Henoch-Schonlein Purpura

AM Report 01/14/09
Jason McClune
 First description was by
Heberden in 1806

Schonlein first described

the association of
arthralgias and purpura,
and he termed it peliosis
rheumatica in 1837.

Henoch (Schonleins
student) described GI and
renal manifestations in
1874 and 1899
respectively.
 HSP= anaphylactoid purpura
Of all cases, 90% children, 10% adults
Classically: leukocytoclastic vasculitis with
deposition of IgA immune complexes.
Most common systemic vasculitis in children, age
4-7 (20 in 100,000), peak incidence in spring
 HSP in Children
Extensively studied
Develop more serious GI vasculitis/involvement.
Intussusception can occur.
Self limited
20% long standing renal involvement. Of that
20%, 7% need HD
Children with renal involvement have a 35-44%
higher incidence of CKD in 24 year follow up.

(Ronkainen J: The adult kidney 24 years after childhood HSP: retrospective

cohort. Lancet 360: 666-670, 2002)
 HSP in Adults
NO comparable incidence in adults
Overall 26% mortality in adults
Knowledge of natural history in adults is limited
due to small series with short follow up.
More serious skin and joint involvement.
Renal disease is more frequent and more
severe in adults, and more likely to progress.
Recurrence 10-40%, within 4 months.
Recurrence does not worsen prognosis.
 Etiology
Unknown! Possible offenders:
URI: half of pediatric cases
linked to streptococcal
infection
Malignancy: Adult form (RR
6.02 compared to 5.25 with
ANCA) with HSP (lymphoma,
carcinoma),
Viral: hepatitis, parvo B19
Vaccines: hept B vaccine
Meds: quinolones,
clarithromycin

Pankhurst T: Malignancy is increased in

ANCA assoc vasculitis, Rheumatology
43:1532, 2004
 Tissue is the Issue
Skin biopsy needed for
diagnosis:
Skin: IgA
(immunofluorescence)
deposited in the small
vessels (primarily
postcapillary venules)
within the papillary dermis
Kidney: IgA and C3
mesangial deposits
No specific Lab test: cbc,
chem, UA, sed rate,
coags
 Tetrad of symptoms
1. Palpable purpura (97%)
r/o thrombocytopenia and coagulopathy
blister and hemorrhagic lesions (35%)

2. Arthritis/arthralgia (61%)
3. Abdominal pain (30%)
4. Renal disease (21-54%)
 Palpable Purpura
Erythematous macular
wheals that coalesce and
evolve to ecchymosis and
palpable purpura.
Occur in crops
Worsen over pressure
areas
Typical distribution of
lower extremities and
buttock.
 Arthralgias
Joint symptoms common with knees and
ankles most common
Transient, migratory, and oligoarticular
No joint space destruction
No longstanding sequlae.
 GI Involvement
Colicky, n/v/d, constipation
Vasculitis anywhere in the GI tract
Submucosal hemorrhage/edema= GI bleeding.
Usually one week after appearance of rash
Complications: gastrointestinal hemorrhage,
bowel ischemia and necrosis, intussusception
(children only), and bowel perforation
54% are hemoccult positive, but rare to have
frank hemorrhage.
 Renal Involvement
Biopsy: segmental glomerulonephritis with
crescents and mesangial deposition of IgA
and C3 (>50% crescents suggest
progression to ESRD)
Proposed mechanism of injury, IgG
directed to immune deposits in the
mesangium.
 Kids versus Adults
95 adults and 57 children, 5 year follow up
Crescents and nephrotic range proteinuria equal
incidence
Residual CKD 31% in adults versus 24% children.
Adults 2x more likely to progress to HD/ESRD. (16
versus 7%)
Poor prognosis: >50% crescents, renal impairment at
presentation, UP/C> 1.5 g/day, HTN

Coppo R: Long term Prognosis of HSP nephritis in adults and children.

Nephrol Dial Transplant 12: 2277-2283, 1997
 Renal Involvement- Children
Only 1-5% progress to ESRD.
10-50% of children get microscopic
hematuria, mild GN, and proteinuria that
resolves spontaneously.
Up to 33% recurrence in children, but
symptoms are milder and shorter duration
 Renal Involvement-Adults
11% on HD
10-30% ESRD at 15 years
27% CrCl <50%
50% persistent hematuria
47% moderate proteinuria
8% nephrotic range
20% remission

Evangeline, P: HSP in adults, Outcome and Prognosis factors, J Am

Soc Nephrology 13:1271: 2002
 Pericarditis
Not part of the classic
presentation,
sometimes seen in
adults, but rare in
children.
 Treatment
1. NSAIDS:
increased risk of GI bleed
2. Steroids:
Prednisone 1 mg/kg
Helps with arthralgias and abdominal sxs
not proven to benefit skin or renal disease
Does not shorten duration of active disease
Does not prevent recurrence
3. Severe renal disease: limited evidence
cyclophosphamide,
Plasmapharesis
IVIG
cyclosporin
 Small prospective trial conducted in France on
biopsy proven adult HSP
140 patient randomized to:
Steroids
steroids with cyclophosphamide
cyclophosphamide alone
No difference in outcome!

Evangeline, P: HSP in adults, Outcome and Prognosis factors, J Am

Soc Nephrology 13:1271: 2002
 Renal Transplant after HSP

35% recurrence
11% graft loss
 Sources
Ronkainen J: The adult kidney 24 years after childhood HSP:
retrospective cohort. Lancet 360: 666-670, 2002
Pankhurst T: Malignancy is increased in ANCA assoc vasculitis,
Rheumatology 43:1532, 2004
Coppo R: Long term Prognosis of HSP nephritis in adults and
children. Nephrol Dial Transplant 12: 2277-2283, 1997
Evangeline, P: HSP in adults, Outcome and Prognosis factors, J Am
Soc Nephrology 13:1271: 2002
Harrisons, 16th edition
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