Juvenile idiopathic arthritis history and symptoms
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Dheeraj Makkar, M.D.[2] Nehal Eid, M.D.[3]
Overview
Juvenile idiopathic arthritis has been recognized for over a century, evolving from early descriptions of chronic childhood arthritis to a modern understanding of a heterogeneous group of immune-mediated diseases. Advances in immunology, classification systems, and targeted therapies have significantly improved diagnosis, management, and long-term outcomes for affected children.
History
The history of juvenile idiopathic arthritis (JIA) dates back to early descriptions of chronic childhood arthritis in the late 19th and early 20th centuries. Historically, these conditions were grouped under the term juvenile rheumatoid arthritis.
- Early classifications were based primarily on clinical patterns of joint involvement
- Advances in immunology led to recognition of distinct autoimmune and autoinflammatory mechanisms
- The term juvenile idiopathic arthritis was adopted to reflect the heterogeneity and unknown etiology of the disease
- Modern classification systems incorporate clinical features, laboratory findings, and disease course
Over time, improved understanding of disease pathogenesis and the introduction of disease-modifying and biologic therapies have transformed the management and outcomes of children with JIA. [1]
Common Symptoms
The onset of symptoms is frequently dependent on the age and subtype of JIA:
- Oligoarticular JIA predominantly affects girls aged 1–5 years.
- Polyarticular JIA:
- RF-Negative subtype has bimodal peak incidence between 1-3 years and >8 years.
- RF-Positive subtype usually affects older children and adolescents, uncommon under the age of 9. It can be more destructive without treatment.
- Systemic JIA can occur at any pediatric age. Early in the disease course, systemic JIA resembles monogenic autoinflammatory syndromes such as familial Mediterranean fever.[2] Chronic arthritis appears later in the disease course.
- Enthesitis-related arthritis is uncommon before 6 years of age usually affecting children older than 9 years. HLA-B27 positivity is associated with more severe disease. In older children, it resembles spondyloarthritis.
- Psoriatic JIA has bimodal peak incidence at 2-4 years of age and >10 years of age. In younger children,it is similar to early-onset oligoarticular and rheumatoid factor–negative polyarticular JIA, with ANA positivty and female predominance.
Symptoms of JIA are often non-specific initially and include lethargy, reduced physical activity, and poor appetite. The first manifestation, particularly in young children, may be limping. Children may also become quite ill, presenting with flu-like syptoms that persist. The cardinal clinical feature is persistent swelling of the affected joint(s), which commonly include the knee, ankle, wrist and small joints of the hands and feet. Swelling may be difficult to detect clinically, especially for joints such as those of the spine, sacroiliac joints, shoulder, hip and jaw, where imaging techniques such as ultrasound or MRI are very useful.
Pain is an important feature of JIA, but young children may have difficulty in communicating this symptom. Late effects of arthritis include joint contracture (stiff, bent joint) and joint damage. Children with JIA vary in the degree to which they are affected by particular symptoms.
Arthritis symptoms:
- Joint stiffness when you wake up in the morning
- Joint pain
- Limited range of motion
- Joints may be warm or swollen and sometimes red
- A child may stop using an affected limb or may limp
- Back pain
Body-wide JRA symptoms:
- Fever, usually high fevers every day
- Rash (trunk and extremities) that comes and goes with the fever
- Swollen lymph nodes (glands)
Extra-articular manifestations
- Eye disease: JIA is associated with inflammation in the front of the eye (chronic anterior uveitis) which affects 10 to 30% of patients. 90% of chronic anterior uveitis occurs in the first 4 years after diagnosis. Acute anterior uveitis affects 11 to 13% of JIA patients. This complication may not have any symptoms or someone may have:
- Red eyes
- Eye pain
- Increased pain when looking at light (photophobia)
- Vision changes
- Growth disturbance: Children with JIA may have reduced overall rate of growth, especially if the disease involves many joints or other body systems. Paradoxically, individually affected large joints (such as the knee) may grow faster, due to inflammation - induced, increased blood supply to the bone growth plates situated near the joints.