Flashcards in Kidney in systemic disease Deck (31):
Diffuse cytoplasmic reactivity due to antibodies directed against proteinase-3.
Granulomatosis with polyangitis ( Wegners )
Perinuclear reactivity only due to lysosomal myeloperoxidase. May be positive in up to 30% of patients
Can Pauci-immune glomerulonephritis be ANCA-negative ?
Yes it can be ANCA negative and without extra-renal disease
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.
What does Wegners cause ?
Rapidly progressive crecentric glomerulonephritis
How can you distinguish TMP from DIC ?
DIC will have an consumptive coagulopathy with prolonged tests of bleeding.
What do TMP and DIC have in common ?
Thrombocytopenia and microangiopathic hemolytic anemia
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
What is HUS a triad of ?
1. Microangiopathic Hemolytic Anemia
What is HUS a normal complication of ?
Gastroenteriris caused by E.Coli
-Shinga Toxin in circulation
What does minor endothelial injury do to patients with HUS ?
Uncontrolled activation of the complement system with intravascular thrombosis
What does thrombotis thrombocytopenic purpura consist of ?
1. Microangiopathic hemolytic anemia
3. Neurologic Dysfunction
4. Renal Dysfunction
What deficiency causes TTP ?
If you find microangiopathic hemolytic anemia and thrombocytopenia in a patient with no other explanation for them besides TPP what should you do ?
What does Lupus cause ?
Immune complex mediatede glomerulonephritis that responds well to immunosupressive drugs
What is vasculitis ?
Inflammation of blood vessels, usually autoimmune
What is polyarteritis nodosa ?
Autoimmune ANCA-negative medium vessel arteritis
What are ANCA's ?
They are antibodies against proteinse 3 or myeloperoxidase associated with specific autoimmune disease
What is microscopic polyangitis ?
Autoimmune nectotizing granulomatous vasculitis of the respiratory tract causing crescentric glomerulonephritis associated with P-anca
What is granulomatosis with polyangiitis ?
Wegners- autoimmune necrotizing granulomatosus vasculitis of respiratory tract and cause of crescentic glomerulonephritis associates with C-ANCA
Hemolytic Uremic Syndrome ?
Thrombotic microangiopathy of glomeruli in small children due to Shinga toxin from diarrheal E-Coli H7: 0157 infection
Thrombotic Thrombocytopenic Purpura
Thrombotic microangiopathy due to VWF multimers from ADAMS13 deficiency
What is Lupus Nephritis ?
Immune Complex-Mediated Glomerulonephritis of systemic lupus erythematosus
What is Scleroderma ?
Rapidly progressive renal failure of systemic sclerosis
What are the two mechanisms in which vascular diseases can affect the kidney ?
1. Inflammation of the blood vessels
2. Loss of thromboresistance
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 - )
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
What is pauci-Immune Glomerulonephritis ?
Refers to the negative immunofluorescence studies usualli in the setting of crescentic glomerulonpphritis
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
What kind of disease is Wegners ?
Sinopulmonary renal syndrome