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[Immunosuppressors in Behçet's disease].
Immunosuppressive therapy in Beh^et's disease
Immunosuppressive therapy seetm to he the treatment ofchoice in Beh<;et's disease if
administered early, at adequate dosages andfor relatively long periods of time. Corticosteroids
can be very effective durlng the initial stages ofthe disease, but the frequeni and rapid
onset of dependence and resistance to these drugs limits their usefulness. The Authors
report a case ofBehget's disease treated with the immunosuppressive alkylating agent chlorambucil.
Thepatient, a 39year old white male, presented the followingclinicaland laboratory
findings: aphthae buccalis andgenitalis, diffused uveitis with marked retinal vasculitis,
pyoderma, diffused arthralgia, relapsis hydrarthrosis, HLA B5 marker, circidating
immimocomplexes and increased serum C4.
Chlorambucil was initially administered in a single 5 mg daily dose, after 3 months of
corticosteroid (prednisone) treatment. The dosage of chlorambucil was itureased by 2.5
mg/week imtil a dady dose of 12.5 mg was reached. MeanwhUe, corticosteroid treatment
was withdrawn. After 5 months of treatment, there was complete remission ofeye inflammation
as well as remarkable general improvement. Dosages of chlorambucd were progressively
decreased over the next 7 months. C4 serum levels returned to norrnal. Relapses
of eye inflammation were sporadic and mild and appeared more amenable to corticosteroid
treatment.
These results confirm the previous observation of5I patients after early administration
of chlorambucd: overall improvement of their clinical condition and prognosis of Behget's
disease was demonstrated.
Key words: Behget's disease, chlorambucil, immunosuppressive drugs, uveitis
Prognosis in Behcet's disease
A retrospective clinical study made to determine the course and prognosis of the disease in 51 cases of Behcet's disease, with a follow-up of at least one year (average five years, range 1 to 15 years), showed that 45% of the eyes kept visual acuities of 1/10 or less, and 44% 8/10 or better, four years after the onset of the ocular symptoms. Ten years later, some 50% of the eyes had visual acuities of 1/10 or less and 32% had acuities of 8/10 or better. In the group of patients who were treated early with chlorambucil, the eye prognosis was significantly improved
Clinical manifestation of Behçet's disease in Italy: study of 155 patients at Rome University
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