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Lupus nephritis

Definition
LN is a severe organ manifestation of the autoimmune disease SLE, characterized by inflammation of the kidneys.
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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.
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Epidemiology
The incidence of LN in the US is estimated at 0.72 per 100,000 person-years.
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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.
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Prognosis and risk of recurrence
The prognosis of LN is severe, with up to 20% of patients progressing to ESRD.
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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 Kidney Disease: Improving Global Outcomes Foundation (KDIGO 2024), the European League Against Rheumatism (EULAR 2023), the European Dialysis and Transplant Association (ERA-EDTA/EULAR 2020), the Royal College of Ophthalmologists (RCOphth 2020), and the American College of Rheumatology (ACR 2012).
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Guidelines

1.Screening and diagnosis

Classification criteria: define LN according to the ACR classification criteria.
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2.Diagnostic investigations

Initial evaluation: obtain the following baseline investigations in patients with active LN:
BP measurement
serum creatinine
urinalysis
urine protein-to-creatinine ratio
C3/C4 levels
anti-DNA levels
B

3.Diagnostic procedures

Kidney biopsy: as per 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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4.Medical management

Goals of treatment: 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

5.Therapeutic procedures

Plasma exchange: initiate plasma exchange therapy therapy in patients with LN and thrombotic microangiopathy.
B

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  • RRT

6.Surgical interventions

Kidney transplantation: as per KDIGO 2024 guidelines, consider offering kidney transplantation, preferred over long-term dialysis, in patients with LN developed kidney failure.
C

7.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

8.Patient education

Pregnancy counseling: as per KDIGO 2024 guidelines, counsel patients with active LN to avoid pregnancy while the disease is active or when treatment with potentially teratogenic drugs is ongoing and for ≥ 6 months after LN becomes inactive.
B

9.Preventative measures

Bone protection: 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

10.Follow-up and surveillance

Assessment of treatment response: 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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  • Follow-up