Addison

History Taking

Chief Complaint:

• Fatigue or general malaise

• Low blood pressure or episodes of dizziness upon standing

• Unexplained weight loss

• Persistent abdominal pain

History of Presenting Complaint:

• Progressive worsening of fatigue over weeks or months

• Episodes of dizziness and faintness especially upon standing, getting worse over time

• Unexpected weight loss over a period of months without changes in diet or exercise

• Chronic, worsening abdominal pain that doesn't correlate with food intake or bowel movements

• Changes in skin color, especially noticeable in scars, creases of the hands, elbows, and knees, lips and inner side of cheeks

System Review:

• Gastrointestinal: Nausea, vomiting, diarrhea, anorexia

• Cardiovascular: Postural hypotension

• Neurological: General weakness, lethargy, depression

• Dermatological: Hyperpigmentation, skin changes

Past Medical History:

• History of autoimmune diseases

• History of recurrent infections

• History of tuberculosis

Drug History:

• Previous or current use of corticosteroids, with recent discontinuation

• Medications that could interfere with adrenal function, such as ketoconazole or etomidate

Family History:

• Relatives with autoimmune diseases

• Relatives with Addison's disease or other endocrine disorders

Personal History:

• Increased salt cravings

• Stressful life events or trauma

Social History:

• Occupational exposure to tuberculosis or fungal infections

• Lifestyle factors such as diet and exercise habits which may be altered due to disease symptoms (such as reduced exercise capacity due to fatigue)

Travel History:

• Travel to regions with high prevalence of infections that can affect the adrenal glands (such as tuberculosis or certain fungal diseases)

Vaccine History:

• Lack of vaccination or irregular vaccination for tuberculosis

OBG History:

• Women may present with amenorrhea or irregular menstrual cycles due to the hormonal imbalances caused by Addison's disease.

Physical Examination

General Examination:

• Pallor or noticeable hyperpigmentation of the skin, particularly in sun-exposed areas, creases of the hands, elbows, knees, scars, and mucous membranes

• Thin or frail appearance due to weight loss

• Signs of dehydration such as dry mucous membranes

Hands and Arms:

• Hyperpigmentation visible in the creases of the hands, knuckles, and nails

• Possible thinning of the skin on the arms

Mouth:

• Pigmented spots on the buccal mucosa, gums or tongue

• Signs of dehydration such as a dry mouth or tongue

Neck:

• Absence of goiter, unless there is coexistent autoimmune thyroid disease

Cardiovascular Examination:

• Low blood pressure, particularly a drop in blood pressure upon standing (postural hypotension)

• Possibly a slow heart rate (bradycardia)

Respiratory Examination:

• No specific changes attributable to Addison's disease

Abdominal Examination:

• Non-specific tenderness in the abdomen due to adrenal crisis

• Absence of hepatosplenomegaly, unless there is concurrent infection or malignancy

Neurological Examination:

• Normal neurological examination unless the patient is in adrenal crisis, which might present with confusion or coma

Additional Examination:

• Orthostatic hypotension assessment: measure blood pressure while patient is lying down and then upon standing; a significant drop in blood pressure can indicate Addison's disease

• Check RBS to exclude hypoglycemia

• Vitiligo rash for associated autoimmune disease

Investigations

Laboratory Investigations:

• Basal cortisol and ACTH levels: Low cortisol and high ACTH levels suggest primary adrenal insufficiency (Addison's disease)

• Synacthen (ACTH) stimulation test: Lack of adequate cortisol response indicates adrenal insufficiency

• Electrolytes: Hyponatraemia, hyperkalaemia, and hypoglycaemia may be present due to aldosterone deficiency

• Renal function tests: Urea and creatinine may be elevated due to dehydration

• Full blood count: May show normocytic normochromic anaemia and relative lymphocytosis

• Autoimmune profile: Positive anti-adrenal or anti-21 hydroxylase antibodies can suggest an autoimmune cause

• Adrenal and gonadal steroid profile: Reduced levels of adrenal androgens (DHEA and androstenedione) can be seen in Addison's disease

Imaging:

• Adrenal CT or MRI: Can help visualize adrenal glands to identify atrophy, calcification, or infiltrative diseases

Invasive Investigations:

• Adrenal biopsy: Rarely performed but can help identify granulomatous diseases or malignancies if imaging is suggestive

Other Tests:

• ECG: May show low voltage QRS complexes or prolonged PR intervals due to hyperkalemia

• Insulin-induced hypoglycemia test: In secondary adrenal insufficiency, this can help differentiate between pituitary and hypothalamic causes

• Tuberculin skin test or Interferon Gamma Release Assay (IGRA): If tuberculosis is suspected as a cause

• ACTH stimulation test with CRH: To differentiate between primary and secondary adrenal insufficiency

Management

General Management:

• Education about disease: Importance of medication compliance, potential triggers for adrenal crises, and when to seek urgent medical attention

• Lifestyle modifications: Balanced diet with adequate sodium intake, regular exercise, and stress management

Medical Management:

• Glucocorticoid replacement: Hydrocortisone or prednisolone to replace cortisol

• Mineralocorticoid replacement: Fludrocortisone to replace aldosterone, if needed

• Management of precipitating factors: Treat any infections, adjust medication doses during times of stress (e.g., surgery, illness)

• Patient should carry an emergency steroid card and consider wearing a medical alert bracelet

Surgical Management:

• Surgery is not typically indicated for Addison's disease itself, but may be necessary for removal of adrenal tumors or tuberculosis granulomas, if present

Other:

• Regular follow-up: Monitor symptoms, blood pressure, electrolyte levels, and adjust medication doses as necessary

• Urgent management during adrenal crisis: Intravenous hydrocortisone, fluids, correction of hypoglycemia and electrolyte imbalances

• Vaccinations: Annual influenza and other routine vaccinations as the patient is immunocompromised

• Psychosocial support: Address psychological impact of chronic disease, provide resources for support groups if necessary