UNDERSTANDING DISEASE
Acromegaly is a disorder that results from an overproduction of growth hormone, typically caused by a benign pituitary tumor. This leads to abnormal growth of bones and tissues, often resulting in enlarged hands, feet, and facial features.
Acromegaly is a rare disease. Often referred to as somatotroph adenoma or growth hormone excess, acromegaly is a rare, chronic metabolic disorder caused by the presence of too much growth hormone.4
Facts & figures:
≥1,000 patients in Malaysia5
130 new cases per year in Malaysia5
Affects men and women equally6
Becomes more common with increasing age6
Incidence 2-11 cases per million per year2
Prevalence 28-137 cases per million2
Acromegaly is caused by abnormal production of growth hormone after normal growth of the skeleton and other organs is complete. In children, too much growth hormone causes a condition called gigantism rather than acromegaly.4
The cause of the increased hormone secretion is usually a benign tumour of the pituitary gland. The pituitary gland, which is located just below the brain, controls the production and release of several different hormones including growth hormone.4
There are no known risk factors for acromegaly other than a prior history of a pituitary tumour.4
Did you know?
The word acromegaly comes from the Greek words “acro,” meaning extremities, and “megaly,” meaning enlargement.
Symptoms often develop slowly and may be missed. Watch for:
The most common symptom of acromegaly is abnormal growth of the hands and feet.7 With swelling of the hands and feet being an early symptom of the condition, people with acromegaly might notice that their rings or shoes have become too tight. Over time, as the bones continue to grow, facial features begin to change.
With the brow and lower jaw becoming more prominent, the bone of the nose becoming larger, and the teeth developing spaces between them. Acromegaly can also lead to arthritis, carpal tunnel syndrome, and enlargement of the internal organs, including the heart.7
Additional symptoms may include:
If left untreated, acromegaly can lead to serious complications like:
High Blood Pressure
18% – 77%
hypertension
Type 2 Diabetes
22.3% –76.8%
higher risk
Cholesterol
up to 61%
dyslipidemia
Obesity
>40%
higher risk
COMPLICATIONS
Cardiovascular
You may develop arterial hyper-tension and or cardiomyopathy (increased size and volume of the heart) with abnormalities in cardiac contractility, sometimes severe heart valve pathologies, or heart rhythm or conduction disorders. Certain cardiac signs may be a result of arterial hyper-tension. GH acts on the muscle of the heart (myocardium), causing hypertrophy (increase in size) of muscle fibres and collagen tissue, thus increasing the size of the heart. 2,15,16
Respiratory
You may suffer from sleep apnoea, and snoring. 2,15,16
Metabolic
GH increases blood glucose levels and exerts a lipolytic effect, which can lead to diabetes and disrupt the balance of fatty acids. 2,17,18
Gastrointestinal tumours
The incidence of polyps in the colon may be higher in patients with acromegaly. 2.15
Endocrine
Excess GH can cause disorders in thyroid function, the development of multinodular thyroid goitres and and hyperparathyroidism. The risk of cancer may be increased ac-cording to certain studies. 2.19
Skeletal and rheumatological
You may suffer from osteoporosis, vertebral fractures, and painful bone and joint pathologies, which can reduce joint mobility and progress to true osteoarthritis. 2.15
Neurological
Carpal tunnel syndrome, most of-ten nocturnal paraesthesia ("tin-gling") can occur in the hands as a result of damage to the median nerve (in the palm of the hand). 2,16
The goal is to bring hormone levels back to normal and relieve symptoms.
Treatment options may include:
LEARN MORE
Surgery – 1st line treatment option
Surgical removal of the adenoma is the treatment of choice for patients with acromegaly.1,2 Surgery can provide immediate control of GH levels in the blood, improve symptoms and reduce adenoma volume.
In some cases, patients may require medical therapy before surgery. Surgery results in remission rates of 66–90% for patients with microadenomas, >70% for patients with macroadenomas confined to the sella turcica, 40–50% for patients with non invasive macroadenomas, and <20% for patients with invasive macroadenomas.3,4 The results are better when the adenoma is small and minimally invasive, and when pre-operative GH levels are low.5
Today, the surgery is usually done using often endoscopic microsurgical techniques, passing through the nostril, following the nasal septum and then the sinuses to reach the sphenoid sinus and, eventually the pituitary gland (this is the transsphenoidal route).4
Surgery may be contraindicated or inappropriate, for example if the adenoma is invasive. Medical treatment can then be offered as first line treatment.2
Medical Therapy – 2nd line option
If surgical treatment does not lead to remission or is not an option, medical treatment is often the second approach recommended.1
Medical treatments currently comprise the following types:1
1. Somatostatin receptor ligands (reduce the amount of GH produced).
2. GH receptor antagonists (block the action of GH on GH receptors).
3. Dopamine receptor agonists (reduce the amount of GH produced).
Radiation therapy
The doctor may also suggest revision surgery or radiation therapy (conventional or stereotactic by gamma knife), depending on the size and location of the adenoma and the residual amount of hormones.2
The goal is to bring hormone levels back to normal and relieve symptoms.
If you've experienced unusual changes in your body or health, don’t ignore them.
Talk to your doctor. Early diagnosis can make a big difference.
Can affect many parts of your body. The manifestations and their intensity can vary.
These includes:
WHAT YOU CAN DO
Know what to do before your visit
When you make the appointment, ask if there's anything you need to do to prepare for testing.
Write down any symptoms you're having
Include any that seem unrelated to the appointment. For example, if you've had headaches more often or if you've been feeling down or more tired than usual, tell your health care provider. Also talk to your provider about changes in your appearance, such as weight gain, new acne or more body hair.
Write down key personal information
Let your provider know if the people closest to you have noticed that you seem irritable or have more mood swings than in the past. It may help to bring a photo of yourself that shows any changes in your appearance since you've started having symptoms.
Make a list of all medicines, vitamins, creams or supplements
Write down the name, dose and dates of any steroid medicines you've taken before, such as cortisone shots.
Take a family member or friend along, if possible
Sometimes it can be hard to remember all the information you get during an appointment. Someone who comes with you may remember something that you missed or forgot.
Write down questions
To ask your health care provider
References
Gatto F, Barbieri F, Arvigo M, et al. Biological and biochemical basis of the differential efficacy of first and second generation somatostatin receptor ligands in neuroendocrine neoplasms. Int J Mol Sci. 2019;20(16):3940. 2. Poullot A-G, Chevalier N. New options in the treatment of Cushing’s disease: a focus on pasireotide. Res Rep Endocr Disord. 2013;3:31-38. 3. SIGNIFOR LAR (pasireotide) for injectable suspension, for intramuscular use [prescribing information]. Lebanon, NJ: Recordati Rare Diseases Inc.; 2020. 4. Acromegaly. UCLA Health System. https://www.uclahealth.org/medical-services/surgery/endocrine-surgery/patient-resources/patient-education/endocrine-surgery-encyclopedia/acromegaly. Accessed August 23, 2022. 5. Malaysia Consensus Statement for The Diagnosis and Management of Acromegaly 1st Edition. https://mems.my/wp-content/uploads/2019/07/Acromegaly-manuscript-and-booklet_20190705_v21_FINAL.pdf. Published July 05, 2019. Accessed Dec 23, 2024. 6. Christofides EA. Clinical importance of achieving biochemical control with medical therapy in adult patients with acromegaly. Patient Prefer Adherence. 2016;10:1217-1225. 7. Acromegaly. National Institute of Diabetes and Digestive and Kidney Diseases. https://www.niddk.nih.gov/health-information/endocrine-diseases/acromegaly. Accessed August 23, 2022. 8. Carmichael JD, Bonert VS, Nu.o M, Ly D, Melmed S. Acromegaly clinical trial methodology impact on reported biochemical efficacy rates of somatostatin receptor ligand treatments: a meta-analysis. J Clin Endocrinol Metab. 2014;99(5):1825-1833. 9. Carroll PV, Jenkins PJ. Acromegaly. In: Feingold KR, Anawalt B, Boyce A, et al, eds. Endotext [Internet]. South Dartmouth, MA: MDText.com, Inc.; 2016 10. Giustina A, Barkhoudarian G, Beckers A, et al. Multidisciplinary management of acromegaly: A consensus. Rev Endocr Metab Disord. 2020;21(4):667-678. 11. Katznelson L, Laws ER Jr, Melmed S, et al. Endocrine Society Acromegaly: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2014;99(11):3933-3951. 12. Lavrentaki A, Paluzzi A, Wass JA, Karavitaki N. Epidemiology of acromegaly: review of population studies. Pituitary. 2017;20(1):4-9. 13. SOMATULINE® DEPOT (lanreotide) injection, for subcutaneous use [prescribing information]. Cambridge, MA: Ipsen Biopharmaceuticals, Inc.; 2019 14. SANDOSTATIN LAR DEPOT (octreotide acetate) for injectable suspension, for gluteal intramuscular use [prescribing information]. East Hanover, NJ: Novartis Pharmaceuticals Corporation; 2021. 15. Coopmans EC, Muhammad A, van der Lely AD, et al. How to position pasireotide LAR treatment in acromegaly. J Clin Endocrinol Metab. 2019;104(6):1978-1988. 16. Shanik MH, Cao PD, Ludlam WH. Historical response rates of somatostatin analogues in the treatment of acromegaly: a systematic review. Endocr Pract. 2016;22(3):350-356. 17. Casar-Borota O, Heck A, Schulz S, et al. Expression of SSTR2a, but not of SSTRs 1, 3, or 5 in somatotroph adenomas assessed by monoclonal antibodies was reduced by octreotide and correlated with the acute and long-term effects of octreotide. J Clin Endocrinol Metab. 2013;98(11):E1730-E1739. 18. Silverstein JM. Hyperglycemia induced by pasireotide in patients with Cushing’s disease or acromegaly. Pituitary. 2016;19:536-543. 19. Zambre Y, Ling Z, Chen MC, et al. Inhibition of human pancreatic islet insulin release by receptor-selective somatostatin analogs directed to somatostatin receptor subtype 5. Biochem Pharmacol. 1999;57(10):1159-1164. 20. Singh V, Brendel MD, Zacharias S, et al. Characterization of somatostatin receptor subtype-specific regulation of insulin and glucagon secretion: an in vitro study on isolated human pancreatic islets. J Clin Endocrinol Metab. 2007;92(2):673-680. 21. Breitschaft A, Hu K, Hermosillo Res.ndiz K, Darstein C, Golor G. Management of hyperglycemia associated with pasireotide (SOM230): healthy volunteer study. Diabetes Res Clin Pract. 2014;103(3):458-465. 22. Henry RR, Ciaraldi TP, Armstrong D, Burke P, Ligueros-Saylan M, Mudaliar S. Hyperglycemia associated with pasireotide: results from a mechanistic study in healthy volunteers. J Clin Endocrinol Metab. 2013;98(8):3446-3453. 23. 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;2(11):875-884. 24. Colao A, Bronstein MD, Freda P, et al. Pasireotide versus octreotide in acromegaly: a head-to-head superiority study. J Clin Endocrinol Metab. 2014;99(3):791-799. 25. Gadelha MR, Gu F, Bronstein MD, et al. Risk factors and management of pasireotide-associated hyperglycemia in acromegaly. Endocr Connect. 2020;9(12):1178-1190. 26. American Diabetes Association. Standards of Medical Care in Diabetes-2020 Abridged for Primary Care Providers. Clin Diabetes. 2020;38(1):10-38. doi:10.2337/cd20-as01. 27. Samson SL, Gu F, Feldt-Rasmussen U, Zhang S, Yu Y, et al. Managing pasireotide-associated hyperglycemia: a randomized, open-label, Phase IV study. Pituitary. 2021;24(6):887-903.
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