41, 42 Henoch schonlein purpura , Kawasaki, MISC Flashcards
Henoch-Schönlein purpura/IgA vasculitis
- Acute immune complex-mediated small vessel vasculitis
- Most commonly occur in children, after the age of 5
- Often preceded by an upper respiratory tract infection
pathophysiology of Henoch-Schönlein purpura/IgA vasculitis
(hypothesis):
exposure to allergen/antigen (eg infection, drugs)
—> stimulation of IgA production
—> deposition of IgA immune complexes in vascular walls
—> activation of complement system
—> vascular inflammation and damage
Henoch-Schönlein purpura/IgA vasculitis typical triad
Typical tetrad:
1) palpable purpura (most commonly on lower extremities, buttocks)
2) arthritis/ arthralgia (most commonly ankles, knees)
3) abdominal pain
4) renal disease (hematuria)
symptoms of Henoch-Schönlein purpura/IgA vasculitis
onset often 1-3 weeks after infection
1) palpable purpura (most commonly on lower extremities, buttocks)
2) arthritis/ arthralgia (most commonly ankles, knees)
3) abdominal pain
4) renal disease (hematuria)
Other:
intussusception,
bloody stool/melena,
nausea/vomiting,
scrotal swelling/pain/tenderness,
headaches, seizures, focal neurological deficits, ataxia,
intracerebral hemorrhage,
mild interstitial pulmonary changes,
pulmonary hemorrhage,
keratitis/uveitis
diagnosis of Henoch-Schönlein purpura/IgA vasculitis
Clinical diagnosis ◦
But can be confirmed with biopsy:
IgA deposits in small vessels of skin or in kidneys
treatment of Henoch-Schönlein purpura/IgA vasculitis
- Most are self-limiting and only require supportive care (NSAIDs for pain, rest, adequate hydration)
- Severe disease —>
*systemic glucocorticoids for severe abdominal pain
*IV fluids for hydration
*for severe renal disease:
methylprednisolone pulse therapy
acute dialysis
antihypertensives
renal transplantation
Kawasaki syndrome
Acute, necrotizing vasculitis of unknown etiology
Primarily affects children, under age of 5, more common in Asian population
diagnosis of kawasaki syndrome
- clinical
- Requires at least 5 days of fever + one of the following:
≥4 other specific symptoms
< 4 specific symptoms but involvement of coronary arteries
The presence of WBC in the urine is not
specific for UTI
Consider appendicitis, group A Streptococcal
infection, Kawasaki disease
specific symptoms of kawasaki syndrome
- Erythema + edema of hands and feet (first week)
- Desquamation of fingertips and toes (2-3 week)
- Polymorphous rash originating from trunk
- Conjunctivitis without exudate
- Oropharyngeal mucositis: strawberry tongue and/or cracked red lips
- Cervical lymphadenopathy
treatment of kawasaki syndrome
- IV immunoglobulins (IVIG):
*high dose to reduce the risk of coronary artery aneurysms
*most effective if given within the first 10 days of onset - High dose oral aspirin: for anti-inflammatory effect, later low dose for anticoagulant effect
Kawasaki disease is a rare exception to the contraindication of giving children aspirin! - IV glucocorticoids: lower risk of coronary involvement
rare exception to the contraindication of giving children aspirin!
- High dose oral aspirin: for anti-inflammatory effect, later low dose for anticoagulant effect
**Kawasaki disease **
complication of kawasaki syndrome
coronary artery aneurysm,
AMI,
myocarditis,
ventricular dysfunction,
arrhythmias
(MISC)
Multisystem inflammatory syndrome in children
MISC is a complication of
Complication of COVID-19 in children that manifests with hyperinflammation + severe illness
symptoms of misc
fever
GI symptoms
mucocutaneous symptoms
