Idiopathic arthritis

Specialties > Child health

Background knowledge 🧠

Definition

Idiopathic arthritis is a term used to describe a group of arthritis conditions without a known cause.
Includes conditions such as juvenile idiopathic arthritis (JIA) and adult-onset idiopathic arthritis.
Characterised by chronic joint inflammation, leading to pain, swelling, and stiffness.
Diagnosis is typically made after excluding other known causes of arthritis.

Epidemiology

Juvenile idiopathic arthritis is the most common type of arthritis in children, affecting approximately 1 in 1,000 children in the UK.
Adult-onset idiopathic arthritis is less well-defined, with variable prevalence depending on specific diagnostic criteria used.
Slight female predominance is noted in most forms of idiopathic arthritis.
No significant racial or ethnic predilection has been identified.

Aetiology and Pathophysiology

Exact cause is unknown, hence the term idiopathic.
Genetic factors may play a role, as some forms of idiopathic arthritis are associated with HLA genes.
Immune system dysregulation is central to the pathophysiology, leading to chronic inflammation and joint damage.
Environmental factors, such as infections, might trigger or exacerbate the condition.

Types

Juvenile Idiopathic Arthritis (JIA): Includes oligoarticular, polyarticular, and systemic onset types.
Adult-onset Idiopathic Arthritis: A less clearly defined group, often overlapping with seronegative rheumatoid arthritis.
Seronegative arthritis: A subgroup with negative rheumatoid factor and anti-CCP antibodies.
Systemic idiopathic arthritis: A form that affects internal organs in addition to joints.

Clinical Features 🌡️

Symptoms

Joint pain, often worse in the morning or after periods of inactivity.
Joint stiffness, particularly in the morning, lasting more than 30 minutes.
Swelling and warmth in the affected joints.
Systemic symptoms in some forms, such as fever, fatigue, and weight loss.

Signs

Tenderness and swelling in the affected joints.
Reduced range of motion in affected joints.
Signs of chronic joint damage, such as deformities or contractures in advanced cases.
In systemic forms, signs of organ involvement such as hepatosplenomegaly or lymphadenopathy.

Investigations 🧪

Tests

Blood tests: CRP, ESR, full blood count, and liver function tests to assess inflammation and systemic involvement.
Autoantibodies: Rheumatoid factor and anti-CCP are typically negative in idiopathic arthritis but may be tested to rule out other conditions.
Imaging: X-rays, MRI, or ultrasound to assess joint damage and inflammation.
Synovial fluid analysis: Excludes infectious and crystal-induced arthritis.
Genetic testing: In some cases, HLA typing may be considered.

Management 🥼

Management

Pharmacological: NSAIDs, DMARDs (e.g., methotrexate), biologics (e.g., TNF inhibitors).
Non-pharmacological: Physiotherapy, occupational therapy, and patient education.
Surgical: Joint replacement or synovectomy in severe cases.
Regular monitoring: To assess disease activity and treatment side effects.

Complications

Chronic joint damage and deformity.
Osteoporosis due to chronic inflammation and steroid use.
Growth retardation in children with JIA.
Psychosocial impact: Depression, anxiety, and reduced quality of life.
Complications of systemic involvement: e.g., pericarditis, uveitis.

Prognosis

Varies widely depending on subtype and severity.
Juvenile forms may remit in adulthood, but some persist.
Risk of long-term disability if not adequately managed.
Early and aggressive treatment improves outcomes.
Regular follow-up is crucial to manage and monitor disease progression.

Key Points

Idiopathic arthritis is a diagnosis of exclusion with unknown cause.
Chronic inflammation leads to joint damage and potential systemic involvement.
Management requires a multidisciplinary approach.
Regular monitoring and early treatment are key to improving outcomes.

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