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Juvenile Idiopathic Arthritis (JIA) - Coggle Diagram
Juvenile Idiopathic Arthritis (JIA)
Intro
commonest chronic inflamm joint disease in children
persistent joint swelling (>6wks)
presents before 16 y/o
absence of infection/any other cause
1 in 1000
Clinical features
Gelling (stiffness after rest e.g. long car rides)
morning stiffness + pain
limp
deterioration in behaviour/mood, avoiding previously enjoyed activities
joint swelling
not always evident initially
fluid in joint
prolif of synovium
swelling of periarticular soft tissues
bone expansion if long term uncontrolled disease
genu valgum (knocked knees)
leg lengthening
digit length discrepancy
advancement of bone age
swan neck deformities in fingers
Subtypes
@ least 7
based on no of joints affected in 1st 6mo (polyarthritis = >4, oligo = up to 4)
systemic
sepsis + malignancy must be considered
presents @ 1-10 y/o
arthralgia, myalgia, high fever, salmon pink macular rash, lymphadenopathy, hepatosplenomegaly, serositis (inflamm of pleura, pericardium or peritoneum)
initially no arthritis
bloods: anaemia, neutrophilia, thrombophilia, high CRP/ESR
prognosis variable/poor
psoriatic arthritis
presents @ 1-16 y/o
dactylitis, nail pitting/dystrophy, chronic ant uveitis in 20%, psoriasis
asymm distribution of large + small joints
enthesitis-related arthritis
presents @ 6-16 y/o
LL large joints 1st, lumbar/sacroiliac joints later
enthesitis = localised inflamm @ tendon/lig insertion esp Achilles
HLA B27+ve
moderate prognosis
can be classified according to presence of RF + HLA subtype
oligoarthritis
persistent
knee, ankle, wrist
excellent prognosis
extended (>4 joints involved after 6mo)
assym distribution of small + large joints
moderate prognosis
chronic ant uveitis in 20%
presents @ 1-6 y/o
polyarthritis
RF -ve
presents @ 1-6 y/o
often fingers, cervical spine, TMJ
moderate prognosis
RF +ve
presents @ 10-16 y/o
marked finger involvement
rheumatoid nodules in 10%
similar to adult RA
poor prognosis
undifferentiated
Comps
chronic ant uveitis
common
asymp
can lead to severe visual impairment
regular ophtho screening with slit lamp
esp oligoarthritis
flexion contractures of joints
may need joint replacement
growth failure
can be due to anorexia, chronic disease or steroids
premature fusion of epiphyses
anaemia of chronic disease
delayed puberty
osteoporosis
caused by diet, reduced weight bearing, steroids, delayed menarche
tx/prevention = ca, vit D, exercise, steroids, bisphosphonates
amyloidosis
v rare now
proteinuria
renal failure
high mortality
Management
aim = induced remission asap
MDT in specialist rheum centre
education + support for child + family
physical therapy to maintain joint function
ophtho
dentistry
ortho
child encouraged to take part in all activities except contact sports during flares
NSAIDs to sx during flares
corticosteroid intra-art injections under US
1st line for oligo
bridging tx for poly before starting MTX
MTX
reduces joint damage
less effective with systemic JIA
regular blood monitoring for LFTs + BM suppression
common SE = nausea
wkly tablet/liquid/subcut injection
systemic corticosteroids
avoided if possible
pulsed IV MTX can be used as an induction agent for severe polyarthritis
Biologics
CK modulators
e.g. anti-TNFa
costly
in severe disease refractory to MTX
BM transplant for refractory disease
Prognosis
1 in 3 will have ongoing disease into adulthood with significant morbidity
joint damage requiring replacement
visual impairment
fractures secondary to osteoporosis
psychosocial morbidity
anticipated that long term outcomes will improve in future thanks to new tx