Kidney in systemic disease Flashcards

1
Q

C-ANCA =

A

Diffuse cytoplasmic reactivity due to antibodies directed against proteinase-3.

Granulomatosis with polyangitis ( Wegners )

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2
Q

P-ANCA

A

Perinuclear reactivity only due to lysosomal myeloperoxidase. May be positive in up to 30% of patients

Microscopic polyangitis

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3
Q

Can Pauci-immune glomerulonephritis be ANCA-negative ?

A

Yes it can be ANCA negative and without extra-renal disease

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4
Q

What do ANCA’s do ?

A

They are pathogenic and cause endothelial cell injury in glomeruli and blood vessels. They bind to neutrophils causing PNM activation and increased contact adhesion with endothelial cells and vascular structures.

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5
Q

What does Wegners cause ?

A

Rapidly progressive crecentric glomerulonephritis

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6
Q

How can you distinguish TMP from DIC ?

A

DIC will have an consumptive coagulopathy with prolonged tests of bleeding.

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7
Q

What do TMP and DIC have in common ?

A

Thrombocytopenia and microangiopathic hemolytic anemia

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8
Q

The sudden onset of irritability, lethargy, weakness, pallor, and Oliguria, in a small child 5-10 days following gastroenteritis will suggest what ?

A

Hemolytic Uremic Syndrome

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9
Q

What is HUS a triad of ?

A
  1. Microangiopathic Hemolytic Anemia
  2. Thrombocytopenia
  3. AKI
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10
Q

What is HUS a normal complication of ?

A

Gastroenteriris caused by E.Coli

-Shinga Toxin in circulation

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11
Q

What does minor endothelial injury do to patients with HUS ?

A

Uncontrolled activation of the complement system with intravascular thrombosis

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12
Q

What does thrombotis thrombocytopenic purpura consist of ?

5 things

A
  1. Microangiopathic hemolytic anemia
  2. Thrombocytopenia
  3. Neurologic Dysfunction
  4. Renal Dysfunction
  5. Fever
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13
Q

What deficiency causes TTP ?

A

ADAMST13

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14
Q

If you find microangiopathic hemolytic anemia and thrombocytopenia in a patient with no other explanation for them besides TPP what should you do ?

A

Immediate plasmaphresis

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15
Q

What does Lupus cause ?

A

Immune complex mediatede glomerulonephritis that responds well to immunosupressive drugs

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16
Q

What is vasculitis ?

A

Inflammation of blood vessels, usually autoimmune

17
Q

What is polyarteritis nodosa ?

A

Autoimmune ANCA-negative medium vessel arteritis

18
Q

What are ANCA’s ?

A

They are antibodies against proteinse 3 or myeloperoxidase associated with specific autoimmune disease

19
Q

What is microscopic polyangitis ?

A

Autoimmune nectotizing granulomatous vasculitis of the respiratory tract causing crescentric glomerulonephritis associated with P-anca

20
Q

What is granulomatosis with polyangiitis ?

A

Wegners- autoimmune necrotizing granulomatosus vasculitis of respiratory tract and cause of crescentic glomerulonephritis associates with C-ANCA

21
Q

Hemolytic Uremic Syndrome ?

A

Thrombotic microangiopathy of glomeruli in small children due to Shinga toxin from diarrheal E-Coli H7: 0157 infection

22
Q

Thrombotic Thrombocytopenic Purpura

A

Thrombotic microangiopathy due to VWF multimers from ADAMS13 deficiency

23
Q

What is Lupus Nephritis ?

A

Immune Complex-Mediated Glomerulonephritis of systemic lupus erythematosus

24
Q

What is Scleroderma ?

A

Rapidly progressive renal failure of systemic sclerosis

25
Q

What are the two mechanisms in which vascular diseases can affect the kidney ?

A
  1. Inflammation of the blood vessels

2. Loss of thromboresistance

26
Q

What is an example of medium vessel disease and what is the pathogenesis ?

A

Medium vessel disease (Polyarterious nodosa) will cause renal infarcts and distal glomerular ischemia associated with a decline in GFR not associated with Glomerular inflammation with RBC casts ( usually ANCA - )

27
Q

What are some small vessel diseases and what is their pathogesis ?

A

( Microscopic polyangietis, wegners, and chrug strauss syndrome ) –> they cause focal necrotizing lesions with crescent formation with active urinary sediment
**Endothelial cells are the primary target in small vessel vasculitis

28
Q

What is pauci-Immune Glomerulonephritis ?

A

Refers to the negative immunofluorescence studies usualli in the setting of crescentic glomerulonpphritis

29
Q

What is the pathogenesis of endothelial cell injury by ANCA’s ?

A

PMN activation by ANCA
Inhibition of PR3 inactivation by ANCA
EC activation /lysis by ANCA

30
Q

What kind of disease is Wegners ?

A

Sinopulmonary renal syndrome

31
Q

What are the characteristic aspects of Wegners ?

A
  1. Multinucleated Giant Cells
  2. Vascular Necrosis and thrombosis
    3.