Juvenile polyarthritis

(idiopathic juvenile arthritis, Chauffard disease, Chauuffard-Still's disease, Still's disease)

Annual incidence approximately 10/10,000 children. By definition: "arthritis of unknown origin beginning before the age of 16 years, lasting for at least 6 weeks and for which the other causes of arthritis have been excluded". Chronic inflammatory disease of the joints which present also some systemic forms. Oligo- and poly-articular forms are more common among girls while systemic forms affects both sexes in a similar manner. Chronic inflammation of the synovium of the joint  with infiltration by lymphocytes and macrophages.

Clinical picture:

-         painful, hot and swollen joint(s) for several weeks; the joint is typically stiff in the morning or after a period of inactivity

-        fever

-        moderate anemia,  high CRP level

-        in case of involvement of the cervical vertebrae: progressive stiffness of the neck or instability of the axo-atloid articulation

-        stridor if the arythenoid cartilage are involved

-        systemic involvement: skin rash, mild hepatitis, pericarditis, adenopathies.

Treatments: intra-articular injections of steroids, systemic corticosteroids, NSAIDs, immunosuppression (methotrexate, cyclosporine) inhibitors of the cytokine receptors (etanecept, infliximab etc).

The following table shows the current classification of the ILAR (International League of Associations for Rheumatology) of the different types of arthritis in children.


Name

Definition and exclusion criteria

Systemic arthritis

  • arthritis of one or more joints
  • accompanied by or preceded by fever for more than 2 weeks (+ febrile peaks for at least 3 days)
  • accompanied by one or more of the following: evanescent erythematosus rash, lymphadenopathy, hepato-splenomegaly or involvement of one serosa (heart, lung)

Oligoarthritis

-        arthritis of 1 to 4 joints during the first 6 months of the disease

Rheumatoid

(positive rheumatoid factor)

-        arthritis of 5 joints or more during the first 6 months of the disease

-        at least twice a positive blood tests for the rheumatoid factor

Polyarthritis

(negative rheumatoid factor)

-        arthritis of 5 joints or more during the first 6 months of the disease

-        negative blood test for the rheumatoid factor

psoriatic

arthritis

  • arthritis in case of psoriasis
  • or arthritis accompanied by at least 2 of the following elements: punctuated nails, onycholysis, psoriasis in a first degree relative

Arthritis
related to

  • arthritis and enthesitis (or inflammation of tendons their bony insertion point)
  • or arthritis or enthesitis accompanied by at least 2 of the following: sacroiliac joint pain, presence of the HLA B27 antigen, onset in a boy of less than 6 years old, acute anterior uveitis, ankylosing spondylitis, Reiter syndrome, digestive  affection associated to a sacroiliitis or an acute anterior uveitis in a first-degree relative

undifferentiated

arthritis

does not meet any criteria of the other forms of juvenile arthritis


Anesthetic implications:

check hemoglobin and liver enzymes; risk of difficult intubation in case of involvement of the temporomandibular joint and/or cervical vertebrae (fused vertebrae or laxity); sometimes small mouth opening and micrognathia. Careful intubation in case of involvement of the arythenoid cartilages; side effects of the treatment: corticosteroid therapy, immunosuppression, immune modulation; fragile skin; difficult peripheral venous access .


References : 

-         Beresford MW. 
Juvenile idiopathic arthritis : new insights into classification, measures of outcome and pharmacotherapy. 
Pediatr Drugs 2001; 13: 161-73.


Updated: March 2019