SM 209 Nephritis Flashcards

1
Q

What are the 3 Nephritic syndromes?

A

Acute Post Streptococcal Glomerulonephritis (PSGN)
Rapidly Progressive Glomerulonephritis (RPGN)
IgA Nephropathy

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2
Q

What is the key feature of a Nephritic syndrome?

A

Hematuria, both micro or macro

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3
Q

What other features besides Hematuria are found in Nephritic syndromes?

A

Oliguria, HTN, Azotemia

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4
Q

Why is HTN a response in Nephritic syndromes?

A

Kidney senses poor blood flow due to inflammation in the Glomerulus, activating RAAS to compensate and resulting in HTN

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5
Q

What is the common histomorphographic feature of Nephritic syndromes?

A

Proliferation of cells in the glomerulus and damage to the GBM

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6
Q

Why are RBC’s in the urine in Nephritic syndromes damaged?

A

The urine environment is electrolytically harsh and causes lysis

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7
Q

How many types of Rapidly Progressive Glomerulonephritis are there?

A

3: Type I, II, and III

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8
Q

Who gets affected by PSGN?

A

Kids 2 weeks after a Group A Beta-hemolytic Strep infection

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9
Q

What does PSGN urine look like?

A

Cola-colored urine

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10
Q

How does PSGN effect serum complement levels?

A

Low serum complement

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11
Q

A child presents with cola-colored urine and low serum complement levels. Diagnosis?

A

Post Streptococcal Glomerulonephritis

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12
Q

How does PSGN present on fluorescent microscopy?

A

Starry-Sky with IgG and Complement

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13
Q

What disease presents with IgG and Complement in a starry-sky pattern on fluorescent microscopy?

A

PSGN

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14
Q

What disease presents with subepithelial humps on electron microscopy?

A

PSGN

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15
Q

How does PSGN appear on electron microscopy?

A

Subepithelial humps

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16
Q

Who gets RPGN?

A

Adults

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17
Q

How does RPGN present?

A

Rapid loss of renal function that needs dialysis

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18
Q

Is RPGN a disease?

A

RPGN is a clinical syndrome

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19
Q

What is seen on slides with RPGN?

A

Lots of Crescents

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20
Q

What causes anti-GBM disease?

A

An antibody against Collagen Type IV

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21
Q

What does RPGN look like on EM?

A

No distinct deposits because the antibody is evenly distributed throughout the GBM

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22
Q

How does RPGN Type I appear on FM?

A

Linear IgG deposits +/- C3

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23
Q

What is Goodpasture syndrome?

A

IgG in RPGN Type I binds to both the glomerular basement membrane as well as the pulmonary basement membrane

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24
Q

How does Goodpasture syndrome present?

A

Hematuria and Hemoptysis

RPGN Type I

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25
Q

Which type of RPGN causes Goodpasture syndrome?

A

Type I RPGN

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26
Q

What is RPGN Type II?

A

An immune complex disease

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27
Q

Which type of RPGN arises as a complication of autoimmune disorders that make immune complexes?

A

RPGN Type II

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28
Q

How is RPGN Type II described on EM?

A

Lumpy Bumpy appearance

29
Q

What disease appears as Lumpy Bumpy on EM?

A

RPGN Type II

30
Q

What types of immune deposits are in RPGN Type II?

A

C3 + IgG or IgA

31
Q

Which type of RPGN is Pauci-immune?

A

RPGN Type III

32
Q

What is the most common type of RPGN?

A

RPGN Type III

33
Q

What type of RPGN has ANCA’s?

A

ANCA’s = anti-neutrophil cytoplasmic antibodies

RPGN Type III

34
Q

What is RPGN Type III?

A

A type of RPGN without deposits on EM and negative on FM

35
Q

What are the subtypes of RPGN Type III?

A

MPA - Vasculitis w/ Neutrophils
GPA - Vasculitis w/ Neutrophils + Granulomas
EGPA - Vasculitis w/ Neutrophils + Granulomas + Eosinophilia

36
Q

What is MPA?

A

A subtype of RPGN Type III that presents as Vasculitis w/ Neutrophils

37
Q

What is GPA?

A

A subtype of RPGN Type III that presents as Vasculitis w/ Neutrophils + Granulomas

38
Q

What is EGPA?

A

A subtype of RPGN Type III that presents as Vasculitis w/ Neutrophils + Granulomas + Eosinophilia

39
Q

In which disease are ANCA seen?

A

ANCA are seen in vasculitis

40
Q

What type of vasculitis is RPGN Type III?

A

RPGN is a necrotizing vasculitis that involves a Glomeruli that involves fibrinoid rings forming around blood vessels

41
Q

Who is affected by IgA Nephropathy?

A

Kids and young adults

42
Q

How does IgA Nephropathy present?

A

Recurrent episodes of Hematuria, typically beginning after an infection

43
Q

What characterizes IgA deposits in IgA Nephropathy?

A

IgA deposits at the GBM

44
Q

How do serum IgA levels change in IgA Nephropathy?

A

Serum IgA levels rise

45
Q

How does IgA in IgA Nephropathy damage the kidney?

A

IgA lodges in the Kidney and activate complement, damaging the GBM and causing RBC leakage into urine

46
Q

How does IgA Nephropathy affect the appearance of glomerulus on slides?

A

The Tree of the Glomerulus expands

47
Q

What is Systemic Lupus Erythematous?

A

An autoimmune disease of unknown etiology

48
Q

Which organ systems does SLE effect?

A

Inflammation of multiple organ systems is characteristic of SLE

49
Q

How is Lupus Nephritis classified?

A

It is classified by the ISN system into 6 classes

50
Q

What sets the treatment of Lupus Nephritis?

A

The class of the Lupus Nephritis sets the treatment

51
Q

How do the changes caused by Lupus Nephritis effect severity and treatment?

A

At higher classes, Lupus has more drastic changes which have more severe symptoms and need more aggressive treatment

52
Q

How does Lupus Nephritis present on fluorescent microscopy?

A

A “full house” of IgG, IgM, IgA, C3, C1q

53
Q

What are active lesions in Lupus Nephritis?

A

Active lesions are hypercellularity and endocapillary fibrin deposits

54
Q

What are chronic changes in Lupus Nephritis?

A

Old injures that lead to scarring and crescents

55
Q

Which stage of Lupus cause “spike and dome” appearance on EM?

A

Class V Lupus Nephritis

56
Q

Which class of Lupus Nephritis is chronic?

A

Class VI Lupus Nephritis

57
Q

What is Amyloidosis?

A

A clinical syndrome that occurs due to deposition of Amyloid in various organ systems

58
Q

What is Amyloid?

A

Deposits of proteins that are capable of forming beta-pleated sheet polymers that resist degradation

59
Q

How does deposition of Amyloid effect normal physiology?

A

Deposition of Amyloid leads to interference with the normal function of the organ

60
Q

How is Amyloid classified?

A

Amyloid is classified by protein type

61
Q

What is AA Amyloid?

A

Amyloid of Serum Amyloid A; occurs in chronic inflammation both infectious and autoimmune

62
Q

What is AL Amyloid?

A

Amyloid of light chains (from IgG) that occurs in Lymphoid neoplasms

63
Q

What is AH Amyloid?

A

Amyloid of heavy chains (from IgG) that occurs in Lymphoid neoplasms

64
Q

What is Ab2M Amyloid?

A

Amyloid of beta2-microglobulin, seen in chronic hemodialysis patients because it’s not filtered

65
Q

What is ATTR Amyloid?

A

Amyloid of Transthyretin, seen in elderly or familial with a propensity for the heart

66
Q

Which type of Amyloid proteins are associated with Lymphoid neoplasms?

A

AH and AL are associated with Lymphoid neoplasms (both are part of the Ig)

67
Q

Which type of Amyloid proteins are associated with Lupus and Hep B/C?

A

Lupus and Hep B/C are sources of chronic inflammation which predispose AA

68
Q

How is Amyloid stained?

A

Apple-green appearance on Congo Rod stain