Glomerular Diseases Flashcards
What glomerular nephropathies are primarily pediatric diseases
minimal change disease and post-striptococcal muriel and nephritis
What are the histologic characteristics of minimal change disease
Normal glomerular membrane with lipoid nephrosis
collapsing glomerulo nephropathy is associated with what
Remember the 4-H’s: Hiv, heroin, hemonopathies, heavy
Compare and contrast the histologic characteristics of collapsing and idiopathic FSGS
collapsing: Very poor prognosis; Epithelioid hyperplasia
Idiopathic: Prognosis is variable; degeneration of the glomerular epithelium
What are common diseases and drugs that cause secondary Membranous nephropathies
Thrombotic vascular diseases; NSAIDs; penicillamine; carcinomas of the lung, colon, & melanoma
Describe the histological characteristics of FSGS
focal Sclerosis and hyalinosis With the destruction of potocytes
Describe the pathogenesis of minimal change disease
cell cytokine storm triggers damage to the foot processes of the potocytes
70% of Membranous neuropathies are associated with deposits of IgG immune complexes. What is the pathogenesis Of these IGG deposits?
Antibodies against phospholipase A2 receptors Activate compliment C5b-C9 Which triggers the deposition of IgG complexes in the sub endothelial layer of the glomerular membrane leading to membrane thickening
describe the histologic features of memberness nephropathy
granular deposits in a spike in dome pattern
What is the pathogenesis of diabetic glomerulonephropathy
Extensive glycosylation of the renal vessels Leads to arterial or membrane thickening; this increases the pressure within the glomeruli and expands the mesangium; this leads to subsequent sclerosis and glomerular hypertrophy
Describe the histologic characteristics of diabetic glomerulonephropathy
arterial hyalinosis; kimmelstiel-wilson nodules in mesangial matrix; Gbm thickening
diabetic glomerulonephroth also causes what complication
4 collagen microangiopathy
What are the unique histologic features of amyloidosis neuropathy
Amyloid deposits within the mesangium; Apple Green Bifringements with bence Jones proteins
Membranous neuropathy is a generic term That can be associated with any disease that causes what?
thickening of the glomerular membrane Isolated to the sub epithelial layer
What nephropathies are isolated to just the mesangium and not the glomerular membrane
Amyloidosis neuropathy And IgA nephropathy; Class I & II SLE nephritis
What nephropathies are isolated to just the glomerular membrane and do not affect the mesagnium
FSGS; Membranous Nephropathy; Alport syndrome And thin basement membrane nephropathy; Class V & VI SLE nephritis
Membrano proliferative comarial nephritis Can cause either a nephronic or nephrotic syndrome. What do Both subtypes of this disease have in common
Both produce a granular pattern along the GBM And mesangium; both will have low serum levels of C3
What is the pathogenesis of type 1 Member no proliferative glomerulonephritis
strong association with hepatitis C; Immune complex deposits mediated by classical complement
What is the pathogenesis of type 2 membrano proliferative glomerulonephritis
Antibody mediated by alternative compliment; Antibodies bind to C3 convertase preventing its degradation and Subsequently overactivating complement
compare and contrast the histologic characteristics of type 1 and type 2 membrano proliferative glomerulonephritis
one: Deposits in the sub endothelial layer of the basement membrane and in the mesangianm
two: intra memberous deposits within the gbm in a ribbon like fashion
Because biopsies are expensive and invasive, wood lab can be used to differentiate type 1 and type 2 membrano proliferative glomerulonephritis
C4 serum levels
Type 1: low
Type 2: normal
Iga nephropathy typically becomes symptomatic in which decade of life
second and third decade
Having a history of what diseases are strongly associated with IGA nephropathy AKA burger disease
Complicated respiratory infections; in stage liver and celiac disease
Describe the pathogenesis of IGA nephropathy
Thought to be due to immunological stresses and insults in the 1st and 2nd decades of life:
Respiratory tract infections can activate expression of GD-IgA; This triggers an autoimmune response against IG-IgA isotypes Which deposit in the mesangium; Subsequent activation of alternative complement causes damage to the glomeruli
What are the histologic features of IGA neuropathy
The sangrial cell hyperplasia And protein deposits within the mesangianm
Describe the pathogenesis Of PSGN
virulent factor: M protein Allows s. pyogenes to persist and survive inside Lukiosites And cause reactivation of infection elsewhere in the body; When these leuke sites deposit and the kidney it activates complement And causes subsequent damage to the glomerulus and the mesandium
describe the distinct histologic characteristics of psgn
PMN Hyperplasia in the mesangium; granular deposits along the eclomycular capillaries Creating a starry Sky Lumpy Bumpy appearance; +IgG & C3
Compare and contrast the different clinical manifestations of good pastures disease and granulomatosis with polyingitis
good pastures is primarily isolated to the lungs and kidneys; Whereas granulomatosis with polyangitis is a vascular disease that affects multiple systems and patients will typically present with complaints of nasal congestion due to nasal septum defects; They also have different antibodies: ANCA for GMP and anit GM for GP
GP Antibodies affect what Protein
alpha 3 chain of type 4 collagen; IgG Type 2 hypersensitivity
For good pasteur syndrome what we’ll be seeing with immunofluorescence that will not be seen with PMG
linear deposits
what are similarities between gp and gmp
Both will present with Crescent shaped glomerulo capillaries; hemoptysis
Both are categorized as subtypes of rapidly progressive glomerulonephritis
What are hallmarks of PSGN
Peri orbital edema; Recent history of a upper respiratory infection; decreased serum levels of C3 and normal serum levels of C4
lab work of Lupus Induced nephritis Low serum levels of both C3 and C4. Therefore which complement pathway has been activated
the classical pathway
what classes of sle nephritis are associated with glomerular crescents
classes 3 and 4
Describe the histologic characteristics of class 3 and 4 lupus induced nephritis
wire looping, crescents, mesangial and sub endothelial electron dense deposits
What antibodies are associated with sle
anti-ANA and anti-dsDNA
What is the pathogenesis of Alport syndrome?
X linked dominant inheritance pattern; mutation of the alpha 3 subtype of collagen 4
what are the clinical manifestations associated with Alport syndrome
Symptomatic onset 5-20 yrs. of age; hearing loss; orbital anomalies
what are the unique histologic characteristics Of Alport syndrome
Interstitial foam cells; Splitting and lamination of the lamina densa Giving a basket like appearance
Thin basement membrane neuropathy pathogenesis is Very similar to that of Alport syndrome. Difference between the two
thin basement membrane nephropathy is not associated at all with the clinical manifestations of Alport syndrome and the prognosis is very very good in comparison
