Phys · endocrine
Pituitary Disease
Also known as pituitary adenoma · prolactinoma · macroprolactinoma · microprolactinoma · acromegaly · gigantism · Cushing disease · non-functioning pituitary adenoma · hypopituitarism · panhypopituitarism · pituitary apoplexy · Sheehan syndrome · craniopharyngioma · diabetes insipidus · central diabetes insipidus · growth hormone deficiency
Consultant-physician-depth guide to pituitary disease — anatomy and physiology, pituitary adenoma classification, prolactinoma, acromegaly, Cushing disease, non-functioning adenoma, hypopituitarism, pituitary apoplexy, craniopharyngioma, and diabetes insipidus — structured for FRACP DWE and DCE preparation.
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Red flags
- Pituitary apoplexy — sudden severe headache with visual loss and hypopituitarism; give emergency hydrocortisone before thyroid
- Bitemporal hemianopia — optic chiasm compression by macroadenoma; urgent MRI and surgical referral
- Adrenal crisis from ACTH deficiency — hypotension, hyponatraemia, hypoglycaemia; never give levothyroxine without cortisol first
- Severe hyperprolactinaemia above 5000 mU/L suggests macroprolactinoma — image with MRI
- Acromegaly with cardiomyopathy or sleep apnoea — increased mortality from cardiovascular disease
- Cushing disease with severe hypokalaemia or psychosis — high cortisol output requiring urgent control
Pituitary Disease
Meet the patient
Meet a 52-year-old man referred for enlarging hands, headaches, and newly diagnosed type 2 diabetes and hypertension. He snores loudly and is sleepy by day. He has coarse facial features, frontal bossing, prognathism with interdental spaces, large hands and feet, a carpal-tunnel release scar, and skin tags. IGF-1 is three times the upper limit of normal and his oral-glucose-tolerance-test growth-hormone nadir fails to suppress. This is acromegaly — and the diagnosis has been delayed for years.[1]
The single rule that runs through every pituitary question is cortisol before thyroid: never give levothyroxine to a patient with untreated ACTH deficiency, because it accelerates cortisol clearance and precipitates adrenal crisis. Hold that rule and the rest of pituitary medicine falls into place.[1]
References10ShowHide
- [1]Katznelson L, Laws ER, Melmed S, et al. Acromegaly: an endocrine society clinical practice guideline J Clin Endocrinol Metab, 2014.PMID 25356808
- [2]Melmed S, Casanueva FF, Hoffman AR, et al. Diagnosis and treatment of hyperprolactinemia: an Endocrine Society clinical practice guideline J Clin Endocrinol Metab, 2011.PMID 21296991
- [3]Rajasekaran S, Vanderpump M, Baldeweg S, et al. UK guidelines for the management of pituitary apoplexy Clin Endocrinol (Oxf), 2011.PMID 21044119
- [4]Nieman LK, Biller BMK, Findling JW, et al. The diagnosis of Cushing's syndrome: an Endocrine Society Clinical Practice Guideline J Clin Endocrinol Metab, 2008.PMID 18334580
- [5]Gadelha MR, Bronstein MD, Brue T, et al. Pasireotide versus continued treatment with octreotide or lanreotide in patients with inadequately controlled acromegaly (PAOLA): a randomised, phase 3 trial Lancet Diabetes Endocrinol, 2014.PMID 25260838
- [6]Trainer PJ, Drake WM, Katznelson L, et al. Treatment of acromegaly with the growth hormone-receptor antagonist pegvisomant N Engl J Med, 2000.PMID 10770982
- [7]Tomlinson JW, Holden N, Hills RK, et al. Association between premature mortality and hypopituitarism. West Midlands Prospective Hypopituitary Study Group Lancet, 2001.PMID 11273062
- [8]Pappachan JM, Raskauskiene D, Kutty VR, Clayton RN Excess mortality associated with hypopituitarism in adults: a meta-analysis of observational studies J Clin Endocrinol Metab, 2015.PMID 25658016
- [9]Refardt J, Winzeler B, Christ-Crain M, et al. A Copeptin-Based Approach in the Diagnosis of Diabetes Insipidus N Engl J Med, 2018.PMID 30067922
- [10]Jasim S, Alahdab F, Ahmed AT, et al. Mortality in adults with hypopituitarism: a systematic review and meta-analysis Endocrine, 2017.PMID 27817141