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Pathology > Glomerular Diseases > Flashcards

Glomerular Diseases Flashcards

1
Q

Description and Diagnosis

A
  • Mesangial cell ingrowth (the arrow) with splitting of the GBM due to subendothelial immune complexes
  • Membranoproliferative Glomerulonephritis (MPGN)
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2
Q

Minimal change disease (Lipoid nephrosis)

(Causes)

A
  • Most common cause of nephrotic syndrome in children 2-6 years of age
  • It could be primary (idiopathic) that can be triggered by recent infection, immunization, or immune stimulus
  • It could be secondary to lymphoma (cytokine-mediated damage) or NSAIDs
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3
Q

Goodpasture syndrome

(Causes)

A
  • Production of anti-bodies against the noncollagenous component of type IV collagen (alpha-3 subunit) of the basement membrane which results in damage of lungs and kidneys \
  • It is type II hypersensitivity reaction
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4
Q

Description and Diagnosis

A
  • Alternating thinning and thickening of the basement membrane with splitting of the lamina densa giving it “Basket-weave” appearance
  • Alport syndrome
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5
Q

Description and Diagnosis

A
  • Electron dense immune deposits within the thickened basement membrane giving the “spike and dome” appearance (the spikes are the intervening basement membrane matrix between the deposits)
  • Membranous glomerulonephritis
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6
Q

IgA nephropathy (Berger disease)

(Causes)

A
  • Production of IgA anti-bodies that target the mesangium
  • After respiratory or GI infections
  • Celiac sprue
  • Henoch-Schonlein purpura
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7
Q

Acute proliferative glomerulonephritis (acute post-streptococcal glomerulonephritis)

(Causes)

A

It is a type III hypersensitivity reaction due to:

  • Beta-hemolytic group A streptococci (after 2-4 weeks of throat or skin infection) is the most common cause, but it can occur after any infection
  • SLE
  • Polyarteritis nodosa
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8
Q

Description and Diagnosis

A

  • Hypercellular and capillary loops are poorly defined due to increased number of epithelial, endothelial, and mesangial cells as well as neutrophils in and around glomerular capillary loops
  • Acute proliferative glomerulonephritis (acute post-streptococcal glomerulonephritis)
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9
Q

Membranoproliferative Glomerulonephritis (MPGN)

(Work up)

A
  • Decreased serum C3 in all types
  • Presence of C3 nephritic factor in type II
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10
Q

Goodpasture syndrome

(Presentation)

A
  • Usually males 20-40 years of age
  • Pulmonary involvement precedes renal one and presents as pulmonary hemorrhage and recurrent hemoptysis
  • Renal involvement will present as gross hematuria
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11
Q

Alport syndrome

(Presentation)

A
  • Gross or microscopic hematuria that begins in childhood
  • Sensorineural deafness
  • Eye problems like retinopathy and lens dislocation (Lenti Conus)
  • “Can’t see, can’t pee, can’t hear a bee”
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12
Q

Description and Diagnosis

A
  • Silver stain that demonstrates double contour of the basement membrane “tram-track” due to GBS splitting caused by mesangial ingrowth due to subendothelial immune complexes
  • Membranoproliferative Glomerulonephritis (MPGN)
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13
Q

Diffuse Proliferative Glomerulonephritis (DPGN)

(Causes)

A
  • SLE (a common cause of death in SLE)
  • Membranoproliferative glomerulonephritis (MPGN)
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14
Q

Focal Segmental glomerulosclerosis

(Causes)

A
  • Most common cause of nephrotic syndrome in African Americans (Lipoprotein L1) and Hispanics
  • Can be primary (idiopathic)
  • Can be secondary to:

* HIV infection

* Sickle cell disease

* Heroin abuse

* Morbid obesity

* Interferon treatment

* Chronic kidney disease due to congenital malformations or some familial case with mutations in NPHS1 (nephrin) and NPHS2 (podocin) genes encoding for proteins forming the slit pores

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15
Q

Membranous glomerulonephritis

(Causes)

A
  • Most common cause of nephrotic syndrome in Caucasian adults
  • 85% is primary (idiopathic) due to anti-bodies against phospholipase A2 receptor
  • Secondary to:

* Drugs: penicillamine and NSAIDs

* Infections: HBV, HCV, HIV, syphilis and malaria

* SLE and DM

* Solid tumors: colon and lung cancers

* Heavy metals: mercury, gold and lead

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16
Q

Acute proliferative glomerulonephritis (acute post-streptococcal glomerulonephritis)

(Treatment and Prognosis)

A
  • Treatment is supportive with antibiotics and diuretics to prevent fluid overload
  • In children good prognosis with > 95% will recover completely, but severe cases may progress to rapidly progressive glomerulonephritis and chronic glomerulonephritis
  • In adults 60% will recover completely and 40% will progress to rapidly progressive glomerulonephritis and chronic glomerulonephritis
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17
Q

Description and Diagnosis

A
  • Dense deposits in the basement membrane
  • Membranoproliferative Glomerulonephritis (MPGN) type II which is now known as Dense deposit disease
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18
Q

Description and Diagnosis

A
  • Pinky hyaline material in capillary loop region due to increase in mesangial matrix due to the non-enzymatic glycosylation of proteins
  • Nodular glomerulosclerosis (Kimmelstiel-Wilson syndrome)
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19
Q

General Principles of Glomerular Diseases

A
  • Generally are chronic
  • Generally are not caused by toxins or hypoperfusion
  • All of them can cause nephrotic syndrome
  • Biopsy is the most accurate test
  • Are often treated with steroids
  • Additional immunosuppressive medications (cyclophosphamide, mycophenolate) are frequently used
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20
Q

Description and Diagnosis

A
  • Diffuse capillary and GBS thickening, but without increase in cellularity
  • Membranous glomerulonephritis
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21
Q

Goodpasture syndrome

(Treatment and Prognosis)

A
  • Emergent plasmapheresis with pulsed steroids and possibly cytotoxic drugs
  • Poor prognosis due to life-threatening pulmonary hemorrhage and RPGN that may lead to renal failure
  • Rapid aggressive treatment may prevent the development of ESRD
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22
Q

Description and Diagnosis

A
  • Trichrome stain with blue collagen deposition
  • Focal Segmental glomerulosclerosis
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23
Q

Rapidly Progressive Glomerulonephritis (RPGN)

(Causes)

A
  • Idiopathic
  • Goodpasture syndrome
  • Granulomatosis with polyangiitis (Wegener)
  • Microscopic polyangiitis
  • SLE
  • Berger disease
  • Acute proliferative glomerulonephritis (post-streptococcal glomerulonephritis)
24
Q

Diffuse proliferative glomerulonephritis (Lupus nephritis)

(Treatment)

A

Prednisone and cytotoxic therapy (cyclophosphamide or mycophenolate) may slow disease progression

25
Q

Description and Diagnosis

A
  • Granular pattern
  • Diffuse proliferative glomerulonephritis (Lupus nephritis)
26
Q

Descritption and Diagnosis

A
  • Crescent shape deposition in Bowman’s space (fibrin, plasma proteins (C3b), glomerular parietal cells, monocytes and macrophages)
  • Rapidly progressive glomerulonephritis (RPGN)
27
Q

Diabetic glomerulonephropathy

(Causes)

A
  • Also called Nodular glomerulosclerosis (Kimmelstiel-Wilson syndrome)
  • Non-enzymatic glycosylation of GBM that will lead to increase permeability and thickening with mesangial expansion
28
Q

Description and Diagnosis

A
  • Thickened basement membrane with subendothelial and intramembranous deposits of IgG and C3 or C1q (SLE specific) immune complexes
  • Diffuse proliferative glomerulonephritis (Lupus nephritis)
29
Q

Description and Diagnosis

A
  • The glomerular capillary loops are pink and thickened with obliteration of their lumen due to mesangial proliferation “wire looping”
  • Diffuse proliferative glomerulonephritis (Lupus nephritis)
30
Q

Alport syndrome

(Treatment and Prognosis)

A
  • No treatment
  • Progresses to renal failure and the nephritis may recur after renal transplant
31
Q

Minimal change disease (Lipoid nephrosis)

(Treatment and Prognosis)

A
  • Steroids
  • Excellent prognosis
32
Q

Membranous glomerulonephritis

(Treatment and Prognosis)

A
  • Steroids
  • Variable prognosis:

* May lead to spontaneous remission

* Persistent proteinuria (especially the primary one)

* ESRD

33
Q

IgA nephropathy (Berger disease)

(Presentation)

A
  • Most common glomerulonephritis in the world
  • It affects children and young adults (usually males)
  • Episodic gross hematuria that occurs concurrently with respiratory or GI tract infection
34
Q

Glomerular Diseases that presents as Nephritic syndrome

A
  • Acute proliferative glomerulonephritis (acute post-streptococcal glomerulonephritis)
  • Rapidly progressive glomerulonephritis
  • IgA nephropathy (Berger disease)
  • Alport syndrome
35
Q

Acute proliferative glomerulonephritis (acute post-streptococcal glomerulonephritis)

(Work up)

A
  • Anti-streptolysin O (ASO), anti-hyaluronidase, and anti-DNase B tests together are very sensitve
  • Decreased complement levels due to consumption
36
Q

Glomerular diseases that presents as Nephritic or Nephrotic syndromes

A
  • Diffuse proliferative glomerulonephritis
  • Membranoproliferative glomerulonephritis
37
Q

Membranoproliferative Glomerulonephritis (MPGN)

(Causes)

A
  • Type I may be idiopathic or may be secondary to hepatitis B or C infection or HIV
  • Type II is due to auto-antibody called C3 nephritic factor. This antibody stabilizes C3 convertase, which leads to enhanced degradation and low serum level C3
  • It also could be due to SLE, cryoglobulinemia, subacute bacterial endocarditis or malignancies like chronic lymphocytic leukemia
38
Q

Description and Diagnosis

A
  • Bright crescent (Pauci-immune [no Ig/C3 deposition])
  • Rapidly progressive glomerulonephritis (RPGN) due to granulomatosis with polyangiitis (Wegener) or microscopic polyangiitis
39
Q

IgA nephropathy (Berger disease)

(Treatment and Prognosis)

A
  • No treatment proven to reverse the disease
  • Severe proteinuria is treated with ACEIs and steroids
  • 30% will completely resolve
  • 40-50% will slowly progress to renal failure (ESRD) over 25 years
40
Q

Description and Diagnosis

A
  • Bright deposits scattered along capillary walls and in the mesangium
  • Membranoproliferative Glomerulonephritis (MPGN) type II which is now known as Dense deposit disease
41
Q

Description and Diagnosis

A
  • Mesangial pattern immune complex deposits (IgA)
  • IgA nephropathy (Berger disease)
42
Q

Description and Diagnosis

A
  • Normal or mild mesangial proliferation
  • IgA nephropathy (Berger disease)
43
Q

Description and Diagnosis

A
  • Fusion of the epithelial foot processes (effacement of podocytes)
  • Minimal change disease (Lipoid nephrosis)
44
Q

Description and Diagnosis

A
  • Subepithelial immune complex humps
  • Acute proliferative glomerulonephritis (acute post-streptococcal glomerulonephritis)
45
Q

Nodular glomerulosclerosis (Kimmelstiel-Wilson syndrome)

(Treatment)

A
  • Tight control of blood sugar
  • ACEIs for type I DM
  • ARBs for type II DM
46
Q

Description and Diagnosis

A
  • In the middle of the glomerulus there is collagenous sclerosis
  • Focal Segmental glomerulosclerosis
47
Q

Glomerular diseases that presents as Nephrotic syndrome

A
  • Minimal change disease
  • Focal segemental glomerulosclerosis
  • Membranous nephropathy
  • Amyloidosis
  • Diabetic glomerulonephropathy
48
Q

Description and Diagnosis

A
  • PAS stain that shows nodular sclerosis of capillary loops and hyaline arteriolosclerosis of the arteriole
  • Nodular glomerulosclerosis (Kimmelstiel-Wilson syndrome)
49
Q

Acute proliferative glomerulonephritis (acute post-streptococcal glomerulonephritis)

(Presentation)

A
  • History of infection
  • Most frequently seen in children
  • Dark (cola-colored) urine
  • Edema that is often periorbital
  • Hypertension
  • Oliguria
50
Q

Goodpasture syndrome

(Work up)

A
  • Anti-GBM anti-bodies
  • Iron deficiency anemia
  • Hemosidren-filled macrophages in sputum
  • CXR will show pulmonary infiltrates
51
Q

Description and Diagnosis

A
  • Granular appearance “lumpy-bumpy” due to IgG, IgM, and C3 deposition along GBM and mesangium
  • Acute proliferative glomerulonephritis (acute post-streptococcal glomerulonephritis)
52
Q

Focal Segmental glomerulosclerosis

(Treatment and Prognosis)

A
  • Prednisone and cytotoxic therapy
  • ACEIs/ARBs to decrease proteinuria
  • Poor prognosis and most progress to chronic renal failure and high rate of recurrence after renal transplantation
53
Q

Description and Diagnosis

A
  • Smooth, diffuse, linear pattern deposition of anti-GBM antibodies (IgG)
  • Goodpasture syndrome
54
Q

Alport syndrome

(Cause)

A
  • X-linked recessive
  • Mutation of COL4A5 gene that encodes for alpha-5 chain of collagen IV
55
Q

Description and Diagnosis

A
  • Sliver stain that shows projections of basement membrane “spikes” between the immune deposits
  • Membranous glomerulonephritis
56
Q

Membranoproliferative Glomerulonephritis (MPGN)

(Treatment and Prognosis)

A
  • Corticosteroids and cytotoxic drugs
  • Slow progression to chronic renal failure over 10 years
  • High incidence of recurrence in transplants

Pathology (9 decks)

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