Nephritic Syndrome

Question 1

What characterizes glomerular disorders in nephritic syndrome?

Answer 1

1. Glomerular inflammation

2. Bleeding

Question 2

What four other symptoms are seen in Nephritic Syndrome?

Answer 2

1. Limited proteinuria (

Question 3

What does salt retention cause in Nephritic Syndrome?

Answer 3

Periorbital edema and hypertension

Question 4

What causes the red blood cell casts seen in Nephritic Syndrome?

Answer 4

Glomerular Bleeding

Question 5

What does biopsy reveal in Nephritic Syndrome?

Answer 5

Hypercellular, inflammed glomeruli

Question 6

What are RBC casts a sign of?

Answer 6

Glomerular bleeding

Question 7

What causes the hypercellularity seen in Nephritic Syndrome?

Answer 7

1. Immune-complex deposition activates complement

2. C5 attracts neutrophils which mediate damage –> hypercellularity and inflammation

Question 8

What is Post-Streptococcal Glomerulonephritis (PSGN)?

Answer 8

Nephritic syndrome that arises after group A beta hemolytic streptococcal infection of the (1) skin (impetigo) or (2) pharynx

Question 9

What two things may cause PSGN?

Answer 9

1. Occurs with nephritogenic strains of strep.

2. May occur after infection with non-streptococcal organisms as well

Question 10

What are nephritogenic strains of strep?

Answer 10

They carry M protein virulence factor!!

Question 11

When does PSGN present?

Answer 11

2-3 weeks after infection

Question 12

What signs and symptoms does PSGN present with?

Answer 12

• Hematuria (cola-colored urine)
• Oliguria
• Hypertension
• Periorbital edema

Question 13

What populations is PSGN usually seen in?

Answer 13

Children, but it may occur in adults

Question 14

What do you see on H&E with PSGN?

Answer 14

Hypercellular, inflamed glomeruli

Question 15

What mediates PSGN? What do you see on IF?

Answer 15

Mediated by immune complex deposition (granular IF)

Question 16

What do you see on EM of PSGN?

Answer 16

Subepithelial ‘humps’

Question 17

What is the treatment for PSGN?

Answer 17

Supportive

Question 18

How many adults and children with PSGN progress to renal failure?

Answer 18

• Children rarely progress to renal failure (1%)

- Some adults (25%) develop rapidly progressive glomerulonephritis (RPGN)

Question 19

What is Rapidly Progressive Glomerulonephritis (RPGN)?

Answer 19

Nephritic syndrome that progresses to renal failure in weeks to months

Question 20

What is the first and second thing you do when working up RPGN?

Answer 20

1. Biopsy

2. IF to determine etiology

Question 21

What are the subepithelial ‘humps’ seen in PSGN?

Answer 21

Immune complexes sitting on the basement membrane –> where subepithelial cells would normally sit

Question 22

What is seen on H&E stain in RPGN?

Answer 22

RPGN is characterized by crescents in Bowman’s space (of glomeruli)

Question 23

What are crescents comprised of?

Answer 23

Fibrin and Macrophages

Question 24

What should you use to help figure out the etiology (cause) of RPGN?

Answer 24

Clinical picture + IF

Question 25

What nephrotic syndrome is SLE associated with?

Answer 25

Membranous Nephropathy

Question 26

What nephritic syndrome is SLE associated with?

Answer 26

RPGN

Question 27

What is a p-ANCA?

Answer 27

Perinuclear - when antibodies from patient serum bind adjacent to the nucleus

Question 28

What is a c-ANCA?

Answer 28

Cytoplasmic - when antibodies bind in the cytoplasm

Question 29

What disease is indicated by a Linear (anti-basement membrane antibody) IF?

Answer 29

Goodpasture Syndrome

Question 30

What is the pathogenesis of Goodpasture syndrome?

Answer 30

Antibody against collagen in glomerular and alveolar basement membranes

Question 31

What does Goodpasture syndrome present with?

Answer 31

Hematuria and hemoptysis

Question 32

What population is Goodpasture syndrome most common in?

Answer 32

Young, adult males

Question 33

What diseases are indicated by Granular (immune complex deposition) IF?

Answer 33

PSGN (most common) OR Diffuse proliferative glomerulonephritis

Question 34

What causes diffuse proliferative glomerulonephritis?

Answer 34

Diffuse antigen-antibody complex deposition, usually sub-endothelial

Question 35

What is Diffuse proliferative glomerulonephritis (form of RPGN) common in?

Answer 35

Most common type of renal disease in SLE | [And renal disease is the most common cause of death in SLE]

Question 36

What diseases are indicated by Negative IF (pauci-immune)?

Answer 36

- Wegener granulomatosis - Microscopic polyangiitis - Churg-Strauss Syndrome

Question 37

What test finding is Wegener granulomatosis associated with?

Answer 37

+ c-ANCA

Question 38

What systems does Wegener granulomatosis effect?

Answer 38

- Lung - Kidney - Nasopharyngeal

Question 39

What if a patient presents with RPGN and recurrent sinus infections?

Answer 39

Think about Wegener granulomatosis (c-ANCA can be used to separate it from Goodpasture syndrome)

Question 40

What conditions are associated with + p-ANCA?

Answer 40

- Microscopic Polyangiitis | - Churg-Strauss Syndrome

Question 41

What three things distinguish Churg-Strauss Syndrome from Microscopic Polyangiitis?

Answer 41

1. Granulomatous inflammation 2. Eosinophilia 3. Asthma

Question 42

What is the most common nephropathy worldwide?

Answer 42

IgA Nephropathy (Berger Disease)

Question 43

What causes IgA Nephropathy (Berger disease)?

Answer 43

IgA immune complex deposition in mesangium of glomeruli

Question 44

Where does IgA deposit in IgA Nephropathy?

Answer 44

Mesangium of glomeruli

Question 45

When do patients usually present with IgA Nephropathy?

Answer 45

Childhood

Question 46

What signs and symptoms does IgA Nephropathy usually present with?

Answer 46

- Episodic gross or microscopic hematuria | - RBC casts

Question 47

What does IgA Nephropathy usually follow?

Answer 47

Mucosal Infections (e.g. gastroenteritis)

Question 48

What is the pattern of disease in IgA Nephropathy?

Answer 48

Relapsing and remitting (symptomatic and asymptomatic) and may lead to renal failure

Question 49

Production of what is increased during infection?

Answer 49

IgA production

Question 50

What is seen on IF in IgA Nephropathy (Berger Disease)?

Answer 50

IgA immune complex deposition in the mesangium

Question 51

What may IgA Nephropathy slowly progress to?

Answer 51

Renal Failure!

Question 52

What is Alport syndrome?

Answer 52

Inherited defect in type IV collagen.

Question 53

What is the inheritance associated with Alport syndrome?

Answer 53

Most commonly X-linked

Question 54

What does the defect in collagen in Alport syndrome result in?

Answer 54

Thinning and splitting or the glomerular basement membrane

Question 55

What does a patient with Alport syndrome present with?

Answer 55

- Isolated hematuria - Sensory hearing loss - Ocular disturbances