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Behçet disease: clinical features and management in a Brazilian tertiary hospital
Ana Cecília Diniz Oliveira1, Ana Letícia Pirozzi Buosi, Lívia Almeida Dutra
1Department of Medicine, Universidade Federal de São Paulo, São Paulo, Brazil.
Insights
This study describes Behçet disease (BD) in Brazil, finding oral ulcers in all patients and higher arthritis rates in women. Brazilian men with BD showed no worse prognosis compared to other regions.
Area of Science:
- Rheumatology
- Immunology
- Dermatology
Background:
- Behçet disease (BD) is a rare multisystem inflammatory disorder with a higher prevalence in East Asia and the Mediterranean.
- Limited research exists on BD patient demographics and clinical profiles in Brazil.
- This study addresses the gap in understanding BD in the Brazilian population.
Purpose of the Study:
- To characterize the clinical manifestations of Behçet disease (BD) in Brazilian patients.
- To document the therapeutic approaches used for BD in a Brazilian cohort.
- To compare BD characteristics between genders in Brazil.
Main Methods:
- A retrospective observational study was conducted on 60 patients diagnosed with BD according to the International Study Group Criteria.
- Patients were recruited from the Vasculitis Unit at Universidade Federal de São Paulo.
- Data collected included demographics, clinical features, and treatment modalities.
Main Results:
- The study population had a mean age of 40.0 years, with a female-to-male ratio of 1.2:1.0.
- High frequencies of oral ulcers (100%), genital ulcers (93.3%), and cutaneous lesions (71.7%) were observed.
- Arthritis (46.7%) and ocular manifestations (63.3%) were common; women showed a higher prevalence of arthritis and erythema nodosum, while men had more papulopustular lesions.
- Neurologic involvement (28.3%) and thrombosis (13.3%) were also noted. Colchicine was the most frequent treatment (78.3%), with immunosuppressants used in 70% of patients.
Conclusions:
- Brazilian patients with BD exhibit distinct clinical patterns, with mucocutaneous and articular manifestations being highly prevalent.
- While women experienced a higher frequency of arthritis, men did not demonstrate a worse prognosis compared to international data.
- The findings underscore the importance of recognizing BD's varied presentation in Brazil and tailoring treatment accordingly.
Background:
Behçet disease (BD) is prevalent in Central and East Asia and in the Eastern Mediterranean area where most studies have been performed. Few studies have evaluated patients with BD in Brazil.
Objectives:
The objective of the study was to describe clinical manifestations of BD and their therapy in a single center in Brazil.
Methods:
Sixty patients who met the International Study Group Criteria for BD and were under follow-up at the Vasculitis Unit of the Universidade Federal de São Paulo were evaluated in a retrospective observational study.
Results:
Mean age at study was 40.0 (SD, 10.7) years, and the female-male ratio was 1.2:1.0. The frequency of disease manifestations was as follows: oral ulcers, 100%; genital ulcers, 93.3%; ocular manifestations, 63.3%; arthritis, 46.7%; cutaneous lesions, 71.7%; positive pathergy test, 22.7%; neurologic involvement, 28.3%; thrombosis, 13.3%; and gastrointestinal involvement, 3.3%. Arthritis and erythema nodosum were more prevalent among women, whereas papulopustular lesions were more common in men. The frequency of each treatment modality was as follows: colchicine, 78.3%; thalidomide, 26.7%; colchicine and penicillin, 21.7%; dapsone, 8.3%; and pentoxyphyline, 8.3%. These treatments were mainly used for mucocutaneous manifestations. Immunosuppressive drugs were prescribed for 70% of the patients, including azathioprine (35.0%), cyclophosphamide (28.3%), cyclosporin A (21.7%), methotrexate (18.3%), and chlorambucil (6.7%). Infliximab was used in 5.0% of refractory patients. No differences were observed between sexes related to severe manifestations of BD.
Conclusions:
Although reported elsewhere, Brazilian men with BD did not have a worse prognosis. Women had a higher frequency of arthritis manifestations.
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