Autoimmune polyendocrine syndrome natural history, complications and prognosis

If left untreated, patients with autoimmune polyendocrine syndrome (APS) may progress to involve other endocrine and non-endocrine organs. APS can be a life-threatening condition if vital hormone producing organs of the body are involved such as adrenal glands, thyroid, parathyroid glands. The complications of APS depend upon the subtype and organ system involved. Common complications of APS include those arising from hypoparathyroidism, Addison’s disease and autoimmune thyroiditis. The prognosis of APS is variable and depends upon the duration and severity of endocrine/non-endocrine organ involved. Patients of APS with endocrine involvement generally require lifelong hormone replacement therapy.

• If left untreated, patients with autoimmune polyendocrine syndrome (APS) may progress to involve other endocrine and nonendocrine organs.
• In APS, involvement of one organ is followed by autoimmune disorder of other endocrine-nonendocrine organs. Thus presentation of APS is highly variable and can lead to involvement of wide variety of organs.
• If untreated, APS can be life threatening condition since it may involve vital hormone producing endocrine glands such as adrenal glands, thyroid, parathyroid or pancreas.

The complications of autoimmune polyendocrine syndrome (APS) depends upon the subtype and endocrine/non-endocrine organ involved.

APS type 1

• Common complications of APS type 1 include those arising from hypoparathyroidism such as:^[1][2][3][4]
  • Renal complications (nephrolithiasis, nephrocalcinosis, impaired renal function, symptomatic hypocalcemia)
  • Posterior subcapsular cataracts
  • Basal ganglia calcifications^[5]
  • Complications of intravenous calcium extravasation
  • Hypocalcemic seizure
  • Dilated cardiomyopathy
  • Pathological fractures
  • Depression and other types of neuropsychiatric diseases
  • Increased risk of infections
• Other common complications of the APS type 1 include Addison’s disease

APS type 2

• Common complications of APS type 2 include those arising from Addison’s disease such as:^[6]
  • Hypoglycemia
  • Addisonian crisis
  • Hypoxia
  • Hypovolemic shock
  • Cardiac arrest
  • Stroke
• Other common complications of the APS type 2 include autoimmune thyroiditis and type 1 diabetes mellitus

APS type 3

• Common complications of APS type 3 include those arising from autoimmune thyroiditis such as:^{[7][8][9][10][11]}
  • Hypothyroidism
  • Hyperthyroidism
  • Heart failure
  • Myxedema Coma
  • Tracheal and/or esophageal compression
  • Papillary thyroid carcinoma (PTC)
  • Thyroid lymphoma
• Other common complications of the APS type 3 include pernicious anemia

• The prognosis of autoimmune polyendocrine syndrome is variable and depends upon the duration and severity of endocrine/non-endocrine organ involved.
• Patients of APS with endocrine involvement generally require lifelong hormone(s) replacement therapy.
• Patients with family history of APS who are screened and diagnosed early have a good prognosis. In other patients, the prognosis depends upon the component diseases.

Template:WH Template:WS