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NEPHROTIC and NEPHRITIC SYNDROMES

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Disease Most Frequent Clinical Presentation Pathogenesis Light Microscope F.M. (Fluorescence Microscope)

E.M. (Electron Microscope)

Age Group Affected Treatment and

Outcome

Minimal Change Disease

(Lipoid Nephrosis)

  • Selective proteinuria (Albumin)
  • Loss of foot processes
  • Loss of GBM polyanionic sites
  • Appearance of villi on epithelial cells
  • Normal

    Lipid in tubules

    F.M. = negative

    E.M. = loss of foot processes, lipid vacuoles

    #1 cause of Nephrotic Syndrome in children, esp. boys younger than 6 yrs. old. Responds well to corticosteroids.

    No progression into chronic renal failure

    Focal Segmental Glomerular Sclerosis
  • Non-selective proteinuria
  • Hypertension
  • Microscopic hematuria
  • Idiopathic
  • Lower renal mass (in obese)
  • 2 causes: heroin use, HIV
  • Focal and segmental sclerosis
  • Hyalinosis
  • Adhesions to Bowman's Capsule
  • Hypercellular mesangium
  • Thick B.M.
  • F.M. = IgM, C3

    E.M. = Loss of foot processes, detachment of epithelium from B.M.

    Majority occur in older children. Also occurs in adults. Does not respond to corticosteroids. Leads to renal failure.
    Membranous Nephropathy

    (Glomerulonephritis)

  • Persistent proteinuria
  • Idiopathic
  • 2 causes: carcinomas, SLE, hepatitis, Diabetes Mellitus, thyroiditis, drugs.
  • Glomeruli are enlarged yet normocellular
  • No cellular proliferation
  • B.M. Thickening
  • F.M. = "Spike and Dome." Granular IgG, C3

    E.M. = Subepithelial immune deposits in B.M., thickened B.M.

    #1 cause of Nephrotic syndrome in adults Benefit of corticosteroids is unknown.
    Diabetic Nephropathy

    (Diabetic Glomerulosclerosis)

  • Proteinuria
  • No hematuria
  • Diabetic microangiopathy
  • Thickened B.M.
  • Massive mesangial growth
  • "Kimmelstiel Wilson" nodular glomerulosclerosis
  • Diffuse glomerulosclerosis
  • Kimmelstiel-Wilson Nodules are pathognomonic.
  • Massive mesangial hypercellularity.
  • F.M. = negative

    E.M. = massive mesangial growth, thickened B.M.

    Diabetics Progresses to renal failure
    Renal Amyloidosis Subendothelial and mesangial amyloid deposits
  • Amyloid deposits are initially mesangial, producing mesangial widening without hypercellularity.
  • Later, the amyloid obliterates the lumen
  • PAS(-)
  • Congo-Red (+)
  • F.M. = negative

    E.M. = characteristic criss-cross fibrillary proteins.

    Any age group Severe amyloid infiltration leads to renal failure
    Alport Syndrome

    (Hereditary Nephritis)

  • Recurrent hematuria before age 20
  • Hypertension
  • Deafness and ocular problems
  • Structural defect in Collagen IV leads to leaky basement membranes. Looks normal F.M. = negative

    E.M. = glomerular B.M. splitting

    Symptoms appear before age 20 Progresses to renal failure
    Benign Familial Hematuria

    (Thin B.M. Disease)

  • Recurrent hematuria
  • Most frequent cause of asymptomatic hematuria.
  • Reduced thickness of glomerular B.M. Looks normal F.M. = negative

    E.M. = reduced glomerular B.M. thickness

    Acute (Post-Streptococcal) Glomerulonephritis
  • Acute nephritis
  • Abrupt oliguria, hematuria, facial edema, hypertension.
  • Immune-complex mediated (Type-III hypersensitivity)
  • Occurs after Streptococcal pharyngitis or Hepatitis-B
  • High ASO-titer, low C3
  • Glomerular hypercellularity
  • Increase in endothelial cells, mesangial cells, and PMN's.
  • No increase in epithelial cells.
  • No B.M. thickening
  • F.M. = "lumpy-bumpy" granular deposits of IgG and C3

    E.M. = Subepithelial (not subendothelial) "humps," otherwise normal appearing B.M.

    Common renal disease in childhood Return to normal in 8 weeks.

    Complete recovery without treatment (especially in kids) within 3 years.

    SLE Nephropathy Degree of kidney involvement correlates with prognosis in SLE. Anti ds-DNA antibodies.
  • WHO I: Normal
  • WHO II: Increased mesangial matrix
  • WHO III: Focal proliferation
  • WHO IV: Diffuse proliferation, worst.
  • WHO V: Identical to Membranous Nephropathy
  • F.M. = IgM, IgG + C3
  • Type-I: Granular appearance
  • Type-II: Pseudo-linear appearance
  • Focal Segmental Glomerulonephritis IgA Nephropathy (Berger's Disease): Most common primary glomerulonephritis Circulating IgA + fibronectin (due to chronic liver disease)
  • Mesangial cell proliferation
  • F.M. = Granular appearance, IgG + C3

    E.M. = Mesangial deposits

    Young men 15-30
    Henoch-Schonlein Purpura Same as above, plus systemic disease: purpura of extremities, arthritis, colicky abdominal pain.
  • Mesangial cell proliferation, more serious than above.
  • F.M. = Granular appearance, IgG + C3

    E.M. = Mesangial deposits

    Children
    Endocarditis S. Aureus
  • Subepithelial immune deposits
  • F.M. = Granular appearance, IgG + C3 Kidney disease resolves when infection is cured.
    Rapidly Progressive Crescentic Glomerulonephritis
  • Wegener's: kidney + upper respiratory tract.
  • Anuria
  • Oliguria
  • Inflamed glomerular capillaries
  • ANCA (+)
  • Cells accumulate in Bowman's Capsule
  • Fibrin trapped in glomeruli
  • Epithelial cell proliferation
  • Macrophage, PMN infiltrates
  • F.M. = Pauci-immune. Irregular

    E.M. = wrinkling, discontinuity of B.M.

    Must be treated or it will go to renal failure within weeks.
    Goodpasture Syndrome

    (Anti-BM Antibody Disease)

    Lung (hemoptysis) + kidneys (hematuria) Anti-B.M. antibodies, against Type-IV collagen Similar to Crescentic glomerulonephritis, as above. F.M. = Linear pattern, IgG + C3

    E.M. = No immune complex deposits

    Males 25-30 Responds to immunosuppressive therapy and plasmapheresis
    Membranoproliferative Glomerulonephritis

    (Mesangiocapillary Glomerulonephritis)

  • B.M. thickening and cellular proliferation
  • Mesangial expansion makes glomerular B.M. appear as though it were in two layers
  • E.M. = "Tram-track" appearance, resulting from double-layer appearance of glomerular B.M.

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    Copyright 1999, Scott Goodman, all rights reserved