Juvenile Idiopathic Arthritis (JIA)

Juvenile Idiopathic Arthritis (JIA) is a group of connective tissue disorder in childhood, in which there is chronic arthritis lasting more than  6 weeks, presenting before the age: 16 years. It is classified according to onset as:

• Systemic
• Poly-articular with more than 4 joints involvement
• Pauci-articular \ Oligo-articular up to & including 4- joints

It is further classified according to the presence of ANA (Anti-nuclear antibodies), HLA-B27, Rheumatoid Factor and clinical examination. Infection and other causes must be excluded.

1. Systemic Arthritis in children (Still’s Disease),

• usually affects young children and is characterized by:
• Acute illness, marked malaise
• High spiking fever
• Salmon-Pink Rash at height of fever
• Aches & pains in joints and muscles(Arthralgia \ myelgia), but no arthritis at presentation.
• Lymphadenopathy, Hepato-spleenomegaly, occasional pericarditis
• Anemia, Increased neutrophils & platelet count & marked increase in acute phase reactants
• Some children recover without developing chronic arthritis. Others progress to chronic polyarthritis.

2. Polyarticular Arthritis:

• Occurs at all ages, more in girls than boys
• Any joint may be affected, usually symmetrical joint involvement of wrist, hands, knees, ankles, Cervical spine and Temporo-Mandibular Joint
• Few female children in 2^nd decade have symmetrical polyarthritis and adult Rheumatoid disease.

3. Pauciarticular / Oligoarthritis:

• Occurs in young children and affects Knees, less often ankles and wrists
• Increased risk of developing Eye disease: chronic anterior uveitis in ANA positive females.
• In some children large joints become involved during first six months, and are categorized as extended oligoarthritis.

4. Enthesities related arthritis (Juvenile Spondylo-arthropathy)

• Have HLA-B27 type tissue, associated arthritis
• Affects older boys who presents with large joint arthropathy usually of lower limbs or swollen digit (sausage finger)
• Positive family history
• Inflammation of tendons into bone; Tendo-Achillis & Planter fascia
• Later there may be Sacro-ileitis & Spinal involvement
• Acute symptomatic Iritis (eye infection) occur in children

5. Juvenile Psoriatic Arthritis

• Involves inter-phalageal (Finger) joints. May present with sausage shaped swelling of digit.
• May occur before onset of skin lesions or nail biting for inclusion in this category. There need to be either arthritis or Psoriasis or arthritis plus two dactylitis, nail abnormality and family history of Psoriasis in 1^st degree relative.

Management requires multi-disciplinary team

Physiotherapy is an essential component to encourage mobility and maintain full range of joint movement and muscle strength.

• Daily exercises
• Hydro-therapy is helpful
• Resting splints to prevent flexion contractures
• Working splints for wrists to maintain posture while writing

Pain control & suppression of inflammation

• By non-steroidal anti-inflammatory (NSAIDS): Naproxan or Ibuprufan
• Inter-articular steroid therapy for Pauci or polyarticular disease

Disease-modifying Anti-Rheumatoid drugs used for persistent active polyarthritis not controlled by NSAID:

• Methotrexate: oral or sub-cutaneous. Monitor side effects
• Salzopyrin: for those refractory to treatment.
• Cyclosporine:
• Corticosteroids systemic for Uveitis (Eye infection), pericarditis
• Anti-TNFα (Anti-Tumor Necrosis Factor-alpha)
• Autologous Bone Marrow Transplant

Dr. U. B. Lall is a practising paediatrician in New Delhi. His practice has spanned over five decades and has retired as the Head of the department, Paediatrics from Moolchand Hospital. He has published various articles in International journals and is a recipient of the prestigious S. T. Achar award in 1974.