ADDISONS

DISEASE
ADDISON'S DISEASE IS A CLINICAL CONDITION
RESULTING FROM ADRENOCORTICAL INSUFFICIENCY DUE
TO PRIMARY ACQUIRED DISEASE OF ADRENAL GLAND
INCIDENCE
ADDISON'S DISEASE IS A RARE ENDOCRINE OR
HORMONAL DISORDER THAT AFFECTS ABOUT 1 IN
100,000 PEOPLE. IT OCCURS IN ALL AGE GROUPS
AND AFFLICTS MEN AND WOMEN EQUALLY.
CAUSES OF ADDISONS DISEASE
COMMON CAUSES:
AUTOIMMUNE MECHANISM- 80% CASES (MORE IN FEMALE)
TUBERCULOSIS (OF ADRENAL GLAND)-10%
SECONDARY DEPOSIT IN ADRENALS
HIV INFECTION
BILATERAL ADRENALECTOMY

OTHER CAUSES:
AMYLOIDOSIS
SARCOIDOSIS
HAEMOCHROMATOSIS
BILATERAL ADRENAL HAEMORRHAGE- FOLLOWING MENINGOCOCCAL
SEPTICAEMIA (WATERHOUSE- FRIEDRICHSON SYNDROME), TRAUMA
LYMPHOMA
PATHOPHYSIOLOGICAL
CHANGES

PRIMARY ADRENOCORTICAL INSUFFICIENCY
(ADDISON'S DIS-EASE) - DESTRUCTION AND
SUBSEQUENT HYPOFUNCTION OF THE ADRENAL
CORTEX, USUALLY CAUSED BY AUTOIMMUNE
PROCESS.
SECONDARY ADRENOCORTICAL INSUFFICIENCY -
ACTH DEFICIENCY FROM PITUITARY DISEASE OR
SUPPRESSION OF HYPOTHALAMIC-PITUITARY AXIS BY
CORTICOSTEROID TREATMENT FOR NONENDOCRINE
DISORDERS CAUSES ATROPHY OF ADRENAL CORTEX.

INADEQUATE ALDOSTERONE PRODUCES
DISTURBANCES OF SODIUM, POTASSIUM, AND
WATER METABOLISM.
CORTISOL DEFICIENCY PRODUCES ABNORMAL FAT,
PROTEIN, AND CARBOHYDRATE METABOLISM; NO
CORTISOL DURING A PERIOD OF STRESS CAN
PRECIPITATE ADDISONIAN CRISIS, AN EXAGGERATED
STATE OF ADRENAL CORTICAL INSUFFICIENCY, AND
CAN LEAD TO DEATH

CLINICAL FEATURES

DUE TO GLUCOCORTICOID INSUFFICIENCY-
WEIGHT LOSS
MALAISE
WEAKNESS
ANOREXIA
NAUSEA
VOMITING
GASTROINTESTINAL-DIARRHOEA OR CONSTIPATION
HYPOGLYCAEMIA
HYPERCALCAEMIA
DUE TO MINERALOCORTICOID INSUFFICIENCY-
HYPOTENSION
SHOCK
HYPONATRAEMIA (DEPLETIONAL)
HYPERKALAEMIA
POSTURAL HYPOTENSION


DUE TO ACTH EXCESS
PIGMENTATION:
SUN-EXPOSED AREAS
PRESSURE AREAS, E.G. ELBOWS, KNEES
PALMAR CREASES
KNUCKLES
MUCOUS MEMBRANES
CONJUNCTIVAE
RECENT SCARS
DUE TO ADRENAL ANDROGEN INSUFFICIENCY:
DECREASED BODY HAIR
LOSS OF LIBIDO, ESPECIALLY IN FEMALE
DIAGNOSTIC CRITERIA OF ADDISONS
DISEASE
TRIAD OF-
WEAKNESS OR EMACIATION (100% CASES)
PIGMENTATION (90% CASES)
HYPOTENSION
INVESTIGATION
BLOOD GLUCOSE-
LOW OR LOWER LIMIT, SPECIALLY
DURING ADDISONIAN CRISIS
ELECTROLYTES-
A)HYPONATRAEMIA.
B)HYPERKALAEMIA.
ONLY HYPONATRAEMIA IS MORE
IMPORTANT.
INVESTIGATION
RANDOM PLASMA CORTISOL LEVEL-
USUALLY LOW BUT MAY BE WITHIN NORMAL RANGE.REFUTE
THE DIAGNOSIS IF THE VALUE IS >460NMOL/L
SHORT ACTH STIMULATION TEST/TETRACOSACTIDE OR SHORT
SYNACTHEN TEST-250MICROGRAM ACTH BY I.M AT ANY TIME OF
DAY -0 AND 30MIN FOR PLASMA CORTISOL-IN ADDISONS
DISEASE PLASMA CORTISOL<460NMOL/L
LONG ACTH STIMULATION TEST-1MG DEPOT ACTH I.M DAILY
FOR 3 DAYS-PLASMA CORTISOL <700NMOL/L AT 8HRS AFTER
LAST INJECTION
TESTS TO FIND OUT CAUSES
A)CHEST X-RAY (TUBERCULOSIS)
B)PLAIN X-RAY OF ABDOMEN (TO SEE ADRENAL
CALCIFICATION IN TUBERCULOSIS).
C)ADRENAL AUTO-ANTIBODY.
D)ULTRASONOGRAPHY OR CT SCAN OF ADRENALS.
E)HIV TEST
OTHER TESTS-
PLASMA CALCIUM-HIGH
PLASMA RENIN ACTIVITY-HIGH
PLASMA ALDOSTERONE-LOW

TREATMENT
REPLACEMENT OF HORMONES
GLUCOCORTICOID
- (HYDROCORTISONE-15 MG ON WAKING AND 5
MG AT 6P.M)
- CORTISONE ACETATE: 25 MG
- PREDNISOLONE: 5 MG
- DEXAMETHASONE: 0.5 MG
MINERALOCORTICOID (FLUDROCORTISONE 0.05 TO
0.1MG DAILY)
SUPPORTIVE TREATMENT AND TREATMENT OF CAUSE
E.G. IF TB- ANTITUBERCULAR THERAPY
GENERAL ADVICE TO THE PATIENT
GOOD NUTRITION
REGULAR MEAL, HIGH CARBOHYDRATE AND
SUFFICIENT SALT
WHEN ORAL THERAPY IS NOT POSSIBLE, INJECTION
HYDROCORTISONE SHOULD BE TAKEN

COMPLICATIONS
THE COMPLICATIONS OF UNTREATED ADDISON'S
DISEASE INCLUDE CARDIOVASCULAR COLLAPSE,
COMA, AND DEATH
ADVICE TO PATIENTS ON
GLUCOCORTICOID REPLACEMENT
INTERCURRENT STRESS
E.G. FEVER, COLD, TRAUMA-DOUBLE DOSE OF HYDROCORTISONE.
DURING SURGERY
MINOR OPERATION-HYDROCORTISONE 100 MG I.M. WITH PRE-MEDICATION .
MAJOR OPERATION-HYDROCORTISONE 100 MG 6-HOURLY FOR 24 HOURS,
THEN 50 MG I.M. 6-HOURLY UNTIL READY TO TAKE TABLETS .
BRACELET AND STEROID CARD
PATIENT SHOULD ALWAYS CARRY THIS. SHOULD HAVE INFORMATION
REGARDING THE DIAGNOSIS, DOSE OF STEROID AND DOCTOR.
PRINCIPLE OF GLUCOCORTICOID THERAPY
DO NOT ADMINISTER GLUCOCORTICOIDS UNLESS
ABSOLUTELY INDICATED OR MORE CONSERVATIVE
MEASURES HAVE FAILED
KEEP DOSAGE AND DURATION OF ADMINISTRATION
TO THE MINIMUM REQUIRED FOR ADEQUATE
TREATMENT
ADVISE TO THE PT.
DIET:
-MONITOR CALORIE INTAKE TO PREVENT WEIGHT
GAIN
-DIABETIC DIET IF GLUCOSE INTOLERANT
-RESTRICT SODIUM INTAKE TO PREVENT OEDEMA
AND MINIMIZE HTN
-PROVIDE SUPPLEMENTARY POTASSIUM IF
NECESSARY
ADMINISTER GLUCOCORTICOIDS WITH MEAL TO
PREVENT ULCER. CONSIDER OMEPRAZOLE 20-40
MG/DAY

MINIMIZE LOSS OF BONE MINERAL DENSITY
-CONSIDER ADMINISTERING GONADAL
HORMONE REPLACEMENT THERAPY IN POST
MENOPAUSAL WOMAN
-ADEQUATE CALCIUM INTAKE
-ADMINISTER A MINIMUM OF 800-1000 IU/DAY
SUPPLEMENTAL VIT D
-CONSIDER ADMINISTERING BIPHOSPHONATE
ADDISONIAN CRISIS
IT IS A MEDICAL EMERGENCY DUE TO ACUTE
ADRENOCORTICAL INSUFFICIENCY
CAUSES
SUDDEN WITHDRAWAL OF STEROID(COMMONEST CAUSE, IF
PT. ON STEROID FOR LONG TIME)
FOLLOWING STRESS E.G.INTERCURRENT DISEASE,TRAUMA,
SURGERY, SEVERE INFECTION OR PROLONGED FASTING IN A
PT WITH LATENT INSUFFICIENCY
FOLLOWING SUDDEN DESTRUCTION OF PITUITARY
GLAND(PITUITARY NECROSIS)OR WHEN THYROID HORMONE
OR DRUGS WHICH INCREASE STEROID METABOLISM(E.G.
PHENYTOIN)GIVEN TO A PT WITH HYPOADRENALISM
FOLLOWING BILATERAL ADRENALECTOMY
FOLLOWING INJURY TO BOTH ADRENALS DUE TO
TRAUMA,ADRENAL VEIN THROMBOSIS,ADRENAL
HAEMORRHAGE DUE TO MENINGOCOCCAEMIA OR
ANTICOAGULANT THERAPY

CLINICAL FEATURES
NAUSEA, VOMITING, DIARRHOEA
ABDOMINAL PAIN
DIARRHOEA
MUSCLE CRAMPS
UNEXPLAINED FEVER
UNCONSCIOUSNESS
SEVERE HYPOTENSION
ELECTROLYTE ABNORMALITIES
TREATMENT
I/V HYDROCORTISONE 100 MG 6 HRLY UNTIL GI
SYMPTOMS ABATE THEN ORAL THERAPY
I/V FLUID NORMAL SALINE AND 10% DEXTROSE FOR
HYPOGLYCAEMIA
PRECIPITATING FACTORS SHOULD BE FIND OUT AND
TREATED
IMMEDIATE TREATMENT IF ADDISONIAN (ADRENAL)
CRISIS OR CIRCULATORY COLLAPSE IS IMMINENT:
I.V. SODIUM CHLORIDE SOLUTION TO REPLACE
SODIUM IONS.
HYDROCORTISONE (CORTEF).
INJECTION OF CIRCULATORY STIMULANTS, SUCH
AS ATROPINE SULFATE (ATROPINE), CALCIUM
CHLORIDE (CALCIUM), EPINEPHRINE (ADRENALIN)