Stills disease (Systemic onset Juvenile Idiopathic Arthritis (JIA)) Chronic disease resulting in joint pain and swelling

lling in children Accounts for 10% cases of JIA Affects girls and boys equally up to age 5, then girls more commonly affected Adult onset Stills disease is extremely rare Cause is thought to be autoimmune

Presentation: - systemic Spiking fever (>39C) Tender joints and muscles (arthralgia, myalgia) Increased liver and spleen size (hepatosplenomegaly) Lymphadenopathy, loss of weight, looks like malignancy Lining of lung and heart inflamed (pleurisy, pericarditis) Salmon-pink rash Differentials: Malignancy (leukaemia, neuroblastoma) Infection

Tests and Investigations: High: WCC (neutrophilia), ESR, CRP, LFT, ferritin, thrombocytosis Low: Hb, albumin Autoantibody and RhF ve X-rays and joint aspiration to exclude other causes

Management: Conservative (non-medical)- regular mild exercise (1 hour a day), physiotherapy, splinting, traction, non-weight bearing exercises?, hot water baths to help with early morning stiffness. Medical- start with aspirin/naproxen (NSAIDs- but beware of Reyes syndrome), in severe systemic disease give prednisolone, if unremitting, destructive disease consider DMARDs. Surgical- may be needed to conserve joint function or to replace damaged joints.