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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
SLE Dx?
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,
26
Drug induced SLE?
procainamide, hydralazine, quinidine, Anti TNF
27
patho in LN?
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
28
LN Tx?
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
29
CR / PR LN Def?
CR: < 0.5g/d proteinuria with no glom hematuria / RBC cast, normal GFR PR: 50% reduction in proteinuria in subnephrotic range, stable GFR
30
what is C1q nephropathy
C1q predom in IF can be MCN or FSGS spectrum to r/o SLE
31
IRGN Tx?
Treat underlying infection | MP if extensive crescent or severe RPGN
32
type of cryoglobulemic GN?
I: monoclonal Ig - M>G>A>BJP: MM, CLL, Waldenstrom II: mixed cryoglobulin with monoclonal Ig - M/G >> G/G: infection HCV, Sjogren, CLL, lymphoma) III: Mixed polyclonal - M/G: Infection (HCV / HBV), SLE, RA, vasculitis, malignancy
33
Cryoglobulinemic GN sym?
palpable non puruitic pupura, arthralgia (not arthritis), PN, AKI CHF, digital necrosis, lung infiltrate, mesenteric ischemica type I usually asymptomatic
34
Ix for cryoglobulinemic GN?
low C3 with C3 normal or mildly low, RF+, 40% type II cryo -ve
35
cryoglobulinemic GN patho?
MPGN 80%, more macrophage than normal poliferative GN, intraluminal thrombi possible, IF diffuse IgM EM subendothelial deposit 'fingerprint pattern of cryo[recipitate
36
Cryoglobulinmic GN Tx?
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
37
MPGN type?
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
38
MPGN IgM predom causes?
Viral related, or RA / Sjrogren | usually IgG3 without LC restriction
39
MPGN Ix?
hepatitis serology, SPE, Ig pattern, FLC, septic workup, Echo
40
IF IgG + C3 ddx?
MPGN, MN, IRGN, CryoGN
41
IgAN Tx?
< 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
IgAN association?
``` Rheumat: RA / AS GI: celiac disease hepatic: cirrhosis lung: sarcoid skin: dermatitis, herpetiformis Infection: HIV, HBV ```
43
IgAN Patho classification?
``` Oxford classification: MEST Mesangial hypercellulity Endocapillary hypecellularity Segmental sclerosis TI inflammation ```
44
Ddx of IgA in renal biopsy?
``` IgA / IgAV LN Alcoholic liver disease IgA monoclonal gammopathy IgA dominant SA associated GN ```
45
PCKD USG criteria
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
PCKD ddx
TSC, ARPKD, VNL, MCKD
47
PCKD prognostic factor?
Pro PKD: early HT / uro symptoms, truncating mutation / PKD2 | TKV
48
PCKD association?
``` IC aneurysm PLD MVP diverticulum cervicocephalic / thoracic artery dissection ```
49
PCKD Tx:
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
Alport MOI?
80% XL, 15% AR, 5% AD
51
PCKD Comp?
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
alport cause?
XLAS: COL4A5 mutation ARAS: COL4A3 / COL4A4 ADAS: COL4A3 / COL4A4 heterozygous
53
alport patho?
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
alport sym
hematuria, proteinuria SNHL anterior lenticonus, maculopathy, corneal endothelial vesicles esophageal / tracheobronchial leiomyomatosis clitoral hypertrophy
55
alport Tx
ACEI for proteinuria
56
Thin GBM dx?
GBM thickness < 250nm
57
Fabry cause?
alpha galactosidase A deficiency
58
Fabry sym?
``` 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
fabry patho:
glomerular epithelial glycosphingolipid depositon / FSGS | EM lysosomal inclusion in epithelial
60
fabry dx?
male: a-galactosidase activity < 15% female: genetic study
61
fabry Tx?
ERT
62
MCN Tx?
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
stone Ix?
``` Hx: onset, hyperCa, gout, drug, UTI, FHx P/E: gouty tophi, RFT, CaPO4, PTH, Urate urine R/M, pH, biochem USG / KUB ```
64
stone urine biochem
``` 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
stone Tx?
``` 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
primary hyperoxaluria cause?
type 1: Alanine glyoxalate aminotransferase inactivation type 2: impaired glyoxalate to glycolate type 3
67
primary hyperoxaluria Tx?
pyridoxine, urine alkalinzation, citrate, Mg, orthophosphate
68
nephrocalcinosis cause?
medullary: - hyperCa, hyperPTH - Drug: acetozolamide, amphoB - tubular: dRTA - PCKD ``` cortical: cortical necrosis rejection GN TB Trauma ```
69
TTP Tx?
TPE Pred ? 200mg, to 60mg/day then 5mg reduction per week IVIG RTX 375mg/m2/wk
70
RVH causes?
FMD artherosclerotic takayasu coarctation and middle aortic syndrome
71
RVH Dx?
doppler PSV > 200 | CT / MRA
72
RVH Tx?
``` FMD: balloon angioplasty artherosclerotic: ASTRAL 2009 STAR 2009 CORAL 2009 BP control / ACEI first, revascularization if fail ```
73
indication for revascularization in RVH?
Worsening kidney function in ischemic renal diease uncontrolled HT intolerance to media therapy flash APO
74
RVT causes?
``` 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
RAT cause?
prothrombotic: transplant, malignancy, APS, vascularitis, TTP, rejection embolism: AF. valvular, paradoxical embolism, cholesterol embolism, atheroemboli
76
ANCA AV TPE indication?
Anti GBM+ Cr > 350 or RRT dependent lung hemorrhage MEPEX: may enhance renal recovery PEXIVAS trial: no different in death / ESRD
77
ANCA induction therapy?
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
ANCA maintenance
``` 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
cast nephropathy Tx?
treat hyperCa IVF to achieve 3L/d UO bortezomib based chemo (CyBorD) TPE or HCO controversal (EULITE: not useful)
80
AKI / CKD in HIV?
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
Cystinuria type?
AR ``` Type I • Obligate heterozygote Type I • Normal aminoaciduria • Associate with 2p21 deletion syndrome, hypotonia-cystinuria syndrome (HCS) & 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
Cystinuria Tx?
low Na low protein high fluid urine alkalinzation > pH 7 penicillinamine, Tioponin Captopril
83
TTP Tx?
TPE steroid RTX Caplacizumab
84
Drug induced TMA?
CNI, mTOR Septrin Quinine Anti cancer
85
AIN causes?
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
AIN sym?
fever, MP rash, arthalgia, eosinophilia, hemolysis, hepatitis, high IgE,
87
granulomatous AIN ddx?
Infection (EBV, toxoplasmosis, Salmonella), sarcoidosis, Sjogren, GPA
88
AIN Ix?
eosinophiuria | gallium scan
89
AIN Tx?
Pred 1mg/kg/d x 4-6 wks if on RRT or RFT fail to improve after 1 week
90
cholesterol emboli patho?
needle-like or slit-like clefts in arterial
91
MCKD MOI?
AD
92
MCKD gene?
Uromodulin REN Mucin1 HNF1B
93
MCKD features?
strong family history early onset gout USG and urine normal renal bx TIN, Eosinophilic "fluffy“/Fibrillar inclusions in tubule, EM shows TBM lamellation
94
MCKD Tx?
allopurinol | renal transplant
95
drug induced ANCA
hydralazine PTU / carbimazole minocycline, penacillamine
96
drug induced lupus
procainamide hydralazine penicillamine

Renal (10 decks)

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