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Juvenile idiopathic arthritis (JIA) is a chronic condition affecting different joints of the body in children below 16 years of age and persisting for at least six weeks. As the name suggests, the exact cause of the condition remains unknown. Both genetics and environmental factors are thought to play a role in its development by promoting immune cell activation and widespread inflammation. The global incidence rate of JIA is about 3.7 per 100,000 children, while the prevalence is 16.8 per 100,000. In India, the estimated prevalence of JIA is 48/100,000.
JIA is associated with significantly poor quality of life in children due to severe joint pain, swelling, stiffness in the joints, fever, rash and limited mobility. About 70 % of those children who have an involvement of 4 or more joints (called polyarticular form) reach adulthood with persistent disease activity. There is no cure for JIA at present. However, several treatment advancements in the recent past have made it possible to effectively manage symptoms and delay the progression of the condition.
JIA is a difficult-to-diagnose and difficult-to-manage condition. A significant portion of patients, have persistent disease when the children reach adulthood. The treatment options in the past, such as NSAIDs and oral corticosteroids, only targeted the symptoms. However, with the emergence of biological and conventional DMARDs, there has been a notable decline in the number of children with persistent disease activity and disability
JIA is difficult to diagnose because the symptoms significantly overlap with other diseases, such as joint infection, malignancy, metabolic bone diseases etc. Further, there are no confirmatory clinical, laboratory or imaging tests for the condition. The only way of confirming the condition is thorough clinical evaluation via appropriate investigations. Evidence suggests that about 76 per cent of JIA cases have a delayed diagnosis by about 3 months. Since the disease affects joints during the growth phase, it can result in severe deformities which persist into adulthood.
A delayed diagnosis delays treatment, resulting in worsening of symptoms. Moreover, management in the past was primarily focused on relief of pain, inflammation, and joint stiffness. analgesics and oral corticosteroids were the most common treatment options.
Little emphasis was given on the remission of the disease due to the unavailability of advanced treatment options. This led to progression and worsening of patient conditions over time leading to the following complications:
Patients with polyarticular JIA which persists into adulthood tend to have a higher disease activity and greater need for biologic agents compared to rheumatoid arthritis.
JIA management has witnessed a significant improvement in the past two decades, thanks to the collaborative efforts of several International Organizations, such as the Pediatric Rheumatology Collaborative Study Group, the Pediatric Rheumatology International Trials Organization, American College of Rheumatology, etc.
Many new treatment options are now available, and several more are in the pipeline. Some of the latest treatments include -
Management of this condition also requires a coordination between different specialties such as rheumatologists, ophthalmologists, and physiotherapists to adequately manage the various complications associated with disease. Intra-articular steroids are primarily used to relieve pain and stiffness by reducing inflammation. DMARDs such as methotrexate helps to slow the progression of JIA and also relieve symptoms in those cases where NSAIDs do not help. Latest American guidelines strongly recommend the use of DMARDs over NSAIDs in children with active disease and with multiple joint involvement. Biologics such as etanercept, adalimumab, golimumab, infliximab, tocilizumab, etc. are effective in reducing systemic inflammation and prevent joint damage.
The goal of JIA treatment should be to prevent joint destruction, promote growth and development in children along with effective relief from symptoms. A multidisciplinary team approach including a rheumatologist, ophthalmologist, physiotherapist, orthopaedic surgeon is required for the optimal management of JIA. In recent years, newer treatment strategies have helped to reduce disease severity and improve quality of life immensely in children with JIA.
(This article is authored by Dr Abhishek Patil, Consultant - Rheumatology, Manipal Hospitals)