Henoch Schonlein

Purpura (HSP)
Simone Sher

HSP: IgA Vasculitis

Most common form of systemic vasculitis in
children

90% of cases occur in pediatric population,

typically is self limited

Characterized by tetrad of: palpable purpura,

arthralgia/arthritis, abdominal pain, renal
disease

Epidemiology of HSP
Most commonly occurs between ages 3-15; peak
incidence between ages 4-6

Annual incidence 20 per 100,000 children <17 years old

Male predominance 1.2 to 1.8 : 1
Most commonly occurs in winter, fall, and spring, rare in
summer months due to association with upper
respiratory infections

Most often preceded by an infection, but vaccinations

and insect bites have also been associated with
development of HSP

Classification of HSP
In 1990 American College of Rheumatology
established criteria. Two or more of these
features >90% sensitivity and specificity for HSP
Palpable purpura
Onset before age 20
Acute abdominal pain
Biopsy showing granulocytes in walls of arterioles or
venules

Classification of HSP
In 2005 European pediatric guidelines for HSP
were created. These criteria are considered more
appropriate for pediatric settings to distinguish
between other processes like gastroenteritis or
appendicitis. Mandatory criteria of purpura or
petechiae plus one or more of the following:
Acute onset abdominal pain
Arthritis/arthralgias
Renal involvement (proteinuria, hematuria)
Leukocytoclastic vasculitis or proliferative
glomerulonephritis with predominant IgA deposition

Pathogenesis of HSP
Immune mediated vasculitis due to IgA deposition
Associated with a variety of infectious and chemical
triggers but exact mechanism is unknown

Characteristic finding is leukocytoclastic vasculitis with

IgA immune complexes in affected organs.

Skin biopsies of the purpuric lesions show small

vessels of the papillar dermis, usually post capillary
venules, to have inflammatory infiltrate

Immunofluorescence shows IgA, C3, and fibrin in the

walls of vessels of affected organs

Clinical Manifestations of
HSP
Develop over the course of weeks and can vary in
order of presentation.

Purpuric skin rash and joint pain are most common

presenting symptoms

Skin: rash often begins with erythematous, macular,

or urticarial wheals. The wheals then coalesce and
evolve into the typical ecchymoses, petechiae, and
palpable purpura. Typically located in pressure
dependent areas such as the lower extremities.
Localized subcutaneous edema can also be seen in
periorbital and dependent areas

Arthritis/Arthralgia
Occurs in 84% of patients
Usually transient, migratory, oligoarticular (1-4
joints), and non-deforming. Usually effects lower
extremities and can have periarticular swelling
and tenderness, but joint will not have effusion,
be red or warm.

Does not cause any permanent joint damage or

sequelae

GI
Occur in about half of patients. Can be mild
(nausea, vomiting, abdominal pain, ileus) to
severe (gi hemorrhage, intussusception,
perforation)

Pain is caused by submucosal bowel

hemorrhage and edema. Purpuric lesions can be
seen on endoscopy

Edema and hemorrhage can act as a pathologic

lead point for intussusception. 60% is in small
bowel, in contrast to idiopathic intussusception
which is typically ileocolic

Renal Disease
More common in older children and adults
Most common presentation is hematuria, with or
without red cell casts and proteinuria

Nephrotic range proteinuria, elevated serum

creatinine, and/or hypertension are present in a
minority of patients

Renal biopsy is identical to IgA nephropathy

Other more rarely affected

organs
Scrotum (scrotal pain/swelling)
Nervous system (headaches, seizures,
neuropathy)

Respiratory Tract (impaired lung diffusion

capacity and interstitial changes)

Eyes (keratitis, uveitis)

Diagnosis
Typically a clinical diagnosis. With unusual
presentation can do biopsy of effected organ
which would show leukocytoclastic vasculitis
with a predominance of IgA deposition

Lab findings (CBC, chem panel, UA) typically

non-specific. Patients may have leukocytosis and
elevated ESR.

Hypocomplementemia found in a large

percentage of patients

Treatment
Most patients recover spontaneously in ambulatory setting with
supportive care of rest, hydration, and pain relief with tylenol or
NSAIDs

Patients with severe abdominal pain that interferes with oral

intake that fails treatment with NSAIDs (after complications such
as intussusception are ruled out) can be given prednisone

Hospitalization is indicated in patients who fail to maintain oral

hydration, have significant gastrointestinal bleeding, severe
abdominal pain, changes in mental status, severe joint
involvement limiting ambulation, or evidence of significant renal
disease (elevated creatinine, hypertension, or proteinuria)

Prognosis is excellent, however a small minority of patients (<1

percent) develop long-term complications, primarily renal disease
2/3 of children will not recur. 1/3 will recur at least once, typically
within 4 months. Each subsequent recurrence is typically milder and
shorter in duration