14/15: Renal Pathology - Fang Flashcards by Elle B

kidneys fail to differentiate

agenesis

incompatible with life if bilateral
adrenal glands will expand if the kidney is not there

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failure of kidneys to develop to a normal size

hypoplasia

commonly unilateral

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ectopic (pelvic) kidney complications

normal size, but kinking or tortuosity of the ureters may occur causing obstruction and predisposing to infection

may see pts with repeated history of stones or infections

horseshoe kidneys have lower risks than ectopic

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Autosomal Dominant cystic diseases affect….

autosomal recessive cystic disease affect…

ADults

children

acquired cystic disease is dialysis associated

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4 leading cause of ESRD

describe autosomal dominant polycystic kidney disease AKPKD

hereditary (genes PKD1 and 2)

multiple expanding cysts in both kidneys that eventually destroy with renal failure in 50s (renal functional decline is related to increase in kidney size (30 cm and dark in color) and cystic volume)

associated with hepatic cysts and berry aneurysms** (fatal subarachnoid hemorrhage - 10%)

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normal size kidney

12 cm length
6 cm width
3 cm depth

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describe autosomal recessive polycycstic kidney disease ARPKD

childhood

huge, white, smooth-surfaced kidneys at birth

small cysts develop from collecting ducts

associated with congenital hepatic fibrossis

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retention aka acquired aka…

simple cysts - cortical

very common - not generally clinically significant

only when too big (5cm) do they become issue

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3 studies done with renal bx

light microscopy LM
immunofluorescence microscopy IF
electron microscopy EM

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podocytes aka

visceral epithelium

ENDOthelium - fenestrated capillary

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focal v. diffuse

global v segmental

primary v. secondary v. idiopathic

WHOLE KIDNEY
less than 50%
greater than 50%

whole glomerulus
portion of glomerulus

renal disease
systemic disease
etiology unknown

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3 patterns of IF staining in glomerulus

linear capillary loop pattern
ex: Anti-GBM disease

granular capillary loop pattern
ex: membranous nephropathy, lupus nephritis

mesangial pattern
ex: lupus nephritis, IgA nephropahty

renal biopsy stains G, A, and M and complements *excludes ED

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describe three main clinical renal syndromes

acute renal failure

nephrotic syndrome

nephritic syndrome

oliguria - tubule injury

proteinuria - glomerulus injury

hematuria - glomerulus injury

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nephritic syndrome

HTN
oliguria
proteinuria
azotemia
hematuria
RBC casts

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nephrotic syndrome

proteinuria greater than 3.5 g/ 24 h
pitting edema and ascities due to hypoalbuminemia
hypercoagulable state
hypercholesterolemia
hypogammaglobulinemia
fatty casts

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due to breaks in the glomerular capillary loops

due to glomerular capillary filtration defects

nephritic

nephrotic

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effacement is associated with …

proteinuria and nephrotic syndrome

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due to tubular injury in most cases

acute renal failure

pathology of chronic renal failure

GROSS
Kidneys are small
Cortex thinned
Increase pelvic fat

MICRO
Glomerular sclerosis
Interstitial fibrosis
Tubular atrophy

LM findings in chronic renal failure **

1- glomerular sclerosis
2 - interstitial fibrosis
3 - tubular atrophy

primary and secondary causes of nephrotic syndrome

primary

minimal change
focal segmental glomerulosclerosis
membranous nephropathy

Secondary
- diabetic nephropathy

minimal change disease looks like ..

children
puffy eyes

most common cause of nephrotic syndrome in children

pathological appearance of minimal change

normal glomeruli on LM an dIF

EFFACEMENT OF FOOT PROCESSES ON EM***

lipid in tubular cells (why aka lipoid nephrosis)

usually respond to steroids

diffuse epithelial foot process effacement =

minimal change disease

proteinuria with progressive glomerular scarring

focal segmental glomerulosclerosis FSGS early in the disease course, glomerulosclerosis is focal and segmental. With progression, more diffuse and global glomerulosclerosis develops 80% idiopathic primary - can be HIV or heroin induced as well

``` nephrotic syndrome HTN common in african american variable degree of decreasing renal function microscopic hematuria ```

FSGS focal segmental glomerulosclerosis

FSGS LM = IF = EM =

segmental sclerosis mild IgM and C3 or negative diffuse epithelial cell injury

how is FSGS different than minimal change

FSGS response to steroids is variable (thus differs from minimal change disease in prognosis)

deposition of Ag-Ab complexes

membranous glomerulopathy

membranous glomerulopathy LM IF EM

LM: thickened capillary walls; “spikes” on silver stain IF: granular IgG and C3 along the GBM EM: subepithelial deposits

Membranoproliferative Glomerulonephritis (MPGN)

usually nephrotic syndrome with a nephritic component (hematuria) - - slowly progressive course - - steroids and immunosuppressive agents NOT effective - - 50% --> chronic renal failure within 10 yrs

mesangial hypercullularity and capillary wall remodeling iwth the formation of double contours =

mesangiocapillary glomerulonephritis aka MPGN

type I v. type II MPGN

Subendothelial deposits –Type I or Intramembranous deposits– Type II Predominant MESANGIAL involvement

LM: “tram tracks” *** (on silver stain) IF: granular C3 (and often with IgG) EM: subendothelial and mesangial deposits -- mesangial interpositioning between endothelial cells and GBM

MPGN Type I Granular deposition of IgG and C3 in type I (C3 alone in type II) Type II --- 70% have C3 nephritic factor (C3NeF; an autoantibody which stabilizes C3 convertase, protecting it from enzymatic degradation) therefore, persistent C3 activation and consumption, causing hypocomplementemia low serum C3 !!!

LM: “tram track” (on silver stain) IF: C3 only EM: Intramembrane deposit. --- lamina densa - electron dense material (“dense deposit”)

MPGN type II also see low serum C3

- dark colored urine | - RBC in urine

nephritic syndrome pt may also have renal filure (increased serum creatinine and BUN - acute nephritic syndrome)

2 conditions that cause acute nephritic syndrome

Diffuse proliferative glomerulonephritis ( Acute post-strep GN ) Crescentic glomerulonepritis ( 1 Goodpasture’s disease 2 Lupus nephritis 3 ANCA-associated diseases)

hematuria, azotemia, oliguria, low C3 in children after a latent period of 1-3 weeks following a strep infection

ACUTE GLOMERULONEPHRITIS ``` POST-STREPTOCOCCAL = old term POST-INFECTIOUS = new term ``` 95% full recovery

LM: diffuse proliferative GN with neutrophils IF: scattered granular (“starry sky”) IgG, IgM, and C3 along GBM and in the mesangium EM: subepithelial “humps” *** -- IgG, IgM, C3 along GBM FOCALLY

Post-strep GN Enlarged, hypercelluar glomeruli with endothelial and mesangial cell proliferation

crescentic - antic GBM Ab - IC - Anti- PMN Ab

crescentic GN components of crescent: Fibrin forms and there is then proliferation of epithelial cells from the parietal wall of Bowman’s capsule, as well as influx of monocytes/macrophages, resulting in crescent formation.

linear "cigarette smoke" capillary loops outlined by linear anti-BM antibody

``` type I crescentric goodpasture disease (antibodies against collagen IV) ```

granular "lumpy-bumpy" capillary loops outlined by granular immune complex deposits

type II crescentic | SLE

rapidly progressive glomerulonephritis is most often associated with..

crescentic GN