Executive summary of the KDIGO 2021 guideline for the management of glomerular diseases

Key points

• The KDIGO 2021 guideline updates the 2012 glomerulonephritis guideline in 11 chapters; where high-quality evidence is lacking, practice points are given, and they greatly outnumber the graded recommendations.
• An ACE inhibitor or angiotensin receptor blocker at the maximally tolerated or allowed dose is first-line for hypertension and proteinuria; target systolic blood pressure in most adults is below 120 mm Hg with standardized office measurement, and dietary sodium should be below 2.0 g/d.
• IgA nephropathy: RAS blockade if proteinuria is above 0.5 g/d (1B); if proteinuria stays above 0.75-1 g/d after at least 90 days of optimized supportive care, a 6-month course of glucocorticoids may be considered (2B), or preferably a clinical trial.
• Membranous nephropathy: biopsy is no longer required in nephrotic syndrome with a positive anti-PLA2R antibody test; with at least one risk factor for progression, rituximab, cyclophosphamide with alternate-month glucocorticoids for 6 months, or tacrolimus-based therapy for at least 6 months is recommended according to risk (1B).
• Children with nephrotic syndrome should receive high-dose glucocorticoids for 8-12 weeks in total instead of 24 weeks (1B).

Overview

This executive summary presents the highlights of the KDIGO 2021 Guideline for the Management of Glomerular Diseases, which replaces the 2012 guideline. It summarises what is the same, what is new and what still needs more evidence for IgA nephropathy and IgA vasculitis, membranous nephropathy, nephrotic syndrome in children, minimal change disease, FSGS, infection-related GN, diseases with a membranoproliferative pattern, ANCA-associated vasculitis, lupus nephritis and anti-GBM disease. This overview is based on the executive summary, which itself is not comprehensive.

General principles

Total proteinuria should be assessed with a 24-hour urine collection in adults and a first morning protein-creatinine ratio in children; eGFR is estimated with the CKD-EPI creatinine equation in adults and the modified Schwartz equation in children. Loop diuretics and sodium restriction are preferred for oedema. Metabolic acidosis should be treated if serum bicarbonate is below 22 mmol/l. Full anticoagulation is indicated for thromboembolic events in nephrotic syndrome. Pneumococcal and influenza vaccination are advised, and trimethoprim-sulfamethoxazole prophylaxis should be considered with high-dose prednisone or other immunosuppressive agents.

Nephrotic syndrome, minimal change disease and FSGS

Children who relapse frequently or are glucocorticoid-dependent should receive 0.5 mg/kg/d prednisone for a week during upper respiratory infections (1C); calcineurin inhibitors are the initial therapy for steroid-resistant nephrotic syndrome (1C). In adults, minimal change disease requires biopsy confirmation and is treated with high-dose oral glucocorticoids (1C) for no longer than 16 weeks. For primary FSGS, high-dose glucocorticoids remain first-line (1D) and a calcineurin inhibitor is recommended for resistant or intolerant patients (1C); the guideline proposes a revised FSGS classification and drops the term idiopathic.

Vasculitis, lupus nephritis and anti-GBM disease

Initial therapy for ANCA-associated vasculitis is cyclophosphamide or rituximab with glucocorticoids, at lower glucocorticoid doses than before; plasma exchange is not suggested routinely, and maintenance therapy, preferably rituximab, should last at least 18 months. In lupus nephritis, antimalarials are recommended for all patients without contraindications, initial treatment uses glucocorticoids with mycophenolate mofetil or low-dose intravenous cyclophosphamide, and total immunosuppression should not be shorter than 36 months after a complete response. Anti-GBM disease is treated with glucocorticoids, cyclophosphamide and plasmapheresis (1C).

Infection-related glomerulonephritis

Patients with replicative hepatitis B (HBV DNA above 2000 IU/ml) and GN should receive nucleos(t)ide analogues (1C), and cyclophosphamide or rituximab should be avoided until the infection is treated. Antiretroviral therapy should be started in all patients with HIV and CKD, regardless of CD4 count (1C).

FAQ

Is a kidney biopsy needed to diagnose membranous nephropathy?

Not in patients with nephrotic syndrome and a positive anti-PLA2R antibody test, although a biopsy can still add important information.

How long should children with nephrotic syndrome receive initial glucocorticoids?

High-dose glucocorticoids for a total of 8-12 weeks instead of 24 weeks (Grade 1B).

Is plasma exchange recommended in ANCA-associated vasculitis?

Not routinely, because the PEXIVAS trial did not show that it delayed kidney failure or death; it should be used if ANCA vasculitis overlaps with anti-GBM disease.

Source

Rovin BH, Adler SG, Barratt J, et al. Executive summary of the KDIGO 2021 Guideline for the Management of Glomerular Diseases. Kidney Int 2021;100:753-779. DOI: 10.1016/j.kint.2021.05.015. Open access under CC BY-NC-ND 4.0. Summary prepared by Medpresso from the original publication; it is not a substitute for the full text or for medical advice.