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CRRAB II > Renal Pathology Lecture II > Flashcards

Renal Pathology Lecture II Flashcards

1
Q

Medullary Sponge Kidney

A
  • -Disease of adults
  • -Often incidental finding
  • -Normal kidney function
  • -Multiple cystic dilations of collecting ducts in medulla
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2
Q

Gross features of medullary sponge kidney

A

Dilated papillary ducts in the medulla

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3
Q

Micro features of medullary sponge kidney

A

Cysts lined by cuboidal or transitional epithelium

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4
Q

Dialysis-Associated (acquired) cystic disease

A
  • -Most asymptomatic
  • -Almost always develop cysts with dialysis
  • -7% of dialysis patients will develop renal cell carcinoma within the cysts
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5
Q

Simple cysts

A
  • -Multiple or single (typically cortical)
  • -1-5 cm in size commonly
  • -Filled with clear fluid (ultrafiltrate)
  • -Pretty avascular on CT-angiography
  • -Single layer of cuboidal or flattened epithelium line the cysts
  • -No clinical significance, but will want to differentiate from possible tumor
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6
Q

Glomerulonephritis

A

IMMUNE MEDIATED DISEASE

–Primary or secondary

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7
Q

Histologic patterns of glomerular injury

A
  1. Hypercellularity (increase in glomerular cells, increase in WBCs, formation of crescents)
  2. Basement membrane thickening
  3. Hyalinization and sclerosis
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8
Q

Diffuse

A

All glomerular involved

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9
Q

Focal

A

Proportion of glomeruli involved (less than 50%)

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10
Q

Global

A

Entire single glom involved

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11
Q

Segmental

A

Part of single glom involved

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12
Q

Antibody-mediated injury

A
  • -In situ immune complex deposition (Goodpasture, hemyann, planted antigens)
  • -Circulating immune complex antigen
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13
Q

Immune mechanisms of glomerular injury

A
  1. Antibody-mediated injury
  2. Cell-mediated immune injury
  3. Activation of alt. complement path
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14
Q

Anti-GBM Glomerulonephritis

A
  • -Abs directed against normal parts of GBM
  • -Ab may cross-react w/other basement membranes (such as pulmonary alveoli)
  • -Ag component of type IV collagen!
  • -
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15
Q

Anti-GBM glomerulonephritis appearance on IF

A

Homogenous, linear/ribbon-like appearance. Diffuse.

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16
Q

Membranous nephritis

A
  • -Antigen: M-type Phospholipase A2 receptor
  • -IF: granular and interrupted pattern
  • -EM: electron dense deposits along subepithelial aspect of GBM
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17
Q

“Planted” antigens

A
  • -Non-glomerular origin, but localize in kidney

- -Abs form against them

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18
Q

Circulating immune complex nephritis

A
  • -Glomerular injury from trapping of circulating Ag/Ab complexes within gloms
  • -Type III hypersensitivity
  • -Antigens endogenous (SLE) or exogenous (streptococci)
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19
Q

IF and EM findings with circulating immune complex nephritis

A

IF: granular deposits
EM: electron-dense deposits, mesangial, subepithelial, or subendothelial

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20
Q

Progression in glomerular disease

A

Once reduced to 30-50% of normal, progress to end-stage renal failure no matter what inciting event was

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21
Q

Histological findings in glomerular disease

A
  • -Focal segmental glomerulosclerosis

- Tubulointerstitial fibrosis

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22
Q

Focal segmental glomerulosclerosis as an adaptive change

A
  • -Compensatory hypertrophy occurs
  • -Hemodynamic changes in ind. glomeruli (increases in flow, filtration, transcapillary pressure)
  • -Leads to segmental sclerosis
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23
Q

Treatment of focal segmental glomerulosclerosis

A

Renin-angiotensin inhibitors

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24
Q

Tubulointerstitial fibrosis

A
  • -Develops with glomerulonephritides over time
  • -Ischemic tubules downstream from sclerotic glomeruli
  • -Proteinuria directly toxic to downstream tubular cells
  • -Increased acute and chronic inflammation occurs
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25
Q

Nephritic syndrome

A
  • -INFLAMMATORY process
  • -Hematuria
  • -RBC casts in urine
  • -Azotemia, oliguria, HTN (from salt retention), proteinuria
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26
Q

Nephrotic syndrome

A
  • -Massive proteinuria (>3.5g/day)
  • -Hypoalbuminemia leading to edema
  • -Hyperlipidemia
  • -Frothy urine w/fatty casts.
  • -Podocyte damage disrupting filtration charge barrier.
  • -Hypercoagulable state
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27
Q

Types of nephritic syndrome

A
  • -Acute poststreptococcal glomerulonephritis
  • -Rapidly progressive (crescenteric) glomerulonephritis (RPGN)
  • -Diffuse proliferative glomerulonephritis (DPGN) (and nephroltic syndrome)
  • -IgA nephropathy (Berger Disease)
  • -Alport syndrome
  • -Membrano-proliferative glomerulonephritis (MPGN) (often also nephrotic syndrome)
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28
Q

Types of nephrotic syndrome

A
  • -Focal segmental glomerulosclerosis
  • -Minimal change disease (lipoid necrosis)
  • -Membranous nephropathy
  • -Amyloidosis
  • -Diabetic glomerulo-nephropathy
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29
Q

Acute poststreptococcal glomerulonephritis general characteristics

A
  • -Occurs 1-4 weeks post-infection of pharynx or skin.
  • -Resolves spontaneously
  • -Type III hypersensitivity reaction
  • -All ages, most common in children
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30
Q

Acute poststreptococcal glomerulonephritis lab findings

A
  • -Decreased C3 serum levels
  • -Seum antistreptolysisin O, antiDNase B titers high
  • -Red cell casts
  • -Mild proteinuria
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31
Q

Acute poststreptococcal glomerulonephritis LM, IF, and EM findings

A
  • -LM: glomeruli enlarged and hypercellular w/proliferation of endothelial and mesangial cells. Infiltration of neutrophils and monocytes
  • -IF: Granular IgG, IgM
  • -EM: sub epithelial immune complex humps
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32
Q

What helps make diagnosis for acute poststreptococcal GN

A

EM

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33
Q

Clinical presentation for Acute poststreptococcal GN

A
  • –Nephritic
  • -“SMOKY URINE” hematuria
  • -Malaise
  • -Oliguria
  • -PERIORIBITAL EDEMA
  • -Mild HTN
  • -Adults may have atypical presentation
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34
Q

Rapidly progressive (crescentic) GN (RPGN) general info

A
  • -SEVERE injury to glomerulus
  • -Not caused by specific entity
  • -Poor prognosis
  • ->50% of glomeruli will have crescents
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35
Q

Crescents

A

Proliferations of parietal epithelial cells of Bowmans capsule mixed with inflammatory cells

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36
Q

Type I RPGN

A
  • -Anti-GBM GN

- -IF show LINEAR deposits of IgG ,C3, in GBM

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37
Q

Type II RPGN

A
  • -Immune complex mediated
  • -IF: GRANULAR pattern, “lumpy-bumpy)
  • -Seen with PSGN, SLE, IgA nephropathy
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38
Q

Type III RPGN

A
  • -Pauci-immune type
  • -LACK OF IF STAINING
  • -Most have P or C-ANCA
  • -Some cases due to vasculitis, most are idiopathic
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39
Q

RPGN gross, micro, EM findings

A
  • -Gross: large, pale kidneys w/petechiae
  • -Micro: crescent formation w/fibrin strands
  • -EM: ruptures in GBM +/- subepithelial deposits
40
Q

Nephrotic syndrome pathophysiology

A
  • -Increased permeability of GBM to plasma proteins
  • -Massive proteinuria
  • -Hypoalbuminemia
  • -Loss of colloid osmotic pressure and edema
  • -Increased liver synthesis of lipoproteins
  • -Infections due to loss of Ig and complement
  • -Hypercoaguable state due to loss of anticoags
41
Q

Number one cause of nephrotic syndrome in children

A

Minimal change disease (75% of cases)

42
Q

Most common causes of nephrotic syndrome in adults

A

Membranous and Focal segmental glomerulosclerosis

43
Q

Membranous GN general info

A

–Can be primary (idiopathic) or secondary to other conditions (DM, NSAIDs, HBV, HCV, SLE, solid tumors, etc.)

44
Q

Pathogenesis of membranous GN

A
  • -Chronic Ag-Ab mediated disease
  • -Antigens can be endogenous or exogenous
  • -Damage to GBM is COMPLEMENT MEDIATED
45
Q

What is often the autoantigen in membranous GN in adults?

A

Phospholipase A2

46
Q

Microscopic findings of membranous GN

A
  • -Normocellular gloms
  • -Uniform, diffuse, thickening of capillary wall (capillary loops look like cheerios)!!!
  • -“Spikes” on silver stain correspond to BM material between deposits
  • -Deposits become part of very thickened GBM
  • -Late mesangial sclerosis and glomerular hyalinization occur
47
Q

IF of membranous GN

A

Granular deposits along GBM–Deposits contain IgG and C3. Nephrotic presentation of SLE

48
Q

EM of membranous GN

A

–Subepithelial deposits
–“Spike and dome” appearance
Spikes of BM between deposits
–Thickened GBM w/lucent defects (deposits resorbed)
–Effacement of foot processes

49
Q

Course of membranous GN

A
  • -Chronic proteinuria and slow deterioration (40% chronic renal insufficiency, 10% die or have renal failure)
  • -Highly variable! Can’t predict who will do well
  • -Corticosteroids are +/-
  • -If secondary treat the cause
50
Q

Minimal change disease (Lipid nephrosis) (MCD) general info

A
  • -Most common cause of nephrotic syndrome in children (peak 2-6 yrs old)
  • -Associated w/atopy, may follow URI or routine immunization
  • -Increased incidence in Hodgkin lymphoma!
  • -RESPONDS TO CORTICOSTEROID TREATMENT
51
Q

Pathogenesis of MCD

A
  • -Visceral epithelial cell injury
  • -T cell dysfunction and release of cytokines
  • -May lead to loss of charge barrier or adhesion defects between epithelial cells
52
Q

Microscopic findings of MCD

A
  • -Normal glomeruli!!

- -Proximal tubules may be fill with lipid

53
Q

IF findings in MCD

A

No staining

54
Q

EM findings in MCD

A
  • -DIffuse effacement (cytokine mediated) of foot processes of visceral epithelial cells (podocytes)
  • -No deposits
55
Q

Clinical course of MCD

A
  • -Massive proteinuria (mostly albumin)
  • -No renal failure, often no HTN
  • ->90% of kids respond to corticosterioids
  • -Can recur
  • -Adults respond more slowly, still recover
  • -Damage to visceral epithelial cells reversed by steroid therapy
56
Q

Focal Segmental Glomerulosclerosis (FSGS) general info

A
  • -Sclerosis of some gloms in portion of glomerulus
  • -Associated with HIV, heroin addiction, sickle cell disease, morbid obesity
  • -Secondary change in area of previous necrotizing lesion
  • -Adaptive response to loss of renal tissue
  • -Currently most common cause of nephrotic syndrome in adults
57
Q

Pathogenesis of FSGS

A
  • -Thought to be related to MCD
  • -Damage to visceral epithelial cells
  • -Genetic abnormalities of proteins which localize to slit diaphragm and lead to FSGS
58
Q

LM findings of FSGS

A
  • -Make diagnosis this way
  • -Focal, need good biopsy
  • -Collapse of GBM, increased mesangial matrix, hyalinization, +/- foam cells
  • -Segmental sclerosis and hyalinization
59
Q

EM findings of FSGS

A
  • -Diffuse effacement of foot processes (similar to MCD)

- -Focal detachment of epithelial cells from GBM

60
Q

IF findings of FSGS

A

Mesangial deposits of IgM and C3 (in sclerotic area)

61
Q

Clinical course in FSGS

A
  • -Doesn’t respond well
  • -Nephrotic syndrome, HTN, reduced GFR
  • -Poor response to corticosteroids
  • -At least 50% have ESRD in 10 yrs
  • -RECURS POST-TRANSPLANTATION
  • -Worse prognosis with HIV nephropathy
62
Q

HIV nephropathy

A
  • -Most commonly FSGS, collapsing variant
  • -See cystically dilated tubules filled with proteinaceous material and inflammation
  • -EM: TUBULORETICULAR INCLUSIONS IN ENDOTHELIAL CELLS
  • -5-10% of HIV positive patients, more commonly in African-Americans
63
Q

Membranoproliferative GN (MPGN) general info

A
  • -5-10% of nephrotic syndrome in children and young adults.
  • -May present with nephritic syndrome
  • -Proliferation of glomerular cells, leukocyte infiltration, changes in GBM
64
Q

Associations w/MPGN

A
  • -Chronic immune complex disease (SLE, Hep B, Hep C, endocarditis, infected ventriculoatrial shunts)
  • -Partial lipodystrophy (type II)
  • -Alpha-1 antitrypsin deficiency
  • -Malignancy (CLL, lymphoma, melanoma)
65
Q

Pathology of MPGN

A

–Type 1 and 2 distinguished by IF, EM. Microscopic (1 and 2 similar).

66
Q

Microscopic findings of MPGN

A
  • -Large, hypercellular glomeruli w/lobular architecture (“flower-like”)
  • -Thickened GBM
  • -Silver stain show “tram track” or “double contour” from mesangial cell interposition into GBM
67
Q

Type I MPGN

A
  • -2/3 of cases
  • -IF: granular C3, IgG, C1q, C4
  • -SUBENDOTHELIAL DEPOSITS +/- subepithelial and mesangial deposits
68
Q

Pathogenesis of Type I MPGN

A
  • -Immune complex disease

- -Activation of classic and alternative complement pathways

69
Q

Type II MPGN or dense deposit disease

A

–IF: granular C3 only
EM: DENSE DEPOSIT DISEASE
Lamina densa RIBBON-LIKE and extremely dense from deposits
–Excess activation of alt complement pathway

70
Q

Clinical course of MPGN

A

50% develop chronic renal failure in 10 years

  • -steroids and immunosuppressive drugs not helpful
  • -COMMONLY RECURS POST-TRANSPLANT
71
Q

IgA nephropathy (Berger disease)

A
  • -Most common type of GN worldwide
  • -Causes recurrent hematuria w/RBC casts
  • -+/- proteinuria (can be in nephrotic range)
  • -HSP systemic disease w/overlapping features
  • -Associated w/celiac sprue, liver disease
72
Q

Pathogenesis of IgA nephropathy

A
  • -Plasma polymeric IgA increased
  • -IgA aberrantly glycosylated
  • -Immune complexes deposit or are formed in mesangium
73
Q

LM of IgA nephropathy

A

Mesangial proliferation

74
Q

IF of IgA nephropathy

A

Mesangial deposition of IgA!!!

+/- C3, properdin, IgG, IgM

75
Q

EM of IgA nephropathy

A

Paramesangial and mesangial immune complex deposits

76
Q

Clinical course of IgA nephropathy

A

–Hematuria following respiratory, GI, UT infection
–Variable course presentation
15-40% develop chronic RF over 20 years
–Disease recurs post-transplantation

77
Q

Types of hereditary nephritis

A
  • -Alport Syndrome

- -Thin membrane disease

78
Q

Alport syndrome presentation

A
  • -Nephritis, eye problems, sensorineural deafness
  • -Most cases X-linked dominant
  • -Males affected more frequently and severely
79
Q

Alport syndrome pathophysiology

A

–Mutation in type IV collagen–> thinning and splitting of GBM

80
Q

Alport syndrome microscopic findings

A
  • -Glomeruli w/segmental proliferation or sclerosis
  • -Mesangial matrix increases
  • -Persistance of fetal-like glomeruli
  • -Foam cells
81
Q

EM findings in Alport Syndrome

A
  • -Splitting of the lamina densa
  • -Changes are widespread
  • -Diagnosis made based on EM findings
82
Q

Clinical course of Alport Syndrome

A
  • -Present w/hematuria at 5-20 yrs
  • -+/- proteinuria (Can be nephrotic)
  • -Renal failure by 20-50
83
Q

Thin membrane disease (Benign familial hematuria)

A
  • -Fairly common
  • -Present w/hematuria
  • -EM shows diffuse thinning of GBM
  • -Abnormal genes encoding collagen chains
  • -Excellent prognosis but homozygous patients may progress to RF
84
Q

Chronic Glomerulonephritis

A
  • -End result of specific types of GN

- -Some present at end stage w/no antecedent disease

85
Q

Gross and micro findings of Chronic GN

A

Gross: small, diffusely granular kidneys
Micro: globally hyalinized glomeruli (hyaline material contains plasma proteins, mesangial matrix, GBM material, collagen) Atrophy and fibrosis of tubules/interstitium

86
Q

Progression to Chronic GN (in order of those most likely to progress)

A
  1. RPGN (90%)
  2. FSGS (50-80%)
  3. Membranous (50%)
  4. Membranoproliferative (50%)
  5. IgA nephropathy (30-50%)
  6. Poststreptococcal (1-2%)
87
Q

SLE general info

A
  • -Autoimmune disease

- -Affects skin, joints, kidney, serosal membranes

88
Q

Lupus nephritis

A
  • -60-70% involvement based on LM exam
  • -Nearly all SLE patients show kidney involvement on IF and EM eval
  • -5 patterns of involvement
89
Q

IF and EM findings in SLE nephritis

A

IF: “full house” stains with everything
EM: subendothelial and sometimes IgG-based immune complexes often with C3 deposition
LM: “WIRE LOOP” LESION (thickening of capillary wall by subendothelial deposits)

90
Q

HSP clinical presentation

A

Purpuric skin lesions on arms, legs and buttocks, abdominal pain, vomiting, bleeding, arthralgia, renal abnormalities (1/3 of patients) (hematuria, proteinuria/nephrotic syndrome, renal issues worse in adults, may have crescentic GN)

91
Q

Most common age of HSP

A

3-8 year old after URI

92
Q

HSP pathophysiology in kidneys

A

IgA deposition in mesangium

93
Q

Diabetic nephropathy general info

A
  • -ESRD in up to 40% of Type I and II DM
  • -Proteinuria in 50% (typically 12-22 yrs after diagnosis)
  • -Micro: capillary BM thickening, diffuse mesangial sclerosis, nodular glomerulosclerosiss
94
Q

Pathogenesis of diabetic nephropathy

A
  • -Metabolic defect: increases type IV collagen, fibronectin, nonenzymatic glycosylation of proteins, leads to thickened GBM, increased mesangial matrix
  • -Hemdynamic effects: increased GFR, glomerular hypertrophy early, leads to glomerulosclerosis
95
Q

Pathology of diabetic nephropathy

A
  • -Capillary basement membrane thickening (see on EM)
  • -Diffuse mesangial sclerosis
  • -Nodular glomerulosclerosis
96
Q

Nodular glomerulosclerosis in DM nephropathy

A

Kimmelstiel-Wilson disease

  • -Hyaline masses in periphery of glomerulus
  • -15-30% of DM patients, assoc. w/ RF
97
Q

Clinical course of diabetic nephropathy

A
  • -Microalbuminuria
  • -HTN
  • -Diabetic nephropathy
  • -ESRD
  • -Treatment options: long-term dialysis, renal transplant, pancreas transplant

CRRAB II (13 decks)

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