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Immunoglobulin A nephropathy

Key sources
The following summarized guidelines for the evaluation and management of immunoglobulin A nephropathy are prepared by our editorial team based on guidelines from the Kidney Disease: Improving Global Outcomes Foundation (KDIGO 2021; 2020) and the Japanese Society of Nephrology (JSN 2016).


1.Classification and risk stratification

Prognosis: consider using clinical and histologic data at the time of biopsy to risk stratify patients with primary IgAN.
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2.Diagnostic investigations

Serum biomarkers: insufficient evidence to support the use of any serum or urine biomarker other thanEGFR and proteinuria for diagnosis or prognosis of primary IgAN.

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  • Evaluation for secondary causes

3.Diagnostic procedures

Kidney biopsy: perform a kidney biopsy for the diagnosis of IgAN.
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4.Medical management

Supportive care: optimize supportive care (including BP management, maximally tolerated dose of ACEIs/ARBs, lifestyle modifications, and addressing cardiovascular risk) as the primary focus of management in patients with IgAN without a variant form of primary IgAN (IgAN with minimal change disease, AKI, or RPGN).

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  • Renin-angiotensin system inhibitors

  • Corticosteroids

  • Immunosuppressive agents

  • Antithrombotic agents

  • Management of IgAN with nephrotic syndrome

  • Management of IgAN with AKI

  • Management of IgAN with RPGN

5.Nonpharmacologic interventions

Smoking cessation: as per KDIGO 2021 guidelines, advise smoking cessation in patients with IgAN.

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  • Weight loss

  • Physical activity

  • Salt restriction

  • Protein restriction

  • Omega-3 fatty acids

6.Surgical interventions

As per KDIGO 2021 guidelines:
Do not perform tonsillectomy for the treatment of IgAN in Caucasian patients. Consider performing tonsillectomy in Japanese patients.
Consider performing tonsillectomy for the treatment of recurrent tonsillitis in patients with IgAN.

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  • Kidney transplantation

7.Specific circumstances

Female patients: provide preconception counseling when appropriate in all female patients of childbearing potential.
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  • Pediatric patients (diagnosis)

  • Pediatric patients (immunosuppressive agents)

  • Pediatric patients (RAS inhibitors)

  • Pediatric patients (follow-up)

  • Patients with IgA vasculitis (diagnosis and evaluation)

  • Patients with IgA vasculitis (prognosis)

  • Patients with IgA vasculitis (management)

  • Patients with IgA vasculitis (IgA vasculitis-associated RPGN)

  • Patients with IgA vasculitis (IgA vasculitis-associated nephritis in pediatric patients)

  • Pediatric patients (lifestyle modifications)

  • Pediatric patients (corticosteroids)

  • Pediatric patients (IgAN specific variants)