Acromegaly is a rare endocrine disorder caused by excess levels of growth hormone in the body. In most cases, the high GH levels are the result of a benign tumor in the pituitary gland.
In rare cases, the acromegaly may be due to excess secretion of growth hormone releasing hormone (GHRH) by the hypothalamus or from neuroendocrine tumors found in other organs (which may oversecrete GH as well).
Early symptoms of acromegaly include abnormal enlargement in bones of the hands, arms, feet, legs, and head. In most cases, the change in appearance is gradual making it difficult to diagnose quickly. Often the most striking initial features are the enlargement of the jaw and forehead. As the disease progresses, continued changes in bone structure as well as overgrowth of soft tissue and cartilage can impact multiple aspects of the body, including malocclusion, osteoarthritis, arthralgia, myalgia, kyphoscoliosis, carpal tunnel syndrome, hirsutism, hepatomegaly, splenomegaly, type 2 diabettes, hyperhidrosis, and cardiovascular problems.
Acromegaly usually begins in the fourth and fifth decade of life but can occur anytime after puberty. This is in contrast to another rare disorder, gigantism, that begins before puberty.
Acromegaly is treated by surgery, medications and/or radiation therapy, depending on the individual.
If surgery is an option, usually when the tumors are small and easy to remove, growth hormone levels will often return to normal levels but some symptoms may persists.
Radiation therapy may accompany surgical or pharmaceutical therapy. It does require daily dosing over a 4 – 6 week period and often the results are not seen for some time later. There are two types of medications approved to treat acromegaly:
- somatostatin analogues (octreotide, lanreotide pasireotide)
- growth hormone receptor antagonists (pegvisomant)
Sometimes, dopamine agonists are used off-label but these medications appear to be effective in only a small proportion of patients.
In addition, to the above treatments, more medications are in development and clinicians should search https://clinicaltrials.gov to learn more about those clinical trials.
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