Acromegaly is abnormally high linear growth due to excessive action of insulinlike growth factor I (IGF-I) that occurs after the growth plate cartilage fuses in adulthood. (Gigantism is the same disorder of IGF-1 but occurs while the epiphyseal growth plates are open during childhood.)
The guidelines include the following:
Biochemical control is the strongest predictor of patient outcome, reflecting improvements in glucose metabolism, obstructive sleep apnea, cardiovascular disease, and vertebral fractures.
Insulinlike growth factor I (IGF-I) levels 6 weeks after surgery can assess remission, but normalization of levels may still occur by 3-6 months.
Predictors of more favorable response to lanreotide, 120 mg therapy every 4 weeks, include older age, female sex, lower IGF-I levels, and hypointensity of tumor T2-weighted MRI images at baseline.
Oral octreotide capsules (OOC), which received US FDA approval in June 2020 for long-term maintenance in acromegaly patients who respond to treatment with octreotide or lanreotide, are suitable for patients who have had a complete or partial response on injectable octreotide or lanreotide.
OOC is not recommended for patients with octreotide-resistant tumors.
OOC is initiated at a dose of 40 mg/day, given as 20 mg capsules twice per day taken 1 hr before or 2 hr after a meal.
OOC dose can be increased by 20 mg every 2-4 wk on the basis of IGF-I levels and clinical symptoms. (Injectable somatostatin receptor ligand [SRL] doses are often increased every 3 mo.)
Pegvisomant in patients with diabetes mellitus improves glucose metabolism independent of IGF-I control.
Higher doses of pegvisomant and a more rapid increase in dose are necessary in patients with diabetes mellitus and those with a higher body mass index to achieve normal IGF-I levels.
For patients who require combination therapy, low-dose octreotide long-acting release or lanreotide plus weekly pegvisomant is a good option.
The combination of pasireotide and pegvisomant can achieve control rates above 70%, but the addition of pegvisomant does not reduce the high rates of pasireotide-induced hyperglycemia.
For more information, please go to Gigantism and Acromegaly.
For more Clinical Practice Guidelines, please go to Guidelines.
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Cite this: Acromegaly Clinical Practice Guidelines (PS, 2021) - Medscape - Feb 26, 2021.