Glomerulonephritis Flashcards
The presence of crescents correlates with the syndrome of _____ and carries a ______ prognosis.
RPGN; ominous
How do children present with Post-infectious Glomerulonephritis?
with edema and sudden weight gain
What lab findings are assoc. with Post-infectious Glomerulonephritis?
elevated levels of antibodies to streptococcal antigens (ASO titer, streptozyme) and decreased levels of complement components C3 with normal levels of C4 suggesting alternate complement pathway activation.
What is the tx for Post-infectious Glomerulonephritis?
none- it’s usually self limiting
What is the tx for auci-immune small vessel vasculitis?
immunosuppressive drugs (high-dose steroids and cyclophosphamide or rituximab)
Who gets MPGN?
older children and adolescents, esp females
What is the pattern of immune complex deposition in anti-GBM disease?
linear
______ is a type of GN that classically occurs after a _____ infection.
Acute post-infectious GN; Group-A strep
Who gets Goodpasture’s Syndrome?
young males
What is the pattern of immune complex deposition in lupus patients?
lumpy bumpy
______ can cause a severe, rapidly progressive GN, which may be accompanied by pulmonary hemorrhage (Goodpasture’s syndrome) or may be renal-limited.
Anti-GBM nephritis
What is Pauci-Immune Renal Vasculitis?
a small vessel vasculitis without immune complex deposition
This is glomerulonephritis from binding of antibody to an antigens in type IV collagen within the GBM.
Anti-GBM Disease
How do IgA nephropathy pts present?
asymptomatic microhematuria , nonnephrotic proteinuria, and normal or only mildly reduced renal function
What is Goodpasture’s syndrome?
the triad of pulmonary hemorrhage, iron deficiency anemia and GN associated with circulating antibody to GBM
How does look histologically?
fibrinoid necrosis and crescents without immune complex deposition
IgA nephropathy is the most common type of acute glomerulonephritis and usually presents in which people?
males 15 to 35 years old
Name 9 diseases with systemic symptoms that are associated with glomerulonephritis.
- Henoch-Schönlein Purpura
- Goodpasture’s syndrome
- Lupus nephritis
- ANCA associated vasculitis
- Infection associated
- Post-infectious
- Endocarditis/shunt nephritis
- Cryoglobulinemia
- Immunoglobulin deposition diseases
MPGN refers to a histologic pattern in which there is both proliferation as well as thickening of the _____.
GBM
MPGN type I is most often idiopathic or associated with _____ infection.
HCV
The excretion of ____, _____, and _____ reflects glomerular inflammation and disruption of the GBM.
red cells, white cells and red cell casts
How is Anti-GBM disease treated?
aggressive treatment with steroids, immunosuppressive agents and plasma exchange
______ is the most common type of acute glomerulonephritis now seen worldwide and usually presents in males 15 to 35 years old.
IgA nephropathy
Post-infectious Glomerulonephritis is diagnosed in patients presenting with nephritic syndrome with a positive ______ test and low ____ levels.
streptozyme; C3
More than ____ of the patients with lupus develop clinically evident renal involvement.
half
______ should be suspected in any patient with known hepatitis C infection who develops renal disease.
Cryoglobulinemic glomerulonephritis
What is Anti-GBM Disease?
Glomerulonephritis from binding of antibody to an antigens in type IV collagen within the GBM
This syndrome is the triad of pulmonary hemorrhage, iron deficiency anemia and GN associated with circulating antibody to GBM.
Goodpasture’s Syndrome
In this disease, the renal lesion is similar to IgA nephropathy but more severe with a focal proliferative necrotizing glomerulonephritis, often with crescent formation, accompanied by mesangial and capillary wall deposits of IgA.
Henoch-Schonlein purpura (HSP)
How does proteinuria occur?
direct damage to the glomerular capillary wall induced by immunologic mechanisms leads to an increase in protein filtration
Which deposits form a characteristic “fingerprint” appearance?
cryoglobulinemia immune complexes
Name 6 diseases, limited to the kidneys, that are associated with glomerulonephritis.
- IgA nephropathy
- Anti-GBM disease
- Membranoproliferative glomerulonephritis (MPGN)
- Renal limited vasculitis (ANCA associated)
- Hemolytic-uremic syndrome
- C3 glomerulopathy/Dense deposit disease
What is pulmonary-renal syndrome?
renal disease with pulmonary hemorrhage
What is IgA nephropathy?
a form of mesangial proliferative glomerulonephritis in which there is a predominance of IgA immune deposits in the mesangium and rarely in subendothelial areas
What are the characteristics of nephritic syndrome?
- hematuria
- dysmorphic red blood cells and/or red blood cell casts
- proteinuria
This disease is thought to be mediated by the deposition of IgA immune complexes to the mesangium with activation of mesangial cells via the Fc alpha receptors, resulting in cell proliferation and matrix expansion.
IgA nephropathy
______ refers to a histologic pattern in which there is both proliferation as well as thickening of the GBM.
MPGN
How does Anti-GBM disease present?
pulmonary hemorrhage with iron deficiency anemia
Approximately 90% of patients with pauci-immune small vessel vasculitis have detectable ______.
anti-neutrophil cytoplasmic antibodies (ANCA)
What is the tx for IgA nephropathy?
ACE inhibitors and possibly steroids with/without azathioprine
Why does HTN occur in nephritic syndrome?
a consequence of salt and water retention
What will the GFR be in nephritic syndrome?
it’s reduced
This is a form of mesangial proliferative glomerulonephritis in which there is a predominance of IgA immune deposits in the mesangium and rarely in subendothelial areas.
IgA nephropathy
What does RPGN stand for?
rapidly progressive glomerulonephritis
Most patients with ______ symptomatic disease develop palpable purpura, arthralgias, and generalized weakness.
cryoglobulinemia
In nephritic syndrome, what will the urine protein value be?
generally less than 3.0 gm/day
Why does edema occur in nephritic syndrome?
an increase in tubular reabsorption of salt and water due to reduced, glomerular perfusion with well preserved tubular function leading to expansion of extracellular fluid volume
The classic clinical manifestations of _____ in children include skin lesions (palpable purpura), arthritis, GI involvement (colic and bleeding) and glomerulonephritis.
Henoch-Schonlein purpura (HSP)
This is a small vessel vasculitis without immune complex deposition.
Pauci-Immune Renal Vasculitis
Glomerular disease should be considered in patients presenting with ____ and/or _____.
proteinuria; hematuria
