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

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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.

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References

  1. 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.2017.12.006

    Article  CAS  PubMed  Google Scholar 

  2. 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.2016.1205486

    Article  CAS  PubMed  Google Scholar 

  3. 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.14601

    Article  CAS  PubMed  Google Scholar 

  4. 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.27568

    Article  CAS  PubMed  Google Scholar 

  5. 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/keaa010

    Article  Google Scholar 

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

    CAS  PubMed  Google Scholar 

  7. 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/10.1097/00005792-200301000-00006

    Article  Google Scholar 

  8. 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/10.1183/13993003.00457-2016

    Article  PubMed  Google Scholar 

  9. 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.0b013e3182428126

    Article  Google Scholar 

  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/s00296-015-3310-5

    Article  PubMed  Google Scholar 

  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.0b013e3283410088

    Article  PubMed  Google Scholar 

  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/s13075-020-2120-7

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  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.2020.11.007

    Article  CAS  PubMed  Google Scholar 

  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.0000000000017735

    Article  Google Scholar 

  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:S30-35

    PubMed  Google Scholar 

  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.2007.11.001

    Article  PubMed  Google Scholar 

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Acknowledgements

We thank the CépiDc for the help in collecting the data and Philip Robinson (DRCI, Hospices Civils de Lyon) for the help in manuscript preparation.

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Authors and Affiliations

  1. Département de Médecine Interne, Hôpital de La Croix-Rousse, Hospices Civils de Lyon, Lyon, France

    Emmanuelle David, Mathieu Gerfaud-Valentin, Pascal Sève & Yvan Jamilloux

  2. Département de Biostatistique - Bioinformatique, Pôle Santé Publique, Hospices Civils de Lyon, Lyon, France

    Mathieu Fauvernier & Delphine Maurcort-Boulch

  3. Laboratoire de Biométrie Et Biologie Évolutive, UMR 5558, Équipe Biostatistique-Santé, Université Lyon 1, CNRS, Villeurbanne, France

    Mathieu Fauvernier

  4. Département de Médecine Interne Et Immunologie Clinique, Groupe Hospitalier Pitié-Salpêtrière, Assistance publique – Hôpitaux de Paris, Paris, France

    David Saadoun

  5. Center National de Références Maladies Autoimmunes Systémiques Rares, Center National de Références Maladies Autoinflammatoires Et Amylose Inflammatoire, Inflammation-Immunopathology-Biotherapy Department (DMU 3iD), Groupe Hospitalier Pitié-Salpêtrière, Assistance publique – Hôpitaux de Paris, INSERM 959, Paris, France

    David Saadoun

  6. Research On Healthcare Performance (RESHAPE), Université Claude Bernard, INSERM U1290Lyon 1, Lyon, France

    Pascal Sève

  7. Lyon Immunology Federation (LIFE), Lyon, France

    Yvan Jamilloux

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  1. Emmanuelle David
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  2. Mathieu Fauvernier
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Correspondence to Yvan Jamilloux.

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David, E., Fauvernier, M., Saadoun, D. et al. Mortality associated with Behçet’s disease in France assessed by multiple-cause-of-death analysis. Clin Rheumatol 41, 1749–1758 (2022). https://doi.org/10.1007/s10067-021-06027-5

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  • DOI: https://doi.org/10.1007/s10067-021-06027-5

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