Paeds: Rheum Flashcards
Henoch-Schonlein Purpura
what is it
an IgA vasculitis that presents with a purpuric rash affecting the lower limbs and buttocks in children.
Inflammation occurs in the affected organs due to IgA deposits in the blood vessels.
Henoch-Schonlein Purpura
What are the 4 classic features
- purpura
- joint pain
- abdo pain
- renal involvement
Henoch-Schonlein Purpura
what age is it most common in
children <10y
Henoch-Schonlein Purpura
what is the condition often triggered by
an upper airway infection or gastroenteritis
Henoch-Schonlein Purpura
what is the rash caused by
inflammation and leaking of blood from small blood vessels under the skin, forming purpura
Henoch-Schonlein Purpura
what is purpura
red-purple lumps under the skin containing blood
Henoch-Schonlein Purpura
which joints are mostly affected
knees and ankles
Henoch-Schonlein Purpura
GI: in severe causes, HSP can lead to?
- GI haemorrhage
- intussusception
- bowel infarction
Henoch-Schonlein Purpura
what can HSP nephritis lead to
microscopic or macroscopic haematuria and proteinuria
Henoch-Schonlein Purpura
Ddx for a non-blanching rash
- meningococcal septicaemia
- leukaemia
- ITP
- haemolytic uraemic syndrome
Henoch-Schonlein Purpura
EULAR/PRINTO/PRES criteria for dx
palpable purpura (not petichiae) + at least one of:
- Diffuse abdo pain
- Arthritis or arthralgia
- IgA deposits on histology (biopsy)
- Proteinuria or haematuria
Henoch-Schonlein Purpura
mnx
supportive: analgesia, rest, hydration
Henoch-Schonlein Purpura
monitoring
- Urine dipstick monitoring for renal involvement
- Blood pressure
Henoch-Schonlein Purpura
prognosis
- abdo pain: few days
- kidney: 4-6w
- 1/3 have recurrence within 6m
- small proportion develop end stage renal failure
Juvenile Idiopathic Arthritis
what is it
autoimmune inflammation in the joints
Juvenile Idiopathic Arthritis
dx criteria
arthritis without any other cause
lasting >6 w
U16
Juvenile Idiopathic Arthritis
key presenting features
joint pain, swelling and stiffness.
Juvenile Idiopathic Arthritis
what are 5 key subtypes of JIA
- Systemic JIA
- Polyarticular JIA
- Oligoarticular JIA
- Enthesitis related arthritis
- Juvenile psoriatic arthritis
Juvenile Idiopathic Arthritis
what is Systemic JIA aka
Still’s disease
Juvenile Idiopathic Arthritis
what is Systemic JIA
a systemic illness that can occur throughout childhood in boys and girls.
idiopathic inflammatory condition
Juvenile Idiopathic Arthritis
Systemic JIA: typical features
- Subtle salmon-pink rash
- High swinging fevers
- Enlarged lymph nodes
- Weight loss
- Joint inflammation and pain
- Splenomegaly
- Muscle pain
- Pleuritis and pericarditis
Juvenile Idiopathic Arthritis
Systemic JIA: lab findings
-ve ANA + RF
raised CRP, ESR, platelets + ferritin
Juvenile Idiopathic Arthritis
Systemic JIA: key complication
macrophage activation syndrome (MAS)
Juvenile Idiopathic Arthritis
Systemic JIA: what is macrophage activation syndrome (MAS)
a severe activation of the immune system with a massive inflammatory response.
Juvenile Idiopathic Arthritis
Systemic JIA: how does someone with macrophage activation syndrome present
- acutely unwell child
- DIC
- anaemia
- thrombocytopenia
- bleeding
- non-blanching rash
life threatening
Juvenile Idiopathic Arthritis
Systemic JIA: key inx finding with someone with MAS
low ESR
for children that have fevers >5d, what are the key non-infective differentials
- Kawasaki disease
- Still’s disease
- rheumatic fever
- leukaemia
Juvenile Idiopathic Arthritis
Polyarticular JIA: what is it
involves idiopathic inflammatory arthritis in 5 joints or more.
equivalent of RA in adults
Juvenile Idiopathic Arthritis
Polyarticular JIA: arthritic presentation
- symmetrical
- small joints of the hands and feet, as well as the large joints such as the hips and knees
Juvenile Idiopathic Arthritis
Polyarticular JIA: are there systemic sx
minimal
- mild fever
- anaemia
- reduced growth
Juvenile Idiopathic Arthritis
Polyarticular JIA: lab findings
most are RF -ve (seronegative)
Seropositive patients tend to be older children and adolescents
Juvenile Idiopathic Arthritis
Oligoarticular JIA: aka
pauciarticular JIA
Juvenile Idiopathic Arthritis
Oligoarticular JIA: how many joints does it involve and which ones
4 joints or less
typically only affecting a single joint (monoarthritis)
knee or ankle
Juvenile Idiopathic Arthritis
Oligoarticular JIA: who does it occur most frequently in
girls <6y
Juvenile Idiopathic Arthritis
Oligoarticular JIA: classic associated feature
anterior uveitis
refer to opthalmologist for mnx and follow up of uveitis
Juvenile Idiopathic Arthritis
Oligoarticular JIA: lab findings
- mildly or normal inflamm markers
- +ve ANA
- RF -ve
Juvenile Idiopathic Arthritis
Enthesitis-Related Arthritis: who is it more common in
boys >6y
Juvenile Idiopathic Arthritis
Enthesitis-Related Arthritis: if this was an adult condition, what is it equivalent to
the seronegative spondyloarthropathy group of conditions:
- ankylosing spondylitis
- psoriatic arthritis
- reactive arthritis
- IBD-related arthritis
Juvenile Idiopathic Arthritis
Enthesitis-Related Arthritis: what is enthesis
the point at which the tendon of a muscle inserts into a bone
Juvenile Idiopathic Arthritis
Enthesitis-Related Arthritis: which gene do majority of patients have
HLA B27 gene
Juvenile Idiopathic Arthritis
Enthesitis-Related Arthritis: what other conditions may they have
- psoriasis
- anterior uveitis
Juvenile Idiopathic Arthritis
Enthesitis-Related Arthritis: why is it worth palpating key areas
to elicit tenderness of the entheses
Juvenile Idiopathic Arthritis
Enthesitis-Related Arthritis: where is it common on the body
- Interphalangeal joints in the hand
- Wrist
- Over the greater trochanter on the lateral aspect of the hip
- Quadriceps insertion at the anterior superior iliac spine
- Quadriceps and patella tendon insertion around the patella
- Base of achilles, at the calcaneus
- Metatarsal heads on the base of the foot
Juvenile Idiopathic Arthritis
Juvenile Psoriatic Arthritis: what is it
seronegative inflammatory arthritis associated with psoriasis
Juvenile Idiopathic Arthritis
Juvenile Psoriatic Arthritis: pattern of joint involvement
varies:
- symmetrical polyarthritis affecting the small joints similar to rheumatoid
- asymmetrical arthritis affecting the large joints in the lower limb
Juvenile Idiopathic Arthritis
Juvenile Psoriatic Arthritis: signs on examination
- Plaques of psoriasis
- nail pitting
- Onycholysis
- Dactylitis
- Enthesitis
Juvenile Idiopathic Arthritis
mnx
- NSAIDs
- PO, IM or intra-articular steroids
- DMARDs
- Biologics: tumour necrosis factor inhibitors etanercept, infliximab and adalimumab
Ehlers-Danlos Syndrome
what is it
umbrella term for a group of genetic conditions that cause defects in collagen,
resulting in hypermobility of the patient’s joints and abnormalities in connective tissue
Ehlers-Danlos Syndrome
name 4 types
- Hypermobile
- Classical
- Vascular
- Kyphoscoliotic
Ehlers-Danlos Syndrome
which type is most common in exams and clinical cases
Hypermobile
Ehlers-Danlos Syndrome
key features of hypermobile EDS
- most common
- joint hypermobility
- soft + stretchy skin
- no gene identified
- no mode of inheritance
Ehlers-Danlos Syndrome
key features of Classical EDS
- stretchy skin that feels smooth and velvety
- severe joint hypermobility
- joint pain
- abnormal wound healing
- lumps over pressure points e.g. elbow
Ehlers-Danlos Syndrome
Classical EDS: what are they prone to
- hernias
- prolapses
- mitral regurgitation
- aortic root dilatation
Ehlers-Danlos Syndrome
Classical EDS: inheritance pattern
autosomal dominant
Ehlers-Danlos Syndrome
what is the most dangerous type
Vascular Ehlers-Danlos syndrome
Ehlers-Danlos Syndrome
Vascular EDS: what is it
blood vessels are particularly fragile as a result of defective collagen
skin, internal organs and arteries are fragile and prone to rupturing.
Ehlers-Danlos Syndrome
Vascular EDS: presentation
thin, translucent skin that you can almost see through.
Ehlers-Danlos Syndrome
Vascular EDS: inheritance pattern
autosomal dominant
Ehlers-Danlos Syndrome
Kyphoscoliotic EDS: presentation
- hypotonia as neonate + infant
- followed by kyphoscoliosis as they grow
- significant joint hypermobility
- tall + slim
Ehlers-Danlos Syndrome
Kyphoscoliotic EDS: risk of what
rupture in the medium sized arteries.
Ehlers-Danlos Syndrome
Kyphoscoliotic EDS: inheritance pattern
autosomal dominant
Ehlers-Danlos Syndrome
presentation
- Hypermobility in joints
- Joint pain after exercise or inactivity
- Joint dislocations
- Soft stretchy skin
- Easy bruising
- Poor healing of wounds
- Bleeding
- Headaches
- Autonomic dysfunction: dizziness, syncope
- GOR
- Abdo pain
- IBS
- Menorrhagia and dysmenorrhea
- Premature rupture of membranes in pregnancy
- Urinary incontinence
- Pelvic organ prolapse
- Temporomandibular joint dysfunction
- Myopia and other
Ehlers-Danlos Syndrome
what is used to assess the extent of hypermobility and support the diagnosis of hypermobility syndrome
Beighton Score
Ehlers-Danlos Syndrome
what is the Beighton Score
1 point for each side of body (max 9):
- Palms flat on floor with straight legs (score 1)
- Elbows hyperextend
- Knees hyperextend
- Thumb can bend to touch the forearm
- Little finger hyperextends past 90 degrees
Ehlers-Danlos Syndrome
mnx
- follow up for complications
- physio
- occupational therapy
- good posture
- moderate intensity of activity
- psychology
Ehlers-Danlos Syndrome
what syndrome can occur as a result of autonomic dysfunction
Postural Orthostatic Tachycardia Syndrome (POTS)
Ehlers-Danlos Syndrome
what is POTS
Postural Orthostatic Tachycardia Syndrome
inappropriate tachycardia on sitting or standing up,
resulting presyncope, syncope, headaches, disorientation, nausea and tremor.
Kawasaki Disease
aka
mucocutaneous lymph node syndrome
Kawasaki Disease
what is it
a systemic, medium-sized vessel vasculitis
Kawasaki Disease
who does it typically effect
children >5y
more common in Japanese and Korean children
B>G
Kawasaki Disease
key complication
coronary artery aneurysm
Kawasaki Disease
clinical features
- fever>39 for >5d
- widespread erythematous maculopapular rash
- desquamation on palms and soles
- strawberry tongue
- cervical lymphadenopathy
- bilateral conjunctivitis
- cracked lips
Kawasaki Disease
inx and results
FBC: anaemia, leukocytosis, thrombocytosis
LFTs: hypoalbuminaemia + elevated liver enzymes
raised ESR
raised WBC on urinalysis
echocardiogram: coronary artery pathology
Kawasaki Disease
what are the 3 phases
- acute phase
- subacute phase
- convalescent stage
Kawasaki Disease
what happens in the acute phase
The child is most unwell with the fever, rash and lymphadenopathy. This lasts 1 – 2 weeks.
Kawasaki Disease
what happens in the subacute phase
desquamation and arthralgia occur and there is a risk of coronary artery aneurysms forming. This lasts 2 – 4 weeks.
Kawasaki Disease
what happens in the convalescent stage
The remaining symptoms settle, the blood tests slowly return to normal and the coronary aneurysms may regress. This last 2 – 4 weeks.
Kawasaki Disease
mnx
- High dose aspirin to reduce the risk of thrombosis
- IV immunoglobulins to reduce the risk of coronary artery aneurysms
Kawasaki Disease
why is aspirin usually avoided in children
risk of Reye’s syndrome
Rheumatic Fever
what is it
an autoimmune condition triggered by streptococcus bacteria.
a multi-system disorder that affects the joints, heart, skin and nervous system
Rheumatic Fever
what is it caused by
antibodies created against the streptococcus bacteria that also target tissues in the body. e,g, myocardium
Rheumatic Fever
what is the causative organism
group A beta-haemolytic streptococcal, typically streptococcus pyogenes causing tonsillitis.
Rheumatic Fever
what type hypersensitivity reaction is it
type 2: the immune system begins attacking cells throughout the body.
Rheumatic Fever
how long after the initial infection does the type 2 hypersensitivity reaction occur
2-4w after initial infection
Rheumatic Fever
presentation (6)
- fever
- joint pain
- rash
- SOB
- Chorea
- Nodules
Rheumatic Fever
what is migratory arthritis
different joints become inflamed and improve at different times
giving the appearance that the arthritis is moving from one joint to the next.
Rheumatic Fever
how is the heart involved
carditis w/ pericarditis, myocarditis + endocarditis leads to:
- tachy/bradycardia
- murmurs from mitral valve disease
- pericardial rub
- heart failure
Rheumatic Fever
what are the 2 key skin findings
Subcutaneous nodules
Erythema marginatum rash
Rheumatic Fever
what is erythema marginatum
a rash which involves pink rings of varying sizes affecting the torso and proximal limbs.
Rheumatic Fever
what is the key nervous system sx
chorea: irregular, uncontrolled and rapid movements of the limbs
aka Sydenham chorea and historically as St Vitus’ Dance.
Rheumatic Fever
inx that can help support dx
- throat swab
- ASO antibody titres
- Echo, ECG + CXR can assess the heart involvement
- Jones Criteria
Rheumatic Fever
dx
Jones Criteria: recent strep infection + 2 major criteria
or 1 major + 2 minor
Rheumatic Fever
Jones Criteria: major criteria
JONES
- Joint arthritis
- Organ inflammation: e.g. carditis
- Nodules
- Erythema marginatum rash
- Sydenham chorea
Rheumatic Fever
Jones Criteria: minor criteria
FEAR
- Fever
- ECG changes (prolonged PR interval) without carditis
- Arthralgia without arthritis
- Raised CRP + ESR
Rheumatic Fever
what are ASO antibody titres
Anti-streptococcal antibodies are antibodies against streptococcus.
they indicate a recent streptococcus infection
Rheumatic Fever
describe the ASO antibody titre levelss after an acute strep infection
Rise over 2 – 4 weeks
Peak around 3 – 6 weeks
Gradually Rheumatic Feverfalls over 3 – 12 months
Rheumatic Fever
why are ASO levels repeated after 2w
Confirm a negative test
Assess whether levels are rising or falling
Rheumatic Fever
what mnx helps prevent the development of rheumatic fever.
trx of strep infection
tonsillitis: phenoxymethylpenicillin (penicillin V) for 10 days
Rheumatic Fever
mnx
- NSAIDs
- aspirin + steroids: carditis
- prophylactic penicillin continued into adulthood
- monitoring complications
Rheumatic Fever
complications (3)
- recurrence
- mitral stenosis
- chronic HF
