nephrotic & nephritic Flashcards
nephrotic syndrome diseases
Minimal change disease, FOCAL SEGMENTAL GLOMERULOSCLEROSIS, Membranous GN
Nephritic Syndrome diseases
ACUTE DIFFUSE PROLIFERATIVE GN, RAPIDLY PROGRESSIVE GLOMERULONEPHRITIS, IgA NEPHROPATHY, MEMBRANOPROLIFERATIVE GN
lipoid nephrosi
Minimal change disease
nil change disease
Minimal change disease
Minimal change disease etiology
Dysfunction of T-cells
Minimal change disease associated with
Podocytes Fusion (foot processes) of epithelial cells
Almost normal glomerulus by Light Microscope (LM)
Most common cause of Nephrotic Syndrome in children
Minimal change disease
Minimal change disease incidence in adults and children
10%, 65%
Minimal change disease age group
2-6 y 1w after uti or immunization
Minimal change disease distungsting feature
selective protinuria
Minimal change disease clinical course
selective proteinuria, normal renal function & respond to steroid
Minimal change disease labratory findingds
lm normal
if negative
em podocytes fusion
FOCAL SEGMENTAL GLOMERULOSCLEROSIS etiology
HIV, heroin addiction, obesity, SCd, glomerular scarring, maladaptation, inherited & primary disease
FOCAL SEGMENTAL GLOMERULOSCLEROSIS congenital forms:
mutations at APOL1 on ch.22 and increased risk of RF and FSGS, in africans
These mutations affect cytoskeletal or related proteins in podocytes (Nephrin)
FOCAL SEGMENTAL GLOMERULOSCLEROSIS lm
Collapse of BM
↑ mesangial matrix
Deposition of hyaline masses (hyalinosis)
FOCAL SEGMENTAL GLOMERULOSCLEROSIS em
Loss of podocytes.
Focal denudation of epithelial cells.
FOCAL SEGMENTAL GLOMERULOSCLEROSIS if
IgM & C3 deposition.
FOCAL SEGMENTAL GLOMERULOSCLEROSIS clinical
higher incidence of hematuria, ↓ GFR, & HT.
Nonselective proteinuria
poor response to steroids.
FOCAL SEGMENTAL GLOMERULOSCLEROSIS incidence
10% CHILDREN, 35% ADULTS
Membranous GN incidence
Adults 30%, children 5%
Membranous GN autoantibodies
M-type phospholipase A2 receptor–70% of the cases (important), Aldose reductase, Maganese superoxide dismutase 2,Membrane metalloendopeptidase.
Membranous GN most common autoantibodies
M-type phospholipase A2 receptor
Membranous GN prognosis
33% spontaneously remission.
33% stable with proteinuria.
33% progress to ESRD
Membranous GN LM
Thick capillary Wall without proliferation
Silver stain: spikes
wire loop lesion.
Membranous GN EM
Diffuse subepithelial deposits.
Membranous GN if
Granular IgG & C3 deposition.
Nephritic Syndrome manifestation
hematuria, htn, high blood urea, high serum creatinine, edema
Nephritic Syndrome pathogensis
Inflammatory lesions of glomeruli
Nephritic Syndrome clinical
Reduced GFR, htn, oliguria, fluid retention, and azotemia
ACUTE DIFFUSE PROLIFERATIVE GN etiology
1-4w after group a streptococcus b hemolytic infection
ACUTE DIFFUSE PROLIFERATIVE GN pathogensis
↑ ASO titer (Anti-streptolysin O)
Immune-complex disease/ Circulating or implanted Ag ↓ serum complement
Implicated Ags, SpeB, GAPDH, endostreptosin & plasmin binding protein
ACUTE DIFFUSE PROLIFERATIVE GN lm
Diffuse proliferation & leukocytic infiltration
ACUTE DIFFUSE PROLIFERATIVE GN em
subepithelial humps
ACUTE DIFFUSE PROLIFERATIVE IF
granular IgG & C3 in GBM and Mesangium
ACUTE DIFFUSE PROLIFERATIVE clinical
Gross hematuria & red casts; mild proteinuria
ACUTE DIFFUSE PROLIFERATIVE children prognosis
> 95% recovery ,1% RPGN ,2% CRF
ACUTE DIFFUSE PROLIFERATIVE adult prognosis
15-50% develop ESRD
RAPIDLY PROGRESSIVE GLOMERULONEPHRITIS types
Anti-GBM antibody–mediated crescentic GN
Pauci-immune type crescentic GN:
Immune-complex–mediated crescentic GN
presence of crescents
RPGN
Anti-GBM antibody–mediated crescentic GN if
Linear pattern for IgG & C3
Anti-GBM antibody–mediated crescentic GN incidence
least common, 12%
hemoptysis & hematouria
Good Pastus syndrome
Anti-GBM antibody–mediated crescentic
Immune-complex–mediated crescentic GN IF
igG & c3
Immune-complex–mediated crescentic GN incidence
44%
Immune-complex–mediated crescentic GN seen in
Primary diseases (post-infect, IgA Nephtopathy)
Systemic (SLE, Henoch-Schoenlein purpura)
Idiopathic
Pauci-immune type crescentic GN IF
negative
Pauci-immune type crescentic GN incidence
44%
ANCA
Pauci-immune type crescentic GN
systemic vasculitis is associated with
Pauci-immune type crescentic GN
rpgn lm
> 50-75% of glomeruli contain crescents
Some areas are still look good.
NL or focal proliferative changes.
systemic vasculits types
Microscopic Polyangiitis
Granulomatosis with polyangiitis.
rpgn em
rupture of GBM only or with electron-dense deposits
rpgn prognosis
More than 80% bad prognosis
rpgn therapy
steroid, cytotoxic, long term dialysis, renal transplant & Plasmapheresis
Plasmapheresis used in
Anti-GBM and pauci-immune
Commonest type of GN
IgA NEPHROPATHY
IgA NEPHROPATHY incidence
10% children 15% adults
IgA NEPHROPATHY etiology
1-2d after uti
IgA NEPHROPATHY pathogensis
abnormality leading to ↑IgA synthesis after Antigenic stimulation, Circulating IgA aggregates or complexes entrapped in mesangium(have CD71), Activation of alternative pathway of complement
Secondary IgA nephropathy
Celiac disease: ↑ IgA secretion due to mucosal inflammation
Liver disease: liver is responsible for clearance of IgA from.
IgA nephropathy LM
Normal (initially) to focal or diffuse proliferative
IgA nephropathy em
electron-dense deposits in the mesangium
IgA nephropathy if
diffuse, usually global mesangial IgA in all cases
IgA nephropathy prognosis
Initial benign course but slowly progressive
20-50% progress to CRF in 20 yrs
20 -60% recur in transplants.
IgA nephropathy Bad prognostic features:
Old age
Heavy proteinuria
Associated HTN
Glomerular sclerosis
MEMBRANOPROLIFERATIVE GN etiology
Chronic immune-complex disorder (SLE, HCV, HBV, HIV) Chronic bacterial infections, schistosomiasis
Malignant conditions (CLL, lymphoma, melanoma)
MEMBRANOPROLIFERATIVE GN incidence
10% in both
MEMBRANOPROLIFERATIVE GN principal presention
nephrotic sy, may begin as acute nephritis or mild proteinuria
MPGN type 1 lm
tram-tracking (thickened reduplicated capillaries)
Tubulointerstitial changes & vascular changes of HTN.
Lobular accentuation
Enlarged glomeruli, proliferation + inflammatory infiltrate
MPGN type 1 em
Subendothelial deposits
Circumferential mesangial interposition
Increase in mesangial cells & matrix.
MPGN type 1 if
C3, C1q, C4 in granular pattern in mesangial area.
mpgn occures in transplanr
Dense Deposit Disease
Dense Deposit Disease
Abnormality that lead to activation of alternative pathway in the presence of C3 nephritic facto (Auto-Ab that stabilizes C3 convertase)
Normal C1 & C4, Low factor B & properdin
Dense Deposit Disease LM
hypercellular glomeruli, duplicated BM & ↑ mesangial matrix
Dense Deposit Disease em
Irregular, Ribbon like, extremely dense structure of the lamina densa & subepithelail space of the GBM
Dense Deposit Disease if
Granular mesangial deposits
Short or discontinuous linear capillary loop deposits of C3.
No early complement components or Ig
mpgn prognosis
40%: progress to ESRF
30%: variable degree of renal insufficiency
30%: persistent nephrotic S without RF.
which worse prognosis mpgn 1 or ddd
ddd
Alport syndrome clincal course
Ear (cochlea): nerve deafness
Eye (cornea): eye disorders.
Kidney (glomerulus): Nephritis
Alport syndrome sex incidence
m>f
HEREDITARY NEPHRITIS
Alport syndrome, THIN MEMBRANE DISEASE
Alport syndrome lm
Secondary sclerosis later
Foam cells (histiocytes) in the interstitium.
Alport syndrome em
GBM shows irregular thickening, lamination
splitting (“basketweave” appearance)
Alport syndrome etiology
Mutation in encoding for alpha-5 chain of collagen type IV
Alport syndrome prognosis
CRF in 20 yrs
THIN MEMBRANE DISEASE etiology
Mutation in gene encoding alpha 3&4 chain of collagen type IV
THIN MEMBRANE DISEASE prognosis
excellent, no systemic manifestation, no progression to rf
