Background: Acromegaly is a growth disorder, but mostly it is a metabolic disease related to excessive production of growth hormone (GH). It is characterized by progressive somatic disfigurement in combination with sometimes severe systemic manifestations. Long-acting somatostatin analog (SSA) therapy normalizes serum insulin-like growth factor I (IGF-I) levels in approximately 55% of patients, but we postulate that these patients still have acromegaly in many tissues other than the liver. Direct and indirect effects of SSA reduce hepatic IGF-I generation and make the liver behave as if it is GH resistant. The remaining ‘peripheral’ or non- hepatic acromegaly has a significant negative impact on the quality of life of these patients. Conclusions: Pegvisomant is the most effective medical treatment for acromegaly. Due to its mode of action and pharmacodynamic properties, it is the ideal partner for combination therapy with an SSA for acromegalic patients with a remaining, peripheral form of the disease.

1.
Matta MP, Couture E, Cazals L, Vezzosi D, Bennet A, Caron P: Impaired quality of life of patients with acromegaly: control of GH/IGF-I excess improves psychological subscale appearance. Eur J Endocrinol 2008;158:305–310.
2.
Webb SM, Badia X: Quality of life in growth hormone deficiency and acromegaly. Endocrinol Metab Clin North Am 2007;36:221–232.
3.
Paisley AN, Rowles SV, Roberts ME, Webb SM, Badia X, Prieto L, Shalet SM, Trainer PJ: Treatment of acromegaly improves quality of life, measured by AcroQoL. Clin Endocrinol (Oxf) 2007;67:358–362.
4.
Webb SM: Quality of life in acromegaly. Neuroendocrinology 2006;83:224–229.
[PubMed]
5.
Kauppinen-Mäkelin R, Sane T, Sintonen H, Markkanen H, Valimaki MJ, Löyttyniemi E, Niskanen L, Reunanen A, Stenman UH: Quality of life in treated patients with acromegaly. J Clin Endocrinol Metab 2006;91:3891–3896.
[PubMed]
6.
van der Lely AJ, Hutson RK, Trainer PJ, Besser GM, Barkan AL, Katznelson L, Klibanski A, Herman-Bonert V, Melmed S, Vance ML, Freda PU, Stewart PM, Friend KE, Clemmons DR, Johannsson G, Stavrou S, Cook DM, Phillips LS, Strasburger CJ, Hackett S, Zib KA, Davis RJ, Scarlett JA, Thorner MO: Long-term treatment of acromegaly with pegvisomant, a growth hormone receptor antagonist. Lancet 2001;358:1754–1759.
[PubMed]
7.
Trainer PJ, Drake WM, Katznelson L, Freda PU, Herman-Bonert V, van der Lely AJ, Dimaraki EV, Stewart PM, Friend KE, Vance ML, Besser GM, Scarlett JA, Thorner MO, Parkinson C, Klibanski A, Powell JS, Barkan AL, Sheppard MC, Malsonado M, Rose DR, Clemmons DR, Johannsson G, Bengtsson BA, Stavrou S, Kleinberg DL, Cook DM, Phillips LS, Bidlingmaier M, Strasburger CJ, Hackett S, Zib K, Bennett WF, Davis RJ: Treatment of acromegaly with the growth hormone-receptor antagonist pegvisomant. N Engl J Med 2000;342:1171–1177.
[PubMed]
8.
Holdaway IM, Rajasoorya RC, Gamble GD: Factors influencing mortality in acromegaly. J Clin Endocrinol Metab 2004;89:667–674.
[PubMed]
9.
Webb SM, Prieto L, Badia X, Albareda M, Catalá M, Gaztambide S, Lucas T, Parámo C, Picó A, Lucas A, Halperin I, Obiols G, Astorga R: Acromegaly Quality of Life Questionnaire (ACROQOL) a new health-related quality of life questionnaire for patients with acromegaly: development and psychometric properties. Clin Endocrinol (Oxf) 2002;57:251–258.
[PubMed]
10.
Neggers SJ, van Aken MO, Janssen JA, Feelders RA, de Herder WW, van der Lely AJ: Long-term efficacy and safety of combined treatment of somatostatin analogs and pegvisomant in acromegaly. J Clin Endocrinol Metab 2007;92:4598–4601.
[PubMed]
11.
Feenstra J, de Herder WW, ten Have SM, van den Beld AW, Feelders RA, Janssen JA, van der Lely AJ: Combined therapy with somatostatin analogues and weekly pegvisomant in active acromegaly. Lancet 2005;365:1644–1646 (erratum in: Lancet 2005;365:1620).
[PubMed]
12.
Lamberts SW, van der Lely AJ, de Herder WW, Hofland LJ: Octreotide. N Engl J Med 1996;334:246–254.
[PubMed]
13.
Wurzburger MI, Prelevic GM, Sönksen PH, Balint-Peric LA, Wheeler M: The effect of recombinant human growth hormone on regulation of growth hormone secretion and blood glucose in insulin-dependent diabetes. J Clin Endocrinol Metab 1993;77:267–272.
14.
Leung KC, Doyle N, Ballesteros M, Waters MJ, Ho KK: Insulin regulation of human hepatic growth hormone receptors: divergent effects on biosynthesis and surface translocation. J Clin Endocrinol Metab 2000;85:4712–4720.
[PubMed]
15.
Pokrajac A, Frystyk J, Flyvbjerg A, Trainer PJ: Pituitary-independent effect of octreotide on IGF1 generation. Eur J Endocrinol 2009;160:543–548.
16.
Plöckinger U, Reuter T: Pegvisomant increases intra-abdominal fat in patients with acromegaly: a pilot study. Eur J Endocrinol 2008;158:467–471.
17.
Moller N, Jorgensen JO: Effects of growth hormone on glucose, lipid, and protein metabolism in human subjects. Endocr Rev 2009;30:152–177.
[PubMed]
18.
Flyvbjerg A, Bennett WF, Rasch R, van Neck JW, Groffen CA, Kopchick JJ, Scarlett JA: Compensatory renal growth in uninephrectomized adult mice is growth hormone dependent. Kidney Int 1999;56:2048–2054.
[PubMed]
19.
Neggers SJ, van Aken MO, de Herder WW, Feelders RA, Janssen JA, Badia X, Webb SM, van der Lely AJ: Quality of life in acromegalic patients during long-term somatostatin analog treatment with and without pegvisomant. J Clin Endocrinol Metab 2008;93:3853–3859.
You do not currently have access to this content.