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 Table of Contents  
Year : 2022  |  Volume : 2  |  Issue : 4  |  Page : 911-912

Tofacitinib in juvenile idiopathic arthritis-associated uveitis

1 Department of Uvea and Ocular Inflammation, Prabha Eye Clinic and Research Centre and Vittala International Institute of Ophthalmology, Bengaluru, Karnataka, India
2 Department of Pediatric Rheumatology, Manipal Hospitals, Indira Gandhi Institute of Child Health, Bengaluru, Karnataka, India

Date of Submission27-Mar-2022
Date of Acceptance11-Jul-2022
Date of Web Publication11-Oct-2022

Correspondence Address:
Dr. Kalpana Babu
504, 40th Cross, Jayanagar 8th Block, Bengaluru – 560 070, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_789_22

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Tofacitinib, a Jannus kinase inhibitor, is used in the treatment of rheumatoid arthritis and recently has been used to treat polyarticular juvenile idiopathic arthritis (JIA). In this case report, we report the resolution of refractory anterior uveitis (OS) in a 5-year-old girl with polyarticular JIA. She had failed conventional immuno-modulation (methotrexate, mycophenolate mofetil, and oral steroids). As she could not afford adalimumab, tofacinitib was added, resulting in resolution of anterior uveitis. During the last follow-up (after 8 months), her best corrected visual acuity is 6/9 (OD) and 6/12 (OS). Tofacitinib may be considered as a cost-effective option for refractory JIA-associated uveitis in the developing world.

Keywords: Biologics, JIA, refractory uveitis, tofacitinib, uveitis

How to cite this article:
Babu K, Mooss V, Rao AP. Tofacitinib in juvenile idiopathic arthritis-associated uveitis. Indian J Ophthalmol Case Rep 2022;2:911-2

How to cite this URL:
Babu K, Mooss V, Rao AP. Tofacitinib in juvenile idiopathic arthritis-associated uveitis. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 Dec 3];2:911-2. Available from: https://www.ijoreports.in/text.asp?2022/2/4/911/358190

We report the resolution of refractory anterior uveitis with tofacitinib in a child with juvenile idiopathic arthritis (JIA) who had failed conventional immunomodulation.

  Case Report Top

A 5-year-old girl with polyarticular JIA and a positive anti-nuclear antibody (ANA) was referred to us by her pediatric rheumatologist for an ophthalmic evaluation. She had a history of knee and elbow joint swellings. Her best corrected visual acuity (BCVA) was 6/6 (OD) and 6/9 (OS). Right eye (OD) examination was normal. Slit lamp examination of the left eye (OS) showed medium-sized keratic precipitates, anterior chamber cells 1+ (SUN),[1] and posterior synechiae [Figure 1]. No vitreous cells were noted. Fundus examination (OS) was normal. She was started on oral steroids 20 mg/day with a slow taper along with subcutaneous injection methotrexate 12.5 mg/week along with topical prednisolone acetate eye drops 6 times/day. Over 2 years, although the systemic disease remained stable, multiple recurrences of anterior uveitis (OS) occurred each time the oral steroids were reduced to less than 2.5 mg/day. Thus, she required a maintenance dose of both topical (1 time/day of prednisolone acetate 1% eye drops) and oral steroids (2.5 mg/day). Adalimumab, the next option after failed conventional immuno-modulation, could not be used here because of financial constraints. Tocilizumab, an IL-6 inhibitor, also could not be used because of cost constraints. Addition of mycophenolate mofetil to this regimen did not prevent recurrences. She eventually developed band-shaped keratopathy changes and cataractous changes in the lens (OS). Last year, she developed pain and swelling in the left knee joint. Tofacitinib 5 mg once a day was added by the pediatric rheumatologist along with continuation of tab wysolone 5 mg/day and injection methotrexate 15 mg/week. At the last follow-up (12 months), her BCVA was 6/6 (OD) and 6/12 (OS). Slit-lamp examination (OS) showed resolution of anterior uveitis [Figure 2]. She is currently on tofacitinib 5 mg once a day along with injection methotrexate 15 mg/week. Her systemic disease and eyes are stable with no flareups and complete resolution of inflammation. No adverse effects were noted with this drug.
Figure 1: Composite photographs showing bilateral knee joint swelling in a child with JIA (a), slit-lamp photographs of the left eye in diffuse illumination (b), and slit illumination (c) showing band keratopathy, medium-sized keratic precipitates, and posterior synechiae

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Figure 2: Slit-lamp photograph of the left eye showing resolution of anterior uveitis

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  Discussion Top

Uveitis is a potentially sight-threatening complication of JIA. In spite of the remarkable progress in the care of these children, blindness can occur as a result of low-grade chronic intra-ocular inflammation. The consequences of this low-grade uveitis occur extremely slowly, resulting in ocular damage including band keratopathy, maculopathy, glaucoma, and cataract.[2],[3],[4],[5] Thus, development of these sequelae is a risk factor resulting in poor visual outcome. Treatment guidelines in JIA-associated uveitis[6] include primary treatment with oral steroids and methotrexate. Biologics was the next option following failure of primary treatment. The SYCAMORE and ADJUVITE randomized controlled trials have proved that adalimumab in combination with methotrexate is a valuable drug in treatment of JIA-associated uveitis.[2],[6] IL-6 inhibitors such as tocilizumab (APTITUDE trial) have been used successively in refractory macular edema because of JIA uveitis.[6] However, the costs and the injection formulations make it difficult for the pediatric population in the developing world.

Tofacitinib is a Jannus kinase (JAK) inhibitor, first used in rheumatoid arthritis. Its cost effectiveness, oral formulation, and efficacy in polyarticular JIA[5] have prompted increased usage of this drug in a country such as ours. There are few reports of its efficacy in JIA-associated uveitis.[4],[5] Our case had polyarticular JIA. Addition of tofacitinib in this case resulted in complete resolution of anterior uveitis which was refractory to conventional immuno-modulation. Also, at 12 months follow-up, there was no untoward side effects because of this drug in our case. Further clinical studies may be required to compare the efficacy of tofacitinib and adalimumab in JIA-associated uveitis. Until then, tofacitinib may be considered as a cost-effective option for refractory JIA-associated uveitis in developing countries where financial constraints prevent the use of adalimumab.

  Conclusion Top

Tofacitinib, a JAK kinase inhibitor, may be considered as a cost-effective option for refractory JIA-associated uveitis in a country such as India.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Jabs DA, Nussenblatt RB, Rosenbaum JT; Standardization of Uveitis Nomenclature (SUN) Working Group. Standardization of uveitis nomenclature for reporting clinical data. Results of the First International Workshop. Am J Ophthalmol 2005;140:509-16.  Back to cited text no. 1
Chen JL, Abiri P, Tsui E. Recent advances in the treatment of juvenile idiopathic arthritis-associated uveitis. Ther Adv Ophthalmol 2021;13:2515841420984572.  Back to cited text no. 2
Miserocchi E, Giuffre C, Cornalba M, Pontikaki I, Cimaz R. JAK inhibitors in refractory juvenile idiopathic arthritis-associated uveitis. Clin Rheumatol 2020;39:847-51.  Back to cited text no. 3
Bauermann P, Heiligenhaus A, Heinz C. Effect of janus kinase inhibitor treatment on anterior uveitis and associated macular edema in an adult patient with juvenile idiopathic arthritis. Ocul Immunol Inflamm 2019;27:1232-4.  Back to cited text no. 4
Ruperto N, Brunner HI, Synoverska O, Ting TV, Mendoza CA, Spindler A, et al. Paediatric rheumatology international trials organisation (printo) and pediatric rheumatology collaborative study group (PRCSG). Tofacitinib in juvenile idiopathic arthritis: A double-blind, placebo-controlled, withdrawal phase 3 randomised trial. Lancet 2021;398:1984-96.  Back to cited text no. 5
Clarke SL, Sen ES, Ramanan AV. Juvenile idiopathic arthritis-associated uveitis. Pediatr Rheumatol Online J 2016;14:27.  Back to cited text no. 6


  [Figure 1], [Figure 2]


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