Nephritic Syndrome Flashcards
What characterizes glomerular disorders in nephritic syndrome?
- Glomerular inflammation
2. Bleeding
What four other symptoms are seen in Nephritic Syndrome?
- Limited proteinuria (
What does salt retention cause in Nephritic Syndrome?
Periorbital edema and hypertension
What causes the red blood cell casts seen in Nephritic Syndrome?
Glomerular Bleeding
What does biopsy reveal in Nephritic Syndrome?
Hypercellular, inflammed glomeruli
What are RBC casts a sign of?
Glomerular bleeding
What causes the hypercellularity seen in Nephritic Syndrome?
- Immune-complex deposition activates complement
2. C5 attracts neutrophils which mediate damage –> hypercellularity and inflammation
What is Post-Streptococcal Glomerulonephritis (PSGN)?
Nephritic syndrome that arises after group A beta hemolytic streptococcal infection of the (1) skin (impetigo) or (2) pharynx
What two things may cause PSGN?
- Occurs with nephritogenic strains of strep.
2. May occur after infection with non-streptococcal organisms as well
What are nephritogenic strains of strep?
They carry M protein virulence factor!!
When does PSGN present?
2-3 weeks after infection
What signs and symptoms does PSGN present with?
- Hematuria (cola-colored urine)
- Oliguria
- Hypertension
- Periorbital edema
What populations is PSGN usually seen in?
Children, but it may occur in adults
What do you see on H&E with PSGN?
Hypercellular, inflamed glomeruli
What mediates PSGN? What do you see on IF?
Mediated by immune complex deposition (granular IF)
What do you see on EM of PSGN?
Subepithelial ‘humps’
What is the treatment for PSGN?
Supportive
How many adults and children with PSGN progress to renal failure?
- Children rarely progress to renal failure (1%)
- Some adults (25%) develop rapidly progressive glomerulonephritis (RPGN)
What is Rapidly Progressive Glomerulonephritis (RPGN)?
Nephritic syndrome that progresses to renal failure in weeks to months
What is the first and second thing you do when working up RPGN?
- Biopsy
2. IF to determine etiology
What are the subepithelial ‘humps’ seen in PSGN?
Immune complexes sitting on the basement membrane –> where subepithelial cells would normally sit
What is seen on H&E stain in RPGN?
RPGN is characterized by crescents in Bowman’s space (of glomeruli)
What are crescents comprised of?
Fibrin and Macrophages
What should you use to help figure out the etiology (cause) of RPGN?
Clinical picture + IF
What nephrotic syndrome is SLE associated with?
Membranous Nephropathy
What nephritic syndrome is SLE associated with?
RPGN
What is a p-ANCA?
Perinuclear - when antibodies from patient serum bind adjacent to the nucleus
What is a c-ANCA?
Cytoplasmic - when antibodies bind in the cytoplasm
What disease is indicated by a Linear (anti-basement membrane antibody) IF?
Goodpasture Syndrome
What is the pathogenesis of Goodpasture syndrome?
Antibody against collagen in glomerular and alveolar basement membranes
What does Goodpasture syndrome present with?
Hematuria and hemoptysis
What population is Goodpasture syndrome most common in?
Young, adult males
What diseases are indicated by Granular (immune complex deposition) IF?
PSGN (most common)
OR
Diffuse proliferative glomerulonephritis
What causes diffuse proliferative glomerulonephritis?
Diffuse antigen-antibody complex deposition, usually sub-endothelial
What is Diffuse proliferative glomerulonephritis (form of RPGN) common in?
Most common type of renal disease in SLE
[And renal disease is the most common cause of death in SLE]
What diseases are indicated by Negative IF (pauci-immune)?
- Wegener granulomatosis
- Microscopic polyangiitis
- Churg-Strauss Syndrome
What test finding is Wegener granulomatosis associated with?
+ c-ANCA
What systems does Wegener granulomatosis effect?
- Lung
- Kidney
- Nasopharyngeal
What if a patient presents with RPGN and recurrent sinus infections?
Think about Wegener granulomatosis (c-ANCA can be used to separate it from Goodpasture syndrome)
What conditions are associated with + p-ANCA?
- Microscopic Polyangiitis
- Churg-Strauss Syndrome
What three things distinguish Churg-Strauss Syndrome from Microscopic Polyangiitis?
- Granulomatous inflammation
- Eosinophilia
- Asthma
What is the most common nephropathy worldwide?
IgA Nephropathy (Berger Disease)
What causes IgA Nephropathy (Berger disease)?
IgA immune complex deposition in mesangium of glomeruli
Where does IgA deposit in IgA Nephropathy?
Mesangium of glomeruli
When do patients usually present with IgA Nephropathy?
Childhood
What signs and symptoms does IgA Nephropathy usually present with?
- Episodic gross or microscopic hematuria
- RBC casts
What does IgA Nephropathy usually follow?
Mucosal Infections (e.g. gastroenteritis)
What is the pattern of disease in IgA Nephropathy?
Relapsing and remitting (symptomatic and asymptomatic) and may lead to renal failure
Production of what is increased during infection?
IgA production
What is seen on IF in IgA Nephropathy (Berger Disease)?
IgA immune complex deposition in the mesangium
What may IgA Nephropathy slowly progress to?
Renal Failure!
What is Alport syndrome?
Inherited defect in type IV collagen.
What is the inheritance associated with Alport syndrome?
Most commonly X-linked
What does the defect in collagen in Alport syndrome result in?
Thinning and splitting or the glomerular basement membrane
What does a patient with Alport syndrome present with?
- Isolated hematuria
- Sensory hearing loss
- Ocular disturbances
