Primary Adrenal Insufficiency (Addison Disease)

Addison's disease, also called adrenal insufficiency, is an uncommon disorder that occurs when your body doesn't produce enough of certain hormones. In Addison's disease, your adrenal glands, located just above your kidneys, produce too little cortisol and, often, too little aldosterone.

Addison's disease occurs in all age groups and both sexes, and can be life-threatening. Treatment involves taking hormones to replace those that are missing.

Characteristic features

• Deficiency of cortisol and mineralocorticoid from destruction of the adrenal cortex
• Weakness, vomiting, diarrhea; abdominal pain, arthralgias; amenorrhea.
• Increased skin pigmentation, especially of creases, pressure areas, and nipples.
• Hypovolemic hypotension, small heart.
• Hyponatremia; hyperkalemia (may be absent with vomiting and diarrhea); hypoglycemia; eosinophilia.
• Elevated plasma ACTH level; cosyntropin unable to stimulate serum cortisol to 20 mcg / dL (550 nmol / L) or more.

Acute adrenal crisis

Above manifestations become critical, with fever, shock, confusion, coma, death

Precipitating factors

Infection, trauma, surgery etc

Investigations

Serum cortisol, RBS, serum electrolytes and CT abdomen.

Management

• Patients with adrenal insufficiency ( and family members ) must be thoroughly educated about adrenal insufficiency. Patients are advised to wear a medical alert bracelet or medal reading ' Adrenal insufficiency - takes hydrocortisone '
• If acute adrenal crisis is suspected but the diagnosis of adrenal insufficiency is not yet established, blood is drawn for routine emergency laboratory tests and blood cultures, as well as serum cortisol and ACTH levels. Without waiting for the results, treatment is initiated immediately
• Inj- Hyzonate ( Hydrocortisone ) 100-300mg IV x stat , then 100 IV x QID . Change to oral steroids after 72h if patient's condition is good. Maintenance therapy dose is 15-30mg / day orally in two or three divided doses. (E.g. 10mg at 7 AM, 10mg at 1 PM and 5 mg at 7 PM ).
• Tab ( Fludrocortisone acetate ) 0.1mg , 1-3 tablets PO x OD or alternate day ( Dosage is 0.05 to 0.3mg orally daily or every other day )
• In the presence of postural hypotension, hyponatremia, or hyperkalemia the dosage is increased
• Inj - Oxidil / Rocephin ( Ceftriaxone ) 1gm IV x BD ( ATD ) ' OR ' Inj- Zinacef ( Cefuroxime ) 1.5gm IV x BD ( ATD )
• Inf - Normal saline 0.9 % 1L , IV x stat in 30-60 minutes.
• Inf- 10 or 25 % dextrose water IV x stat, if patient is hypoglycemic. (Continue IV fluids , more slowly . Be guided by clinical state)
• Once the crisis has passed, the patient must be evaluated to assess the degree of permanent adrenal insufficiency and to establish the cause, if possible.