Flashcards in 10.28: Renal II Deck (31):
What is RPGN?
- Rapidly progressing glomerulonephritis to renal failure
- Gross hematuria with red urine
3 types of RPGN?
1. Anti GBM antibody
2. Severe immune complex
3. ANCA: anti-neutrophilic cytoplasmic antibodies
***Crescents seen in all 3 types
Presentation of RPGN?
1. Rapidly progressing loss of renal function
2. Nephritis with gross hematuria: red urine
3. Crescents always seen
What is a crescent?
- Glomerular stopper that stops bleeding when glomerular vasculature is punctured
- Forms in a crescent shape of parietal epithelial cells
- Decreases GFR leading to rapid renal failure
When are crescents seen?
Severe glomerular basement membrane injury with necrosis and breaks
Presentation of RPGN I?
1. Gross hematuria
2. Decreased urine output
3. Hemoptysis: injury to pulmonary alviola too
4. History of smoking, working in car garage or industrial exposure
5. Urine Cr
6. Chest x ray with bilateral pulmonary opacities
Who is RPGN I usually seen in?
Pathogenesis of RPGN I?
- Expose to virus, smoking, dyes or drugs
- Body forms anti-glomerular basement membrane antibodies
- Antibodies are deposited along entire length of glomerulus
- Creates sieve like holes allowing RBCs to leak through
What is goodpasture syndrome?
- Antibody formed in RPGN I has cross reactivity with alveolar basement membrane
- Leads to hemoptysis
What is hemoptysis?
What is the most deadly part of RPGN I?
- Pulmonary fibrosis
- Kidneys can be saved with dialysis but not lungs
- Plasmapheresis used for treatment
Pathogenesis of RPGN II?
- Severe immune complex formation with necrosis and breaks in glomerular membrane
- Can be associated with lupus or post infectious
Presentation of RPGN III?
1. Usually in older patients
2. Low urine output with hematuria
3. Hemoptysis with SOB
4. Complexes not seen in laboratory test: test for ANCA
Pathogenesis of RPGN III?
c-ANCA: "Antineutrophil cytoplasmic autoantibodies"
- React with neutrophil antigens
What is pulmonary angiitis and granulomatosis characteristic of?
- AKA "Wegener's syndrome"
- RPGN III "ELK"
E- ear, nose, throat
L - Lungs
K - Kidneys
What is ELK?
Infection is also seen in ear, lung, kidney
What is goodpasture syndrome?
What is wegener granulomatosis?
What is c-ANCA seen in? p-ANCA?
c-ANCA: Wegener's granulomatosis
p-ANCA: Churg strauss
What is foamy urine characteristic of?
Is hematuria seen in nephrotic syndrome?
- No, holes in glomeruli are only big enough for proteins
How does glomeruli prevent albumin filtration?
- Holes in it are too small for albumin to pass
- Negative charge repels albumin
Characteristics of nephrotic syndrome?
1. Urine protein > 3.5g/day
Clinical presentation of membranous nephropathy?
- Generalized edema seen around eyes as well
- Thrombosis: antithrombin III is lost in urine
What is puffy eyes characteristic of?
Pathogenesis of membranous nephropathy ?
- In situ subepithelial immune complex formation
- Occurs on basal surface of podocytes losing slit diaphragms
- Leads to capillary wall thickening
- No cellular reaction, inflammation, or proliferation
- Can be caused by cancer, penicillin, gold
What is spike and dome pattern characteristic of?
What needs to be ruled out in membranous nephropathy?
Does membranous nephropathy lead do drop in complement?
- No, slower more chronic development
Typical characteristic of membranous nephropathy?
- Edema with eye puffiness
- Foamy urine