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Chapter: Paediatrics: Bones and joints

Paediatrics: Systemic arthritis

Multisystem disease is often diagnosed late as joint involvement is often late. Peak age 2–3yrs; 10–20% JIA; equal male:female.

Systemic arthritis

 

Multisystem disease is often diagnosed late as joint involvement is often late. Peak age 2–3yrs; 10–20% JIA; equal male:female.

 

Clinical features

 

   Fever is essential, typically quotidian up to 39°C, returning to normal between attacks.

   Rash: salmon pink, macular/urticarial on chest, trunk, and intertrigones. Present when warm and disappears within minutes.

   Myalgia, arthralgia, and arthritis. Arthritis often appears after first 6mths of illness and can be oligo- or polyarthritis.

   Generalized lymphadenopathy and hepatosplenomegaly.

   Polyserositis with pericarditis, pleuritis, and sterile peritonitis. Silent pericardial effusions (15%). Myocarditis + tachycardia, cardiomegaly, and congestive cardiac failure is rare.

   Growth retardation s to disease, steroids, or joint damage.

   Late complications: amyloidosis (difficult to treat).

   MAS: rare, life-threatening; precipitated by infection or NSAIDs. Haemophagocytic bone marrow with falling WBC, platelets, and ESR, and very high ferritin.

 

Investigations

 

   FBC (normocytic or hypochromic anaemia; leucocytosis; thrombocytosis).

   ESR/CRP can be high: use to monitor disease during treatment.

   Hypoalbuminaemia: multifactorial—poor diet, general ill health with catabolism, possibly proteinuria secondary to renal amyloid.

   ANA and RF usually –ve

   Viral titres and blood cultures.

   Malignancy screen: CXR, US abdomen.

   ECG and echocardiogram.

 

Management

 

   NSAIDs for initial management of pain, fever, and serositis. Indomethacin often used for pericarditis.

   Pulsed IV corticosteroids if no improvement after 1wk of NSAIDs.

   Oral steroids at 1mg/kg in divided doses until fever settled and inflammatory markers normal. Taper dose to reduce side-effects. Use alternate day doses and add steroid-sparing agent.

   MTX is used, but is not as effective as in other JIA subsets.

   Intra-articular corticosteroid for flares of single joints.

   Biological therapy: anti-TNF and anti-IL6 in resistant cases.

 

Prognosis

 

Three groups—monocyclic (11%); recurrent or polycyclic (34%); and per-sistent (55%). Monocyclic patients do well. More than 33% of the others will have permanent disability with active disease in adult life. Death from infection, MAS, or amylodosis.

 

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Paediatrics: Bones and joints


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