Goal 5 Nephritic & Nephrotic syndromes

Question 1

Causes of Nephritic syndrome

Answer 1

acute proliferative (post infectious) GN
IgA nephropathy
Lupus Nephritis
RPGN (crescentic):
a. Anti GBM antibody (type 1)
b. complication of immune complex type 2 (HSP, Post infectious GN, Lupus nephritis, IgA nephropathy)
c. pauci immune (gpa wegeners, eosinophilic gpa churg strauss, mpa)

Question 2

Acute Proliferative GN (post streptococcal) [Nephritic]

Answer 2

gas, pyogenes, type 12,4,1 are
nephritogenic
3-4 weeks post inf.
immune complexes
6-10 yo, adults also
aso titres increase
complement C3 decrease
EM:subepithelial humps
IF: granular deposits of IgG, IgM, C3
exogenous: PSGN
endogenous: SLE

Question 3

IgA nephropathy, Berger disease
[Nephritic]

Answer 3

most common type of GN
hematuria
genetic; HLA association
acquired: resp or gi exposure to virus, bacteria, food proteins, etc
secondary: celiac, chronic liver disease
smokey urine
recurrence in transplanted kidneys
IF: mesangial deposits of IgA, C3
EM: electron dense deposits in the mesangium

Question 4

Rapidly Progressive GN
[Nephritic]

Answer 4

type 1: anti GBM Ab with or without lung involvement, If: linear IgG , C3
type 2: post infectious GN, HSP, Lupus (C3 C4), IgA, If: granular Ag Ab complexes
type 3: pauci immune, anca assoc, wegener, microscopic polyangiitis, If: no deposits (circulating ancas)
LM: crescents
hematuria, red cell casts, GP may have hemoptysis or pulmonary hemorrhage
EM: deposits of immune complex

Question 5

Hereditary Nephritis
Alport syndrome

Answer 5

x linked dominant
hematuria, nerve deafness, lens dislocation, cataracts, corneal dystrophy
proteinuria later
mutation of COL4A3, 4, 5
abnormal type 4 collagen
5-20 yo
renal failure 20-50 men
basket weave
finally leads to FSGN, global GS

Question 6

Nephrotic syndrome

Answer 6

massive proteinuria >3.5g/day
hypoalbuminuria (<3g/dL)
anasarca
hyperlipidemia, lipiduria
children (peak 2-6): MCD, FSGS
adults: secondary to diabetes, SLE, amyloidosis, drugs (penicillamine, Nsaids), infections, malignancies, misc,
primary causes: membranous glomerulopathy, PSGS

Question 7

Membranous Nephropathy (MGN)

Answer 7

most common nephrotic syn in elderly
primary
secondary: drugs, malignancy, infection (hep b c ) SLE
response to steroids not so good
autoimmune-antibodies to M type phospholipase A2 receptor (PLA 2 R) –>complement activation–>C5b-C9 activates–> immune complexes along subepithelial aspect of bm
nonselective proteinuria
renal vein thrombosis
oval fat bodies, fatty casts
spikes on silver stain
EM: effaced foot processes
IF: IgG along GBM

Question 8

Minimal change disease MCD

Answer 8

most common nephrotic syn in children
systemic t cell dysfunction, foot process fusion, resp inf, immuni, atopic disorders, hodgkin lymphoma, dramatic response to corticosteroid therapy.
normal looking glomeruli
lipoid nephrosis

Question 9

FSGS

Answer 9

most common nephrotic syn in adults
mutations in slit diaphragm proteins, nephrin, podicin, Ca channel.
poor response to corticosteroids

Question 10

HIV associated nephropathy

Answer 10

most common-a severe form of collapsing variant of FSGS
tubules filled with proteinaceous material
EM: tubuloreticular inclusions within endothelial cells
glomerular and tubular injury due to inf of epith by HIV (PCR)

Question 11

MPGN

Answer 11

primary:
type 1: classical and alternate complement pathways, immune complex subendothelial electron dense deposits IF: IgG & C3 granular
type 2: dense deposit disease (alternate complement pathway)
secondary: SLE, HBV, HCV, CLL, Lymphoma
GBM thick-tram track appearance (PAS or silver stains)
crescents may be present