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Renal > PATHOLOGY GLOMERULAR DISEASE > Flashcards

PATHOLOGY GLOMERULAR DISEASE Flashcards

1
Q

What are the features of a normal glomerulus?

What is :

V

T

I

C

M

U

on the picture

A
  1. The overall cellularity
  2. The symmetry (which of course is broken at the hilum if there is a section through it)
  3. The thickness of the capillary walls (arrow).
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2
Q

How is the glomerulus able to filter waste?

What are they regulated by?

A

1) size (regulated by the endothelial fenestrea/pores)
2) charge (i.e., anionic or cationic) by the delicate semi permeable basement membranes and the epithelial foot processes.

Any disturbances (directly or indirectly) in the endothelium, basement membrane, or foot processes can therefore result in abnormalities of proper filtration process.

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3
Q

How are renalbiopsies sually examined?

A

•Light microscopy
–H&E
–Special stains
•Immunofluorescence (IF)
•Electron microscopy (EM)
•
•

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4
Q

What are these stains?

What do they highlight?

A

PAS

Trichrome

H and E

Reticulin

The PAS stain accentuates matrix (m) constituents (e.g. proteins) in the mesangium or basement membrane (BM).

The trichrome stain highlights collagen fibers in blue or green colors .

The silver stain accentuates connective tissue (c.t.) reticulin fibers (e.g., in the mesangial matrix and the glomerular basement membrane).

the reticulin fibers of the capillary wall basement membranes are extremely thin, and uniformly delicate.

As you can see, in these images of normal glomeruli, there is very little matrix or collagen. And

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5
Q

What are the histologic alterations indicative of glomerulus injury?

A
  • Hypercellularity
  • Basement Membrane Thickness
  • Hyalinosis & Sclerosis
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6
Q

What are the immune mechanisms that underlie glommerlar disease?

A

glomerular deposition of immune complexes- formed by autoantibodies reactive with circulating antigens

Glomerulonephritis resulting from antibodies against the in situ glomerular basement membrane

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7
Q

How do nephritic syndromes presents?

A

–Hematuria/red blood cell casts
–Hypertension

Mild to moderate proteinuria

This is the classic prsentation of acute poststreptococcal glomerulonephritis

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8
Q

What are the presentations of nephrotic syndromes?

A

heavy proteinuria (more than 3.5 gm/day),

hypoalbuminemia,

severe edema,

hyperlipidemia,

lipiduria (lipid in the urine).

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9
Q

What are causes of Nephritic syndromes?

A

– 1˚: Acute proliferative GN & crescentic GN

2˚: SLE, microscopic polyangiitis, etc

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10
Q

What are causes of Nephrotic syndromes?

A

–

1˚: Membranous GN, minimal change disease, focal segmental glomerulosclerosis, & membranoproliferative GN
–

2˚: Diabetes, amyloidosis, SLE, drugs, CA, etc.

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11
Q

When do sympyoms appear from Poststinfectious (Proliferative) GN?

What cuases it?

A
  • Symptoms appear 1-4 weeks after an URI
  • Usually group A β-hemolytic strep
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12
Q

What is the pathophisiology of Poststinfectious (Proliferative) GN?

A

•Antigen (Ag)-antibody (Ab) complex:

antigen is usually streptococcal exotoxin B or enzymes released from the streptococcal surface. These may be circulating antigens or antigens planted in the glomerulus. The antibody is an IgG immunoglobulin. The immune complexes appear as in situ deposits in the glomerulus, capable of activating compliments..

–Patients may recover or progress to RPGN

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13
Q

What is this a picture of?

What is happening compared to normal?

A

Kidney, acute postinfectious (proliferative) glomerulonephritis:

the glomeruli of patients with proliferative glomerulonephritis are conspicuously hypercellular compared to normal.

many neutrophils in glomerular tufts resulting in the hypercellularity of the glomeruli.

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14
Q

What are the classification of Crescentic (or RP) GN?

A
  1. Type I RPGN (anti-GBM) has a linear IF pattern.
  2. Type II RPGN, caused by immune complexes, has a granular pattern, a complication of any of the immune complex nephritides
  3. Type III RPGN, pauci-immune type, lack anti-GBM or immune complexes, so IF is normal. This type of RPGN may be a component of a systemic vasculitis. In many cases, however, it is isolated and hence idiopathic.
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15
Q

What isRPGN (Crescentic GN), type I due too?

With what may it cross react?

What will this lead too?

What is a poor prognosis?

A
  • Antibody against type IV collagen in GBM
  • May cross-react with lung alveolar BM with pulmonary hemorrhage (Goodpasture syndrome)
  • Anti-GBM Ab results in BM breakage
  • Inflammatory cells/cytokines leak into Bowman space cause cell injury/crescent
  • >80% glomerular involvement = poor prognosis
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16
Q

What is this an image of?

What is inside the crescent?

A

The crescent (arrow) is composed of proliferated parietal epithelial cells and some mononuclear inflammatory cells.

The crescent is a result of severe glomerular injury

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17
Q

What is this a pic of?

What is it showing?

A

crescentic types of glomerulonephritis, the glomerular basement membrane is damaged, as shown here with broken ends of the basement membrane.

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18
Q

What is this image showing?

A

EM clearly shows broken ends of a basement membrane with no evidence of antibody deposition along the basement membrane.

These finding are not compatible with findings of lupus

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19
Q

What is Type II RPGN caused by?

What does the biopsy show?

A

•Complication of any of the immune complex nephritides
–Systemic lupus erythematosus,
–IgA nephropathy
–Henoch-Schönlein purpura
•idiopathic

Biopsy shows granular patterns

20
Q

What is TYPE lll RPGN associated with?

What does it show on IF?

What is treatment for these patients?

A

Lack anti-GBM or immune complexes

associated vasculitis so has circulating ANCA. and a necrosis with rupture of capillary loop.

They benefit fromplamapheresis

21
Q

What are the primary and secondary causes of nephrotic syndromes?

A

• 1˚:
–Membranous GN
–Minimal change disease
–Focal segmental glomerulosclerosis
–Membranoproliferative GN
•2˚:
–Diabetes
–Amyloidosis
–SLE
–Drugs, CA, etc.
–

22
Q

What are the characteristics of Membranous Glomerunephritis?

A
  • Mostly an indolent autoimmune GN
  • Usually with no antecedent illness
  • Nonselective proteinuria with intra-epithelial reabsorption droplets
  • May be primary or secondary (e.g. infections, malignancy, SLE, drugs)
  • Nonresponsive to steroids
  • May lead to glomerulosclerosis over time
23
Q

What is this imaging showing?

A

The capillary loops show subtle, uniform thickening with spikes (arrow).

The capillary loops are open (no inflammation),

the mesangial areas and cellularity are normal.

24
Q

What is the most common cause of nephrotic syndrome?

What does it become in adults?

What is the pathogenisis?

What is the prognosis?

What does it respond?

A
  • minimal change disease is the Most common cause of nephrotic syndrome in children
  • In adults it may be associated with Hodgins lymphomas/leukemias or NSAID therapy
  • Unknown pathogenesis, possibly immune-stimulated cytokine-release damage
  • Good prognosis, most respond to corticosteroids
25
Q

Which disease is this associated with?
What does this show?

A

Foam (lipoid) cells reflect tubular reabsorption of lipoproteins passing through diseased glomeruli.

The renal tubules of this patient with heavy proteinuria may show lipid-laden macrophages due to reabsorption of lipoproteins passing through the glomeruli.

26
Q

What is this image showing?

A

conspicuous fusion (effacement) of foot processes.

Normal foot processes separated by regular spaces are shown in the lower left inset..

Schematic diagram illustrates diffuse effacement of foot processes of podocytes (epithelial cells) with no immune deposits.

27
Q

Is focal segmental glomerulsclerosis primary or secondary?

What is it due too?

What are the mutations that cause it?

What is it responsive too?

A
  • May be 1˚or 2˚ to other conditions (HIV, heroin use, IgA nephropathy)
  • Due to podocyte-damaging cytokines disruption of visceral epithelial cells•Some are familial, linked to mutations in proteins forming the slit pores:

–NPHS1 (nephrin)

–NPHS2 (podocin)

•Nonresponsive to steroids, 50% progress

28
Q

What are the characteristics of Focal segmental glomerularsclerosis?

A

characterized by Sclerosis of some focal renal glomeruli, but not other glomeruli, and in the affected glomerulus, segmental involvement of only a portion of the capillary tuft identifies the glomerular involvement as well as the name as focal segmental.

29
Q

What are these images of?

A

The basic lesion in primary FSGS is disruption of visceral epithelial cells.

A.FSGS (periodic acid–Schiff stain): mass of scarred, obliterated capillary lumens with accumulations of matrix material has replaced a portion of the glomerulus.

B.Trichrome stain demonstrates blue collagen deposition.

C.IF is positive in the sclerotic focus with antibodies to IgM and C3 due to nonspecific entrapment of proteins rather than immune complexes. They result from hyperpermeability at sites of epithelial cell damage. IF is negative in the nonsclerotic part.

D.EM: effacement of foot processes (arrows), as in minimal-change disease (MCD).

30
Q

What are the types ofMembranoproliferative GN (MPGN)?

What is the prognosis

A

•Type I (80%) is due to:

–Ab against an unknown Ag forming immune complexes, in-situ or circulating, with complement activation

–May be 2˚ hep B, hep C, SLE, infections

•Type II (dense deposit disease) due to:

–Excessive complement activation

  • Auto Ab against C3 nephritic factor (NeF)
  • Mutations in, or autoAb against, Factor H
  • Poor prognosis: progress to renal failure
31
Q

What is this pathology?

A

a lobular hypercellular glomerulus with capillary wall double contours (periodic acid methenamine silver stains)

in MPGN type II

32
Q

What is the pathology of this disease?

A

EM: Discrete subendothelial electron-dense deposits

IF: Peripheral granular staining for C3, IgG, and early compliments.

33
Q

What are the isolated urinary abnormalities?

A
  • IgA nephropathy
  • Alport syndrome (hereditary nephritis)
34
Q

What does IgA nephropathy cause?

How is IgA caused?

What is IgA nephropathy associated with?

A
  • An important cause of recurrent hematuria in children and young adults.
  • May represent a hereditary defect in IgA production and clearance.
  • May be associated with celiac sprue, liver disease and occurrence within 1 or 2 days of a mucosal infection.
35
Q

What are the characteristics of this image?

A

No inflammation is visible.

There is mesangial widening which may be the result of cell proliferation, accumulation of matrix, immune deposits, or some combination of these abnormalities.

The glomeruli may be normal or may show mesangial widening and endocapillary proliferation (mesangioproliferative glomerulonephritis), segmental proliferation confined to some glomeruli (focal proliferative glomerulonephritis), or rarely, overt crescentic glomerulonephritis. The presence of leukocytes within glomerular capillaries is a variable feature.

36
Q

What are the characteristics of this image?

A

IgA synthesis is thought to be increased in response to mucosal infections. IgA forms immune complexes that deposit in the mesangium.

37
Q

What cause Hereditary nephtitis?

What is it associated with?

What are the characteristics?

A
  • EM shows characteristic abnormalities /thin BM
  • Hereditary mutations in GBM type IV collagen gene
  • Associated with nerve deafness and ocular disorders

Its a FSGS

38
Q

What is this image of?

A

hereditary nephritis

typical features of FSGS (i.e. involvement of some, but not all, glomeruli with segmental fibrosis).

39
Q
  • What is this image ilustrating?
A

consists of diffuse GBM thinning.

40
Q

What are the characteristics of Nodular Glomerulosclerosis?

A

•Kommetjie-Wilson lesion; Virtually diagnostic of DM

–In ~15% to 30% of long-term diabetics

–A major cause of morbidity and mortality

–First present with microalbuminuria(>30 mg/day, but <300 mg/day)

41
Q

What are the characteristics of this image?

A

positive nodules , situated in the periphery of the glomerulus

They lie within the mesangial core of the glomerular lobules surrounded by patent peripheral capillary loops.

capillary loops (“fibrin caps”) or adherent to Bowman’s capsules (“capsular drops”).

42
Q

what is amyloisosis associted with?

What can it cause?

A

Occurs in 6 to 15% of multiple myeloma
Always associated with free light-chain in urine
Can subsequently cause marked vascular narrowing

43
Q

What are the characteristics of the image?

A

amyloidosis

the deposits are primarily in the glomeruli, but the interstitial peritubular tissue, arteries, and arterioles are also affected.

Under polarized light the Congo red–stained amyloid shows so-called apple-green birefringence.

EM reveals amorphous nonoriented thin fibrils.

44
Q

What are the lupus nephritis classicification?

A 
–Minimal mesangial lupus nephritis (class I)
–Mesangial proliferative lupus nephritis (class II)
–Focal lupus nephritis (class III)
–Diffuse lupus nephritis (class IV)
–Membranous lupus nephritis (class V)
–Advanced sclerosing lupus nephritis (class VI)
45
Q

What are the patterns of glomulenephritis?

A

A. Lupus nephritis, focal proliferative type. There are two focal necrotizing lesions in the glomerulus (arrows).

B. Lupus nephritis, diffuse proliferative type. Note the marked increase in cellularity throughout the glomerulus.

C. Lupus nephritis. A glomerulus with several “wire loop” lesions representing extensive subendothelial deposits of immune complexes is seen. (Periodic acid-Schiff [PAS] stain.)

D. EM shows subendothelial dense deposits (*) corresponding to “wire loops” seen by light microscopy. Deposits are also present in the mesangium.

E. Immunofluorescence shows granular mesangial and capillary wall deposits of IgG (anti-DNA-DNA immune complexes).

46
Q

What are the characteristics of chronic glomerulanephritis?

A

Chronic glomerulonephritis. A Masson trichrome preparation shows complete replacement of virtually all glomeruli by blue-staining collagen.

Chronic glomerulonephritis is best considered a pool of end-stage glomerular disease fed by several streams of specific types of glomerulonephritis.

Renal (15 decks)

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