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GN Flashcards

1
Q

FSGS causes?

A

idiopathic
Familial: NPHS1 (nephrin), NPHS2 (podocin), WT1
Infection: HIV, B19, SV40, CMV
Drug induced: Pamidronate, Sirolimus, heroin, interferon, Lithium, anabolic steroid, TKI
reduced renal mass: unilateral renal agenesis, oligomeganephroia, VLBW, renal dysplasia, reflux nephropathy, CAN, sequela to cortical necrosis, advanced renal disease
normal renal mass: HT, RAS, obesity, CRHD, sickle cell

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2
Q

FSGS sym?

A

HT, microhematuria, proteinuria (non selective), occ glucouria, aminoaciduria, PO4uria, conc defect

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3
Q

FSGS subtypes?

A
  • NOS: accumulation of ECM that occlude glom capillary, forming discrete segmental solidifications
    IF shows IgM, C3
  • perihilar: SS and hyalinosis at vascular pole, usually in 2nd FSGS
  • cellular: segmental endocapillary hypercellularity, usually 1st FSGS
  • collapsing: at least 1 segmental or global collapse, prominent IFTA, commonly by HIV, B19, lupus podocytopathy, hemophagocytic syndrome, IF therapy, TKI pamidronate
  • tip: adhesion to pole
  • diffuse mesangial hypercellularity, IF IgM, C3 EM extensive foot process effacement; exclusively no children
    C1qN: dominant or codominant C1q stain with LM FSGS or MCD like, need to exclude LN
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4
Q

Diff 1st and 2nd FSGS?

A
  • HIV: tubular microcyst, EM tubuloreticular inclusion
  • adaptive: glomerulomegaly, perihilar lesion, EM foot process effacement tends to be mild
  • loss of renal mass: background global GS, IFTA, arteriosclerosis
  • SCD: capillary congestion by sickled erythrocyte, double contour of GBM
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5
Q

FSGS prognosis?

A

some spontaneously remit, unresponsive case to ESRD in 5 - 20 years
worst for collapse, best for tip, NOS an cellular middle

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6
Q

FSGS Tx?

A

subnephrotic: BP < 125 / 75, ACEI, statin, low protein
nephrotic: + Pred 1mg/kg/d x 4 - 16wks then taper over 6m
SR or CI to steroid: CsA 3-5mg/kg/d x 4 - 6m, FK 2-4 bd x 4-6m, CYC 2mg/kg/d x 2-4m, MMF 1-1.5g bd x 4-6m
? RTX, Abatacept, ACTH

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7
Q

MN patho features?

A

LM: thickening of glom capillary wall, spike in silver stain
IF: diffuse finely granular IC IgG (IgG4, if other Ig suggest LN / 2nd cause) and EDD in subepithelial space, C3 common, C1q suggest LN, PLA2R suggest 1st, mesangial Ig present suggest 2nd
EM: subepithelial deposit without subendothelial

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8
Q

MN causes?

A

Anti PLA2R
Anti THSD7A
Autoimmune: Class V LN, uncommon RA, autoimmune thyroid, IgG4, Anti GBM, ANCA
Infection: HBV, common HCV, HIV, syphilis, schistosomiasis
malignancy: solid tumor, uncommon NHL, CLL, melanoma
Drug: NSAID, PPI, uncommon Hg, gold, D penicillamine
Other: sarcoidosis, GVHD, post transplant

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9
Q

MN typical age and sex?

A

M:F 2:1, peak 40s - 50s

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10
Q

MN features?

A

nephrotic, microscopic hematuria common but RBC cast rare, 10% HT, Cr n

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11
Q

MN Ix?

A

BP, RFT, urinalysis, 24hr uP, LDL, Anti PLA2R, HBV,HCV, ANA, dsDNA, C3, C4
doppler / MRA / CTA if frankl pain hematuria AKI
Anti GBM and ANCA if active urine
cancer screening if > 50y/o

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12
Q

NS ddx

A

DM, MCN, FSGS, MN, MPGN (IC or C3), amyloid / LCDD, LN

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13
Q

MN Tx?

A

BP 125 / 75 with ACEI
statin
low protein diet
anticoagulate if Alb < 25
immunosuppresant if uPCR > 4 without 50% decline over 6m despite ACEI (unless disabling or llife threatening sym or Cr rise 30% over 12m after r/o RVT / crescentic variant / AIN (or 3m if heavy NS >8g)
- Ponticelli: MP 1g x 3 days then pred 0.5mg/kg/d for 1,3,5m; CYC 2mg/kd/d 2,4,6m
- CNI: CsA 3.5-5mg/kg/d + 0.15mg/kg/d Pred x 6m, or FK 0.05-0.075mg/kg/d x 6m
? RTX / ACTH

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14
Q

Diff TTP and HUS?

A

TTP more severe low plt and less severe AKI

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15
Q

HUS causes?

A

typical:
- Shiga toxin bacteria (STE Coli, shigella)
- Strep pneumoniae (neuraminidase related)
Atypical:
- familial: CFH, C3, MCP, THBD, CFB, CHF / CHFR hybrid, DGKE
- sporadic
- idiopathic
- pregnancy related / HELLP
- Transplant

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16
Q

TTP cause?

A

congenital: homozygous / compound heterozygous mutation in ADAMTS13
Acquired: Anti ADAMTS13 AutoAb

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17
Q

TMA causes?

A 
HUS / TTP
Cobalamin C deficiency (MMACHC mutation)
Drug induced: Ticlopidine and plavix: Anti ADAMTS13
BM / HSC transplant
Malignancy
HIV
SLE, APS
malignant HT
scleroderma
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18
Q

TTP pentad?

A

fever, MAHA, fever, CNS AKI

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19
Q

Diff TMA from DIC?

A

normal PT, APTT, fibrinogen, coagulation factor

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20
Q

MIDD secondary cause?

A

MM, waldenstrom, CLL, NHL

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21
Q

MIDD sym?

A

hematuria, proteinuria may be NS, CKD or RPGN, hepatosplenomegaly, CHf, arrhythmia, neuropathy, GI upset, lung nodules, arthropathy

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22
Q

MIDD patho features?

A

eosinophilic tubular lesions, refractile, PAS+ ribbon like
or nodular GS
cresecent possible
arterial and ptc can have PAS+ deposit
IF mainly kappa
EM coarsely / fine granular EDD in tubular, BM or subendothial in GBM

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23
Q

MIDD Tx?

A

underlying cause

Melphalan +/- ASCT

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24
Q

Diff amyloid, fibrillary and Immunotactoid in patho?

A

congo red only in amyloid
size of deposit: amyloid 8 - 15nm, FGN 12-22, IGN > 30
alignment of deposit: only IGN are parallel
Ig: Amyloid LC lambda, FGN polychonal IgG4, IGN monoclonal IgGk or IgGL
extrarenal: amyloid systemic, FGN lung hemorrhage,
Asso with other disease: amyloid: MM, FGN: MM, IBD, SLE, IGN NHL, CLL
Tx: amyloid: mephalan + dexa, FGN steroid+/- CYC, IGN treat PLD

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25
Q

SLE Dx?

A

SLICC ACR Criteria: 4 of following, at least 1 clinical and 1 immunological criteria OR biopsy proven LN with ANA / dsDNA+
Clinical: acute / subacute cutaneous lupus, chronic cutaneous lupus, non scarring alopecia, oral / nasal ulcer, synovitis > 2 joints, serositis, renal disease (RBC cast / uPCR > 0.5g/d), neurological disorder, hemolytic anaemia, leukppenia OR lymphopenia, thrombocytopenia
Immunological criteria: ANA, dsDNA, APAb+, low C3 / C4, DAT+ without hemolytic anaemia,

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26
Q

Drug induced SLE?

A

procainamide, hydralazine, quinidine, Anti TNF

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27
Q

patho in LN?

A

I: normal LM, mesangial deposit in IF
II: LM mesangial hypercellularity
III: LM endocapillary or extracapillary GN in < 50% glom
IV: same as III with > 50%
V: GBM thickening with subepitheliam and mesangial IC
IV 90% sclersed

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28
Q

LN Tx?

A

III or IV:
PRed 0.5 - 1mg/kg/d titrate down in 6-12m +/- MP 1g 1-3days
NIH regime: IV CYC 0.5-1g/m2 monthly x 6
Euro-Lupus: IV CYC 500mg, Q2weeks x 3 months
PO CYC: 1-1.5mg/kd/d (Max 150mg/d) x 3 months
MMF up to 3g/d x 6m
If refractory: RITUXILUP: RTX 1g x 2,
FK / CsA, IVIG, TPE

? Abatacept CTLA4 fusion protein

maintainence: MAINTAIN: MMF 1-2g/d or AZA 1-2.5mg/kd/d or CsA 2.5-4mg/kd/d or FK to trough 4-6ng/ml

V and NS / AKI / persistently < 1g despite ACEI:
MMF 2-3g/d or AZA 1-2.5mg/kg/d +/- steroid x 6m
Alt use IV CYC 0.5-1g/,2 monthly x 6 / CsA 4-6mg/d x 12m / FK 0.1-0.2mg/kd/d x 12 m

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29
Q

CR / PR LN Def?

A

CR: < 0.5g/d proteinuria with no glom hematuria / RBC cast, normal GFR
PR: 50% reduction in proteinuria in subnephrotic range, stable GFR

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30
Q

what is C1q nephropathy

A

C1q predom in IF
can be MCN or FSGS spectrum
to r/o SLE

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31
Q

IRGN Tx?

A

Treat underlying infection

MP if extensive crescent or severe RPGN

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32
Q

type of cryoglobulemic GN?

A

I: monoclonal Ig - M>G>A>BJP: MM, CLL, Waldenstrom
II: mixed cryoglobulin with monoclonal Ig - M/G&raquo_space; G/G: infection HCV, Sjogren, CLL, lymphoma)
III: Mixed polyclonal - M/G: Infection (HCV / HBV), SLE, RA, vasculitis, malignancy

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33
Q

Cryoglobulinemic GN sym?

A

palpable non puruitic pupura, arthralgia (not arthritis), PN, AKI
CHF, digital necrosis, lung infiltrate, mesenteric ischemica
type I usually asymptomatic

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34
Q

Ix for cryoglobulinemic GN?

A

low C3 with C3 normal or mildly low, RF+, 40% type II cryo -ve

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35
Q

cryoglobulinemic GN patho?

A

MPGN 80%, more macrophage than normal poliferative GN, intraluminal thrombi possible,
IF diffuse IgM
EM subendothelial deposit ‘fingerprint pattern of cryo[recipitate

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36
Q

Cryoglobulinmic GN Tx?

A

RP or organ threatening: MP + CYC / RTX
life threatening (lung hemorrhage, hyperviscosity) should have TPE
can IC first before treating HCV, but HIV / HBV should treat with antiviral first

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37
Q

MPGN type?

A

type I: subendothelial + mesangial
type II: intramembranous EDD + mesangial
type III: mesangial + subendothelial + subepithelial

Mayo clinic classification:
IC: infection HBV / HCV, IE, shunt, abscess, leprosy, meningiococcal, mycoplasma, malaria, haanta, schistsomiasis; autoimmune: SLe, sjogren, RA, PSC; neoplastic: monoclonal gammopathy
Complement related: C3 / DDD, due to CFH, CHI or C3NF
Non IC / complement: healing HUS / TTP, APS, POEMS, TMA, SCA, post transplant
Idiopathic

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38
Q

MPGN IgM predom causes?

A

Viral related, or RA / Sjrogren

usually IgG3 without LC restriction

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39
Q

MPGN Ix?

A

hepatitis serology, SPE, Ig pattern, FLC, septic workup, Echo

40
Q

IF IgG + C3 ddx?

A

MPGN, MN, IRGN, CryoGN

41
Q

IgAN Tx?

A

< 1g/d proteinuria: ACEI / ARB, BP control 130 / 70, statin, fish oil
> 1g/d despite ACEI x 6m and GFR > 60: 6m steroid

German STOP IgAN
Chinese TESTING

MMF in Chinese population, CsA maybe tried

Manno: Pred 0.8-1mg/kg/d x 2 months then reduce by 0.2mg/kg/d for next 4 months
Pozzi: MP 1g x 3d at month 1,3,5, followed by 0.5mg/kg/d alt day x 6m

42
Q

IgAN association?

A 
Rheumat: RA / AS
GI: celiac disease
hepatic: cirrhosis
lung: sarcoid
skin: dermatitis, herpetiformis
Infection: HIV, HBV
43
Q

IgAN Patho classification?

A 
Oxford classification: MEST
Mesangial hypercellulity
Endocapillary hypecellularity
Segmental sclerosis
TI inflammation
44
Q

Ddx of IgA in renal biopsy?

A 
IgA / IgAV
LN
Alcoholic liver disease
IgA monoclonal gammopathy
IgA dominant SA associated GN
45
Q

PCKD USG criteria

A

With FHx PKD1 (Ravine’s):
< 30, total 2 cyst
30 - 60, 2 in each kidney
> 60, 4 in each kidney

With FHx PKD unknown gene (Revised unified diagnostic criteria):
< 40, total 3 cyst
40 - 60, 2 in each kidney
> 60, 4 in each kidney

Rule out:
< 30/40: need MRI < 5 cyst
30 - 40: no renal cyst
> 40: 1 or 0 renal cyst

Without FHx:
> 10 cyst in each kidney

46
Q

PCKD ddx

A

TSC, ARPKD, VNL, MCKD

47
Q

PCKD prognostic factor?

A

Pro PKD: early HT / uro symptoms, truncating mutation / PKD2

TKV

48
Q

PCKD association?

A 
IC aneurysm
PLD
MVP
diverticulum
cervicocephalic / thoracic artery dissection
49
Q

PCKD Tx:

A

fluid intake > 3L to keep Uosm < 250
Na restriction
MDRD: BP control ACEI MAP < 92

V2RA (SE polyuria , dLFT, high urate): 
- if Mayo classification 1C-E, 
- 18 - 55y/o with GFR 25 - 65
- PRO PKD > 6
kidney length > 16.5
TKV > 750ml

ALADIN: octreotide

50
Q

Alport MOI?

A

80% XL, 15% AR, 5% AD

51
Q

PCKD Comp?

A

cystic infection: septrin / FQ, aspirate if persistent
hemorrhage: conservative
Frank pain ddx: stone, hemorrhage, infection, tumor
stone: K citrate
PLD: avoid alcohol, caffine, OCP, ERT, somatostatin, H2RA
Aneurysm: monitor if < 5mm, surgery if > 5mm

52
Q

alport cause?

A

XLAS: COL4A5 mutation
ARAS: COL4A3 / COL4A4
ADAS: COL4A3 / COL4A4 heterozygous

53
Q

alport patho?

A

LM: mesangial hypercellularity, FSGS / IFTA
EM: variable thickening, thinning, lamellation of GBM, spliting of lamina densa

IF:
XLAS: no a3/a4/a5, a1/a2 normal
ARAS: GBM no a3/a4/a5, a5/a6 present in bowmen capsule / distal TBM
ADAS: normal staining

54
Q

alport sym

A

hematuria, proteinuria
SNHL
anterior lenticonus, maculopathy, corneal endothelial vesicles
esophageal / tracheobronchial leiomyomatosis
clitoral hypertrophy

55
Q

alport Tx

A

ACEI for proteinuria

56
Q

Thin GBM dx?

A

GBM thickness < 250nm

57
Q

Fabry cause?

A

alpha galactosidase A deficiency

58
Q

Fabry sym?

A 
arcoparasthesia
ESRD
MI / CVA
autonomic: hypodidrosis, GI dysmotility
skin: angiokeratoma at LL
eye: verticulata (whitish discoloration radiation from center to cornea)
lung: obstructive

Urine oval fat body, maltese cross configuration

59
Q

fabry patho:

A

glomerular epithelial glycosphingolipid depositon / FSGS

EM lysosomal inclusion in epithelial

60
Q

fabry dx?

A

male: a-galactosidase activity < 15%
female: genetic study

61
Q

fabry Tx?

A

ERT

62
Q

MCN Tx?

A

Pred 1mg/kg/d 4 - 16wks, titrate down over 6 months
if SR: rebiopsy
if FR / SD: CYC 2mg/kg/d x 3m or IV CYC 0.5-1g/m2
or CsA 4-6mg/kg/d air trough 50 - 150 x 1 year
MMF
RTX 1g x 2

63
Q

stone Ix?

A 
Hx: onset, hyperCa, gout, drug, UTI, FHx
P/E: gouty tophi, 
RFT, CaPO4, PTH, Urate
urine R/M, pH, biochem
USG / KUB
64
Q

stone urine biochem

A 
Vol: 2-2.5
Ca < 0.1mmol/kg
Oxalate < 0.36
urate < 4.5 (M) / 4.7 (F)
citrate > 17
Ng < 2g
PO4 < 35
cystine < 1.04 (usually 0.13)
65
Q

stone Tx?

A 
general: 
fluid > 2L
Na restriction < 2g
moderate protein intake
moderate Ca diet

Oxalate stone:
- high UCa: thiazide, treat hyperCa
- high oxalate:
r/o primary hyperoxaluria (>0.7, can cause CMP, BM supp, CKD)
r/o enteric hyperoxaluria (Crohn, celiac, chronic pancreatitis, short bowel, post bariatric)
- hypocitrate (due to hypoK, metabolic acidosis, hypoMg, azetozolamide): K citrate
- RTA: K citrate

Urate stone:

  • urine alkalinzation till urine pH > 6.5
  • allopurinol
  • acetozolamide if hyperK

Struvite stone:
- treat bacteruria till 3m sterile, remove stone
urease ihibitor: aceohydroxaic acid
chemolysis

Cystine stone:
urine alkalinzation > 7.5
tiopronin, D penicillamine
captopril

66
Q

primary hyperoxaluria cause?

A

type 1: Alanine glyoxalate aminotransferase inactivation
type 2: impaired glyoxalate to glycolate
type 3

67
Q

primary hyperoxaluria Tx?

A

pyridoxine, urine alkalinzation, citrate, Mg, orthophosphate

68
Q

nephrocalcinosis cause?

A

medullary:

  • hyperCa, hyperPTH
  • Drug: acetozolamide, amphoB
  • tubular: dRTA
  • PCKD
cortical:
cortical necrosis
rejection
GN
TB
Trauma
69
Q

TTP Tx?

A

TPE
Pred ? 200mg, to 60mg/day then 5mg reduction per week
IVIG
RTX 375mg/m2/wk

70
Q

RVH causes?

A

FMD
artherosclerotic
takayasu
coarctation and middle aortic syndrome

71
Q

RVH Dx?

A

doppler PSV > 200

CT / MRA

72
Q

RVH Tx?

A 
FMD: balloon angioplasty
artherosclerotic:
ASTRAL 2009
STAR 2009
CORAL 2009
BP control / ACEI first, revascularization if fail
73
Q

indication for revascularization in RVH?

A

Worsening kidney function in ischemic renal diease
uncontrolled HT
intolerance to media therapy
flash APO

74
Q

RVT causes?

A 
malignancy: tumor invasion
NS: LN / MN
complication of IVC / PICC line
pyelonephritis, pancreatitis
IBD
inflammatory aortic aneurysm
hypercoagulable state: APS, Factor V leiden, AT3 deficiency, Protein C/ S
OCP
high Plt / Hct
post transplant
surgicla compression
pregnancy
Behcet
Bidd Chiari
75
Q

RAT cause?

A

prothrombotic:
transplant, malignancy, APS, vascularitis, TTP, rejection
embolism:
AF. valvular, paradoxical embolism, cholesterol embolism, atheroemboli

76
Q

ANCA AV TPE indication?

A

Anti GBM+
Cr > 350 or RRT dependent
lung hemorrhage

MEPEX: may enhance renal recovery
PEXIVAS trial: no different in death / ESRD

77
Q

ANCA induction therapy?

A

MP 1g x 3 days, then 1mg/kg/d x 3-4m
+ IV CYC 0.5 - 0.75g/m2 monthly x 3m
or + PO CYC 2mg/kg/d x 3-6m
or + RTX 375mg/m2 x 4 week (RAVE vs PO CYC, RITUXVAS vs IV CYC

78
Q

ANCA maintenance

A 
Aza 2mg/kg/d
MMF 1-2g/d
MTX 0.3mg/kg/week if GFR > 60
RTX Q6 months
septrin if upper airway disease
79
Q

cast nephropathy Tx?

A

treat hyperCa
IVF to achieve 3L/d UO
bortezomib based chemo (CyBorD)
TPE or HCO controversal (EULITE: not useful)

80
Q

AKI / CKD in HIV?

A

AKI:
Pre renal
Renal: ATN (sepsis, medication, pigment), TMA, HIV related GN (IgAN, MPGN, Cryoglobulimic, LN like, IRGN), AIN
Post renal

CKD:
HIVAN: Collapsing FSGS
ARTAN: AIN, crystaluria, fanconi
other causes: HT, DM

81
Q

Cystinuria type?

A

AR

Type I
• Obligate heterozygote Type I
• Normal aminoaciduria
• Associate with 2p21 deletion syndrome, hypotonia-cystinuria syndrome (HCS)
&amp; atypical HCS
  • Type II and III
  • Hyperexcretion of dibasic amino acid
  • Very infrequently formation of cystine stones

Type A – Patient with 2 mutations of SLC3A1 gene
• Type B – Patient with 2 mutations of SLC7A9 gene
• Type AB – Patient with 1 mutation on both SLC3A1 and SLC7A9 genes

82
Q

Cystinuria Tx?

A

low Na low protein high fluid
urine alkalinzation > pH 7
penicillinamine, Tioponin
Captopril

83
Q

TTP Tx?

A

TPE
steroid
RTX
Caplacizumab

84
Q

Drug induced TMA?

A

CNI, mTOR
Septrin
Quinine
Anti cancer

85
Q

AIN causes?

A

Drug: beta lactam, sulfonamide, FQ, fibampicin, NSAID, PPI, diuretics, phenytoid, allopurinol, HARRT, TKI, ifosfamide, PD1, CTLA4, Aspirin
Infection: campylobacter, GNB, CMV, EBV, HIV, Brucella, toxoplasmosis, mycoplasma, chamydia
Systemic: sarcoidosis, Sjogren, SLE, IgG4, cryoglobulin
Malignancy: leukemia / lymphoma
TINU

86
Q

AIN sym?

A

fever, MP rash, arthalgia, eosinophilia, hemolysis, hepatitis, high IgE,

87
Q

granulomatous AIN ddx?

A

Infection (EBV, toxoplasmosis, Salmonella), sarcoidosis, Sjogren, GPA

88
Q

AIN Ix?

A

eosinophiuria

gallium scan

89
Q

AIN Tx?

A

Pred 1mg/kg/d x 4-6 wks if on RRT or RFT fail to improve after 1 week

90
Q

cholesterol emboli patho?

A

needle-like or slit-like clefts in arterial

91
Q

MCKD MOI?

A

AD

92
Q

MCKD gene?

A

Uromodulin
REN
Mucin1
HNF1B

93
Q

MCKD features?

A

strong family history
early onset gout
USG and urine normal
renal bx TIN, Eosinophilic “fluffy“/Fibrillar inclusions in tubule, EM shows TBM lamellation

94
Q

MCKD Tx?

A

allopurinol

renal transplant

95
Q

drug induced ANCA

A

hydralazine
PTU / carbimazole
minocycline, penacillamine

96
Q

drug induced lupus

A

procainamide
hydralazine
penicillamine

Renal (10 decks)

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