THU0406 Rituximab Therapy in Patients with Refractory Skin Manifestation of Lupus Erythematosus During Long-Term Follow-Up

ObjectivesTo evaluate the efficacy and usefulness of anti-B-cell therapy with rituximab (RTM) in patients with different subtype of skin manifestations of lupus erythematosus (LE) refractory to standard therapy during the long-term follow-up.Methods32 patients with various subtype of skin manifestation of LE were included in our study: generalized acute cutaneous LE was observed in 6, subacute cutaneous LE in 7, butterfly rash in 7, panniculitis in 1, discoid lupus erythematosus in 2, ulcerative vasculitis in 9 patients. Mucosal LE was observed in 23 patients. The median age of the patients was 27 [21-40] years, the duration of the diseases was 44 [17-84] months. Before the treatment of the disease SLEDAI2K was 20 [15-27], the concentration of a-dsDNA was 61,5 [24,6-111] U/ml, C3 was 0,58 [0,49-0,76] g/l, C4 was 0,1 [0,08-0,15] g/l, the concentration of B-cells was 6,6 [3,5-14,7]%. Previous puls-therapy was not effective in 66% of patients who received the cyclophosphamide (CF) and high dosage of glucocorticoids (GC), in 34% of patients who received previous therapy with mycofemolate mofetil (MFM) and azathioprine (AZA).Only 1 course of RTM received 19 patients, 2 courses - 5 patients, 3 courses - 5 patients, 4 courses – 3 patients. After the first course of RTM 30 patients received maintenance therapy with cytostatics and hydroxychloroquine: CF-10, AZA-1, MFM - 4 patients and hydroxychloroquine received 5 patients. CF was changed to AZA in 3 patients after the 3 months of the therapy. 1 patient received combined therapy with CF and MFM.ResultsDuring the long-term follow-up (12 [12-24] months, from 6 to 48 months) the repeated courses of rituximab were effective in 84% of patients (complete response in 56%, partial response in 28%) and were not effective in 16% of patients. The B-cell depletion was observed in 72% of patients during a month after the therapy with RTM. The mean time of B-cell recovery was 6 [1-12] months. The exacerbations were noticed in 25% of patients during 12 [6-21] months of follow-up.Reducing signs of skin manifestations occurred gradually with the following sequence: the most rapid effect was observed in subacute LE, generalized acute cutaneous LE, discoid LE and further in ulcerative vasculitis. Complete response was observed in 60% of patients during a month after the first course of anti-B-cell therapy.During the long-term follow-up we observed the improvements of disease activity by SLEDAI2K, concentrations of a-dsDNA and compon