Polyendocrine Syndrome also referred to as Polyglandular Autoimmune Syndrome (PAS) or (PGA)
Complicating the diagnosis of unexplained shifts in blood glucose levels experienced by brittle diabetics are conditions such as PAS in which multiple organs are involved. To better understand the condition let’s breakdown its name.
Poly – meaning more than one, many.
Endocrine – a system or series of hormone producing glands that chemically regulate a number of bodily functions.
Syndrome – a group or set of signs and symptoms that occur together (as an association) and characterize a particular abnormality. The presence of one should serve to alert the physician to look for the others.
Autoimmune – the body’s defensive mechanism mistakes these hormone producing glands as foreign and attacks them as if they were foreign invaders.
Poly-glandular failure which results in hypo-secretion of their respective hormones occurs in three forms expressed as PAS type I, PAS type II and PAS type III. Let’s consider each briefly.
PAS-1 is rare and normally occurs in childhood. These individuals exhibit at least two of the three symptoms: recurring fungal infections of the skin and mucous membranes usually occurring early in life (ages 3-5) but normally before 30; followed by a decrease in function in the parathyroid glands (by the age of ten) that regulates the amount of calcium and phosphorus in the bone and blood. This can lead to weakness, fatigue, muscle cramps, abdominal pain, extremity pain, dry skin. The third symptom to appear is as a result of an underactive adrenal gland leading to Addison’s disease usually by age 20.
PAS - II is the most common of the three. It is characterized by a combination of Addison’s disease, thyroid autoimmune disease , and/or type 1 diabetes. Celiac disease, myasthenia gravis and primary hypogonadism are sometime seen in this syndrome. This syndrome normally occurs in middle-aged adults 30 to 49 years of age.
PAS – III although not as well defined includes thyroid autoimmune disorders in conjunction with two other organ specific autoimmune diseases ( celiac disease, hypogonadism or myasthenia gravis) but does not include adrenal cortex conditions like Addison’s.
When evaluating patients with underactive endocrine gland function, physicians should consider the possibility that other endocrine glands may also be exhibiting similar hypo-functions complicating type 1 diabetes conditions resulting in unstable, uncontrollable blood glucose levels.