Glomerular Dz

Mild clinical abnormalities

Primary

XLR, Hearing, Ocular, a5 of Type IV collagen

Alport's

r

Rx: Renal transplant

LM

↑ed mesangial matrix

IF

EM

Multi-layer laminated

r

"basket-weave" appearance

Thin Membrane Dz

r

AKA Benign familial hematuriaAKA Benign idiopathic hematuriaBe cautious when making diagnosis.There is no universally-accepted standard for normal GBM thickness

LM

IF

EM

BM Thinning

IgA Nephropathy

r

aka Berger's dzEpisodic bouts of hematuriaCommon: young, malebut can affect anyone at any ageProgression to CHRONIC RENAL FAILURE IN 50% of casesNot to be confused with Buerger's dz (thromboangiitis obliterans)

LM

Normal or...

Mesangioprolif

IF

IgA Mesangial deposits

r

Before Dx, rule out the followingLupusHenoch-Schonlein PurpuraMay see IgG and C3 also.

EM

IgA Mesangial deposits

Secondary

LN, mesangioprolif.

r

5 yr survival: 100%

LM

Proliferation of mesangial cells, matrix

IF

IgG, Complement, IgA in Mesangium

EM

Dense deposit in mesangium

Mild Diabetes

Benign HTN

Nephritic

r

Think inflammationNephrItic = Inflammation

Primary

"Lumpy-bumpy", GAS

PIGN

r

Group A Strep (12, 4, 1, 49)PharyngitisTonsillitisOtitis mediaMastoiditisPeritonsillar abscessSkin infectionLatent period of 1-4 weeksyoung>adultmale>femaleCrescents possible

LM

r

Crescents may be seen

Diffuse proliferative

PMNs

IF

Coarsely granular = "lumpy bumpy"

r

Compare to membranous GN

EM

2 or 3 Large Humps

Hypocomplementemia

r

Both types are RESISTANT to STEROID THERAPYMesangial proliferation which advanced to the Basement membrane (HENCE MEMBRANOPROLIFERATIVE)

Immune Complex

r

Low C3Low C4

MPGN-1

LM

Duplicated BM

IF

IgG, C3

EM

Split BM

Subendothelial deposits

Alt. path of Complement

r

Low C3Normal C4

MPGN-2 (DDD)

LM

Duplicated BM

IF

C3 Nephritic Factor

r

Mentioned in Intro. lecture as well

EM

Entire BM is darkened

IgA Nephropathy

r

aka Berger's dzEpisodic bouts of hematuriaCommon: young, malebut can affect anyone at any ageProgression to CHRONIC RENAL FAILURE IN 50% of casesNot to be confused with Buerger's dz (thromboangiitis obliterans)

LM

Normal OR

Mesangioprolif

IF

IgA Mesangial deposits

EM

IgA Mesangial deposits

Secondary

Malar rash, Anti-Sm Ab

All Glomeruli involved

LN, diffuse prolif.

r

Most seriousMost commonCan also present w/VasculitisInterstitial nephritisFemale, 20s-40s

LM

Wire Loop

Hyaline thrombi

IF

"Full House"

r

"Full House" = Positive for the entire panelIgGIgAIgMC3

EM

Subendothelial, Mesangial Deposits

Few glomeruli, segment of glom. tuft

LN, focal prolif.

r

Females, 20s-40s

LM

IF

"Full House"

r

"Full House" = Positive for the entire panelIgGIgAIgMC3

EM

Child: GI, Joint, Skin

Henoch-Schonlein purpura

r

Syndrome:Abdominal PainArthralgiaPurpura

Vasculitis

C-ANCA

Wegener's Granulomatosis

Lung Granuloma

P-ANCA

Hypersensitivity angiitis (microPAN)

r

Crescents possible

Crescents

Fibrinoid necrosis

Focal necrotizing GN

HBsAg

Classic PAN

Renal infarct

anti-BM

r

Crescents possible

GN, no lung

GN+Lung, Type II Hypersens.

Male, Hemoptysis, Dyspnea, Discolored urine

Goodpasture's

r

5:1 M/F ratioPt develops renal failure typically?

Nephrotic

r

Nephrotic syndromeProteinuria: > 3 g / 24 hrsHypoalbuminemiaHypercholesterolemiaEdemaNephrOtic = PrOtein

Primary

1-3 yo, T-cell cytokines affect BM charge, steroids

Min. Change Dz

r

Rx: Steroid therapyGood prognosis (despite massive proteinuria)

LM

r

May see mild increase in mesangial matrix

IF

EM

Effacement of epithelial foot processes

Black, ↓GFR, HTN, Poor response to steroids

Idiopathic FSGS

r

Prognosis: ESRD in 5-20 yrs

LM

Segmental, Focal lesions

r

Juxtamedullary glomeruli

Obliterated capillary lumen

Variant FSGS

Collapse of glomerular capillary loops

IF

EM

Hyaline

Foam cells

Focal damage of VECs

r

Visceral epithelial cells (Rapid Review)

Effacement of epithelial foot processes

Epithelial cell detachment from BM

White adult, Poor response to steroids

Membranous GN

r

WhiteMales > FemalesRARE in childrenALL glomeruli involvedCompare microscopic findings to Acute-PIGN.Stages (I-IV) determined by EMIf immune complex deposition is stopped at early stages, changes are reversible. Immune: LupusSTD: SyphilisInfectious: Malaria, Viral hepatitis (Australia Ag)Iatrogenic: Gold therapy (for RA)Cancer

LM

Diffuse, thickened BM

IF

Finely Granular

r

Opposed to Coarse granular (lumpy-bumpy) in PIGN

IgG, C3

EM

Numerous, small subepithelial deposits

Renal transplant, Neg. for Immune complex Dz

Transplant GN

r

Most common cause of immune complex GN = Membranous GNMust use IF and EM to rule out Membranous GN.

LM

IF

EM

Subendothelial electron-lucent widening

Secondary

(Pos) Congo Red, Crystal Violet, Thioflavin T

Amyloidosis

r

Causes:TBOsteomyelitisBronchiectasisLeprosyUlcerative colitisRheumatoid arthritisEosinophilic, Glossy material

Gross

Dark brown w/ Iodine

Microscopic

Homogenous, pink

(Neg) Congo Red

Fibrillary GN

Fibrils larger than amyloid

LN,membranous

Membranous (Other)

FSGS

HIV

Collapsing features

r

Compression of glomerular tuftHyperplasia of VECs

Rapid progression to ESRD w/in months

Heroin

Obesity

Reflux Neph: Unilateral renal agenesis