Kidney in systemic disease Flashcards Preview

Organ Systems- Renal > Kidney in systemic disease > Flashcards

Flashcards in Kidney in systemic disease Deck (31):
1

C-ANCA =

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

Granulomatosis with polyangitis ( Wegners )

2

P-ANCA

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

Microscopic polyangitis

3

Can Pauci-immune glomerulonephritis be ANCA-negative ?

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

4

What do ANCA's do ?

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.

5

What does Wegners cause ?

Rapidly progressive crecentric glomerulonephritis

6

How can you distinguish TMP from DIC ?

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

7

What do TMP and DIC have in common ?

Thrombocytopenia and microangiopathic hemolytic anemia

8

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

Hemolytic Uremic Syndrome

9

What is HUS a triad of ?

1. Microangiopathic Hemolytic Anemia
2. Thrombocytopenia
3. AKI

10

What is HUS a normal complication of ?

Gastroenteriris caused by E.Coli
-Shinga Toxin in circulation

11

What does minor endothelial injury do to patients with HUS ?

Uncontrolled activation of the complement system with intravascular thrombosis

12

What does thrombotis thrombocytopenic purpura consist of ?
5 things

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

13

What deficiency causes TTP ?

ADAMST13

14

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

Immediate plasmaphresis

15

What does Lupus cause ?

Immune complex mediatede glomerulonephritis that responds well to immunosupressive drugs

16

What is vasculitis ?

Inflammation of blood vessels, usually autoimmune

17

What is polyarteritis nodosa ?

Autoimmune ANCA-negative medium vessel arteritis

18

What are ANCA's ?

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

19

What is microscopic polyangitis ?

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

20

What is granulomatosis with polyangiitis ?

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

21

Hemolytic Uremic Syndrome ?

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

22

Thrombotic Thrombocytopenic Purpura

Thrombotic microangiopathy due to VWF multimers from ADAMS13 deficiency

23

What is Lupus Nephritis ?

Immune Complex-Mediated Glomerulonephritis of systemic lupus erythematosus

24

What is Scleroderma ?

Rapidly progressive renal failure of systemic sclerosis

25

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

1. Inflammation of the blood vessels
2. Loss of thromboresistance

26

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

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

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

( 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

What is pauci-Immune Glomerulonephritis ?

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

29

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

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

30

What kind of disease is Wegners ?

Sinopulmonary renal syndrome

31

What are the characteristic aspects of Wegners ?

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