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Original article

Vol. 149 No. 2930 (2019)

Behçet’s syndrome: clinical presentation and prevalence in Switzerland

  • Rahel A. Villiger
  • Anna-Luisa Stefanski
  • Véronique Grobéty
  • Sabine Adler
  • Peter M. Villiger
DOI
https://doi.org/10.4414/smw.2019.20072
Cite this as:
Swiss Med Wkly. 2019;149:w20072
Published
22.07.2019

Summary

OBJECTIVE

Behçet’s syndrome is a rare systemic autoimmune/autoinflammatory disease affecting mucocutaneous tissues, the skin and the eyes, as well as the joints, the central nervous system, the gastrointestinal tract and blood vessels. Because of the lack of clinical data in Switzerland, the aims of this cohort study were to calculate the disease prevalence and to analyse the disease manifestations and the immune-suppressive medication.

METHODS

Data were extracted from 52 patient charts. Thereafter, all patients were interviewed with a questionnaire and 46 had an additional physical examination and laboratory analyses. For calculation of prevalence, data of the national statistical bureau were used.

RESULTS

A disease prevalence of 4.03/100,000 inhabitants was calculated. The mean delay between first disease manifestation and diagnosis was 8 years. It was 2 years longer for Swiss than for non-Swiss individuals (p = 0.45). The time intervals between diagnosis and occurrence of different organ manifestations ranged from +8 to −11 years. There was no difference in organ involvement between different ethnicities. Colchicine was prescribed for 52% of patients only, whereas tumour necrosis factor (TNF) inhibitors and glucocorticoids were most frequently prescribed (80 and 64%, respectively). In almost half of the patients, TNF blockers could be stopped and replaced by conventional immunosuppressive drugs.

CONCLUSION

The data from this cohort of Behçet’s syndrome patients, the largest in Switzerland, documents a prevalence higher than anticipated. The diagnostic delay underlines an urgent need to improve awareness of the disease and allow timely treatment.

References

  1. Behçet H. Über rezidivierende aphtöse, durch Virus verursachte Geschwüre am Mund, am Auge und an den Genitalien. Dermatol Monatsschr. 1937;105:1152–7.
  2. Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013;65(1):1–11. doi:.https://doi.org/10.1002/art.37715
  3. Wechsler B, Davatchi F, Mizushima Y, et al.; International Study Group for Behçet’s Disease. Criteria for diagnosis of Behçet’s disease. Lancet. 1990;335(8697):1078–80.
  4. Davatchi F, Assaad-Khalil S, Calamia KT, Crook JE, Sadeghi-Abdollahi B, Schirmer M, et al. The International Criteria for Behçet’s Disease (ICBD): a collaborative study of 27 countries on the sensitivity and specificity of the new criteria. J Eur Acad Dermatol Venereol. 2014;28(3):338–47 https://doi.org/10.1111/jdv.12107
  5. Meguro A, Inoko H, Ota M, Katsuyama Y, Oka A, Okada E, et al. Genetics of Behçet disease inside and outside the MHC. Ann Rheum Dis. 2010;69(4):747–54. doi:.https://doi.org/10.1136/ard.2009.108571
  6. Turan B, Gallati H, Erdi H, Gürler A, Michel BA, Villiger PM. Systemic levels of the T cell regulatory cytokines IL-10 and IL-12 in Bechçet’s disease; soluble TNFR-75 as a biological marker of disease activity. J Rheumatol. 1997;24(1):128–32.
  7. Geri G, Terrier B, Rosenzwajg M, Wechsler B, Touzot M, Seilhean D, et al. Critical role of IL-21 in modulating TH17 and regulatory T cells in Behçet disease. J Allergy Clin Immunol. 2011;128(3):655–64. doi:.https://doi.org/10.1016/j.jaci.2011.05.029
  8. Lopalco G, Lucherini OM, Vitale A, Talarico R, Lopalco A, Galeazzi M, et al. Putative Role of Serum Amyloid-A and Proinflammatory Cytokines as Biomarkers for Behcet’s Disease. Medicine (Baltimore). 2015;94(42):e1858. doi:.https://doi.org/10.1097/MD.0000000000001858
  9. Desbois AC, Vallet H, Domont F, Comarmond C, Cacoub P, Saadoun D. Management of severe complications in Behçet’s disease with TNF inhibitors. Expert Opin Biol Ther. 2017;17(7):853–9. doi:.https://doi.org/10.1080/14712598.2017.1328496
  10. Davatchi F, Chams-Davatchi C, Shams H, Shahram F, Nadji A, Akhlaghi M, et al. Behcet’s disease: epidemiology, clinical manifestations, and diagnosis. Expert Rev Clin Immunol. 2017;13(1):57–65. doi:.https://doi.org/10.1080/1744666X.2016.1205486
  11. Maldini C, Druce K, Basu N, LaValley MP, Mahr A. Exploring the variability in Behçet’s disease prevalence: a meta-analytical approach. Rheumatology (Oxford). 2018;57(1):185–95. doi:.https://doi.org/10.1093/rheumatology/kew486
  12. Tursen U, Gurler A, Boyvat A. Evaluation of clinical findings according to sex in 2313 Turkish patients with Behçet’s disease. Int J Dermatol. 2003;42(5):346–51. doi:.https://doi.org/10.1046/j.1365-4362.2003.01741.x
  13. Alpsoy E, Dönmez L, Önder M, Gunasti S, Usta A, Karincaoglu Y, et al. Clinical features and natural course of Behçet’s disease in 661 cases: a multicentre study. Br J Dermatol. 2007;157(5):901–6. doi:.https://doi.org/10.1111/j.1365-2133.2007.08116.x
  14. Hatemi G, Silman A, Bang D, Bodaghi B, Chamberlain AM, Gul A, et al.; EULAR Expert Committee. EULAR recommendations for the management of Behçet disease. Ann Rheum Dis. 2008;67(12):1656–62. doi:.https://doi.org/10.1136/ard.2007.080432
  15. Table of the Bundesamt für Statistik, as of 31 December 2016: Bilanz der ständigen Wohnbevölkerung nach institutionellen Gliederungen, Staatsangehörigkeit (Kategorie), Geschlecht und demographischen Komponenten. Published 30 August 2017; BFS-Nummer: px-x-0102020000_201; Available at: https://www.bfs.admin.ch/bfs/de/home/statistiken/kataloge-datenbanken/tabellen.assetdetail.3262106.html
  16. Table of the Bundesamt für Statistik, as of 31 December 2015; Regionalporträts 2017: Kennzahlen aller Gemeinden. Published 18 May 2017; BFS-Nummer: je-d-21.03.01; Available at: https://www.bfs.admin.ch/bfs/de/home/statistiken/regionalstatistik/regionale-portraets-kennzahlen/gemeinden/daten-erlaeuterungen.assetdetail.2422865.html
  17. Table of the Bundesamt für Statistik. as of 31 December 2015; Ausgewählte Indikatoren im regionalen Vergleich, 2017 (Kantone). Published 28 March 2017; BFS-Nummer: je-d-21.03.02; Available at: https://www.bfs.admin.ch/bfs/de/home/statistiken/regionalstatistik/regionale-portraets-kennzahlen/kantone.assetdetail.1922812.html
  18. Shapefiles of the Bundesamt für Landestopografie swisstopo; „swissBOUNDARIES3D“, as of January 2018; Link: https://shop.swisstopo.admin.ch/de/products/landscape/boundaries3D
  19. Mohammad A, Mandl T, Sturfelt G, Segelmark M. Incidence, prevalence and clinical characteristics of Behcet’s disease in southern Sweden. Rheumatology (Oxford). 2013;52(2):304–10. doi:.https://doi.org/10.1093/rheumatology/kes249
  20. Kötter I, Vonthein R, Müller CA, Günaydin I, Zierhut M, Stübiger N. Behçet’s disease in patients of German and Turkish origin living in Germany: a comparative analysis. J Rheumatol. 2004;31(1):133–9.
  21. Hatemi G, Melikoglu M, Tunc R, Korkmaz C, Turgut Ozturk B, Mat C, et al. Apremilast for Behçet’s syndrome--a phase 2, placebo-controlled study. N Engl J Med. 2015;372(16):1510–8. doi:.https://doi.org/10.1056/NEJMoa1408684
  22. Adler S, Baumgartner I, Villiger PM. Behçet’s disease: successful treatment with infliximab in 7 patients with severe vascular manifestations. A retrospective analysis. Arthritis Care Res (Hoboken). 2012;64(4):607–11. doi:.https://doi.org/10.1002/acr.21557
  23. Yurdakul S, Mat C, Tüzün Y, Ozyazgan Y, Hamuryudan V, Uysal O, et al. A double-blind trial of colchicine in Behçet’s syndrome. Arthritis Rheum. 2001;44(11):2686–92. doi:.https://doi.org/10.1002/1529-0131(200111)44:11<2686::AID-ART448>3.0.CO;2-H
  24. Kötter I, Zierhut M, Eckstein AK, Vonthein R, Ness T, Günaydin I, et al. Human recombinant interferon alfa-2a for the treatment of Behçet’s disease with sight threatening posterior or panuveitis. Br J Ophthalmol. 2003;87(4):423–31. doi:.https://doi.org/10.1136/bjo.87.4.423
  25. Alpsoy E, Durusoy C, Yilmaz E, Ozgurel Y, Ermis O, Yazar S, et al. Interferon alfa-2a in the treatment of Behçet disease: a randomized placebo-controlled and double-blind study. Arch Dermatol. 2002;138(4):467–71. doi:.https://doi.org/10.1001/archderm.138.4.467
  26. Baki K, Villiger PM, Jenni D, Meyer T, Beer JH. Behcet’s disease with life-threatening haemoptoe and pulmonary aneurysms: complete remission after infliximab treatment. Ann Rheum Dis. 2006;65(11):1531–2. doi:.https://doi.org/10.1136/ard.2005.045195

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