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NEPHROTIC and NEPHRITIC SYNDROMES

Disease Most FrequentClinical Presentation

Pathogenesis Light Microscope F.M.(FluorescenceMicroscope)

E.M. (ElectronMicroscope)

Age Group Affected Treatment and

Outcome

Minimal Change Disease

(Lipoid Nephrosis)

• Selectiveproteinuria (Albumin)

• Loss of footprocesses• Loss of GBMpolyanionic sites• Appearance of villion epithelial cells

Normal

Lipid in tubules

F.M. = negative

E.M. = loss of footprocesses, lipidvacuoles

#1 cause of NephroticSyndrome in children,esp. boys younger than6 yrs. old.

Responds well tocorticosteroids.

No progressioninto chronic renalfailure

Focal SegmentalGlomerular Sclerosis

• Non-selectiveproteinuria• Hypertension• Microscopichematuria

• Idiopathic• Lower renal mass(in obese)• 2 causes: heroin use,HIV

• Focal andsegmental sclerosis• Hyalinosis• Adhesions toBowman's Capsule• Hypercellularmesangium• Thick B.M.

F.M. = IgM, C3

E.M. = Loss offoot processes,detachment ofepithelium fromB.M.

Majority occur in olderchildren. Also occursin adults.

Does not respondtocorticosteroids.Leads to renalfailure.

MembranousNephropathy

(Glomerulonephritis)

• Persistentproteinuria

• Idiopathic• 2 causes:carcinomas, SLE,hepatitis, DiabetesMellitus, thyroiditis,drugs.

• Glomeruli areenlarged yetnormocellular• No cellularproliferation• B.M.Thickening

F.M. = "Spike andDome." GranularIgG, C3

E.M. =Subepithelialimmune depositsin B.M., thickenedB.M.

#1 cause of Nephroticsyndrome in adults

Benefit ofcorticosteroids isunknown.

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Diabetic Nephropathy

(DiabeticGlomerulosclerosis)

• Proteinuria• No hematuria

• Diabeticmicroangiopathy• Thickened B.M.• Massive mesangialgrowth• "KimmelstielWilson" nodularglomerulosclerosis• Diffuseglomerulosclerosis

• Kimmelstiel-WilsonNodules arepathognomonic.• Massive mesangialhypercellularity.

F.M. = negative

E.M. = massivemesangial growth,thickened B.M.

DiabeticsProgresses to renalfailure

Reoidosisnal AmylSubendothelial andmesangial amyloiddeposits

• Amyloid depositsare initially mesangial,producing mesangialwidening withouthypercellularity.• Later, the amyloidobliterates the lumen• PAS(-)• Congo-Red (+)

F.M. = negative

E.M. =characteristic criss-cross fibrillaryproteins.

Any age groupSevere amyloidinfiltration leads torenal failure

Alport Syndrome

(Hereditary Nephritis)

• Recurrenthematuria before age20• Hypertension• Deafness andocular problems

Structural defect inCollagen IV leads toleaky basementmembranes.

Looks normal F.M. = negative

E.M. = glomerularB.M. splitting

Symptoms appearbefore age 20

Progresses torenal failure

Benign FamilialHematuria

(Thin B.M. Disease)

• Recurrenthematuria• Most frequentcause ofasymptomatichematuria.

Reduced thickness ofglomerular B.M.

Looks normal

F.M. = negative

E.M. = reducedglomerular B.M.thickness

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Acute (Post-Streptococcal)Glomerulonephritis

• Acute nephritis• Abrupt oliguria,hematuria, facialedema, hypertension.

• Immune-complexmediated (Type-IIIhypersensitivity)• Occurs afterStreptococcalpharyngitis orHepatitis-B• High ASO-titer, lowC3

• Glomerularhypercellularity• Increase inendothelial cells,mesangial cells,and PMN's.• No increase inepithelial cells.• No B.M.thickening

F.M. = "lumpy-bumpy" granulardeposits of IgGand C3

E.M. =Subepithelial (notsubendothelial)"humps,"otherwise normalappearing B.M.

Common renal diseasein childhood

Return to normalin 8 weeks.

Completerecovery withouttreatment(especially inkids) within 3years.

SLE Nephropathy

Degree of kidneyinvolvementcorrelates withprognosis in SLE.

Anti ds-DNAantibodies.

• WHO I: Normal• WHO II:Increasedmesangial matrix• WHO III: Focalproliferation• WHO IV:Diffuseproliferation,worst.• WHO V:Identical toMembranousNephropathy

F.M. = IgM, IgG +C3• Type-I:Granularappearance• Type-II:Pseudo-linearappearance

IgA Nephropathy(Berger's Disease):Most commonprimaryglomerulonephritis

Circulating IgA +fibronectin (due tochronic liver disease)

• Mesangial cellproliferation

F.M. = Granularappearance, IgG +C3

E.M. = Mesangialdeposits

Young men 15-30Focal SegmentalGlomerulonephritis

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Henoch-SchonleinPurpura

Same as above, plussystemic disease:purpura ofextremities, arthritis,colicky abdominalpain.

• Mesangial cellproliferation, moreserious than above.

F.M. = Granularappearance, IgG +C3

E.M. = Mesangialdeposits

Children

Endocarditis S. Aureus• Subepithelialimmune deposits

F.M. = Granularappearance, IgG + C3

Kidney diseaseresolves wheninfection is cured.

Rapidly ProgressiveCrescenticGlomerulonephritis

• Wegener's: kidney +upper respiratory tract.• Anuria• Oliguria

• Inflamedglomerular capillaries• ANCA (+)

• Cells accumulate inBowman's Capsule• Fibrin trapped inglomeruli• Epithelial cellproliferation• Macrophage, PMNinfiltrates

F.M. = Pauci-immune. Irregular

E.M. = wrinkling,discontinuity ofB.M.

Must be treated or itwill go to renal failurewithin weeks.

Goodpasture Syndrome

(Anti-BM AntibodyDisease)

Lung (hemoptysis) +kidneys (hematuria)

Anti-B.M. antibodies,against Type-IVcollagen

Similar to Crescenticglomerulonephritis, asabove.

F.M. = Linearpattern, IgG + C3

E.M. = Noimmune complexdeposits

Males 25-30

Responds toimmunosuppressivetherapy andplasmapheresis

MembranoproliferativeGlomerulonephritis

(MesangiocapillaryGlomerulonephritis)

• B.M. thickening andcellular proliferation• Mesangialexpansion makesglomerular B.M.appear as though itwere in two layers

E.M. = "Tram-track" appearance,resulting fromdouble-layerappearance ofglomerular B.M.