Rheumatology Flashcards
What are the causes of a non-blanching purpuric rash in children?
- henoch-schonlein purpura
- ITP
- meningitis
- following viral infection
- haemolytic uraemic syndrome
What is henoch-schonlein purpura (HSP)?
IgA vasculitis
Inflammation occurs in affected organs due to IgA deposits.
Which organs does henoch-schonlein purpura affect?
Skin
Kidneys
GI tract
What can precipitate HSP?
URTI
Gastroenteritis
What ages is HSP most common?
Children <10 years
What are the 4 classic features of HSP?
- purpuric rash (100%)
- arthralgia/arthritis (75%)
- abdominal pain (50%)
- renal involvement, IgA nephritis (50%)
What causes the purpuric rash?
Inflammation and leaking of blood from small blood vessels under the skin, forming purpura.
Purpura are red-purple lumps under the skin containing blood.
Where is the purpura mostly found in HSP?
Typically start on the legs; spread to the buttocks
Can also affect the trunk and arms.
In severe cases of HSP what skin changes can occur?
Skin ulceration and necrosis
What joints are most commonly affected in HSP?
Knees
Ankles
Become swollen, painful, reduced ROM
In severe cases of HSP, what GI involvment can occur?
- gastrointestinal haemorrhage
- intussusception
- bowel infarction
What is present in IgA nephritis?
- haematuria
- proteinuria
What is recorded on the urine dipstick that would indicate the child has developed nephrotic syndrome in HSP?
- 2+ protein
Will also have a degree of oedema
What serious pathology should be ruled out in investigations for HSP?
- meningococcal septicaemia
- leukaemia
- ITP
- haemolytic uraemic syndrome
What investigations should be done for HSP?
- FBC, blood film (thrombocytopenia, sepsis, leukaemia)
- renal profile (kidney involvement)
- serum albumin (nephrotic syndrome)
- CRP (sepsis)
- blood cultures (sepsis)
- urine dipstick (proteinuria)
- urine protein:creatinine ratio (quantify proteinuria)
- BP (HTN)
What set of criteria is used to diagnose HSP?
EULAR/PRINTO/PRES criteria
Requires patient to have palpable purpura AND at least one of:
- diffuse abdo pain
- arthritis/arthralgia
- IgA deposits on histology
- proteinuria/haematuria
What is the management for HSP?
Supportive
- simple analgesia
- rest
- hydration
Consider steroids in severe GI pain, renal involvement
Monitor:
- renal involvement (proteinuria/haematuria) with urine dipstick
- HTN with BP
What is the prognosis for HSP?
- abdo pain should settle within a few days
- patients without kidney involvement should recover within 4-6 weeks
- third have recurrence within 6 months
- small & develop end stage renal failure
What is Kawasaki disease?
A systemic, medium sized vessel vasculitis
Who does Kawasaki affect?
Young children <5 years
More common in Asian children (Japanese, Korean)
More common in boys
What are the causes of Kawasaki disease?
No clear cause of trigger
How does Kawasaki disease present?
“warm cream”
- persistent fever >39C for >5 days
- Conjunctivitis
- Rash (widespread erythematous, maculopapular)
- Erythema/Oedema to palms/soles (and desquamation, skin peeling)
- Adenopathy, cervical
- Mucositis, mucous membranes dry, red, strawberry tongue (red tongue with large papillae), cracked lips
What investigations should be done in Kawasaki disease?
- FBC (anaemia, leukocytosis, thrombocytosis)
- LFTs (hypoalbuminaemia, elevated liver enzymes)
- ESR, CRP (raised)
- urinalysis (raised WBC without infection)
- ECHO (coronary artery pathology)
What are the 3 phases to Kawasaki disease?
1) Acute phase
2) Subacute phase
3) Convalescent stage
What happens in acute phase of Kawasaki disease?
- most unwell with fever, rash, lymphadenopathy
- lasts 1-2 weeks
What happens in subacute phase of Kawasaki disease?
- acute symptoms setlle
- desquamation, arthralgia occur
- risk of coronary artery aneurysms forming
- lasts 2-4 weeks
What happens in the convalescent stage of Kawasaki disease?
- remaining symptoms settle
- blood tests slowly return to normal
- coronary artery aneurysms may regress
- lasts 2-4 weeks
What is the management for Kawasaki disease?
- High dose aspirin (reduce thrombosis risk)
- IV immunoglobulins (reduce risk of coronary artery aneurysms)
- monitor with echocardiograms for evidence of coronary artery aneurysms
Why is aspirin usually avoided in children?
The risk of Reye’s syndrome (very rare; encephalopathy and liver damage)
