Acromegaly

Overview 
Theory 
Diagnosis 
Management 
Follow up 
Resources 

Last reviewed: 30 Sep 2024

Last updated: 09 Jan 2024

Acromegaly is a rare, chronic disease caused by excessive secretion of growth hormone (GH), usually due to a pituitary somatotroph adenoma. It is associated with increased morbidity and premature mortality if not appropriately treated.

The diagnosis is often delayed. Early recognition and appropriate treatment are crucial for reducing the potentially debilitating complications of the disease.

Must be screened for in the presence of pituitary adenoma, profuse sweating, acral growth, coarsening of facial features, and when suspected in conjunction with commonly associated conditions, such as carpal tunnel syndrome, arthralgia, glucose intolerance or diabetes mellitus, amenorrhea, hypertension, and sleep apnea.

Modern surgical and pharmacologic modalities are associated with improved outcomes. Normalization of plasma insulin-like growth factor 1 (IGF-1) and a decrease of plasma GH to below 1 microgram/L bring the mortality rate to normal.

Monitoring and treatment of the comorbidities associated with acromegaly are essential for improving patient quality of life.

Definition

The term acromegaly is derived from the Greek akros (meaning extremity) and megas (large). It refers to the characteristic growth of extremities, which describes one aspect of the disease. Acromegaly is a chronic, progressive, multisystemic disease associated with significant morbidity and increased mortality. It is caused by excessive secretion of growth hormone, usually due to a pituitary somatotroph adenoma. Gigantism occurs with disease onset in childhood (prior to epiphyseal closure).

History and exam

Key diagnostic factors

coarsening of facial features
soft-tissue and skin changes
carpal tunnel syndrome
joint pain and dysfunction
snoring
alterations in sexual functioning
history or family history of inherited syndrome

Full details

Other diagnostic factors

fatigue
hypertension, arrhythmias
organomegaly
increased appetite, polyuria/polydipsia
headaches
visual field defects
signs and symptoms of hypopituitarism
cranial nerve palsies (e.g., ophthalmoplegia)

Full details

Risk factors

GPR101 overexpression
multiple endocrine neoplasia type 1 syndrome
isolated familial acromegaly
McCune-Albright syndrome
Carney complex

Full details

Diagnostic tests

1st tests to order

serum insulin-like growth factor 1 (IGF-1)
oral glucose tolerance test (OGTT)
random serum growth hormone (GH)
pituitary MRI or CT scan

Full details

Tests to consider

GH-releasing hormone
chest and/or abdominal CT scanning
total body scintigraphy with radiolabeled somatostatin analog (octreoscan)
PET scan with radiolabeled somatostatin analog (Gallium-68 DOTATATE)
plasma cortisol
prolactin
thyroid-stimulating hormone (TSH) and free thyroxine
estradiol or testosterone
visual field testing

Full details

Treatment algorithm

ACUTE

enclosed pituitary tumor

unresectable pituitary tumor (with neural or vascular impingement/invasion)

nonpituitary adenoma etiology

ONGOING

pituitary adenoma progression or recurrence

Contributors

Authors

Maria Fleseriu, MD, FACE

Professor of Medicine (Endocrinology) and Neurological Surgery

Director

Pituitary Center

Oregon Health & Science University

Portland

Disclosures

MF declares that she receives grants to her institution from Sparrow, Xeris (formerly Strongbridge), and Crinetics; personal honoraria for consulting and advisory boards from Sparrow, Xeris, HRA Pharma, and Recordati. MF is an author of a number of references cited in this topic and has served on the Board of the Pituitary Society.

Acknowledgements

Professor Fleseriu would like to gratefully acknowledge Professor Ariel Barkan, Dr Omar Serri and Dr Sophie Vallette, previous contributors to this topic.

Disclosures

AB has received lecturing fees and research support from Novartis and Ipsen and is an author of several references cited in this topic. OS has received lecturing and educational program fees from Novartis Canada and is an author of a reference cited in this topic. SV has received fees for lecturing and attending a symposium and is an author of a reference cited in this topic.

Peer reviewers

Robert D. Murray, MBBS, BSc, FRCP, MD

Consultant Endocrinologist & Honorary Senior Lecturer

Department of Endocrinology

Leeds Centre for Diabetes & Endocrinology

Leeds Teaching Hospitals NHS Trust

St James’s University Hospital

Leeds

Disclosures

RDM declares that he has no competing interests.

Shlomo Melmed, MD

Senior Vice President and Dean

Cedars Sinai Medical Center

Los Angeles

Disclosures

SM has research grants of over 6 figures USD. SM is an author of a number of references cited in this topic.

Laurence Kennedy, MD, FRCP

Professor and Chief

Chairman

Department of Endocrinology, Diabetes and Metabolism

Cleveland Clinic

Cleveland

Disclosures

LK declares that he has no competing interests.

Differentials
- acromegaloidism or pseudoacromegaly
More Differentials
Guidelines
- A Pituitary Society update to acromegaly management guidelines
- A consensus on the diagnosis and treatment of acromegaly comorbidities: an update
More Guidelines
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Acromegaly

Summary

Definition

History and exam

Key diagnostic factors

Other diagnostic factors

Risk factors

Diagnostic tests

1st tests to order

Tests to consider

Treatment algorithm

enclosed pituitary tumor

unresectable pituitary tumor (with neural or vascular impingement/invasion)

nonpituitary adenoma etiology

pituitary adenoma progression or recurrence

Contributors

Authors

Maria Fleseriu, MD, FACE

Disclosures

Acknowledgements

Disclosures

Peer reviewers

Robert D. Murray, MBBS, BSc, FRCP, MD

Disclosures

Shlomo Melmed, MD

Disclosures

Laurence Kennedy, MD, FRCP

Disclosures

Differentials

Guidelines

Log in or subscribe to access all of BMJ Best Practice

Log in or subscribe to access all of BMJ Best Practice

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