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Finals > Paediatric rheumatology > Flashcards

Paediatric rheumatology Flashcards

1
Q

What is JIA

A
  • Autoimmune condition causing inflammatory arthritis in a patient <16yrs and lasting more than 6 weeks
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2
Q

What are the 5 subtypes of JIA

A
  • Systemic JIA
  • Oligoarticular JIA
  • Polyarticular JIA
  • Enthesitis related JIA
  • Juvenile posoriatic arthritis
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3
Q

Give 6 features of systemic JIA

A
  • High swinging fevers
  • Salmon-pink rash
  • Lymphadenopathy
  • Arthritis
  • Uveitis
  • Anorexia and weight loss
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4
Q

What is usually seen on bloods in systemic JIA ?

A
  • ANA and RF is usually -ve
  • Inflammatory markers will be raised : CRP, ESR, plts and serum ferritin.
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5
Q

What is a key complications of systemic JIA and how does it present?

A
  • Macrophage activation syndrome (MAS)
  • Acutely unwell child : DIC, anaemia, thrombocytopenia, bleeding and non blanching rash
  • LOW ESR
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6
Q

Give 4 key differentials in a child present with a fever lasting >5 days

A
  • Kawasaki
  • Still’s (systemic JIA)
  • Leukaemia
  • Rheaumatic fever
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7
Q

What is polyarticular JIA and explain the pattern of join inflammation

A
  • Idiopathic inflammatory arthritis in 5 joints or more
  • The inflammation tends to be : symmetrical, affect small joints of hands and feet = larger joints
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8
Q

what is olgioarticular JIA and what is the pattern of joint inflammation

A
  • Involves 4 or less joints
  • Usually monoarthritis
  • Usually larger joints
  • More common in females <6yrs
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9
Q

What is a classic associaed feature of oligoarticular JIA

A
  • Anterior uveitis
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10
Q

What antiboides are usually +ve in Oligoarticular JIA

A

ANA

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11
Q

What is entehsitis-related arthritis, who is it more common in and what gene is it associated with ?

A
  • Inflammatory arthritis in the joints ASWELL as enthesitis
  • More common in male children >6yrs
  • HLA B27 gener
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12
Q

What is seen on examination in children with juvenile psoriatic arthritis ?

A
  • Psoriasis
  • Nail pitting
  • Onycholysis, separation of the nail from the nail bed
  • Dactylitis,
  • Enthesitis
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13
Q

What is the stepwise management of JIA

A
  • 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
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14
Q

What is Ehler-Danlos Syndrome ?

A
  • Genetic condition causing a defect in collagen
  • Leeds to hypermobility in the joints and abnormalities in connective tissue
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15
Q

What are the 4 types of EDS?

A
  • Hypermobile
  • Classical
  • Vascular
  • Kyphoscoliotic
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16
Q

Give the key features of hypermobile EDS

A
  • Most common and least severe
  • Joint hypermobility with soft and stretchy skin
  • Thought to be autosomal dominant
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17
Q

Give the key features of classical EDS

A
  • Stretchy skin that feels smooth and velvety
  • Severe joint hypermobility, joint pain and abnormal wound healing
  • Lumps over pressure points
  • Autosomal dominant
18
Q

What are patients with classical EDS prone to ?

A
  • Hernias
  • Prolapses
  • Mitral regurg
  • Aortic root dilatation
19
Q

Give the key features of vascular EDS

A
  • Most severe and dangerous form - > blood vessels are fragile and prone to rupture
  • Thin, translucent skin
  • Autosomal dominant
20
Q

What are the key features of kyphoscoliotic EDS

A
  • Hypotonia as a neonate and infant
  • Kyphoscoliosis as they grow
  • Significant joint hypermobility making dislocation common
  • Autosomal recessive
21
Q

What syndrome can occur with hypermobile EDS ?

A
  • POTS (postural orthostatic tachycardia syndrome)
  • Tachycardia on sitting or standing causing presyncope, syncope, headaches, disorientation, nausea and tremor
22
Q

How is hypermobility assessed ?

A

BEIGHTON SCORE

  • Palms flat on floor with straight legs
  • Hyperextend elbows
  • Hyperextend knees
  • Bend thumb to touch forearm
  • Hyperextend little finger past 90 degrees.
23
Q

What is Henoch-Schonlein Purpura ?

A
  • IgA small vessel vasculitis due to IgA depositis in blood vessels
24
Q

What are the 4 classic features of HSP and what is it often triggered by ?

A
  • Common in children <10
  • Triggered by upper airway infection of gastroenteritis
  1. Purpura
  2. Joint pain
  3. Abdo pain
  4. Renal involvement
25
Q

Explain the Sx seen in HSP

A
  • 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
26
Q

How is HSP managed ?

A
  • Supportive with simple analgesia, rest and hydration
  • Monitoring with urine dip and BP for renal involement
27
Q

What is kawasaki disease and who is it most common in ?

A
  • Medium vessel vasculitis
  • Children <5
  • Asian children (Japanese, Korean)
  • M>F
28
Q

What are the key features of kawasaki disease

A
  • 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
29
Q

How is Kawasaki disease managed ?

A
  • High dose aspirin to reduce the risk of thrombosis
  • IV immunoglobulins to reduce the risk of coronary artery aneurysms
30
Q

What imaging is inolved in the follow up of Kawasaki disease and why ?

A
  • Echo
  • Increased risk of coronary artery aneurysms
31
Q

Why is aspirin usually avoided in children ?

A
  • Risk of Reye’s syndrome
32
Q

What is reye’s syndrome ?

A
  • Severe, progressive encephalopathy
  • Peak incidence 2yrs.

Features
- Encephalopathy: confusion, seizures, cerebral oedema, coma
- Fatty infiltration of the liver, kidneys and pancreas
- Hypoglycaemia

33
Q

What are two complications of a streptococcus pyogenes infection ?

A
  • Post streptococcal glomerulonephritis
  • Rheumatic fever
34
Q

What is rheumatic fever

A
  • 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
35
Q

What kind of hypersensitivity reaction is rheumatic fever?

A
  • Type 2
  • Delayed by 2-4 from initial tonsilitis infection
36
Q

What is the criteria for diangosing rheumatic fever ?

A

JONES
- Two major OR
- One major and 2 minot

37
Q

What are the major features of the Jones criteria ?

A

JONES
- J : Joint arthritis
- O : Organ inflammation, such as carditis
- N : Nodules
- E : Erythema marginatum rash
- S : Sydenham chorea

38
Q

What are the minor features of the Jones criteria ?

A

FEAR
- F : Fever
- E : ECG Changes (prolonged PR interval) without carditis
- A : Arthralgia without arthritis
- R : Raised inflammatory markers (CRP and ESR)

39
Q

How is rheumatic fever managed ?

A
  • NSAIDs for joint pain
  • Aspirin and steroid for carditis
  • Abx (Oral or IM penicillin)
40
Q

How is rheumatic fever preventedf ?

A
  • Treating streptococcal infections with Abc
  • Tonsilitis = penicillin V for 10 days
41
Q

What are 3 complications of rheumatic fever ?

A
  • Recurrence
  • Valvular heart disease -> mitral stenosis
  • Chronic HF

Finals (92 decks)

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