Table of contents
Lupus nephritis
What's new
Updated 2024 KDIGO guidelines for the diagnosis and management of lupus nephritis.
Background
Overview
Definition
LN is a severe organ manifestation of the autoimmune disease SLE, characterized by inflammation of the kidneys.
1
Pathophysiology
The pathophysiology of LN involves multiple pathways, including aberrant apoptosis, autoantibody production, immune complex deposition, and complement activation. These processes lead to the formation of autoantibodies that deposit in the kidney, triggering inflammation and damage to the renal tissue.
2
Epidemiology
The incidence of LN in the US is estimated at 0.72 per 100,000 person-years.
3
Disease course
LN is a severe organ manifestation of SLE, with clinical features that can range from asymptomatic proteinuria to manifestations associated with nephritic and nephrotic syndromes and ESRD.
4
Prognosis and risk of recurrence
The prognosis of LN is severe, with up to 20% of patients progressing to ESRD.
4
Guidelines
Key sources
The following summarized guidelines for the evaluation and management of lupus nephritis are prepared by our editorial team based on guidelines from the European League Against Rheumatism (EULAR 2024), the Kidney Disease: Improving Global Outcomes Foundation (KDIGO 2024), the European Dialysis and Transplant Association (ERA-EDTA/EULAR 2020), the Royal College of Ophthalmologists (RCOphth 2020), and the American College of Rheumatology (ACR...
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Screening and diagnosis
Diagnostic investigations
Diagnostic procedures
Kidney biopsy: as per ERA-EDTA/EULAR 2020 guidelines, Consider performing kidney biopsy if there is evidence of kidney involvement, especially in the presence of persistent proteinuria ≥ 0.5 g/24 hours or urine protein-to-creatine ratio ≥ 500 mg/g in morning first void urine
C
and/or an unexplained decrease in GFR. C
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Medical management
Goals of treatment: as per ERA-EDTA/EULAR 2020 guidelines, Set the following as goals of treatment:
patient survival
long-term preservation of kidney function
prevention of disease flares
prevention of organ damage
management of comorbidities
improvement in disease-related QoL.
B
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Immunosuppressive therapy (general principles)
Immunosuppressive therapy (class I-II LN)
Immunosuppressive therapy (class III and IV LN)
Immunosuppressive therapy (class V LN)
Hydroxychloroquine
Belimumab
Management after adequate response
Management of inadequate treatment response
Management of relapse after complete or partial remission
RAAS blocker therapy
Statin therapy
Antithrombotic therapy
Antihypertensive therapy
Adjunctive therapies
Therapeutic procedures
Plasma exchange: as per ACR 2012 guidelines, Initiate plasma exchange therapy therapy in patients with LN and thrombotic microangiopathy.
B
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RRT
Surgical interventions
Specific circumstances
Pregnant patients
As per KDIGO 2024 guidelines:
Continue hydroxychloroquine during pregnancy and start low-dose aspirin before 16 weeks of gestation to reduce the risk of pregnancy complications.
B
Recognize that corticosteroids, hydroxychloroquine, azathioprine, tacrolimus, and cyclosporine are considered safe immunosuppressive treatments during pregnancy.
B
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Pediatric patients
Patients with thrombotic microangiopathy
Patient education
Preventative measures
Bone protection: as per ERA-EDTA/EULAR 2020 guidelines, Offer calcium/vitamin D supplementation and/or antiresorptive agents for bone protection to reduce treatment-related and disease-related comorbidities in patients with LN.
B
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Routine immunizations
Follow-up and surveillance
Assessment of treatment response: as per KDIGO 2024 guidelines, Define response to treatment in patients with LN as follows:
Situation
Guidance
Complete response
Reduction in proteinuria < 0.5 g/g (< 50 mg/mmol) measured as the protein-creatinine ratio from a 24-hour urine collection (proteinuria < 0.5 g/1.73 m²/day or < 300 mg/m²/day based on a 24-hour urine specimen in pediatric patients)
Stabilization or improvement in kidney function (±10-15% of baseline)
Within 6-12 months of initiating therapy, but could take > 12 months
Primary efficacy renal response
Protein-creatinine ratio ≤ 0.7 g/g (≤ 70 mg/mmol)
Estimated glomerulat filtration ration that was no worse than 20% below the pre-flare value or ≥ 60 mL/min/1.73 m²
No use of rescue therapy for treatment failure
Partial response
Reduction in proteinuria by at least 50% and to < 3 g/g (< 300 mg/mmol) measured as the protein-creatinine ratio from a 24-hour urine collection
Stabilization or improvement in kidney function (±10-15% of baseline)
Within 6-12 months of initiating therapy
No kidney response
Failure to achieve a partial or complete response within 6-12 months of initiating therapy
B
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Surveillance for hydroxychloroquine retinopathy
Follow-up