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ADDISONS DISEASE BASIC INFO Adrenal hypofunction from primary disease (partial or complete destruction) of the adrenal gland with inadequate secretion of glucocorticoids and mineralocorticoids. An autoimmune process is the most common cause (80% of the cases) followed by tuberculosis. AIDS is becoming a more frequent cause. Addison disease (primary adrenocortical insufficiency) is differentiated from secondary (pituitary failure) and tertiary (hypothalamic failure) causes of adrenocortical insufficiency. Mineral corticoid function usually remains intact in secondary and tertiary adrenocorticoid insufficiency. Addisonian (adrenal) crisis - acute complication of adrenal insufficiency (circulatory collapse, dehydration, hypotension, nausea, vomiting, hypoglycemia); usually precipitated by acute physiologic stressor such as surgery, illness, exacerbation of comorbid process, acute withdrawal of long term corticosteroid therapy System(s) affected: Endocrine/Metabolic Genetics: Autoimmune adrenal insufficiency shows some hereditary disposition. Familial glucocorticoid insufficiency may have recessive pattern; adrenomyeloneuropathy is X-linked. Frequent association with other autoimmune disorders. Incidence/Prevalence in USA: Prevalence 4:100,000; incidence 0.6:100,000 Predominant age: All ages; usually 3rd to 5th decade Predominant sex: Females > Males (slight) SIGNS & SYMPTOMS Weakness, fatigue, tiredness Weight loss Dizziness; low blood pressure, orthostatic hypotension Increased pigmentation (extensor surfaces, hand creases, dental-gingival margins, buccal and vaginal mucosa, lips, areola, pressure points, scars; tanning; freckles; vitiligo) Anorexia; nausea; vomiting Chronic diarrhea Abdominal pain Decreased cold tolerance Salt craving Hair loss in females Depression (60-80% of patients) Vitiligo CAUSES Autoimmune adrenal insufficiency (80% of cases in USA) Tuberculosis (most common infectious cause worldwide) HIV (most common infectious cause in USA) Waterhouse-Friderichsen syndrome (disseminated adrenal infection and subsequent infarction; meningococcemia most common; Pseudomonas aeruginosa common in children; atypical pathogens, CMV, Cryptococcus, MAC in immunosuppressed and AIDS) Fungal disease (histoplasmosis, blastomycosis, coccidioidomycosis) Bilateral adrenal hemorrhage and infarction (anticoagulants; 50% are in therapeutic range at time of hemorrhage) Antiphospholipid syndrome Metastatic (lung, breast, kidney, colon, melanoma), lymphoma, Kaposi sarcoma (tumor must destroy 90% of gland to produce hypofunction) Drugs (ketoconazole, etomidate) Shock Surgical adrenalectomy

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Radiation therapy Sarcoidosis RISK FACTORS Family history of autoimmune adrenal insufficiency. About 40% of patients have a first- or second-degree relative with one of the associated disorders. Taking steroids for prolonged periods, then experiencing severe infection, trauma or surgical procedures DIAGNOSIS LABORATORY Low serum sodium Elevated serum potassium Elevated BUN, creatinine Elevated serum calcium Hypoglycemia when fasted Metabolic acidosis Low cortisol level (between 8 and 9 a.m.) Elevated ACTH level Moderate neutropenia Eosinophilia Relative lymphocytosis Anemia, normochromic, normocytic Adrenal-cortex autoantibody (ACA/21-hydroxylase) Low aldosterone levels TSH - repeat when condition has stabilized. Thyroid hormone levels may normalize with treatment of Addison disease. Drugs that may alter lab results: Digitalis Disorders that may alter lab results: Diabetes mellitus PATHOLOGICAL FINDINGS Atrophic adrenals in autoimmune adrenalitis. Infiltrative and hemorrhagic disorders produce enlargement with destruction of entire gland. SPECIAL TESTS Rapid ACTH stimulation test: Cosyntropin 0.25 mg IV, measure pre-injection and 60 minute post-injection cortisol levels. Patients with Addison disease have low to normal values that do not rise Metapyrone test Insulin-induced hypoglycemia test CRH may help distinguish secondary from tertiary adrenal insuffi ciency Autoantibody tests 21-Hydroxylase (most common and specifi c) 17-Hydroxylase 17-alfa-Hydroxylase (may not be associated) Adrenomedullin IMAGING Abdominal CT scan Small adrenal glands in autoimmune adrenalitis Enlarged adrenal glands in infiltrative and hemorrhagicdisorders Abdominal x-ray: may show adrenal calcifications CXR: may show adrenal calcifications, small heart size, calcification of cartilage DIAGNOSTIC PROCEDURES A work-up to determine the cause of Addison disease. CT guided fine-needle biopsy of adrenal masses may be helpful. TREATMENT

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APPROPRIATE HEALTH CARE Outpatient Inpatient during adrenal crisis GENERAL MEASURES Treatment for adrenal insufficiency is with glucocorticoid and mineralocorticoid replacement 5 Ss of management of adrenal crisis: salt, sugar, steroids, support, search for precipitating illness Appropriate treatment for underlying cause (e.g., tuberculosis) SURGICAL MEASURES N/A ACTIVITY As tolerated DIET Arrange for a diet that maintains water, sodium and potassium balances PATIENT EDUCATION For patient education materials, contact: National Addison Disease Foundation, 505 Northern Blvd., Suite 200, Great Neck, NY 11021, (516)487-4992 Patient should wear or carry medical identification with information about the disease and the need for hydrocortisone or other replacement therapy Instruct patient in self-administering of parenteral hydrocortisone for emergency situations (e.g., traveling in remote areas away from medical help) MEDICATIONS DRUG(S) OF CHOICE For chronic adrenal insufficiency: Hydrocortisone 15-20 mg orally each morning upon arising and 10 mg at 4-5 each afternoon is usual dosage (dosage may vary and is usually less in children) PLUS Fludrocortisone 0.05-0.2 mg orally once/day plus Dehydroepiandrosterone 25-50 mg orally once a day (monitor lipid profile, breast or prostate cancer) May require salt supplementation Acute adrenal insufficiency Hydrocortisone 100 mg IV followed by 10 mg/hr infusion IV glucose, saline, plasma expanders Fludrocortisone 0.05 mg qd For acute illnesses (fever, stress, minor trauma) Double the patients usual steroid dose. Taper gradually over a week or more, monitor VS and serum sodium. Supplement for surgical procedures: 25-150 mg hydrocortisone or 5-30 mg methylprednisolone IV on day of procedure in addition to maintenance therapy. Taper gradually to usual dose over 1-2 days. Contraindications: Refer to manufacturers literature Precautions: Patients with hepatic disease may need a reduced dose Elderly should have a slightly reduced dose Excessive corticosteroid doses or excessive duration of supplemental treatment of those who are acutely ill or undergoing surgery may increase the mortality rate Refer to manufacturers literature for other precautions Significant possible interactions: Refer to manufacturers literature. Rifampin, phenytoin, and barbiturates may precipitate adrenal insufficiency in addisonian patients by inducing steroid-metabolizing liver enzymes. Patients on these drugs may require higher doses of corticosteroid due to increased steroid metabolism. ALTERNATIVE DRUGS Prednisone 5 mg in AM and 2.5 mg at hs plus fludrocortisone, and DHEA; dexamethasone 0.5 mg in AM plus fludrocortisone plus DHEA FOLLOWUP

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PATIENT MONITORING Verify adequacy of therapy - normal blood pressure, serum electrolytes normal, normal plasma renin, improvement of appetite and strength, increase in heart size to normal, normal fasting blood glucose level Lifelong medical supervision for signs of continued adequate therapy and avoidance of overdose PREVENTION/AVOIDANCE No preventive measures known for Addison disease Prevention of complications Anticipate adrenal crisis and treat before symptoms begin If nausea and vomiting preclude oral therapy, patient should seek medical help to start parenteral therapy Elective surgical procedures require upward adjustment in steroid dose Prevent exposure to infections POSSIBLE COMPLICATIONS Hyperpyrexia Psychotic reactions Complications from underlying disease Over- or under-steroid treatment Hyperkalemic paralysis (rare) Addisonian crisis EXPECTED COURSE/PROGNOSIS Requires lifetime treatment Good outlook with appropriate treatment. With adequate replacement therapy, life expectancy approximates normal. 100% lethal without treatment MISCELLANEOUS AGE-RELATED FACTORS Pediatric: Hydrocortisone and fludrocortisone doses are lower than adults More difficult to diagnose Occurs in siblings Geriatric: Acute adrenal crisis more likely SYNONYMS Adrenocortical insufficiency Waterhouse-Friderichsen syndrome (adrenal crisis) Corticoadrenal insufficiency Primary adrenocortical insufficiency REFERENCES Coursin DB, Wood KE. Corticosteroid supplementation for adrenal insuffi ciency. JAMA 2002;287(2):236-40 Dale DC, Federman DD, eds. Scientific American Medicine. New York, Scientific American, Inc., 1997 Oelkers W. Adrenal insufficiency. NEJM 1996;335(16):1206-12 Oelkers W. Dehydroepiandrosterone for adrenal insufficiency. NEJM 1999;341(14):1073-4 King MS. Adrenal insufficiency: an uncommon cause of fatigue. J Am Board Fam Pract 1999;12(5):386-90 Web references: 6 available at www.5mcc.com

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