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Path Exam 4 > Kidney II: Glomerular Diseases > Flashcards

Kidney II: Glomerular Diseases Flashcards

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1
Q

What is the glomerulus?

A

A network of capillaries supported by mesangial cells and matrix.

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2
Q

What highlights the glomerular basement membrane (GBM) in light microscopy?

A

PAS and silver stains.

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3
Q

What are the main cell types present in the glomerulus?

A
  • Endothelial cells
  • Mesangial cells
  • Podocytes
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4
Q

What is the composition of the glomerular basement membrane (GBM)?

A

Type IV collagen.

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5
Q

What is the function of podocytes in the glomerulus?

A

They have foot processes that interdigitate to form filtration slits.

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6
Q

What are the components of the filtration barrier in the glomerulus?

A
  • Fenestrated endothelium
  • Glomerular basement membrane (GBM)
  • Slit diaphragm
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7
Q

What is the permeability of the filtration barrier?

A

Permeable to water, small solutes, and small proteins; impermeable to larger molecules like albumin.

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8
Q

What is the primary cause of most glomerular diseases?

A

Immune mechanisms, primarily involving immune complex formation.

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9
Q

What is in situ immune complex formation?

A

Antibodies bind to antigens that are either planted in the glomerulus or are intrinsic glomerular antigens.

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10
Q

What happens during circulating immune complex deposition?

A

Circulating antigen-antibody complexes are trapped in the glomerulus, activating complement and recruiting inflammatory cells.

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11
Q

What is characteristic of Goodpasture syndrome?

A

Antibodies target the GBM antigens, leading to linear deposition of immune complexes along the GBM.

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12
Q

What does the granular pattern in immunofluorescence indicate?

A

Scattered, granular deposits along the GBM or within the mesangium.

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13
Q

Which diseases commonly show a granular pattern?

A
  • Lupus nephritis
  • Post-infectious glomerulonephritis
  • Membranous nephropathy
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14
Q

What is the defining feature of the linear pattern in immunofluorescence?

A

Continuous, linear staining along the GBM.

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15
Q

What is nephritic syndrome?

A

A clinical syndrome characterized by inflammation of the glomeruli, leading to hematuria and proteinuria.

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16
Q

What are the major clinical signs of nephritic syndrome?

A
  • Hematuria
  • Proteinuria
  • Oliguria
  • Azotemia
  • Hypertension
  • Edema
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17
Q

What are the features of nephrotic syndrome?

A
  • Massive proteinuria (>3.5 g/day)
  • Hypoalbuminemia
  • Generalized edema
  • Hyperlipidemia
  • Lipiduria
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18
Q

What is the definition of diffuse histologic changes in the glomerulus?

A

Involves all glomeruli in the kidney.

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19
Q

What does focal histologic change refer to?

A

Involves some, but not all, glomeruli.

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20
Q

What is the concept of ‘Progression of Injury’ in renal disease?

A

Once GFR is reduced to less than 30-50% of normal, progression to ESRD occurs at a steady rate.

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21
Q

What is the major morphologic change in focal segmental glomerulosclerosis (FSGS)?

A

Hyperfiltration and compensatory hypertrophy resulting in segmental sclerosis.

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22
Q

What are the histologic changes in Minimal Change Disease (MCD)?

A
  • Normal glomeruli on LM
  • Negative IF
  • Podocyte effacement on EM
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23
Q

What is the typical light microscopy finding in acute proliferative glomerulonephritis?

A

Enlarged, hypercellular glomeruli.

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24
Q

What is the defining feature of rapidly progressive glomerulonephritis (RPGN)?

A

Crescents formed by proliferating parietal epithelial cells and monocytes in Bowman’s space.

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25
What does IgA nephropathy (Berger disease) show on immunofluorescence?
Mesangial deposition of IgA immune complexes.
26
What is the definition of chronic glomerulonephritis?
Progressive and irreversible glomerular and tubulointerstitial fibrosis leading to CKD and ESRD.
27
What is the light microscopy finding in membranous nephropathy?
Diffuse thickening of the capillary wall with normal cellularity.
28
What is the characteristic finding in dense deposit disease?
Dense, homogeneous, ribbon-like intramembranous deposits.
29
What is the main feature of Alport syndrome observed under electron microscopy?
Alternating thickening, thinning, and splitting of the GBM.
30
What is the clinical setting for acute proliferative glomerulonephritis?
Typically occurs in children aged 6-10 years, following a Group A Streptococcal infection.
31
What does the 'tram track' appearance indicate in MPGN Type I?
New GBM synthesis leading to a lobular appearance.
32
What are the defining features of nephritic syndrome?
* Hematuria * Proteinuria * Oliguria * Azotemia * Hypertension * Edema
33
What is the relationship between nephron destruction and GFR in renal disease?
Destruction leads to reduced GFR, progressing to ESRD independent of the original cause.
34
What is azotemia?
A condition characterized by elevated blood urea nitrogen (BUN) and creatinine levels.
35
What are the major diseases associated with nephritic syndrome?
* Acute Proliferative Glomerulonephritis * Rapidly Progressive Glomerulonephritis
36
What are the morphologic features of Acute Proliferative Glomerulonephritis?
* Hypercellular glomeruli * Granular IF deposits * Subepithelial 'humps' on EM
37
What are the morphologic features of Rapidly Progressive Glomerulonephritis?
* Crescents on LM * Linear or granular IF patterns * GBM ruptures and crescents on EM
38
Define nephrotic syndrome.
A clinical syndrome characterized by significant proteinuria, hypoalbuminemia, hyperlipidemia, and edema.
39
What is the threshold for proteinuria in nephrotic syndrome?
>3.5 grams per day.
40
What is considered hypoalbuminemia?
Serum albumin <3 grams/dL.
41
What are the clinical features of nephrotic syndrome?
* Proteinuria * Hypoalbuminemia * Edema * Hyperlipidemia * Lipiduria
42
What is the most common primary glomerular disease in children presenting with nephrotic syndrome?
Minimal Change Disease (MCD).
43
What are the morphologic features of Minimal Change Disease (MCD)?
* Normal glomeruli on LM * Negative IF * Uniform and diffuse effacement of podocyte foot processes on EM
44
What is the most common primary glomerular disease in adults presenting with nephrotic syndrome?
Focal Segmental Glomerulosclerosis (FSGS).
45
What are the clinical features of Focal Segmental Glomerulosclerosis (FSGS)?
* Proteinuria (often non-selective) * Hypertension * Microscopic hematuria * Mild azotemia
46
What are the morphologic features of Focal Segmental Glomerulosclerosis (FSGS)?
* Focal and segmental sclerosis on LM * Granular deposits of IgM and C3 in the mesangium on IF * Podocyte effacement and segmental collapse of capillary loops on EM
47
What is Membranous Nephropathy characterized by?
* Insidious onset of nephrotic syndrome * Non-selective proteinuria * Mild hypertension
48
What are the morphologic features of Membranous Nephropathy?
* Diffuse thickening of the capillary wall on LM * Granular deposits of IgG and C3 along the GBM on IF * Subepithelial deposits with 'spikes' of GBM on EM
49
What is Membranoproliferative Glomerulonephritis (MPGN)?
A disease that can present with nephrotic or combined nephrotic-nephritic syndrome.
50
What are the morphologic features of Membranoproliferative Glomerulonephritis (MPGN)?
* Large, hypercellular glomeruli with a lobular appearance on LM * Granular deposits of IgG and C3 on IF * Subendothelial immune complex deposits on EM
51
What are the defining features of Alport Syndrome?
* Hematuria * Proteinuria * Progressive renal failure * Extrarenal manifestations like nerve deafness and eye disorders
52
What are the morphologic features of Alport Syndrome?
* Alternating thickening, thinning, and splitting of the GBM on EM * Progression to FSGS on LM
53
What is Thin Basement Membrane Disease (TBMD) also known as?
Benign Familial Hematuria.
54
What are the clinical features of Thin Basement Membrane Disease (TBMD)?
* Familial asymptomatic hematuria * Proteinuria and hypertension usually absent
55
What are the gross features of Chronic Glomerulonephritis (GN)?
* Symmetrically contracted kidneys * Diffusely granular cortical surfaces * Thinned cortex
56
What are the microscopic features of Chronic GN?
* Hyaline obliteration of glomeruli * Tubular atrophy * Interstitial fibrosis * Lymphocytic infiltrate present
57
What is the leading cause of renal failure in the US?
Diabetic Nephropathy.
58
What are the notable morphologic changes in the glomeruli of diabetics?
* GBM thickening * Diffuse mesangial sclerosis * Nodular glomerulosclerosis (Kimmelstiel-Wilson Lesions)
59
What is a key clinical feature of IgA Nephropathy (Berger Disease)?
Recurrent hematuria, often following mucosal infections.
60
What is the pathogenesis of IgA Nephropathy?
* Aberrant glycosylation of IgA1 * Formation of autoantibodies against galactose-deficient IgA1 * Immune complex deposition in the glomerular mesangium
61
What is the most common and severe form of lupus nephritis?
Class IV: Diffuse Lupus Nephritis.
62
What are the electron microscopy features of Class IV Lupus Nephritis?
Extensive subendothelial immune complex deposits.
63
What is the typical demographic for Goodpasture Syndrome?
Most common in young adult males, particularly smokers.
64
What are the renal symptoms of Goodpasture Syndrome?
* Hematuria * Rapidly progressive glomerulonephritis (RPGN) * Severe oliguria
65
What is a key histological feature associated with lupus nephritis?
The 'wire loop' appearance ## Footnote This appearance is identified on light microscopy.
66
What demographics are most commonly affected by Goodpasture syndrome?
Young adult males, particularly smokers ## Footnote This syndrome is more prevalent in this demographic.
67
What pulmonary symptom is associated with Goodpasture syndrome?
Hemoptysis (coughing up blood) ## Footnote This occurs due to pulmonary intra-alveolar hemorrhages.
68
What renal symptoms are typically seen in Goodpasture syndrome?
Hematuria and rapidly progressive glomerulonephritis (RPGN) ## Footnote RPGN leads to severe oliguria and renal failure.
69
What is the defining finding on renal biopsy for Goodpasture syndrome?
Linear deposition of IgG along the glomerular basement membrane (GBM) ## Footnote This is observed through immunofluorescence microscopy.
70
What light microscopy finding indicates severe glomerular injury in Goodpasture syndrome?
Crescent formation in the glomeruli ## Footnote This finding is crucial for diagnosis.
71
What distinguishes the linear IF pattern in Goodpasture syndrome?
Smooth, continuous linear staining of IgG along the GBM ## Footnote This pattern is distinct from the granular pattern seen in other glomerulonephritides.
72
What are the characteristics of Minimal Change Disease (MCD)?
* Most common in children * Light Microscopy: Normal * IF: Negative * EM: Diffuse effacement of podocyte foot processes ## Footnote MCD is a type of nephrotic syndrome.
73
What defines Focal Segmental Glomerulosclerosis (FSGS)?
* Most common in adults * Light Microscopy: Focal and segmental sclerosis * IF: Granular IgM and C3 in sclerotic segments * EM: Diffuse effacement of podocyte foot processes ## Footnote FSGS is a type of nephrotic syndrome.
74
What are the key features of Membranous Nephropathy?
* Common in older adults * Light Microscopy: Diffuse thickening of capillary walls * IF: Granular IgG and C3 along GBM * EM: Subepithelial deposits with 'spikes' of GBM ## Footnote This condition is also a variant of nephrotic syndrome.
75
What describes Membranoproliferative Glomerulonephritis (MPGN) Type I?
* Light Microscopy: Large, hypercellular glomeruli with lobular appearance, 'tram tracks' * IF: Granular IgG and C3 * EM: Subendothelial deposits ## Footnote MPGN Type I is characterized by these findings.
76
What are the characteristics of MPGN Type II (Dense Deposit Disease)?
* Light Microscopy: 'Tram tracks', variable proliferative changes * IF: Bright C3 granular, no IgG * EM: Dense, ribbon-like intramembranous deposits ## Footnote This disease is a specific subtype of MPGN.
77
What are the findings in Acute Proliferative (Poststreptococcal/Postinfectious) GN?
* Light Microscopy: Enlarged, hypercellular glomeruli * IF: Granular deposits of IgG, IgM, and C3 * EM: Subepithelial 'humps' ## Footnote This condition is a form of nephritic syndrome.
78
What defines Rapidly Progressive (Crescentic) GN (RPGN)?
* Light Microscopy: Crescents in glomeruli * IF: Linear (Goodpasture's) or granular (immune complex diseases) * EM: Ruptures in GBM, crescents ## Footnote RPGN is characterized by these findings.
79
What is the definition of Transient Proteinuria?
Associated with hemodynamic changes, lasts 24-48 hours ## Footnote Protein excretion is < 1 gm/day.
80
What characterizes Orthostatic (Postural) Proteinuria?
Increased protein excretion < 2 gm/day in upright position; normal in recumbent position ## Footnote This condition is often benign.
81
What is crucial for diagnosing and differentiating between different types of nephrotic and nephritic syndromes?
Key features of various glomerular diseases, including their common patient demographics, light microscopy, immunofluorescence, and electron microscopy findings ## Footnote This information aids in understanding benign causes of proteinuria.

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