Smarter Decisions,
Better Care

UpToDate synthesizes the most recent medical information into evidence-based practical recommendations clinicians trust to make the right point-of-care decisions.

  • Rigorous editorial process: Evidence-based treatment recommendations
  • World-Renowned physician authors: over 5,100 physician authors and editors around the globe
  • Innovative technology: integrates into the workflow; access from EMRs

Choose from the list below to learn more about subscriptions for a:


Subscribers log in here


Diagnosis of acromegaly

INTRODUCTION

Acromegaly results from persistent hypersecretion of growth hormone (GH). Excess GH stimulates hepatic secretion of insulin-like growth factor-1 (IGF-1), which causes most of the clinical manifestations of acromegaly.

The clinical diagnosis is often delayed because of the slow progression of the signs of acromegaly over a period of many years. Growth hormone excess that occurs before fusion of the epiphyseal growth plates in a child or adolescent is called pituitary gigantism and is discussed separately.

The diagnostic approach to acromegaly will be reviewed here. Growth hormone excess in children and adolescents and the causes, clinical manifestations, and treatment of acromegaly are discussed separately. (See "Pituitary gigantism" and "Causes and clinical manifestations of acromegaly" and "Treatment of acromegaly".)

EPIDEMIOLOGY

Acromegaly has been considered to be a rare disease, with an estimated prevalence in Europe of 30 to 70 individuals per million [1,2]. However, current estimates are considerably higher [2-4]. In one report, seven new cases of acromegaly were identified among 6773 unselected adults in a primary care population who underwent plasma insulin-like growth factor-1 (IGF-1) screening; this suggests a population prevalence as high as 1000 per million individuals [5]. A similar uncontrolled IGF-1 screening study in over 2000 individuals with type 2 diabetes found a prevalence of approximately 480 per million individuals [6].

WHEN TO SUSPECT ACROMEGALY

The diagnosis of acromegaly should be suspected in individuals who present with the typical clinical features of growth hormone excess, which include the enlargement during adulthood of the jaw (macrognathia), hands, and feet, which result in increasing shoe and glove size and the need to enlarge finger rings. The facial features become coarse, with enlargement of the nose and frontal bones as well as the jaw, and the upper incisors may become spread apart. Despite the prominence of these findings at the time of diagnosis, the rate of change is so slow that few patients seek care because their appearance had changed. Other features include cardiovascular disease, sleep apnea, type 2 diabetes, arthropathies, and problems directly related to the pituitary tumor size (headache, visual loss).

            

Subscribers log in here

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information or to purchase a personal subscription, click below on the option that best describes you:
Literature review current through: Aug 2014. | This topic last updated: Jan 29, 2014.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2014 UpToDate, Inc.
References
Top
  1. Bengtsson BA, Edén S, Ernest I, et al. Epidemiology and long-term survival in acromegaly. A study of 166 cases diagnosed between 1955 and 1984. Acta Med Scand 1988; 223:327.
  2. Ribeiro-Oliveira A Jr, Barkan A. The changing face of acromegaly--advances in diagnosis and treatment. Nat Rev Endocrinol 2012; 8:605.
  3. Fernandez A, Karavitaki N, Wass JA. Prevalence of pituitary adenomas: a community-based, cross-sectional study in Banbury (Oxfordshire, UK). Clin Endocrinol (Oxf) 2010; 72:377.
  4. Daly AF, Rixhon M, Adam C, et al. High prevalence of pituitary adenomas: a cross-sectional study in the province of Liege, Belgium. J Clin Endocrinol Metab 2006; 91:4769.
  5. 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.
  6. Rosario PW. Frequency of acromegaly in adults with diabetes or glucose intolerance and estimated prevalence in the general population. Pituitary 2011; 14:217.
  7. Melmed S. Medical progress: Acromegaly. N Engl J Med 2006; 355:2558.
  8. Clemmons DR, Van Wyk JJ, Ridgway EC, et al. Evaluation of acromegaly by radioimmunoassay of somatomedin-C. N Engl J Med 1979; 301:1138.
  9. Stoffel-Wagner B, Springer W, Bidlingmaier F, Klingmüller D. A comparison of different methods for diagnosing acromegaly. Clin Endocrinol (Oxf) 1997; 46:531.
  10. Clemmons DR. Clinical utility of measurements of insulin-like growth factor 1. Nat Clin Pract Endocrinol Metab 2006; 2:436.
  11. Iranmanesh A, Grisso B, Veldhuis JD. Low basal and persistent pulsatile growth hormone secretion are revealed in normal and hyposomatotropic men studied with a new ultrasensitive chemiluminescence assay. J Clin Endocrinol Metab 1994; 78:526.
  12. Chapman IM, Hartman ML, Straume M, et al. Enhanced sensitivity growth hormone (GH) chemiluminescence assay reveals lower postglucose nadir GH concentrations in men than women. J Clin Endocrinol Metab 1994; 78:1312.
  13. Costa AC, Rossi A, Martinelli CE Jr, et al. Assessment of disease activity in treated acromegalic patients using a sensitive GH assay: should we achieve strict normal GH levels for a biochemical cure? J Clin Endocrinol Metab 2002; 87:3142.
  14. Jaffe CA, Pan W, Brown MB, et al. Regulation of GH secretion in acromegaly: reproducibility of daily GH profiles and attenuated negative feedback by IGF-I. J Clin Endocrinol Metab 2001; 86:4364.
  15. Carmichael JD, Bonert VS, Mirocha JM, Melmed S. The utility of oral glucose tolerance testing for diagnosis and assessment of treatment outcomes in 166 patients with acromegaly. J Clin Endocrinol Metab 2009; 94:523.
  16. Freda PU, Post KD, Powell JS, Wardlaw SL. Evaluation of disease status with sensitive measures of growth hormone secretion in 60 postoperative patients with acromegaly. J Clin Endocrinol Metab 1998; 83:3808.
  17. Irie M, Tsushima T. Increase of serum growth hormone concentration following thyrotropin-releasing hormone injection in patients with acromegaly or gigantism. J Clin Endocrinol Metab 1972; 35:97.
  18. Freda PU. Current concepts in the biochemical assessment of the patient with acromegaly. Growth Horm IGF Res 2003; 13:171.
  19. Grinspoon S, Clemmons D, Swearingen B, Klibanski A. Serum insulin-like growth factor-binding protein-3 levels in the diagnosis of acromegaly. J Clin Endocrinol Metab 1995; 80:927.
  20. Melmed S, Colao A, Barkan A, et al. Guidelines for acromegaly management: an update. J Clin Endocrinol Metab 2009; 94:1509.
  21. Famini P, Maya MM, Melmed S. Pituitary magnetic resonance imaging for sellar and parasellar masses: ten-year experience in 2598 patients. J Clin Endocrinol Metab 2011; 96:1633.
  22. Garby L, Caron P, Claustrat F, et al. Clinical characteristics and outcome of acromegaly induced by ectopic secretion of growth hormone-releasing hormone (GHRH): a French nationwide series of 21 cases. J Clin Endocrinol Metab 2012; 97:2093.