Glomerular Injury/Disease Pathogenesis Flashcards
What defines a Primary Glomerulopathy?
ONLY the kidney is involved
List 4 responses that the glomerulus tissue can have in response to injury
- Hypercellularity
- Basement membrane thickening
- Hyalinosis
- Sclerosis
List 4 responses that the glomerulus tissue can have in response to injury
- Hypercellularity
- Basement membrane thickening
- Hyalinosis
- Sclerosis
Hypercellularity of the glomerulus tissue can result from a few different things. It can result from proliferation of what type of cells?
Mesangial/endothelial cell proliferation
Hypercellularity of the glomerulus tissue can result from a few different things. It can result from infiltration of?
Leukocyte infiltration
If Hypercellularity of the glomerulus tissue is due to mesangial/endothelial cell proliferation AND leukocyte infiltration, what is that called?
Endocapillary proliferation
Hypercellularity of the glomerulus tissue can result from a few different things. What specific structures can form and and what are they made of that may cause hypercellularity?
Formation of Crescents
= Epithelial cells + leukocytes
Crescents are made of what things?
Epithelial cells + leukocytes
What 2 glomerulus tissue responses to injury can cause/result in capillary wall injuries?
Hyalinosis and Sclerosis
Homogenous eosinophilic material
Hyalinosis
What proteins make up hyalinosis?
Plasma proteins
Extracellular collagenous matrix deposition
Sclerosis
There are many different clinical manifestations of Glomerular Diseases. List some of them (5)
- Nephritic or Nephrotic syndrome
- Rapidly Progressive Glomerulonephritis
- CKD
- Urinary abnormalities (hematuria/proteinuria)
There are many different clinical manifestations of Glomerular Diseases. List some of them (5)
- Nephritic or Nephrotic Syndromes
- Rapidly Progressive Glomerulonephritis
- CKD
- Urinary abnormalities (hematuria/proteinuria)
Glomerular Injury is usually ____ mediated
Immune mediated
What is the most common Glomerular Injury pathogenesis?
In situ formation of immune complexes
- Antibodies react with intrinsic or planted tissue in glomerulus
Glomeruluar injury caused by in situ formation of immune complexes involves?
Antibodies that react with intrinsic tissue antigens or planted antigens in the glomerulus
Diseases that are caused by In situ formation of immune complexes show what type of immunofluorescence?
Granular
Diseases that are caused by antibodies against components of the GBM show what type of immunofluorescence?
Diffuse Linear
Diseases that are caused by circulating immune complexes are trapped how in the glomeruli?
Antigen-Antibody complexes get trapped due to the glomeruli hemodynamics
– not because they are reacting with intrinsic tissue
Diffuse linear immunofluorescence pattern suggests that the antibodies are against?
GBM
Granular immunofluorescence suggests that the antibodies are against?
Intrinsic tissue antigens or planted antigens
– in situ formation of complexes
ALL immune complex depositions in the glomeruli cause what to occur?
Local inflammatory reaction that produces an injury
What main inflammatory reactants do immune complex deposition glomerular diseases activate?
Complement
Neutrophils
Macrophages
What main inflammatory reactants do T-cell mediated Glomerular injuries activate?
Macrophages
T cells
Mesangial cells
Platelets
What specific things can cause the injury to the glomerulus?
Oxidants, cytokines, chemokines
Proteases
Nitric oxide, Eicosanoids
Immune Complex deposition that causes glomerular injury involves what type of deposits?
Electron dense deposits made of immune complexes
Electron dense deposits between the endothelial cells and the GBM are considered?
Subendothlelial
Electron dense deposits between the outer surface of the GBM and the podocytes are considered?
Subepithelial
Electron dense deposits can also occur in the?
Mesangium
Glomerular injury can also occur due to epithelial cell injury. This involves injury to?
Podocytes
What occurs with podocyte injuries?
Effacement of foot processes, vacuolization, retraction and cell detachment
What are 2 examples of diseases that specifically cause podocyte injury?
Focal Segmental Glomerulosclerosis
Diabetic Nephropathy
With Glomerular injuries, what will it progress to once the GFR is < 30-50% of normal?
ESRD
What are the 2 histologic manifestations of ESRD?
Glomerulosclerosis
Tubular injury/interstitial fibrosis
What are 2 Hereditary Nephritis’s?
Alport Syndrome
Thin Basement Membrane Nephropathy
What are 2 Hereditary Nephritis’s?
Alport Syndrome
Thin Basement Membrane Nephropathy
Hereditary Nephritis diseases have mutations in?
Collagen type IV
Alport Syndrome is inherited how?
X-linked
What are the symptoms of Alport syndrome?
- Hematuria that progresses to renal failure
- Nerve Deafness and Eye disorders
Hematuria, nerve deafness and eye disorders suggests what hereditary nephritis?
Alport Syndrome
How does Alport Syndrome’s GBM look?
Basket weave appearance
– alternating thin and thick areas
What is a sign of Alport Syndrome?
Alpha 3,4,5 collagen antibodies all fail to stain
Thin Basement Membrane Nephropathy is inherited how?
Autosomally
What are the symptoms of Thin Basement Membrane Nephropathy?
Asymptomatic hematuria
How does Thin Basement Membrane Nephropathy GBM look?
Thin
Benign Familial Hematuria is also known as?
Thin Basement Membrane Nephropathy
Asymptomatic hematuria suggests what hereditary nephritis?
Thin Basement Membrane Nephropathy