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I&I WC2 > Nephrology > Flashcards

Nephrology Flashcards

1
Q

Which symptoms often occur with mebranous nephropathy? (4)

A
  1. Hypertension
  2. Oedema
  3. Proteinuria
  4. Erythrocytes
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2
Q

What is the main problem in membranous nephropathy?

A

Inhibited podocyte function

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3
Q

Which type(s) of casts can be found in a nephrotic syndrome?

A

Fatty casts

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4
Q

Which type(s) can be found in nephritic syndrome?

A

Erythrocyte casts

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5
Q

Proteinuria occurs in case of [nephrotic/nephritic] syndrome

A

Proteinuria is characteristic for nephrotic syndrome

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6
Q

What is the upper limit of proteinuria in nephritic syndrome?

A

3,5 g/day

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7
Q

What is the lower limit of proteinuria in nephrotic syndrome?

A

3,5 g/day

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8
Q

Haematuria is characteristic of [nephrotic syndrome/nephritic syndrome], but can also occur in [nephrotic syndrome/nephritic syndrome]

A

Characteristic of nephritic syndrome, can also occur in nephrotic syndrome

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9
Q

Oedema is generally more extreme in [nephrotic syndrome/nephritic syndrome]

A

Nephrotic syndrome

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10
Q

What are the clinical features of nephrotic syndrome? (3)

A
  1. Generalized oedema
  2. Periorbital oedema
  3. Hypertension
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11
Q

What are the clinical features of nephritic syndrome? (2)

A
  1. Oedema
  2. Hypertension
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12
Q

What is the most common aetiology of chronic kidney disease?

A

Diabetes (44%)

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13
Q

What are the 5 most common aetiologies of chronic kidney disease?

A
  1. Diabetes (44%)
  2. Hypertension (29%)
  3. Other (18,4%)
  4. Glomerular disease (7%)
  5. Polycystic kidney disease (1,6%)
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14
Q

Into which two major groups can glomerular kidney disease be divided?

A
  1. Primary: caused by processes inside the kidney
  2. Secondary: caused by processes outside the kidney
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15
Q

What are primary glomerular diseases? (5)

A
  1. Minimal change disease
  2. Primary focal and segmental glomerulosclerosis (FSGS)
  3. Membranous nephropathy
  4. Membranoproliferative glomerulonephritis
  5. IgA nephropathy
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16
Q

What are secondary glomerular diseases? (6)

A
  1. Lupus nephritis
  2. Anti-GBM disease
  3. Amyloidosis
  4. Infection-related glomerulonephritis
  5. ANCA-associated vasculitis
  6. Atypical HUS
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17
Q

What are diagnostic blood tests for chronic kidney disease?

A
  1. Anti-nuclear antibodies (ANA)
  2. Anti-neutrophil cytoplasmic antibodies (ANCA)
  3. Anti-GBM antibodies
  4. Complement C3/C4
  5. Biomarkers of underlying disease
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18
Q

Which biomarkers of underlying disease should be checked in case of chronic kidney disease? (4)

A
  1. M-protein (haematological malignancy)
  2. Virology
  3. Anti-streptolysin titre
  4. Cryoglobulins
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19
Q

What is the cause of decreased podocyte function in membranous nephropathy?

A

Subendothelial deposits of antibodies inside the GBM

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20
Q

What is the causative antibody of primary membranous nephropathy?

A

anti-M-type phospholipase A2 receptor = PLA2R

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21
Q

What is the function of the M-type phospholipase A2 receptor?

A

Lipid homeostasis of podocytes

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22
Q

True or false: anti-PLA2R receptors always have affinity towards the same epitope

A

False; multiple parts of the receptor may raise a reaction

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23
Q

What happens after deposition of subendothelial antibodies in membranous nephropathy?

A
  1. Activation of complement
  2. Podocyte damage -> disturbed filtration capacity -> proteinuria
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24
Q

True or false: membranous nephropathy can only be caused by kidney-specific antibodies

A

False; secondary membranous nephropathy is caused by deposition of non-specific antibodies in the GBM

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25
Q

In which two ways can anti-PLA2R antibodies in membranous nephropathy be raised?

A
  1. Autoreactive B-cells bind PLA2R, leading to B-cell activation, antigen internalization & presentation on MHCII
  2. Endocytosis of PLA2R by professional APCs in the presence of microbial agents
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26
Q

Why is the presence of microbial agents key for APCs to be able to raise an auto-immune response after phagocytosing PLA2R?

A

Causes the APC to give pro-inflammatory costimulation to CD4+ T-cells

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27
Q

What happens after activation of CD4+ T-cells by B-cells/APCs in the raising of anti-PLA2R antibodies in membranous nephropathy?

A

Germinal centre reaction

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28
Q

Which processes happen in the germinal centre reaction of PLA2R-specific B-cells?

A
  1. Affinity maturation
  2. Class switch to IgG4
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29
Q

What is the most common isotype of anti-PLA2R antibodies in membranous nephropathy?

A

IgG4

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30
Q

Which steps in the raising of anti-PLA2R antibodies in membranous nephropathy may be blocked? (3)

A
  1. DC activation/co-stimulation of T-cells
  2. Clonal expansion of autoreactive B-cells
  3. Production of auto-antibodies by plasmablasts
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31
Q

How is membranous nephropathy diagnosed?

A

Serology + kidney biopsy

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32
Q

How can a kidney biopsy confirm membranous nephropathy?

A

Anti-PLA2R antibodies concentrated on the GBM of the kidney

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33
Q

Why is kidney biopsy more sensitive for the diagnostics of membranous nephropathy than serology?

A

Anti-PLA2R titres in serum may be low, while these antibodies accumulate in the kidney due to their specificity

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34
Q

What is the 5-, 10- and 15-year prognosis of (primary) membranous nephropathy?

A

5-year: ~60% survival
10-year: ~40% survival
15-year: ~30% survival

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35
Q

What is the main treatment goal when treating membranous nephropathy?

A

Prevent kidney failure

36
Q

True or false: membranous nephropathy can resolve itself through spontaneous remission

37
Q

Which targets can be identified for the treatment of membranous nephropathy?

A
  1. T-cells & cytokines
  2. B-cells
  3. Complement
38
Q

What is the most frequently used treatment for membranous nephropathy?

A

Rituximab (anti-CD20)

39
Q

What is the second line treatment for membranous nephropathy (after rituximab failure)?

A

Cyclophosphamide

40
Q

What are the side effects of treatment with cyclophosphamide? (2)

A
  1. Infertility
  2. Kaposi sarcoma
41
Q

How can kidney failure be treated if it does occur in membranous nephropathy?

A
  1. Dialysis (=bridging therapy)
  2. Kidney transplant (=only permanent solution)
42
Q

Which parameters are used to determine the treatment success of membranous nephropathy treatment?

A
  1. Proteinuria (filtration capacity)
  2. eGFR (kidney function)
  3. Creatinine (kidney function)
  4. Serum albumin (filtration capacity)
  5. Antibody titre
43
Q

What are fragmentocytes in a blood sample indicative of?

A

Erythrocyte lysis

44
Q

What is a low haptoglobin indicative of?

A

Erythrocyte lysis

45
Q

What is the function of haptoglobin?

A

Binding of free haemoglobin in circulation

46
Q

What are the three hallmarks of haemolytic uraemic syndrome?

A
  1. Microangiopathic haemolytic anaemia
  2. Thrombocytopenia
  3. Acute kidney injury
47
Q

Which to processes occur in microangiopathic haemolytic anaemia?

A
  1. Small vessel disease
  2. Destruction of erythrocytes
48
Q

How does erythrocyte destruction occur in microangiopathic haemolytic anaemia?

A

Fibrin sheaths in the small blood vessels lyse erythrocytes

49
Q

How does acute kidney injury in haemolytic anaemic syndrome occur?

A

Narrowing & occlusion of renal arterioles by thrombi & erythrocytes

50
Q

What is ‘typical’ HUS?

A

STEC-HUS -> caused by Shiga toxin-producing E. coli (STEC)

51
Q

In which group does STEC-HUS most frequently occur?

A

Childeren <5 years (90%)

52
Q

What is the incidence of STEC-HUS in children?

A

~3/100.000

53
Q

What is the epidemiological pattern of STEC-HUS?

A

Outbreaks -> occurs in parallel with Shiga toxin-producing E. coli outbreaks

54
Q

What is the most common trigger/cause of HUS in adults?

A

Coexisting disease

55
Q

Which coexisting diseases can trigger HUS in adults? (7)

A
  1. HSCT
  2. Solid organ transplantation
  3. Malignancy
  4. Auto-immune diseases
  5. Drugs
  6. Malignant hypertension
  7. Pre-existing nephropathy
56
Q

What is atypical HUS?

A

Complement-mediated TMA

57
Q

What is the incidence of atypical HUS? In which group does it mostly occur?

A

~7/million, mostly in children

58
Q

What is TMA? (complement-mediated TMA = atypical HUS)

A

Thrombocytic microangiopathy

59
Q

What is the cause of atypical HUS/anti-TMA syndrome? What are the underlying problems?

A

Dysregulation of the alternative complement pathway due to
1. Antibodies against complement factor H
2. Mutations in complement factor H

60
Q

What is an important underlying principle of the complement system?

A

Depends on cleavage concepts of readily available proteins, which leads to rapid amplification of the response

61
Q

What is the end stadium of all complement pathways?

A

Formation of membrane attack complexes (MAC)

62
Q

Which two steps are central to all complement pathways?

A
  1. C3 convertases
  2. C5 convertases
63
Q

How is the alternative complement pathway activated?

A

Continuous low-level deposition of C3 on all cells

64
Q

How does the alternative complement pathway prevent attack on human cells?

A

Human cells express inhibitory proteins that block this complement pathway

65
Q

Which two mechanisms do human cells use to inhibit the alternative complement pathway?

A
  1. Prevention of association between C3b and Bb
  2. Regulation of MAC-formation
66
Q

How do self-cells prevent the association between C3b and Bb?

A
  1. Decay-associated factor prevents association between C3b and fB on the cell surface
  2. CD46 stabilizes factor I on the surface -> inhibitory
  3. Soluble factor H prevents factor B binding to C3 & promotes their dissociation
67
Q

What is de CD number of decay-associated factor? What is its abbreviation?

A

CD55 = DAF

68
Q

What is the precursor of factor Bb?

69
Q

What is the function of DAF in the regulation of the alternative complement pathway?

A

Prevents association between C3b and Bb fB on the cell surface -> stops early activation of the alternative complement pathway

70
Q

What is the function of factor I in the regulation of the alternative complement pathway?

A

Stabilizes association of fH to C3b -> prevents C3b binding to fB

71
Q

Which molecule do self-cells use to prevent MAC-formation on their surface?

72
Q

What is the mechanism by which CD59 prevents MAC formation?

A

Prevents C8-C9 association

73
Q

What happens when human cells cannot effectively regulate (the alternative) complement pathway(s)?

A

Complement attack on se-flcells

74
Q

Which molecule inhibits the classical and mannose complement pathways?

75
Q

What is the mechanism by which C1-inh prevents complement activation?

A

Irreversibly inactivates C1r/C1s (classical pathway) or MASP (mannose pathway)

76
Q

What are the main complement factors that affect clotting?

77
Q

Which effects does MAC have on clotting?

A
  1. Activates platelets
  2. Induces erythrocyte lysis
  3. Induces endothelial cell damage –> extra clotting surface
78
Q

What effects does C5a have on clotting?

A
  1. Activates platelets
  2. Expression of tissue factor
  3. Plasminogen-activator inhibitor 1 (PA-I1)
79
Q

Which major factors can disturb complement regulation? (2)

A
  1. Gene mutations
  2. Anti-fH antibodies
80
Q

Which gene mutations can cause complement dysregulation?

A
  1. Factor H/factor I mutations
  2. CD46/MCP defects
  3. C3 mutations blocking fH-binding
  4. Gain-of-function mutation of fB
81
Q

Which strategies can be used for the treatment of aHUS? (4)

A
  1. Plasmapheresis
  2. Dialysis to replace kidney function
  3. Kidney transplantation
  4. Complement-targeting biologicals
82
Q

What is the biggest determinant of aHUS disease recurrence after kidney transplantation?

A

Dependent on underlying mutation ->
-If mutation in soluble factor (fH, fI, C3, fB) -> not removed by replacing organ
-If mutation on cell surface (CD46) -> solved with donor organ

83
Q

Which two drugs are currently commonly used for complement inhibition? Which protein do they target?

A

Eculizumab/ravulizumab -> target C5

84
Q

Which 3 goals are the main research goals needed to improve treatment for aHUS?

A
  1. Improved understanding of complement regulation
  2. Discovery of new causative mutations/antibodies
  3. Diagnosis of previously undiagnosed complement dysregulation
85
Q

Why are there likely a high number of undiagnosed aHUS cases?

A

Often misdiagnosed as end-stage kidney disease due to hypertensive nephropathy -> underlying complement disorder missed

I&I WC2 (18 decks)

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