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Paeds > Rheumatology > Flashcards

Rheumatology Flashcards

1
Q

What is juvenile idiopathic arthritis?

A

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

diagnosed when:
no other cause
> 6 wks
< 16

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2
Q

Types of juvenile idiopathic arthritis?

A

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

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3
Q

What is systemic JIA?

A

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

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4
Q

Key differentials in children with fever > 5 days?

A

infections
Still’s disease
Kawasaki disease
rheumatic fever
ALL

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5
Q

Investigations for Still’s disease?

A

ANA and RF negative
raised CRP, ESR, serum ferritin

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6
Q

Complication of Still’s disease?

A

macrophage activation syndrome
life-threatening

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7
Q

What is polyarticular JIA?

A

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

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8
Q

What is oligoarticular JIA?

A

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

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9
Q

What is enthesis-related athritis?

A

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

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10
Q

What is juvenile psoriatic arthritis?

A

seronegative inflammatory arthritis associated with psoriasis

plaques of psoriasis
nail pitting
onycholysis
dactylitis
enthesitis

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11
Q

Mx of JIA?

A

coordinated by paed rheum specialist with MDT involvement

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

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12
Q

What is Ehlers-Danlos syndrome?

A

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

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13
Q

Types of Ehlers-Danlos syndrome?

A

hypermobile (most common, least severe)
classical
vascular
kyphoscoliotic

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14
Q

Presentation of Ehlers-Danlos syndrome?

A

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

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15
Q

Assessing for hypermobility?

A

Beighton score
scored out of 9

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16
Q

What is the Beighton score used for?

A

to assess for hypermobility

17
Q

Mx of Ehlers-Danlos syndrome?

A

MDT approach
physio
OT
moderating activity
psychology
monitor and treat complications

18
Q

What is Henoch-Schoenlein Purpura?

A

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

19
Q

Features of HSP?

A

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

20
Q

Gastrointestinal involvement in HSP?

A

GI haemorrhage
intussusception
bowel infarction

21
Q

Renal involvement in HSP?

A

IgA nephritis
microscopic/macroscopic haematuria
proteinuria
+/- oedema

22
Q

Diagnosis of HSP?

A

exclude other causes of non-blanching rash

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

23
Q

Mx of HSP?

A

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

24
Q

Prognosis of HSP?

A

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

25
Q

What is Kawasaki disease?

A

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

26
Q

Features of Kawasaki disease?

A

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

27
Q

Investigations for Kawasaki disease?

A

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

28
Q

Disease course in Kawasaki disease?

A

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

29
Q

Mx of Kawasaki disease?

A

high dose aspirin (reduce thrombosis)
IVIG (reduce risk of coronary artery aneurysms)

follow up with Echo

30
Q

When is aspirin prescribed in children?

A

Kawasaki disease

(usually avoided due to Reye’s syndrome)

31
Q

What is rheumatic fever?

A

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

32
Q

Presentation of rheumatic fever?

A

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

33
Q

Investigations for rheumatic fever?

A

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

34
Q

What are the Jones criteria used for?

A

the diagnosis of rheumatic fever

35
Q

What are the Jones criteria?

A

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

36
Q

Major criteria for rheumatic fever?

A

JONES

Joint arthritis
Organ involvement
Nodules
Erythema marginatum
Sydenham chorea

37
Q

Minor criteria for Rheumatic fever?

A

FEAR

Fever
ECG changes without carditis
Arthralgia without arthritis
Raised inflammatory markers

38
Q

Mx of rheumatic fever?

A

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

39
Q

Complications of rheumatic fever?

A

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

Paeds (16 decks)

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