Type II Autoimmune Polyglandular Syndrome

Type II autoimmune polyglandular syndrome, which is characterised by the presence of autoimmune Addison disease, thyroid autoimmune disease and insulin-dependent diabetes mellitus (IDDM) that is also generally called as type 1 diabetes mellitus, is the most common immunoendocrinopathy syndrome. PGA-II, which occurs primarily in adulthood, is found usually around the third or fourth decades of life. Middle aged women are more likely to suffer from type II autoimmune polyglandular syndrome.

Symptoms

Autoimmune polyglandular syndrome type II, even though is not a common disease, may be life-threatening, unless diagnosed at proper time. In addition to the autoimmune adrenal insufficiency, type I autoimmune diabetes mellitus and autoimmune thyroid disease some other conditions such vitiligo, Hypergonadotrophic hypogonadism, chronic autoimmune hepatitis, hypophysitis, alopecia, myasthenia gravis, thrombocytic purpura, rheumatoid arthritis and chronic atrophic gastritis with or without pernicious are also associated with type II autoimmune polyglandular syndrome.

Adrenal insufficiency, the most common disease associated with autoimmune polyglandular syndrome type II, has many common and non-specific symptoms that can fluctuate the disease even it its early stages. Its symptoms commonly include fatigue, anorexia, weakness, nausea and vomiting. The less common symptoms of adrenal insufficiency are salt craving, abdominal pain, constipation, diarrhea and syncope. The patients may experience hypotension, hypoglycaemia, weight loss, and cutaneous and mucosal pigmentation.

Incidence

The Type II autoimmune polyglandular syndrome, which is also called as Schmidt syndrome, is a rare autoimmune disease that will result in a steep fall in production of necessary hormones by the glands.

Type II autoimmune polyglandular syndrome is a relatively rare disease with a prevalence of 1.5-2: 100,000. But, women are more likely to suffer from this disease. Among women, the prevalence is two time more frequent. The initial signs of autoimmune polyglandular syndrome type 11 usually start to develop between the age of 20 and 30.

Treatment

Treatments of Type II autoimmune polyglandular syndrome depend on which endocrine gland is affected. Insulin is required in pancreatic dysfunction, cortisol is required in adrenal dysfunction and thyroid hormone replacement is essential for thyroid hormone dysfunction. But all these treatments should be followed only with the consultation of a physician. So you have to ask the advice of a professional medical officer about the treatments of autoimmune polyglandular syndrome type II or change in treatment plans.

Treatment of type II autoimmune polyglandular syndrome is based on the identification and management of the underlying autoimmune conditions. Autoimmune thyroid disease is commonly found in people with APS 2 and so patients with type 1 A diabetes mellitus and Addison disease should determine the levels of TSH at least once a year. Patients with Addison disease should monitor the other autoimmune disorders, as they are at 50% of risk of developing additional autoimmune disease. These people must be screened for auto-antibodies against insulin, GAD and IA-2 for type 1A diabetes mellitus.

Treatment of type 1A diabetes mellitus mainly includes immunotherapies, as an effort to halt the underlying autoimmune disease process.

Diagnosis

The patients should be screened continuously until they are aged of 50 years. The screening includes an assessment of electrolytes, autoantibodies, thyroid function tests, vitamin B-12 levels, liver function tests, fasting blood glucose, cortrosyn-stimulation test, plasma renin activity, gonadotrophins, CBCs and testosterone. Family members should also be screened for autoimmune disease and considered for genetic counselling. People with autoimmune polyglandular syndrome type II, should use minerals, blood work and vitamins for lifelong.

Preventions

Education about the syndrome is important in case of type II autoimmune polyglandular syndrome, because the patients should understand the importance of lifelong medication the increase of dose at times of stress or illness. Patients with APS II should carry a card with information on their current therapy and treatment plans in case of emergency. A medical alert necklace or bracelet noting the condition can be worn and the patients are directed to contact their doctors whenever they become ill.