Long-term outcome of neuro-Behçet's disease.
OBJECTIVE
To report the long-term outcome of neurologic involvement in patients with Behçet's disease (BD).


METHODS
We performed a retrospective analysis of 115 patients who fulfilled the international criteria for BD (57% male; median age 37 years [interquartile range (IQR) 30-46 years]) and had neuro-BD (NBD) after exclusion of cerebral venous thrombosis. Factors associated with relapse of NBD, inability to perform activities of daily living, and mortality were assessed.


RESULTS
Seventy-eight patients (68%) presented with acute NBD and 37 (32%) presented with a progressive course. The HLA-B51 allele was carried by 49% of the patients. Overall, 46 of 115 patients (40%) had severe disability at baseline, represented by a Rankin score of ≥3. The 5- and 7-year event-free survival rates were 65% and 53%, respectively. In multivariate analysis, a positive HLA-B51 status was independently associated with the risk of NBD relapse, with an odds ratio (OR) of 3.6 (95% confidence interval [95% CI] 1.5-9.1). After a median followup of 73 months (IQR 59-102 months), 29 patients (25.2%) became dependent (were unable to perform activities of daily living) or died. Factors independently associated with poor outcome were paresis at onset (OR 6.47 [95% CI 1.73-24.23]) and location of inflammatory lesions at the brainstem on magnetic resonance imaging (OR 8.41 [1.03-68.43]). All 115 patients were treated with corticosteroids; 53 (46.1%) also took cyclophosphamide and 40 (34.8%) also took azathioprine. A trend toward longer event-free survival was observed in patients with severe NBD (i.e., with a Rankin score of ≥3 at onset) receiving intravenous cyclophosphamide compared with those receiving azathioprine (P = 0.06).


CONCLUSIONS
Our findings indicate that NBD is a severe condition in which patients with the HLA-B51 allele appear to experience a worse prognosis.
APHP Groupe Hospitalier Pitié Salpétrière, and DHU Inflammation, Immunopathologie, Biothérapie, Université Pierre et Marie Curie, Paris VI, Paris, France.
Nicolas
N
Noel
Remy
R
Bernard
Bertrand
B
Wechsler
Matthieu
M
Resche-Rigon
Raphaël
R
Depaz
Du
D
Le Thi Huong Boutin
Jean-Charles
JC
Piette
Aurélie
A
Drier
Didier
D
Dormont
Patrice
P
Cacoub
David
D
Saadoun
D000328:Adult; D000483:Alleles; D001528:Behcet Syndrome / Q000175:diagnosis* / Q000453:epidemiology / Q000235:genetics*; D015897:Comorbidity; D005260:Female; D059949:HLA-B51 Antigen / Q000235:genetics; D006801:Humans; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D009422:Nervous System Diseases / Q000175:diagnosis* / Q000453:epidemiology / Q000235:genetics*; D011379:Prognosis; D012189:Retrospective Studies; D015996:Survival Rate
D059949:HLA-B51 Antigen