Juvenile Idiopathic Arthritis Flashcards
Whats makes an arthritis JIA?
Systemic Inflammatory disorders
Has to be <16yrs old
What is required for us to diagnose JIA?
Onset <16yrs Duration >6wks Symptoms: Joint Swelling OR 2 of: - Tenderness - Painful/limited ROM - Warmth
There are 3 major subtypes of JIA (only determinable after 6 months)?
Pauciarticular (55%) - <5 joints Polyarticular (25%) - >4 joints Systemic Onset (20%) - Still's Disease
There are 3 subtypes of Pauciarticular JIA (Type 1, 2 & 3). Here’s some characteristics of each:
1) 1-3yrs so tend to present with unusual gait rather than complaining of pain
2) Can feature hip/back pain which can rarely progress to ankylosing spondylitis
3) Ass with psoriasis & Dactylitis
What are the subtypes of Polyarticular JIA?
RF -ve (15%)
RF +ve (10%
What sets polyarticular apart from pauciarticular (other than more joints)?
Potential Systemic Symptoms:
- Low grade fever
- Malaise & weight loss
- Hepato-splenomegaly
- Mild Anaemia
- Growth abnormalities
- Nodules
Can be complicated by vasculitis, Sjogren’s or pulm fibrosis
Whats special about how Still’s Disease (Systemic onset JIA) presents?
Systemic features start first with arthritis not occuring till 3-12 months in.
Who gets Still’s Disease?
1.5F:1M, mostly 4-6yrs old
How does Still’s Disease Start?
Fever.
It rises daily in the afternoon/evening for atleast 2 weeks.
Look for a child toxic with fever that goes away in the morning
50-75% of Still’s Patients get Abdominal symptoms such as…
Hepatosplenomegaly
Abdo Pain
Raised Transaminases
How many stills patients get lymph symptoms?
50-75% geet non-tender Generalised Lymphadenopathy
90% of Still’s Patients present with a rash, descirbe it?
Evanescent Salmon Red Eruption on their trunk/thighs along with the fever.
It can be brought on by scratching (aka Koebner’s Phenomenon)
Very rarely Still’s Patients can get pulmonary symptoms like..
Pleural effusion
Pulm. Fibrosis
Inflammation of Serous membranes can come with Still’s Disease, what types are common?
36% of patient’s get Polyserositis including pericarditis
Rarely they can get tamponade and myocarditis
How do the actual arthritis symptoms appear in Still’s Disease?
Start 3-12months after the fever onsets
Wrist, ankle, knee, C-spine, hips & TMJ
What tests can we do to diagnose and determine the subtype of JIA?
1) ANA (commonest in pauciarticular) & RF (subtype of polyarticular)
2) ESR/CRP (Most elevated in systemic onset)
3) FBC (May see anaemia, thrombocytosis & leucocytosis. Esp in polyarticular and Systemic onset)
4) Opthalmology screening for uveitis (mostly in pauciarticular)
1st Line treatment of JIA?
SImple Pain Killers NSAIDs Local Steroids: - IA in Pauciarticular JIA - For Eye disease
What if NSAIDs & Steroids fail?
Methotrexate
What if NSAIDs, steroids and MTX fail?
Anti-TNFalpha - Infliximab
How do we treat Refractory Systemic Arthritis?
IL-1 Receptor Antagonist - Anakinra
IL-6 Antagonist (Tocilizumab)
When would we risk using systemic steroids?
Systemic JIA
Serious compliated JIA e.g. pericardial effusion, tamponade, vasculitis, severe anaemia or severe eye disease
Bridge between DMARDs
Surgery
What are the risks of systemic steroids?
Osteoporosis
Infection
GRowth Abnormality
So summarise the likely presentations of Systemic Onset JIA?
1st = Daily rising and falling fever 2nd: - Generalised Lymphadenoapthy - Evanescent Salmon red rash - ~ serositis - ~ Abdo pain, hepatosplenomegaly & raised transaminases
Then 3-12months later the arthritis starts
