Type I Polyglandular Autoimmune Syndrome Follow-up

Updated: Nov 15, 2016
  • Author: Saleh A Aldasouqi, MD, FACE, ECNU; Chief Editor: Romesh Khardori, MD, PhD, FACP  more...
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Follow-up

Further Outpatient Care

Apart from the usual medications, enforce the following measures:

  • The patient's diet should be high in calcium, fresh fruits, and vegetables and low in simple carbohydrates.
  • In addition to any other stress management techniques, encourage moderate exercise. This is mainly relevant for patients with adrenal insufficiency.
  • Patients may need a dual-energy radiographic absorptiometry (DEXA) scan to assess any degree of osteoporosis due to long-term steroid use.
  • Inform patients about the symptoms of an acute exacerbation, such as dizziness, lightheadedness, abdominal pain, and nausea and vomiting, as will need stress dosing of the chosen glucocorticoid therapy.
  • In addition, make patients aware of the signs and symptoms of hypoparathyroidism, including muscle cramps or spasms.
  • If evidence of hypothyroidism exists, perform an adrenal evaluation before any thyroid replacement. If replacement of thyroid hormones is urgent or emergent, draw blood for later adrenal evaluation, and administer steroids before starting thyroid replacement dosing.
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Further Inpatient Care

If evidence of hypothyroidism is present, perform an adrenal evaluation before any thyroid replacement. If replacement of thyroid hormones is urgent, draw blood for later adrenal evaluation, and administer steroids before starting thyroid replacement dosing.

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Inpatient & Outpatient Medications

These medications depend on the components present in individual patients and range from agents used for hormone replacement to medications employed to manage fungal infections and other complications/deficiencies.

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Deterrence/Prevention

Strongly advise patients to wear medical alert bracelets indicating that they have adrenal insufficiency.

Provide patients with increased steroid coverage before surgeries or periods of stress (for example, in the case of a febrile illness).

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Complications

Hypoparathyroidism

  • Laryngospasm
  • Basal ganglial calcification
  • Renal stones may arise from vitamin D use due to possible excessive urine Ca ++ excretion. Urine calcium excretion may be monitored in these patients.

Addison disease

  • Arrhythmias secondary to electrolyte imbalance
  • Loss of libido
  • Psychotic illnesses
  • Gastrointestinal complaints
  • Complications from treatment, such as osteoporosis or gastrointestinal ulceration with concurrent use of nonsteroidal anti-inflammatory drugs (NSAIDs)

Other complications include the following:

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Prognosis

The prognosis is variable, depending on how organs are affected and the severity of the disease.

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Patient Education

Outpatient management should include patient education on the various components of polyglandular autoimmune (PGA) syndrome, type I, and the need to screen close relatives as appropriate. An important aspect of patient education is the provision of information about adrenal deficiency; subtle deficiency that goes unnoticed in normal, daily-life situations may become life-threatening in stressful situations.

See Further Outpatient Care.

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