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Nephritic Syndromes. Dr. Raid Jastania. Nephritic Syndrome. Diffuse Proliferative (post infectious) GN Rapidly Progressive GN (Crescentic GN) IgA Nephropathy Chronic Glomerulonephritis. Acute Proliferative/Diffuse Proliferative/Post-infectious Glomerulonephritis.
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Nephritic Syndromes Dr. Raid Jastania
Nephritic Syndrome • Diffuse Proliferative (post infectious) GN • Rapidly Progressive GN (Crescentic GN) • IgA Nephropathy • Chronic Glomerulonephritis
Acute Proliferative/Diffuse Proliferative/Post-infectious Glomerulonephritis • Post strep (staph, measles, mumps, HepB, HepC) • Immune complex
Acute Proliferative/Diffuse Proliferative/Post-infectious Glomerulonephritis • Light microscopy
Acute Proliferative/Diffuse Proliferative/Post-infectious Glomerulonephritis • Light microscopy: • Proliferative: mesangial, endothelial, inflammation (neutrophils) • Thrombi • necrosis
Acute Proliferative/Diffuse Proliferative/Post-infectious Glomerulonephritis • Electron microscopy:
Acute Proliferative/Diffuse Proliferative/Post-infectious Glomerulonephritis • Electron microscopy: • Sub-epithelial humps • Other deposits
Acute Proliferative/Diffuse Proliferative/Post-infectious Glomerulonephritis • Immuno Fluorescence: • Ig, Comp
Acute Proliferative/Diffuse Proliferative/Post-infectious Glomerulonephritis • Prognosis
Acute Proliferative/Diffuse Proliferative/Post-infectious Glomerulonephritis • Prognosis: • Progression is uncommon • 15-50% may progress to CRF
Rapidly Progressive GN (Crescentic GN) • Type I: Anti GBM • 12% • LM: Crescent • IF: Linear deposits, IgG, C3 • EM: deposits, GBM rupture
Rapidly Progressive GN (Crescentic GN) • Type II: Immune complex • 44% • Post strep, IgA nephropathy • LM: crescent • IF: deposits similar to the primary disease • EM: deposits, GBM rupture
Rapidly Progressive GN (Crescentic GN) • Type III: Pauci-immune, ANCA positive • 44% • Vasculitis: Wegener granulomatosis, microscopic polyarteritis • LM: crescent • IF: neg • EM: neg, GBM rupture
IgA nephropathy • Children, young adults • Microscopic, gross hematuria, recurrent • Loin pain • Association: Henoch-Schonlein purpura, Celiac disease, Liver disease
IgA nephropathy • Pathogenesis
IgA nephropathy • Pathogenesis: • Abnormal IgA production and clearance • High level of IgA • Deposits of IgA • Immune complex • Activation of alternative complement system (C3 only)
IgA nephropathy • Light micorscopy
IgA nephropathy • Light microscopy: • Normal or mesangial expansion
IgA nephropathy • Immuno Fluorescence:
IgA nephropathy • Immuno Fluorescence: • IgA in mesangium
IgA nephropathy • Electron microscopy
IgA nephropathy • Electron microscopy: • deposits
IgA nephropathy • Prognosis:
IgA nephropathy • Prognosis: • 25-50% progress to CRF
Chronic Glomerulonphritis • Late stage of glomerular disease • Found in end-stage renal disease/CRF • Represent 30-50% of patients on hemodialysis • Young and middle age
What are the possible causes of this appearance of the kidneys?
Describe the four compartments (glomeruli, tubules, interstitium, and vasculature)
Chronic Glomerulonphritis • Gross: • Contracted kidneys • Atrophic with granular surface
Chronic Glomerulonphritis • Micro: • Glomerular sclerosis/hyalinizaiton • Interstitial fibrosis/inflammation • Tubular atrophy • Thick vessels (hypertension)