Flashcards in Juvenile Idiopathic Arthritis Deck (32)
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1
What is juvenile idiopathic arthritis?
-Group of systemic inflammatory disorders affecting children below age of 16 years.
-The most commonly diagnosed Rheumatic disease in children.
2
What is juvenile idiopathic arthritis an important cause of?
Disability and blindness
3
What is the aetiology and pathogenesis of JIA?
-JIA is an auto-immune disease.
-Etiopathogenesis is multi-factorial and different from that of adult RA.
-Strong subset-specific genetic markers may affect immune response
4
What is the criteria for diagnosis of JIA?
Age of onset
-<16 years
Duration of disease
->6 weeks
Presence of arthritis (Joint swelling or 2 of the following:)
-Painful or limited joint movement
-Tenderness
-Warmth
5
When can subtypes of JIA be identified?
After 6 months
6
What are the 3 major subtypes of JIA?
-Pauciarticular 55%
-Polyarticular 25%
-Systemic onset 20%
7
What clinical subtypes of JIA have recently been identified?
-Enthesopathy related arthritis
-Juvenile psoriatic arthritis: oligo or spondylo with psoriasis or potential psoriasis
-Others: unclassified under above criteria
8
How is pauciarticular JIA divided?
-Type I 25%
-Type II 15%
-Type III 15%
9
What is the classical presentation of type I pauciarticular JIA?
-Preschool girls
-Limp rather than pain
-Mainly LL joints
-Knee>ankle>hand or elbow (hip very rare)
-Chronic uveitis
-Irregular iris due to posterior synechiae
- +ve ANA
-Can be asymptomatic
10
How is pauciarticular JIA defined?
4 or less joints
11
What is the classical presentation of type II pauciarticular JIA?
-Primary aged boys (8-9)
-Limp due to LL affection
-Mainly LL joints: knee and ankle
-Hips may be affected early with rapid damage requiring THR
-Enthesitis
-Can develop AS or spondyloarthritis
12
Which individuals with type II pauciarticular JIA are categoreised as juvenile ankylosing spondylitis?
Those with HLA-B27 + back involvement
13
What is the classical presentation of type III pauciarticular JIA?
-Girls during childhood
-Asymmetric UL and LL arthritis
-Dactylitis
-Chronic iridocyclitis
-FH of psoriasis
14
What can happen to those with extended oligoarthritis?
30% of these presenting with pauciarticular JIA can go on to more severe polyarticular course.
15
How is polyarticular JIA defined?
5 or more joints
16
What is the classical presentation of RF- polyarticular JIA?
-Girls of any age (often early)
-Constitutional manifestations (low grade fever, malaise)
-Hepato-splenomegaly
-Mild anemia
-Growth abnormalities
-Symmetric large and small joints affection: knees, wrists, ankles, MCPs, PIPs, neck
-Iridocyclitis rare
17
What is the classical presentation of RF+ polyarticular JIA?
-Girls in later childhood (including teens)
-Constitutional manifestations (low grade fever, malaise, weight loss)
-Anaemia
-Nodules
Iridocyclitis is rare
-Similar to adult RA
18
What can be seen on x-ray of RF+ polyarticular JIA?
Erosions which occur early
19
What can complicate RF+ polyarticular JIA?
-Sjorgen's
-Felty
-Vasculitis
-AR
-Pulmonary fibrosis
-AAS
-CTS
20
How is systemic onset JIA defined?
Extra-articular features which start early and disappear after 2-5 years
21
What is another name for systemic onset JIA ?
Still's disease
22
What is the classical presentation of Still's disease?
-F:M 1.5:1
-Occurs throughout childhood (4-6 years)
-Joints affected include wrists, knees, ankles, cervical spine, hips and TMJ
-Evanescent salmon red eruption on trunk and thighs accompanised by fever. Exhibits Koebner's phenomenon
23
What extra-articular features can be present in Still's disease?
-Pleural effusion
-Pulmonary fibrosis
-Polyserositis
-Pericarditis
-Tamponade and myocarditis
-Generalised non-tender lymphadenopathy
-Hepatosplenomegaly
-Abdominal pain +- transaminases
24
Describe the associated fever of Still's disease.
-Rise to 39.5 C daily for at least 2 weeks
-Late in afternoon or evening and returns to normal or subnormal in the morning
-Child appears toxic with fever +/- chills but looks normal when fever goes away
25
What is the 1st line therapy for JIA?
-Simple painkillers
-NSAIDs
26
What is the 2nd line therapy for JIA?
If no response to NSAIDs/ joint (steroid) injections.
(Rarely needed in oligoarticular JIA)
-Methotrexate (pharmocokinetics is age related).
-Anti-TNF Rx. (all 3): In methotrexate failure.
-IL-1 R-antagonist (Anakinra) in refractory systemic arthritis.
-IL-6 antagonist (Tocilizumab) for refractory systemic disease.
27
Why is there limited indications for systemic steroids?
Due to their serious side effects
28
What are systemic steroids used in?
-Systemic JIA (control pain and fever)
-Serious disease complications with any subtype e.g. periocordial effusion, tamponade, vasculitis, severe auto-immune anemia, severe aye disease
-As a bridge between DMARDs
-Children undergoing surgery
29
What are the risks of systemic steroid use?
-Osteoporosis
-Infections
-Growth abnormalities
30
