.hge (WHFS)
TB Amyloidois, Hoemochromotosis
HIV/AIDS
Metastatic Carcinoma
Bilateral adrenalectomy

8. Addison’s Disease ( ACTH) C/F
I. Ad.cortex Hormone Effects
ACTH increases Cortisol decreases Skin & mucous membrane
manifestation
Z.Glm…
Minerelo Cort /Aldoestrone
decrease
Decrease NA – Hypotension
Increase K – Elect-dist
Z.Fas… Gluco Cort decrease Intermediary metabolism,
Immune response
Z.Ret…. Ad-androgens (sex-st)
decrease
Sec.Sex character decreased.
II. Ad. Medulla Adranaline, Nor
Adrenaline decrease
Hypotension
Ad.crisis

9. AIPGS – Diabetic mellitus
 Type I – A – Immune mediated – 90%
B – Idiopathic – 10%
 Type II – Insulin resistance & deficiency
 Other specific types – Genetic defects β cell faults /
Insulin actions, Exocrine
Pancreatic disease,
Endocrinopathis, Drug induced,
Infection, other genetic syndrome
 GDM

10. AIPGS – Hypo Parathyroidism
 Most common cause & more common in AIPGS T1
Post operative thyroid surgery,
Rare – infiltration of glands – Hoemochromotosis &
copper in Wilson’s disease
Congenital or inherited – AIPGS – T1
 Pseudo Hyporparathyroidism
Functionally hyporparathyroidism but PTH not in def,
instead of PTH increases due to tissue resistance
 C/F due to Hypocalcaemia – Acute & Chronic symptoms/signs
 Treatment – Oral calcium salts & Vit D analogous
Recombinant PTH – subcut Inj for osteoporosis
but not in Pseudo (no use because already resist)

11. AIPGS - Investigations
Depends on the endocrine organs involvement
 Urine - Sugar, Cortisol, Calcium.
 Blood – Sugar, hormones level, Auto immune hormone
antibodies, serum electrolytes.
 Images – X Ray, CT, USG, MRI – to R/O Tumours & Calcif
 Biopsy

12. AIPGS - Treatment
 Depends on Hormone involved
 Hormone replacement in deficiency – life long
 Supportive treatment when emergency crisis as in
- Adrenal crisis
- Myxoedema coma
 Co – morbidities treatment

13. AIPGS - Prognosis
 Hormone replacement in deficiency – life long
 Interrupted treatment – bad prognosis
 Emergency crisis – if not in appropriate treatment
very bad prognosis even  100%
mortality