Nephritic Syndrome - Pathoma Flashcards
What is the hallmark of Nephritic Syndrome?
- Glomerular inflammation
- Bleeding from glomerulus
What are the common signs and symptoms of Nephritic Syndrome?
- Hematuria
- Oliguria (small urine production)
- Azotemia (high BUN)
- Salt retention with periorbital edema
- fluid retention
- HTN
- RBC casts
- Dysmorphic RBCs in urine
What would be seen in general on a biopsy of glomeruli in Nephritic Syndrome?
- Hypercellular, inflamed glomeruli
- Immune-complex deposition → activates complement
- C5a attracts neutrophils → mediate damage
What nephritic syndrome occurs mostly in children after impetigo or strep?
Poststreptococcal Glomerulonephritis
(arises after a Beta-hemolytic Strep infection of skin/pharynx)
What bacterial strains cause Poststreptococcal Glomerulonephritis?
- Nephritogenic strains
- Carry M protein virulence factor
- may occur after infection with non-strep organisms as well
What is the classic presentation of Poststreptococcal Glomerulonephritis?
- Presentation 2-3 weeks after infection
- usually child, but may occur in adults
- Hematuria
- “coca-cola” colored urine
- Oliguria
- Hypertension
- Periorbital edema
What would you see on kidney biopsy in Poststreptococcal Glomerulonephritis?
-
Hypercellular, inflamed glomeruli on H&E
- Mediated by immune complex deposition
- granular IF
- Mediated by immune complex deposition
-
Subepithelial “humps” on EM
- deposits pile up on BM
What is the treatment for Poststreptococcal Glomerulonephritis?
-
SUPPORTIVE
- children rarely progress to renal failure
- some adults develop Rapidly Progressive Glomerulonephritis → renal failure in weeks-months
How soon do patients with Rapidly Progressive Glomerulonephritis progress to renal failure?
Weeks to Months
What findings are characteristic of Rapidly Progressive Glomerulonephritis in a kidney biopsy ?
- Crescents in Bowman’s Space on H&E
- comprised of fibrin and macrophages (inflammatory debris)
What test after renal biopsy will help you figure out the etiology of Rapidly Progressive Glomerulonephritis?
Immunofluorescence
What does a finding of Linear immunofluorescence suggest as the etiology of Rapidly Progressive Glomerulonephritis?
- Antibody against the collagen in basement membrane
- binds basement membrane → creates sharp line = linear immunofluorescence
- SUGGESTS: Goodpasture Syndrome
- binds basement membrane → creates sharp line = linear immunofluorescence
What is the classic presentation of Goodpasture Syndrome?
- Hemoptysis
- antibody against collagen in alveolar basement membrane
- Hematuria
- antibody against collagen in glomerular basement membrane
- Young, adult male
What does a finding of Granular immunofluorescence suggest as the etiology of Rapidly Progressive Glomerulonephritis?
- Immune complex deposition
-
Poststreptococcus Glomerulonephritis
- most common
-
Diffuse Proliferative Glomerulonephritis
- diffuse deposition of antibody-antigen complexes subendothelially
- common in SLE with renal disease
- diffuse deposition of antibody-antigen complexes subendothelially
-
Poststreptococcus Glomerulonephritis
What does a finding of Negative immunofluorescence suggest as the etiology of Rapidly Progressive Glomerulonephritis?
- Pauci-Immune (limited immune)
- Could be:
- Wegener granulomatosis
- Microscopic polyangiitis
- Churg-Strauss Syndrome
- Could be:
After finding a Negative immunofluorescence in the setting of Rapidly Progressive Glomerulonephritis, what is your next step in differentiating between the possible etiologies?
-
Perform ANCA test
- Wegener granulomatosis (c-ANCA)
- nasopharynx, lung, kidney
- Microscopic polyangiitis (p-ANCA)
- Churg-Strauss Syndrome (p-ANCA)
- Wegener granulomatosis (c-ANCA)
How do you differentiate between Churg-Strauss and Microscopic Polyangiitis when you get a p-ANCA result?
- Churg-Strauss has three things MP does not:
- granulomatous inflammation
- eosinophilia
- asthma
What is the most common cause of Nephropathy worldwide?
IgA Nephropathy
Where does IgA deposit in IgA nephropathy?
Mesangium of glomeruli
What is the common presentation of IgA Nephropathy?
- Episodic gross or microscopic hematuria
- RBC casts
- Following mucosal infections
- produce too much IgA
What is the histological hallmark of IgA nephropathy?
- IgA immune complex deposition in the mesangium
- seen in Immunofluorescence (granular)
What syndrome is characterized by an inherited defect in Type IV collagen (most commonly X-linked) that results in thinning and splitting of the glomerular basement membrane?
Alport Syndrome
How does Alport Syndrome classically present?
- Isolated hematuria
- Sensory hearing loss
- Ocular disturbances
- lens dislocation
- cataracts
- corneal dystrophy
