Notch signaling: its roles and therapeutic potential in hematological malignancies.

OBJECTIVES

Behçet's disease (BD) is a chronic multisystem inflammatory disorder associated to uveitis that may represent a serious sight-threatening condition. The purpose of the present study is to assess the effectiveness of adalimumab as new strategic therapeutic approach in patients affected by severe Behçet's uveitis.

METHODS

Clinical data from twelve selected patients (22 eyes) were retrospectively analysed. All patients received 40 mg of adalimumab subcutaneously, once every 2 weeks, in addition to traditional immunosuppressive on-going therapy and eight of them were switched to adalimumab after failure of infliximab therapy. Primary outcome measures included ocular inflammatory activity, frequency of uveitis attacks and steroid-sparing effect. Secondary outcomes were changes of best-corrected visual acuity (BCVA), impact on traditional immunosuppressive therapy and occurrence of adalimumab-related side effects.

RESULTS

Mean age of patients (11 males and 1 female) at the onset of disease was 24.34 years (±8.62 SD). Ocular involvement resulted bilateral in 83% of cases and mainly consisted in panuveitis (68% of eyes). After mean follow-up of 21 months (±9.63 SD) all patients but one (92%) achieved uveitis remission with BCVA improvement at least in one eye. Average uveitis attacks decreased from 2 to 0,42 during adalimumab (p<0.001) and daily-steroid dose was tapered in all adalimumab responders up to suspension in seven of them. No patient developed related side effects during adalimumab administration.

CONCLUSIONS

Our results demonstrate that adalimumab is a very effective and safe option for treatment of patients with severe and resistant Behçet's uveitis, providing an appropriate and long-term control of ocular inflammation.