Juvenile Idiopathic Arthritis Flashcards
Whats makes an arthritis JIA? [2]
Systemic inflammatory disorders
Has to be <16yrs old
Diagnostic criteria for JIA [3 components]
Onset <16yrs Duration >6wks Symptoms: Joint Swelling OR 2 of: - Tenderness - Painful/limited ROM - Warmth
There are 3 major subtypes of JIA which are 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). How does Type 1 present?
Gender, age, sites
2 features
Female dominant
Peaks at 1-3yrs.
Limp due to LL arthritis
20% also get chronic uveitis
How can we test for Type 1 Pauciarticular JIA? [1]
40-75% are ANA +ve
How does Type 2 Pauciarticular JIA present?
Gender and demographic
2 features
NB Watch out for hip or back involvement, while rare it can progress rapidly to AS (worth testing for HLA-B27)
Pre-school boys >8 yo
Limp due to LL arthritis
10-20% get Acute Iridocyclitis
How Does Type 3 Pauciarticular JIA present?
Gender, age
4 features
Female Predominant
Any age during childhood
Asymmetric UL and LL arthritis Dactylitis \+/- Nail pitting Psoriasis 40% have FH/o Psoriasis, develops later in life
What are the subtypes of Polyarticular JIA? [2]
NB in both iridocyclitis is rare
RF -ve (15%)
RF +ve (10%
How does RF -ve Polyarticular JIA present? Gender Main presenting complaint Constitutional manifestations [2] Signs [3]
Female predominant, any age
•Symmetric large and small joint effusions •Constitutional manifestations § Low grade fever § Malaise • Hepato-splenomegaly • Mild anemia • Growth abnormalities
How does RF +ve Polyarticular JIA present? Gender, age Main presenting complaint + Systemic sx [4] Complications [5] X-ray signs
Female mostly, 12-16yrs
5 or more joints + systemic features:
- Anaemia
- Low grade fever
- Malaise
- Rheumatoid Nodules
Can be complicated by:
- Secondary Sjogren’s
- Atlanto-Axial subluxation
- Pulm. Fibrosis
- Felty’s disease
- Cubital tunnel syndrome
• X-ray erosion seen early
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?
1.5 F:M, mostly 4-6yrs old
Still’s disease fever [2]
It rises daily in the afternoon/evening for at least 2 weeks.
Look for a child toxic with fever that goes away in the morning
50-75% of Still’s Patients get Abdominal features such as… [3]
2 symptoms and 1 sign
Hepatosplenomegaly
Abdo Pain
~Raised Transaminases
Lymphadenopathy problems in 50-75% of still’s patients:
2 features
Non-tender and generalized
Describe Still’s rash (90%) [1]
Name the phenomenon associated with this rash
Evanescent Salmon Red Eruption on their trunk/thighs along with the fever.
It can be brought on by scratching (aka Koebner’s Phenomenon)
Pulmonary sx in Still’s patients are rare [2]
Pleural effusion
Pulm. Fibrosis
Inflammation of Serous membranes can come with Still’s Disease, what type is most common?
Give 2 rare complications assoc with inflammation of serous membranes
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? (Name 6 sites)
Wrist, ankle, knee, C-spine, hips, TMJ
Many types of JIA can present with Eye symptoms such as uveitis, these are often asymptomatic but can lead to blindness, how do we respond?
Every JIA patient gets regular Ophthalmology Screenings (PArticularly those with Pauciarticular JIA)
1st Line treatment of JIA? [3]
Treatment for pauciartuclar
Treatment for polyarticular
Simple analgesia
NSAIDs
Intra-articular steroids for single joint involvement
Systemic steroids for polyarticular
What if NSAIDs and local steroids fail? [4] options
Methotrexate
Anti-TNF - infliximab
IL-1R antagonist
IL-6 antagonist tocilizumab
How do we treat Refractory Systemic Arthritis?
IL-1 Receptor Antagonist - Anakinra
IL-6 Antagonist (Tocilizumab)
When would we risk using systemic steroids? [3]
- Serious disease complications [5]
Systemic JIA to control pain and fever
Bridge between DMARDs
Surgery
Serious disease complications in JIA e.g. pericardial effusion, tamponade, vasculitis, severe anaemia or severe eye disease
What are the risks of systemic steroids?
Osteoporosis
Infection
Growth abnormality
Major difference between pauciarticular and polyarticular JIA in presentation
Rare constitutional manifestations in pauci vs
poly
Surgical management of JIA involves [2]
• Surgical mx
○ Synovectomy
Reconstructive/joint replacement surgery
Examinations [2] Investigations No specific tests for JIA But what lab investigations can you consider [4] What imaging techniques can you do [2]
pGALS as screening measure and pREMS ANA HLA-B27 Inflammatory markers RF X-ray, MRI
Management approach
Do’s [2]
Dont’s [3]
Do - intensify treatment, remain active Don't: - Advise rest - Splints, crutches, wheelchairs - Accept less than normal function
