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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
Are ANCAs diagnostic or pathogenic?
ANCAs are pathogenic, meaning they are not usually present.
26
Describe how ANCAs work? What 3 adhesion molecules are important?
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
27
What are the primary target cells in small vessel vasculitis?
Endothelial cells
28
What are the 2 common disorders affecting BOTH lungs and kidneys?
1. Goodpasture syndrome 2. Granulomatosis with polyangiitis
29
Wegener's is considered what type of renal syndrome?
Sinopulmonary renal syndrome
30
3 sites involved in Wegener's
Nasopharynx, Lungs, Kidney
31
Symptoms associated with Wegener's
Rhinorrhea, sinusitis, nasopharyngeal mucosal ulcerations, dyspnea, hemopytis, transietn pulmonary infiltrates fever, weight loss, arthralgias/arthritis, skin lesions
32
Main difference in renal function between Wegener's and Goodpasture syndrome/Anti-GBM?
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
Pathology of Wegener's. What key feature is present (hence the granulomatosis)
Necrotizing granulomatous inflammation with multinucleated giant cells (red circles) - can see vascular necrosis with thrombosis - can see crescentic glomerulonephritis
34
Treatment for granulomatosis with polyangiitis
Cyclophosphamide, steroids, plasmapheresis
35
Prognosis of Wegener's?
Mortality is 80-90% if untreated Relapse in 25-50% of patients with 3-5 years of follow up
36
2 syndromes of thrombotic microangiopathies (TMA)
1. Hemolytic uremic syndrome (HUS) 2. Thrombotic thrombocytopenic purpura (TTP)
37
3 common findings of HUS?
1. Hemolytic anemia (schistocytes on blood smear) 2. Renal dysfunction 3. Thrombocytopenia (due to platelet consumption)
38
5 common findings of TTP?
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
Pathogenesis of thrombotic microangiopathies?
**Loss of thromboresistance by the endothelial cell**, leads to platelet activation and deposition (along with fibrin) in lumen of affected vessels
40
2 main deposits in thrombotic microangiopathies?
Platelet and fibrin deposition in affected vessels
41
Buzzword for thrombotic angiopathies?
**Onionskin** apperance, however this is not specific for thrombotic microangiopathies
42
Onionskinning is common in what other syndromes besides thrombotic microangiopathies
Common in: HTN, Scleroderma, sometimes Lupus
43
In what vessels does onionskinning occur? Why?
Occurs in arterioles due to thrombosis of capillaries (leading to increased back pressure)
44
Endothelial damage in thrombotic microangiopathies are commonly caused by what 3 mechanisms?
1. E. coli H7:0157 (in HUS) 2. Chemotherapeutic agents (cyclosporine, gemcitabine, bleomycin/cisplatinum) 3. Radiation (bone marrow transplant)
45
Hemolytic uremic syndrome commonly caused by? Mechanism?
E.Coli H7:O157 verotoxin, causes production of cytotoxic anti-endothelial Ab's
46
Increased levels of von Willebrand factor causes what?
Enhanced platelet aggregation
47
Familial TTP mechanism? What percentage of TTP patients are familial TTP?
genetic deficiency of vWF cleaving protease (**Low ADAMTS13** activity) 10% of TTP
48
Autoimmune TTP mechanism?
autoantibody to vWF cleaving protease develops (**ADAMTS13 autoantibodies**)
49
Buzzword. Schistocytes?
HUS, TTP
50
Treatment for TTP?
Plasmaphoresis
51
2 Key differences between thrombotic microangiopathies and DIC?
1. PT, PTT, INR: TMA - PT, PTT, and INR are **normal** DIC - PT, PTT, INR are prolonged (think, prolonged DIC(k)!) 2. Diathesis (tendency to suffer from medical condition) TMA - associated with **thrombotic** diathesis DIC - associated with **bleeding** diathesis
52
Lupus epidemiology?
Black women, but can be present in almost all races/ages
53
Lupus antibodies most common?
ANA - antinuclear antibody anti-dsDNA antibody anti-smith antibody Last 2 are specific for Lupus
54
What test is commonly done to screen for syphilis? What is the relation to lupus?
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
How many classes of Lupus nephritis are there? What are they? Which is most common?
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
Class II Lupus nephritis = ? %? Severity?
Class II = Mesangial proliferative 15% Mild severity
57
Class III Lupus Nephritis = ? %? Severity?
Class III = Focal Proliferative 25% Moderate severity
58
Class IV Lupus Nephritis = ? %? Prognosis?
Class IV = Diffuse proliferative ## Footnote **50%** **SEVERE**
59
Class V Lupus Nephritis = ? %? Description?
Class V = Membranous 10% Nephrotic
60
Type IV Lupus nephritis = ? What 3 types of proliferative cells are present
Class IV = Diffuse proliferative lupus nephritis = most common Lots of excess cells (inflammatory cells, mesangial cells, capillary endothelial cells)
61
Class IV Lupus Nephritis is associated with what buzzword pathology?
**Wireloop** lesions
62
Crescent formation is possible in what class of Lupus nephritis?
Class IV (diffuse proliferative). If in doubt, pick Class IV, its the most talked about in lecture
63
Immunofluorescence of Lupus Nephritis? What is present?
**FULL HOUSE** immunofluoresence IgG, IgA, IgM, C3, and C4
64
Which class is associated with poor renal survivial?
Class IV (both global and segmental) has poor prognosis. Will need to put on dialysis
65
Treatment for SLE?
Aspirin **Glucocorticoids**, Immunosuppressants:**Cyclophosphamide** (IV only), **Mycophenolate mofetil,** TLR inhibitors: **Hydroxychloroquine** **Bold** = preferential therapy
66
Another name for scleroderma?
Systemic sclerosis
67
Scleroderma MOA?
Involves CT and microvasculature with fibrosis and vascular occlusion, causing onion skin sclerotic thickening of interlobar arteries (larger arteries)
68
Scleroderma epidemiology?
Black females (1/4000 adults in US)
69
2 subtypes of scleroderma? Mechanism of differentiation?
Limited cutaneous Diffuse cutaneous Separated based on pattern of skin involvement
70
Which subtype of Scleroderma is more likely to have renal involvement?
Diffuse scleroderma
71
What is scleroderma renal crisis?
New onset of accelerated arterial HTN and/or rapidly progressive oliguric renal failure
72
How do you treat scleroderma renal crisis?
Treat with ACE-I. **This is the one time where you give ACE-I to patient with poor renal function.**
73
What drug can cause scleroderma?
Corticosteroid therapy can increase risk for scleroderma

Renal 2 (11 decks)

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