Henoch-Schönlein purpura - IgA vasculitis (< 18 years)
Henoch-Schönlein purpura - IgA vasculitis (< 18 years)

Consider other diagnosis, off pathway

Signs of severe renal disease

  • Hypertension
  • Nephrotic syndrome
  • Renal insufficiency
  • Severe proteinuria (> 1 g/day)

Off pathway, treat appropriately

Inclusion Criteria
  • Clinical concern for Henoch-Schönlein purpura
Exclusion Criteria
  • History of thrombocytopenia or coagulopathy
  • History of cancer or immunosuppression
  • History of rheumatologic or vasculitis disease
  • Complex medical disorder
  • Ill appearance or mental status changes
  • Pregnancy

Consider rheumatology or nephrology consult

Clinical finidings consistant with HSP?

Severe secondary effects of pain?

Signs of severe renal disease?

Tolerating PO?

Clinical findings in HSP

  • Palpable purpura (75% of patients, commonly preceeds other sx (avg. 4 days) however it is not the presenting sign in 25% of patients)
  • Arthritis/arthralgia (50-75% of patients)
  • Abdominal pain (50% of patients with GI bleeding in 20-30%)
  • Renal disease (20-50% of patients)
  • Scrotal pain, tenderness, and swelling may be present in 2-40% of patients

Epidemiology

  • 90% in pediatric age group
  • Male-Female ratio 1.2:1 to 1.8:1
  • Primarily a fall, winter, and spring disease (rare in summer)
  • 50% will have recent URI
  • History of familial Mediterranean fever (~5 percent)

Severe abdominal pain?

Consider additional imaging other than US if clinical signs concerning for bowel infarction, or perforation

Ultrasound Abdomen Limited Intussusception

Prednisone tabs

Methylprednisolone injection

Need for hospitalization?

Consider nephrology consult

Potential reasons for hospitalizations

  • Severe abdominal pain or gastrointestinal bleeding
  • Mental status changes or confusion
  • Severe joint involvement
  • Poor PO intake
  • Signs of renal insufficiency (elevated creatinine), hypertension, or nephrotic syndrome

Admit, treat appropriately for associated complications

  • Discharge home
  • Mid-moderate pain
    • Naproxen 20 mg/kg BID x5 days (max daily dose 1000 mg/day)
  • Severe pain
    • Naproxen as above
    • Prednisone 1 mg/kg Qday (max 60 mg) tapered over 1-2 months
  • If no renal involvement: UA and BP monitoring weekly for the first 1-2 months, then once a month to once every other month for 1 year
  • If limited renal involvement: urine protein excretion and serum creatinine once per week for 1 mo, then every 2wks for 2 mos, then decreased frequency for the next year
  • If severe renal involvement: follow nephrology recommendations

Complete Blood Count, Serum
Urinalysis with micro, Urine
Basic Metabolic Panel, Serum
Prothrombin time (PT)
Partial Thromboplastin Time (PTT)

Severe secondary effects of pain

  • Poor oral intake
  • Impairment of ADLs
  • Hospitalization

Hemorrhagic stroke may occur in the setting of HSP, however it is very rare. Most CNS findings in HSP are transitory.




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