The typical ocular manifestation of pauciarticular juvenile idiopathic arthritis (JIA) is an insidious, non-granulomatous chronic anterior uveitis (CAU). The main risk factors for the development of uveitis in JIA are being female, antinuclear antibody (ANA) positivity, and oligoarticular disease at onset.^1,2 Even during acute exacerbations of JIA associated CAU with severe anterior chamber (AC) cellular reaction, it is rare for patients to have symptoms and the eye is invariably white.^1,2 This is in distinct contrast to the acutely symptomatic red eye of acute anterior uveitis (AAU). Hypopyon can form within the AC in severe forms of anterior uveitis, particularly HLA-B27 associated AAU.^3–5 We report a case of CAU in a patient with pauciarticular JIA, which was complicated by the development of recurrent hypopyon.

Case report

A 4 year old white girl first presented with blurred vision in her right eye and was found to have a unilateral, non-granulomatous CAU with extensive posterior synechiae and early band keratopathy. She was initially treated with intensive topical corticosteroids and mydriatics. She was ANA positive and had an ankle effusion, resulting in the diagnosis of pauciarticular JIA associated CAU. Repeated orbital floor steroid injections and systemic low dose methotrexate were required for the control of persistent anterior uveitis activity. Control of AC inflammatory activity was then maintained with regular topical prednisolone and oral methotrexate treatment.

At a routine follow up, 6 months after her initial presentation with CAU, the patient was found to have an asymptomatic inflammatory hypopyon and +4 cells in the AC of the right eye without conjunctival injection. There was no history of ocular surgery or trauma and the patient was well systemically. Topical steroids were increased to hourly leading to a significant improvement in the anterior uveitis activity with resolution of the hypopyon and the achievement of a quiet AC with no cells. Further investigations revealed that the patient was HLA-B27 positive and the angiotensin converting enzyme level was not elevated. There were no clinical features of a seronegative spondyloarthropathy. Two months later, the hypopyon recurred upon attempted tapering of topical steroid treatment, once again occurring in a painless “white” eye of CAU (fig 1). The hypopyon again demonstrated therapeutic response to hourly topical steroids and was completely resolved within 1 week. A quiet eye was eventually achieved and maintained with systemic methotrexate and regular topical prednisolone therapy, with last follow up being 9 months after the resolution of the recurrent hypopyon.

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Figure 1

 Slit lamp biomicroscopy examination demonstrating a hypopyon in a painless “white” eye with chronic anterior uveitis that is associated with ANA positive pauciarticular JIA. Note the lack of ciliary conjunctival injection.

Comment

JIA is by far the most common identifiable clinical association of uveitis in children. There have been no previous reports describing the development of hypopyon in the typical clinical phenotype of CAU associated with pauciarticular JIA.^1,2,5,6 Our case exhibited all of the characteristic clinical features of non-granulomatous CAU of ANA positive pauciarticular JIA, including its insidious onset and asymptomatic chronic disease course requiring long term anti-inflammatory treatment for uveitis control.

Although our patient was also found to be HLA-B27 positive, her clinical features were not consistent with HLA-B27 associated AAU, which includes an abrupt onset of red and painful anterior uveitis that is typically of short duration, predominantly occurring in boys who are ANA negative.^3,6 HLA-B27 antigen is of clinical significance in AAU; however, in contrast, HLA-B27 positivity does not occur more frequently in CAU than in the general healthy population.^3,7 JIA associated uveitis that is positive for both ANA and HLA-B27 appears to be a relatively rare clinical subgroup and whether these represent a distinct clinical entity remains unclear.^2,6,8 Three patients with ANA positive CAU in early childhood who later developed HLA-B27 associated recurrent attacks of AAU during adolescence or adulthood have been reported.⁸ In one of these reported patients, who were positive for both ANA and HLA-B27, hypopyon had been described in association with the recurrent episodes of AAU that developed during adulthood. The hypopyon described in that case occurred in the context of HLA-B27 associated AAU in an adult, distinct from and temporally following, clinical features of childhood ANA positive CAU.⁸ In contrast, our reported patient developed recurrent spontaneous hypopyon (at the age of 5) in the context of the typical, insidious CAU without any clinical features of HLA-B27 associated AAU.

It remains unclear whether the presence of HLA-B27 antigen in our patient is a coincidence or whether it is of pathogenic significance. HLA-B27 antigen may represent a risk factor for the development of hypopyon in anterior uveitis, even in those that do not display the typical phenotype of HLA-B27 associated AAU. It would be of interest to follow the clinical course of our patient into her adulthood to determine whether she later develops ocular or systemic features of HLA-B27 associated AAU. Further studies of a cohort of ANA and HLA-B27 positive children with uveitis are indicated to investigate their clinical phenotype and the potential interaction between ANA and HLA-B27.