Addison's Disease

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Addison’s Disease

Title

Ñ    Addison’s Disease

Definition

Ñ    Destruction of the entire adrenal cortex

Ñ    Reduced production of:

·          Glucocorticoid

·          Mineralocorticoid

·          Sex steroids

Pathogenesis

Causes

Ñ    Inflammation:

Inflammation of the adrenal gland may be:

Autoimmune

·          Sporadic

·          Polyglandular deficiency type 1:

Addison’s disease, chronic mucocutaneous candidiasis, dental enamel hypoplasia, alopecia, primary gonadal failure

·          Polyglandular deficiency type 2:

Addison’s disease, primary hypothyroidism, primary hypogonadism, insulin-dependent diabetes mellitus, pernicious anaemia, vitiligo

Inflammation of the adrenal gland may also occur in

·          Tuberculosis, sarcoidosis, fungal infections, cytomegalovirus infection, AIDS

Ñ    Trauma:

Bilateral adrenalectomy

Ñ    Neoplasia:

Secondary deposits, lymphoma

Ñ    Vascular Lesions:

Meningococcal septicaemia, adrenal haemorrhage, adrenal vein thrombosis

Ñ    Degenerative:

Adrenoleucodystrophy, adrenomyeloneuropathy

Ñ    Metabolic:

Haemochromatosis

Ñ    Interstitial Infiltration:

Amyloidosis

Pathophysiology

Ñ    Decreased physiological effects of glucocorticoid, mineralocorticoid and sex steroids

Clinical Features

History

Sex

Ñ    Females > Males

E&M

Ñ    Weight loss

Ñ    Malaise

Ñ    Weakness

Ñ    Fever

CVS

Ñ    Syncope:

Postural hypotension

GIT

Ñ    Anorexia

Ñ    Nausea and vomiting

Ñ    Abdominal pain

Ñ    Diarrhoea

RAG

Ñ    Impotence

Ñ    Amenorrhoea

CNS

Ñ    Depression

Ñ    Confusion

LMS

Ñ    Joint pains

Ñ    Back pain

Ñ    Myalgia

Examination

E&M

Ñ    Decreased weight

Ñ    Wasting

IS

Ñ    Buccal pigmentation

Ñ    Pigmentation of palmar creases, scars

Ñ    Vitiligo:

Autoimmune

Ñ    Loss of body hair

Ñ    Decreased skin turgor:

Salt and water loss

CVS

Ñ    Postural hypotension

Investigations

Fluids

Blood

Biochemistry

Ñ    Hyponatraemia

Ñ    Hyperkalaemia

Ñ    Blood urea:

Increased

Ñ    Blood glucose low

Ñ    Cortisol levels low

Ñ    Short synacthen test:

Impaired response

Ñ    0900 ACTH level:

Increased

Ñ    Serum aldosterone:

Decreased with increased plasma renin activity

Immunology

Ñ    Adrenal antibodies:

Positive in autoimmune adrenalitis

Haematology

Ñ    Anaemia

Ñ    Lymphocytosis

Ñ    Eosinophil count:

Increased

Imaging

Chest x-ray

Ñ    Tuberculosis

Abdominal X-ray

Ñ    Calcified adrenals

CT scan

Ñ    Tuberculosis

Ñ    Adrenal infiltration

Management

Support

E&M

Drugs

Ñ    Glucocorticoid:

Hydrocortisone, prednisolone, dexamethasone

Ñ    Mineralocorticoid:

Fludrocortisone

 

 

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