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Nephrotic Syndrome Flashcards

1
Q

What is the nephrotic syndrome?

A

Glomerular disease associated with heavy albuminurea (>3-3.5 g/day)

Also:
Hypoalbunimemia

Edema: periorbital and pedal

Hyperlipidemia

Thrombotic tendency

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2
Q

How does hypoalbuminemia develop in patients with nephrotic syndrome?

A

Glomerular disease –>

(1) proteinurea
(2) increased albumin catabolism bc it’s filtered, reabsorbed by the filters, and catabolized

Both lead to –> hypoalbumemia

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3
Q

What is the pattern of edema found in pt’s with nephrotic syndrome? Why do they develop characteristic pattern of edema?

A

Periorbital edema in the morning: bc they can sleep on their back unlike in CHF or ascites

Rings don’t fit their fingers

Pedal edema during the day

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4
Q

What is the pathogenesis of nephrotic edema?

A

Hypoalbuminema –> low oncotic pressure

Na and water retention –> high hydrostatic pressure

So there’s more pressure dirving it out of cells and less oncotic pressure pulling it into the cells

Note that there are 2 theories about the salt retention:

(1) primary renal salt retention: bc during glomerular dz, the kidney becomes a salt retaining organ immediately
(2) secondary renal sodium retention due to the loss of plasma oncotic pressure

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5
Q

What is the best treatment of edema in nephrotic syndrome?

A

Low Na diet

Oral loop diuretics: start with low dose, then double doses

IV diuretics, colloid rarely needed

Goal is 1-2# edema loss/day

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6
Q

Why do you get high lipids/cholesterol in nephrotic syndrome?

A

Glomerulus leaks protein –> low albumin –> liver perceives this & increases its production of albumin as well as other things i.e. lipids, cholesterol

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7
Q

What are maltese crosses? Where and why do you find them?

A

Cholesterol esters that under polarized light look like this

They escaped filtration barrier and come out in the urine

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8
Q

How do you treat hyperlipidemia in nephrotic syndrome?

A

Select high risk pt (high LDL, low HDL)

Atteempt to induce a remission o fthe proteinurea (ACEI, ARBs, specific immunosuppressors…)

Dietary therapy

Medical therapy (statins+)

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9
Q

What are the 3 main things you want to address in your treatment of nephrotic syndrome?

A

(1) Treat the primary dz i.e. with immune modulating meds
(2) Symptomatic treatment: diuretics, statins, diet, anticoag

(3) Reduction of proteinurea/slowing progression
- blood pressure reduction
- inhibiting the renin-angiotensin axis

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10
Q

What are the 5 major diseases in nephrotic syndrome?

A
  1. Minimal change dz (most common idiopathic type in kids)
  2. Focal segmental glomerulosclerosis (most common idiopathic in blacks)
  3. Membranous glomerulopathy (most common idiopathic in whites)
  4. Diffuse diabetic glomerulosclerosis
  5. Amyloidosis
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11
Q

Minimal change disease: findings, signs, symptoms, and course

A
  • *Findings**: low serum albumin, normal 24h urine protein, high serum cholesterol, serologic tests normal
  • 30% have high blood pressure
  • 30% have microhematurea +/- low GFR
  • Volume depletion
  • *Histological patterns**:
  • lipid nephrosis: pale, lipid rich kidneys
  • tubules stuffed with lipids
  • oval fat body: sloughed epithelial cells filled with lipid; hallmark of the urine analysis
  • glomeruli appear normal!! hence the name of the dz
  • no immune reactants
  • it’s a podocyte dz:foot process effacement –> reduced filtration

Symptoms: general like nephrotic syndrome

Course: responds to steroids, relapse, no RF

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12
Q

What evidence supports the idea that there are immunologic derangements in MCD?

A

May follow viral infection, recent immunizations

Altered in vitro response to mitogens

Circulating lymphocytotoxins (permeability factors)

Association with Hodgkins’s Disease and other lymphoproliferative disease in some patients

Responds to steroids

** probably some factor is toxic to the podocytes –> debasement –> can’t maintain the normal glomerular barrier & size/charge selection

** highly selective proteinurea with albumin only –> tells liver to make more lipids

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13
Q

Do you need to biopsy in minimal change dz?

A

In adults: yes

In a kid: assume it’s MCD, bc 80% of kids with nephrotic syndrome have MCD. if they don’t respond, then you need a biopsy

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14
Q

How do you treat MCD?

A

Prednisone

Furosemide

Pt should get better!

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15
Q

Focal segmental glomerulosclerosis: signs, histology, symptoms, treatment, course

A

Signs: low albumin, normal creat, high cholesterol, proteinurea, negative serologic tests
- high blood pressure and microhematuria in 30%, renal dysfunction in 50%

Histology: sclerosis is focal (involves some of the glomeruli but not others) and segmental (portion of the glomerular tuft)

  • debasement of the food processes in the whole glomerulus (not focal) –> podocyte effacement and detachment
  • immuno staining: for IgM and C3
  • can progress to global glomerulosclerosis –> extensive tubular atrophy & interstitial fibrosis –> ESRD
  • in HIV: enlarged, hyperechoic kidneys, also can become global not segmental and lead to ESRF; you also get tubular microcysts

Treatment: 50% respond to steroids, reoccurs in 1/3 of transplant pt’s

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16
Q

Compare and contrast MCD and FSGS:

A

Similarities: might share the same permeability factor that leads to foot process debasement

Differences: FSGS - nonselective proteinurea; MCD - only albumin
- FSGS can progress to ESRF; MCD usually doesn’t

17
Q

What can cause FSGS?

A

Primary FSGS: unknown cause, prob circulating permeability factors

Secondary FSGS: genetic (mutation in podocyte genes- nephrin, podocin)

  • viral, esp HIV
  • drugs (heroin, adriamycin in rodents)
  • adaptive: in repsonse to elevated glomerular capillary pressures and flows i.e. if you’re born with one kidney, reflux nephropathy (if urine goes from bladder back to ureter and kidney causing damage & scarring –> hyperfiltration in remnant nephrons), any chronic renal dz, obesity

All cause podocyte injury and depletion –> glomerulosclerosis

18
Q

When FSGS is adaptive, how does “stress” on kidneys lead to FSGS?

A

Loss of functioning nephrons –>

(1) increased glomerular volume –> increased capillary radius
(2) increased P and Q (increased cap radius also causes this)

–>

Tension of GCW

–> microaneurisms

–> segmental capillary collapse and mesangial sclerosis

This leads to a loss of functioning nephrons and creates a viscious cycle!

19
Q

Who gets focal segmental glomerulosclerosis?

A

More common in blacks and they tend to do worse even with the same #s

20
Q

Membranous glomerulopathy: signs, histology, symptoms, course, treatment

A

Signs: edema, proteinurea, normal GFR, low serum albumin, high cholest

  • you can test for anti-PLA2R in blood (this helps you rule it in bc 70% of patients have circulating antibody)
  • normal serologic tests

Histology:

  • GBM thickening but no hypercellularity on H&E stain
  • GBM spikes on silver stain, around the subepithelial deposits
  • red staining subepithelial deposits on trichrome stain
  • granular IgG and C3
  • foot process effacement

Course: variable, but usually slow progressing; most common idiopathic NS in whites

Treatment: conservative therapy, not all pt’s need treatment - steroids, cyclosporine, anti C5 Ab, rituximab

21
Q

In membranous glomerulopathy, what are the deposits from?

A

Antigen-antibody complexes composed of IgG bound to GP330/megalin are capped and shed from clathrin coated pits at soles of foot processes to form subepithelial deposits

Binding of complement –> formation of C5b9 complex –> activation of podocyte scavenging of C5b-9 & activation of cytochrome b558 –> formation of ROS –> deposition of ROS in GBM –> proteinurea

22
Q

What is the target antigen in human primary membranous glomerulopathy?

A

Phospholipase A2 receptor (PLA2R)

23
Q

What conditions are associated with secondary membranous glomerulopathy?

A

Infections: Hep B and C, secondary and congenital syphilis, malaria, schistosomiasis

Drugs: gold, penicillamine

Collagen vascular dz: SLE, Hashimoto’s thyroiditis, RA, mixed conn tiss disorder

Neoplasia: carcinoma- lung breast colon stomach

24
Q

Which nephrotic syndrom dz do you see thrombosis most commonly?

A

Membranous glomerulopathy!!

It’s possible to get it in the other ones, but most commonly seen in MG!

Increased coagulation tendency

DVT, RVT, pulmonary emboli are possible

Membranous NS greatest risk (35%)

Most RVT asymptomatic but flank pain, microhematurea, low GFR

Sometimes you will see lots of collateral circulation if pt has total venous thrombosis

25
Q

Diffuse Diabetic Glomerulosclerosis: signs, histoloty, treatment, course

A
  • *Signs**: proteinurea, diabetes, edema (periorbital and pedal)
  • microaneurisms, severe retinopathy: do opthalmic exam
  • *Histology**:
  • thickening of all the extracellular components in the glomerulus
  • nodules made up of RBC; sometimes they can dilate and even burst –> hematuria; parallel to what’s happening in the retina!
  • hyalinosis: lipid mixed with protinaceous material that gets trapped here
  • arteries –> hyalinosis; thickening
  • mesangial sclerosis and thick GBM; note there are no deposits; massive nodular areas of sclerosis obliterate teh glomerulus = mesantial nodule

Course: can progress to end stage kidney dz

Treatment: try to limit renal injury in diabetic nephropathy to prevent it; if they have proteinurea- treat symptoms with diuretic, reduce proteinurea

26
Q

In diabetes mellitus, what happens to the basement membrane? Which body parts are affected?

A

Thickening due to glycation of basement membranes

Vascular BM: glomerular capillaries, muscle capillaries, retinal capillaries, and arterioles

Other: renal tubules, mammary ducts, schwann cells

27
Q

What is the effect of nonenzymatic glycosylation of glomerular BM in diabetic glomerulsclerosis?

A

(1) accumulation of circulating plasma proteins –> gets trapped –> defective mesangial clearing –> mesangial sclerosis
(2) decrease in sialic acid content and increase in cationic charge –> protein can get thru more easily bc GBM becomes more + charged –> proteinurea
(3) increase in disulfide bonding GBM collagen –> decrease in GBM degradation and increase in GBM synthesis (altered homeostasis) –> GBM thickening

28
Q

What are the 5 stages of diabetic renal dz in type 1 diabetes? (applies to type 2 but harder to characterize bc not a sudden onset)

A

1 hyperfiltration

2 clinically silent

3 incipient nephropathy: microalbuminemia you cant see on dipstick

4 overt nephropathy- overt proteinurea

5 ESRD

29
Q

How can you limit renal injury in diabetic nephropathy?

A

blood pressure reduction

inhibition of the renin angiotensin aldosterone axis

blood sugar control

metabolic manipulation

30
Q

Amyloidosis NS: signs, histology, course, treatment

A

Signs: another associated cause
- proteinurea, negative serologic tests

Histology: amyloid- pale staining infiltrate (paler staining than matrix)

  • congo red polarization: apple green birefringence
  • fibrils infiltrated at the glomerular basement membrane- this destroys the barrier
  • immunostain tells you if you have amyloid (different type depending on the dz)
  • lambda light chain in AL amyloidosis lights up in immunofluroesence
  • end stage kidney is large and pale

Treatment: treat symptoms, reduce proteinurea, treat the underlying dz

31
Q

What is amyloid?

A

homogenous, hyaline, eosinophilic proteinacious substance
- congo stain is the gold standard to see it

Fibrillar constituent: random arrays of non-branching fibrils

Nonfibrillar constituent: pentameric discs of protein in beta pleated sheet

32
Q

What are teh 2 main types of amyloid and what are the causes?

A

Primary = AL = light chain
- dysproteinemias

Secondary = AA = acute phase reactant
- inflammatory or infectious states

33
Q

What chronic dz’s are associated wtih AA amyloidosis?

A

TB

leprosy

chronic osteomyelitis

paraplegia

chronic bronchiectasis

CF

chronic heroin addiction

RA

psoriasis

familial mediterranean fever

Renal (21 decks)

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