Henoch-Schonlein purpura: Difference between revisions
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[[File:HSPVasc01.jpg| thumb|Palpable Purpura]] | [[File:HSPVasc01.jpg| thumb|Palpable Purpura]] | ||
''Symptoms may develop over the course of days to weeks and vary in order of presentation'' | ''Symptoms may develop over the course of days to weeks and vary in order of presentation'' | ||
#Rash: palpable purpura | #[[Rash]]: palpable purpura | ||
#*Often begins with erythematous, macular, or urticarial wheals, progressing into the typical ecchymoses, petechiae, and palpable purpura (see picture) | #*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) | #*Typically appears in crops, symmetrically distributed, and located primarily in gravity/pressure-dependent areas (e.g. lower extremities, buttocks) | ||
#Acute abdominal pain | #Acute [[Abdominal pain (peds)|abdominal pain]] | ||
#*Typically diffuse and colicky | #*Typically diffuse and colicky | ||
#*May have blood in the stool (massive bleeding is rare) | #*May have blood in the stool (massive bleeding is rare) | ||
#Arthritis/arthralgia | #[[Arthritis]]/arthralgia | ||
#*Migratory, usually involves knees/ankles | #*Migratory, usually involves knees/ankles | ||
#Nephritis/renal disease | #[[Nephritis]]/renal disease | ||
#*(~50% of the time)<ref>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.</ref> | #*(~50% of the time)<ref>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.</ref> | ||
#*Hematuria +/- proteinuria | #*Hematuria +/- proteinuria | ||
===Rare manifestations=== | ===Rare manifestations=== | ||
* Melena, hematemesis, hepatosplenomegaly | * [[Melena]], [[hematemesis]], hepatosplenomegaly | ||
* Headache, seizures | * [[Headache]], [[seizures]] | ||
* Fever | * [[Fever]] | ||
* Non-pitting edema of the extremities and face | * Non-pitting edema of the extremities and face | ||
*Nephrotic | *[[Nephrotic syndrome]] | ||
**Long-term mortality directly related to renal involvement<ref>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 </ref><ref>Saulsbury, FT (1999) Henoch-Schönlein purpura in children. Report of 100 patients and review of the literature. Medicine (Baltimore) 78: pp. 395-409 </ref> | **Long-term mortality directly related to renal involvement<ref>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 </ref><ref>Saulsbury, FT (1999) Henoch-Schönlein purpura in children. Report of 100 patients and review of the literature. Medicine (Baltimore) 78: pp. 395-409 </ref> | ||
Revision as of 20:27, 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
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 w/ rheum f/u
- 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.