Treatment

Most cases of IgAV are self-limiting and only require supportive care (e.g., pain management) with regular outpatient follow-up. Severe IgAV requires hospitalization and intensive medical therapy.

• Outpatient treatment

• Usually no treatment necessary

• NSAIDs for pain management, rest, and adequate hydration

• Discontinuation of suspected precipitating drug, if applicable

• Inpatient treatment

• Systemic glucocorticoids for severe abdominal pain not relieved by NSAIDs

• IV fluids to maintain hydration

• In case of severe renal disease

  • IV methylprednisolone pulse therapy [8]

  • Renal transplantation in end-stage renal disease (ESRD)

  • Acute dialysis in the case of acute kidney injury (AKI)

  • Consider antihypertensive agents if hypertension is present.

  • Reduce salt intake

• Renal

  • In some cases, IgAV nephritis may progress to nephrotic syndrome.

  • Serious complication: rapid-progressive glomerulonephritis (RPGN) with crescent formation

• Gastrointestinal

  • Intussusception

  • Small bowel infarction or perforation

• All patients

  • Blood pressure monitoring and urinalysis

  • Timing

    • Symptomatic patients: weekly or biweekly testing

    • After symptoms subside: monthly testing for the first 6 months, then every other month for an additional 6 months

    • Symptom-free for > 12 months: perform tests at subsequent well-child visits

• In patients with abnormal blood pressure or urinalysis

  • Measure serum creatinine

  • Referral to a pediatric nephrologist if serum creatinine levels are abnorm

• Usually resolves with full recovery, however, relapse is likely in patients with previous renal involvement.

• The prognosis is worse in patients with nephrotic range proteinuria. In rare cases (∼ 1%), ESRD may occur.