Clinical Management Of Nephrotic And nephritic Syndromes Flashcards Preview

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1

Nephritic vs nephrotic syndrome

Nephritic
- acute or chronic
- reversible
- inflammatory process with active urinary sediment
- 3 primary symptoms:
1) proteinuria of <2gm/dL
2) WBCs (neutrouria)
3) RBCs (hematuria)
** may show RBC casts but must have glomerular disease present also
- can show (+/-) HTN, edema, renal failure

Nephrotic
- acute or chronic
- noninflammatory proces
- 3 primary symptoms:
1) proteinuria of >3,5 bpm/dL
2) mass edema
3) Hypercholesterolemia with hypoalbuminemia
- (+/-) show oval fat bodies and HTN

2

What does the term “active urinary sediment” mean?

Urine contains
- proteinuria of <2gm/dL
- RBCs >10/HPF
- WBCs

Can contain casts also

**is seen in nephritic syndrome only**

3

Types of casts and associated pathology

Red cell casts : glomerulonephritis

White cell casts : pyelonephritis, interstitial nephritis

Waxy casts : advanced renal diseases

Hyaline casts : concentrated urine or use of diuretics only (may not be pathology)

Fatty casts = nephrotic syndrome

Granular Casts = renal failure (ATN if muddy brown)

Epithelial cell/renal tubular cell casts = ATN (acute tubular necrosis)

4

What does the term “bland urinary sediment mean”

Contains
- heavy proteinuria (>3.5)
- urine fat/oval fat bodies
- fatty/waxy cats
- renal tubular cells with lipid droplets
- NO inflammation


**seen in nephrotic syndrome**

5

3 broad catagories of glomerulonephritis

1) ANCA is present

2) anti-GBM antibodies are present

3) immune complexes are present

6

What are possible causes of glomerulonephritis with ANCA bodies present

ANCA-associated crescentic GN:
- if there is no extra-renal symptoms

Microscopic polyangitis:
- if systemic necrotizing symptoms are present

Granulomatosis polyangitis
- if respiratory symptoms with granulomas are present

Eosinophilic granulomatosis w/ polyangitis
- if asthma and eosinophila are present

7

What are possible causes of glomerulonephritis if anti-GBM antibodies are present?

Goodpasture syndrome
- if lung hemorrhages are present

Anti-GBM specific GN
- if no lung hemorrhage is present

8

What are possible causes of glomerulonephritis with immune complex markers present?

Lupus GN
- if ANAs are present and lupus systemic symptoms

Postinfectious GN
- if recent strep/staph infection and antipathogenic antibodies are present

IgA nephropathy
- if IgA complexes are present

Cryoglobulinemic GN
- if cryoglobulins are present

MPGN
- if complement is active

9

Post infectious Glomerulonephritis (PIGN)

A cause of nephritic syndrome w/ low complement and primarily kidney presentation only

Almost ONLY seen in recent staph/strep infections (1-3 weeks after)
- presents with ASO antibodies if strep

Urine shows hematuria and sub nephrotic proteinuria (<3.5)

Imaging shows granular deposition of IgG and C3 in GBM
- EM will show large dense endothelial deposits (humps)

10

Membranoproliferative glomerulonephritis (MPGN)

Rare pattern of glomerular injury and can be seen across the entire nephritic spectrum

Can be idiopathic or associated with chronic HCV or autoimmune infections/disorders

Type 1: MOST COMMON
- shows low C3/C4 levels in blood and urine
- all over the spectrum (can be asymptomatic hematuria to gross hematuria to extreme proteinuria)
- biopsy shows “tram-track” apperance with hypercellularity
- shows C3 deposition and immunoglobulin staining on IF stains

Type 2: “dense deposit disease
- shows low levels of C3 only in blood and urine
- is an inherited disease
- kidney EM biopsy shows ribbon-like electron dense deposits on the GBM

11

How do you treat type one MPGN?

Mild disease = ACE-I/ARB

Severe disease = cyclophosphamide or mycophenolate mofetil + steroids


**even with treatment, end stage renal disease (ESRD) is very likely**


***type 2 treatment is novel therapies and experiment processes, no first line***

12

IgA nephropathy (Berger disease)

Cause of nephritic syndrome with normal complement

*most common primary glomerular disease worldwide*

**classic presentation: acute viral illness with gross hematuria/proteinuria with “coca-cola” urine**

Commonly seen in females, children and Asians

Serum complement levels are NORMAL

Biopsy shows focal glomulonephritis w/ IgA and C3 depositions and proliferation

13

How to treat IgA nephropathy

Mild = just monitor
- NO HTN and within normal limits of GFR and minimal proteinuria

Severe: ACE-I/ARB to reduce HTN (125/75) and proteinuria (< 1g/d)
- HTN, reduced GFR and >1g/d proteinuria
- * if this doesn’t solve it, add corticosteroids

IgA + RPGN (“crescent nephropathy”)
- treat with corticosteroids and cyclophosphamide

IgA + ESRD = renal transplant

14

Alport syndrome (hereditary nephritis)

X-linked nephritic syndrome with normal complement levels

Presents with chronic glomerulonephritis w/ hearing and eye issues

*caused by defects in (a)-3/4 type 4 collagen subunits*

Kidney biopsy shows split lamina densa of the GBM (hallmark finding)

Treatment = ACE-I/ARB
- * if ESRD is present = renal transplant, however need to be careful with risk of developing anit-GBM disease against the normal type 4 collagen seen in the transplant kidney*

15

ANCA+ w/ renal-limited glomerulonephritis

Cause of nephritic syndrome w/ normal compliment levels

Presents idiopathic crescentic GN and is essentially RPGN w/:
- HTN
- nephritic urine
- ANCA antibodies present

Kidney biopsy shows NO immunoglobulin or compliment deposition

Treatment = cyclophosphamide and high dose corticosteroids

16

SLE

Is a cause of nephritic syndrome w/ low complement

Has 6 separate classes
- 1 ) normal mesangial
- 2) mesangial
- 3) focal proliferative
- 4) diffuse proliferative
- 5) membranous
- 6) ESRD w/ sclerosis

Treatment = aggressive high dose corticosteroids + cyclophosphamide or mycophenolate mofetil
- can also use rituximab in refractory cases

17

Cryoglobulinemia glomerulonephritis (MPGN)

Cause of nephritic syndrome w/ low complement and systemic presentations

Is a vasculitis with cold-precipitate immunoglobulins
- **most common underlying etiology = HCV infections

Can also be caused by underlying bacterial/viral/fungal pathogens, infective endocarditis, myeloma present

Shows high levels of rheumatoid factor, HCV antigens and polyclonal IgG complexes

18

Presentation and treatment of cryoglobulinemia glomerulonephritis

Purpura rash with necrotizing skin lesions w/ fever, arthralgia and hepatosplenomegaly

Low complement levels with high RF present

Kidney biopsy = various patterns

Treatment = aggressive treatment of underlying cause, high dose corticosteroids, hemodialysis
- can also use rituximab, cyclosporine or mycophenolate mofetil

19

What CANT you use for HCV-associated renal disease?

Ribavirin
- normally this can be used in HCV infections, however with renal disease, casues hemolysis and toxic effects so it is contraindicated if renal disease is also present

20

Goodpasture syndrome and anti-GBM glomerulonephritis

Both of the above show nephrotic syndrome w/ normal complement and primary systemic presentations

- Good pasture = has pulmonary hemorrhages
- anti-GBM disease = NO pulmonary hemorrhages

Bimodal peak with males in ages of 20-30 yrs and 60-70yrs

**can also show HLA-DR2 and B7 antigens but always NORMAL compliment

21

What is the presentation of goodpasture disease and anti-GBM disease

Shared:
- sometimes starts with a URI
- dyspnea
- glomerular hematuria
- proteinuria
- minimal renal dysfunction

Goodpasture = hemoptysis and infiltrates in lungs on CXR

Treatment for both = plasmapheresis and corticosteroids and cyclophosphamide

**monitor levels of anti-GBM to determine effectiveness**

22

Henoch-Schonlein purpura (HSP)

Presents mostly in children after a GAS infection
- affects skin/GI tract/joints and kidneys

Presentation:
- GI disturbances
- joint pains
- erythema
- purpura rash (especially on lower extremities)
- hematuria

**kidney biopsy looks identical to IgA nephropathy

23

What are the 3 major pathogenic causes of RPGN

1) anti-GBM disease/ good pasture disease

2) immune complex deposition

3) pauci-immune disorder

24

Nephrotic syndrome symptoms

Bland urinary sediment (no casts or very few)

Heavy proteinuria >3.5g

Hypoalbuminemia <3g/dL
- causes vit D, zinc and copper deficiencies also as a side effect

Peripheral edema (usually lower extremity)
- can shows dyspnea and SOB because of this

Hyperlipidemia (50% of patients)

Hypercoagulable state

Oval fat bodies in urine

**most common cause in US is diabetes mellitus**

25

Why is proteinuria so high in nephrotic syndrome?

Serious effacement of podocytes and alteration of all 3 layers negative charges in the GBM

26

What do oval fat bodies look like under microscopy?

Light microscopy = cluster of grapes

Polarized light = “Maltese cross”

27

Why does hyperlidemia occur in nephrotic syndrome?

Low oncotic pressure triggers lipid production of cholesterol and apolipoprotein B

Low oncotic pressure also decreases clearance of VLDL proteins

28

What are the relative contraindications for ACE-Is and ARBs?

Hyperkalemia

Serum creatinine rise of greater than 30%

**if either of these occurs stop use.**

29

why shouldnt you use a statin and gemfibrozil together?

Causes rhabdomyolysis

30

Minimal change disease

*most common nephrotic syndrome in children and presents in 10-15% of adults*

T-cell dysfunction leading to fusion in the podocytes of the glomerular capillary walls and a leaky GBM
- causes significant proteinuria

**high rates seen in lithium, heavy NSAID use and lymphomas**

Presentation:
- anasarca/severe peripheral edema
- NO HTN
- shows fatty casts in urine
- nephrotic proteinuria