Juvenile Idiopathic Arthritis Flashcards
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
An important cause of disability and blindness
a type of arthritis that causes joint inflammation and stiffness for more than six weeks in a child aged 16 or younger.
what is the Etiology and Pathogenesis of JIA?
- nJIA 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.
what is the criteria for diagnosis?
Age of onset = < 16 years
Duration of disease = > 6weeks
Presence of arthritis = Joint swelling or 2 of the following:
- Painful or limited joint motion
- Tenderness
- Warmth
Clinical subtypes of JIA: After 6 months 3 major subtypescan be identified
Which will help to suspect:
- The natural history
- Complications
- Prognosis
- Decide the strategy of treatment
what are the different subtypes?
summary table of the different types:
what is and how common is Pauciarticular JIA?
4 or less joints
Oligoarticular juvenile idiopathic arthritis (formerly called pauciarthritis or pauciarticular-onset juvenile rheumatoid arthritis) is defined as juvenile idiopathic arthritis (JIA) involving fewer than five joints. It is the most common subgroup, constituting approximately 50 percent of cases of JIA
who does type 1 Pauciarticular JIA occur in?
- Majority of pauci (25%)
- Age: before 5 years, peak 1-3 years
- Girls: boys = 8:1
what are the symptoms of type 1 Pauciarticular JIA?
- Presentation: limp rather than pain
- No constitutional manifestations
- +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
where does type 1 Pauciarticular JIA affect?
- Mainly LL joints
- Knee> ankle> hand or elbow (hip very rare).
who does type 2 pauciarticular JIA affect?
- 15%
- Age: after 8-9
- Girls: boys = 1:7
what are the symptoms and presentation of type 2 pauciarticular JIA?
- Presentation: constitutional rare, limp due to LL affection
- 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
- Acute iridocyclitis in 10-20%
in type 2 pauciarticular JIA, those with HLA-B27 + back involvement will be categorized as what?
Juvenile Ankylosing spondylitis
Type 3 pauciarticular JIA affects who?
- 15%
- Age: any age during childhood
- Girls: boys = 4:1
what are the symptoms and presentation of type 3 pauciarticular JIA?
Presentation:
- constitutional rare
- asymmetric UL and LL arthritis
- dactylitis (severe inflammation of the finger and toe joints)
- Arthritis can be very destructive
- Family history of psoriasis in 40%
- +/- nail pitting
- These patients may develop psoriasis later in life
- Chronic iridocyclitis in 10-20%.
whatis extended oligoarthritis?
30% of these presenting with pauciarticular JIA (less than4 joints involved) can go on to more severe polyarticular course
what is the seocnd most ocmmon type of JIA?
what are the 2 different types of Polyarticular JIA?
Polyarticular JIA RF negative and RF positive
who does polyarticular JIA RF negative occur in?
- 15% of JIA
- Age: any age, often early
- Girls: boys = 9:1
what is the presentation of RF negative polyarticular JIA like?
Constitutional manifestations (low grade fever, malaise)
Hepato-splenomegaly
Mild anemia
Growth abnormalities
• Iridocyclitis rare
where does RF negative polyarticular JIA affect?
• Symmetric large and small joints affection: knees, wrists, ankles, MCPs, PIPs, neck
who does rF positive polyarticular JIA occur in?
- 10% of JIA
- Age: late childhood (teens, 12-16 years)
- Girls: boys = 7:1
what is the presentation and symptoms of RF positive polyarticular JIA?
Constitutional manifestations (low grade fever, malaise, weight loss)
Anemia
Nodules
- 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
- Iridocyclitis rare
what is the leats common type of JIA?
Systemic onset JIA
The least common (20%)
but most serious short and long term morbidity and mortality
what defines systemic onset JIA?
Extra-articular features define the disease
(start early and disappear after 2-5 years)
what is another name for systemic onset JIA?
stills disease
who does systemic onset JIA occur in?
- 20% of JIA
- Age: throughout childhood (4-6 years)
- Girls: boys = 1.5:1
what fever may occur in systemic onset JIA?
- 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
what rash may occur in systemic onset JIA?
- 90%
- Evanescent salmon red eruption
- On trunk and thighs
- Accompanies fever
- Can be brought by scratching (+ve Koebner’s phenomenon)
what lymph node problems may occur in systemic onset JIA?
- 50-75%
- Generalized lymphadenopathy
- Non tender
what abdominal problems may occur in systemic onset JIA?
- Hepatosplenomegaly
- 50-75%
- Abdominal pain
- +/- transaminases
what serositis (inflammation of a serous membrane) may occur in systemic onset JIA?
- Polyserositis
- Pericarditis in 36%
- Tamponade and myocarditis rare
what pulmonary problems may occur in systemic onset JIA?
- Rare
- Pleural effusion
- Pulmonary fibrosis
what arthiritis may occur in systemic onset JIA?
- 75%
- Within 3-12 months of onset of fever
- Wrists, knees, ankles, cervical spine, hips and TMJ
What are some things to look out for in children with arthritis?
- Sowllen knee
- Children are bendy – hypermobile so an important clinical sign is a loss of this hypermobility
- Jaw involvement – children growing so the effect on a growing skeleton is different from an adults skeleton, joint can be effect unilaterally or bilaterally
when does uveitis occur in JIA?
what does uveitis in JIA cause?
what is 1st line therapy in JIA?
- Simple pain killers
- NSAIDs:
– Difference between adults and children half life
– Can control disease
– Doses
– Same compounds only
what is 2nd line therapy in JIA?
If no response to NSAIDs/ joint (steroid) injections
- Rarely needed in oligoarticular JIA
(1) 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
how are systemic steroid used in JIA?
- Limited indications due to serious side effects
- 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
whata re the risks of used systemic steroid in JIA?
•Risk of osteoporosis, infections, growth abnormalities
another kind of manage is rehabilitation and that is done by who?
- Physiotherapy
- Occupational therapy
when would local steroids be used?
- Intra-articular mainly in Oligo-articular JIA
- Eye disease (ANA +ve oligo-articular disease)
what surgery may be done? (Try avoid this in young people)
- Synovectomy
- Reconstructive / joint replacement surgery
what growth failure may occur in JIA?
Micrognathia is a condition in which the lower jaw is undersized
Summary:
- JIA is not ____
- Subtypes guide management and prognosis
- Eye screening for all patients _______ (more common in the ______ subtype
- ANA is not _________
rare
important
oligos
diagnostic
