Juvenile idiopathic arthritis Flashcards
What is JIA?
What can it cause in young people as well?
Group of systemic inflammatory disorders affecting children below age of 16 years.
The most commonly diagnosed Rheumatic disease in children.
An important cause of disability and blindness.
history and pathogenesis
- what may affect the immune response?
auto immune
- Etiopathogenesis is multi-factorial and different from that of adult RA.
- Strong subset-specific genetic markers may affect immune response.
Most common cause for physical disability ?
JIA
Etiology and Pathogenesis
Age of onset?
Duration of disease?
Presence of arthritis?
<16 years
> 6 weeks
Joint swelling or 2 of the following:
Painful or limited joint motion
Tenderness
Warmth
Clinical subtypes of JIA
-most common subtype?
Pauciarticula
most common subtype divided into
Rheumatoid factor positive and negative
all subtypes are greater in females apart from what one?
enthesitis related
Pauciarticular JIA (4 or less joints) - describe the 3 types - type 1
Majority of pauci (25%)
Age: before 5 years, peak 1-3 years
Girls: boys = 8:1
Presentation of children with arthritis
- limp rather than pain
No constitutional manifestations
Pauciarticular JIA (4 or less joints) - what joints are involvement? - type 1
Mainly LL joints
Knee> ankle> hand or elbow (hip very rare).
What is positive in Pauciarticular JIA - type 1
- what is common that can be asymptomatic?
ve ANA in 40-75%
Chronic uveitis in 20% of cases (95% if female < 2years old)
Asymptomatic in 50%
Irregular iris due to posterior synechiae (get eyes looked at)
Pauciarticular JIA - type 2
- what joints are affected?
More common in boys
can get acute episodes
Mainly LL joints: knee, ankle.
Hip can be affected early with rapid damage requiring THR early in life + enthesitis + many have sacroilliac joints and may evolve AS or spondyloarthritis
20% difficult to classify to particular spondyloarthropathy group.
Pauciarticular JIA - type 2 - those who lack back problems called?
slightly older patients
into sero-ve enthesopathy arthropathy (SEA) syndrome who less frequently inherit HLA-B27
Those with HLA-B27 + inflammatory back symptoms can be termed as having?
Juvenile Ankylosing spondylitis.
Pauciarticular JIA - type 3 - features - what can it be associated with?
younger patients
constitutional rare
asymmetric UL and LL arthritis
dactylitis.
Arthritis can be very destructive
Family history of psoriasis in 40%
+/- nail pitting
These patients may develop psoriasis later in life
Pauciarticular JIA - type 3 - what can they develop
Chronic iridocyclitis in 10-20%.
Pauciarticular JIA - what can they go on to develop?
Extended oligoarthritis:
30% of these presenting with pauciarticular JIA can go on to more severe polyarticular course.
WHta is dactylitis?
nail bed
linked with psoriasis
- fungal infections
Polyarticular JIA (5 or more joints) - rheumatoid factor negative?
girls more than boys
- early age
Polyarticular JIA (5 or more joints) - rheumatoid factor negative - presentation
Constitutional manifestations (low grade fever, malaise)
Hepato-splenomegaly
Mild anemia
Growth abnormalities
Symmetric large and small joints affection: knees, wrists, ankles, MCPs, PIPs, neck
What is rare in Polyarticular JIA negative
Iridocyclitis rare.
Polyarticular JIA (5 or more joints) - rheumatoid factor positive - presentation
later years
Presentation:
Constitutional manifestations (low grade fever, malaise, weight loss)
Anemia
Nodules.
Systemic complications of Polyarticular JIA (5 or more joints) - rheumatoid factor positive
Can be complicated by sjogren’s, Felty or vasculitis, AR, pulmonary fibrosis, AAS, CTS.
Similar to adult RA but in a child
Erosions in x ray occur early
Systemic onset JIA - what defines ?
Extra-articular features define the disease
Systemic onset JIA - presentation
fever that rises daily and goes back to normal - presentationt for 2 weeks
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
Systemic onset JIA - what is there a typical clinical sign of? GIVE features
RASH 90% Evanescent salmon red eruption On trunk and thighs Accompanies fever Can be brought by scratching (+ve Koebner’s phenomenon).
Systemic features of Systemic onset JIA
Generalized lymphadenopathy Hepatosplenomegaly 50-75% Abdominal pain \+/- transaminases - Polyserositis Pericarditis in 36% Tamponade and myocarditis rare
Rare
Pleural effusion
Pulmonary fibrosis
Systemic onset JIA - what is the arthritis like
when does it present?
joints affected?
poly arthritis
Within 3-12 months of onset of fever
Wrists, knees, ankles, cervical spine, hips and TMJ.
Important clinical sign
loss of hyper mobility
What should you detect in children with arthritis?
jaw movement
- jaw ma be pulled towards the affected side
When are there higher levers of uveitis (2)
oligo-articular and extended oligo groups
- related to ANA positivity in EOIJA
Uveitis complications (3)
lots of patients will develop it
potential blindness
- can affect all groups
early in the disease course
- posterior synechiae - iris sticks onto the Lens
- cataract
- band keratopathy
- glaucoma
- eye surgery
- Sicca syndrome - dry eyes
Treatment and management of uveitis
screening!!! - all age groups
treatment:
- steroids : typical: intraoccular
- mydriatic agents/cycloplegic agents
- Methotrexate
- MMF
- Ciclosporin
Anti-TNF
1st line therapy?
some features you must consider with NSAIDS
Simple pain killers NSAIDs: Difference between adults and children half life Can control disease Doses Same compounds only
2nd line therapy in children? what drugs do you use (1-4)
when are these drugs used?
when are these drugs rarely needed?
Methotrexate (pharmocokinetics is age related).
(2) Anti-TNF Rx. (all 3): In methotrexate failure.
(3) IL-1 R-antagonist (Anakinra) in refractory systemic arthritis.
(4) IL-6 antagonist (Tocilizumab) for refractory systemic disease
If no response to NSAIDs/ joint (steroid) injections.
- Rarely needed in oligoarticular JIA
Systemic steroids
When are they used?
Limited indications due to serious side effects
- 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
What are the risks of systemic steroids?
of osteoporosis, infections, growth abnormalities
Local steroids are used mainly in?
how are they used?
- Oligo-articular JIA
- TOPICALLY for the Eye disease (ANA +ve oligo-articular disease).
Surgical treatment (2)
- Synovectomy
- Reconstructive / joint replacement surgery
Growth failure in JIA? the 2 types and features of both
localised - leg length discrepancies, shortening of fingers, hands, forearms, toes and feet, JAW - Mircrognathia (poor growth of the mandible) - receding chin
Generalised
- related to severe systemic
- short stature
- delayed puberty
- systemic steroids
Mircrognathia - often need (3)
orthodontic intervention
receding chin
- TMJ resistant = vigilant in MRI scanning, intra- ocular steroids to prevent growth failure
What is not a diagnostic factor?
ANA - important to do it - helps to categorise their arthritis as much as possible
