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GN Flashcards

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1
Q

What’s nephrotic syndrome?

A
  • Glomerular disorders characterised by proteinuria (>3.5g/day) resulting in
    ○ Hypoalbuminemia <30g/L - pitting oedema
    ○ Hypogammaglobulinemia - increased risk of infection
    ○ Hypercoagulable state - due to loss of antithrombin III (breaks up thrombin and destroys coagulation factors)
    ○ Hyperlipidaemia and hypercholesterolemia - may result in fatty casts in urine

Way to remember: lose a lot of protein –> thin blood –> body increases cholesterol and lipids in blood to “beef” it up

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2
Q

What can UA pick up?

A

Picks up haem pigments (not specific for RBC or Hb; will be positive in myoglobin) - need to do urinalysis if there is “blood” on UA

Picks up albumin only in terms of protein. Insensitive for low levels of albumin (microalbuminuria). Doesn’t pick up other proteins like light chains.

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3
Q

What’s nephritic syndrome?

A
  • Glomerular disorders characterised by glomerular inflammation and bleeding
    ○ Limited proteinuria (<3.5g/day)
    ○ AKI +/- Oliguria
    ○ Salt retention with periorbital oedema and hypertension
    ○ Haematuria with RBC casts and dysmorphic RBCs in urine
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4
Q

RBC casts in the urine are diagnostic of

A

GN

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5
Q

Relationship between albuminuria and proteinuria

A

As you increase in proteinuria, the % of albuminuria increases

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6
Q

Gold standard to look for proteinuria

A

24h urine collection is gold standard

But usually we do spot urine albumin creatinine ratio, but important to check it over time due to significant variability e.g. sepsis, time of day

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7
Q

What’s GN? What are the 5 clinical classification?

A

Inflammation of the glomerulus

1) Asymptomatic urinary abnormalities
2) Nephritic syndrome
3) RPGN
4) Nephrotic syndrome
5) Chronic GN

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8
Q

Nephrotic syndrome DDx

A 
Minimal change
FSGS
Membranous
Lupus nephritis class V(membranous)
Diabetic nephropathy
Amyloid
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9
Q

Nephritic syndrome DDx

A 
ANCA vasculitis
Anti GBM 
Infection-associated GN (post strep/infection)
Lupus nephritis class III/IV
TMA (TTP, aHUS, HUS, APS)
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10
Q

Overlap between nephrotic and nephritic syndrome DDx

A

IgA nephropathy (most common GN)
MPGN (immune complexes, cryoglobulins, complement)
Lupus
Myeloma/MGRS

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11
Q

Approach to management of primary GN

A

Pathogenesis is unknown/evolving, therefore
Therapy is non-specific
Supportive measures are important
Consider side effects

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12
Q

Minimal change disease presentation and histology

A

Pure nephrotic syndrome
Children and young adult

Histology
Normal light microscopy
\+/- slight mesangial proliferation
IF +/- mesangial IgM 
EM - flattened podocytes (epithelial layer disrupted)
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13
Q

Minimal change disease poor prognostic features

A

Haematuria
Reduced GFR
HTN

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14
Q

Minimal change disease treatment

A

VERY responsive to steroids

If not responding to steroids, consider FSGS (looks the same on electron microscopy but maybe there is a sampling error and you haven’t picked up the glomeruli segments with sclerosis)

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15
Q

FSGS (primary) presentation and histology and prognosis

A

Note this is NOT secondary or genetic FSGS. Different entities.

Sudden onset nephrotic syndrome
Often HTN, reduced GFR +/- haematuria (overlap with nephritic)

Histology
- Focal and segmental glomerulosclerosis

Prognostic factor
Reduced GFR, degree of proteinruai, fibrosis on biopsy

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16
Q

FSGS treatment

A

Prednisolone

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17
Q

FSGS and suPAR?

A

Not specific to FSGS
Seen in other inflammatory conditions
But we do think there is increased permeability factor but this is unknown at the moment

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18
Q

FSGS prognosis

A

Prognosis
If left untreated, will progress to ESKD
If treated and remission - good outcome
High recurrence rate in transplant patients

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19
Q

Primary membranous nephropathy presentation, histology, poor prognostic factor

A

Presentation
Nephrotic range proteinuria

Histology
LM: thickened GBM
IF: granular IgG +/- C3
EM: subepithelial deposits 
Silver stain: spikes seen (immunoglobulin deposits)

Poor prognostic factor
Late sign - interstitial fibrosis + less deposits
Degree of proteinuria, HTN, reduced GFR, age >50, male

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20
Q

Most common GN in nephrotic syndrome in adults

Study These Flashcards
A

Primary membranous nephropathy

21
Q

Whats important to exclude in Primary membranous nephropathy?

Study These Flashcards
A

Exclude secondary causes

SLE, hepatitis, melignancy, drugs

22
Q

Primary membranous nephropathy prognosis

Study These Flashcards
A

Variable course

25% progress to ESKD in 10 years

23
Q

Pathophysiology of Primary membranous nephropathy

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A

Ag-Ab complex deposit in epithelial layer of GBM

24
Q

Primary membranous nephropathy associated with which marker

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A

Anti-PLA2R ab
Can be used instead of kidney biopsy
Can be used to risk stratify patient
Can be monitored for treatment response and relapse
If high levels, consider immunosuppressive therapy upfront

Treatment associated with fast decline (quicker than resolution of proteinuria)

25
Anti-PLA2R present for .... of Primary membranous nephropathy
70%
26
TSHD71 Ab and Primary membranous nephropathy
Also expressed on podocytes Present in a small number of anti-PLA2R negative cases Associated with malignancy
27
If negative anti-PLA2R in Primary membranous nephropathy....
``` Look for secondary causes Drugs NSAIDs, penicillamine gold, Anti-TNF gold Lupus HBV Malignancy - CXR, screen for iron deficiency - Breast and colon malignancy screening - PSA in males >50-60 years ```
28
Management Primary membranous nephropathy
Rule out secondary causes - treat those! Risk assessment - GFR, level of proteinuria, complications such as albumin, thrombotic events - Level of PLA2R ab and pace of kidney deterioration Supportive care always come first - BP reduction - ACEI/ARB - Some patients with spontaneously remit +/- Immunosuppression (only those at risk of progression to ESKD) - Steroids alone - no benefit - Rituximab + CNI (tacrolimus, cyclosporin) - Cyclophosphamide + steroids (higher risk) Anticoagulation - Prophylaxis vs warfarin if serum albumin <20mg/day
29
Most common primary GN
IgA nephropathy
30
IgA nephropathy presentation
Can range from asymptomatic haematuria, synpharyngitic haematuria to RPGN HTN, proteinuria, reduced GFR M>F (Note can happen in context of vasculitis e.g. HSP - considered secondary GN) Histology LM: Mesangial hypercellulartiy and matrix expansion IF: IgA+ Poor prognosis: Interstitial damage and fibrosis, occasional crescents Prognosis 1/3 benign cause 1/3 slowly progressive (most) 1/3 progress to ESKD Prognostic factor Degree of proteinuria, HTN, reduced GFR Old age Interstitial fibrosis or crescents on biopsy
31
Pathogenesis of IgA nephropathy
"Multi-hit" model 1) IgA producing plasma cells in mucosal surface and lymphoid tissue 2) Increased levels of abnormal galactose deficient IgA Get Ab response to this abnormal IgA Ag-Ab immune complex deposit in kidneys (mesangium and endothelial side of GBM) and cause damage
32
IgA nephropathy which score can be used to predict kidney prognosis?
MEST-C biopsy score at time of biopsy + takes into account clinical factors
33
IgA nephropathy treatment
Supportive care always come first - ACEI/ARB - HCQ reduces proteinuria (awaiting phase 3 trial) - Fish oil (stabilise GFR) +/- 6/12 steroids are controversial - Trials have shown some benefits in stabilising GFR but increased serious adverse effects +++ and death - KDIGO recommends discussion with patient re toxicity especially when eGFR <50
34
RPGN presentation and histology
Medical emergency - Kidney failure within 6 weeks - Nephritic syndrome + constituitional symptoms + orther organ involvement - M=F, bimodal distribution Histology - Crescents >25% glomeruli - ANCA - necrosis, pauci-immune (no ab that stain) - Anti-GBM - linear IgG on IF - SLE - full house IF with both ab and complement deposited on multiple sites High risk of death
35
Most common cause for RPGN
ANCA associated vasculitis
36
RPGN management
Urgent diagnosis - Kidney biopsy, serology SLE, ANCA, anti-GBM Prednisolone - Pulse x3 then high dose oral +/- Consider cyclophosphamide (vasculitis, anti-GBM, SLE) or MMF (SLE) +/- Consider plasmapharesis - Anti-GBM - yes if any residual kidney function - ANCA - not really required ?only if lung haemorrhage/life threatening/Cr >500/anuric (no improved survival in trial) - SLE - no proven benefit
37
ANCA-associated vasculitis What is it? Histology Who?
Small vessel vasculitis, crescenteric, pauci immune Older patients Clinicopathologic variants and phenotypes - GPA - granulomas (PR3 +) - EGPA - asthma + eosinophilia + granulomas (either PR3/MPO+) - MPA (no granulomas) (MPO+) - Renal limited vasculitis (MPO+) Specify serotype - PR3 (C-ANCA) or MPO (P-ANCA), ANCA negative (10%), dual positive with anti-GBM PR3 more likely to have refractory disease/relapse, need longer maintenance
38
Pathogenesis of ANCA-associated vasculitis and GN
Neutrophils IN response to infection/inflammation, neutrophils are exposed to inflammatory cytokines, their intranuclear proteins (PR3, MPO) become expressed on cell surface --> primes neutrophils which then bind to ANCA, also express adhesion molecules and bind to vascular endothelium --> primed neutrophils undergo a specific type of cell death + collateral damage (neutrophil mediated destruction of endothelium)
39
ANCA-associated vasculitis and GN treatment
Induction + maintenance Steroids + cyclophosphamide or RTX ---> maintenance with AZA or MTX or MMF or RTZ Supportive care
40
Should persistence of ANCA positivity or increase in level in ANCA-associated vasculitis and GN change management?
No | Should not guide decisions
41
Lupus nephritis Type III, IV, V Why are these types important?
III: Focal proliferative GN IV: Diffuse proliferative FN V: Membranous GN Benefits of immunopression established
42
Lupus nephritis histology
"Full house" disease | Affects all components of the glomuerli and all complements and ab are present
43
Lupus nephritis type III, IV, V treatment
Induction IV methylpred followed by oral pred + MMF or cyclophosphamide Also HCQ, other kidney protective measures (BP mx), low salt diet Maintenance MMF or azathioprine Taper steroids Refractory Consider repeating kidney bx Multi target therapy (MMF + CNI) or RTX or IVIG or lefluonamide Wait 6/12 before changing treatment
44
``` Membranoproliferative GN What is it? Presentation Histology Prognosis Poor prognostic markers ```
What the biopsy looks like This is a pattern of injury on biopsy that have a number of different dx associated with complement Nephritic syndrome Histology Duplication of GBM "double contour" Chronic deposition of immunoglobulin, complement, fibrin along glomerular capillary wall --> cycles of inflammation and repair --> double contour IF: C3 only = C3GN, C3 + Ig = MPGN (IF staining for immunoglobulin and complement tells us cause) Prognosis 40% ESKD in 10 years Poor prognostic factors Nephrotic syndrome Reduced GFR Interstitial disease on biopsy
45
Membranoproliferative GN | Why is IF staining important?
Tells us underlying disease IF positive for Ig +/-C3 - Immunoglobulin/immune-complex mediated - Monoclonal: monoclonal gammopathies e.g. MM, amyloid - Polyclonal: autoimmune disease e.g. SLE, infection IF positive for C3 only - C3 glomerulopathies IF negative for both - Rule out thrombotic microangiopathy
46
Membranoproliferative GN | Management
Treat the underlying disease Idiopathic MPGN Supportive management +/- immunosuppression ?benefit Balance risk vs benefit But if RPGN - use strong immunosuppression ie cyclophosphamide
47
Pathogenesis of TMA/aHUS
Unregulated complement activation --> endothelial dysfunction --> immune activation --> platelet activation --> haemolysis
48
Eculizumab in aHUS/TMA
Significant time-dependent improvement in renal function

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