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)

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

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 Glomerulonephritis

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)

Describe the abnormality

Chronic Glomerulonphritis

• Gross:

• Contracted kidneys

• Atrophic with granular surface

Chronic Glomerulonphritis

• Micro:

• Glomerular sclerosis/hyalinizaiton

• Interstitial fibrosis/inflammation

• Tubular atrophy

• Thick vessels (hypertension)