Henoch-Schonlein purpura: Difference between revisions
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*UA ([[hematuria]], proteinuria) | *UA ([[hematuria]], proteinuria) | ||
*Stool guaiac (positive in >50%)<ref>Chang WL, Yang YH, Lin YT, Chiang BL. "Gastrointestinal manifestations in Henoch-Schönlein purpura: a review of 261 patients." Acta Paediatr. 2004;93(11):1427.</ref> | *Stool guaiac (positive in >50%)<ref>Chang WL, Yang YH, Lin YT, Chiang BL. "Gastrointestinal manifestations in Henoch-Schönlein purpura: a review of 261 patients." Acta Paediatr. 2004;93(11):1427.</ref> | ||
*Consider abdominal ultrasound if concerns for [[intussusception]] | *Consider abdominal ultrasound if concerns for [[intussusception]] (at higher risk) | ||
===Evaluation=== | ===Evaluation=== | ||
Revision as of 20:29, 27 October 2015
Background
- Most common vasculitis in childhood
- Small vessel
- Most cases preceded by a URI
- Usually affects 2-11 yr
- 5% of cases associated with intussusception (abdominal vasculitis)
- Renal involvement is feared complication
- 95% recover completely after 3-4wk
Clinical Presentation
Classic
Symptoms may develop over the course of days to weeks and vary in order of presentation
- Rash: palpable purpura
- Often begins with erythematous, macular, or urticarial wheals, progressing into the typical ecchymoses, petechiae, and palpable purpura (see picture)
- Typically appears in crops, symmetrically distributed, and located primarily in gravity/pressure-dependent areas (e.g. lower extremities, buttocks)
- Acute abdominal pain
- Typically diffuse and colicky
- May have blood in the stool (massive bleeding is rare)
- Arthritis/arthralgia
- Migratory, usually involves knees/ankles
- Nephritis/renal disease
- (~50% of the time)[1]
- Hematuria +/- proteinuria
Rare manifestations
- Melena, hematemesis, hepatosplenomegaly
- Headache, seizures
- Fever
- Non-pitting edema of the extremities and face
- Nephrotic syndrome
Differential Diagnosis
Pediatric Abdominal Pain
0–3 Months Old
- Emergent
- Nonemergent
3 mo–3 y old
- Emergent
- Nonemergent
3 y old–adolescence
- Emergent
- Nonemergent
Petechiae/Purpura (by cause)
- Abnormal platelet count and/or coagulation
- Septicemia
- Idiopathic thrombocytopenic purpura (ITP)
- Hemolytic uremic syndrome
- Leukemia
- Coagulopathies (e.g. hemophilia)
- Henoch-Schonlein Purpura (HSP)
- Acute hemorrhagic edema of infancy (AHEI)
- Hypersensitivity vasculitis
- Primary vasculitides
- Wegener's
- Microscopic polyangiitis
- Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)
- Secondary vasculitides
- Trauma
Causes of Glomerulonephritis
- Poststreptococcal glomerulonephritis
- Hemolytic-uremic syndrome
- Henoch-Schonlein purpura
- IgA nephropathy
- Lupus nephritis
- Alport syndrome
- Goodpasture syndrome
- Paraneoplastic
Diagnosis
Work-Up
- CBC (normal)
- Chemistry
- PT/PTT (normal)
- UA (hematuria, proteinuria)
- Stool guaiac (positive in >50%)[4]
- Consider abdominal ultrasound if concerns for intussusception (at higher risk)
Evaluation
- Usually a clinical diagnosis
- Rule out other possible causes (see differential diagnosis)
- In patients with an incomplete or unusual presentation, no lab test is diagnostic but biopsy of the affected organ (e.g. skin or kidney) with predominantly immunoglobulin A (IgA) deposition supports diagnosis
Treatment
- Supportive
- NSAIDs for pain, may worsen renal disease or GI disease
- Consider prednisone 1mg/kg/day for severe arthralgias, abdominal or scrotal disease
Disposition
- Outpt management for most with rheumatology follow up
- Recurrence rate of up to 33%
See Also
References
- ↑ Chen JY et al. Henoch-Schönlein purpura nephritis in children: incidence, pathogenesis and management. World J Pediatr. 2015 Feb;11(1):29-34. doi: 10.1007/s12519-014-0534-5. Epub 2014 Dec 29.
- ↑ Calviño, MC, Llorca, J, García-Porrúa, C, Fernández-Iglesias, JL, Rodriguez-Ledo, P, González-Gay, MA (2001) Henoch-Schönlein purpura in children from northwestern Spain: a 20-year epidemiologic and clinical study. Medicine (Baltimore) 80: pp. 279-290
- ↑ Saulsbury, FT (1999) Henoch-Schönlein purpura in children. Report of 100 patients and review of the literature. Medicine (Baltimore) 78: pp. 395-409
- ↑ Chang WL, Yang YH, Lin YT, Chiang BL. "Gastrointestinal manifestations in Henoch-Schönlein purpura: a review of 261 patients." Acta Paediatr. 2004;93(11):1427.