PAI-BEL: a Belgian multicentre survey of primary adrenal insufficiency

Author:

Driessens Natacha1ORCID,Prasai Madhu1,Alexopoulou Orsalia2,De Block Christophe3ORCID,Van Caenegem Eva4,T’Sjoen Guy5ORCID,Nobels Frank6,Ghys Christophe7,Vroonen Laurent8,Jonas Corinne9,Corvilain Bernard1,Maiter Dominique2ORCID

Affiliation:

1. Université libre de Bruxelles (ULB), Hôpital Universitaire de Bruxelles (HUB), CUB Hôpital Erasme, Department of Endocrinology, Route de Lennik, Brussels, Belgium

2. Department of Endocrinology, Cliniques Universitaires Saint Luc, Brussels, Belgium

3. Department of Endocrinology-Diabetology-Metabolism, Universitair Ziekenhuis Antwerpen & University of Antwerp, Drie Eikenstraat, Edegem, Belgium

4. Department of Endocrinology, Academisch Ziekenhuis Sint-Jan Brugge – Oostende AV, Ruddershove, Brugge, Belgium

5. Department of Endocrinology, Ghent Universitary Hospital, C. Heymanslaan, Gent, Belgium

6. Department of Endocrinology, Onze-Lieve Vrouw Ziekenhuis, Moorselbaan, Aalst, Belgium

7. Department of Endocrinology, Universitair Ziekenhuis Brussel, Laarbeeklaan, Brussels, Belgium

8. Department of Endocrinology, Cliniques Universitaires de Liège, Avenue de l’hôpital, Liège, Belgium

9. Department of Endocrinology, CHU UCL Namur - Godinne, Avenue Docteur Gaston Thérasse, Yvoir, Belgium

Abstract

Objective Primary adrenal insufficiency (PAI) is a rare disease with an increasing prevalence, which may be complicated by life-threatening adrenal crisis (AC). Good quality epidemiological data remain scarce. We performed a Belgian survey to describe the aetiology, clinical characteristics, treatment regimens, comorbidities and frequency of AC in PAI. Methods A nationwide multicentre study involving 10 major university hospitals in Belgium collected data from adult patients with known PAI. Results Two hundred patients were included in this survey. The median age at diagnosis was 38 years (IQR 25–48) with a higher female prevalence (F/M sex ratio = 1.53). The median disease duration was 13 years (IQR 7–25). Autoimmune disease was the most common aetiology (62.5%) followed by bilateral adrenalectomy (23.5%) and genetic variations (8.5%). The majority (96%) of patients were treated with hydrocortisone at a mean daily dose of 24.5 ± 7.0 mg, whereas 87.5% of patients also received fludrocortisone. About one-third of patients experienced one or more AC over the follow-up period, giving an incidence of 3.2 crises per 100 patient-years. There was no association between the incidence of AC and the maintenance dose of hydrocortisone. As high as 27.5% of patients were hypertensive, 17.5% had diabetes and 17.5% had a diagnosis of osteoporosis. Conclusion This study provides the first information on the management of PAI in large clinical centres in Belgium, showing an increased frequency of postsurgical PAI, a nearly normal prevalence of several comorbidities and an overall good quality of care with a low incidence of adrenal crises, compared with data from other registries.

Publisher

Bioscientifica

Subject

Endocrinology,Endocrinology, Diabetes and Metabolism,Internal Medicine

Reference31 articles.

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2. Adrenal insufficiency: review of clinical outcomes with current glucocorticoid replacement therapy;Johannsson,2015

3. Mortality data from the European Adrenal Insufficiency Registry-Patient characterization and associations;Quinkler,2018

4. Clinical, immunological, and genetic features of autoimmune primary adrenal insufficiency: observations from a Norwegian registry;Erichsen,2009

5. Primary adrenal insufficiency - quality of life and long-term outcome with current treatment strategies;Esposito,2022

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1. Iatrogenic adrenal insufficiency in adults;Nature Reviews Endocrinology;2024-01-25

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