henoch scholein purpura
DESCRIPTION
pediatric cases for HSPTRANSCRIPT
Henoch Schonlein Purpura (HSP)
Simone Sher
HSP: IgA VasculitisMost 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 HSPMost commonly occurs between ages 3-15; peak
incidence between ages 4-6Annual incidence 20 per 100,000 children <17 years oldMale predominance 1.2 to 1.8 : 1Most 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
In 1990 American College of Rheumatology established criteria. Two or more of these features >90% sensitivity and specificity for HSP
Palpable purpuraOnset before age 20Acute abdominal painBiopsy 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 painArthritis/arthralgiasRenal involvement (proteinuria, hematuria)Leukocytoclastic vasculitis or proliferative
glomerulonephritis with predominant IgA deposition
Classification of HSP
Pathogenesis of HSP Immune mediated vasculitis due to IgA depositionAssociated with a variety of infectious and chemical
triggers but exact mechanism is unknownCharacteristic 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/ArthralgiaOccurs in 84% of patientsUsually 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
GIOccur 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 DiseaseMore common in older children and adultsMost common presentation is hematuria, with or
without red cell casts and proteinuriaNephrotic 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)
DiagnosisTypically 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