Lupus Nephritis

Lupus Nephritis Is Among the Most Severe and Dangerous Complications of Systematic Lupus Erythematosus

Systematic lupus erythematosus (SLE), commonly known as lupus, is a chronic autoimmune disease where the body's immune system mistakenly attacks its own healthy tissues and organs. Over 200,000 people in the United States are estimated to have SLE, ^a of which 20% to 60% develop lupus nephritis (LN). ^b

Lupus Nephritis (LN), a condition caused by the immune system attacking the kidneys. On the left, a diagram of the human body highlights affected systems: skin (malar/discoid rash), CNS (neurological damage, affective disorder), lungs (inflammation), blood (anemia, thrombocytopenia, IFN signature), serum (anti-nuclear antibodies), spleen (splenomegaly), joints (arthritis), and kidneys (glomerulonephritis). On the right, four key facts are listed:

LN occurs when the immune system attacks the kidneys.
SLE/LN disproportionately affects women and people of color.
Measuring proteinuria (protein in the urine) is critical for monitoring disease activity and therapy response.
Inflammation causes blood and protein in urine, impaired kidney function, and potential kidney failure.

^aU.S. Centers for Disease Control and Prevention 2024
‍^b KDIGO Lupus Nephritis Work Group, Kidney Int 2024
‍^c Tamirou et al., Ann Rheum Dis 2016

Proteinuria Reduction Is Associated with Long-Term Renal Preservation

The larger the initial reduction in proteinuria in the first several months of management, the lower the risk of end-stage kidney disease.

Kaplan-Meier survival curve titled “Kidney Survival Based on Proteinuria Response Status.”
The x-axis shows time from study entry in months (0 to 200), and the y-axis shows kidney survival percentage (0% to 100%).

Three survival curves are displayed:

Teal line – labeled “Complete Response”: Shows consistently high kidney survival over time, ending with 94% survival at 10 years.
Blue line – labeled “Partial Response”: Shows a gradual decline in kidney survival, with 45% survival at 10 years.
Light purple line – labeled “No Response”: Shows a steep decline in kidney survival, with 19% survival at 10 years.
Three orange circles highlight the 10-year kidney survival rates for each group:

94% for complete response
45% for partial response
19% for no response
The graph demonstrates a strong correlation between proteinuria response and long-term kidney survival, with better outcomes in patients who achieve a complete response.

^a Adapted with permission from Chen et al., Clin J Am Soc Nephro 2008
‍^b Retrospective analysis of patients (N=86) enrolled in the prospective, controlled study of plasmapheresis in severe LN to determine long-term prognosis of achieving partial response. Complete response was defined as SCr ≤1.4 mg/dL and proteinuria ≤0.33 g/day within 5 years of study entry, and partial response was defined as ≤25% increase in baseline SCr and ≥50% reduction in baseline proteinuria to ≤1.5 g/day (but >0.33 g/day) within 5 years of entering the study. Kidney survival was determined by kidney failure (≥6 mg/dL SCr or the initiation of kidney replacement therapy).

Recent American College of Rheumatology (ACR) Lupus Nephritis Treatment Guideline Update

In November 2024, the ACR released an updated guideline for the treatment of LN that emphasizes early and aggressive treatment to preserve kidney function. Specifically, this updated guideline now details the following:

‍^a2024 ACR Guideline for the Screening, Treatment, and Management of Lupus Nephritis: Guideline Summary 2024