Type II Polyglandular Autoimmune Syndrome Clinical Presentation

Updated: Nov 08, 2016
  • Author: Surendra Sivarajah, MD; Chief Editor: Romesh Khardori, MD, PhD, FACP  more...
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Presentation

History

Polyglandular autoimmune syndrome type II ( PGA-II) consists of Addison disease plus either an autoimmune thyroid disease or type 1 diabetes mellitus associated with hypogonadism, pernicious anemia, celiac disease, and recent primary biliary cirrhosis. [7] The clinical features consist of a constellation of the individual endocrinopathies.

For type 1 diabetes mellitus, some of these clinical features closely mimic those of primary adrenal insufficiency. Note the following:

  • Symptoms - Polyuria, polydipsia, polyphagia, unexplained weight loss, intermittent blurred vision, and lethargy (may present initially with diabetic ketoacidosis and coma)
  • Signs - Depend on the severity; consist of poor skin turgor, orthostasis, and hypotension

For Hashimoto thyroiditis (chronic lymphocytic thyroiditis), note the following:

  • Symptoms - Usually nonspecific and include cold intolerance, fatigue, somnolence, poor memory, constipation, menorrhagia, myalgias, and hoarseness
  • Signs - Slow tendon reflexes, bradycardia, facial and periorbital edema, dry skin and nonpitting edema, carpal tunnel syndromes, deafness, and pericardial or pleural effusions

For Graves disease, note the following:

  • Symptoms - Heat intolerance, weight loss, weakness, palpitations, oligomenorrhea, and anxiety
  • Signs - Brisk tendon reflexes, fine tremor, proximal weakness, stare and eyelid lag, exophthalmos, atrial fibrillation, and sinus tachycardia

For Addison disease (primary adrenal insufficiency), note the following:

  • Symptoms - Anorexia, nausea, vomiting, weight loss, weakness, and fatigue
  • Signs - Chronic hyperpigmentation of creases and scars, as well as orthostatic hypotension

For celiac disease, symptoms are weight loss, steatorrhea, bloating, cramping, and malnutrition.

For pernicious anemia, symptoms are pallor, jaundice, ataxia, glossitis, impaired cognition, impaired vibratory and position sense, and impaired cognition.

Other disorders associated with PGA-II include the following:

  • Hypogonadism (usually autoimmune oophoritis) and hypopituitarism
  • Idiopathic thrombocytopenic purpura
  • Myasthenia gravis
  • Parkinson disease
  • Vitiligo
  • Alopecia
  • Seronegative arthritis
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Physical

See History.

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Causes

The etiology of polyglandular autoimmune syndrome type II (PGA-II) is very poorly understood. Note the following:

  • Some association has been seen between diabetes or hypothyroidism and congenital rubella infection.
  • Immune stimulation by certain dietary proteins is a possible etiologic factor.
  • Additional possibilities include genetic susceptibility and idiopathic immunopathologic dysfunction.
  • Animal models have demonstrated that cytomegalovirus-infected mice may develop PGA-II with lymphocytic infiltration of the thyroid, liver, myocardium, adrenals, pancreatic islets, and salivary glands. At this time, however, no infectious agents or noticeable immunodeficiency states have been associated with human PGA-II.
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