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The changing landscape of acromegaly – an epidemiological perspective

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Abstract

Acromegaly is a rare disease and thus challenging to accurately quantify epidemiologically. In this comprehensive literature review, we compare different approaches to studying acromegaly from an epidemiological perspective and describe the temporal evolution of the disease pertaining to epidemiological variables, clinical presentation and mortality. We present updated epidemiological data from the population-based Danish cohort of patients with acromegaly (AcroDEN), along with meta-analyses of existing estimates from around the world.

Based on this, we conclude that the incidence, prevalence and age at acromegaly diagnosis are all steadily increasing, but with considerable variation between studies. An increased number of incidental cases may contribute to the increase in incidence and age at diagnosis, respectively. The clinical features at presentation are trending toward a milder disease phenotype at diagnosis, and advances in therapeutic options have reduced the mortality of patients with acromegaly to a level similar to that of the general population. Moreover, the underlying cause of death has shifted from cardiovascular to malignant neoplastic diseases.

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Data availability

Data from the present study is available from the authors upon request.

Abbreviations

GH:

Growth Hormone

IGF-I:

Insulin-like Growth Factor 1

ICD:

International Classification of Disease

MRI:

Magnetic Resonance Imaging

95%CI:

95% Confidence Interval

AIP:

Aryl hydrocarbon receptor-Interacting Protein

MEN1:

Multiple Endocrine Neoplasia type 1

LAS:

Liége Acromegaly Survey

SMR:

Standardized Mortality Ratio

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Authors and Affiliations

  1. Department of Endocrinology, Aalborg University Hospital, Aalborg, Denmark

    Christian Rosendal, Eigil Husted Nielsen & Jakob Dal

  2. Department of Endocrinology and Internal Medicine, Aarhus University Hospital, Aarhus, Denmark

    Mai Christiansen Arlien-Søborg & Jens Otto Lunde Jørgensen

  3. Department of Endocrinology, Odense University Hospital, Odense, Denmark

    Marianne Skovsager Andersen

  4. Department of Medicine, Copenhagen University Hospital - Herlev and Gentofte Hospital, Herlev, Denmark

    Claus Larsen Feltoft

  5. Department of Hormone and Metabolism, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark

    Caroline Kistorp

  6. Department of Clinical Medicine, Faculty of Health and Medical Sciences, University of Copenhagen, Copenhagen, Denmark

    Caroline Kistorp

  7. Department of Clinical Epidemiology, Leiden University Medical Center, Leiden, The Netherlands

    Olaf M. Dekkers

  8. Department of Endocrinology, Leiden University Medical Center, Leiden, The Netherlands

    Olaf M. Dekkers

  9. Aarhus KEA, Aarhus University Hospital, Aarhus, Denmark

    Olaf M. Dekkers

  10. Steno Diabetes Center North Jutland, Aalborg, Denmark

    Jakob Dal

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  1. Christian Rosendal
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Contributions

All authors contributed to the study conception and design, as well as the establishment of the database from which the presented original epidemiological data is derived. The first draft of the manuscript was written by J.D. and C.R. and all authors commented on previous versions of the manuscript. All authors read, critically revised and approved the final manuscript.

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Correspondence to Christian Rosendal.

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JD: Unrestricted research grant from Pfizer and Ipsen.

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The construction of the AcroDEN cohort was supported by unrestricted research grants from Pfizer and Ipsen.

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Rosendal, C., Arlien-Søborg, M.C., Nielsen, E.H. et al. The changing landscape of acromegaly – an epidemiological perspective. Rev Endocr Metab Disord (2024). https://doi.org/10.1007/s11154-024-09875-z

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