enthesis related juvenile idiopathic arthritis

Last reviewed 01/2018

enthesitis realted juvenile idiopathic arthritis

Enthesitis related arthritis (ERA) is a condition which mainly affects male patients after the age of 6 years and is characterized by the association of enthesitis and arthritis.

  • it is considered as one of the most controversial conditions in paediatric rheumatology
  • both JIA and juvenile spondyloarthropathie features are seen in these patients
  • several names were used to describe ERA - e.g. - type 2 oligoarticular JIA, juvenile chronic arthritis (JCA)  with late onset, seronegative enthesopathy and arthropathy, arthropathy associated with HLA B27 or juvenile spondyloarthropathy with early onset. 

In patients with ERA:

  • RF and ANA are negative
  • HLA B27 positivity is reported in 65-80 % 
  • enthesopathy and asymmetric arthritis of the lower extremities are characteristic features   
    • enthesopathy
      • presence of  inflammation of the attachment sites of the tendons to the bones
      • Achilles tendon is the most commonly affected site while patellar insertion of the quadriceps tendon, and calcaneal and metatarsal insertions of the plantar fascia could be affected 
      • there is pain and sensitivity at the affected site
    • arthritis
      • asymmetric oligoarticular lower-extremity arthritis are seen typically
      • knee and ankle are affected commonly
      • arthritis could progress to affect the sacroiliac and spinal joint
  •  primary extra articular manifestation in acute anterior uveitis

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