Acromegaly is characterized by increased release of
growth hormone and, consequently,
insulin-like growth factor I (IGF1), most often by a
pituitary adenoma. Prolonged exposure to excess
hormone leads to progressive somatic disfigurement and a wide range of systemic manifestations that are associated with increased mortality. Although considered a
rare disease, recent studies have reported an increased incidence of
acromegaly owing to better disease awareness, improved diagnostic tools and perhaps a real increase in prevalence.
Acromegaly treatment approaches, which include surgery,
radiotherapy and medical
therapy, have changed considerably over time owing to improved
surgical procedures, development of new
radiotherapy techniques and availability of new medical
therapies. The optimal use of these treatments will reduce mortality in patients with
acromegaly to levels in the general population. Medical
therapy is currently an important treatment option and can even be the first-line treatment in patients with
acromegaly who will not benefit from or are not suitable for first-line neurosurgical treatment. Pharmacological treatments include
somatostatin receptor ligands (such as
octreotide,
lanreotide and
pasireotide),
dopamine agonists and the
growth hormone receptor antagonist
pegvisomant. In this Primer, we review the main aspects of
acromegaly, including scientific advances that underlie expanding knowledge of disease pathogenesis, improvements in disease management and new medical
therapies that are available and in development to improve disease control.