PATH: Kidney in Systemic Disease I Flashcards

1
Q

Microscopic polyangitis is associated with what?

A

P-ANCA (antibodies against myeloperoxidase)

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2
Q

Small vessel vasculitis usually causes what type of glomerulonephritis?

A

pauci-immune crescentic (have few Abs or ICs but lots of ANCAs)

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3
Q

What are the thrombotic microangiopathies?

A

HUS

TTP

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4
Q

Before ANCA-associated glomerulonephritis occurs, what sort of symptoms can occur?

A

extrarenal symptoms: joint pains, muscle aches, flu-like symptoms

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5
Q

Where do ANCAs act in the glomerulus?

A

cause ENDOTHELIAL injury in glomeruli and blood vessels

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6
Q

What normally happens when neutrophils release proteinase 3?

A

circulating alpha-1 antitrypsin rapidly breaks it down

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7
Q

What can increase the surface expression of proteinase 3 and MPO on the surface of the neutrophil?

A

TNF-alpha and IL-8 (synergistic inflammatory event)

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8
Q

If C-ANCA is present, what happens to neutrophils coated with proteinase 3?

A

the ANCA “coats” the proteinase 3 and “hides” it from alpha-1 antitrypsin

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9
Q

What happens when ANCAs bind to neutrophils?

A

they are activated and adhere to endothelial cells by beta-2 integrins, MAC-1, and Fc-Gamma

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10
Q

What type of complement pathway is activated by ANCA/neutrophil binding to endothelium?

A

alternative pathway

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11
Q

What is the overall result of the ANCA/neurtophil binding to the endothelium?

A

endothelial surface damage, ADCC, internalization of MPO adn proteinase 3 –> apoptosis

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12
Q

What else can ANCAs bind to in the glomerulus?

A

monocytes –> activates them to become macrophages in the crescents of Bowman’s space (release chemotactic mediators and toxic oxygen metabolites)

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13
Q

Wht type of ANCAs are seen in granulomatosis with polyangiitis?

A

C-ANCA (diffuse cytoplasmic)- directed at proteinase 3

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14
Q

What is the triad seen in granulomatosis with polyangitis patients?

A

Kidney problems
Lung problems
Pharynx problems (ulcers)

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15
Q

Who gets HUS?

A

children under 5 (sometimes connected with inherited factor H mutation 10%)

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16
Q

What is the clinical triad of HUS?

A

Microangiopathic hemolytic anemia, thrombocytopenia, acute kidney injury

17
Q

What is the underlying cause of HUS?

A

Complication of an intestinal infection with E Coli (H7:O157) that produces Shiga Toxin and changes ednothelium to a pro-coagulant state

18
Q

What is the main form of treatment for HUS?

A

blood transfusion (80%), dialysis (60%), Eculizumab (10%)

19
Q

What causes TTP?

A

deficiency (or antibody formed during pregnancy, infection, malignancy) against ADAMTS13

20
Q

What does ADAMTS13 do?

A

von-Willegrand factor-cleaving protease that cleaves and inactivates it to prevent interaction with platelets

21
Q

What does a mutation in ADAMTS13 do?

A

allows vWF to uncontrollably activate platelet aggregation and causes systemic microvascular thrombosis

22
Q

What are the symptoms of TTP?

A

FAT RN

23
Q

What is the treatment for TTP?

A

plasmapheresis

24
Q

What is the most severe form of lupus?

A

type IV (diffuse proliferative)

25
Q

What is the most common form of lupus?

A

type IV (diffuse proliferative)

26
Q

What Abs are postiive in Lupus?

A

Anti-DNA, Anti-Sm

27
Q

What does IF look like of a lupus glomerulus?

A

“Full House” where IgG, IgM, IgA, C3, and C4 are all positive

28
Q

What is scleroderma?

A

systemic sclerosis (CT and microvascular disease with fibrosis and vascular occlusion)

29
Q

What are the symptoms of limited cutaneous SS?

A

severe Raynaud’s, ulcers, gangrene, NO renal involvement

30
Q

What type of SS has renal involvement?

A

Diffuse cutaneous SS

31
Q

What are the s/s of diffuse cutenaous systemic sclerosis?

A

fatigue/weight loss, skin tight and thick, renal mainfestations, myalgia

32
Q

What is a scleroderma renal crisis?

A

new-onset of accelerated HTN and/or rapidly progressive oliguric renal failure

33
Q

How do you treat a scleroderma renal crisis?

A

ACE inhibitors

34
Q

What is the pathology of SS?

A

Arcuate arteries have intimal/medial proliferation and luminal narrowing (onion skinning of interlobar arteries)

  • Get fibrinoid necrosis and thrombosis
  • Vascular changes–> poor outcome