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28 Cards in this Set

  • Front
  • Back
Renal Diseases which are Secondary to Systemic Diseases
Systemic Lupus Erythematosus (SLE)
Diabetes Mellitus
Amyloidosis

Bacterial Endocarditis–Associated Glomerulonephritis
Henoch-Schonlein Purpura
Fibrillary Glomerulonephritis and Immunotactoid Glomerulopathy
Goodpasture syndrome
Thickening of GBM and Mesangial expansion (Diffuse glomerulosclerosis)
Kimmelsteil-Wilson nodules (Nodular glomerulosclerosis)
Arteriolar hyalinosis (pathognomonic when changes in both afferent and efferent arterioles)
Diabetic Nephropathy (Nephrotic syndrome)
Amyloidosis effect on the kidney
Particularly glomeruli, causes proteinuria, advanced cases uremia and death
Nephritic syndrome diseases
IgA nephropathy
Poststreptococcal glomerulonephritis
Rapidly progressing glomerulonephritis
Membranoproliferative glomerulonephritis
What are red cell casts?
Always pathologic
Yellowish-brown color
Generally cylindrical with sometimes ragged edges; their fragility makes inspection of a fresh sample paramount
Usually associated with nephritic syndromes
Strongly indicative of glomerular damage
what are granular casts?
Result either from the breakdown of cellular casts or the inclusion of aggregates of plasma proteins
Based on size of inclusions, classified as fine or coarse
Seen in renal parenchymal disease
Appears 1-4 weeks after a group A Beta hemolytic streptococcal infection of pharynx or skin in children 6-10
Post Streptococcal Glomerulonephritis. Immunologically mediated
Post Streptococcal Glomerulonephritis lab findings
elevated antibody titres against streptococcal antigens, lower serum C3 and cryoglobulins in the serum
-Latent period between infection and onset of nephritis is due to the time needed for antibody and immune-complex production
Post Streptococcal Glomerulonephritis Immunofluoresence
Electron microscopy
Immunofluoresnce: Coarse granular immunofluorescence for IgG and C3-lumpy bumpy as in starry sky pattern
EM: Electron dense humps on the epithelial side of the bsmt membrane (subepithelial deposits)
Focal proliferative glomerulonephritis
Hematuria
Proteinuria

Moderate increase in mesangial matrix and mild mesangial hypercellularity
IgA Nephropathy/Henoch-Schonlein purpura
IgA Nephropathy
immunofluorescence
EM
Immuno: uniform mesangial positivity for IgA

EM:Mesangial deposits of IgA
Clinical-pathologic syndrome characterized by the accumulation of cells in the Bowman space as crescents with rapid decline in renal function
Proliferation of parietal cells in Bowman space
Crescents eventually undergo sclerosis
Crescentic Glomerulonephritis/ Rapidly Progressive Glomerulonephritis
Rapidly Progressive (Crescentic) Glomerulonephritis Type I:
Anti-GBM IgG and C3
Idiopathic
Goodpasture syndrome
Rapidly Progressive (Crescentic) Glomerulonephritis Type II
: Immune complex
-Idiopathic
-Post-infectious
-Systemic lupus erythematosus
-Henoch-Schonlein purpura (IgA)
Rapidly Progressive (Crescentic) Glomerulonephritis Type III
Pauci-immune (ANCA associated)
Idiopathic
Wegener granulomatosis
Microscopic polyarteritis nodosa
In goodpasture syndrome Rapidly Progressive (Crescentic) Glomerulonephritis presents with
Nephritic syndrome with hemoptysis and antiglomerular basement membrane antibodies the noncollagenous (NC1) domain of the alpha 3 chain of type IV collagen
Good pasture syndrome elisa testing
NEGATIVE
Normal glomerulus incubated with patient’s serum shows no GBM positivity, indicating that no anti-GBM antibody is present in the patient’s serum.

+
Normal glomerulus stained with serum from a patient who has anti-GBM antibodies
Focal Necrotizing glomerulonephritis, Pauci-Immune,
due to Wegener Granulomatosis
microscopic findings
Pulmonary and renal lesions
Serum c-ANCA positive
Fibrinoid necrosis
Pyknotic nuclei and nuclear fragments
Hypercellularity and early crescent formation
PAS-fibrin
IF-fibrinogen
EM-fibrin
Thin Basement Membrane Disease Differential Diagnosis with Alport Syndrome
Bengin familial hematuria
Heredofamilial disorder or sporadic
Glomerular basement membrane thinning
Hematuria/proteinuria
20-25% of pediatric/adult patients with persistent isolated hematuria
ADULTS <250 nm
Alport syndrome (Hereditary nephritis) has a triad of symptoms
Nephritis, nerve deafness, cataracts. It first presents with hematuria and erythrocyte casts
What causes Alport syndrome?
X-linked disorder that results in the mutation of the alpha 5 chain of collagen type 4.
Alport syndrome EM
Irregular foci of thickening or attenuation in the glomerular basement membrane with longitudinal splitting of the lamina densa
Diseases Presenting as Both Nephritic and Nephritic Syndrome
Membranoproliferative Glomerulonephritis
(MPGN)
Systemic Lupus Erythematosus
Membranoproliferative (Mesangiocapillary) Glomerulonephritis type 1 and 2 general characteristics
1)mesangiocapillary glomerulonephritis seen in SLE, hep B and c and involves the alternative pathway activation as well as the classical so deposits of C3, C4 and C1q
2) (Dense deposit disease and involves only the alternative pathway so C3 deposits. Recurs in transplant kidney

Most pts with Membranoproliferative Glomerulonephritis are under 30!
Membranoproliferative Glomerulonephritis type 1 and 2 EM
TYpe 1: Subendothelial dense deposits
Type 2: Dense sausage-like, deposits within glomerular bsmt membrane
Membranoproliferative Glomerulonephritis type 1 and 2 light microscopy reveals a characteristic feat
Railroad track or tram track appearance of the replicated basement membrane:
Membranoproliferative Glomerulonephritis type 2 can be screened by circulating antibody
autoantibody called C3 nephritic factor, which binds to the C3 convertase of the alternative pathway
The greatest contributions to Chronic glomerulonephritis are
Rapidly progressive glomerulonephritis
FSGS
Membranoproliferative GN