A23: Favorable Response to Belimumab in Childhood‐Onset Systemic Lupus Erythematosus. Issue 11 (March 2014)

• Record Type: Journal Article
• Authors: Hui‐Yuen, Joyce S; Bermudez‐Santiago, Liza Mariel; Isgro, Josephine; Eichenfield, Andrew H.; Starr, Amy J.; Imundo, Lisa F.; Askanase, Anca
• Is Part Of: Arthritis & rheumatology. Volume 66:Issue 11(2014)supplement
• Page Start: S37
• Page End: S37
• Publication Date: 2014-03
• Subjects: Arthritis -- Periodicals; Rheumatism -- Periodicals; 616.72
• DOI: 10.1002/art.38439
• Languages: English
• ISSNs: 2326-5191

Abstract

Abstract : Background/Purpose: Belimumab (Benlysta) is a human monoclonal antibody inhibiting soluble B‐lymphocyte stimulator that was approved by the Food and Drug Administration in 2011 for treatment of active, autoantibody‐positive, systemic lupus erythematosus (SLE) in adults. To date, there have been no published data on the use of belimumab in childhood‐onset SLE. Methods: This is a case series of patients with childhood‐onset SLE treated with belimumab at our institution. Data were collected on demographic and disease characteristics, clinical manifestations requiring treatment, concomitant medications, disease course, and treatment outcomes. Descriptive statistics were used when appropriate. Results: We describe 7 patients with childhood‐onset SLE treated with belimumab, 5 of whom are still receiving belimumab infusions at our institution, with a mean follow‐up of 9.3 months on belimumab. The mean age was 18.5 years old, mean disease duration 5.8 years at initiation of belimumab. Eighty‐six percent of the patients were female, 57% African American, 28% Hispanic, and 14% Caucasian. All patients were taking other background medications prior to initiation of belimumab (hydroxychloroquine 7/7, prednisone 7/7, azathioprine 2/7, mycophenolate mofetil 3/7). The most common indications for initiation of therapy were the inability to taper steroids (100%, mean prednisone equivalent dose 0.54mg/kg/day), rash (43%), fatigue (29%), and arthritis (14%), accompanied by worsening serologic activity (increasing anti‐dsDNA titers and hypocomplementemia). At the last follow‐up (mean 9.3 months after initiation of belimumab), average SLEDAI decreased from 6.4 to 4. We were able to taper steroids in 57% of patients, with 3/7 (43%) able to completely discontinue steroids. The average complement levels increased from 71 to 74 (lower limit of normal 83) for C3 and 8 to 10 (lower limit of normal 16) for C4. Six of the seven patients clinically responded to belimumab within three months, with marked improvement in rash, arthritis, and fatigue. Six of seven patients tolerated infusions well, with 1 patient who discontinued treatment due to recurrent vaginal moniliasis. Conclusion: This is the first study investigating the use of belimumab in childhood‐onset SLE across major ethnic groups. Our data demonstrate favorable clinical outcomes including the discontinuation of steroids in 3/7 (43%) of our patients, and improvement in C3 and C4 as early as 3 months after the initiation of belimumab, suggesting a quicker onset of action for belimumab in children than that observed in adult studies, which may support a larger role for belimumab in childhood‐onset SLE.