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


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


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.


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).


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.


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.

Age, years, months17 y, 6 m18 y, 4 m14 y, 1 m9 y, 4 m
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
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
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

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