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Ovid: Oxford Handbook of Acute Medicine

Editors: Ramrakha, Punit S.; Moore, Kevin P. Title: Oxford Handbook of Acute Medicine, 2nd Edition Copyright ©1997,2004 Oxford University Press (Copyright 1997, 2004 by Punit S Ramrakha and Kevin P Moore) > Table of Contents > Chapter 9 – Endocrine emergencies > Addisonian crisis: assessment Addisonian crisis: assessment Adrenocortical insufficiency may be sub-clinical for days or months in otherwise well individuals. Stress, such as infection, trauma, or surgery, may precipitate an Addisonian crisis with cardiovascular collapse and death if the condition is not suspected. Crises may also occur in patients with known Addison’s disease on replacement hydrocortisone if they fail to increase their steroid dose with infections. Presentation

  • Hypotension and cardiovascular collapse (shock)
  • Faintness, particularly on standing (postural hypotension)
  • Anorexia, nausea, vomiting, and abdominal pain
  • Hyponatraemia
  • Dehydration (thirst may not be apparent because of the low sodium)
  • Diarrhoea in 20% of cases
  • Symptoms of precipitant [fever, night sweats (infection); flank pain (haemorrhagic adrenal infarction); etc]. Note signs/symptoms of other endocrinopathies.
  • Non-specific symptoms: weight loss, fatigue, weakness, myalgia.
  • Hyperpigmentation suggests chronic hypoadrenalism.
  • Psychiatric features are common and include asthenia, depression, apathy, and confusion (treatment with glucocorticoids reverses most psychiatric features).

Malignant secondaries Present in the adrenals of a high percentage of patients with lung cancer, breast tumours, and malignant melanomas. Adrenal failure will only occur when over 90% of the gland is replaced by metastases. Adrenal haemorrhage This may complicate sepsis (meningococcal septicaemia, the Waterhouse–Friderichsen syndrome), traumatic shock, coagulopathies, and ischaemic disorders.

  • Severe stress substantially increases the arterial blood supply to the adrenals. However the adrenal gland has only one or two veins, making it vulnerable to venous thrombosis.
  • Blood tests: a precipitous drop in haemoglobin, hyponatraemia, hyperkalaemia, acidosis, uraemia, and neutrophilia.
  • The Waterhouse–Friderichsen syndrome is the association of bilateral adrenal haemorrhage with fulminant meningococcaemia. Adrenal haemorrhage is also seen with other gram-negative endotoxaemias such as Diplococcus pneumoniae, Haemophilus influenzae B and DF-2 bacillus infections.

Hypopituitarism As there is no mineralocorticoid deficiency, the salt and water loss and shock are less profound than in primary Addison’s disease. Drugs Rifampicin, phenytoin, and phenobarbitone accelerate the metabolism of cortisol and may precipitate Addisonian crisis in partially compromised individuals, or in those on a fixed replacement dose. Most adrenal crises precipitated by rifampicin occur within 2 weeks of initiating therapy. P.585
Recognized causes of adrenal failure

  • Autoimmune adrenalitis (70%)
  • Tuberculosis of the adrenals (10–20%)
  • Malignant secondaries in the adrenal glands
  • Adrenal haemorrhage incl. meningococcal septicaemia
  • Diseminated fungal infection (histoplasmosis, paracoccidioidomycosis)
  • Hypopituitarism
  • Drugs: metyrapone or aminoglutethimide can precipitate adrenal failure. Other drugs (see below) may cause relative adrenal insufficiency
  • Congenital conditions
    • Adrenoleukodystrophy
    • Congenital adrenal hyperplasia
    • Familial glucorticoid deficiency

Causes of relative adrenal insufficiency

  • Drugs
    • Metyrapone or aminoglutethimide
    • Ketoconazole
    • Etomidate
    • Rifampicin, phenytoin, and phenobarbitone
    • Trilostane
    • Megestrol acetate
    • Suramin
  • HIV
  • Severe sepsis
  • Burns
  • Acute or chronic liver failure

Practice points

  • ~50% of patients with autoimmune adrenalitis have one or more other autoimmune disorders such as polyglandular autoimmune syndrome type 1 or 2.
  • Never forget Addison’s disease in a sick patient when the diagnosis is unclear.

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