Juvenile idiopathic arthritis: improved outcome requires improved access to care

Driving referral to paediatric rheumatology Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease in children worldwide [1] with a range of clinical presentations and outcomes [2]. Improving the outcome for children with JIA is an important goal in paediatric rheumatology, and current management of severe JIA involves increasingly aggressive immunosuppression [2, 3], although often based on extrapolation of clinical experience in adult RA. The advent of immunomodulatory treatments have resulted in an improved quality of life for many children and the need for improved clinical trials in JIA has been highlighted [4]. Undoubtedly, improving outcome in JIA depends on the availability of effective, safe treatments; but if a child does not have access to treatment at all or presents late in the disease course, then outcome is likely to be sub-optimal. This editorial focuses on delay and inequity to appropriate care in JIA, although similar problems may be encountered in other childhood rheumatic diseases, and we note a recent publication highlighting sub-optimal access to care for adults with RA [5]. Table 1 summarizes studies within different health-care systems around the world, demonstrating that for many children, there is a prolonged interval from onset of disease to paediatric rheumatology care, although notably these studies report different methodologies with selection bias towards specialist centres. Despite there being no published data from developing countries, anecdotal observations suggest that poor access to optimal care is likely a global issue. Summary of studies reporting interval from onset of symptoms to paediatric rheumatology care Summary of studies reporting interval from onset of symptoms to paediatric rheumatology care Defining delay in access to care is not easy given that the definition of JIA requires the presence of 6 weeks of persistent arthritis [15]; pragmatically, in a cohort study of incident cases of JIA in the UK [8], delay was defined as 10 weeks from onset of symptoms to first paediatric rheumatology assessment. Within this cohort, >75% of the children attending paediatric rheumatology clinics for the first time exceeded this interval, and notably before this none had been referred for eye screening (to detect chronic anterior uveitis), many had not received appropriate analgesia and, at first assessment, many had prolonged untreated active disease and presented with multiple restricted joints. Furthermore, for those requiring MTX or joint injections, the median interval from onset of symptoms to receiving treatment was 10 months. The impact of such a delay is as yet unknown in the long term but likely to adversely affect clinical outcomes, given that lower treatment response is associated with a longer interval from onset to starting MTX [16]: the longer the interval to injection of the joints, especially in young children, the greater the risk of unequal leg length, muscle wasting and functional disability [17]; and delay in eye screening results in many children being at a risk of undetected, untreated uveitis with poor visual outcomes [18]. Furthermore, the psychosocial effect of the child in pain and the family living with an uncertain diagnosis is profound albeit difficult to quantify. Access to appropriate treatments is often limited by cost, such as parenteral MTX and, in particular, biologic agents that are not available in many parts of the world. In the UK and within rheumatology, rationing of health care for expensive treatments such as biologics is reported and often geographically dependent on local health budgets. In the USA, children with JIA covered by basic health-care insurance are less likely to have screening of the eyes [19]. Inequity in care includes access to appropriately trained specialists and, in many parts of the world, there is sub-optimal provision of paediatric rheumatologists and, in many areas, clinical care is often delivered by adult rheumatologists [20, 21]. Notably, adult rheumatology training in the UK no longer includes paediatric rheumatology and clinical care currently provided by adult rheumatologists poses a potential problem for the future. In many parts of the UK, current paediatric rheumatology services are under resourced even when judged against national recommendations set out many years ago [22], and this shortfall is likely to be greater with increasingly complex treatment regimes. Inappropriate care refers to management without the norms of current paediatric rheumatology clinical practice, with many anecdotal reports of children with incident JIA being subjected to multiple and often invasive investigations (such as arthroscopy or synovial biopsy); such procedures are unnecessary in most cases and likely contribute to delay in referral to paediatric rheumatology [23]. The reasons for delay, inequity and inappropriate care are likely to be multi-factorial but increasingly important to address as a major challenge to improve outcome for children with JIA. Pathways of care for children with suspected JIA are complex, vary across health-care systems and invariably children present to health-care professionals in primary or secondary care who are most probably not experts in paediatric musculoskeletal medicine; orthopaedics are reported to have the longest interval in the pathways of referral to paediatric rheumatology care [8, 13]. The clinical presentation at onset may well have an effect on the referral process, because children with severe subtypes of JIA are profoundly unwell and hence tend to have shorter referral intervals [7, 8, 13]. Many doctors in primary and secondary care (such as general paediatrics and orthopaedics) are not confident in their musculoskeletal assessment of children [24, 25], which is not surprising as paediatric musculoskeletal medicine is not part of the core training in medical schools [26], and this may contribute to delay in diagnosis of JIA and other conditions presenting with musculoskeletal features [27, 28]. Awareness and recognition of suspected JIA needs to be raised among doctors in primary and secondary care who are likely to encounter children (especially doctors working within orthopaedics, accident and emergency and general paediatrics). In the UK, an important step forward has been the introduction of competency frameworks for all general paediatricians in training [29], which include musculoskeletal themes and have been incorporated in the mandatory professional assessments since early 2009. The paucity of appropriately trained paediatric rheumatology multi-disciplinary teams contributes to inequity to appropriate care. The British Society for Paediatric and Adolescent Rheumatology Standards of Care for children with JIA [30] stipulate optimal paediatric rheumatology services; these standards used to audit current clinical provision will aid efforts to lobby for further personnel and resources to deliver high-quality care. Moreover, hitherto unaddressed is the need to understand the factors that drive or hinder the referral to paediatric rheumatology. Irrespective of the diagnosis, the visibility, persistence and perceived severity of symptoms are central to a parent's actions around help-seeking behaviour [31]. Parents will notice a problem, but may not be able to judge the actual severity and need to seek expert advice [32]. In consultations with health professionals, parents often act as advocates for their sick child and have to balance between being seen as responsible and vigilant against being labelled as overly neurotic, protective or even exploitative [31]. When parents of children with cancer first reported that their child was unwell, their concerns were often discounted initially and they struggled to obtain further investigations and a diagnosis of cancer [33]. Anecdotally, parents of children with JIA are sometimes spurred on to seek health care by observations from teachers or nursery workers who may have noticed altered function in the child during school or play activities. Centrally, inequalities of referrals to appropriate care often stem from inequalities of knowledge and experience of the health professionals; experience with a prior case or some prior contact with a specialist service, or both, are likely key drivers in triggering concern and a prompt referral. Clearly, more work is required with need to identify and understand the barriers and drivers in the referral process, identify and address the training needs of doctors likely to encounter children presenting with suspected JIA, and also identify strategies to empower parents and carers to seek appropriate health-care attention. Disclosure statement: The authors have declared no conflicts of interest.