What are Polyglandular Autoimmune Syndromes (PGS)?

Autoimmune diseases occur when the immune system is directed towards its own organs. In the case of polyglandular autoimmune syndrome (PGS) the endocrine glands are involved and the immune attack results in a mixture of hyper- and hypo-function of those involved. There are two main types, compared below:

PGS type 1

PGS type 2

Occurs mainly
in childhood

Occurs mainly
in adulthood

Decreased
adrenal function

Decreased
adrenal function

Decreased
parathyroid function

Increased or decreased thyroid function possible

Mucocutaneous candidiasis (thrush in the mucous membranes of the mouth)

Possible diabetes mellitus type 1

Of relevance to pancreatic problems is PGS 2 where diabetes mellitus can develop due to autoimmune destruction of insulin-producing beta cells by autoantibodies.

With such a wide range of possible endocrine dysfunction, symptoms can be many and varied. In PGS 2, the diabetes mellitus symptoms (such as excessive thirst and an increased frequency of urination at night) will be associated with problems such as low blood pressure and increased susceptibility to infection resulting from adrenal insufficiency. Increased thyroid function would cause symptoms such as anxiety, tremor, weight loss, diarrhoea, menstrual irregularity, palpitations and heat intolerance. Symptoms of reduced thyroid function include weight gain, cold intolerance, mental slowing, dry skin and low blood pressure. As all these can occur in any combination, a diverse clinical picture is often presented.

PGS 1 and 2 are often associated with other autoimmune conditions such as rheumatoid arthritis, vitiligo and systemic lupus erythematosus.