Background Behçet's disease is a recurrent systemic vasculitis which may have neurological manifestations: Neuro-Behçet (NB). The estimated frequency is 1-59%. It is more common in males. Central neurological system (CNS) involvement, presenting as pseudotumoral masses are very rare
Objectives To describe the clinical characteristics, incidence, treatment, and outcome of patients diagnosed of pseudotumoral neuro-Behçet disease. We performed a systematic review of cases reported in the literature.
Methods Desing: retrospective (1984-2014). Location: Two University hospitals. A hundred and fifty five patients records were reviewed with a diagnosis of Behçet's disease from the Rheumatology service data base. Inclusion criteria: patients with pseudotumoral neuro-Behçet. A systematic search of all published cases with this diagnosis through PubMed was also performed. (1987-2014, keywords: pseudotumoral, neuro-Behçet)
Results Three new cases of pseudotumoral Neuro-Behçet were identified. After a systematic review, other 27 cases were found with this diagnosis. The whole series were composed of 30 patients: a predominance of male (73.3%) was observed, with a mean age: 39.2±12.1 (range: 23-63 years). The mean duration of Behçet's disease prior to the diagnosis of NB, was 9.3±7.8 years, in 50% of patients.
The diagnosis was performed simultaneously or later in the remaining patients. The location of the lesion was: capsular thalamus region 15 (50%), peduncle and cerebellar 4 (13.3%), fronto parietal region 2 (6.7%), fronto temporal and temporo -occipital region in 1 (3.3%). Four (13.3%) patients presented more than one lesion. In 14 (46.7%) patients biopsy of the brain mass was performed in order to confirm the diagnostic. All reported patients were initially treated with high-dose of glucocorticosteroids (oral or intravenous), other immunosuppressive agents such as azathioprine, cyclophosphamide, methotrexate or chlorambucil were added. Three (10%) patients (2 of own serie and 1 described in literature) were treated with biological agents (anti TNF alpha, rituximab and tocilizumab). Only in one case plasmapheresis was performed because of refractory disease. Most patients showed progressive improvement, and three (10%) patients died.
Conclusions The presence of pseudotumoral lesions is a very rare presentation of Neuro-Behçet. It is more common in males and occurs years after diagnosis; however it can also occurs simultaneously. A differential diagnosis with other CNS diseases and malignancy is mandatory. The response to glucocorticoides plus immunosuppressive drugs is good. Biological agents may be useful for refractory cases.
Disclosure of Interest None declared