HSP
Summaried Nelsons.
most common vasculitis in children (3-10yo)
leukocytoclastic vasculitis and IgA deposition in the small vessels of the skin, joints, GIT, and kidney
Often post URTI (inc GAS) hence less in summer
FHx can be positive for HSP, genetic component
HLA-B34 and HLA-DRB1*01 alleles have been linked to nephritis
Ex
Palpable purpura (haemorrhage into skin),
at pressure or dependent areas
occasionally progressing to bullae then ulceration
Evolve in groups lasting 3-10days
recur for up to 4 months
Subcutaneous Oedema
oligoarthralgia/itis 75%: resolving over 2w but recurring
GIT 80%: abdominal pain, vomiting, diarrhoea, paralytic ileus, melena, intussusception, and mesenteric ischemia or perforation
Renal 50%: hematuria, proteinuria, hypertension,
Neuro: ICH, seizures, headaches, behaviour changes
Other: orchitis, carditis, inflammatory eye disease, testicular torsion, and pulmonary hemorrhage
Ix
Must BP, U/a, Cr
US if thinking intussusception, will also show bowel wall oedema
(may find ↑WCC/Plt/ESR/CRP ↓Hb, occult blood in stool)
AXR if ?perforation
nephrotic syndrome
acute or chronic renal failure
- petechia <3mm capillaries
- ecchymosis (aka bruise if traumatic) >3mm arterioles/venuoles
Dx
Palpable purpura + 1 of arthralgia/gut angina/biopsy consistant
skin biopsy shows vasculitis of capillaries and venuoles, with neutrophil and infiltration
Renal biopsy shows glomerulonephritis
Both under immunofluorescence show IgA1 deposition
DDx
<2yo with isolated skin ?Acute Haemorragic Oedema
also leukoclastic vasculitis
fever
Tender oedema
Ecchymosis
Rx
Hydration
Analgesia
?1mg/kg daily for 1-2 weeks then taper, treats pain, doesn't alter prognosis
? IVIg ?plasma exchange
Chronic ?azathioprine, cyclophosphamide, or mycophenolate mofetil
Prognosis
30% recurrence within 4-6 months (usually subsequent are milder)
1-2% of total > Chronic Renal Disease
8% of HSP nephritis > end stage renal failure
May not develop for 6months
Therefore monitor BP and UA for 6 months
most common vasculitis in children (3-10yo)
leukocytoclastic vasculitis and IgA deposition in the small vessels of the skin, joints, GIT, and kidney
Often post URTI (inc GAS) hence less in summer
FHx can be positive for HSP, genetic component
HLA-B34 and HLA-DRB1*01 alleles have been linked to nephritis
Ex
Palpable purpura (haemorrhage into skin),
at pressure or dependent areas
occasionally progressing to bullae then ulceration
Evolve in groups lasting 3-10days
recur for up to 4 months
Subcutaneous Oedema
oligoarthralgia/itis 75%: resolving over 2w but recurring
GIT 80%: abdominal pain, vomiting, diarrhoea, paralytic ileus, melena, intussusception, and mesenteric ischemia or perforation
Renal 50%: hematuria, proteinuria, hypertension,
Neuro: ICH, seizures, headaches, behaviour changes
Other: orchitis, carditis, inflammatory eye disease, testicular torsion, and pulmonary hemorrhage
Ix
Must BP, U/a, Cr
US if thinking intussusception, will also show bowel wall oedema
(may find ↑WCC/Plt/ESR/CRP ↓Hb, occult blood in stool)
AXR if ?perforation
nephrotic syndrome
acute or chronic renal failure
- petechia <3mm capillaries
- ecchymosis (aka bruise if traumatic) >3mm arterioles/venuoles
Dx
Palpable purpura + 1 of arthralgia/gut angina/biopsy consistant
skin biopsy shows vasculitis of capillaries and venuoles, with neutrophil and infiltration
Renal biopsy shows glomerulonephritis
Both under immunofluorescence show IgA1 deposition
DDx
<2yo with isolated skin ?Acute Haemorragic Oedema
also leukoclastic vasculitis
fever
Tender oedema
Ecchymosis
Rx
Hydration
Analgesia
?1mg/kg daily for 1-2 weeks then taper, treats pain, doesn't alter prognosis
? IVIg ?plasma exchange
Chronic ?azathioprine, cyclophosphamide, or mycophenolate mofetil
Prognosis
30% recurrence within 4-6 months (usually subsequent are milder)
1-2% of total > Chronic Renal Disease
8% of HSP nephritis > end stage renal failure
May not develop for 6months
Therefore monitor BP and UA for 6 months