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1 Mega Step 1 Deck > Nephrotic Syndrome > Flashcards

Nephrotic Syndrome Flashcards

1
Q

clinical clues for glomerular disease

A

PROTEINURIA and/or HEMATURIA (dysmorphic RBC, RBC cast)
+/- drop in GFR
+/- electrolyte/acid abnormalities
+/- change in urine output

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2
Q

nephrotic vs. nephritic syndromes - detailed

A

*nephritic syndrome:
-location of immune complex deposition = SUBENDOTHELIAL (b/w fenestrated endothelium and GBM - BAD)
-damage = INFLAMMATION of glomerular barrier
-classic clinical finding = mostly HEMATURIA

*nephrotic syndrome:
-location of immune complex deposition = SUBEPITHELIAL (b/w GBM and foot processes)
-damage = LEAKY glomerular barrier
-classic clinical finding = mostly PROTEINURIA

*mixed:
-location of immune complex deposition = mesangial
-damage = both inflammation & leakage
-finding = blend of hematuria and low-level proteinuria

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3
Q

subendothelial deposits - location

A

*between endothelial cells and basement membrane
*endothelial cells face the capillary side
*more commonly seen in nephritic syndrome

*glomerular inflammation → GBM damage → loss of RBCs into urine → dysmorphic RBCs, hematuria

naming is counterintuitive: subENDOthelial is NEPHRITIC syndrome (even though it has an “O” in it)

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4
Q

subepithelial deposits - location

A

*between the podocytes and the basement membrane

*podocytes face the urinary space/lumen

*more commonly seen in NEPHROTIC syndrome

naming is counterintuitive: subEPIthelial is nephrotic (even though it has the letter “I” in it)

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5
Q

immune complex deposition → glomerular damage

A

*type 3 hypersensitivity reaction (antigen:antibody immune complexes) → damage of the glomerular barrier
*location of immune complex deposition results in the associated syndromes:
-subendothelial deposits → nephritic syndrome
-subepithelial deposits → nephrotic syndrome

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6
Q

nephrotic syndrome - classic findings

A

*nephrotic syndrome:
1. large proteinuria (>3.5 grams/day)
2. hypoalbuminemia
3. edema
4. hypercholesterolemia

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7
Q

nephritic syndrome - classic findings

A

*nephritic syndrome:
1. hematuria
2. azotemia
3. oliguria
4. hypertension

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8
Q

nephrotic syndrome - overview

A

*clinical syndrome caused by pathological glomerular protein losses

*hallmarks:
1. proteinuria ( > 3.5 grams/24 hours)
2. hypoalbuminemia
3. edema
4. hyperlipidemia

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9
Q

how much protein must be in the urine to define nephrotic syndrome

A

> 3.5 grams per day of protein in the urine

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10
Q

edema in nephrotic syndrome

A

edema can manifest in various ways with nephrotic syndrome, including:
*periorbital edema
*puffy pale face
*lips may be swollen
*moderate to severe edema in the legs

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11
Q

nephrotic syndrome - urine casts

A

*oval fat bodies (“fatty casts”) - lipid-laden cells, associated with “Maltese cross” sign

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12
Q

complications of nephrotic syndrome

A
  1. hyperlipidemia
    -due to elevated production, abnormal transport, and decreased catabolism of lipids
  2. hypercoagulability (DVT, renal vein thrombosis)
    -due to loss of anticoagulation proteins
  3. relative increased risk of infection (staph, pneumococcus)
    -due to loss of IgG, complement factors
  4. hypovitaminosis D
    -due to proximal tubular dysfunction and vitamin D binding protein losses
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13
Q

quantifying proteinuria

A

*pathologic amounts of proteinuria: > 150 mg
150 mg - 3.5 grams/day = sub-nephrotic proteinuria
> 3.5 grams/day = nephrotic range proteinuria

albuminuria:
< 30 mg/day = normal
30-300 mg/day = “microalbuminuria” (moderately increased)
> 300 mg/day = “macroalbuminuria” (severely increased)

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14
Q

classifying proteinuria

A
  1. transient vs. isolated:
    -occurs INTERMITTENTLY due to stress which alter renal hemodynamics (fever, vigorous exercise, exposure to extreme cold)
  2. orthostatic vs. postural:
    -occurs in chidren
    -proteinuria when upright > supine
  3. PERSISTENT:
    -glomerular source: increased movement of protein across GBM
    -tubular source: decreased reabsorption of protein in PCT (Fanconi)
    -overflow: increased production of plasma proteins overwhelms GBM filtering capacity and tubular reabsorption (multiple myeloma)
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15
Q

diseases that cause nephrotic syndrome

A
  1. minimal change disease (most common in children)
  2. membranous nephropathy
  3. focal segmental glomerulosclerosis (FSGS)
  4. amyloidosis
  5. diabetic glomerulonephropathy (MOST COMMON CAUSE)

note - each disorder can be primary or secondary

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16
Q

information obtained from kidney biopsy

A
  1. light microscopy: allows for identification of structural changes
    -thickening of the GBM, nephrosclerosis, tubular atrophy, intra-capillary proliferation
  2. immunofluorescence: allows for identification and location of antibody deposition
  3. electron microscopy: allows for identification of ultrastructural changes
    -location and appearance of immune complexes
    -podocyte effacement
    -deposited protein fibril size/orientation
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17
Q

minimal change disease - overview

A

*most common cause of nephrotic syndrome in children
*hallmark pathology: visceral epithelial cell (podocyte) damage

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18
Q

minimal change disease - mechanism/etiology

A

*primary (idiopathic)
*secondary:
-NSAIDs
-lymphoma
-triggered by recent infection, immunization, or immune stimulus

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19
Q

minimal change disease - light microscopy findings

A

normal glomeruli

proximal tubule cells may be laden with lipids (lipid necrosis)

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20
Q

minimal change disease - immunofluorescence findings

A

negative (not an immune-complex disease)

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21
Q

minimal change disease - electron microscopy

A

effacement of podocyte foot processes

this is the ONLY biopsy finding associated with minimal change disease

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22
Q

minimal change disease - epidemiology

A

*most common cause of nephrotic syndrome in CHILDREN
*can occur in adults too

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23
Q

minimal change disease - clinical presentation

A

*sudden onset of massive proteinuria and nephrotic syndrome (proteinuria, hypoalbuminemia, edema, hyperlipidemia)
*usually triggered by recent infection, immunization, or immune stimulus

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24
Q

minimal change disease - treatment

A

*primary: corticosteroids & other immunosuppression
*secondary: treat underlying cause (stop NSAIDs, treat lymphoma, etc)

25
Q

minimal change disease - prognosis

A

*1/3 remit, but most relapse/remit
*children have better outcomes
*HTN and ESRD are rare

26
Q

focal segmental glomerulosclerosis (FSGS) - light microscopy findings

A

*focal and segmental sclerosis and hyalinosis

27
Q

focal segmental glomerulosclerosis (FSGS) - immunofluorescence

A

*not an immune complex disease (often NEGATIVE)
*but can see focal IgM, C3 in sclerotic areas / mesangium

28
Q

focal segmental glomerulosclerosis (FSGS) - electron microscopy findings

A

*effacement of podocyte foot processes

29
Q

focal segmental glomerulosclerosis (FSGS) - overview

A

*causes nephrotic syndrome
*hallmark: focal degeneration and disruption of visceral epithelial cells
*likely caused by circulating cytokines & genetically determined defects of the slit diaphragm complex
*hyalinosis and sclerosis thought to result from hyperpermeable areas of the GBM and extracellular matrix deposition

30
Q

PRIMARY focal segmental glomerulosclerosis (FSGS) - pathogenesis

A

*proteinuria can recur within 24 hours after transplant, suggesting a circulating factor as the cause
*hereditary/genetic: associations with mutations in podocyte proteins, including APOL1 variants

31
Q

SECONDARY focal segmental glomerulosclerosis (FSGS) - pathogenesis

A

*HIV
*SICKLE CELL DISEASE
*hyperfiltration
*toxins/drugs (PAMIDRONATE, HEROIN)
*reflux nephropathy

32
Q

focal segmental glomerulosclerosis (FSGS) - epidemiology

A

*more common in adults than children

33
Q

focal segmental glomerulosclerosis (FSGS) - clinical presentation

A

*varies; from gradually progressive (secondary) to nephrotic syndrome (primary)

34
Q

focal segmental glomerulosclerosis (FSGS) - treatment

A

*ACEi / ARB

*for primary with persistent nephrotic syndrome: immunosuppression / corticosteroids

35
Q

focal segmental glomerulosclerosis (FSGS) - prognosis

A

*spontaneous remission rare
*common cause of ESRD
*can recur within days to weeks in transplant

36
Q

HIV associated nephropathy (HIVAN) - pathology

A

*a COLLAPSING variant of focal segmental glomerulosclerosis (FSGS)
*sclerotic tuft is retracted with proliferation of overlying visceral epithelial cells
*cystic dilation of tubule segments
*tubuloreticular inclusions of endothelial cells

37
Q

HIV associated nephropathy (HIVAN) - pathogenesis

A

*direct infection of glomerular/tubular cells with HIV

38
Q

HIV associated nephropathy (HIVAN) - epidemiology

A

*more common in patients with APOL1 high risk variants

39
Q

HIV associated nephropathy (HIVAN) - clinical presentation

A

*usually seen in uncontrolled HIV, but can precede AIDS
*nephrotic syndrome common

40
Q

HIV associated nephropathy (HIVAN) - treatment

A

*treat HIV (anti-retroviral therapy)

41
Q

HIV associated nephropathy (HIVAN) - prognosis

A

*poor
*common cause of ESRD in HIV patients

42
Q

membranous nephropathy - overview

A

*an immune complex deposition cause of nephrotic syndrome
*hallmark finding: granular immune complex deposition on IF

43
Q

membranous nephropathy - light microscopy findings

A

*diffuse capillary wall and GBM thickening
*“spikes and domes” on silver stain

44
Q

membranous nephropathy - immunofluorescence findings

A

*granular staining in GBM due to immune complex deposition (IgG, C3)

45
Q

membranous nephropathy - electron microscopy

A

*foot process effacement
*subepithelial deposits with “spike and dome” appearance

46
Q

membranous nephropathy - pathogenesis

A

*in situ immune complex formation against various antigens
*complement activation and formation of the MAC → activates glomerular epithelial and mesangial cells → capillary wall injury and protein leakage

47
Q

PRIMARY membranous nephropathy - pathogenesis

A

*thought to be genetic susceptibility + antibodies to renal in-situ autoantigen
*antigen in neonates = neutral endopeptidase
*antigen in adults = M type phospholipase A2 receptors (PLA2R)

48
Q

SECONDARY membranous nephropathy - causes

A

*drugs (penicillamine, captopril, gold, NSAIDs)
*SOLID TUMOR MALIGNANCIES
*autoimmune diseases (LUPUS, etc)
*infections (HEPATITIS B, etc)

49
Q

membranous nephropathy - epidemiology

A

*think middle aged males of caucasian descent
*more common in adults than children

50
Q

membranous nephropathy - clinical presentation

A

*primary: sudden onset proteinuria with nephrotic syndrome
*secondary: can have more subtle onset

51
Q

membranous nephropathy - treatment

A

*primary: corticosteroids and immunosuppression
*secondary: treat underlying condition
*age/sex appropriate cancer screening

52
Q

membranous nephropathy - prognosis

A

*for primary disease: 1/3 spontaneously remit, 1/3 persistent, 1/3 progressive

53
Q

diabetic glomerulonephropathy - light microscopy findings

A
  1. afferent & efferent arteriolar hyalinosis
  2. mesangial expansion
  3. GBM thickening
  4. nodular glomerulosclerosis (Kimmelstiel-Wilson nodules)
54
Q

diabetic glomerulo-nephropathy - immunofluorescence

A

*negative (not an immune complex disease)
*can have nonspecific focal IgG

55
Q

diabetic glomerulo-nephropathy - electron microscopy findings

A

*foot process effacement
*GBM thickening

56
Q

glomerular amyloidosis - light microscopy findings

A

*fluffy “cotton candy” deposition in mesangium
*apple-green birefringence on congo red staining

57
Q

glomerular amyloidosis - immunofluorescence

A

most commonly lambda light chains > kappa

58
Q

glomerular amyloidosis - electron microscopy findings

A

*randomly arranged fibrils
*mesangial expansion by amyloid fibrils

59
Q

glomerular amyloidosis - pathogenesis

A

*extracellular tissue deposition of protein fibrils in beta pleated sheets
*AL: plasma cell dyscrasia → deposition of light chains
*AA: inflammation → deposition of serum amyloid A (acute phase reactant)
*AF: family of inherited protein deformities such as transthyretin (TTR)

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