II. Epidemiology
- Renal disease or Lupus Nephritis is ultimately present in 50 to 60% of SLE cases
III. Grading
- Class 1: Minimal Mesangial (earliest and most mild form)
- Minimal Proteinuria
- Normal Serum Creatinine
- Histology with immune complex deposition on immunofluorescence, but otherwise normal glomeruli
- Class 2: Mesangial Proliferative
- Microscopic Hematuria with or without Proteinuria
- Histology with mesangial matrix expansion and immune complex deposition
- Class 3: Focal Lupus Nephritis
- Microscopic Hematuria
- Proteinuria (Nephrotic Syndrome may be present)
- Hypertension
- eGFR decreased
- Histology with Glomerulonephritis (focal, segmental or global) affecting <50% of glomeruli
- Class 4: Diffuse Lupus Nephritis (most common)
- Includes Class 3 features
- Complement C3 low
- Anti-Double Stranded DNA Antibody (dsDNA) elevated
- Histology with Glomerulonephritis affecting >50% of glomeruli and subendothelial diffuse immune deposits
- Diffuse Segmental Subtype (4S): Segmental lesions affecting >50% of glomeruli
- Diffuse Global Subtype (4G): Global lesions affecting >50% of glomeruli
- Subtypes may be further characterized: Active, Active and chronic, and Inactive with glomerular scar
- Class 5: Membranous Nephropathy
- Nephrotic Syndrome
- Microscopic Hematuria
- Hypertension
- Serum Creatinine normal
- Membranous Nephropathy may be the only SLE manifestation, and may occur combined with Class 3 ot 4
- Histology with subepithelial immune deposits (global or segmental)
- Class 6: Advanced Sclerosing Lupus
- Slowly progressive Chronic Kidney Disease to end-stage Renal Failure
- Proteinuria with otherwise negative Urine Microscopic Exam
- Histology with global sclerosis affecting >90% of glomeruli
IV. Labs
- Screening in Systemic Lupus Erythematosus patients (without diagnosed renal disease)
- Screen Urinalysis and Serum Creatinine every 3-6 months
- Reflex abnormal findings to Urine Protein to Creatinine Ratio
- Renal biopsy indications
- Proteinuria >=1 g per 24 hours OR
- Hematuria > 0.5 g per 24 hours OR
- Cellular Casts
V. Management
-
General
- See Prevention of Kidney Disease Progression
- Consult nephrology and Rheumatology
- Class 1 and 2
- Renin-Angiotensin System agents (e.g. ACE Inhibitors or Angiotensin Receptor Blockers)
- Class 3, 4 and 5
- Induction Phase
- Pulse IV Corticosteroids AND
- Cyclophosphamide IV for 6 doses (over 3-6 months) OR Mycophenolate daily for 6 months
- Cyclosporine or Tacrolimus may be used as alternative in Class 5 disease
- Maintenance Phase
- Wean Corticosteroids to lowest effective dose
- Longterm Mycophenolate or Aziothioprine
- Induction Phase
- Class 6
- Renin-Angiotensin System agents (e.g. ACE Inhibitors or Angiotensin Receptor Blockers)
- Prepare for renal replacment therapy (e.g. Hemodialysis, Renal Transplant)
VI. Resources
- Lupus Nephritis (StatPearls)