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Recurrent immunoglobulin A nephropathy — Clinical Guidelines

Overview

Immunoglobulin A nephropathy (IgAN) is a primary glomerulonephritis characterized by mesangial deposition of polymeric IgA1, often leading to progressive renal disease. Recurrent episodes can exacerbate renal damage, necessitating careful management to preserve kidney function 1 4.

Diagnosis

Key Diagnostic Criteria: Mesangial IgA deposition on renal biopsy 1.

Recommended Tests: Renal biopsy for histopathological assessment 1.

Grading: Utilize the Oxford Classification for risk stratification 1.

Management

First-Line Treatments:

- Targeted-release budesonide (Nefecon): For reducing pathogenic IgA production 1. - Reduced-dose systemic corticosteroids: Alternative for managing IgA production 1. - Mycophenolate mofetil: Specifically noted for efficacy in Chinese patients 1.

Adjunctive Treatments:

- Strict proteinuria control: Aim for <0.5 g/day, ideally <0.3 g/day 1. - Lifestyle modifications: Healthy lifestyle education to manage nephron loss consequences 1. - Tonsillectomy: Considered effective in cases linked to recurrent tonsillitis 7. - Steroid pulse therapy: Combined with tonsillectomy shows promise 8.

Special Populations

Pediatrics: Specific dosing and management strategies may differ but are not detailed in provided abstracts 1.

Elderly: Considerations for comorbidities and treatment tolerance are not explicitly addressed 1.

Comorbidities: Management strategies should account for coexisting conditions, though specific guidance is limited 1.

Key Recommendations

Adopt a liberal kidney biopsy policy for recurrent IgAN to guide management (Evidence: Moderate 1).

Aim for stringent proteinuria control with a target of <0.5 g/day, ideally <0.3 g/day (Evidence: Strong 1).

Consider initiating treatment with therapies targeting IgA production, such as targeted-release budesonide or reduced-dose corticosteroids (Evidence: Moderate 1).

Evaluate the role of tonsillectomy in patients with recurrent IgAN secondary to tonsillitis (Evidence: Moderate 7 8).

Incorporate lifestyle modifications to manage the consequences of nephron loss (Evidence: Expert opinion 1).

References

1 Floege J, Barratt J, Cook HT, Noronha IL, Reich HN, Suzuki Y et al.. Executive summary of the KDIGO 2025 Clinical Practice Guideline for the Management of Immunoglobulin A Nephropathy (IgAN) and Immunoglobulin A Vasculitis (IgAV). Kidney international 2025. link 2 Sato R, Inoue T, Wakisaka R, Kono M, Yamaki H, Ohara K et al.. (no title). Renal failure 2025. link 3 Harabuchi Y. Tonsil-induced autoimmune/inflammatory syndrome: Current insights into the pathogenic role of tonsils in immunoglobulin A nephropathy, palmoplantar pustulosis and psoriasis. Auris, nasus, larynx 2025. link 4 Yamaguchi H, Goto S, Takahashi N, Tsuchida M, Watanabe H, Yamamoto S et al.. Aberrant mucosal immunoreaction to tonsillar microbiota in immunoglobulin A nephropathy. Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association 2021. link 5 Niel O, Bastard P. Artificial Intelligence in Nephrology: Core Concepts, Clinical Applications, and Perspectives. American journal of kidney diseases : the official journal of the National Kidney Foundation 2019. link 6 . You're the Flight Surgeon. Aerospace medicine and human performance 2017. link 7 Liess BD, Mytyk C, Calhoun KH, Whaley-Connell AT. Tonsillectomy for the treatment of tonsillitis-induced immunoglobulin A nephropathy. American journal of otolaryngology 2010. link 8 Iio K, Nagasawa Y, Iwatani H, Yamamoto R, Horii A, Okuzaki D et al.. Microarray analysis of tonsils in immunoglobulin A nephropathy patients. Biochemical and biophysical research communications 2010. link

Recurrent immunoglobulin A nephropathy