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Neurological manifestations of Behçet's disease: evaluation of 40 patients treated by cyclophosphamide
E H Ait Ben Haddou1, F Imounan, W Regragui
1Service de neurologie B et de neurogénétique, hôpital des spécialités, ONO, CHU de Rabat-Salé, BP 6444, Rabat-Instituts, Rabat, Maroc. elaitbenha@yahoo.fr
Insights
Aggressive cyclophosphamide treatment shows promise for severe neuro-Behçet's disease, improving outcomes and tolerance. Further multicenter studies are needed to confirm efficacy and long-term safety.
Area of Science:
- Neurology
- Rheumatology
- Immunology
Background:
- Neuro-Behçet's disease (NBD) is a severe complication of Behçet's disease, leading to significant morbidity and mortality.
- Neurological manifestations account for 4-49% of systemic Behçet's disease cases.
Purpose of the Study:
- To evaluate the efficacy and tolerance of aggressive cyclophosphamide treatment in severe NBD patients.
- To analyze the clinical presentation and outcomes of NBD.
Main Methods:
- Retrospective analysis of 40 severe NBD cases (June 2004 - December 2010).
- Patients received high-dose cyclophosphamide (600 mg/m(2) BSA) pulses for 2 years.
- Clinical, ophthalmologic, laboratory, and imaging assessments were performed.
Main Results:
- The average age at diagnosis was 34±13 years; meningoencephalitis (48.8%) and cerebral deep venous thrombosis (43.6%) were common.
- Aggressive cyclophosphamide treatment led to positive clinical improvement and good tolerance in most patients.
- Two cases showed disease aggravation despite treatment.
Conclusions:
- Immediate, aggressive cyclophosphamide therapy may improve the prognosis of severe NBD.
- The findings suggest a potential therapeutic benefit of cyclophosphamide in NBD.
- Multicenter studies are required to validate cyclophosphamide's efficacy and long-term safety.
Introduction:
Neurological manifestations in Behçet's disease represent between 4 to 49% of systemic manifestations and remain, in the long term, the leading cause of morbidity and mortality.
Methods:
Retrospective series of 40 severe Neurobehçet cases fulfilling the International Study Group criteria for Behçet's disease were consecutively recruited over a period from June 2004 to December 2010. All patients had clinical and ophthalmologic examinations; they underwent laboratory and imaging investigations. They received corticosteroids and cyclophosphamide as initial bolus of 600 mg/m(2) of BSA in the 1st, 2nd, 4th, 6th and 8th day followed by a bolus of 600 mg/m(2) BSA every 2 months for 2 years. Antithrombotic therapy was given to patients with cerebral deep venous thrombosis. Patient follow-up and tolerance to treatment were analyzed.
Results:
The average age at diagnosis was 34±13 years, with a sex-ratio of 1.78. The clinical presentation was dominated by the meningoencephalitis in 48.8% of cases, cerebral deep venous thrombosis in 43.6% of cases and myelopathy in 7.7% of cases. The 40 patients receiving cyclophosphamide bolus, despite two aggravated cases, evolved positively with clinical improvement and good tolerance.
Conclusion:
The demographic and clinical aspects of our series are similar to those reported in the literature. In contrast to previously reported cases of a poor prognosis in severe neurobehçet's disease, our study suggests that immediate and aggressive treatment by cyclophosphamide may ameliorate the prognosis. However, a multicenter study is needed to confirm the possible efficacy of cyclophosphamide and further assess the long-term tolerance.
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