Renal Pathology Pt. 2 Flashcards

1
Q

what are the causes of nephritic syndrome?

A

post-streptococcal/post-infectious glomerulonephritis (acute proliferative)

rapidly progressive (crescentic) glomerulonephritis

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2
Q

what is acute proliferative glomerulonephritis?

A

diffuse proliferation of glomerular cells associated with influx of leukocytes caused by immune complexes

*most common is post-strep

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3
Q

what are the pathologic characterizations of acute proliferative glomerulonephritis?

A

hypercellularity
leukocyte infiltration
granular deposits of IgG, IgM and C3
subepithelial “humps” of immune complexes

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4
Q

what is the clinical presentation of post-strep glomerulonephritis in children?

A

malaise, fever, nausea, oliguria and hematuria 1-2 weeks after recovery from a sore throat

dysmorphic RBC or RBC casts with mild proteinuria

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5
Q

what is rapidly progressive glomerulonephritis?

A

syndrome associated with severe glomerular injury - typically immunologically mediated

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6
Q

what are the classifications of RPGN?

A

Type 1 - anti-GBM antibody
Type 2 - immune complex
Type 3 - ANCA +/Pauci immune

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7
Q

what is Goodpasture syndrome?

A

Type 1 RPGN
anti-basement membrane antibody (IgG) deposition in kidneys and lungs
manifests as pulmonary and renal hemorrhage

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8
Q

what HLA subtype is associated with Goodpasture?

A

HLA-DRB1

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9
Q

How is Goodpasture treated?

A

plasmaphoresis

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10
Q

what diseases are associated with Type 2 RPGN?

A

acute proliferative glomerulonephritis
SLE
IgA nephropathy
Henoch-Schonlein purpura

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11
Q

what is the definition of Type 3/Pauci immune RPGN?

A

lack of detectable anti-GBM antibodies or immune complexes by immunofluorescence or electron microscopy

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12
Q

what is typically found on serology with Type 3 RPGN?

A

p-ANCA or c-ANCA

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13
Q

what disorders are associated with type 3 RPGN?

A

vasculitides such as Wegeners or polyangiitis

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14
Q

what are the causes of nephrotic syndrome?

A
Membranous nephropathy
Minimal change disease
Focal segmental glomerulosclerosis
Membranoproliferative glomerulonephritis
IgA nephropathy
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15
Q

what is membranous nephropathy?

A

diffuse thickening of the glomerular capillary wall due to the accumulation of IgG deposits along the basement membrane

can be primary (idiopathic) or secondary

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16
Q

what is the pathogenesis of primary membranous nephropathy?

A

autoimmune disease linked to PLA-2 Ab

17
Q

what are common causes of secondary membranous nephropathy?

A
drugs (NSAIDs)
malignancy
SLE
infections 
other autoimmune disorders (thyroiditis)
18
Q

what are the histological hallmarks of membranous nephropathy?

A

effaced foot processes (podocytes)
thick basement membrane
subepithelial IgG deposits

19
Q

what is minimal change disease?

A

podocytopathy

relatively benign disorder characterized by effacement of podocytes detectable only by electron microscopy

most frequent cause of nephrotic syndrome in children

20
Q

what is the peak incidence of minimal change disease?

A

2-6 y/o

21
Q

what features of minimal change disease indicate its immunologic basis?

A
  1. immune dysfunction often following respiratory infection or routine prophylactic immunization
  2. responsive to steroids/immunosuppressives
  3. association with other atopic disorders
  4. increased incidence with certain HLA haplotypes
  5. increased incidence in patients with Hodgkin lymphoma
22
Q

what is focal segmental glomerulosclerosis?

A

podocytopathy

sclerosis of some but not all glomeruli with only a portion of the capillary tuft involved

most common cause of nephrotic syndrome in adults

23
Q

what are the classifications of FSGS?

A
primary/idiopathic *most common*
in association with other conditions
secondary event due to antecedent glomerular injury
adaptive response to loss of renal mass
hereditary
24
Q

how does FSGS differ from MCD?

A
  1. higher incidence of hematuria, reduced GFR and HTN
  2. proteinuria tends to be non-selective
  3. poor response to steroids
  4. significant progression to CKD
25
Q

what is termed the “collapsing variant” FSGS?

A

HIV associated FSGS

26
Q

what gene variation is associated with FSGS and renal failure in patients of African descent?

A

APOL1 on Chr. 22

27
Q

what is membranoproliferative glomerulonephritis?

A

considered a pattern of immune-mediated injury to the mesangial cells or capillary loops

28
Q

what are the classes of MPGN?

A

Type 1 - IgG and complement deposition

Type 2 - complement deposition and activation

29
Q

what are the histologic findings of MPGN?

A

alterations in the glomerular basement membrane
proliferation of glomerular cells
leukocyte infiltration

30
Q

what is the clinical presentation of primary MPGN Type 1?

A

typically occurs in children or young adults
nephrotic syndrome with nephritic features
mild HTN
50% of patients develop CKD

31
Q

what is the clinical presentation of secondary MPGN Type 1?

A

occurs exclusively in adults
chronic antigenemia causing immune complex deposition
associated with other disease or malignancy
50% of patients develop CKD

32
Q

what is the clinical presentation of MPGN Type 2/Dense Deposit Disease?

A

generally occurs in adults
hematuria
50% have nephritic syndrome
poorer prognosis than MGPN Type 1

33
Q

what is the pathogenesis of MPGN type 2?

A

C3NeF or “nephritic factor” IgG autoantibody binds C3 convertase leading to continuous activation of the alternative complement pathway

34
Q

what two diseases often manifest with IgA nephropathy?

A

Berger disease

Henoch-Scholein purpura

35
Q

what are the clinical characteristics of IgA nephropathy?

A

older children and young adults most common
Caucasian and Asian
male > female
genetic susceptibility
hematuria with the presence of significant infection (URI/respiratory/GI/abscess)
association with gluten enteropathy and liver disease

36
Q

what is chronic glomerulonephritis?

A

end-stage glomerular disease may occur with or without an antecedent history of acute glomerulonephritis

37
Q

what primary glomerular diseases are most likely to progress to chronic glomerulonephritis?

A
crescentic GN (90%)
focal segmental GS (50-80%)
membranoproliferative GN (50%)
membranous nephropathy (30-50%)
IgA nephropathy (30-50%)