Renal Disorders II Flashcards Preview

MODHIII - Unit 3 > Renal Disorders II > Flashcards

Flashcards in Renal Disorders II Deck (38)
1

Features of nephritic syndrome

- hematuria
- microscopic analysis of urine will show erythrocytes
- may also have kidney injury (increased creatinine and BUN) in which case this is call acute nephritic syndrome

2

Diseases that cause asymptomatic isolated hematuria

- IgA nephropathy
- alport syndrome

3

IgA nephropathy facts

- most common type of primary glomerulonephritis worldwide
- affects children and young adults
- quite common in young adults (non african americans)
- recurrent hematuria

4

Histologic feature of IgA nephropathy and immunofluorescence

- mesangial proliferation: mesangial hypercellularity of > 3 cells per mesangial region
- granular mesangial pattern of staining for IgA

5

IgA nephropathy prognosis and treatment

- most patients have good prognosis
- proteinuria is a predictor of progressive disease
- no specific treatment available: in progressive cases steroids and immunosuppressive agents may be tried

6

Henoch Schonlein Purpura (HSP)

- vasculitic variant of IgA nephropathy
- usually seen in CHILDREN
- features of vasculitis, arthritis, abdominal pain, rashes

7

Kidney and Skin biopsy findings in HSP

- kidney: IgA in the mesangium
- skin: IgA in sub dermal vessels

8

Alport syndrome facts

- defect in glomerular basement membrane due to mutation in alpha-5 chain of collagen type IV
- X linked dominant inheritance
- COL4A5 gene (Xq22)
- affects males, females are carriers

9

Alport syndrome features

- presents with microscopic hematuria
- sensorineural deafness
- progresses to chronic kidney disease
- proteinuria is a sign of progression

10

Alport syndrome key lesions

- GBM: thickening, splitting, and lamination
- "basket-weave" pattern

11

Isolated hematuria is caused by 2 diseases

- IgA nephropathy
- alport's disease

12

Acute nephritic syndrome caused by 2 conditions

1. crescentic glomerulonephritis
2. diffuse proliferative glomerulonephritis

13

Crescentic glomerulonephritis facts

- glomerular crescents are formed
- cause severe glomerular injury
- severe kidney injury and death can result if untreated

14

Crescentic glomerulonephritis types

Type 1: Anti GBM disease
Type 2: Immune complex type
Type 3: Pauci-immune type

15

Anti GBM disease immunofluorescence

- linear capillary loop IgG

16

Anti GBM disease lab values

- elevated serum anti-GBM antibody

17

Anti GBM disease causes

- Anti GBM disease, Goodpasture's disease

18

Immune complex type immunofluorescence

- granular deposits (IgG/IgA) in mesangium or capillary loops

19

Immune complex type lab values

- elevated serum anti nuclear antibodies, anti dsDNA

20

Immune complex type causes

- lupus nephritis
- IgA nephropathy (only Henoch-Schonlein pupura)

21

Pauci-immune type immunofluorescence

- negative for deposits

22

Pauci-immune type lab values

- elevated serum anti neutrophil cytoplasmic antibodies (ANCA)

23

Pauci-immune type causes

- ANCA associated diseases: wegener's granulomatosis, microscopic polyarteritis

24

Anti-GBM disease (goodpasture's disease)

- autoimmune disease: abnormal production of antibodies directed against collagen IV, which constitutes the basement membranes of glomerulus and lung

25

Anti-GBM Disease treatment

- steroids, cytotoxic agents, PLASMAPHERESIS

26

Pauci-immune crescentic GN

- group of vasculitic disorders affecting small vessels
- can cause pulmonary renal syndrome
- associated with anti-neutrophil cytoplasmic antibody (ANCA)********
- classic example of wegener's granulomatosis

27

Treatment of Crescentic GN

- immunosuppressive therapy: corticosteroids
- cytotoxic agents: cyclophosphamide
***in addition to above anti-GBM needs plasmapheresis***

28

Three causes of crescentic GN

1. anti GBM disease
2. immune complex GM
3. pauci-immune disorders

29

Diffuse proliferative GN (DPGN) causes

- acute post streptococcal glomerulonephritis
- proliferative (active) lupus nephritis

30

Acute post streptococcal GN facts: who's affected

- affects children
- history of respiratory infection 2 weeks prior
- only certain "nephritogenic" strains of B-hemolytic streptococci cause this

31

Acute post-streptococcal GN: presentation

- acute nephritic syndrome
- kidney injury
- Hypertension
- Low Complement 3 levels
- ASO titer serially elevated

32

Key features of acute post-streptococcal GN

- diffuse endocapillary proliferative GN with subepithelial "humps"
- IgG and C3 deposits by immunofluorescence

33

Proliferative lupus nephritis

- acute nephritic syndrome
- systemic symptoms of active lupus: arthritis, rashes

34

Proliferative lupus nephritis evidence

- serology: anti nuclear antibody, anti-dsDNA+, low complements C3 and C4

35

Key features of lupus nephritis

- endocapillary proliferation, crescents, necrosis
- FULL house pattern by immunofluorescence
- subendothelial deposits

36

Three types of hypercellularity

- mesangial
- endocapillary
- crescents

37

Causes of acute nephritic syndrome

1. crescentic GN (3 types)
2. diffuse proliferative GN (2 types)

38

Types of nephritic syndrome

- isolated hematuria
- acute nephritic syndrome