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Nephritic syndrome Flashcards

1
Q

What is RPGN

A

Severe glomerulonephritis fulminant course with rapid detoriaration in renal function over days to weeks to months - type of nephritic syndrome

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2
Q

What is nephritic syndrome

A

Haematruia, proteinuria, renal impairment, HPTN

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3
Q

Cuases of nephritic syndrome

A

Immune complex deposition (AI, post strep, IgA)
AntiGBM deposition
Small vessel vasculitis (polyangitis)

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4
Q

Causes of immune nephritic syndrome

A

SLE
Post strep glomerulonephritis
IgA nephropathy
Cryoglobuniaemia

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5
Q

Antibodies ass with small vessel vasculitis

A

ANCA
anti-GBM -

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6
Q

ANCA realted vasculitis can cause

A

Microscopic polyangitis - MPA
Grnaulomatosis with polyangitis
Eosinophilic granulomatosis w polyangtitis

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7
Q

Anti GBm targets

A

GBM antibodies target type I collagen within basement membrane
Linear depositon of IgG in glomerular capillaries

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8
Q

Symptoms of nephritic syndrome

A

Lethargy
Recent infection - fever, sore throat, coryzal symptoms - post strep glom and IgA nephropathy
Haematuria
Oliguria
Oedema - peripheral, periorbital
SOB - fluid overload
Haemoptysis - due to pulmonary haemorrhage eg anti-GBM ANCA-vasculitis

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9
Q

Signs of nephritic syndrome

A

Haematuria
HPTN
Oedema - peripheral, periorbital
Fluid overload - raised JVP, bibasal crackles on auscultation, peripheral oedema, ascites
Reduced urine output

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10
Q

Extra renal manifestations

A

Skin - vasculitis rash - palpable purpura
ENT - rhinosinusitis, nasal discharge, polychondritis (cartliage inflammation)
Eyes - red, painful eyes (eg conjunctivitis, scleritis, uveitis)
Lungs - haemoptysis, pleuritic pain, wheeze
Heart - chest pain - pericardial or MI involvement
Nervous system - mononeuropathies

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11
Q

How is nephritic syndrome diagnosed

A

Haematuria, proteinuria, deteriorating renal function

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12
Q

Glomerular origin of haematuria evidence

A

Dysmorphic RNC
Red blood cell casts

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13
Q

What is a cast

A

Cluster of particles wrapped in uromodulin - secreted by epothelial cells lining loop of Henle, distal tube and collecting duct

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14
Q

Manaegment of nephritic syndrome

A

General supportive measures for AKI
Temporary RRT
Immunosupressive therapy

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15
Q

Complications nephritic syndrome

A

Can cause severe AKI and ass complications
Can lead to widespread organ dysfunction

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16
Q

What does post infective glomerulonephritis occur after

A

Group A beta haemolytic streptococcus

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17
Q

What population is PSGN most common in

A

Children

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18
Q

post exposure length after infection for PSGN

A

1-3 weeks

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19
Q

Diagnosis

A

Haematuria, proteinuria, decline renal function + recent strep infetion
Anti-streptolysin titre - ASOT - evidence for recent infection
Renal biopsy in adults

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20
Q

RSPN management

A

Managing complications
Resolution normally rpaid and outcome excellent in children
Adults more varibale
Active infection - antibiotic

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21
Q

Renal biopsy findings of post infective glomerulone[hritis

A

Diffuse proliferative and exudative glomerular hisotry
Dominant C3 staining and subepithelial humps

22
Q

SLE nephropathy what causes

A

Anti double stranded DNA deposit in glomeruli, other immune complexes, area of deposition can include mesangium, enothelium or epithelium

23
Q

Classiications of lupus nephritis

A

Class I - minimal mesangial
Class II - mesangial proliferative
Class III - focal
Class IV - diffuse
Class V - membranous
Class VI - advance scleorisis

24
Q

Most common and severe form of SLE nephritis

A

Class IV diffuse - nephrotic or nephritis syndrome

25
How do class I, II and III of SLE nephritis present
I - asymptomatic II - non visible haem and proteinuria III - variabel, may have haematuria, proteinuria, renal impairment and/or nephrotic syndrome
26
Class V SLE nephritis vs Class VI presentation
V - nephrotic syndrome similar to membranous nephropathy VI - advance sclerosis - global sclerosis and slowly worsening renal function
27
Management SLE nephritis
Class IV and above High dose systemic immunosuppression require RRT
28
What causes IgA nephropathy
IgA Deposition of IgA nephropathy in the glomeruli
29
Treatment of IgA nephropathy
High dose prednisolone Immunosupressive drugs Good BP control, largely supportive
30
How does IgA nephropathy present
Nephritic syndrome 24-48 hours after URTI
31
Investigations for IgA nephropathy
Increased mesangial cells Increased matrix IgA deposition in matric
32
Prognosis of IgA nephropathy
20-30% -> renal failure
33
Pathology of anti GBM disease
antiGBM attacks basement membrane in lungs and kidney -> glomerulo/pulm haemorrhage
34
Treatment antiGBM
Potent immunosupression Plasmapheresis
35
What is membranoproliferative GN?
Glomerular basement membrane thickening Proliferative changes on light microscopy
36
What is membranoproliferative GN ass with
Hepatitis C AI eg SLE
37
Appearance of membranoproliferative disease on biospy
Thickened basement membrane Thickened mesangium Tram tracking appearance Subendothelial deposition of IgG - on immunoflurescence
38
Managmenet of membranoproliferative GN
Dipyridamole + aspirin Kidney transplantation - end stage renal disease
39
Managmenet of membranoproliferative GN in children with nephrotic range proteinuria
Corticosteroids
40
What is RPGN
Acute nephritic syndrome with microscopic glomerular crescent formation
41
What antibody do patients usually have in small vessel vasculitis
ANCA
42
What is GPA
Wegners - extremely rare long term systemic disorder - formation of granulomas + inflammation of small and medium blood vessles URTI, lungs, kidneys
43
What is GPa caused by AB?
c-ANCA
44
Biopsy of GPA shows
Leukocytoclastic vasculitis with necrotic changes and granulomatous inflammation
45
Management of GPA
Immunosupression - remission with rituximab or cyclophosphamide in combination with high dose corticosteroids
46
What is microscopic polyangtitis
Systemic small vessel vasculitis
47
Clinical features of microscopic polyangitis
weight loss, fevers, fatigue, renal failure
48
Managemnet of microscopic polyangitis
Long term prednisolone Cyclophosphamide therapy - cycled Plasmapheresis
49
What is goodpasteurs syndrome
When lung involvement with anti-GBM (haemoptysis) IgG
50
Management of anti-GBM disease
IV prednisolone Cyclophophamide Plasmapheresis

GI, liver, kidney (50 decks)

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