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Block 4: SD3 Week 11 > Glomerular Disease 4 > Flashcards

Glomerular Disease 4 Flashcards

1
Q

What age group typically has post infectious nephritis/post-streptococcal glomerulonephritis?

A

5-15 years

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2
Q

When does the GN associated with post-streptococcal infection usually occur?

A

1-4 weeks after streptococcal infection of the pharynx or skin

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3
Q

What type of HSR type is post-streptococcal GN?

A

type 3 HSR

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4
Q

What infection usually leads to post-streptococcal GN?

A

Group A B hemolytic strep infection or skin infection

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5
Q

What is the latent period before onset of GN causing PSGN?

A

2-4 weeks latent activity

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6
Q

Describe immune complex patter on IF of one with PSGN?

A

lumpy-bumpy pattern

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7
Q

Is characteristic deposit of complex in PSGN subepithelial or sub endothelial?

A

supithelial

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8
Q

What are the CF hinting you to believe that a child has PSGN?

A

Children
-preceding history of sore throat (1-2 weeks)
-acute onset of malaise, fever, oliguria, periorbital edema
-smoky or coca-cola colored urine
-HTN mild to moderate

Nephritic presentation remember

Adults

Atypical features
Acute onset of HTN, edema, azotemia

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9
Q

What may you see upon microscopy of one with PSGN?

A

In severe cases - epithelial crescent +ve

Hypercellularity and neutrophilic infiltration

Increased cells within the glomerulus
Obliterated capillaries- end epithelial proliferation

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10
Q

Describe characteristic EM of one with PSGN.

A

sub epithelial deposits commonly seen as humps

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11
Q

Upon IF of one with PSGN what can be seen?

A

IgG and C3 deposits along BM (sub epithelial) and mesangium

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12
Q

What are some characteristic lab findings for one with PSGN?

A

Urine- creanated cells: red cell casts (possible cola colored urine)
ASO elevation (anti strep ab titre)
C3 levels decreased (for 1st 2 weeks
BUN may be elevated

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13
Q

Prognosis of PSGN in kids?

A

good

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14
Q

Prognosis of PSGN in adults?

A

relatively bad compared to having the condition in kids

40% progress to RPGN or chronic GN

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15
Q

What are some bacterial infections that can lead to Postinfectious GN?

A

staphylococcal endocarditis, pneumococcal pneumonia and meningococcemia

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16
Q

What are some viral diseases that can lead to non streptococcal acute GN?

A

Hep B, Hep C, mumps, HIV infection, varicella and infectious mononucleosis

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17
Q

What are some parasitic infections that can lead to post infectious GN?

A

malaria, toxoplasmosis

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18
Q

Are histology and EM for those with Nonstreptococcal acute GN the same as those with PSGN?

A

yes

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19
Q

What is the most common type of GN “worldwide”?

A

IgA nephropathy

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20
Q

In Berger’s Disease IgA typically deposits in what area of the glomerulus?

A

mesangial regions

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21
Q

Can diagnosis of IgA be made on LM?

A

no

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22
Q

A Disease that presents with nephritic presentation that is a frequent cause of recurrent gross or microscopic hematuria?

A

Berger’s Disease

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23
Q

What conditions will Berger’s Disease recur in?

A

transplanted kidney

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24
Q

What are other primary GN which will recur in transplanted kidneys?

A

FSGS, IgA nephropathy, MPGN type 2

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25
Q

Explain pathogenesis behind Berger’s Disease>

A

Genetic or acquire abnormality of immune regulation > Increased mucosal IgA synthesis ( in response to respiratory (pharyngitis) or GI exposure (acute gastroenteritis) to environmental agents) e.g viruses, bacteria(URI), food proteins)

IgA1 immune complexes trapped in mesangium activate alternative complement pathway and initiate glomerular injury

26
Q

What is a GI disease that IgA nephropathy commonly presents with?

A

gluten enteropathy (celiac disease)

27
Q

What is a condition that IgA nephropathy has some common presentation with?

A

HSP

28
Q

Disease of what organ can bring about secondary IgA nephropathy?

A

liver disease

29
Q

Histology of glomeruli in one with Berger’s disease may have what characteristics?

A

normal

mesangioproliferative glomerulonephritis

focal proliferative glomerulonephritis

rarely crescentic GN

30
Q

What conditions is there an increased risk of progression of Berger’s disease?

A

Onset in old age, heavy proteinuria, HTN, and extent of glomerulosclerosis on biopsy

31
Q

Features of HSP.

A

Purpuric skin lesions that can involve arms but mostly involves buttocks and legs

abdominal pain and vomiting

Intestinal bleeding

renal abnormalities

preceding viral infection (URI, pharyngitis)

non migratory arthralgia

IgA nephropathy

32
Q

Can a presentation of HSP in adults include gluten sensitive enteropathy and dermatitis herpetiformis?

A

yes

33
Q

T/F. IgA nephropathy and Henoch-Schonlein pursuer are spectra of the same disease.

A

True

34
Q

What is a nephritic syndrome characterized by rapid and progressive losss of renal function?

A

RPGN

35
Q

Prognosis of RPGN?

A

death from renal failure within weeks month is untreated

36
Q

Histology of RPGN.

A

crescentic GN

Diffuse crescents formed by proliferation of parietal cells, monocytes and lymphocytes

Fibrin contribute to crescent formation

37
Q

What is the best treatment for RPGN?

A

early diagnosis and use of cytotoxic drugs to disrupt crescents are the key to halt disease progression

38
Q

Describe gross morphology characteristic of crescentic GN.

A

enlarged, pale kidneys with petechial hemorrhages on cortical surface

39
Q

How does crescentic GN look upon LM?

A

proliferation of parietal epithelial cells admixed with macrophages and fibers filling Bowman’s/urinary space

40
Q

What stain shows locations of crescents in crescentic GN?

A

PAS stain shows location of crescent

41
Q

Be able to classify different causes for Crescentic GN based on if. Create a slide.

A
42
Q

Linear deposits along the GBM are characteristic to what nephritic syndrome?

A

anti GBM/ Good Pasture’s

43
Q

What type of deposits are characteristic of Pauci Immune?

A

no deposits

44
Q

Post infectious causes of crescentic GN have what pattern?

A

granular

45
Q

C/F of Goodpasture’s Syndrome.

A

hemoptysis and RPGN

46
Q

Treatment for Goodpastures?

A

plasmapheresis and immunosupression

47
Q

HLA DRB1 is associated with what nephritis syndrome?

A

Goodpastures ag

48
Q

Can good pastures syndrome develop in a patient with Alport’s?

A
49
Q

RPGN Immune Complex Deposition that has granular deposits on IF are due to what complications?

A

complication of post infectious, IgA nephropathy, MPGN, SLE

50
Q

What are the Labs to check for SLE?

A

ANA/dsDNA+ve

51
Q

Prescription for RPGN?

A

treat underlying condition

52
Q

Does Wegner’s granulamatosis have a +ve or Negative IF?

A

negative

53
Q

LM of kidney in one with Wegner’s?

A

neurosis of glomeruli

54
Q

Labs to check of Wegner’s Granulomatosis?

A

C-ANCA +ve

55
Q

Clinical features of Wegner’s granulomatosis?

A

saddle nose; cavitary lung lesion
-hemoptysis
- necrotizing granulomas

56
Q

A person with C-ANCA +ve labs will have ab directed agains what?

A

proteinase 3 of neutorophils

57
Q

What type of string pattern is characteristic of C ANCA?

A

cytoplasmic

58
Q

Ab are directed agains what in p-ANCA +ve labs?

A

myeloperoxidase of neutrophils

59
Q

p-ANCA + conditions?

A

microscopic polyangitis, Churg Strauss, PAN

60
Q

What is the pathology triggering crescent formation in RPGN?

A

disruption of BM

fibrin leaks into Bowman’s space triggering crescent formation

61
Q

Be able to reproduce Chart on comparison and Contrast between Alport’s Syndrome and Thin BM disease.

A
62
Q

What are some characteristic features upon EM of Alpert’s?

A

thickening alternating with attenuation (thinning)
pronounced BM splitting (black thin arrows)

Block 4: SD3 Week 11 (13 decks)

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