Glomerular Diseases

Publication Date: October 1, 2021
Last Updated: January 3, 2024

Chapter 2: Immunoglobulin A nephropathy (IgAN)/immunoglobulin A vasculitis (IgAV)

Recommendation 2.3.1

We recommend that all patients have their blood pressure managed, as described in Chapter 1. If the patient has proteinuria >0.5 g/d, we recommend that initial therapy be with either an angiotensin-converting enzyme inhibitor (ACEi) or angiotensin II receptor blocker (ARB). (Level 1, B)
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Recommendation 2.3.1.1

We suggest that patients who remain at high risk of progressive CKD despite maximal supportive care be considered for a 6-month course of glucocorticoid therapy. The important risk of treatment-emergent toxicity must be discussed with patients, particularly those who have an eGFR <50 ml/min per 1.73 m2. (Level 2, B)
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Recommendation 2.3.2

We recommend that all patients with proteinuria >0.5 g/d, irrespective of whether they have hypertension, be treated with either an ACEi or ARB. (Level 1, B)
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Recommendation 2.7.1.1

We recommend not using glucocorticoids to prevent nephritis in patients with isolated extrarenal IgAV. (Level 1, B)
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Chapter 3: Membranous nephropathy

Recommendation 3.3.1

For patients with MN and at least one risk factor for disease progression, we recommend using rituximab or cyclophosphamide and alternate month glucocorticoids for 6 months, or CNI-based therapy for ≥6 months, with the choice of treatment depending on the risk estimate. (Level 1, B)
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Chapter 4: Nephrotic syndrome in children

Recommendation 4.3.1.1

We recommend that oral glucocorticoids be given for 8 weeks (4 weeks of daily glucocorticoids followed by 4 weeks of alternate-day glucocorticoids) or 12 weeks (6 weeks of daily glucocorticoids followed by 6 weeks of alternate-day glucocorticoids). (Level 1, B)
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Recommendation 4.3.2.1

For children with frequently relapsing and steroid-dependent nephrotic syndrome who are currently taking alternate-day glucocorticoids or are off glucocorticoids, we recommend that daily glucocorticoids 0.5 mg/kg/d be given during episodes of upper respiratory tract and other infections for 5–7 days to reduce the risk of relapse. (Level 1, C)
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Recommendation 4.3.2.2

For children with frequently relapsing nephrotic syndrome who develop serious glucocorticoid-related adverse effects and for all children with steroid-dependent nephrotic syndrome, we recommend that glucocorticoid-sparing agents be prescribed, rather than no treatment or continuation with glucocorticoid treatment alone. (Level 1, B)
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Recommendation 4.4.1

We recommend using cyclosporine or tacrolimus as initial second-line therapy for children with steroid-resistant nephrotic syndrome. (Level 1, C)
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Chapter 5: Minimal change disease (MCD) in adults

Recommendation 5.3.1

We recommend high-dose oral glucocorticoids for initial treatment of MCD. (Level 1, C)
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Recommendation 5.3.1.1

We recommend cyclophosphamide, rituximab, CNIs, or mycophenolic acid analogs (MPAA) for the treatment of frequently relapsing/steroid-dependent MCD, rather than prednisone alone or no treatment. (Level 1, C)
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Chapter 6: Focal segmental glomerulosclerosis (FSGS) in adults

Recommendation 6.2.2.1

We recommend that highdose oral glucocorticoids be used as the first-line immunosuppressive treatment for primary FSGS. (Level 1, D)
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Recommendation 6.3.1.1

For adults with steroid-resistant primary FSGS, we recommend that cyclosporine or tacrolimus be given for ≥6 months rather than continuing with glucocorticoid monotherapy or not treating. (Level 1, C)
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Chapter 7: Infection-related glomerulonephritis

Recommendation 7.2.2.3.1

We recommend that patients with replicative HBV infection (as denoted by HBV DNA levels >2000 IU/ml) and GN receive treatment with nucleos(t)ide analogues as recommended for the general population by standard clinical practice guidelines for HBV infection. (Level 1, C)
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Recommendation 7.2.3.3.1

We recommend that antiretroviral therapy be initiated in all patients with HIV and CKD, especially biopsy-proven HIV-associated nephropathy (HIVAN), regardless of CD4 count, adjusted to the degree of kidney function. (Level 1, C)
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Chapter 9: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis

Recommendation 9.3.1.1

We recommend that glucocorticoids in combination with cyclophosphamide or rituximab be used as initial treatment of new-onset AAV. (Level 1, B)
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Recommendation 9.3.2.1

We recommend maintenance therapy with either rituximab or azathioprine and lowdose glucocorticoids after induction of remission. (Level 1, C)
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Chapter 10: Lupus nephritis

Recommendation 10.2.1.1

We recommend that patients with SLE, including those with lupus nephritis (LN), be treated with hydroxychloroquine or an equivalent antimalarial unless contraindicated. (Level 1, C)
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Recommendation 10.2.3.1.1

We recommend that patients with active Class III or IV LN, with or without a membranous component, be treated initially with glucocorticoids plus any one of the following:
mycophenolic acid analogs (MPAA) (Level 1, B)
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low-dose intravenous cyclophosphamide (Level 1, B)
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belimumab and either MPAA or low-dose intravenous cyclophosphamide (Level 1, B)
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MPAA and a calcineurin inhibitor (CNI) when kidney function is not severely impaired (i.e., estimated glomerular filtration rate [eGFR] ≤45 ml/min per 1.73 m2) (Level 1, B)
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Recommendation 10.2.3.2.1

We recommend that after completion of initial therapy, patients should be placed on MPAA for maintenance. (Level 1, B)
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Chapter 11: Anti-glomerular basement membrane (Anti-GBM) antibody glomerulonephritis

Recommendation 11.2.1

We recommend initiating immunosuppression with cyclophosphamide and glucocorticoids plus plasmapheresis in all patients with anti-GBM GN except those who are treated with dialysis at presentation, have 100% crescents or >50% global glomerulosclerosis in an adequate biopsy sample, and do not have pulmonary hemorrhage. (Level 1, C)
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Recommendation Grading

Overview

Title

Management of Glomerular Diseases

Authoring Organizations

Kidney Disease Improving Global Outcomes

National Kidney Foundation

Publication Month/Year

October 1, 2021

Last Updated Month/Year

September 3, 2024

Document Type

Guideline

External Publication Status

Published

Country of Publication

US

Inclusion Criteria

Male, Female, Adolescent, Adult, Child, Older adult

Health Care Settings

Ambulatory, Childcare center, Hospital, Outpatient

Intended Users

Clinical researcher, physician, nurse, nurse practitioner, physician assistant

Scope

Assessment and screening, Treatment, Management

Diseases/Conditions (MeSH)

D005921 - Glomerulonephritis

Keywords

KDIGO, glomerulonephritis, nephrotic syndrome, Clinical Practice Guideline, systematic review, evidence-based recommendations

Source Citation

Kidney Disease: Improving Global Outcomes (KDIGO) Glomerular Diseases Work Group. KDIGO 2021 Clinical Practice Guideline for the Management of Glomerular Diseases. Kidney Int. 2021 Oct;100(4S):S1-S276. doi: 10.1016/j.kint.2021.05.021. PMID: 34556256.

Methodology

Number of Source Documents
976
Literature Search Start Date
September 1, 2018
Literature Search End Date
September 1, 2019