Skip to main content

Advertisement

SpringerLink
Log in

The natural course and factors affecting severity of Behçet’s disease: a single-center cohort of 368 patients

  • Short Communication - Observational Research
  • Published:
Rheumatology International Aims and scope Submit manuscript

Abstract

Our goal was to determine, retrospectively, the occurrence of the symptoms of Behçet’s disease in chronological order and the course of the disease. Additionally, probable factors affecting the clinical severity were investigated. A total of 368 patients (171 females and 197 males; aged 41.11 ± 10.9 years) were included in this retrospective cohort study. The chronological order of the clinical manifestations was recorded. Patients were also assessed for clinical severity score. Oral ulcer was the most common manifestation (100 %) followed by genital ulcer (89.4 %), papulopustular lesions (75 %) and articular involvement (60.1 %). Oral ulcer was the most common onset manifestation (66.8 %) followed by genital ulcer (4.9 %), erythema nodosum (3.3 %) and ocular involvement (1.4 %). The duration between the onset symptom and the fulfillment of the diagnostic criteria was 4.67 ± 5.9 years. The duration between the time point of fulfillment of diagnostic criteria and the diagnosis (2.5 ± 2.1 years) was longer in patients having only mucocutaneous lesions (2.8 ± 2.2 years) than in patients having serious organ involvements (1.9 ± 1.6 years; p < 0.01). Serious involvements such as neurological involvement and large vessel involvement had their onsets later. Mean clinical severity score was higher in male patients (5.3 ± 2.1 vs 4.8 ± 1.7; p < 0.05). In logistic regression analysis, male gender (p = 0.03) and increased number of symptoms at diagnosis (p < 0.001, R 2 = 0.73) were found to be significant risk factors for severity. Mucocutaneous lesions, especially oral and/or genital ulcers, usually precede possible serious involvements; therefore, careful follow-up is mandatory. Males with increased number of organ involvements at the diagnosis are associated with more severe disease.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

References

  1. Alpsoy E, Kodelja V, Goerdt S et al (2003) Serum of patients with Behçet’s disease induces classical (pro-inflammatory) activation of human macrophages in vitro. Dermatology 206:225–232

    Article  CAS  PubMed  Google Scholar 

  2. Yazici H, Tuzun Y, Pazarli H et al (1984) Influence of age of onset and patient’s sex on the prevalence and severity of manifestations of Behçet’s syndrome. Ann Rheum Dis 43:783–789

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  3. Zouboulis CC, Vaiopoulos G, Marcomichelakis N et al (2003) Onset signs, clinical course, prognosis, treatment and outcome of adult patients with Adamantiades–Behcet’s disease in Greece. Clin Exp Rheumatol 21(4 Suppl 30):S19–S26

    CAS  PubMed  Google Scholar 

  4. Kural-Seyahi E, Fresko I, Seyahi N et al (2003) The long-term mortality and morbidity of Behcet syndrome: a 2-decade outcome survey of 387 patients followed at a dedicated center. Medicine (Baltimore) 82:60–76

    Article  Google Scholar 

  5. International Study Group for Behçet’s disease (1990) Criteria for diagnosis of Behçet’s disease. Lancet 335:1078–1080

    Google Scholar 

  6. Krause I, Mader R, Sulkes J et al (2001) Behçet’s disease in Israel: the influence of ethnic origin on disease expression and severity. J Rheumatol 28:1033–1036

    CAS  PubMed  Google Scholar 

  7. Cho SB, Cho S, Bang D (2012) New insights in the clinical understanding of Behçet’s disease. Yonsei Med J 53:35–42

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  8. Vaiopoulos G, Konstantopoulou P, Evangelatos N et al (2010) The spectrum of mucocutaneous manifestations in Adamantiades–Behçet’s disease in Greece. J Eur Acad Dermatol Venereol 24:434–438

    Article  CAS  PubMed  Google Scholar 

  9. El Menyawi MM, Raslan HM, Edrees A (2009) Clinical features of Behcet’s disease in Egypt. Rheumatol Int 29:641–646

    Article  PubMed  Google Scholar 

  10. Pipitone N, Boiardi L, Olivieri I et al (2004) Clinical manifestations of Behçet’s disease in 137 Italian patients: results of a multicenter study. Clin Exp Rheumatol 22(6 Suppl 36):S46–S51

    CAS  PubMed  Google Scholar 

  11. Salvarani C, Pipitone N, Catanoso MG et al (2007) Epidemiology and clinical course of Behçet’s disease in the Reggio Emilia area of Northern Italy: a seventeen-year population-based study. Arthritis Rheum 57:171–178

    Article  PubMed  Google Scholar 

  12. Shafie N, Shahram F, Nadji A et al (1997) Iran’s aspects of Behçet’s disease in children. The 1996 survey. In: Hamsa M (ed) Behçet’s disease. Pub Adhoua, Tunis, pp 125–129

    Google Scholar 

  13. Krause I, Uziel Y, Guedj D et al (1998) Mode of presentation and multisystem involvement in Behçet’s disease: the influence of sex and age of disease onset. J Rheumatol 25:1566–1569

    CAS  PubMed  Google Scholar 

  14. Ideguchi H, Suda A, Takeno M et al (2011) Behçet disease: evolution of clinical manifestations. Medicine (Baltimore) 90:125–132

    Article  Google Scholar 

  15. Wang LY, Zhao DB, Gu J et al (2010) Clinical characteristics of Behçet’s disease in China. Rheumatol Int 30:1191–1196

    Article  CAS  PubMed  Google Scholar 

  16. Zhang Z, He F, Shi Y (2013) Behcet’s disease seen in China: analysis of 334 cases. Rheumatol Int 33:645–648

    Article  CAS  PubMed  Google Scholar 

  17. Alpsoy E, Donmez L, Bacanli A et al (2003) Review of the chronology of clinical manifestations in 60 patients with Behcet’s disease. Dermatology 207:354–356

    Article  PubMed  Google Scholar 

  18. Alpsoy E, Donmez L, Onder M et al (2007) Clinical features and natural course of Behçet’s disease in 661 cases: a multicentre study. Br J Dermatol 157:901–906

    Article  CAS  PubMed  Google Scholar 

  19. Sachetto Z, Mahayri N, Ferraz RH et al (2012) Behçet’s disease in Brazilian patients: demographic and clinical features. Rheumatol Int 32:2063–2067

    Article  PubMed  Google Scholar 

  20. Koç Y, Güllü I, Akpek G et al (1992) Vascular involvement in Behcet’s disease. J Rheumatol 19:402–410

    PubMed  Google Scholar 

  21. Yazici H, Tüzün Y, Tanman AB et al (1985) Male patients with Behçet’s syndrome have stronger pathergy reactions. Clin Exp Rheumatol 3:137–141

    CAS  PubMed  Google Scholar 

  22. Chang HK, Cheon KS (2002) The clinical significance of a pathergy reaction in patients with Behcet’s disease. J Korean Med Sci 17:371–374

    Article  PubMed Central  PubMed  Google Scholar 

  23. Bonitsis NG, Luong Nguyen LB, LaValley MP et al (2015) Gender-specific differences in Adamantiades–Behçet’s disease manifestations: an analysis of the German registry and meta-analysis of data from the literature. Rheumatology (Oxford) 54:121–133

    Article  Google Scholar 

  24. Bang DS, Oh SH, Lee KH et al (2003) Influence of sex on patients with Behcet’s disease in Korea. J Korean Med Sci 18:231–235

    Article  PubMed Central  PubMed  Google Scholar 

  25. Alli N, Gur G, Yalcin B et al (2009) Patient characteristics in Behçet disease: a retrospective analysis of 213 Turkish patients during 2001–4. Am J Clin Dermatol 10:411–418

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Dermatology and Venereology, Akdeniz University School of Medicine, 07059, Antalya, Turkey

    Nilay Ugurlu, Ayse Akman-Karakas, Soner Uzun & Erkan Alpsoy

  2. Department of Biostatistics and Medical Informatics, Akdeniz University School of Medicine, 07059, Antalya, Turkey

    Selen Bozkurt

  3. Department of Dermatology and Venereology, Antalya Education and Research Hospital, Antalya, Turkey

    Ali Bacanli

Authors
  1. Nilay Ugurlu
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Selen Bozkurt
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Ali Bacanli
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Ayse Akman-Karakas
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Soner Uzun
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Erkan Alpsoy
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Erkan Alpsoy.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

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

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00296-015-3310-5

Keywords

Search

Navigation