Henoch-Schonlein Purpur (IgA Vasculitis) Flashcards
A 5-year-old boy presents with a 4-day history of a purpuric rash on his lower extremities, mild abdominal cramping, and diffuse ankle pain. His mother reports that he recently had symptoms of an upper respiratory tract infection.
Impression
given distribution of the rash, and concomitant arthralgia + abdominal sx likely HSP.
Common vasculitis in paeds, low mortality rate and majority will improve. Rarely leads to RPGN.
Ddx to consider
- other vasculitides: granulomatosis with polyangitis
- rule out meningococcal meningitis, and other causes of sepsis
- ITP
HSP - IgA Vasculitis - History
History
- sx: abdo pain, cramping, N/V/D, polyarthralgia, palpable purpura (on legs and buttocks), haematuria. time course, progression, etc
- details of recent illness, had this before, details of rash (itchy, painful, spreading)
- meningitis: photophobia, systemically unwell, sick contacts, neck rigidity
- fam hx: bleeding disorders, autoimmune disorders
- paeds hx: development, growth, vaccinations, pregnancy/birth
HSP - IgA vasculitis - Examination
Examination
- general appearance + vital signs
- features of HSP: rash, location, palpable? blanch vs non-blanch,
- distribution of arthralgia
- abdo examination: tenderness
- systems review
HSP - IgA vasculitis - Investigations
INvestigations
Is a clinical diagnosis, labs are in consideration of differentials;
in HSP;
- mild leukocytosis, eosinophilia is rare, elevated IgA
- Bedside: UA, urine MCs, ?LP in consideration of meningitis
- Bloods: cultures, FBC, UEC, LFT, CRP/ESR, ANA/ENA, ANCA, serum IgA level
- Imaging: nil
HSP - IgA vasculitis - Management
Management
- consult paeds +/- nephrology for management depending on severity of symptoms
Supportive
Most will resolve over weeks to months with supportive treatment (95% of paeds cases)
- analgesia
- fluids
Definitive
- oral prednisolone (1mg/kg/day) if mild nephritis
- consider steroid-sparing immunomodulator (azathioprine, mycophenolate)
- consider addition of ACEi/ARB
