Renal Pathology Pt. 2 Flashcards by Allison Crecelius

what are the causes of nephritic syndrome?

post-streptococcal/post-infectious glomerulonephritis (acute proliferative)

rapidly progressive (crescentic) glomerulonephritis

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what is acute proliferative glomerulonephritis?

diffuse proliferation of glomerular cells associated with influx of leukocytes caused by immune complexes

*most common is post-strep

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what are the pathologic characterizations of acute proliferative glomerulonephritis?

hypercellularity
leukocyte infiltration
granular deposits of IgG, IgM and C3
subepithelial “humps” of immune complexes

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what is the clinical presentation of post-strep glomerulonephritis in children?

malaise, fever, nausea, oliguria and hematuria 1-2 weeks after recovery from a sore throat

dysmorphic RBC or RBC casts with mild proteinuria

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what is rapidly progressive glomerulonephritis?

syndrome associated with severe glomerular injury - typically immunologically mediated

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what are the classifications of RPGN?

Type 1 - anti-GBM antibody
Type 2 - immune complex
Type 3 - ANCA +/Pauci immune

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what is Goodpasture syndrome?

Type 1 RPGN
anti-basement membrane antibody (IgG) deposition in kidneys and lungs
manifests as pulmonary and renal hemorrhage

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what HLA subtype is associated with Goodpasture?

HLA-DRB1

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How is Goodpasture treated?

plasmaphoresis

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what diseases are associated with Type 2 RPGN?

acute proliferative glomerulonephritis
SLE
IgA nephropathy
Henoch-Schonlein purpura

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what is the definition of Type 3/Pauci immune RPGN?

lack of detectable anti-GBM antibodies or immune complexes by immunofluorescence or electron microscopy

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what is typically found on serology with Type 3 RPGN?

p-ANCA or c-ANCA

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what disorders are associated with type 3 RPGN?

vasculitides such as Wegeners or polyangiitis

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what are the causes of nephrotic syndrome?

Membranous nephropathy
Minimal change disease
Focal segmental glomerulosclerosis
Membranoproliferative glomerulonephritis
IgA nephropathy

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what is membranous nephropathy?

diffuse thickening of the glomerular capillary wall due to the accumulation of IgG deposits along the basement membrane

can be primary (idiopathic) or secondary

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what is the pathogenesis of primary membranous nephropathy?

autoimmune disease linked to PLA-2 Ab

what are common causes of secondary membranous nephropathy?

drugs (NSAIDs)
malignancy
SLE
infections 
other autoimmune disorders (thyroiditis)

what are the histological hallmarks of membranous nephropathy?

effaced foot processes (podocytes)
thick basement membrane
subepithelial IgG deposits

what is minimal change disease?

podocytopathy

relatively benign disorder characterized by effacement of podocytes detectable only by electron microscopy

most frequent cause of nephrotic syndrome in children

what is the peak incidence of minimal change disease?

2-6 y/o

what features of minimal change disease indicate its immunologic basis?

immune dysfunction often following respiratory infection or routine prophylactic immunization
responsive to steroids/immunosuppressives
association with other atopic disorders
increased incidence with certain HLA haplotypes
increased incidence in patients with Hodgkin lymphoma

what is focal segmental glomerulosclerosis?

podocytopathy

sclerosis of some but not all glomeruli with only a portion of the capillary tuft involved

most common cause of nephrotic syndrome in adults

what are the classifications of FSGS?

primary/idiopathic *most common*
in association with other conditions
secondary event due to antecedent glomerular injury
adaptive response to loss of renal mass
hereditary

how does FSGS differ from MCD?

higher incidence of hematuria, reduced GFR and HTN
proteinuria tends to be non-selective
poor response to steroids
significant progression to CKD

what is termed the "collapsing variant" FSGS?

HIV associated FSGS

what gene variation is associated with FSGS and renal failure in patients of African descent?

APOL1 on Chr. 22

what is membranoproliferative glomerulonephritis?

considered a pattern of immune-mediated injury to the mesangial cells or capillary loops

what are the classes of MPGN?

Type 1 - IgG and complement deposition | Type 2 - complement deposition and activation

what are the histologic findings of MPGN?

alterations in the glomerular basement membrane proliferation of glomerular cells leukocyte infiltration

what is the clinical presentation of primary MPGN Type 1?

typically occurs in children or young adults nephrotic syndrome with nephritic features mild HTN 50% of patients develop CKD

what is the clinical presentation of secondary MPGN Type 1?

occurs exclusively in adults chronic antigenemia causing immune complex deposition associated with other disease or malignancy 50% of patients develop CKD

what is the clinical presentation of MPGN Type 2/Dense Deposit Disease?

generally occurs in adults hematuria 50% have nephritic syndrome poorer prognosis than MGPN Type 1

what is the pathogenesis of MPGN type 2?

C3NeF or "nephritic factor" IgG autoantibody binds C3 convertase leading to continuous activation of the alternative complement pathway

what two diseases often manifest with IgA nephropathy?

Berger disease | Henoch-Scholein purpura

what are the clinical characteristics of IgA nephropathy?

older children and young adults most common Caucasian and Asian male > female genetic susceptibility hematuria with the presence of significant infection (URI/respiratory/GI/abscess) association with gluten enteropathy and liver disease

what is chronic glomerulonephritis?

end-stage glomerular disease may occur with or without an antecedent history of acute glomerulonephritis

what primary glomerular diseases are most likely to progress to chronic glomerulonephritis?

``` crescentic GN (90%) focal segmental GS (50-80%) membranoproliferative GN (50%) membranous nephropathy (30-50%) IgA nephropathy (30-50%) ```