Paediatric rheumatology Flashcards
What is JIA
- Autoimmune condition causing inflammatory arthritis in a patient <16yrs and lasting more than 6 weeks
What are the 5 subtypes of JIA
- Systemic JIA
- Oligoarticular JIA
- Polyarticular JIA
- Enthesitis related JIA
- Juvenile posoriatic arthritis
Give 6 features of systemic JIA
- High swinging fevers
- Salmon-pink rash
- Lymphadenopathy
- Arthritis
- Uveitis
- Anorexia and weight loss
What is usually seen on bloods in systemic JIA ?
- ANA and RF is usually -ve
- Inflammatory markers will be raised : CRP, ESR, plts and serum ferritin.
What is a key complications of systemic JIA and how does it present?
- Macrophage activation syndrome (MAS)
- Acutely unwell child : DIC, anaemia, thrombocytopenia, bleeding and non blanching rash
- LOW ESR
Give 4 key differentials in a child present with a fever lasting >5 days
- Kawasaki
- Still’s (systemic JIA)
- Leukaemia
- Rheaumatic fever
What is polyarticular JIA and explain the pattern of join inflammation
- Idiopathic inflammatory arthritis in 5 joints or more
- The inflammation tends to be : symmetrical, affect small joints of hands and feet = larger joints
what is olgioarticular JIA and what is the pattern of joint inflammation
- Involves 4 or less joints
- Usually monoarthritis
- Usually larger joints
- More common in females <6yrs
What is a classic associaed feature of oligoarticular JIA
- Anterior uveitis
What antiboides are usually +ve in Oligoarticular JIA
ANA
What is entehsitis-related arthritis, who is it more common in and what gene is it associated with ?
- Inflammatory arthritis in the joints ASWELL as enthesitis
- More common in male children >6yrs
- HLA B27 gener
What is seen on examination in children with juvenile psoriatic arthritis ?
- Psoriasis
- Nail pitting
- Onycholysis, separation of the nail from the nail bed
- Dactylitis,
- Enthesitis
What is the stepwise management of JIA
- NSAIDs, such as ibuprofen
- Steroids, either oral, intramuscular or intra-artricular in oligoarthritis
- Disease modifying anti-rheumatic drugs (DMARDs), such as methotrexate, sulfasalazine and leflunomide
- Biologic therapy, such as the tumour necrosis factor inhibitors etanercept, infliximab and adalimumab
What is Ehler-Danlos Syndrome ?
- Genetic condition causing a defect in collagen
- Leeds to hypermobility in the joints and abnormalities in connective tissue
What are the 4 types of EDS?
- Hypermobile
- Classical
- Vascular
- Kyphoscoliotic
Give the key features of hypermobile EDS
- Most common and least severe
- Joint hypermobility with soft and stretchy skin
- Thought to be autosomal dominant
Give the key features of classical EDS
- Stretchy skin that feels smooth and velvety
- Severe joint hypermobility, joint pain and abnormal wound healing
- Lumps over pressure points
- Autosomal dominant
What are patients with classical EDS prone to ?
- Hernias
- Prolapses
- Mitral regurg
- Aortic root dilatation
Give the key features of vascular EDS
- Most severe and dangerous form - > blood vessels are fragile and prone to rupture
- Thin, translucent skin
- Autosomal dominant
What are the key features of kyphoscoliotic EDS
- Hypotonia as a neonate and infant
- Kyphoscoliosis as they grow
- Significant joint hypermobility making dislocation common
- Autosomal recessive
What syndrome can occur with hypermobile EDS ?
- POTS (postural orthostatic tachycardia syndrome)
- Tachycardia on sitting or standing causing presyncope, syncope, headaches, disorientation, nausea and tremor
How is hypermobility assessed ?
BEIGHTON SCORE
- Palms flat on floor with straight legs
- Hyperextend elbows
- Hyperextend knees
- Bend thumb to touch forearm
- Hyperextend little finger past 90 degrees.
What is Henoch-Schonlein Purpura ?
- IgA small vessel vasculitis due to IgA depositis in blood vessels
What are the 4 classic features of HSP and what is it often triggered by ?
- Common in children <10
- Triggered by upper airway infection of gastroenteritis
- Purpura
- Joint pain
- Abdo pain
- Renal involvement
Explain the Sx seen in HSP
- 100% = purpura, starting on the legs and spreading to the buttocks.
- 75% = arthralgia in knees and ankles.
- 50% = abdo pain
- 50% = renal with IgA nephritis. Proteinuria, haematuria and reduced kidney function
How is HSP managed ?
- Supportive with simple analgesia, rest and hydration
- Monitoring with urine dip and BP for renal involement
What is kawasaki disease and who is it most common in ?
- Medium vessel vasculitis
- Children <5
- Asian children (Japanese, Korean)
- M>F
What are the key features of kawasaki disease
- High-grade fever (>39) which lasts for > 5 days.
- Bilateral conjunctivits
- Bright red, cracked lips
Strawberry tongue - Cervical lymphadenopathy
- Red palms of the hands and the soles of the feet which later peel
How is Kawasaki disease managed ?
- High dose aspirin to reduce the risk of thrombosis
- IV immunoglobulins to reduce the risk of coronary artery aneurysms
What imaging is inolved in the follow up of Kawasaki disease and why ?
- Echo
- Increased risk of coronary artery aneurysms
Why is aspirin usually avoided in children ?
- Risk of Reye’s syndrome
What is reye’s syndrome ?
- Severe, progressive encephalopathy
- Peak incidence 2yrs.
Features
- Encephalopathy: confusion, seizures, cerebral oedema, coma
- Fatty infiltration of the liver, kidneys and pancreas
- Hypoglycaemia
What are two complications of a streptococcus pyogenes infection ?
- Post streptococcal glomerulonephritis
- Rheumatic fever
What is rheumatic fever
- Autoimmune conditions triggered by group A beta-haemolytic streptococaal (strep.pyogenes)
- Abx are produced against the bacteria which alspo attack the cells of the body
What kind of hypersensitivity reaction is rheumatic fever?
- Type 2
- Delayed by 2-4 from initial tonsilitis infection
What is the criteria for diangosing rheumatic fever ?
JONES
- Two major OR
- One major and 2 minot
What are the major features of the Jones criteria ?
JONES
- J : Joint arthritis
- O : Organ inflammation, such as carditis
- N : Nodules
- E : Erythema marginatum rash
- S : Sydenham chorea
What are the minor features of the Jones criteria ?
FEAR
- F : Fever
- E : ECG Changes (prolonged PR interval) without carditis
- A : Arthralgia without arthritis
- R : Raised inflammatory markers (CRP and ESR)
How is rheumatic fever managed ?
- NSAIDs for joint pain
- Aspirin and steroid for carditis
- Abx (Oral or IM penicillin)
How is rheumatic fever preventedf ?
- Treating streptococcal infections with Abc
- Tonsilitis = penicillin V for 10 days
What are 3 complications of rheumatic fever ?
- Recurrence
- Valvular heart disease -> mitral stenosis
- Chronic HF
