Rheumatology

Question 1

What is juvenile idiopathic arthritis?

Answer 1

condition affecting children and adolescents where autoimmune inflammation occurs within the joints

diagnosed when:
no other cause
> 6 wks
< 16

Question 2

Types of juvenile idiopathic arthritis?

Answer 2

systemic JIA
polyarticular JIA
oligoarticular JIA
enthesitis related arthritis
juvenile psoriatic arthritis

Question 3

What is systemic JIA?

Answer 3

aka Still’s Disease
systemic illness that can occur throughout childhood
presents with:
subtle salmon-pink rash
high swinging fevers
lymphadenopathy
weight loss
joint inflammation and pain
splenomegaly
muscle pain
pleuritis and pericarditis

Question 4

Key differentials in children with fever > 5 days?

Answer 4

infections
Still’s disease
Kawasaki disease
rheumatic fever
ALL

Question 5

Investigations for Still’s disease?

Answer 5

ANA and RF negative
raised CRP, ESR, serum ferritin

Question 6

Complication of Still’s disease?

Answer 6

macrophage activation syndrome
life-threatening

Question 7

What is polyarticular JIA?

Answer 7

idiopathic inflammatory arthritis in 5 or more joints
minimal systemic symptoms but mild fever, anaemia or reduced growth

Question 8

What is oligoarticular JIA?

Answer 8

aka pauciarticular JIA
4 joints or less
usually monoarthritic
tends to affect young girls <6yrs
anterior uveitis is classic complication
ANA positive, RF negative

Question 9

What is enthesis-related athritis?

Answer 9

paediatric version of seronegative spondyloarthropathy
inflammatory arthritis in joints and enthesis (where the tendon of a muscle inserts into bone)
HLA B27 gene

look for psoriasis, IBD and anterior uveitis

Question 10

What is juvenile psoriatic arthritis?

Answer 10

seronegative inflammatory arthritis associated with psoriasis

plaques of psoriasis
nail pitting
onycholysis
dactylitis
enthesitis

Question 11

Mx of JIA?

Answer 11

coordinated by paed rheum specialist with MDT involvement

NSAIDs
steroids (oral, IM or intra-articular)
DMARDs
biologics (anti-TNFs)

Question 12

What is Ehlers-Danlos syndrome?

Answer 12

group of AD genetic disorders involving defects in collagen
causing hypermobility of the joints and abnormalities in the CT of the skin, bones, blood vessels and organs

Question 13

Types of Ehlers-Danlos syndrome?

Answer 13

hypermobile (most common, least severe)
classical
vascular
kyphoscoliotic

Question 14

Presentation of Ehlers-Danlos syndrome?

Answer 14

joint pain
joint hypermobility
joint dislocations
soft and stretchy skin
striae
easy bruising
poor wound healing
bleeding
chronic pain
chronic fatigue
headaches
POTS
GOR
abdo pain
IBS
menorrhagia
dysmenorrhoea
PROM
urinary incontinence
pelvic organ prolapse

Question 15

Assessing for hypermobility?

Answer 15

Beighton score
scored out of 9

Question 16

What is the Beighton score used for?

Answer 16

to assess for hypermobility

Question 17

Mx of Ehlers-Danlos syndrome?

Answer 17

MDT approach
physio
OT
moderating activity
psychology
monitor and treat complications

Question 18

What is Henoch-Schoenlein Purpura?

Answer 18

IgA vasculitis that presents with a purpuric rash typically affecting the lower limbs and buttocks in children
causes inflammation in affected organs due to IgA deposits
usually triggered by upper airway infection or gastroenteritis

Question 19

Features of HSP?

Answer 19

purpura (100%)
joint pain (75%)
abdo pain (50%)
renal involvement (50%)

Question 20

Gastrointestinal involvement in HSP?

Answer 20

GI haemorrhage
intussusception
bowel infarction

Question 21

Renal involvement in HSP?

Answer 21

IgA nephritis
microscopic/macroscopic haematuria
proteinuria
+/- oedema

Question 22

Diagnosis of HSP?

Answer 22

exclude other causes of non-blanching rash

FBC, blood film
U&E
serum albumin
CRP
blood cultures
urine dipstick
urine protein: creatinine ratio
BP

Question 23

Mx of HSP?

Answer 23

supportive (rest, analgesia, hydration)
steroids
monitor urine dipstick and BP

Question 24

Prognosis of HSP?

Answer 24

patients w/o renal involvement can expect to fully recover within 4-6wks
1/3 relapse within 6 months
very few develop end-stage kidney disease

Question 25

What is Kawasaki disease?

Answer 25

aka mucocutaneous lymph node syndrome systemic, medium-sized vasculitis affects young children, typically <5 more common in boys and Asians

Question 26

Features of Kawasaki disease?

Answer 26

persistent high fever (>39) for more than 5 days widespread erythematous maculopapular rash desquamation on palms and soles strawberry tongue cervical lymphadenopathy cracked lips bilateral conjunctivitis

Question 27

Investigations for Kawasaki disease?

Answer 27

FBC (anaemia, leucocytosis, thrombocytosis) LFTs (hypalbuminaemia, raised LFTs) inflammatory markers (ESR) urinalysis Echo (coronary artery aneurysm)

Question 28

Disease course in Kawasaki disease?

Answer 28

acute phase (1-2wks) subacute phase (2-4wks, risk of coronary artery aneurysms forming) convalescent stage (2-4wks, symptoms settle, aneurysm regresses)

Question 29

Mx of Kawasaki disease?

Answer 29

high dose aspirin (reduce thrombosis) IVIG (reduce risk of coronary artery aneurysms) follow up with Echo

Question 30

When is aspirin prescribed in children?

Answer 30

Kawasaki disease (usually avoided due to Reye's syndrome)

Question 31

What is rheumatic fever?

Answer 31

autoimmune multi-system condition triggered by Group A streptococcus bacteria caused by antibodies that form against the strep bacteria that also target the tissues in the body type 2 hypersensitivity reaction rare in developed world due to early treatment of strep infections

Question 32

Presentation of rheumatic fever?

Answer 32

occurs 2-4wks after strep A infection, such as tonsillitis fever rash dyspnoea joint -> migratory arthritis cardiac -> pericarditis, myocarditis, endocarditis skin -> subcutaneous nodules, erythema marginatum CNS -> chorea

Question 33

Investigations for rheumatic fever?

Answer 33

throat swab ASO antibody titre Echo, ECG, CXR Jones criteria used for diagnosis

Question 34

What are the Jones criteria used for?

Answer 34

the diagnosis of rheumatic fever

Question 35

What are the Jones criteria?

Answer 35

recent evidence of strep infection + two major criteria OR one major + two minor

Question 36

Major criteria for rheumatic fever?

Answer 36

JONES Joint arthritis Organ involvement Nodules Erythema marginatum Sydenham chorea

Question 37

Minor criteria for Rheumatic fever?

Answer 37

FEAR Fever ECG changes without carditis Arthralgia without arthritis Raised inflammatory markers

Question 38

Mx of rheumatic fever?

Answer 38

prevention by treating strep infections with penicillin V for 10 days refer to specialist NSAIDs for joint pain aspirin and steroids for carditis prophylactic Abx to prevent further strep infections monitoring and management of complications

Question 39

Complications of rheumatic fever?

Answer 39

recurrence valvular heart disease (mitral stenosis most common) chronic heart failure