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Behçet disease.

H Yazici1, S Yurdakul, V Hamuryudan

  • 1Division of Rheumatology, Department of Medicine. Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey. hyazici@attglobal.net

Current Opinion in Rheumatology
|January 10, 2001
PubMed
Summary

Behçet disease (BD) central nervous system involvement, particularly parenchymal, indicates a worse prognosis. While HLA B51 is implicated, familial aggregation and coagulation factors require further study for BD pathogenesis.

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Area of Science:

  • Neurology
  • Immunology
  • Genetics

Background:

  • Behçet disease (BD) can affect the central nervous system (CNS) through parenchymal or nonparenchymal involvement.
  • Parenchymal CNS involvement and abnormal cerebrospinal fluid findings are linked to a poorer prognosis in BD patients.
  • The human leukocyte antigen B51 (HLA B51) is a potential self-antigen in BD pathogenesis, with a 3.6% sibling occurrence rate.

Purpose of the Study:

  • To review the central nervous system manifestations of Behçet disease.
  • To discuss the prognostic factors associated with CNS involvement in BD.
  • To explore the potential roles of genetic and coagulation abnormalities in BD pathogenesis and management.

Main Methods:

  • Literature review of studies on Behçet disease and central nervous system involvement.
  • Analysis of prognostic indicators, including cerebrospinal fluid findings and parenchymal disease.
  • Discussion of the proposed role of HLA B51 and familial aggregation in BD.

Main Results:

  • Central nervous system involvement in BD carries a significant prognostic weight, especially parenchymal forms.
  • Familial aggregation is noted, potentially more so in pediatric BD cases.
  • Vasculitis of the vasa vasorum is identified as a key pathology in large vessel disease within BD.

Conclusions:

  • Optimal treatment strategies for BD, particularly neuro-Behçet disease, remain debated.
  • Low-dose thalidomide shows efficacy for mucocutaneous lesions but carries a risk of polyneuropathy.
  • Cyclosporine A's neurological side effects necessitate caution in CNS-involved BD patients; surgical risks due to heightened inflammatory responses are manageable.

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