Rheumatology Flashcards
What is juvenile idiopathic arthritis?
condition affecting children and adolescents where autoimmune inflammation occurs within the joints
diagnosed when:
no other cause
> 6 wks
< 16
Types of juvenile idiopathic arthritis?
systemic JIA
polyarticular JIA
oligoarticular JIA
enthesitis related arthritis
juvenile psoriatic arthritis
What is systemic JIA?
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
Key differentials in children with fever > 5 days?
infections
Still’s disease
Kawasaki disease
rheumatic fever
ALL
Investigations for Still’s disease?
ANA and RF negative
raised CRP, ESR, serum ferritin
Complication of Still’s disease?
macrophage activation syndrome
life-threatening
What is polyarticular JIA?
idiopathic inflammatory arthritis in 5 or more joints
minimal systemic symptoms but mild fever, anaemia or reduced growth
What is oligoarticular JIA?
aka pauciarticular JIA
4 joints or less
usually monoarthritic
tends to affect young girls <6yrs
anterior uveitis is classic complication
ANA positive, RF negative
What is enthesis-related athritis?
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
What is juvenile psoriatic arthritis?
seronegative inflammatory arthritis associated with psoriasis
plaques of psoriasis
nail pitting
onycholysis
dactylitis
enthesitis
Mx of JIA?
coordinated by paed rheum specialist with MDT involvement
NSAIDs
steroids (oral, IM or intra-articular)
DMARDs
biologics (anti-TNFs)
What is Ehlers-Danlos syndrome?
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
Types of Ehlers-Danlos syndrome?
hypermobile (most common, least severe)
classical
vascular
kyphoscoliotic
Presentation of Ehlers-Danlos syndrome?
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
Assessing for hypermobility?
Beighton score
scored out of 9
What is the Beighton score used for?
to assess for hypermobility
Mx of Ehlers-Danlos syndrome?
MDT approach
physio
OT
moderating activity
psychology
monitor and treat complications
What is Henoch-Schoenlein Purpura?
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
Features of HSP?
purpura (100%)
joint pain (75%)
abdo pain (50%)
renal involvement (50%)
Gastrointestinal involvement in HSP?
GI haemorrhage
intussusception
bowel infarction
Renal involvement in HSP?
IgA nephritis
microscopic/macroscopic haematuria
proteinuria
+/- oedema
Diagnosis of HSP?
exclude other causes of non-blanching rash
FBC, blood film
U&E
serum albumin
CRP
blood cultures
urine dipstick
urine protein: creatinine ratio
BP
Mx of HSP?
supportive (rest, analgesia, hydration)
steroids
monitor urine dipstick and BP
Prognosis of HSP?
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
What is Kawasaki disease?
aka mucocutaneous lymph node syndrome
systemic, medium-sized vasculitis
affects young children, typically <5
more common in boys and Asians
Features of Kawasaki disease?
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
Investigations for Kawasaki disease?
FBC (anaemia, leucocytosis, thrombocytosis)
LFTs (hypalbuminaemia, raised LFTs)
inflammatory markers (ESR)
urinalysis
Echo (coronary artery aneurysm)
Disease course in Kawasaki disease?
acute phase (1-2wks)
subacute phase (2-4wks, risk of coronary artery aneurysms forming)
convalescent stage (2-4wks, symptoms settle, aneurysm regresses)
Mx of Kawasaki disease?
high dose aspirin (reduce thrombosis)
IVIG (reduce risk of coronary artery aneurysms)
follow up with Echo
When is aspirin prescribed in children?
Kawasaki disease
(usually avoided due to Reye’s syndrome)
What is rheumatic fever?
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
Presentation of rheumatic fever?
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
Investigations for rheumatic fever?
throat swab
ASO antibody titre
Echo, ECG, CXR
Jones criteria used for diagnosis
What are the Jones criteria used for?
the diagnosis of rheumatic fever
What are the Jones criteria?
recent evidence of strep infection + two major criteria OR one major + two minor
Major criteria for rheumatic fever?
JONES
Joint arthritis
Organ involvement
Nodules
Erythema marginatum
Sydenham chorea
Minor criteria for Rheumatic fever?
FEAR
Fever
ECG changes without carditis
Arthralgia without arthritis
Raised inflammatory markers
Mx of rheumatic fever?
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
Complications of rheumatic fever?
recurrence
valvular heart disease (mitral stenosis most common)
chronic heart failure
