About acromegaly

A brief introduction to the rare condition, its effects and implications

Pathology
Acromegaly is a chronic disease of adulthood resulting from excessive secretion of growth hormone (GH) and the subsequent elevation of insulin-like growth factor-I (IGF-I).1 It is usually caused by a benign tumour in the anterior pituitary gland.2

 

Prevalence
Acromegaly is equally prevalent in men and women, with a worldwide estimate of 60 cases per million,3 although more recent studies have estimated this number to be higher,4 and an annual incidence of 3-4 cases per million.

 

Mortality
The mean age of diagnosis is 41–48 years,5 and diagnosis takes an average of 8 years from onset of symptoms.6 If left untreated or unsuccessfully treated, acromegaly is associated with increased mortality and risk of metabolic and cardiovascular complications;1 the mortality rate for patients with uncontrolled acromegaly is approximately 23 times higher than that of the general population.7

 

Signs and symptoms
Acromegaly affects virtually all organs and tissues, causing metabolic, cardiovascular, neurological and musculoskeletal morbidities.7 The clinical signs can result from chronic exposure to the effects of GH and IGF-I and/or a secondary effect of the mass of the tumour.8

 

 

 

References:

  1. Aron DC, Findling JW, Tyrrell JB. Hypothalamus and pituitary. In: Greenspan FS, Gardner DG., editors. Basic and clinical endocrinology. 6th ed. Lange Medical Books/ McGraw-Hill Medical Publishing Division; 2001. p. 100-162.
  2. Melmed S, Kleinberg D. Anterior pituitary. In: Larsen PR, Kronenberg HM, Melmed S et al., editors. Williams textbook of endocrinology. 10th ed. Philadelphia, Pennsylvania: Saunders (Elsevier); 2003. p. 180-238.
  3. Freda PU. Acromegaly diagnostic pitfalls. The Endocrinologist. 2004; 14(5):277-287.
  4. Schneider HJ, Sievers C, Saller B, et al. High prevalence of biochemical acromegaly in primary care patients with elevated IGF-1 levels. Clin Endocrinol (Oxf) 2008; 69:432.
  5. Mestrón A, Webb SM, Astorga R et al. Epidemiology, clinical characteristics, outcome, morbidity and mortality in acromegaly based on the Spanish Acromegaly Registry (Registro Español de Acromegalia, REA). Eur J Endocrinol. 2004; 151:439-446.
  6. Melmed S. Medical progress: acromegaly. N Engl J Med. 2006;355:2558-73
  7. Clemmons DR, Chihara K, Freda PU et al. Optimizing control of acromegaly: integrating a growth hormone receptor antagonist into the treatment algorithm. J Clin Endocrinol Metab. 2003; 88(10):4759-4767.
  8. Melmed S, Colao A, Barkan A et al. Guidelines for acromegaly management: an update. J Clin Endocrinol Metab. 2009; 94(5):1509-1517.

PP-SOM-GBR-0519. May 2020.

Presentation
Most patients have unrecognised signs and symptoms for many years and may be diagnosed through a dental examination or testing done for other reasons.1Although their outward appearance may have altered, it is often the symptoms of complications that lead patients to seek medical care.

 

Diagnostic blood tests
The diagnosis can be confirmed with an Oral Glucose Tolerance Test (OGTT) or measurement of serum IGF-I levels (age and sex-adjusted). Although both growth hormone (GH) and IGF-I levels have been implicated as indicators of mortality, IGF-I is considered to be indicative of persistent clinical disease activity.2,3

 

Imaging
Imaging techniques such as computed tomography (CT) or magnetic resonance imaging (MRI) are used to determine the cause of GH overproduction and provide further confirmation of the acromegaly diagnosis.4

 

 

 

References:

  1. Freda PU. Acromegaly diagnostic pitfalls. The Endocrinologist. 2004; 14(5):277-287.
  2. Alexopoulou O, Bex M, Abs R et al. Divergence between growth hormone and insulin-like growth factor-I concentrations in the follow-up of acromegaly. J Clin Endocrinol Metab. 2008; 93(4):1324-1330.
  3. Melmed S, et al. A Consensus Statement on acromegaly therapeutic outcomes. Nat Rev Endocrinol 2018;26:1.
  4. Ilie I, et al. Diagnosis of AcromegalyEncyclopedia of Endocrine Diseases, Second Edition Vol 2 (Elsevier). 2018 p223-229.

 PP-SOM-GBR-0519. May 2020.

Typical characteristics of acromegaly1,2 

Local tumour effects

Visual impairment, headaches, pituitary infarction/destruction, optic/cranial nerve impingement

General

Soft-tissue swelling, fatigue, enlarged organs

Cutaneous and soft tissue

Enlarged hands, ‘spade-like’ fingers, feet, nose, coarsening of facial features, thick oily skin, increased sweating and skin odour, sweaty handshake, skin tags, increased heel pad thickness, thick lips and skin folds

Endocrine/metabolic

Carbohydrate intolerance, diabetes mellitus, hyperlipidaemia, goitre, hyperprolactinaemia, galactorrhoea

Cardiovascular

Congestive heart failure, hypertension, cardiomyopathy, left ventricular hypertrophy

Respiratory

Sleep apnoea, tongue enlargement, voice change

Gastrointestinal

Colonic polyps, diverticulosis

Neurological

Paraesthesia, somnolence, carpal tunnel syndrome

Musculoskeletal

Malocclusion, widely spaced teeth, prognathismfrontal bossing, enlarged sinuses and larynx, weakness, joint pains (shoulders, back, knees), osteoarthritis, degenerative arthritis of the spine, hips and knees

Psychosexual

Decreased libido, impotence, menstrual disturbance, depression, mania

 

 

 

References:

  1. Aron DC, Findling JW, Tyrrell JB. Hypothalamus and pituitary. In: Greenspan FS, Gardner DG., editors. Basic and clinical endocrinology. 6th ed. Lange Medical Books/ McGraw-Hill Medical Publishing Division; 2001. p. 100-162.
  2. Melmed S, Kleinberg D. Anterior pituitary. In: Larsen PR, Kronenberg HM, Melmed S et al., editors. Williams textbook of endocrinology. 10th ed. Philadelphia, Pennsylvania: Saunders (Elsevier); 2003. p. 180-238.

PP-SOM-GBR-0519. May 2020.

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