CHALLENGES IN THE TREATMENT OF NEURO-BEHÇET’S DISEASE — a case report

Samir Mehmedagić, Nejra Džananović

Behçet’s disease is a multisystem disease of insufficiently known etiology, with genetics playing a significant role in its development, featuring an increased frequency of the HLA-B51 gene in the genome of patients, but it can sometimes also be associated with the presence of certain strains of bacteria. Pathogenetically, this disease is considered to be small-vessel vasculitis. The disease most often manifests itself in oral and genital ulcerations with frequent uveitis and skin changes, and, in very rare cases, there are neurological manifestations, which occur in less than 10 percent of patients that suffer from this disease, and in that case we call it the neuro-Behçet’s disease. The treatment of this disease depends on the clinical condition and the treatment approach is individual for each patient. The greatest challenge is the treatment of neuro-Behçet’s disease due to the severity of the clinical condition and frequent inadequate response to therapy. Neuro-Behçet’s disease is treated with corticosteroids, various immunosuppressants and biological therapy – e.g., TNF-alpha inhibitors as the latest form of therapy with the best results. This paper intends to present the case of a patient with neurological manifestations of the disease, the challenges that we faced during the treatment, the complications that occurred, and the resulting changes in therapy that were sometimes necessary.

Behçetova bolest je multisistemska bolest nedovoljno poznate etiologije, no genetika ima značajnu ulogu u njenom nastanku, s većom učestalošću prisutnosti gena HLAB51 u genomu oboljelih, no ponekad se može povezati i s prisutnošću nekih sojeva bakterija. Patogenetski to je vaskulitis malih krvnih žila. Bolest se najčešće manifestira oralnim i genitalnim ulceracijama uz čestu pojavu uveitisa, kožnih promjena, te vrlo rijetku prisutnost neuroloških manifestacija koje se javljaju u manje od 10% pacijenata koji boluju od ove bolesti (nazivamo je neuro-Behçetova bolest). Liječenje ove bolesti ovisi o kliničkoj slici i pristup je individualan za svakog bolesnika, a najveći izazov predstavlja liječenje neuro oblika Behçetove bolesti zbog težine kliničke slike i vrlo često neadekvatnog odgovora na terapiju koja je uključena. Neuro-Behçet se liječi kortikosteroidima, raznim imunosupresivima i biološkom terapijom – TNF alfa inhibitorima kao najnovijom vrstom terapije kojom se postižu najbolji rezultati. U ovome radu želimo prikazati slučaj bolesnika s neurološkim manifestacijama bolesti, izazove koji su nas pratili tijekom liječenja, komplikacije koje su se dogodile, a samim time i promjene u terapiji koje su ponekad bile potrebne.