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#Phase 2.1: GI > Glomerular Diseases > Flashcards

Glomerular Diseases Flashcards

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1
Q

What are Glomerular Diseases?

A

Group of conditions that damage Renal Glomeruli (site of ultrafiltration)

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2
Q

What can Glomerular diseases cause?

A

AKI / CKD

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3
Q

What are the 2 general clinical manifestations of Glomerular disease?

A

Nephrotic Syndrome

Nephritic Syndrome

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4
Q

What are the Kindey’s uses? (5 things)

A
  1. Waste product excretion
  2. Acid-base balance
  3. Fluid balance
  4. Calcium-phosphate balance
  5. BP control
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5
Q

What does a Bowman’s Capsule involve?

A
  1. Capillaries
  2. Mesanguim (Cells + CT supporting capillaries)
  3. Filtration barrier:
  • Endothelial cell (capillary wall)
  • Basement Membrane (made from Collagen + Glycoproteins)
  • Foot processes of Podocytes
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6
Q

What is the essence of Glomerular disease pathophysiology?

A

Damage to Filtration barrier –> leakage of Protein / Blood

aka Proteinuria / Haematuria

aka The Hallmarks of Glomerular Disease

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7
Q

What are the layers of the Filtration Barrier of the Glomerulus? (3 things)

A
  1. Endothelial cell (capillary wall)
  2. Basement Membrane (made from Collagen + Glycoproteins)
  3. Foot processes of Podocytes (specialised epithelial cells)
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8
Q

What are the different ways Glomerular Diseases are classified? (4 things)

A
  1. Primary vs Secondary
  2. Focal vs Diffuse
  3. Global vs Segmental
  4. Immune vs Non-immune mediated (Pathophysiological Classificiation)
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9
Q

What is the difference between Primary n Secondary Glomerular Diseases?

A

Primary: Glomerular injury bc renal pathology

Secondary: Bc systemic process

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10
Q

What are examples of PRIMARY Glomerular Diseases? (3 things)

A
  1. IgA nephropathy
  2. Minimal change disease
  3. Focal segmental glomerulosclerosis
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11
Q

What are examples of systemic processes causing SECONDARY Glomerular Diseases? (3 things)

A
  1. Vasculitis
  2. Amyloidosis
  3. DM
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12
Q

What is the difference between Focal n Diffuse Glomerular Diseases?

A

Focal: Less than 50% of glomeruli damaged

Diffuse: More than 50% of glomeruli damaged

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13
Q

How can you diagnose between Focal n Diffuse Glomerular diseases?

A

Renal biopsy will tell you

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14
Q

What is the difference between Global n Segmental Glomerular Diseases?

A

Global = More than 50% of each individual glomerulus damaged

Segmental = Less than 50% of each individual glomerulus damaged

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15
Q

What is the Pathophysiology of IMMUNE Mediated Glomerular Disease? (2 types)

A
  1. Deposition / In-situ formation of Immune Complexes (iG collections) –> activate Inflamm response
  2. Circulating iGs come n clart proteins on BM / Endothelial cells –> activate Inflamm response (Vasculitis)
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16
Q

What is the Pathophysiology of NON-IMMUNE Mediated Glomerular Disease? (2 types)

A
  1. Structure / Function of Podocytes clarted –> Macromolecule leak thru
  2. Protein accum –> disrupts glomeruli structure –> Dysfunction (DM / Amyloidosis)
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17
Q

What are the different histopathological changes in Glomerular disease? (3 things)

A
  1. Structural
  2. Proliferative
  3. Crescents
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18
Q

What are the features of STRUCTURAL Histopathological patterns in Glomerular Disease? (2 things)

A
  1. Structural changes in Glomerulus
  2. Sclerosis (scarring of glomerulus)
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19
Q

What are the STRUCTURAL Histopathological patterns in Glomerular Disease associated with?

A

Excess prot loss (Nephrotic Syndrome)

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20
Q

What are the features of PROLIFERATIVE Histopathological patterns in Glomerular Disease?

A

Increase in number of cells in Glomerulus: In Mesanguim / Capillary Wall / Bowman’s Space

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21
Q

What are the PROLIFERATIVE Histopathological patterns in Glomerular Disease associated with? (2 things)

A
  1. Inflamm response bc immune complex deposition
  2. Haematuria +/e Nephritic Syndrome
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22
Q

What are the features of CRESCENTS Histopathological patterns in Glomerular Disease? (2 things)

A
  1. Extra-Capillary lesions in Bowman’s Capsulse
  2. Bc accum of: Immune cells / Epithelial Cells / Fibroblasts / Fibrin
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23
Q

What do the CRESCENTS Histopathological patterns in Glomerular Disease represent?

A

Represent: SEVERE injury –> Glomerular Membrane Rupture

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24
Q

What are the CRESCENTS Histopathological patterns in Glomerular Disease associated with? (2 things)

A
  1. Any Inflamm glomerular diseases
  2. BUT MAINLY: Rapidly Progressive Glomerulonephritis (RPGN)
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25
What are the general manifestations of Glomerular Disease? (4 things)
1. Isolated haematuria (by itself) 2. Isolated proteinuria (by itself) 3. Nephrotic Syndrome 4. Nephritic Syndrome
26
What are typical causes of Isolated haematuria? (3 things)
1. IgA nephropathy 2. Alport syndrome 3. Thin basement membrane disease
27
What is Nephrotic Syndrome? (3 things)
Triad of: 1. Heavy Proteinuria (3.5+ g / day) (aka Nephrotic Range) 2. Hypoalbuminaemia (35- g / day) 3. Peripheral Oedema
28
What can also accompany the Triad in Nephrotic Syndrome? (4 things)
1. Hyperlipidaemia 2. Thrombotic disease (venous / arterial clots) 3. Ascites 4. Pleural effusions
29
What are the PRIMARY Glomerular diseases that present w Nephrotic Syndrome? (3 things)
1. Minimal change disease 2. Focal Segmental Glomerulosclerosis (FSGS) 3. Membranous Nephropathy
30
What are the SECONDARY systemic conditions that present w Nephrotic Syndrome? (4 things)
1. DM 2. Amyloidosis 3. Lupus 4. HIV
31
What is Nephritic Syndrome? (4 things)
1. Haematuria 2. Variable Proteinuria (can be Nephrotic Range) 3. Oliguria (AKI) 4. HTN
32
What is the term Nephritic Syndrome used interchangeably with?
Glomerulonephritis (inflamm of glomerulus)
33
How does Mild vs Severe Glomerulonephritis present?
Mild: Minimal haematuria + proteinuria ONLY Severe: Nephrtic Syndrome (all 4 parts)
34
What are main causes of Glomerulonephritis? (3 things)
1. Immune complex deposition 2. Anti-GBM deposition (Anti Glomerular Basement Bembrane Disease) 3. Small vessel vasculitis
35
What are the Glomerular diseases that do the Immune Complex deposition cause of Nephritic Syndrome? (3 things)
1. IgA Nephropathy 2. Poststreptococcal glomerulonephritis 3. Lupus (SECONDARY)
36
What are the Glomerular diseases that do the Small-vessel vasculitis cause of Nephritic Syndrome? (3 things)
1. Eosinophilic Granulomatosis w Polyangiitis 2. Microscopic Polyangiitis 3. Polyangiitis w Granulomatosis
37
What investigations should you do for sus Glomerular Diseases? (5 things)
1. Renal biopsy (GOLD) 2. Urinary tests 3. Blood tests 4. Renal screen 5. Imaging
38
What is a Renal Screen? (2 things)
1. Special blood tests 2. Look for causes of AKI / Glomerular disease
39
What imaging should you do for sus Glomerular Diseases? (2 things)
1. US 2. CT KUB (Kindeys + Ureters + Bladder)
40
What is the use of imaging in sus Glomerular Diseases? (2 things)
1. Exclude obst 2. Look at kidney structure
41
When should you NOT do a Renal Biopsy in sus Glomerular Diseases? (5 things)
1. CI: Uncorrectable Bleeding Diathesis 2. CI: Uncontrollable severe HTN 3. CI: Active Renal / Perirenal infection 4. Diagnosis already confirmed w Serology 5. Presentation beh stereotypical (e.g Nephrotic Syndrome in kids is probs Minimal Change Disease)
42
What are NEPHRITIC Glomerulonephritis Diseases from Oxf Handbook? (5 things)
1. IgA nephropathy 2. Henoch–Schönlein purpura (HSP) 3. Post-Streptococcal Glomerulonephritis 4. Anti-glomerular basement membrane (anti-GBM) disease 5. Rapidly Progressive Glomerulonephritis (RPGN)
43
What is IgA Nephropathy?
1. aka Berger's Disease 2. Most common cause of Glomerulonephritis
44
What are the CF of IgA Nephropathy? (4 things)
1. Asymptomatic 2. Nephritic Syndrome
45
What is the classic patient who presents with IgA Nephropathy?
Young male w UPRTI
46
What is the Pathophysiology of IgA Nephropathy?
Mesangial deposition of IgA immune complexes (Mesangium: Cells + CT surrounding capillaries)
47
What is the speed of progression of IgA Nephropathy? (2 things)
1. Slow 2. Progresses to Renal Failure over 30 years
48
How is IgA Nephropathy diagnosed? What will you see? (2 things)
Renal biopsy: 1. IgA deposition in mesangium 2. Mesangial proliferation (cah dey usually happen bc immune complex deposition)
49
What are the management options for IgA Nephropathy? (3 things)
1. ACE Inhibitors / Angiotensin Receptor Blockers (ARB) * Reduce proteinuria * Protect renal function 1. Corticosteroids (stop immune system from making defective IgA 2. Fish oil (prevent inflammation --\> slows down disease progression) (Give Corticosteroids / Fish oil if 3-6 months of ACEi/ARB not fixing proteinuria)
50
What is Henoch–Schönlein purpura (HSP)? (2 things)
1. Small vessel vasculitis 2. Systemic variant of IgA nephropathy
51
Where is IgA depositied in Henoch–Schönlein purpura (HSP)? (4 things)
1. Skin 2. Joints 3. Gut 4. As well as Kidney
52
What are the CF of Henoch–Schönlein purpura (HSP)? (4 things)
1. Nephritis (Haematuria, Proteinuria, Oliguria, HTN) (KIDNEY) 2. Abd pain (GI bleed) (GUT) 3. Flitting polyarhritis (one joint gets better --\> another gets worse) (JOINTS) 4. Purple rash on legs (SKIN)
53
How is Henoch–Schönlein purpura (HSP) diagnosed? (2 things)
1. Clinical diagnosis 2. Confirmed w: Positive Immunofluorescence for IgA (biopsy) (Renal biopsy same as IgA Nephropathy)
54
What are the management options for Henoch–Schönlein purpura (HSP)?
Same as IgA Nephropathy 1. ACE Inhibitors / Angiotensin Receptor Blockers (ARB) * Reduce proteinuria * Protect renal function 1. Corticosteroids (stop immune system from making defective IgA **(+ steroids help gut involvement in HSP)** 2. Fish oil (prevent inflammation --\> slows down disease progression)
55
What is Post-streptococcal Glomerulonephritis (PSGN)?
Complication of Group A Streptococci (GAS) infection (aka bac skin / throat infection)
56
What is the pathophysiology of Post-streptococcal glomerulonephritis? (2 things)
1. Streptococcal antigen deposits in Glomerulus --\> Immune complex formation + Inflamm 2. Proliferation (PSGN is aka Acute Proliferative GN)
57
What are the risk factors for Post-streptococcal glomerulonephritis? (2 things)
1. Kid 2. Male
58
What are the CF of Post-streptococcal glomerulonephritis? (3 things)
1. Nephritic Syndrome 2. Generalised oedema 3. Fever / headache (from GAS infection)
59
Why is a Renal Biopsy rarely needed for Post-streptococcal glomerulonephritis, even though it would give a Definitive diagnosis?
Post-streptococcal glomerulonephritis is self-limiting, so wts da point
60
How is a diagnosis of Post-streptococcal glomerulonephritis made (3 things)
1. Serology (anti-DNase B antibody = evidence of GAS infection) 2. Culture (throat / skin swab = to confirm GAS infection) 3. Urinalysis (haematuria)
61
What are the management options for Post-streptococcal glomerulonephritis? (2 things)
1. Diuretics (to clear generalised oedema + high BP) 2. Abx (if evidence of persistent GAS infection)
62
What is Anti-glomerular basement membrane (anti-GBM) disease? 2 things)
1. aka Goodpasture's Disease 2. Rare
63
What is the pathophysiology of Anti-glomerular basement membrane (anti-GBM) disease? (2 things)
1. Antibodies attack alpha 3 subunit of type 4 collagen (in BM of kidney glomeruli + lung alveoli) 2. Auto-reactive T cells also attack alpha 3 subunits --\> Crescents (indicate severe damage)
64
What are the CF of Anti-glomerular basement membrane (anti-GBM) disease? (4 things)
Renal CF: Nephritic Syndrome + Renal Failure Lung CF (bc pulmonary haemorrhage) 1. Haemoptysis 2. SOB 3. Cough
65
What indicate poor prognosis of Anti-glomerular basement membrane (anti-GBM) disease? (2 things)
1. Dialysis dependance 2. Crescents in biopsy
66
How is Anti-glomerular basement membrane (anti-GBM) disease diagnosed? (2 things)
1. Renal biopsy (crescenteric GN) 2. Bloods: Anti-GBM antibodies
67
What are the management options for Anti-glomerular basement membrane (anti-GBM) disease? (3 things)
1. Plasma exchange (to remove anti-GBM antibody + inflamm mediators) 2. Corticosteroids 3. Cyclophosphamide (chemo to suppress immune system)
68
What is Rapidly Progressive Glomerulonephritis?
Any aggressive GN that progresses to Renal failure in days / weeks
69
What are the causes of Rapidly Progressive Glomerulonephritis? (5 things)
1. Small vessel vasculitis 2. Lupus nephritis 3. Anti-GBM disease 4. IgA Nephropathy 5. Membranous GN
70
How is Rapidly Progressive GN diagnosed?
Renal biopsy (crescenteric GN: bc breaks in GBM allow inflamm cell influx)
71
What are the management options for Rapidly progressive GN? (4 things)
1. Plasma-exchange (if caused by anti-GBM) 2. Corticosteroids 3. Cyclophosphamide (chemo to suppress immune system) 4. Monoclonal antibodies (if caused by Lupus Nephritis)
72
What are NEPHROTIC Glomerulonephritis Diseases from Oxf Handbook? (4 things)
1. Minimal Change Disease 2. Focal Segmental Glomerulosclerosis (FSGS) 3. Membranous Glomerulonephritis 4. Membranoproliferative Glomerulonephritis
73
What is Minimal Change Disease? (2 things)
1. Most common cause of Nephrotic Syndrome in kids 2. 25% of Nephrotic Syndrome in adults
74
What is the pathophysiology of Minimal Change Disease? (2 things)
1. Podocytes damaged by T cells + Cytokines 2. Foot processes of Podocytes damaged --\> filtration barrier more permeable to Albumin (explain Hypoalbuminaemia of Nephrotic Syndrome)
75
What are the causes of Minimal Change Disease? (3 things)
1. Idiopathic (mostly) 2. Drugs: NSAIDs / Lithium / Rifampicin 3. Haematological Malignancy (usually Hodgkin’s lymphoma)
76
What is good about Minimal Change Disease?
It doesn't progress to Renal Failure (if you see dat, den sus Focal Segmental Glomerulosclerosis FSGS)
77
What are the CF of Minimal Change Disease?
Nephrotic Syndrome (Proteinuria + Hypoalbuminaemia + Peripheral Oedema)
78
How is Minimal Change Disease diagnosed? (2 things)
Renal Biopsy: 1. Electron Microscopy: Shows erasure of Foot processes of Podocytes 2. Light Micropscopy: Normal (hence the name "_Minimal_ Change Disease"
79
What are the management options for Minimal Change Disease? (2 things)
1. Corticosteroids (80% steroid responsive) 2. Cyclophosphamide (chemo to suppress immune system) (give if unresponsive to steroids)
80
What is Focal Segmental Glomerulosclerosis (FSGS)? (3 things)
1. Focal: less than 50% of glomeruli damaged 2. Segmental: less than 50% of each individual glomerulus is damaged 3. Glomerusclerosis: scarring (--\> proteinuria)
81
What are the causes of Focal Segmental Glomerulosclerosis (FSGS)? (6 things)
1. Idiopathic 2. 2ndary to IgA Nephropathy 3. HIV 4. Heroin 5. Alport's syndrome 6. Sickle-cell
82
What are the CF of Focal Segmental Glomerulosclerosis (FSGS)? (5 things)
1. Nephrotic Syndrome (proteinuria / hypoalbuminaemia / peripheral oedema) 2. Hyperlipidaemia 3. Haematuria (50% 4. Swelling around eye (80%) 5. Facial swelling (60%)
83
How is Focal Segmental Glomerulosclerosis (FSGS) diagnosed? (2 things)
Renal biopsy 1. Light microscopy: Focal Segmental Glomerulosclerosis (literally what u see) 2. Electron miscroscopy: Erasure of foot processes of Podocytes
84
What is bad about Focal Segmental Glomerulosclerosis? (2 things)
1. Risk of Progressive CKD + Renal Failure 2. Will recur in 30-50% of Kidney transplants
85
What tells you if Focal Segmental Glomerulosclerosis has a bad prognosis?
High proteinuria
86
What are the management options for Focal Segmental Glomerulosclerosis? (4 things)
1. ACE inhibitors / ARB (to reduce BP + proteinuria) 2. Diuretics (for oedema) 3. Corticosteroids (only if idiopathic) 4. Plasma exchange (if recurrence after transplants)
87
What is Membranous Glomerulonephritis? (2 things)
1. Most common GN in adults 2. 3rd most common cause of End-Stage Renal Failure (ESRF)
88
What are the causes of Membranous GN? (5 things)
1. Idiopathic (bc anti-phospholipase A2 antibodies) 2. Inf: Hep B / Malaria / Syphilis 3. Cancer: Lung cancer / Lymphoma / Leukaemia 4. AI: Lupus / RA / Thyroiditis 5. Drugs: Gold / Penicillamine / NSAIDs
89
What are the CF of Membranous GN? (4 things)
1. Often asymptomatic 2. Nephrotic Syndrome (Proteinuria / Hypoalbuminaemia / Peripheral Oedema) 3. Haematuria (50% 4. Facial swelling (40%)
90
How is Membranous GN diagnosed? (2 things)
1. Renal biopsy: * Light microscopy: Diffuse thickening of GBM (bc subepithelial deposits) * Electron Microscopy: Spiky appearance bc thick GBM 1. Serology: Anti-phospholipase A2 receptor antibody
91
What are the management options for Membranous GN? (3 things)
1. ACE inhibitors / ARB (to reduce BP + proteinuria) 2. Corticosteroids + Cyclophosphamide (to suppress immune system)
92
What are the 2 types of Membranoproliferative Glomerulonephritis?
1. Immune Complex associated 2. C3 Glomerulopathy
93
What is the pathophysiology of Immune Complex associated Membranoproliferative GN? (2 things)
1. Subendothelial immune deposits of electron dense material 2. Caused by: Infection (Hep C) / Cryoglobulinaemia / AI
94
What is the pathophysiology of C3 Glomerulopathy Membranoproliferative GN?
Genetic / acquired defect in complement pathway e.g C3b nephritic factor found in 70%
95
What are the CF of Membranoproliferative GN? (2 things)
1. Nephrotic Syndrome (Proteinuria / Hypoalbuminaemia / Peripheral Oedema) 2. Nephritic Syndrome (Proteinuria / Haematuria / Oliguria / High BP)
96
How is Membranoproliferative GN diagnosed? (2 things)
1. Renal biopsy: * Mesangial cell proliferation (hence da name) * Subendothelial deposits * BM thickening 1. Serology: C3 nephrotic factor (distinguishes C3 glomerulopathy from Immune Complex associateD)
97
What are the management options for Membranoproliferative GN? (3 things)
1. ACE inhibitors / ARB (to reduce BP + proteinuria) 2. Treat underlying cause in Immune Complex associated 3. Corticosteroids + Cyclophosphamide (to suppress immune system) (if no underlying cause found)

#Phase 2.1: GI (29 decks)

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