Addison’s disease

Published on 02/04/2015 by admin

Filed under Internal Medicine

Last modified 22/04/2025

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144 Addison’s disease

Instruction

Look at this patient, who presented with weakness, loss of appetite and weight loss.

Salient features

History

• Dizziness, syncope

• Skin pigmentation (ask if the patient has been sitting in the sun)

• Ask questions regarding fatigue, weakness, apathy, anorexia, nausea, vomiting, weight loss and abdominal pain

• Depression.

Examination

• The striking abnormality is hyperpigmentation.

Proceed as follows:

• Examine for hyperpigmentation, typically localized to palmar creases and knuckles:

• Hand: compare the creases with your own
• Mouth and lips for pigmentation
• Areas not usually covered by clothing: nipples, areas irritated by belts, straps, collars or rings
• Look for vitiligo.

• Tell the examiner that you would like to investigate as follows:

• BP, in particular for postural hypotension
• Looking for sparse axillary and pubic hair
• The abdomen for adrenal scar (if the scar is pigmented, think of Nelson syndrome and examine field defects).

• If you suspect Addison’s disease, tell the examiner that you would like to do a short tetracosactrin test (synacthen test).

Diagnosis

This patient has postural hypotension, marked hyperpigmentation and sparse axillary hair (lesion) caused by autoimmune Addison’s disease (aetiology). The patient has marked hypoadrenalism (functional status).

Questions

What is the size of the heart in Addison’s deficiency?

The heart is small.

Mention some causes of hyperpigmentation

• Suntan

• Race

• Uraemia

• Haemochromatosis

• Primary biliary cirrhosis

• Ectopic ACTH

• Porphyria cutanea tarda

• Nelson syndrome

• Malabsorption syndromes.

Mention some causes of Addison’s disease

• 80% idiopathic

• TB

• Metastasis

• HIV infection.

Which conditions may be associated with Addison’s disease?

• Graves’ disease

• Hashimato’s thyroiditis

• Primary ovarian failure

• Pernicious anaemia

• Polyglandular syndromes.

Advanced-level questions

What are the components of Schmidt syndrome?

Addison’s disease and hypoparathyroidism. (In these patients cortisol should be administered before thyroxine to avoid exacerbating the hypoadrenalism.)

What do you know about polyglandular syndromes?

They are autoimmune in nature and are of two types:

I: chronic mucocutaneous candidiasis, hypoparathyroidism and Addison’s disease

II: Addison’s disease, insulin-dependent diabetes and thyroid disease (hyperthyroidism or hypothyroidism); syndrome is also known as ‘autoimmune polyendocrinopathy–candidiasis–ectodermal dysplasia’.

What are the features of Allgrove syndrome?

Adrenal insensitivity to ACTH (resulting in cortisol deficiency), achalasia, alacrima and neurological disease.

How would you investigate this patient?

• FBC (for lymphocytosis, eosinophilia)

• Electrolytes (for hyponatraemia, hyperkalaemia, hyperchloraemic acidosis, hypercalcaemia)

• Blood glucose, looking for hypoglycaemia

• Short tetracosactrin (synacthen) test; if positive, follow-up with a prolonged ACTH stimulation test

• ACTH and cortisol levels: in Addison’s disease the 9 am ACTH is elevated (>300 ng/l)

• Adrenal autoantibodies: 21-hydroxylase autoantibodies are elevated in 80%

• Chest radiography for TB

• Plain radiograph of the abdomen for adrenal calcification

• CT scan of the adrenals.

How would you manage this patient if the underlying aetiology is autoimmune?

• Immediate treatment of adrenal failure with replacement steroids: prednisolone 5 mg in the morning and 2.5 mg at night; adjust dose depending on serum levels and clinical well-being

• Fludrocortisone 0.025–0.15 mg daily; adjust dose depending on postural hypotension

• Give steroid card and Medic Alert bracelet

• Stress the importance of regular therapy and increase the dose in the event of stress such as dental extraction or urinary tract infection. It is also important to tell the patient that this therapy is lifelong and that an ampoule of hydrocortisone should be kept at home

• Follow up every 6 months.

Note: In Addisonian crisis, intravenous fluids and hydrocortisone should be administered (after drawing a blood sample for cortisol determination).

Thomas Addison (1793–1860) qualified from Edinburgh and worked at Guy’s Hospital, London. He wrote ‘The constitutional and local effects of the disease of the suprarenal capsules’. He was one of the three ‘Giants of Guy’s Hospital’ and all three studied in Edinburgh. The others were Richard Bright (1789–1858) and Thomas Hodgkin (1798–1866).

Armand Trosseau in Paris labelled the disease ‘maladie d’Addison’. Addison also first described morphoea. The original description of the disease was reported in the following paper: Addison T. Disease of the suprarenal capsules. London Med Gaz 1855;43:517.

John F Kennedy, past president of the USA, reportedly had Addison’s disease and was on replacement corticosteroid therapy.

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