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Clinical manifestations of Behçet's disease in 137 Italian patients: Results of a multicenter study

N. Pipitone¹, L. Boiardi¹, I. Olivieri², F. Cantini³, F. Salvi⁴, R. Malatesta⁵, R. La Corte⁶, G. Triolo⁷, A. Ferrante⁷, D. Filippini⁸, G. Paolazzi⁹, P. Sarzi-Puttini¹⁰, G. Restuccia¹, C. Salvarani¹

¹Unitá di Reumatologia, Arcispedale Santa Maria Nuova, Reggio Emilia; ²Unitá di Reumatologia, Ospedale di Potenza; ³Unitá di Reumatologia, Ospedale di Prato; ⁴Dipartimento Scienze Neurologiche and ⁵Unitá di Reumatologia, Ospedale Bellaria, Bellaria; ⁶Unitá di Reumatologia, Ospedale S. Anna, Ferrara; ⁷Cattedra di Reumatologia, Universitá di Palermo; ⁸Unitá di Reumatologia, Ospedale Niguarda; ⁹Unitá di Reumatologia, Ospedale di Trento; ¹⁰Unitá di Reumatologia, Ospedale L. Sacco, Milan, Italy.

ABSTRACT
Objective
To determine the type and frequency of clinical features of Behçet's disease in a population of Italian patients.

Methods
We retrospectively studied 137 Italian patients (76 males and 61 females, age at onset 29.6 ±12.2 [mean ±SD] years) seen consecutively in nine different referral centers. The duration of follow-up at study entry was 10.9 ± 8.2 years. Virtually all patients fulfilled the classification criteria developed by the International Study Group for Behçet's disease. The clinical manifestations of the patients were recorded by the attending physicians using specifically designed forms.

Results
The most frequent manifestations at disease onset were oral (78.3%) and genital aphthae (29.2%) followed by inflammatory ocular involvement (20%) and arthritis (14.2%). The commonest (>50% of cases) manifestations observed throughout the disease course were oral aphthae (99.3%), genital aphthae (62.8%), various cutaneous lesions including erythema nodosum (81.8%), and inflammatory ocular disease (60.6%). Panuveitis and posterior uveitis/retinitis occurred more frequently in males compared with females (28.9% versus 11.5% and 57.9% versus 36.1%, respectively; p < 0.05). 61.6% of our patients were HLA-B51 positive.

Conclusion
Behçet.s disease in Italian patients is characterized by a variety of clinical manifestations in agreement with the medical literature. Panuveitis and posterior uveitis/retinitis occur more frequently in male patients.

Key words
Behçet syndrome, signs and symptoms, Italy.

Please address correspondence and reprint requests to: Dr. Carlo Salvarani, Unitá di Reumatologia, Arcispedale Santa Maria Nuova, Reggio Emilia, 42100, Italy.
E-mail: Carlo.Salvarani@asmn.re.it