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CNS involvement occurs more frequently in patients with Behçet's disease under cyclosporin A (CSA) than under other medications—results of a retrospective analysis of 117 cases

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Abstract

The aim of this study was to evaluate the incidence of neurological manifestations of Behçet's disease (BD) in patients on cyclosporin A (CSA) compared with those on other medications. The records of 117 patients with BD who visited our hospital between 1990 and 2003 were reviewed with respect to symptoms and medication. All episodes of constant therapy prior to central nervous system (CNS) involvement were counted, and then the associations were analysed by the exact Fisher–Freeman–Halton test and adjusted for multiple tests by the Bonferroni–Holm method. We observed ten new cases of CNS manifestations in our patients with BD being regularly seen and treated in our tertiary care centre. The overall prevalence of neuro-BD in our patient group was 8.5%. In a retrospective analysis, the incidence of new-onset neurological disease (neuro-BD) in all patients with BD who regularly visited our hospital was significantly higher in patients on CSA than in those on other medications (6 of 21 vs 0 of 175 episodes, P<0.0001). This contrasts the obvious efficacy of CSA on extracerebral manifestations of BD, such as severe ocular disease, mucocutaneous lesions or arthritis. CSA exerts differential efficacy on various manifestations of BD. It is very effective for severe ocular and other moderate to severe manifestations of BD, but its efficacy for the prevention of neuro-BD seems to be inferior to that of other medications used in BD, such as azathioprine or interferon-α. The reasons for this are unclear, but the potential toxic effects of CSA on the CNS may be a predisposing factor for CNS vasculitis in BD.

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References

  1. International Study Group for Behcet's Disease (1990) Criteria for diagnosis of Behcet's disease. Lancet 335:1078–1080

    Google Scholar 

  2. Kidd D, Steuer A, Denman AM, Rudge P (1999) Neurological complications in Behcet's syndrome. Brain 122(Pt 11):2183–2194

    Article  PubMed  Google Scholar 

  3. Akman-Demir G, Serdaroglu P, Tasci B (1999) Clinical patterns of neurological involvement in Behcet's disease: evaluation of 200 patients. The Neuro-Behcet Study Group. Brain 122(Pt 11):2171–2182

    Article  PubMed  Google Scholar 

  4. Siva A, Kantarci OH, Saip S, et al (2001) Behcet's disease: diagnostic and prognostic aspects of neurological involvement. J Neurol 248:95–103

    Article  PubMed  CAS  Google Scholar 

  5. Dilsen N, Konice M, Aral O (1985) Our diagnostic criteria of Behcet's disease—an overview. In: Lehner T, Barnes CG (eds) Recent advances in Behçet's disease. Royal Society of Medicine Services, London, pp 177–180

    Google Scholar 

  6. Krause I, Rosen Y, Kaplan I, et al (1999) Recurrent aphthous stomatitis in Behcet's disease: clinical features and correlation with systemic disease expression and severity. J Oral Pathol Med 28:193–196

    Article  PubMed  CAS  Google Scholar 

  7. Kotter I, Vonthein R, Muller CA, Gunaydin I, Zierhut M, Stubiger N (2004) Behcet's disease in patients of German and Turkish origin living in Germany: a comparative analysis. J Rheumatol 31:133–139

    PubMed  Google Scholar 

  8. Saenz A, Ausejo M, Shea B, Wells G, Welch V, Tugwell P (2000) Pharmacotherapy for Behcet's syndrome. Cochrane Database Syst Rev 2:CD001084

    Google Scholar 

  9. Hinchey J, Chaves C, Appignani B, et al (1996) A reversible posterior leukoencephalopathy syndrome. N Engl J Med 334:494–500

    Article  PubMed  CAS  Google Scholar 

  10. Gijtenbeek JMM, van den Bent MJ, Vecht CJ (1999) Cyclosporine neurotoxicity: a review. J Neurol 246:339–346

    Article  PubMed  CAS  Google Scholar 

  11. Filley CM, Kleinschmidt-DeMasters BK (2001) Toxic leukoencephalopathy. N Engl J Med 345:425–432

    Article  PubMed  CAS  Google Scholar 

  12. Lee SH, Yoon PH, Park SJ, Kim DI (2001) MRI findings in neuro-Behçet's disease. Clin Radiol 56:485–494

    Article  PubMed  CAS  Google Scholar 

  13. Kato Y, Numaga J, Kato S, Kaburaki T, Kawashima H, Fujino Y (2001) Central nervous system symptoms in a population of Behcet's disease patients with refractory uveitis treated with cyclosporine A. Clin Experiment Ophthalmol 29:335–336

    Article  PubMed  CAS  Google Scholar 

  14. Kotake S, Higashi K, Yoshikawa K, Sasamoto Y, Okamoto T, Matsuda H (1999) Central nervous system symptoms in patients with Behcet disease receiving cyclosporine therapy. Ophthalmology 106:586–589

    Article  PubMed  CAS  Google Scholar 

  15. Meusser S, Eger G, Anders M, Burkhardt H, Kalden JR (1997) Manifestation of neuro-Behcet disease in cyclosporin A therapy. Z Rheumatol 56:31–39

    Article  PubMed  CAS  Google Scholar 

  16. Lannuzel A, Lamaury I, Charpentier D, Caparros-Lefebvre D (2002) Neurological manifestations of Behcet's disease in a Caribbean population: clinical and imaging findings. J Neurol 249:410–418

    Article  PubMed  Google Scholar 

  17. Serdaroglu P (1998) Behcet's disease and the nervous system. J Neurol 245:197–205

    Article  PubMed  CAS  Google Scholar 

  18. McLean BN, Miller D, Thompson EJ (1995) Oligoclonal banding of IgG in CSF, blood–brain barrier function, and MRI findings in patients with sarcoidosis, systemic lupus erythematosus, and Behcet's disease involving the nervous system. J Neurol Neurosurg Psychiatry 58:548–554

    PubMed  CAS  Google Scholar 

  19. Motomura S, Tabira T, Kuroiwa Y (1980) A clinical comparative study of multiple sclerosis and neuro-Behcet's syndrome. J Neurol Neurosurg Psychiatry 43:210–213

    Article  PubMed  CAS  Google Scholar 

  20. Krause I, Leibovici L, Guedj D, Molad Y, Uziel Y, Weinberger A (1999) Disease patterns of patients with Behcet's disease demonstrated by factor analysis. Clin Exp Rheumatol 17:347–350

    PubMed  CAS  Google Scholar 

  21. Yurdakul S, Mat C, Tuzun Y, et al (2001) A double-blind trial of colchicine in Behcet's syndrome. Arthritis Rheum 44:2686–2692

    Article  PubMed  CAS  Google Scholar 

  22. Hamuryudan V, Mat C, Saip S, et al (1998) Thalidomide in the treatment of the mucocutaneous lesions of the Behcet syndrome. A randomized, double-blind, placebo-controlled trial. Ann Intern Med 128:443–450

    PubMed  CAS  Google Scholar 

  23. Kotter I, Vonthein R, Zierhut M, et al (2004) Differential efficacy of human recombinant interferon-alpha2a on ocular and extraocular manifestations of Behcet disease: results of an open 4-center trial. Semin Arthritis Rheum 33:311–319

    Article  PubMed  CAS  Google Scholar 

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Acknowledgements

We thank Prof. Hasan Yazici for his critical review of the manuscript, and Prof. CA Mueller for the human leukocyte antigen (HLA) analyses.

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Authors and Affiliations

  1. Department of Internal Medicine II (Hematology/Oncology/Immunology/Rheumatology), University Hospital, Otfried-Müller. Str. 10, 72076, Tübingen, Germany

    Ina Kötter & Ilhan Günaydin

  2. Department of Neuro-Radiology, University Hospital, Tübingen, Germany

    Marion Batra

  3. Department of Medical Biometry, University Hospital, Tübingen, Germany

    Reinhard Vonthein

  4. Department of Ophthalmology, University Hospital, Tübingen, Germany

    Nicole Stübiger

  5. Department of Dermatology, University Hospital, Tübingen, Germany

    Gerhard Fierlbeck

  6. Department of Neurology, University Hospital, Tübingen, Germany

    Arthur Melms

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  1. Ina Kötter
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  2. Ilhan Günaydin
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  3. Marion Batra
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  4. Reinhard Vonthein
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  5. Nicole Stübiger
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  6. Gerhard Fierlbeck
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  7. Arthur Melms
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Correspondence to Ina Kötter.

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Kötter, I., Günaydin, I., Batra, M. et al. CNS involvement occurs more frequently in patients with Behçet's disease under cyclosporin A (CSA) than under other medications—results of a retrospective analysis of 117 cases. Clin Rheumatol 25, 482–486 (2006). https://doi.org/10.1007/s10067-005-0070-8

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  • DOI: https://doi.org/10.1007/s10067-005-0070-8

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