Renal Disorders II

Question 1

Features of nephritic syndrome

Answer 1

• 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

Question 2

Diseases that cause asymptomatic isolated hematuria

Answer 2

• IgA nephropathy

- alport syndrome

Question 3

IgA nephropathy facts

Answer 3

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

Question 4

Histologic feature of IgA nephropathy and immunofluorescence

Answer 4

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

Question 5

IgA nephropathy prognosis and treatment

Answer 5

• 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

Question 6

Henoch Schonlein Purpura (HSP)

Answer 6

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

Question 7

Kidney and Skin biopsy findings in HSP

Answer 7

• kidney: IgA in the mesangium

- skin: IgA in sub dermal vessels

Question 8

Alport syndrome facts

Answer 8

• 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

Question 9

Alport syndrome features

Answer 9

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

Question 10

Alport syndrome key lesions

Answer 10

• GBM: thickening, splitting, and lamination

- “basket-weave” pattern

Question 11

Isolated hematuria is caused by 2 diseases

Answer 11

• IgA nephropathy

- alport’s disease

Question 12

Acute nephritic syndrome caused by 2 conditions

Answer 12

1. crescentic glomerulonephritis

2. diffuse proliferative glomerulonephritis

Question 13

Crescentic glomerulonephritis facts

Answer 13

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

Question 14

Crescentic glomerulonephritis types

Answer 14

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

Question 15

Anti GBM disease immunofluorescence

Answer 15

• linear capillary loop IgG

Question 16

Anti GBM disease lab values

Answer 16

• elevated serum anti-GBM antibody

Question 17

Anti GBM disease causes

Answer 17

• Anti GBM disease, Goodpasture’s disease

Question 18

Immune complex type immunofluorescence

Answer 18

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

Question 19

Immune complex type lab values

Answer 19

• elevated serum anti nuclear antibodies, anti dsDNA

Question 20

Immune complex type causes

Answer 20

• lupus nephritis

- IgA nephropathy (only Henoch-Schonlein pupura)

Question 21

Pauci-immune type immunofluorescence

Answer 21

• negative for deposits

Question 22

Pauci-immune type lab values

Answer 22

• elevated serum anti neutrophil cytoplasmic antibodies (ANCA)

Question 23

Pauci-immune type causes

Answer 23

• ANCA associated diseases: wegener’s granulomatosis, microscopic polyarteritis

Question 24

Anti-GBM disease (goodpasture’s disease)

Answer 24

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

Question 25

Anti-GBM Disease treatment

Answer 25

- steroids, cytotoxic agents, PLASMAPHERESIS

Question 26

Pauci-immune crescentic GN

Answer 26

- 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

Question 27

Treatment of Crescentic GN

Answer 27

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

Question 28

Three causes of crescentic GN

Answer 28

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

Question 29

Diffuse proliferative GN (DPGN) causes

Answer 29

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

Question 30

Acute post streptococcal GN facts: who's affected

Answer 30

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

Question 31

Acute post-streptococcal GN: presentation

Answer 31

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

Question 32

Key features of acute post-streptococcal GN

Answer 32

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

Question 33

Proliferative lupus nephritis

Answer 33

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

Question 34

Proliferative lupus nephritis evidence

Answer 34

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

Question 35

Key features of lupus nephritis

Answer 35

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

Question 36

Three types of hypercellularity

Answer 36

- mesangial - endocapillary - crescents

Question 37

Causes of acute nephritic syndrome

Answer 37

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

Question 38

Types of nephritic syndrome

Answer 38

- isolated hematuria | - acute nephritic syndrome