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Henoch-Schönlein purpura

Guidelines

Key sources

The following summarized guidelines for the evaluation and management of Henoch-Schönlein purpura are prepared by our editorial team based on guidelines from the Single Hub and Access Point for Pediatric Rheumatology in Europe (SHARE initiative 2019) and the Kidney Disease: Improving Global Outcomes Foundation (KDIGO 2012). ...
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Classification and risk stratification

Classification: as per SHARE initiative 2019 guidelines, use the EULAR/PRINTO/PReS-endorsed Ankara 2008 criteria for the classification of IgA vasculitis (formerly known as HSP).
B
EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura
Purpura or petechiae with lower limb predominance (not related to thrombocytopenia)
Acute onset diffuse abdominal colicky pain assessed by history and physical examination (may include intussusception and gastrointestinal bleeding)
Leucocytoclastic vasculitis or proliferative glomerulonephritis with predominant IgA deposit on biopsy
Acute onset arthritis, defined as joint swelling or joint pain with limitation on motion
Acute onset arthralgia, defined as joint pain without joint swelling or limitation on motion
Proteinuria > 0.3 g/24 hours or > 30 mmol/mg of urine albumin/creatinine ratio on a spot morning sample
Hematuria or RBC cast (> 5 RBCs/hpf or RBCs casts in the urinary sediment or ≥ 2+ on dipstick)
Criteria not met
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Diagnostic investigations

Evaluation for renal involvement: as per SHARE initiative 2019 guidelines, obtain EGFR measurement and urinalysis (for hematuria and urine protein/urine creatinine ratio or urine albumin/urine creatinine ratio) for the evaluation of renal involvement in patients with IgA vasculitis.
B

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  • Abdominal ultrasound

Diagnostic procedures

Skin biopsy: as per SHARE initiative 2019 guidelines, perform skin biopsy with specific immunofluorescence stating for IgA in patients with atypical rash and/or to exclude alternative diagnosis.
B
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  • Kidney biopsy

Medical management

Corticosteroids: as per SHARE initiative 2019 guidelines, initiate corticosteroids in patients with IgA vasculitis for the treatment of:
orchitis
cerebral vasculitis
pulmonary hemorrhage
other severe organ- or life-threatening vasculitis manifestations.
B
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  • Analgesics

Specific circumstances

Patients with IgA nephritis, ACEIs and ARBs: as per SHARE initiative 2019 guidelines, consider administering ACEIs to prevent/limit secondary glomerular injury in patients with IgA nephritis with persistent proteinuria.
C

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  • Patients with IgA nephritis (corticosteroids)

  • Patients with IgA nephritis (immunosuppressants)

  • Patients with IgA nephritis (combination therapy)

  • Patients with crescentic nephritis

Preventative measures

Prevention of IgA nephritis: as per SHARE initiative 2019 guidelines, do not administer prophylactic corticosteroids to prevent the development of IgA vasculitis-associated nephritis.
D

Follow-up and surveillance

Indications for specialist consultation: as per SHARE initiative 2019 guidelines, consult with a pediatric nephrologist:
if the patient with IgA vasculitis has moderate proteinuria and/or impaired GFR
when starting treatment for IgA nephritis.
B