DPGN
Summary
Diffuse Proliferative Glomerulonephritis (DPGN) is the most severe and common renal manifestation of systemic lupus erythematosus (SLE), classified as WHO/ISN-RPS Class IV lupus nephritis. It presents with hematuria, proteinuria (often nephrotic-range), hypertension, and progressive renal insufficiency due to immune complex deposition affecting >50% of glomeruli.
Detail
DPGN is characterized by immune complex deposition (containing DNA, anti-DNA antibodies, and complement) in the subendothelial space, leading to diffuse proliferation of endothelial and mesangial cells affecting the majority of glomeruli (>50%) and often involving >50% of each glomerulus. This results in a 'wire-loop' appearance on light microscopy due to thickened capillary walls from subendothelial immune deposits. Immunofluorescence shows granular 'full-house' staining (IgG, IgA, IgM, C3, C1q). Electron microscopy reveals subendothelial dense deposits. Clinically, patients present with nephritic-nephrotic features: hematuria with RBC casts, proteinuria (can be nephrotic-range >3.5g/day), hypertension, and decreasing GFR. It's associated with hypocomplementemia (low C3/C4) due to complement consumption. This is the most common and most severe class of lupus nephritis, carrying the worst prognosis among lupus nephritis classes if untreated, with risk of progression to end-stage renal disease. Treatment involves aggressive immunosuppression with corticosteroids plus cyclophosphamide or mycophenolate mofetil. Distinguishing DPGN from other lupus nephritis classes (especially Class III focal proliferative GN) is crucial as it dictates treatment intensity and prognosis. Renal biopsy is required for diagnosis and classification.
Sources
- Robbins and Cotran Pathologic Basis of Disease
- First Aid for the USMLE Step 1
- Harrison's Principles of Internal Medicine
- UpToDate: Diagnosis and classification of renal disease in systemic lupus erythematosus
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