Lupus Nephritis Class IV: Diagnosis and Treatment
Diagnostic Findings
- The American College of Rheumatology recommends that the urine sediment will show activity with granular and erythrocytic casts, which is a marker of active glomerular inflammation in lupus nephritis 1
- The presence of dysmorphic erythrocytes and cellular casts (not hyaline) is characteristic of glomerular lesion, not non-glomerular hematuria 1
- The combination of nephrotic proteinuria (3+), glomerular hematuria (20-30 erythrocytes/field), deteriorated renal function (Cr 1.8, BUN 35), hypoalbuminemia (3.0 g/dL), and hypertension (140/85) defines an active nephritic-nephrotic syndrome 1
Classification of Renal Damage
- The American Journal of Kidney Diseases states that this case corresponds to rapidly progressive glomerulonephritis (RPGN) 1, 2
- The active sediment with casts and the progression of symptoms in 3 weeks indicate an acute proliferative process 2
Histological Classification of Biopsy
- The International Society of Nephrology and the Renal Pathology Society recommend that the findings described correspond to Lupus Nephritis Class IV (diffuse) 1, 3
- The diffuse endocapillary and extracapillary proliferation with subendothelial deposits in >50% of glomeruli specifically defines Class IV according to the ISN/RPS classification 1, 3
Treatment of Choice
- The American College of Rheumatology and Kidney International recommend that the optimal treatment is prednisone + cyclophosphamide 1, 3
- Cyclophosphamide or mycophenolate mofetil are the first-line options for induction in Class IV, both with Level A evidence 1, 3
- The regimen should include: methylprednisolone IV pulses (500-1000 mg/day for 3 days) followed by oral prednisone 0.5-1 mg/kg/day 1
Prognosis without Adequate Treatment
- The American Journal of Kidney Diseases states that without proper treatment, the evolution is towards terminal chronic kidney disease 1, 6
- Lupus nephritis Class IV that is not treated or is undertreated progresses to terminal kidney disease in most cases 1, 6
- With adequate treatment, 60-80% of patients achieve complete or partial remission 3