Retrospective analysis of long-term outcome of chronic progressive neurological manifestations in Behcet's disease.

OBJECTIVES

Chronic progressive neuro-Behcet's disease (CPNBD) is characterized by progressive deterioration leading to disability and death. Although methotrexate has been found effective for CPNBD, its influences on the long-term outcome remain unclear. We therefore explored the effects of various treatments on the prognosis.

METHODS

Thirty-seven patients, who met the international classification criteria for BD and developed chronic progressive neuropsychiatric manifestations after 1988, were followed up until October 2013. The effects of various treatments on prevention of death or severe disability of bedridden state were examined by Kaplan-Meier analysis and Cox's proportional hazard model.

RESULTS

Twenty-eight of 37 patients with CPNBD (75.7%) received methotrexate. Among the 28 patients, none died and only 5 patients progressed to disability with bedridden state. By contrast, among the 9 patients without methotrexate, 5 patients died and 3 patients progressed to bedridden state. Thus, methotrexate significantly improved the survival of patients with CPNBD (HR 0.0507, p=0.020) as well as reduced the rate of progression into bedridden state or death (HR 0.2082, p=0.0126), but none of high doses of steroids, azathioprine or cyclophosphamide did.

CONCLUSION

The results indicate that methotrexate, but not high doses of steroids, azathioprine or cyclophosphamide, is effective to prevent the progression of CPNBD.