Nephritic Syndrome Flashcards Preview

Pathoma - Kidney and Urinary Tract Pathology > Nephritic Syndrome > Flashcards

Flashcards in Nephritic Syndrome Deck (55):
1

What characterizes glomerular disorders in nephritic syndrome?

1. Glomerular inflammation
2. Bleeding

2

What four other symptoms are seen in Nephritic Syndrome?

1. Limited proteinuria (

3

What does salt retention cause in Nephritic Syndrome?

Periorbital edema and hypertension

4

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

Glomerular Bleeding

5

What does biopsy reveal in Nephritic Syndrome?

Hypercellular, inflammed glomeruli

6

What are RBC casts a sign of?

Glomerular bleeding

7

What causes the hypercellularity seen in Nephritic Syndrome?

1. Immune-complex deposition activates complement
2. C5 attracts neutrophils which mediate damage --> hypercellularity and inflammation

8

What is Post-Streptococcal Glomerulonephritis (PSGN)?

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

9

What two things may cause PSGN?

1. Occurs with nephritogenic strains of strep.
2. May occur after infection with non-streptococcal organisms as well

10

What are nephritogenic strains of strep?

They carry M protein virulence factor!!

11

When does PSGN present?

2-3 weeks after infection

12

What signs and symptoms does PSGN present with?

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

13

What populations is PSGN usually seen in?

Children, but it may occur in adults

14

What do you see on H&E with PSGN?

Hypercellular, inflamed glomeruli

15

What mediates PSGN? What do you see on IF?

Mediated by immune complex deposition (granular IF)

16

What do you see on EM of PSGN?

Subepithelial 'humps'

17

What is the treatment for PSGN?

Supportive

18

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

-Children rarely progress to renal failure (1%)
-Some adults (25%) develop rapidly progressive glomerulonephritis (RPGN)

19

What is Rapidly Progressive Glomerulonephritis (RPGN)?

Nephritic syndrome that progresses to renal failure in weeks to months

20

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

1. Biopsy
2. IF to determine etiology

21

What are the subepithelial 'humps' seen in PSGN?

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

22

What is seen on H&E stain in RPGN?

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

23

What are crescents comprised of?

Fibrin and Macrophages

24

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

Clinical picture + IF

25

What nephrotic syndrome is SLE associated with?

Membranous Nephropathy

26

What nephritic syndrome is SLE associated with?

RPGN

27

What is a p-ANCA?

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

28

What is a c-ANCA?

Cytoplasmic - when antibodies bind in the cytoplasm

29

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

Goodpasture Syndrome

30

What is the pathogenesis of Goodpasture syndrome?

Antibody against collagen in glomerular and alveolar basement membranes

31

What does Goodpasture syndrome present with?

Hematuria and hemoptysis

32

What population is Goodpasture syndrome most common in?

Young, adult males

33

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

PSGN (most common)
OR
Diffuse proliferative glomerulonephritis

34

What causes diffuse proliferative glomerulonephritis?

Diffuse antigen-antibody complex deposition, usually sub-endothelial

35

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

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

36

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

-Wegener granulomatosis
-Microscopic polyangiitis
-Churg-Strauss Syndrome

37

What test finding is Wegener granulomatosis associated with?

+ c-ANCA

38

What systems does Wegener granulomatosis effect?

-Lung
-Kidney
-Nasopharyngeal

39

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

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

40

What conditions are associated with + p-ANCA?

-Microscopic Polyangiitis
-Churg-Strauss Syndrome

41

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

1. Granulomatous inflammation
2. Eosinophilia
3. Asthma

42

What is the most common nephropathy worldwide?

IgA Nephropathy (Berger Disease)

43

What causes IgA Nephropathy (Berger disease)?

IgA immune complex deposition in mesangium of glomeruli

44

Where does IgA deposit in IgA Nephropathy?

Mesangium of glomeruli

45

When do patients usually present with IgA Nephropathy?

Childhood

46

What signs and symptoms does IgA Nephropathy usually present with?

-Episodic gross or microscopic hematuria
-RBC casts

47

What does IgA Nephropathy usually follow?

Mucosal Infections (e.g. gastroenteritis)

48

What is the pattern of disease in IgA Nephropathy?

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

49

Production of what is increased during infection?

IgA production

50

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

IgA immune complex deposition in the mesangium

51

What may IgA Nephropathy slowly progress to?

Renal Failure!

52

What is Alport syndrome?

Inherited defect in type IV collagen.

53

What is the inheritance associated with Alport syndrome?

Most commonly X-linked

54

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

Thinning and splitting or the glomerular basement membrane

55

What does a patient with Alport syndrome present with?

-Isolated hematuria
-Sensory hearing loss
-Ocular disturbances