Nephrotic and Nephritic syndromes Flashcards
Nephrotic syndrome
- -Proteinurina >3.5 g/ day *
- -Hypoalbuminemia
- Hypogammaglobulinemia (increased risk of infection)
- Hyper coagulable state (loss of antithrombin III)
- -Hyperlipidemia and hypercholesterolemia
- edema
Nephritic syndrome
characterized by glomerular inflammation and bleeding
- Proteinuria less than 3.5 grams per day
- Hypertension due to salt retention
- Azotemia (increase BUN and creatinine)
- oliguria
- RBC casts
- Hematuria
Who are the nephritis syndromes
- Acute post streptococcal glomerulonephritis
- Rapidly progressive glomerulonephritis
- IgA nephropathy (Berger disease)
- Alport syndrome
- Membranoproliferative glomerulonephritis
Who are the nephrotic syndromes
- Focal segmental glomerulosclerosis
- Minimal change disease
- Membranous nephropathy
- Amyloidosis
- Diabetic glomerulonephropathy
Acute post streptococcal glomerulonephritis
LM?
IF?
EM?
Nephritic
LM: glomeruli enlarged and hyper cellular
IF: “starry sky”; “Lumpy-Bumpy” due to IgG, IgM, and C3 deposition along GBM and mesangium
EM– subepithelial immune complex (IC) humps
Clinically – children, 2 week after group A streptococcal infection; Type III hypersensitivity reaction
Coca-colored urine, HTN
Increase in anti- DNase B titers
Tx: supportive. Children rarely progress to renal failure, however, it is more likely for adults to progress to renal failure
Rapidly progressive glomerulonephritis
LM?
IF?
subtypes?
Nephritic
LM and IF crescent moon shape; crescents consist of fibrin and plasma proteins
Goodpasture syndrome – type II hypersensitivity; antibodies to GBM and alveolar basement membrane –> Linear IF
Granulomatosis with polyangiitis – PR3-ANCA/ c-ANCA
Microscopic polyangiitis MPO-ANCA
Diffuse proliferative glomerulonephritis (DPGN)
LM?
IF?
EM?
Nephritic
due to SLE or membranoproliferazive glomerulonephritis
LM: wire looping of capillaries
IF: granular
EM: subendothelial IgG-based ICs often with C3
IgA nephropathy (Berger disease)
Nephritic
Renal pathology of Henoch-Schonlein purport
LM- mesangial proliferation
IF: IgA based IC deposits in mesangium
EM: mesangial IC depositis
childhood disease and happens a few days after an upper respiratory tract infection
Alport syndrome
cause?
Nephritic
Mutation in type 4 collagen (basement menbranes)
X-linked
“Can’t see, Can’t pee, can’t hear high C”
Basket weave appearance on EM
Membranoproliferative glomerulonephritis
Type 1 and type 2?
LM of type 1?
Cause of type 2?
Any associations?
Nephritic and Nephrotic presentation
Type 1: sub-endothelial immune complexes
LM: Tram track appearance on PAS stain and Hand E stain due to GBM splitting due to mesangial ingrowth
Type 1 may be secondary to HepB or HepC infection
Type 2: intramembranous IC deposits
-associated with C3 nephritic factor : stabilizes C3 converts — decrease serum C3 levels
Focal segmental glomerulosclerosis
LM?
IF?
EM?
Population?
Nephrotic
LM: segmental sclerosis and hyalinosis
IF: deposits of IgM, C3, and C1
EM: effacement of foot process similar to minimal change disease
Most common in african americans and hispanics and HIV patients.
Minimal change disease
LM?
IF?
EM?
Population?
Tx?
Nephrotic – aka Lipoid nephrosis
LM: normal
IF: normal
EM: effacement (fusion) of foot processes
Children; linked to hodgkin Lymphoma and changes are believed to be due to the cytokines present from lymphoma
Highly responsive to corticosteroids
Membranous Nephropathy
LM?
IF?
EM?
Population?
Nephrotic presentation of SLE
LM: GBM thickening
IF: granular due to immune complex deposition.
EM: “Spike and Dome” – subepithelial deposits
Most common in caucasian adults
Associated with IgG4 antibodies to phospholipase A2 receptor
Primary disease has a poor prognosis.
Amyloidosis
LM?
Associations?
Nephrotic
LM: Congo red stain shows apple-green birefringence under polarized light
- associated with chronic conditions such as: Multiple Myeloma, TB, Rheumatoid arthritis
Diabetic glomerulonephropathy
What is classically seen on LM?
Nephrotic
LM: Mesangial expansion, GBM thickening, EOSINOPHILIC nodular glomerulosis
- Kimmelstiel-wilson lesions
Increase permeability
