Top

Published in:

01-06-2022 | Original Article

Mortality associated with Behçet’s disease in France assessed by multiple-cause-of-death analysis

Authors: Emmanuelle David, Mathieu Fauvernier, David Saadoun, Mathieu Gerfaud-Valentin, Delphine Maurcort-Boulch, Pascal Sève, Yvan Jamilloux

Published in: Clinical Rheumatology | Issue 6/2022

Abstract

Objectives

To examine the mortality rates and causes of death among French decedents with Behçet’s disease (BD).

Methods

Data collected between 1979 and 2016 by the French Epidemiological Center for the Medical Causes of Death was used to calculate BD-related mortality rates and examine differences according to age and sex, determine underlying and non-underlying causes of death (UCD/NUCD), and compare with the general population (observed/expected ratios, O/E).

Results

A total of 328 death certificates mentioned BD as UCD (n = 171) or NUCD (n = 157). The mean (± SD) age at death was 52.8 (± 11.3) years in men and 58.4 (± 16.5) in women (vs. 71.2 and 79.8 in the general population, respectively). The age-standardized mortality rate was 0.15/million person-years and significantly increased over the study period (p < 0.01). When BD was the UCD, the most frequent associated causes were infections (25.1%), underlying organ dysfunction (21.6%), and arterial events (15.2%). As compared to the general population, BD decedents < 45 years were more likely to die from a cardiovascular event (O/E = 6.25, p < 0.001); decedents > 45 years were more likely to die from infection (O/E = 9.69, p < 0.001). Between 1979 and 2016, BD as UCD decreases whereas NUCD increased.

Conclusion

This study found an earlier mean age at death for BD compared to the general population; young patients were more prone to die from cardiovascular complication whereas older patients died more frequently from infections. Over the years, the mean age at death increased and BD was less frequently considered as an UCD, suggesting a better knowledge of the disease and complications.

Key Points

• Patients with Behçet’s disease die earlier than the general population.

• Young patients were more prone to die from cardiovascular complication.

• Older patients died more frequently from infections.

Greco A, De Virgilio A, Ralli M, Ciofalo A, Mancini P, Attanasio G et al (2018) Behçet’s disease: new insights into pathophysiology, clinical features and treatment options. Autoimmun Rev 17:567–575. https://doi.org/10.1016/j.autrev.2017.12.006CrossRefPubMed

Davatchi F, Chams-Davatchi C, Shams H, Shahram F, Nadji A, Akhlaghi M et al (2017) Behcet’s disease: epidemiology, clinical manifestations, and diagnosis. Expert Rev Clin Immunol 13:57–65. https://doi.org/10.1080/1744666X.2016.1205486CrossRefPubMed

Lee YB, Lee SY, Choi JY, Lee JH, Chae HS, Kim JW et al (2018) Incidence, prevalence, and mortality of Adamantiades-Behçet’s disease in Korea: a nationwide, population-based study (2006–2015). J Eur Acad Dermatol Venereol JEADV 32:999–1003. https://doi.org/10.1111/jdv.14601CrossRefPubMed

Saadoun D, Wechsler B, Desseaux K, Le Thi HD, Amoura Z, Resche-Rigon M et al (2010) Mortality in Behçet’s disease. Arthritis Rheum 62:2806–2812. https://doi.org/10.1002/art.27568CrossRefPubMed

Thomas T, Chandan JS, Subramanian A, Gokhale K, Gkoutos G, Harper L et al (2020) Epidemiology, morbidity and mortality in Behçet’s disease: a cohort study using The Health Improvement Network (THIN). Rheumatol Oxf Engl. https://doi.org/10.1093/rheumatology/keaa010CrossRef

Yazici H, Esen F (2008) Mortality in Behçet’s syndrome. Clin Exp Rheumatol 26:S138-140PubMed

Kural-Seyahi E, Fresko I, Seyahi N, Ozyazgan Y, Mat C, Hamuryudan V et al (2003) The long-term mortality and morbidity of Behçet syndrome: a 2-decade outcome survey of 387 patients followed at a dedicated center. Medicine (Baltimore) 82:60–76. https://doi.org/10.1097/00005792-200301000-00006CrossRef

Jamilloux Y, Maucort-Boulch D, Kerever S, Gerfaud-Valentin M, Broussolle C, Eb M et al (2016) Sarcoidosis-related mortality in France: a multiple-cause-of-death analysis. Eur Respir J 48:1700–1709. https://doi.org/10.1183/13993003.00457-2016CrossRefPubMed

Wood S (2017) Generalized additive models: an introduction with R, Second Edition. Undefined

10.

Saadoun D, Asli B, Wechsler B, Houman H, Geri G, Desseaux K et al (2012) Long-term outcome of arterial lesions in Behçet disease: a series of 101 patients. Medicine (Baltimore) 91:18–24. https://doi.org/10.1097/MD.0b013e3182428126CrossRef

11.

Ugurlu N, Bozkurt S, Bacanli A, Akman-Karakas A, Uzun S, Alpsoy E (2015) The natural course and factors affecting severity of Behçet’s disease: a single-center cohort of 368 patients. Rheumatol Int 35:2103–2107. https://doi.org/10.1007/s00296-015-3310-5CrossRefPubMed

12.

Calamia KT, Schirmer M, Melikoglu M (2011) Major vessel involvement in Behçet’s disease: an update. Curr Opin Rheumatol 23:24–31. https://doi.org/10.1097/BOR.0b013e3283410088CrossRefPubMed

13.

Stewart S, Yang KCK, Atkins K, Dalbeth N, Robinson PC (2020) Adverse events during oral colchicine use: a systematic review and meta-analysis of randomised controlled trials. Arthritis Res Ther 22:28. https://doi.org/10.1186/s13075-020-2120-7CrossRefPubMedPubMedCentral

14.

McEwan T, Robinson PC (2021) A systematic review of the infectious complications of colchicine and the use of colchicine to treat infections. Semin Arthritis Rheum 51:101–112. https://doi.org/10.1016/j.semarthrit.2020.11.007CrossRefPubMed

15.

Wang X, Peng Y, Gao J, Han S, Li Y (2019) Risk of malignancy in Behcet disease: a meta-analysis with systematic review. Medicine (Baltimore) 98:e17735. https://doi.org/10.1097/MD.0000000000017735CrossRef

16.

Épidémiologie France Suicides – Infosuicide.org n.d. https://www.infosuicide.org/reperes/epidemiologie/epidemiologie-france-suicides/. Accessed Feb 17 2021.

17.

Saygin C, Uzunaslan D, Hatemi G, Hamuryudan V (2015) Suicidal ideation among patients with Behçet’s syndrome. Clin Exp Rheumatol 33:S30-35PubMed

18.

Akpolat T, Dilek M, Aksu K, Keser G, Toprak O, Cirit M et al (2008) Renal Behçet’s disease: an update. Semin Arthritis Rheum 38:241–248. https://doi.org/10.1016/j.semarthrit.2007.11.001CrossRefPubMed

Title: Mortality associated with Behçet’s disease in France assessed by multiple-cause-of-death analysis
Authors: Emmanuelle David
Mathieu Fauvernier
David Saadoun
Mathieu Gerfaud-Valentin
Delphine Maurcort-Boulch
Pascal Sève
Yvan Jamilloux
Publication date: 01-06-2022
Publisher: Springer International Publishing
Published in: Clinical Rheumatology / Issue 6/2022
Print ISSN: 0770-3198
Electronic ISSN: 1434-9949
DOI: https://doi.org/10.1007/s10067-021-06027-5

Live Webinar | 27-06-2024 | 18:00 (CEST)

Keynote webinar | Spotlight on medication adherence

Reserve your place

Live: Thursday 27th June 2024, 18:00-19:30 (CEST)

WHO estimates that half of all patients worldwide are non-adherent to their prescribed medication. The consequences of poor adherence can be catastrophic, on both the individual and population level.

Join our expert panel to discover why you need to understand the drivers of non-adherence in your patients, and how you can optimize medication adherence in your clinics to drastically improve patient outcomes.

Prof. Kevin Dolgin

Prof. Florian Limbourg

Prof. Anoop Chauhan

Developed by: Springer Medicine

Obesity Clinical Trial Summary

At a glance: The STEP trials

A round-up of the STEP phase 3 clinical trials evaluating semaglutide for weight loss in people with overweight or obesity.

Developed by: Springer Medicine

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Objectives

Methods

Results

Conclusion

Please log in to get access to this content

Other articles of this Issue 6/2022

Erosive Hand OsteoArthritis (EHOA): analysis of consecutive patients presenting with EHOA in a hospital-based rheumatology practice and its implications for an upcoming interventional study

Persistent enlargement of the pancreatic gland after glucocorticoid therapy increases the risk of relapse in IgG4-related autoimmune pancreatitis

Efficacy and safety of corticosteroid in the treatment of hand osteoarthritis: a systematic review and meta-analysis of randomized controlled trials

Kawasaki disease does not affect coronaries alone: large vessels can be involved as well

A case of eosinophilic granulomatosis with polyangiitis presented with blisters on the tongue

Symptoms related to gastrointestinal tract involvement and low muscularity in systemic sclerosis

Keynote webinar | Spotlight on medication adherence

Live: Thursday 27th June 2024, 18:00-19:30 (CEST)

At a glance: The STEP trials