Luigi Maione1,2,3, Thierry Brue4, Albert Beckers5, Brigitte Delemer6, Patrick Petrossians5, Françoise Borson-Chazot7,8,9, Olivier Chabre10, Patrick François11, Jérôme Bertherat12, Christine Cortet-Rudelli13, Philippe Chanson1,2,3. 1. Assistance Publique-Hôpitaux de ParisHôpital de Bicêtre, Service d'Endocrinologie et des Maladies de la Reproduction, Le Kremlin Bicêtre, France. 2. Univ Paris-SudFaculté de Médecine Paris-Sud, Unité Mixte de Recherche-S1185, Le Kremlin Bicêtre, France. 3. Institut National de la Santé et de la Recherche Médicale (INSERM) U1185Le Kremlin Bicêtre, France. 4. Université Aix-Marseille and Assistance Publique-Hôpitaux de MarseillePôle ENDO (Endocrinologie, Diabète, Obésité Nutrition), Hôpital de la Conception, Marseille, France. 5. Département de Médecine InterneService d'Endocrinologie, Centre Hospitalier Universitaire de Liège, Université de Liège, Domaine Universitaire du Sart-Tilman, Liège, Belgium. 6. Service d'EndocrinologieDiabète et Nutrition, Hôpital Robert Debré, Centre Hospitalier Universitaire de Reims, Reims, France. 7. Université Lyon 1Lyon, France. 8. Hospices Civils de LyonFédération d'Endocrinologie du pôle Est, Lyon, France. 9. INSERM U1052UMR CNRS 5286, Centre de Recherche en Cancérologie de Lyon, Equipe Tumeurs Endocrines, Lyon, France. 10. Service d'Endocrinologie-Diabétologie-NutritionPôle Digidune, Centre Hospitalier Universitaire de Grenoble and Université Grenoble Alpes, Grenoble, France. 11. Service de NeurochirurgieCHRU de Tours; and Université François-Rabelais de Tours, Tours, France. 12. Assistance Publique Hôpitaux de ParisService d'Endocrinologie, Hôpital Cochin, and Université Paris Descartes, Sorbonne Paris Cité, and INSERM U1016, CNRS UM8104, Institut Cochin, Paris, France. 13. Service de DiabétologieEndocrinologie et des Maladies Métaboliques, Hôpital Huriez, CHU de Lille, Lille, France.
Abstract
CONTEXT: Acromegaly is a rare disease associated with chronic multisystem complications. National registries have been created in several countries. DESIGN: The French Registry contains data on acromegaly epidemiology, management and comorbidities recorded over more than three decades, retrospectively until 1999 and prospectively from 1999 to 2012. RESULTS: Data could be analyzed for 999 of the 1034 patients included in the registry (46% males). Disease control, defined as IGF-I normalization (adjusted for age and sex), was achieved in 75% of patients at the last follow-up visit. Half the patients with uncontrolled disease had IGF-I levels below 1.5 times the upper limit of normal (ULN). The proportion of patients with surgically cured disease did not change markedly over time, whereas the proportion of patients with uncontrolled disease fell and the proportion of patients with medically controlled disease rose. Cardiovascular, metabolic, respiratory and rheumatologic comorbidities and their outcomes were recorded for most patients, and no noteworthy overall deterioration was noted over time. Cancer occurred in 10% of patients, for a standardized incidence ratio of 1.34 (95% CI: 0.94-1.87) in men and 1.24 (0.77-1.73) in women. Forty-one patients died during follow-up, for a standardized mortality ratio of 1.05 (0.70-1.42). Most deaths were due to cancer. CONCLUSIONS: The majority of patients with acromegaly now have successful disease control thanks to the multistep management. The incidence of comorbidities following diagnosis of acromegaly is very low. Life expectancy is now close to that of the general population, probably owing to better management of the GH/IGF-I excess and comorbidities.
CONTEXT: Acromegaly is a rare disease associated with chronic multisystem complications. National registries have been created in several countries. DESIGN: The French Registry contains data on acromegaly epidemiology, management and comorbidities recorded over more than three decades, retrospectively until 1999 and prospectively from 1999 to 2012. RESULTS: Data could be analyzed for 999 of the 1034 patients included in the registry (46% males). Disease control, defined as IGF-I normalization (adjusted for age and sex), was achieved in 75% of patients at the last follow-up visit. Half the patients with uncontrolled disease had IGF-I levels below 1.5 times the upper limit of normal (ULN). The proportion of patients with surgically cured disease did not change markedly over time, whereas the proportion of patients with uncontrolled disease fell and the proportion of patients with medically controlled disease rose. Cardiovascular, metabolic, respiratory and rheumatologic comorbidities and their outcomes were recorded for most patients, and no noteworthy overall deterioration was noted over time. Cancer occurred in 10% of patients, for a standardized incidence ratio of 1.34 (95% CI: 0.94-1.87) in men and 1.24 (0.77-1.73) in women. Forty-one patients died during follow-up, for a standardized mortality ratio of 1.05 (0.70-1.42). Most deaths were due to cancer. CONCLUSIONS: The majority of patients with acromegaly now have successful disease control thanks to the multistep management. The incidence of comorbidities following diagnosis of acromegaly is very low. Life expectancy is now close to that of the general population, probably owing to better management of the GH/IGF-I excess and comorbidities.
Authors: Roberto Salvatori; Pietro Maffei; Susan M Webb; Thierry Brue; Jane Loftus; Srinivas Rao Valluri; Roy Gomez; Michael P Wajnrajch; Maria Fleseriu Journal: Pituitary Date: 2022-01-12 Impact factor: 4.107
Authors: Maria Rosaria Ambrosio; Irene Gagliardi; Sabrina Chiloiro; Ana Gonçalves Ferreira; Marta Bondanelli; Antonella Giampietro; Antonio Bianchi; Laura De Marinis; Maria Fleseriu; Maria Chiara Zatelli Journal: Endocrine Date: 2020-02-14 Impact factor: 3.633