HSP

Henoch-Schonlein purpura (HSP) is the most

common small vessel vasculitis in childhood.
It is characterized by a purpuric rash and
commonly accompanied by arthritis and
abdominal pain.
Approximately 50% of patients with HSP develop
renal manifestations, which vary from
asymptomatic microscopic hematuria to severe,
progressive glomerulonephritis.

Pathogenesis and Pathology

The pathogenesis of HSP nephritis appears to be mediated
by the deposition of polymeric immunoglobulin A (IgA) in
glomeruli.
This is analogous to the same type of IgA deposits seen in
systemic small vessels, primarily those of the skin and
intestine.
The glomerular findings can be indistinguishable from those
of IgA nephropathy.
IgA deposits are present by immunofluorescence, and a
broad spectrum of glomerular lesions that can range from
mild proliferation to necrotic and crescentic changes can be
seen.

Clinical and Laboratory

Manifestations
The nephritis that can accompany HSP usually follows onset of the
rash, often weeks or even months after the initial presentation of
the disease.
Nephritis can be manifest at initial presentation but only rarely
before onset of the rash.
Patients at presentation rarely display a severe combined acute
nephritic and nephrotic picture (hematuria, hypertension, renal
insufficiency, significant proteinuria, and nephrotic syndrome).
Most patients have only mild renal manifestations, principally
isolated microscopic hematuria without significant proteinuria.
Initial mild renal involvement can progress to more severe nephritis
despite resolution of all other features of HSP.

 The severity of the systemic

manifestations does not correlate with the
severity of the nephritis.
Most patients who develop nephritis have
urinary abnormalities by 1 month, and
nearly all have abnormalities by 3 months
after onset of HSP.
Therefore, a urinalysis should be
performed weekly in patients with HSP
during the period of active clinical disease.

Prognosis and Treatment

The prognosis of HSP nephritis for most patients is
excellent.
Spontaneous and complete resolution of the
nephritis typically occurs in those with mild initial
manifestations (isolated hematuria with insignificant
proteinuria).
Patients with acute nephritic or nephrotic syndrome
at presentation have a guarded renal prognosis,
especially if they are found to have concomitant
necrosis or substantial crescentic changes on renal
biopsy.

 Tonsillectomy
Mild HSP nephritis does not require treatment
Several uncontrolled studies have reported benefit
from aggressive immunosuppression (high-dose
and extended courses of corticosteroids with
cyclophosphamide or azathioprine) in patients with
poor prognostic features who might be expected to
have a high risk of chronic renal failure.
Aggressive therapy with careful monitoring may be
reasonable in those with the most severe HSP
nephritis (>50% crescents on biopsy).