SM 209 Nephritis

Question 1

What are the 3 Nephritic syndromes?

Answer 1

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

Question 2

What is the key feature of a Nephritic syndrome?

Answer 2

Hematuria, both micro or macro

Question 3

What other features besides Hematuria are found in Nephritic syndromes?

Answer 3

Oliguria, HTN, Azotemia

Question 4

Why is HTN a response in Nephritic syndromes?

Answer 4

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

Question 5

What is the common histomorphographic feature of Nephritic syndromes?

Answer 5

Proliferation of cells in the glomerulus and damage to the GBM

Question 6

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

Answer 6

The urine environment is electrolytically harsh and causes lysis

Question 7

How many types of Rapidly Progressive Glomerulonephritis are there?

Answer 7

3: Type I, II, and III

Question 8

Who gets affected by PSGN?

Answer 8

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

Question 9

What does PSGN urine look like?

Answer 9

Cola-colored urine

Question 10

How does PSGN effect serum complement levels?

Answer 10

Low serum complement

Question 11

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

Answer 11

Post Streptococcal Glomerulonephritis

Question 12

How does PSGN present on fluorescent microscopy?

Answer 12

Starry-Sky with IgG and Complement

Question 13

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

Answer 13

PSGN

Question 14

What disease presents with subepithelial humps on electron microscopy?

Answer 14

PSGN

Question 15

How does PSGN appear on electron microscopy?

Answer 15

Subepithelial humps

Question 16

Who gets RPGN?

Answer 16

Adults

Question 17

How does RPGN present?

Answer 17

Rapid loss of renal function that needs dialysis

Question 18

Is RPGN a disease?

Answer 18

RPGN is a clinical syndrome

Question 19

What is seen on slides with RPGN?

Answer 19

Lots of Crescents

Question 20

What causes anti-GBM disease?

Answer 20

An antibody against Collagen Type IV

Question 21

What does RPGN look like on EM?

Answer 21

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

Question 22

How does RPGN Type I appear on FM?

Answer 22

Linear IgG deposits +/- C3

Question 23

What is Goodpasture syndrome?

Answer 23

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

Question 24

How does Goodpasture syndrome present?

Answer 24

Hematuria and Hemoptysis

RPGN Type I

Question 25

Which type of RPGN causes Goodpasture syndrome?

Answer 25

Type I RPGN

Question 26

What is RPGN Type II?

Answer 26

An immune complex disease

Question 27

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

Answer 27

RPGN Type II

Question 28

How is RPGN Type II described on EM?

Answer 28

Lumpy Bumpy appearance

Question 29

What disease appears as Lumpy Bumpy on EM?

Answer 29

RPGN Type II

Question 30

What types of immune deposits are in RPGN Type II?

Answer 30

C3 + IgG or IgA

Question 31

Which type of RPGN is Pauci-immune?

Answer 31

RPGN Type III

Question 32

What is the most common type of RPGN?

Answer 32

RPGN Type III

Question 33

What type of RPGN has ANCA's?

Answer 33

ANCA's = anti-neutrophil cytoplasmic antibodies RPGN Type III

Question 34

What is RPGN Type III?

Answer 34

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

Question 35

What are the subtypes of RPGN Type III?

Answer 35

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

Question 36

What is MPA?

Answer 36

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

Question 37

What is GPA?

Answer 37

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

Question 38

What is EGPA?

Answer 38

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

Question 39

In which disease are ANCA seen?

Answer 39

ANCA are seen in vasculitis

Question 40

What type of vasculitis is RPGN Type III?

Answer 40

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

Question 41

Who is affected by IgA Nephropathy?

Answer 41

Kids and young adults

Question 42

How does IgA Nephropathy present?

Answer 42

Recurrent episodes of Hematuria, typically beginning after an infection

Question 43

What characterizes IgA deposits in IgA Nephropathy?

Answer 43

IgA deposits at the GBM

Question 44

How do serum IgA levels change in IgA Nephropathy?

Answer 44

Serum IgA levels rise

Question 45

How does IgA in IgA Nephropathy damage the kidney?

Answer 45

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

Question 46

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

Answer 46

The Tree of the Glomerulus expands

Question 47

What is Systemic Lupus Erythematous?

Answer 47

An autoimmune disease of unknown etiology

Question 48

Which organ systems does SLE effect?

Answer 48

Inflammation of multiple organ systems is characteristic of SLE

Question 49

How is Lupus Nephritis classified?

Answer 49

It is classified by the ISN system into 6 classes

Question 50

What sets the treatment of Lupus Nephritis?

Answer 50

The class of the Lupus Nephritis sets the treatment

Question 51

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

Answer 51

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

Question 52

How does Lupus Nephritis present on fluorescent microscopy?

Answer 52

A "full house" of IgG, IgM, IgA, C3, C1q

Question 53

What are active lesions in Lupus Nephritis?

Answer 53

Active lesions are hypercellularity and endocapillary fibrin deposits

Question 54

What are chronic changes in Lupus Nephritis?

Answer 54

Old injures that lead to scarring and crescents

Question 55

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

Answer 55

Class V Lupus Nephritis

Question 56

Which class of Lupus Nephritis is chronic?

Answer 56

Class VI Lupus Nephritis

Question 57

What is Amyloidosis?

Answer 57

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

Question 58

What is Amyloid?

Answer 58

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

Question 59

How does deposition of Amyloid effect normal physiology?

Answer 59

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

Question 60

How is Amyloid classified?

Answer 60

Amyloid is classified by protein type

Question 61

What is AA Amyloid?

Answer 61

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

Question 62

What is AL Amyloid?

Answer 62

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

Question 63

What is AH Amyloid?

Answer 63

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

Question 64

What is Ab2M Amyloid?

Answer 64

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

Question 65

What is ATTR Amyloid?

Answer 65

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

Question 66

Which type of Amyloid proteins are associated with Lymphoid neoplasms?

Answer 66

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

Question 67

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

Answer 67

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

Question 68

How is Amyloid stained?

Answer 68

Apple-green appearance on Congo Rod stain