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Renal 2 > Path - Kidney in Systemic Disease I > Flashcards

Path - Kidney in Systemic Disease I Flashcards

1
Q

Name the 4 vascular injury syndromes?

A

ANCA-associated glomerulonephritis

Thrombotic microangiopathy

Lupus nephritis

Scleroderma

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2
Q

What are the 2 forms of pathogenesis in vascular disorders?

A
  1. Inflammation of blood vessels (seen in vasculitides)
  2. Loss of thromboresistance (seen in thrombotic microangiopathies)
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3
Q

Most common medium vessel disease?

A

Polyarteritis nodosa

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4
Q

Name 3 most common small vessel diseases?

A

Microscopic polyangiitis, granulomatosis with polyangiitis (aka Wegeners), Churgg Stauss syndrome

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5
Q

Describe key difference between medium vessel disease and small vessel disease

A

Medium vessel

  • causes distal glomerular ischemia leading to renal infarcts. It is NOT associated with glomerulonephritis
  • Not associated with glomerular inflammation with RBC casts (usually ANCA negative)

Small vessel

  • causes focal necrotizing lesions with crescent formation (aka glomerulonephritis)
  • active urinary sediment and rapid progression of kidney failure (usually ANCA positive)
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6
Q

Medium vessel disease UA findings?

A

No RBC casts

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7
Q

Small vessel disease UA findings?

A

Active urinary sediment = hematuria (+ RBC casts)

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8
Q

3 disorders involving Hep B or C? (nephrotic or nephritic syndromes included)

A

Membranous - more commonly Hep B

MPGN - more commonly Hep C

Polyarteritis nodosa - more commonly Hep C

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9
Q

Polyarteritis nodosa affects what type of vessels?

A

Medium sized muscular arteries (does not affect veins)

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10
Q

Which type of vascular disorder is associated with ANCAs? (medium or small vessel)

A

Small vessel disorders

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11
Q

Polyarteritis nodosa epidemiology?

A

Most common in middle aged or older adults with peak incidence in sixth decade of life

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12
Q

Causes of PAN (polyarteritis nodosa)?

A

Idiopathic, also associated with Hep B or Hep C (more common than B), and hairy cell leukemia

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13
Q

Common presentation of PAN (polyarteritis nodosa)?

A

Fever, fatigue, rash, weight loss, arthralgia.

May see HTN, renal insufficiency, neurologic dysfunction, abdominal pain

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14
Q

Pathogenesis of PAN?

A

Unknown

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15
Q

Pathology of PAN (polyarteritis nodosa)

A

Buzzword. Segmental transmural necrotizing vasculitis

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16
Q

Early stage PAN - what pathologic finding?

Later stage PAN - what pathologic finding?

A

Early - lots of PMNs (neutrophils)

Later - fibrinoid necrosis

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17
Q

What disease process is shown in this kidney? Describe white arrows, black arrows, and red arrow

A

This is a kidney in polyarteritis nodosa

White arrows = renal cortical infarcts

Black arrows = aneurysms

Red arrow = ruptured aneurysm

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18
Q

In PAN, patient with ruptured aneurysm will present how? Significance of ruptured aneurysm in kidney?

A

Ruptured aneurysm causes painless hemorrhage shock (no nerve endings in retroperitoneum) - causing death if not treated immediately

  • They will be hypotensive, syncopal, and have extremely low Hgb
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19
Q

Pauci immune means?

A

Negative immunofluoresence stains usually in setting of crescentic glomerulonephritis

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20
Q

Pauci-immune glomerulonephritis is often associated with what 2 findings?

A

ANCAs and extrarenal findings (arthritis, arthralgias, myalgias, fatigue)

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21
Q

2 Key points about pauci-immune glomerulonephritis and ANCAs

A
  1. ANCA titers may not always parallel disease activity
  2. Patient can be ANCA negative and without extrarenal findings, but still have pauci-immune glomerulonephritis
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22
Q

Another name for c-ANCA? Most commonly associated with?

A

anti-proteinase 3

seen in granulomatosis with polynagiitis (Wegeners)

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23
Q

Another name for p-ANCA? Commonly associated with?

A

anti-myeloperoxidase

seen in Microscopic polyangiitis or Churg-Strauss syndrome

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24
Q

Which ANCA has a greater specificity?

A

c-ANCA has a 95% specificty for Wegener’s. p-ANCA is not as specific

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25
Q

Are ANCAs diagnostic or pathogenic?

A

ANCAs are pathogenic, meaning they are not usually present.

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26
Q

Describe how ANCAs work? What 3 adhesion molecules are important?

A

ANCAs bind to neutrophils, activating them. This causes increased contact and adhesion with endotheilal cells and vascular structures.

The three main adhesion factors are Beta 2 integrin, Mac-1, and Fc gamma

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27
Q

What are the primary target cells in small vessel vasculitis?

A

Endothelial cells

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28
Q

What are the 2 common disorders affecting BOTH lungs and kidneys?

A
  1. Goodpasture syndrome
  2. Granulomatosis with polyangiitis
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29
Q

Wegener’s is considered what type of renal syndrome?

A

Sinopulmonary renal syndrome

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30
Q

3 sites involved in Wegener’s

A

Nasopharynx, Lungs, Kidney

31
Q

Symptoms associated with Wegener’s

A

Rhinorrhea, sinusitis, nasopharyngeal mucosal ulcerations, dyspnea, hemopytis, transietn pulmonary infiltrates

fever, weight loss, arthralgias/arthritis, skin lesions

32
Q

Main difference in renal function between Wegener’s and Goodpasture syndrome/Anti-GBM?

A

Wegener’s will not commonly have renal impairment at presentation (only 10% have azotemia) vs goodpasture that will commonly have renal impairment at presentation

33
Q

Pathology of Wegener’s. What key feature is present (hence the granulomatosis)

A

Necrotizing granulomatous inflammation with multinucleated giant cells (red circles)

  • can see vascular necrosis with thrombosis
  • can see crescentic glomerulonephritis
34
Q

Treatment for granulomatosis with polyangiitis

A

Cyclophosphamide, steroids, plasmapheresis

35
Q

Prognosis of Wegener’s?

A

Mortality is 80-90% if untreated

Relapse in 25-50% of patients with 3-5 years of follow up

36
Q

2 syndromes of thrombotic microangiopathies (TMA)

A
  1. Hemolytic uremic syndrome (HUS)
  2. Thrombotic thrombocytopenic purpura (TTP)
37
Q

3 common findings of HUS?

A
  1. Hemolytic anemia (schistocytes on blood smear)
  2. Renal dysfunction
  3. Thrombocytopenia (due to platelet consumption)
38
Q

5 common findings of TTP?

A
  1. Fever
  2. Hemolytic anemia
  3. Thrombocytopenia
  4. Renal dysfunction
  5. Neurologic dysfunction

Note: Now patients are being diagnosed even in the absence of 1 or more findings

39
Q

Pathogenesis of thrombotic microangiopathies?

A

Loss of thromboresistance by the endothelial cell, leads to platelet activation and deposition (along with fibrin) in lumen of affected vessels

40
Q

2 main deposits in thrombotic microangiopathies?

A

Platelet and fibrin deposition in affected vessels

41
Q

Buzzword for thrombotic angiopathies?

A

Onionskin apperance, however this is not specific for thrombotic microangiopathies

42
Q

Onionskinning is common in what other syndromes besides thrombotic microangiopathies

A

Common in:

HTN, Scleroderma, sometimes Lupus

43
Q

In what vessels does onionskinning occur? Why?

A

Occurs in arterioles due to thrombosis of capillaries (leading to increased back pressure)

44
Q

Endothelial damage in thrombotic microangiopathies are commonly caused by what 3 mechanisms?

A
  1. E. coli H7:0157 (in HUS)
  2. Chemotherapeutic agents (cyclosporine, gemcitabine, bleomycin/cisplatinum)
  3. Radiation (bone marrow transplant)
45
Q

Hemolytic uremic syndrome commonly caused by? Mechanism?

A

E.Coli H7:O157 verotoxin, causes production of cytotoxic anti-endothelial Ab’s

46
Q

Increased levels of von Willebrand factor causes what?

A

Enhanced platelet aggregation

47
Q

Familial TTP mechanism? What percentage of TTP patients are familial TTP?

A

genetic deficiency of vWF cleaving protease (Low ADAMTS13 activity)

10% of TTP

48
Q

Autoimmune TTP mechanism?

A

autoantibody to vWF cleaving protease develops (ADAMTS13 autoantibodies)

49
Q

Buzzword. Schistocytes?

A

HUS, TTP

50
Q

Treatment for TTP?

A

Plasmaphoresis

51
Q

2 Key differences between thrombotic microangiopathies and DIC?

A
  1. PT, PTT, INR:

TMA - PT, PTT, and INR are normal

DIC - PT, PTT, INR are prolonged (think, prolonged DIC(k)!)

  1. Diathesis (tendency to suffer from medical condition)

TMA - associated with thrombotic diathesis

DIC - associated with bleeding diathesis

52
Q

Lupus epidemiology?

A

Black women, but can be present in almost all races/ages

53
Q

Lupus antibodies most common?

A

ANA - antinuclear antibody

anti-dsDNA antibody

anti-smith antibody

Last 2 are specific for Lupus

54
Q

What test is commonly done to screen for syphilis? What is the relation to lupus?

A

If patient has +RPR test (which is used to screen for syphilis) you must follow up with anti-smith and anti-dsDNA antibody screening to r/o lupus

BRS version:

+RPR, - antismith, - dsDNA = possible Syphilis

+RPR, +antismith, + dsDNA = Lupus

55
Q

How many classes of Lupus nephritis are there? What are they? Which is most common?

A

6 classes

Class I = Minimal mesangial, rare

Class II = Mesangial proliferative, 15%, mild

Class III = Focal proliferative, 25%, moderate

Class IV = Diffuse proliferative, 50%, severe

Class V = Membranous, 10%, nephrotic

Class VI = Advanced sclerosing, ? %

56
Q

Class II Lupus nephritis = ?

%?

Severity?

A

Class II = Mesangial proliferative

15%

Mild severity

57
Q

Class III Lupus Nephritis = ?

%?

Severity?

A

Class III = Focal Proliferative

25%

Moderate severity

58
Q

Class IV Lupus Nephritis = ?

%?

Prognosis?

A

Class IV = Diffuse proliferative

50%

SEVERE

59
Q

Class V Lupus Nephritis = ?

%?

Description?

A

Class V = Membranous

10%

Nephrotic

60
Q

Type IV Lupus nephritis = ?

What 3 types of proliferative cells are present

A

Class IV = Diffuse proliferative lupus nephritis = most common

Lots of excess cells (inflammatory cells, mesangial cells, capillary endothelial cells)

61
Q

Class IV Lupus Nephritis is associated with what buzzword pathology?

A

Wireloop lesions

62
Q

Crescent formation is possible in what class of Lupus nephritis?

A

Class IV (diffuse proliferative). If in doubt, pick Class IV, its the most talked about in lecture

63
Q

Immunofluorescence of Lupus Nephritis? What is present?

A

FULL HOUSE immunofluoresence

IgG, IgA, IgM, C3, and C4

64
Q

Which class is associated with poor renal survivial?

A

Class IV (both global and segmental) has poor prognosis. Will need to put on dialysis

65
Q

Treatment for SLE?

A

Aspirin

Glucocorticoids,

Immunosuppressants:Cyclophosphamide (IV only), Mycophenolate mofetil,

TLR inhibitors: Hydroxychloroquine

Bold = preferential therapy

66
Q

Another name for scleroderma?

A

Systemic sclerosis

67
Q

Scleroderma MOA?

A

Involves CT and microvasculature with fibrosis and vascular occlusion, causing onion skin sclerotic thickening of interlobar arteries (larger arteries)

68
Q

Scleroderma epidemiology?

A

Black females (1/4000 adults in US)

69
Q

2 subtypes of scleroderma? Mechanism of differentiation?

A

Limited cutaneous

Diffuse cutaneous

Separated based on pattern of skin involvement

70
Q

Which subtype of Scleroderma is more likely to have renal involvement?

A

Diffuse scleroderma

71
Q

What is scleroderma renal crisis?

A

New onset of accelerated arterial HTN and/or rapidly progressive oliguric renal failure

72
Q

How do you treat scleroderma renal crisis?

A

Treat with ACE-I. This is the one time where you give ACE-I to patient with poor renal function.

73
Q

What drug can cause scleroderma?

A

Corticosteroid therapy can increase risk for scleroderma

Renal 2 (11 decks)

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