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Rheumatology Advance Access originally published online on February 7, 2009
Rheumatology 2009 48(4):330-331; doi:10.1093/rheumatology/kep002
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© The Author 2009. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

EDITORIALS

‘The eyes have it!’ The need to improve awareness and access to early ophthalmological screening for juvenile idiopathic arthritis associated uveitis

Helen Foster1,2 and Athimalaipet V. Ramanan3

1Musculoskeletal Research Group, Newcastle University, 2Paediatric Rheumatology, Newcastle Hospitals NHS Trust, Newcastle upon Tyne and 3Paediatric Rheumatology, Royal Bristol Children's Hospital and Royal National Hospital for Rheumatic Diseases, Bristol, UK

Correspondence to: Helen Foster, Musculoskeletal Research Group, Medical School, Framlington Place, Newcastle upon Tyne NE2 4HH, UK. E-mail: h.e.foster{at}ncl.ac.uk

Juvenile idiopathic arthritis (JIA) is a heterogeneous group of diseases that differ considerably from adult RA with different clinical, serological and genetic associations [1]. JIA is the commonest cause of acquired chronic disability in children and a major cause of visual loss in children as a result of uveitis. The association between JIA and uveitis is important because visual complications are common (up to 40% of children with uveitis) [2, 3], and can result in decreased visual acuity, and also blindness from untreated macular oedema, ambylopia and glaucoma. Acute anterior uveitis, presenting with a painful red eye is unusual in children and tends to associate typically with enthesitis-related arthritis and the presence of HLA-B27 [4]. The most common form of JIA-associated uveitis is chronic anterior uveitis, which is typically asymptomatic, occurs in all types of JIA with the highest risk being observed in young girls (often pre-school age) with oligo-articular onset and within the first few months of onset of arthritis [1]. JIA-associated uveitis (accounting for 75% of all cases of childhood uveitis) occurs in 12–38% of children with JIA [4–7], with variation in prevalence between JIA subtypes and across different ethnic groups [8]. JIA-associated uveitis may be bilateral and may indeed precede the onset of joint symptoms; a child presenting with visual symptoms usually indicates established eye involvement and has a worse visual prognosis [9]. Invariably, in the early stages, chronic anterior uveitis is asymptomatic and may only be detected by slit lamp examination by an experienced ophthalmologist.

One of the main prognostic factors associated with adverse visual outcome is the presence of uveitis at first ophthalmological evaluation [9, 10]; indeed studies have shown that a significant proportion of children with JIA-associated uveitis have ocular complications including cataract, band keratopathy, posterior synechiae and glaucoma at first assessment [3, 11]. Early detection of uveitis is therefore the most important step in reducing ocular morbidity and children with suspected JIA should be referred for a slit lamp examination as soon as possible and entered into a regular screening programme with a period of surveillance which may last several years. Indications for urgent referral to an ophthalmologist would be the presence of visual symptoms such as blurring, and the presence of an irregular pupil, suspicion of cataract or an absent red reflex. Current guidelines for eye screening are based on consensus between the Royal College of Ophthalmologists and the British Society for Paediatric and Adolescent Rheumatology (http://www.bspar.org.uk/downloads/clinical_guidelines/BSPAR_guidelines_eye_screening_2006.pdf).

The treatment of JIA-associated uveitis depends on the severity of the ocular findings. Essentially, topical corticosteroids remain the mainstay of therapy for mild involvement and this is often all that is required. Peri-ocular corticosteroids and systemic corticosteroids are used for severe exacerbations of uveitis. MTX is used increasingly in management of children with moderate to severe uveitis and follows the observed trend over the last two decades for earlier and more aggressive immunosuppressive treatment of JIA. A significant proportion (at least one-third) of children with oligo-articular onset JIA will develop an aggressive polyarthritis (termed extended oligo-articular JIA) with a guarded articular prognosis. Increasingly, many of these children will receive MTX for their joint disease and despite there being no clinical trials to support MTX use in JIA-associated uveitis, clinical experience suggests that this agent is an effective treatment for uveitis.

The frequency of blindness secondary to JIA-associated chronic anterior uveitis has reduced and generally visual outcomes have improved over the last two decades [5, 6]. Several retrospective studies suggest that improved visual outcomes may be related to early use of MTX [5, 12, 13], although it remains unclear as to whether the observed improvement is due to earlier and greater use of MTX or improved eye screening programmes. For severe uveitis unresponsive to conventional immunosuppressive therapy (including MTX, cyclosporin or mycophenolate mofetil), anecdotal studies suggest that monoclonal antibodies to TNF are effective [14–18]; case reports and small case series show that infliximab is useful in the management of refractory JIA-associated uveitis [19] and recent data suggest that adalimumab may also be efficacious [20, 21]. In contrast, with etanercept, de novo cases of uveitis have been reported in children with JIA [22, 23] suggesting that this agent may not prevent the onset of uveitis. It is clearly important that all children with JIA-associated uveitis are managed by ophthalmologists with an interest in paediatric uveitis and working in close collaboration with paediatric rheumatologists so that early aggressive immunosuppressive therapy can be instituted if required and monitored appropriately (http://www.bspar.org.uk/pages/clinical_guidelines.asp).

The commonest presentation of JIA in the UK is oligo-articular onset subtype and these children have the highest risk of developing chronic anterior uveitis which is invariably asymptomatic in the early stages. Hence, all children with suspected JIA (most of whom are likely to have oligo-articular onset and therefore have high risk of uveitis), are recommended to be referred for ophthalmological screening as soon as possible. However, herein lies a major problem. Several studies have shown that there is a significant delay (often of several months and even years) [10, 24], between the diagnosis of JIA and the initial eye screening visit [10, 24]; this observation is likely to reflect the fact that most referrals for eye screening are initiated by paediatric rheumatologists and delay in access to paediatric rheumatology care is reported in the UK and further afield [25, 26]. The reasons for this delay are undoubtedly complex, but likely to include referral pathways as most children with incident JIA do not present directly to paediatric rheumatologists but to other health care professionals working within primary and secondary care [25].

Children with musculoskeletal problems are common and present in different guises to specialities in primary and secondary care. It is imperative therefore that all doctors are able to identify children with suspected JIA, are aware of the association with uveitis and the need for urgent referral to specialist paediatric rheumatology care and access to eye screening. Currently however, many doctors working in primary and secondary care have poor self-rated confidence in paediatric musculoskeletal clinical skills [27], largely because paediatric musculoskeletal medicine (including clinical skills and knowledge) is not part of core training at medical schools [28] and this needs to be addressed. At postgraduate level within primary care and general paediatrics, the revised ARC GP Learning Guides for primary care (www.arc.org.uk), the e-learning for Health (an NHS initiative with the Royal College of General Practitioners (http://www.rcgp.org.uk) and the emergence of Core Competency Frameworks for all general paediatricians (http://www.rcpch.ac.uk/Training), will incorporate paediatric musculoskeletal clinical skills and knowledge including the association of JIA with uveitis and the importance of early referral to specialist care to optimize outcome. However, the current syllabus for adult rheumatology (http://www.jrcptb.org.uk) has scant reference to paediatric rheumatology, the association of JIA and uveitis is not specifically included and trainees emerging from training programmes are not equipped to deliver clinical care for children with JIA; this reflects a significant change in the delivery of clinical care over the last decade with the emergence of paediatric rheumatology as a sub-specialty and higher specialist training within paediatrics. In many parts of the UK, however, many established adult rheumatologists continue to play an important role in the care of children with JIA although involvement within clinical networks with regional paediatric rheumatology centres is advocated and the role of adult rheumatologists is likely to shift more towards transitional care. In the absence of uniform exposure to paediatric rheumatology within current training programmes, and the likelihood of children presenting with JIA to many specialities within primary and secondary care, we strongly recommend that trainees in adult rheumatology (as well as trainees within orthopaedics, accident and emergency, general paediatrics and primary care), are exposed to such clinical networks to gain an understanding of the importance of early referral to specialist paediatric rheumatology care, and facilitate access to ophthalmological screening. This approach will improve earlier detection and treatment of JIA-associated uveitis, and ultimately improve visual outcomes for these children.

Disclosure statement: The authors have declared no conflicts of interest.

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Accepted 5 January 2009


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E. B. NORDAL, N. T. SONGSTAD, L. BERNTSON, T. MOEN, B. STRAUME, and M. RYGG
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[Abstract] [Full Text] [PDF]

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