Article Text
PDF
Abstract
Objective To evaluate the efficacy of anifrolumab (ANI) in refractory cutaneous manifestations of systemic lupus erythematosus (SLE) in real-world settings.
Methods Patients with SLE, according to ACR 1997 criteria and/or SLICC 2012 criteria, and refractory cutaneous manifestations who received anifrolumab were included. Demographic and clinical characteristics and laboratory values were documented at baseline and last visit of follow-up. Prior use of immunosuppressives and glucocorticoids, as well as adverse events were also recorded. SLE disease activity was measured with Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) and Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI).
Results Eighteen patients (94%female, mean age 44.3±12.6years, mean disease duration 13.7±7.6years) received ANI. In all patients, active skin involvement was the main reason for ANI initiation. Mean (SD) SLEDAI at baseline was 7.1 (2.6) and mean prednisone dose was 6 (4.9) mg/day. Additionally, 8 and 4 patients had low levels of C3/C4, and positive anti-dsDNA. Mean (SD) CLASI (Actvity/Damage) at ANI baseline was 12/3.4 (2.9/4.6). Skin rash had previously proven refractory to a mean (SD) 4.5 (1.9) prior immunosuppressive agents (excluding hydroxychloroquine and glucocorticoids). Fifteen patients had not responded to belimumab, seven to cyclophosphamide, and four each to rituximab, thalidomide. We observed an impressive and rapid response of active skin lesions very early following ANI initiation, even after the first infusion. After a mean (SD) 7.1 (5.8) months of follow-up, fifteen patients are still receiving ANI without loss of efficacy and mean (SD) daily prednisone dose is 3.4 (3.5). Mean (SD) SLEDAI and CLASI (Activity/Damage) at last visit were 1.8 (2.4) and 1.6/1.3 (2.4/1.7) respectively. C3/C4 normalized in 3/8, while one patient remained anti-dsDNA positive (figure 1). ANI was discontinued in 2 patients after 4 and 3 months due to zoster infection and generalized lymphadenopathy, respectively.
Conclusions Our initial experience suggests high efficacy of ANI for skin manifestations of SLE that have previously failed multiple treatment options.
C3/C4 and anti-dsDNA levels at ANI initiation (baseline) and at last visit
This is an open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited, appropriate credit is given, any changes made indicated, and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/ .