Renal: 12.4: Nephritic Syndromes Flashcards
What is seen on immunofluorescence in IgA nephropathy?
IgA-based immune complex deposits in mesangium
Dx?
- thinning/splitting of the GBM due to type IV collagen defect
- isolated hematuria, sensory hearing loss, ocular disturbances
Alport Syndrome
What are the s/s of post-strep glomerulonephritis (PSGN)?
- hematuria (Coca-Cola colored urine)
- oliguria
- HTN
- periorbital edema
- *** 2-3 weeks after a strep infection***
Dx?
- linear immunofluorescence pattern
Goodpasture syndrome
Your pt with nephritic signs has a negative immunofluorescence pattern (pauci-immune). What do you do next?
test for ANCA
What is the tx for post-strep glomerulonephritis (PSGN)?
supportive care
What is Alport Syndrome?
- thinning/splitting of the GBM due to type IV collagen defect
- isolated hematuria, sensory hearing loss, ocular disturbances
What is Wegener granulomatosis/granulomatosis with polyangiitis?
- a small vessel vasculitis
- involves lung, kidneys, and skin WITH nasopharyngeal involvement
- c-ANCA positive
Dx?
- crescents in Bowman’s space
rapidly progressive glomerulonephritis (RPGN)
In Goodpasture syndrome, an antibody against the collagen and alveolar basement membrane create a _____ immunofluorescence pattern.
linear
Dx?
- hematuria (Coca-Cola colored urine)
- oliguria
- HTN
- periorbital edema
- 2-3 weeks after a strep infection
post-strep glomerulonephritis (PSGN)
This is the most common cause of systemic vasculitis in children and is associated with IgA nephropathy/Berger’s disease.
Henoch-Schönlein purpura
What is post-strep glomerulonephritis (PSGN)?
nephritic syndrome after a Group A hemolytic strep infection
What is seen on light microscopy in IgA nephropathy?
mesangial proliferation
Your pt with nephritic signs has a negative immunofluorescence pattern (pauci-immune) and is postitive for p-ANCA. Dx?
- microscopic polyangiitis
- Churg-Strauss
Dx?
- an X-linked type IV collagen defect
Alport Syndrome
Dx?
- negative immunofluorescence pattern (pauci-immune)
- Wegener granulomatosis/granulomatosis with polyangiitis
- Churg-Strauss
Dx?
- Vasculitis 2° to IgA immune complex deposition
- triad of palpable purpura on buttocks/legs + arthralgias + ab pain
- post-URI
Henoch-Schönlein purpura
In rapidly progressive glomerulonephritis (RPGN), what are the crescents made of?
fibrin and macs
In Goodpasture syndrome, an antibody against the ____ and _____ create a linear immunofluorescence pattern.
collagen alveolar basement membrane
What causes nephritic syndrome?
GBM disruption
Dx?
- episodic gross hematuria with RBC casts
- respiratory or GI infection
- granular mesangial deposition of IgA
IgA nephropathy/Berger’s disease
What is rapidly progressive glomerulonephritis (RPGN)?
nephritic syndrome –> renal failure in weeks to months
What is Henoch-Schönlein purpura?
- Vasculitis 2° to IgA immune complex deposition
- triad of:
- palpable purpura on buttocks/legs
- arthralgias
- ab pain
- ***post-URI***
In ______, an antibody against the collagen and alveolar basement membrane create a linear immunofluorescence pattern.
Goodpasture syndrome
What is the tx for Wegener granulomatosis/granulomatosis with polyangiitis?
- cyclophosphamide
- corticosteroids
What is cyclophosphamide? What is it used to treat?
- an alkylating agent that cross-links DNA at guanine
- tx for CA, vasculitis
What causes Alport Syndrome?
an X-linked type IV collagen defect
What is microscopic polyangiitis?
- a small vessel necrotizing vasculitis involving lung, kidney, and skin
- NO nasopharyngeal involvement
- p-ANCA positive
Dx?
- hypercellular, inflamed glomerulus
- granular immune complex deposition
- subepithelial humps
post-strep glomerulonephritis (PSGN)
What is the most common nephropathy?
IgA nephropathy/Berger’s disease
What does C5a attract?
neutrophils
What virulence factor of Strep causes postpost-strep glomerulonephritis (PSGN)?
M protein
What is seen on EM in IgA nephropathy?
mesangial immune complex deposits
What are the biopsy findings in post-strep glomerulonephritis (PSGN)?
- hypercellular, inflamed glomerulus
- granular immune complex deposition
- subepithelial humps
Ddx?
- granular immunofluorescence pattern
- post-strep glomerulonephritis (PSGN)
- diffuse proliferative glomerulonephritis
- SLE
What is Churg-Strauss?
- a small vessel vasculitis
- p-ANCA positive
- s/s include:
- skin nodules/purpura
- peripheral neuropathy
- granulomatous inflammation
- eosinophilia
- asthma
- sinusitis
- coronary arteritis
Dx?
- skin nodules/purpura
- peripheral neuropathy
- granulomatous inflammation
- eosinophilia
- asthma
- sinusitis
- coronary arteritis
- p-ANCA positive
Churg-Strauss
This is a c-ANCA positive small vessel vasculitis involving the lung, kidneys, and skin WITH nasopharyngeal involvement.
Wegener granulomatosis/granulomatosis with polyangiitis
Dx?
- URI: sinusitis
- Lower respiratory tract: hemoptysis, cough, dyspnea
- Renal: hematuria, red cell casts, RPGN with crescents
- c-ANCA positive
Wegener granulomatosis/granulomatosis with polyangiitis
Your pt with nephritic signs has a negative immunofluorescence pattern (pauci-immune) and is postitive for c-ANCA. Dx?
Wegener granulomatosis/granulomatosis with polyangiitis
Dx?
- a small vessel necrotizing vasculitis involving lung, kidney, and skin
- NO nasopharyngeal involvement
- p-ANCA positive
microscopic polyangiitis
