Kidney in systemic disease Flashcards
C-ANCA =
Diffuse cytoplasmic reactivity due to antibodies directed against proteinase-3.
Granulomatosis with polyangitis ( Wegners )
P-ANCA
Perinuclear reactivity only due to lysosomal myeloperoxidase. May be positive in up to 30% of patients
Microscopic polyangitis
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 ?
- Microangiopathic Hemolytic Anemia
- Thrombocytopenia
- AKI
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 ?
5 things
- Microangiopathic hemolytic anemia
- Thrombocytopenia
- Neurologic Dysfunction
- Renal Dysfunction
- Fever
What deficiency causes TTP ?
ADAMST13
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
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
