Addison’s disease in Italy: mortality and survival by etiology

BACKGROUND: Data on survival and mortality in Addison's disease (AD) are contradictory: some studies suggest a mortality rate two times higher than in the general population, but this is not consistently reported in other studies. METHODS: We evaluated survival and mortality in 1,826 Italian adult patients with AD of different etiologies, collected from 1947 to 2024 across 30 Endocrine Units. These outcomes were compared to those of the age- and sex-matched Italian reference population using a standardized mortality ratio (SMR) in a subgroup of 1,575 cases. RESULTS: Sex and age adjusted survival analysis showed increased mortality in genetic AD, cancer-related-AD and cases with type 1 autoimmune polyendocrine syndrome (APS-1) compared to other etiologies. The SMR analysis demonstrated a significantly increased mortality risk of the overall patients with AD compared to the general population (SMR 1.34, p < 0.01). However, 4 etiologies presented survival similar to the general population: patients with type 2 or type 4 APS or isolated autoimmune AD (SMR 0.91, p = 0,57), vascular/post-tuberculosis AD (SMR 0.90, p = 0.69), bilateral adrenalectomy due to benign endocrine diseases (SMR 0.91. p = 0.64) and not defined AD (SMR 1.06, p = 0.93). These groups included 1,188 patients, representing 75.4% of the population. On the contrary, a significantly increased mortality risk compared to the general population was reported in the following 3 groups: patients with APS-1 (SMR 5.98, p < 0.01), genetic AD (SMR 5.09, p < 0.01), and cancer-related AD (SMR 4.64, p < 0.01), accounting for 387 patients (24.5%). CONCLUSION: Our study has shown that in adult patients with AD there is a large difference in survival and mortality based on the various etiological forms. In order to evaluate survival and mortality each form of AD must be considered separately and compared with an age- and sex-matched population.

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Journal
Journal of Endocrinological Investigation
Published
2026-05-22
DOI
https://doi.org/10.1007/s40618-026-02904-5
Primary Topic
Adrenal Hormones and Disorders
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article
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0.00

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article

Addison’s disease in Italy: mortality and survival by etiology

Antonella Meloni, Mariluce Barrasso, Mattia Barbot, Alessandro Mondin et al.
Journal of Endocrinological Investigation
Adrenal Hormones and Disorders
article

Addison’s disease in Italy: mortality and survival by etiology

Antonella Meloni, Mariluce Barrasso, Mattia Barbot, Alessandro Mondin, Guido Di Dalmazi, Antonio Stigliano, Fausto Bogazzi, Marco Cappa, Carlotta Keiko Vedolin, Giulia Marconcini, Silvia Longhi, Valentina Guarnotta, Fabio Presotto, Silvia Garelli, Elisabetta Cavedon, Valentina Camozzi, Roberto Perniola, Stefano Masiero, Alessandra Fierabracci, Chiara Sabbadin, C Scaroni, Alberto Falorni, Giuseppe Picca, Giuseppe Francia, Anna Maria De Bellis, Francesca Pigliaru, Malgorzata Wasniewska, Giorgio Radetti, Roberta Giordano, Corrado Betterle, Donatella Capalbo, Claudio Letizia, Chiara Simeoli, Carlo De Riva, Uberto Pagotto, Filippo Ceccato, Antonio Crinò, Alessandro Delitala, Carolina Salerno, Caterina Mian, Carla Giordano, Rosario Pivonello, Valentina Morelli
article en

Abstract

BACKGROUND: Data on survival and mortality in Addison's disease (AD) are contradictory: some studies suggest a mortality rate two times higher than in the general population, but this is not consistently reported in other studies. METHODS: We evaluated survival and mortality in 1,826 Italian adult patients with AD of different etiologies, collected from 1947 to 2024 across 30 Endocrine Units. These outcomes were compared to those of the age- and sex-matched Italian reference population using a standardized mortality ratio (SMR) in a subgroup of 1,575 cases. RESULTS: Sex and age adjusted survival analysis showed increased mortality in genetic AD, cancer-related-AD and cases with type 1 autoimmune polyendocrine syndrome (APS-1) compared to other etiologies. The SMR analysis demonstrated a significantly increased mortality risk of the overall patients with AD compared to the general population (SMR 1.34, p < 0.01). However, 4 etiologies presented survival similar to the general population: patients with type 2 or type 4 APS or isolated autoimmune AD (SMR 0.91, p = 0,57), vascular/post-tuberculosis AD (SMR 0.90, p = 0.69), bilateral adrenalectomy due to benign endocrine diseases (SMR 0.91. p = 0.64) and not defined AD (SMR 1.06, p = 0.93). These groups included 1,188 patients, representing 75.4% of the population. On the contrary, a significantly increased mortality risk compared to the general population was reported in the following 3 groups: patients with APS-1 (SMR 5.98, p < 0.01), genetic AD (SMR 5.09, p < 0.01), and cancer-related AD (SMR 4.64, p < 0.01), accounting for 387 patients (24.5%). CONCLUSION: Our study has shown that in adult patients with AD there is a large difference in survival and mortality based on the various etiological forms. In order to evaluate survival and mortality each form of AD must be considered separately and compared with an age- and sex-matched population.

Journal of Endocrinological Investigation
University of Foggia (IT), University of Pisa (IT), University of Messina (IT), University of Verona (IT), University of Padua (IT), University of Cagliari (IT), University of Perugia (IT), Agostino Gemelli University Polyclinic (IT), Ospedale di Bolzano (IT), Ospedali Riuniti di Foggia (IT), Azienda Ospedaliero Universitaria di Sassari (IT), Istituto Nazionale di Fisica Nucleare, Sezione di Napoli (IT), Federico II University Hospital (IT), Azienda USL di Bologna (IT), Bambino Gesù Children's Hospital (IT), Azienda Ospedaliera Sant'Andrea (IT), Ospedale Microcitemico (IT), University of Campania "Luigi Vanvitelli" (IT), Department of Medical Sciences (BY), IRCCS Istituto Auxologico Italiano (IT), Ospedale dell' Angelo (IT), Azienda Ospedaliera Universitaria Policlinico "Paolo Giaccone" di Palermo (IT), Ospedale Vito Fazzi (IT), Link Campus University (IT), University of Naples Federico II (IT), Sapienza University of Rome (IT), University of Bologna (IT)
Università degli Studi di Padova
Good health and well-being
Openalex Percentile: Top 6%
Adrenal Hormones and Disorders
DOIOpen Access PDFOpenAlex
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