Paeds rheum Flashcards
List 4 non infective causes of a fever lasting > 5 days
- Kawasaki disease
- Still’s disease
- Rheumatic fever
- Leukaemia
What is another name for systemic JIA?
Still’s disease
Child B presents with a salmon pink rash, fevers and joint pain. What’s the likely diagnosis?
Still’s disease
What is a complication of Still’s disease? What is the key investigation finding?
Macrophage activation syndrome. Low ESR
What 3 key things are required for diagnosing JIA?
- Arthritis without a cause
- Lasting more than 6 wks
- In someone < 16 years old
How many joints need to be affected to diagnose polyarticular JIA?
5 or more joints
Oligoarticular JIA:
1. How many joints are affected?
2. Typical gender and age?
3. What features is classical associated?
- 4 or less
- Girls under 6
- Anterior uveitis
Enthesitis related arthritis:
- Typical gender and age?
- What is this the ‘paediatric version of’?
- What gene is it commonly associated with?
- Boys over 6
- Seronegative spondyloarthropathy
- HLA B27
Give 4 types of medication used to manage JIA + examples
- NSAIDs (ibuprofen)
- Steriods (prednisolone/hydrocortisone)
- DMARDs (methotrexate/sulfasalazine/leflunomide)
- Biologics (infliximab/adalimumab)
What gender and age does Kawasaki typically affect?
Boys under 5
What type of vasculitis is Kawasaki?
Systemic, medium vessel vasculitis
What is the management of Kawasaki
High dose aspirin and IVIG
What is the role of IVIG in Kawasaki?
To prevent coronary artery aneurysms
Why is aspirin normally avoided in children?
Risk of Reye syndrome - swelling in the liver and brain
What are typical features of Kawasaki disease?
- Fever > 5 days
- Widespread erythematous maculopapular rash
- Desquamation on palms and soles
- Strawberry tounge
- Conjunctivitis
- Lymphadenopathy
What is Henoch-Schonlein Purpura?
An IgA vasculitis
What parts of the body are affected in HSP?
The skin, kidneys and GI tract
What are classic features of HSP?
- Purpura
- Joint pain
- Abdominal involvement
- IgA nephritis
What is the management of HSP?
- Supportive
- Monitor BP for hypertension
- Urine dipstick to monitor renal involvement
How many patients have a reoccurrence of HSP within 6 months?
1/3rd
What is the most common type of Ehlers-Danlos syndrome?
Hypermobile Ehlers-Danlos
What type of Ehlers-Danlos is associated with abnormal wound healing?
Classical Ehlers-Danlos
What score can be used to assess hyper mobility? What do you get points for?
Beighton Score (one point score for each side of body, max nine):
- Palms flat on floor with straight legs
- Elbows hyperextended
- Knees hyperextended
- Them can bend to forearm
- Little finger hyperextends past 90 degrees
What is rheumatic fever?
An autoimmune condition in response to strep infection in which antibodies against the bacteria also attack the joints, heart and skin
What criteria is used to diagnose rheumatic fever?
Jones criteria
According to the Jones Criteria, which is required to diagnoses rheumatic fever
- Evidence of recent strep infection and…
- Two major criteria or…
- One major and two minor criteria
What are major features according to Jones Criteria?
JONES
- Joint arthritis
- Organ inflammation
- Nodules
- Erythema marginatum rash
- Sydenham chorea
What are minor features according to Jones Criteria?
FEAR
- Fever
- ECG changes
- Arthralgia without arthritis
- Raised inflammatory markers
Which valvular heart disease is most likely to be caused by rheumatic fever?
MS
What is the follow up for HSP?
BP and early morning dipstick at..
- 1 and 2 weeks
- 1, 3 and 6 months
What would bloods show in JIA?
- Raised inflammatory markers (ESR/CRP)
- Negative ANA and rheumatoid factor
What part of the skin is spared in HSP?
Trunk is normally spared (from rash)