Vol 70, No 1 (2019)
Guidelines / Expert consensus
Published online: 2019-02-22

open access

Page views 12804
Article views/downloads 8732
Get Citation

Connect on Social Media

Connect on Social Media

The content of this article is also available in the following languages:
Polski

Diagnostics and treatment of acromegaly — updated recommendations of the Polish Society of Endocrinology [Rozpoznanie i leczenie akromegalii — aktualizacja rekomendacji Polskiego Towarzystwa Endokrynologicznego]

Marek Bolanowski1, Marek Ruchała2, Wojciech Zgliczyński3, Beata Kos-Kudła4, Alicja Hubalewska-Dydejczyk5, Andrzej Lewiński6,
Pubmed: 30843181
Endokrynol Pol 2019;70(1):2-18.

Abstract

Akromegalia jest rzadką chorobą spowodowaną nadmiernym wydzielaniem hormonu wzrostu (GH), zwykle przez guz przysadki. Rozpoznanie jest zwykle stawiane z opóźnieniem, w związku z czym często dochodzi do rozwoju różnych powikłań choroby będących przyczyną zwiększonego zagrożenia zgonem. U chorych z nadciśnieniem, niewydolnością serca, cukrzycą, artropatiami, nietypowymi dla wieku, należy zwrócić uwagę na występowanie objawów akromegalii. Oznaczanie stężenia insulinopodobnego czynnika wzrostu 1 (IGF-1) powinno być stosowane jako badanie przesiewowe przy podejrzeniu akromegalii. Dalsza diagnostyka i leczenie powinny być prowadzone w wyspecjalizowanych ośrodkach. Leczeniem pierwszego rzutu jest wybiórcze usunięcie gruczolaka przysadki z dostępu przez zatokę klinową. Pacjentów, u których jest szansa na wyleczenie w wyniku operacyjnego usunięcia guza przysadki, po przygotowaniu farmakologicznym należy kierować do ośrodków mających doświadczenie w tego typu zabiegach. U pozostałych chorych oraz u pacjentów, u których leczenie neurochirurgiczne było nieskuteczne, należy stosować przewlekłe leczenie, w pierwszej kolejności analogami somatostatyny pierwszej generacji. W leczeniu drugiego rzutu należy rozważyć pazyreotyd, pegwisomant, kabergolinę lub ich kombinacje. W każdym przypadku chorych należy objąć obserwacją przez całe życie w celu monitorowania i aktywnego leczenia następstw akromegalii. Obecne zalecenia są uaktualnioną wersją rekomendacji publikowanych w Endokrynologii Polskiej w 2014 roku. Uwzględniają one krajowe realia i powinny być pomocne w postępowaniu z pacjentami chorymi na akromegalię. 

Article available in PDF format

View PDF Download PDF file

References

  1. Bolanowski M, Ruchała M, Zgliczyński W, et al. Acromegaly — a novel view of the patient. Polish proposals for diagnostic and therapeutic procedures in the light of recent reports. Endokrynol Pol. 2014; 65(4): 326–331.
  2. Giustina A, Bronstein MD, Casanueva FF, et al. Current management practices for acromegaly: an international survey. Pituitary. 2011; 14(2): 125–133.
  3. Katznelson L, Laws ER, Melmed S, et al. Endocrine Society. Acromegaly: an endocrine society clinical practice guideline. J Clin Endocrinol Metab. 2014; 99(11): 3933–3951.
  4. Melmed S, Casanueva FF, Klibanski A, et al. Pituitary Society and the European Neuroendocrine Association. A consensus on the diagnosis and treatment of acromegaly complications. Pituitary. 2013; 16(3): 294–302.
  5. Melmed S, Bronstein MD, Chanson P, et al. A Consensus Statement on acromegaly therapeutic outcomes. Nat Rev Endocrinol. 2018; 14(9): 552–561.
  6. Paragliola RM, Salvatori R. Novel somatostatin receptor ligands therapies for acromegaly. Front Endocrinol (Lausanne). 2018; 9: 78.
  7. Chanson P, Salenave S, Kamenicky P, et al. Pituitary tumours: acromegaly. Best Pract Res Clin Endocrinol Metab. 2009; 23(5): 555–574.
  8. Melmed S. Medical progress: acromegaly. N Engl J Med. 2006; 355(24): 2558–2573.
  9. Reid TJ, Post KD, Bruce JN, et al. Features at diagnosis of 324 patients with acromegaly did not change from 1981 to 2006: acromegaly remains under-recognized and under-diagnosed. Clin Endocrinol (Oxf). 2010; 72(2): 203–208.
  10. Holdaway IM, Rajasoorya C. Epidemiology of acromegaly. Pituitary. 1999; 2(1): 29–41.
  11. Orlewska E, Kos-Kudła B, Sowiński J, et al. Wyniki fazy retrospektywnej badania obserwacyjnego Lanro-Study oceniającego zużycie zasobów ochrony zdrowia w populacji polskich pacjentów z akromegalią, leczonych preparatem Somatuline AUTOGEL [Results of the retrospective phase of an observational study Lanro-Study evaluating the use of health care resources in the population of Polish patients with acromegaly, treated with Somatuline Autogel]. Nowa Medycyna. 2012; 3: 39–46.
  12. Fernandez A, Karavitaki N, Wass JAH. Prevalence of pituitary adenomas: a community-based, cross-sectional study in Banbury (Oxfordshire, UK). Clin Endocrinol (Oxf). 2010; 72(3): 377–382.
  13. Beckers A, Lodish MB, Trivellin G, et al. X-linked acrogigantism syndrome: clinical profile and therapeutic responses. Endocr Relat Cancer. 2015; 22(3): 353–367.
  14. Stelmachowska-Banas M, Zgliczynski W, Tutka P, et al. Fatal Carney Complex in Siblings Due to De Novo Large Gene Deletion. J Clin Endocrinol Metab. 2017; 102(11): 3924–3927.
  15. Bernabeu I, Aller J, Álvarez-Escolá C, et al. Criteria for diagnosis and postoperative control of acromegaly, and screening and management of its comorbidities: Expert consensus. Endocrinol Diabetes Nutr. 2018; 65(5): 297–305.
  16. Potorac I, Petrossians P, Daly AF, et al. T2-weighted MRI signal predicts hormone and tumor responses to somatostatin analogs in acromegaly. Endocr Relat Cancer. 2016; 23(11): 871–881.
  17. Giustina A, Chanson P, Kleinberg D, et al. Acromegaly Consensus Group. Expert consensus document: A consensus on the medical treatment of acromegaly. Nat Rev Endocrinol. 2014; 10(4): 243–248.
  18. Bolanowski M, Bar-Andziak E, Kos-Kudła B, et al. Polish Society for Endocrinology. Consensus statement of the Polish Society for Endocrinology: presurgical somatostatin analogs therapy in acromegaly. Neuro Endocrinol Lett. 2008; 29(1): 59–62.
  19. Annamalai AK, Webb A, Kandasamy N, et al. A comprehensive study of clinical, biochemical, radiological, vascular, cardiac, and sleep parameters in an unselected cohort of patients with acromegaly undergoing presurgical somatostatin receptor ligand therapy. J Clin Endocrinol Metab. 2013; 98(3): 1040–1050.
  20. Bates PR, Carson MN, Trainer PJ, et al. UK National Acromegaly Register Study Group (UKAR-2). Wide variation in surgical outcomes for acromegaly in the UK. Clin Endocrinol (Oxf). 2008; 68(1): 136–142.
  21. Colao A, Attanasio R, Pivonello R, et al. Partial surgical removal of growth hormone-secreting pituitary tumors enhances the response to somatostatin analogs in acromegaly. J Clin Endocrinol Metab. 2006; 91(1): 85–92.
  22. Schopohl J, Strasburger CJ, Caird D, et al. German Lanreotide Study Group. Efficacy and acceptability of lanreotide Autogel® 120 mg at different dose intervals in patients with acromegaly previously treated with octreotide LAR. Exp Clin Endocrinol Diabetes. 2011; 119(3): 156–162.
  23. Colao A, Pivonello R, Auriemma RS, et al. Beneficial effect of dose escalation of octreotide-LAR as first-line therapy in patients with acromegaly. Eur J Endocrinol. 2007; 157(5): 579–587.
  24. Murray RD, Melmed S. A critical analysis of clinically available somatostatin analog formulations for therapy of acromegaly. J Clin Endocrinol Metab. 2008; 93(8): 2957–2968.
  25. Gomez-Panzani E, Chang S, Ramis J, et al. Sustained biochemical control in patients with acromegaly treated with lanreotide depot 120 mg administered every 4 weeks, or an extended dosing interval of 6 or 8 weeks: a pharmacokinetic approach. Res Rep Endocrine Dis. 2012; 2: 79–84.
  26. Witek P, Mucha S, Ruchała M. Patient satisfaction and preferences of lanreotide Autogel treatment in acromegaly. Endokrynol Pol. 2016; 67(6): 572–579.
  27. Cuevas-Ramos D, Fleseriu M. Somatostatin receptor ligands and resistance to treatment in pituitary adenomas. J Mol Endocrinol. 2014; 52(3): R223–R240.
  28. Colao A, Bronstein MD, Freda P, et al. Pasireotide C2305 Study Group. Pasireotide versus octreotide in acromegaly: a head-to-head superiority study. J Clin Endocrinol Metab. 2014; 99(3): 791–799.
  29. Gadelha MR, Bronstein MD, Brue T, et al. Pasireotide C2402 Study Group. 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.
  30. Muhammad A, Van der Lely AJ, Delhanty P, et al. Efficacy and Safety of Switching to Pasireotide in Patients With Acromegaly Controlled With Pegvisomant and First-Generation Somatostatin Analogues (PAPE Study). J Clin Endocrinol Metab. 2018; 103(2): 586–595.
  31. Shimon I, Adnan Z, Gorshtein A, et al. Efficacy and safety of long-acting pasireotide in patients with somatostatin-resistant acromegaly: a multicenter study. Endocrine. 2018; 62(2): 448–455.
  32. Leonart LP, Tonin FS, Ferreira VL, et al. Effectiveness and safety of pegvisomant: a systematic review and meta-analysis of observational longitudinal studies. Endocrine. 2018 [Epub ahead of print].
  33. Buchfelder M, van der Lely AJ, Biller BMK, et al. Long-term treatment with pegvisomant: observations from 2090 acromegaly patients in ACROSTUDY. Eur J Endocrinol. 2018; 179(6): 419–427.
  34. Strasburger CJ, Mattsson A, Wilton P, et al. Increasing frequency of combination medical therapy in the treatment of acromegaly with the GH receptor antagonist pegvisomant. Eur J Endocrinol. 2018; 178(4): 321–329.
  35. Ramos-Leví AM, Bernabeu I, Álvarez-Escolá C, et al. Long-term treatment with pegvisomant for acromegaly: a 10-year experience. Clin Endocrinol (Oxf). 2016; 84(4): 540–550.
  36. Kuhn E, Chanson P. Cabergoline in acromegaly. Pituitary. 2017; 20(1): 121–128.
  37. Sandret L, Maison P, Chanson P. Place of cabergoline in acromegaly: a meta-analysis. J Clin Endocrinol Metab. 2011; 96(5): 1327–1335.
  38. Lim DS, Fleseriu M. The role of combination medical therapy in the treatment of acromegaly. Pituitary. 2017; 20(1): 136–148.
  39. Wass JAH. Radiotherapy in acromegaly: a protagonists viewpoint. Clin Endocrinol (Oxf). 2003; 58(2): 128–131.
  40. Thorner MO. Controversy: radiotherapy for acromegaly. Clin Endocrinol (Oxf). 2003; 58(2): 136–137.
  41. Casanueva FF, Barkan AL, Buchfelder M, et al. Pituitary Society, Expert Group on Pituitary Tumors, Pituitary Society, Expert Group on Pituitary Tumors. Criteria for the definition of Pituitary Tumor Centers of Excellence (PTCOE): A Pituitary Society Statement. Pituitary. 2017; 20(5): 489–498.
  42. Woliński K, Stangierski A, Gurgul E, et al. Thyroid lesions in patients with acromegaly - case-control study and update to the meta-analysis. Endokrynol Pol. 2017; 68(1): 2–6.
  43. Webb SM. Quality of life in acromegaly. Neuroendocrinology. 2006; 83(3–4): 224–229.
  44. Giustina A, Bevan JS, Bronstein MD, et al. SAGIT Investigator Group. SAGIT®: clinician-reported outcome instrument for managing acromegaly in clinical practice--development and results from a pilot study. Pituitary. 2016; 19(1): 39–49.
  45. van der Lely AJ, Gomez R, Pleil A, et al. Development of ACRODAT, a new software medical device to assess disease activity in patients with acromegaly. Pituitary. 2017; 20(6): 692–701.
  46. Elbaum M, Mizera Ł, Bolanowski M. Real costs of acromegaly: analysis of different therapies. Endokrynol Pol. 2018; 70(2): (accepted, in press).
  47. Neggers SJ, Pronin V, Balcere I, et al. LEAD Study Group. Lanreotide Autogel 120 mg at extended dosing intervals in patients with acromegaly biochemically controlled with octreotide LAR: the LEAD study. Eur J Endocrinol. 2015; 173(3): 313–323.
  48. Bolfi F, Neves AF, Boguszewski CL, et al. Mortality in acromegaly decreased in the last decade: a systematic review and meta-analysis. Eur J Endocrinol. 2018; 179(1): 59–71.
  49. Gadelha MR, Kasuki L, Lim DSt, et al. Systemic complications of acromegaly and the impact of the current treatment landscape: an update. Endocr Rev. 2018 [Epub ahead of print].
  50. Dekkers OM, Biermasz NR, Pereira AM, et al. Mortality in acromegaly: a metaanalysis. J Clin Endocrinol Metab. 2008; 93(1): 61–67.