Arthritis & Rheumatism, Volume 62,
November 2010 Abstract Supplement

Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Atlanta, Georgia November 6-11, 2010.


Use of Abatacept for Childhood Refractory Vision-Threatening Uveitis.

Simonini2,  Gabriele, De Cinzia1,  Libero, Caputo1,  Roberto, Giani2,  Teresa, Pascoli2,  Laura, Pagnini2,  Ilaria, Cimaz2,  Rolando

Opthalmology Unit, A Meyer Children's Hospital, Florence, Italy
Rheumatology Unit Dpt of Pediatrics, A.Meyer Children's Hospital-University of Florence, Italy

Background:

Improvement of refractory JIA-related uveitis has been reported with Abatacept.

Objective:

Aim of our study was to evaluate the efficacy and safety of Abatacept in an open-label prospective, monocenter, study of childhood non-infectious chronic uveitis.

Methods:

Four patients with refractory, vision-threatening non-infectious uveitis were enrolled to receive Abatacept 10 mg/kg at weeks 0, 2, 4, and thereafter monthly, for at least 6 months. Absence/recurrence rate of uveitis throughout the study period, according to the SUN Working Group criteria, visual acuity pre- and post-Abatacept treatment, and tapering of steroid medication were recorded. Improved visual acuity was defined as a doubling of the visual angle (converted in a LogMAR format) in at least one eye. All children had active uveitis: 6 out of 8 eyes were involved, despite treatment with methotrexate (MTX) (15 mg/m2/weekly) in association with Infliximab (6 mg/kg every 6–8 weeks) followed by Adalimumab (24 mg/m2, every other week), then by mycophenolate mofetil in 3 cases; and by Adalimumab in 1 case. Due to active uveitis, along with topical steroids, all children were also receiving oral prednisone (1–2 mg/kg/day), at stable doses for at least 6 weeks (range 45–55 days).

Results:

Demographics, clinical characteristics and outcomes on treatment are reported in the Table.

Median time of abatacept treatment was 10 months (range 6–14). During treatment, all children achieved a complete remission over a median period of 12 weeks (range 8–14). Steroid administration was discontinued during the first 6 months (range 4–6) in 3/4 children. The patient with Blau syndrome was able to taper the dose down to 0.3 mg/kg/day. In all children, no relapse of uveitis occurred during treatment, whilst during the 6 month-period before starting Abatacept, the median number of relapses was 4 (range 3–6). At 6 months of treatment, all patients, (5/8 eyes), met the criteria for improved visual acuity. Two children reached a completely normal visual acuity, while the other two improved, eventhough the pre-existing complications, due to chronic uveitis, affected a complete recovery. No major side effects, infusion reactions and/or drug-related adverse events were recorded during the treatment period.

Conclusion:

Even if limited to a small group, our results suggest that Abatacept may be effective and safe in chronic refractory childhood uveitis, both in JIA-uveitis and in not JIA-uveitis.

 1234
Age, years, months17 y, 6 m18 y, 4 m14 y, 1 m9 y, 4 m
GenderFFMF
Age at onset of uveitis, (years, months)10 y, 10 m3 y, 5 m4 y, 10 m2 y, 11 m
Uveitis duration7 y, 4 m14 y, 11 m10 y, 9 m7 y, 7 m
Diagnosis:    
Age at onset of disease, (years, months)Idiopathic uveitis 10 y, 10 mJIA 1 y, 5 mBlau syndrome 4 yJIA 2 y, 7 m
Previous DMARD/anti-TNF-a treatment, duration (months)Infliximab 28Infliximab 37Infliximab 32
 Adalimumab 8Adalimumab 7Adalimumab 21Adalimumab 17
 MMF 16MMF 18MMF 11
N* of previous flares, 6 months before3654
Time to remission (weeks)8121412
Time to steroid discontinuation (months)465
Visual acuity 6 months before ® 6 months afterR 20/60 ® 20/30R 20/50 ® 20/40R 20/20 ® 20/20R 20/25 ® 20/20
 L 20/20 ® 20/20L 20/60 ® 20/40L 20/30 ® 20/20L 20/20 ® 20/20
Concomitant therapy (duration, months)MTX: 15 mg/m2MTX: 15 mg/m2MMF: 1 gm/m2MTX: 15 mg/m2
 6646
Follow-up duration on treatment121486
JIA = Juvenile Idiopathic Arthritis, MTX = methotrexate, MMF = mycophenolate mofetil R = right eye, L = left eye

To cite this abstract, please use the following information:
Simonini, Gabriele, De Cinzia, Libero, Caputo, Roberto, Giani, Teresa, Pascoli, Laura, Pagnini, Ilaria, et al; Use of Abatacept for Childhood Refractory Vision-Threatening Uveitis. [abstract]. Arthritis Rheum 2010;62 Suppl 10 :240
DOI: 10.1002/art.28009

Abstract Supplement

Meeting Menu

2010 ACR/ARHP