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3B week 1 - abnormal urine > glomerulonephritis > Flashcards

glomerulonephritis Flashcards

1
Q

what are mesangial cell and what is their function

A

smooth muscle–like cells that contain actin and myosin -> they connect to each other via gap junctions and to the Glomerular Basement Membrane via cell processes;
Contraction of mesangial cells regulates the size of the capillary lumen and thus the amount of glomerular blood flow

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2
Q

what is a focal glomerulonephritis

A

affecting only some glomeruli

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3
Q

what is a diffuse glomerulonephritis

A

affecting all glomeruli

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4
Q

what is a segmental glomerulonephritis

A

affecting only one part of the glomerulus

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5
Q

what is a global glomerulonephritis

A

affecting the whole glomerulus

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6
Q

what is proliferation

A

increase in number of cells

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7
Q

what is cell expansion

A

increase in intracellular matrix

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8
Q

what is crescent proliferation

A

proliferation of cells within the bowman’s space

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9
Q

2 mechanisms underlying glomerulonephritides

A
  1. immunological
  2. vascular
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10
Q

types of immunological glomerulonephritides + examples !!! (4)

A
  1. antibody binding to INTRINSIC glomerular antigens (i.e. the body happens to have auto-antibodies to glomerular antigens) e.g. goodpastures;
  2. antibody binding to PLANTED glomerular antigens e.g. post strep glomerulonephritis;
  3. deposition of circulating antigen-antibody complexes -> in active autoimmine disease, complexes can deposit nonspecifically in the BM e.g. lupus nephritis
  4. NON-SPECIFIC DEPOSITION of circulating antibodies -> binds through the heavy chain (constant region) resulting in more sticky antibodies that can bind randomly e.g. IgA nephropathy
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11
Q

brief pathophys of goodpasture’s disease

A

antibodies against T4 collagen -> T4 collagen is used to form the basement membrane => destruction of glomerular basement membrane

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12
Q

what is “pauci-immune” crescentic glomerulonephritis

A

an idiopathic form of crescentic glomerulonephritis that typically lacks significant deposits within glomeruli and most often is associated with ANCA -> systemic vasculitis

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13
Q

indications for renal biopsy (6)

A
  1. nephrotic syndrome
  2. renal dysfunction of unknown cause (e.g. AKI with unknown cause)
  3. to guide treatment of assess prognosis where diagnosis is known
  4. dysfucntion of kidney transplant (sus of rejection)
  5. haematuria
  6. proteinuria

might not biopsy in last 2

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14
Q

complications of renal biospy (2)

A
  1. pain
  2. bleeding -> may need embolisation/nephrectomy, blood transfusion and may result in death
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15
Q

contraindications to renal biopsy

A
  1. abnormal clotting/thrombocytopenia -> take a drug history!!
  2. drugs (e.g. aspirin, clopi, warfarin)
  3. uncontrolled hypertension
  4. single kidney (risk of damaging only working kidney, can still biopsy but only if absolutely necessary)
  5. hydronephrosis
  6. urinary tract infection
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16
Q

what imaging guidance is used in renal biopsies

A

ultrasound

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17
Q

what does the EM of minimal change disease biopsy show

A

fusion of podocyte foot processes -> biopsy looks normal on light microscopy so EM must be done

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18
Q

what drug class is often implicated in minimal change disease

A

NSAIDs

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19
Q

what malignancy is often implicated in minimal change disease

A

lymphoma; leukemia

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20
Q

how does focal segmental glomerulosclerosis present

A

with nephrotic syndrome and renal impairment

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21
Q

what is seen on biopsy of focal segmental glomerulosclerosis (if correct area taken)

A

segmental sclerotic lesions with C3 and IgM deposition

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22
Q

if nephrotic syndrome doesn’t improve with steroids, what underlying cause should be suspected

A
  1. focal segmental glomerulosclerosis !
  2. membranous nephropathy
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23
Q

what can FSGS and membranous nephrapthy progress to

A

CKD

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24
Q

secondary causes of focal segmental glomerulosclerosis (4)

A
  1. obesity (hyperfiltration causes damage)
  2. IV heroin use
  3. HIV
  4. drugs (e.g.pamidronate)
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25
Q

what is the commonest cause of nephrotic syndrome in adults

A

membranous nephropathy

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26
Q

pathophys of membranous nephropathy

A

an autoimmune disorder in which immune complexes deposit along the subepithelial region of the glomerular basement membrane (IgG)

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27
Q

what is membranous nephropathy associated with (3)

A
  1. solid organ malignancy
  2. drugs (e.g. gold, penicillamine, captopril)
  3. infections (e.g. hepaitis, malaria)
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28
Q

what is the rule of 1/3s if membranous nephropathy is untreated

A

1/3 improve spontaneously
1/3 remain the same
1/3 develop progressive disease

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29
Q

how can mesangiocpillary glomerulonephritis (aka membranoproliferative GN) present

A

nephrotic or nephritic syndrome

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30
Q

mesangiocpillary glomerulonephritis biopsy findings

A
  1. mesangial proliferation (often lobular)
  2. thickened capillary walls -> “double contouring” of basement membrane
  3. +ve immunoflouresence
31
Q

what other conditions is mesangiocapillary glomerulonephritis associated with (3)

A
  1. infections eg. hepatitis, malaria, endocarditis, shunt nephritis
  2. cryoglobinaemia (abnormal antibodies that precipitate in the cold)
  3. malignancy
32
Q

what is shunt nephritis

A

a rare disease of the kidney that can occur in patients being treated for hydrocephalus with a cerebral shunt (infection of shunts being used to drain)

33
Q

what glomerulonephritis may recurr post transplant

A

mesangiocapillary glomerulonephritis

34
Q

what is the earliest clinical feature of diabetic nephropathy

A

microalbuminuria -> low level proteinuria (too low for dipstick detection)

35
Q

how does diabetic nephropathy progress

A
  1. microalbuminuria
  2. protienuria becomes heavier (+/- nephrotic syndrome)
  3. progressive decline in GFR (goes up before it comes down due to hyperfiltration and then damage)
36
Q

what does a diabetic nephropathy renal biopsy often show

A

kimmelstiel-wilson lesions -> Nodules of pink hyaline material form in regions of glomerular capillary loops in the glomerulus

37
Q

what are the 2 types of amyloidosis

A

AL - primary amyloidosis, light chain deposition e.g. myeloma
AA - chronic inflammation e.g. infection, connective tissue disorders

38
Q

what is amyloidosis

A

deposition of amyloid within multiple organs, including kidneys

39
Q

causes of nephrotic syndrome (9)

A
  1. minimal change (children)
  2. FSGS
  3. membranous nephropathy (single commonest cause in adults)
  4. mesangiocapillary glomerulonephritis
  5. other proliferative
  6. diabetes
  7. amyloid
  8. lupus
40
Q

what is the commonest glomerulonephritis in the world

A

IgA nephropathy (berger’s disease)

41
Q

buerger’s disease presentation (5)

A
  1. painful blue fingertips
  2. rest pain
  3. Hx of smoking (and being a young man)
  4. microscopic haematuria/episodic macroscopic haematuria
  5. nephrotic syndrome
42
Q

IgA vasculitis (henoch-schonlein purpura) presentation (5)

A
  1. symmetrical rashes usually located in the lower extremities
  2. palpable purpura
  3. migratory arthritis in the lower large joints
  4. nausea/ vomiting
  5. abdominal pain
43
Q

IgA nephropathy (berger’s disease) presentation (3)

A
  1. nephritic syndrome
  2. dark urine
  3. oedema
44
Q

what is buerger’s disease

A

a non-atherosclerotic vasculitis resulting in segmental occlusions of small and medium-sized arteries, commonly affecting the lower limbs of young men who smoke

45
Q

what is IgA nephroapthy (berger’s disease)

A

a disease in which IgA protein builds up in and damages the filtering part of the kidney (glomerulus)

46
Q

what other conditons is IgA nephropathy associated with

A
  1. henoch-schonlein purpura aka IgA vasculitis (abdo pain, joint pain, skin rash)
  2. liver disease
47
Q

what is the triad of nephritic syndrome

A
  1. hypertension
  2. haematuria
  3. renal impairment
48
Q

what are causes for nephritic syndrome (5)

A
  1. post-infectious glomerulonephritis
  2. systemic vasculitis
  3. goodpasture’s disease (Anti-GBM disease)
  4. cresentic nephritis
  5. lupus nephritis
49
Q

when does post-infectious glomerulonephritis usually present

A

2-3 weeks after group A strep infection

50
Q

post-infectious glomerulonephritis brief pathophsy

A

immune-complex to strep A results in damage to glomerulus

51
Q

why are C3 levels usually low in post-infectious glomerulonephritis

A

alternate complement pathway is activated -> C3 consumed in complement cascade (C4 will be normal as not consumed)

52
Q

what will the renal biopsy of post-infectious glomerulonephritis show (4)

A
  1. neutrophil infiltration
  2. mesangial and epithelial cell proliferation
  3. IgG and C3 deposition
  4. subepithelial deposits
53
Q

what is a red cell cast

A

clear cylinders containing red blood cells and may have an orange-red tinge seen in the urine -> indicates microscopic bleeding in the kidneys i.e. glomerulonephritis

54
Q

what condition are red cell casts seen in

A

nephritic syndrome

55
Q

3 examples of systemic vasculitises that cause glomerulonephropathy

A
  1. GPA (wergener’s granulomatosis)
  2. microscopic polyangiitis (MPA)
  3. eosinophilic granulomatosis with polyangiitis (churg-strauss)
56
Q

what are the other names for cANCA and pANCA

A

cANCA= PR3
pANCA = MPO

57
Q

what does ANCA stand for

A

Antineutrophilic cytoplasmic antibody

58
Q

what ANCA is seen in GPA

A

cANCA

59
Q

what ANCA is seen in MPA

A

pANCA

60
Q

what is seen on renal biopsy in systemic vasculitis

A

crescents, pauci-immune

61
Q

pauci etymology

A

latin for small/few

62
Q

systemic vasculitis mgx

A

immunosuppression +/- plasma exchange

63
Q

how does goodpasture’s disease present

A
  1. nephritic syndrome
  2. AKI
  3. pulmonary haemorrhage
64
Q

goodpasture’s disease biopsy

A

crescents with linear antibody deposition

65
Q

why must goodpasture’s disease be treated early and how

A

treat with immunosuppression +/- plasma exchange - organ damage happens very quickly so must be treated early

66
Q

crescentic nephritis presentation

A

nephritic illness with rapidly deteriorating renal function

67
Q

what conditions can preceed crescentic nephritis

A

many incl. goodpasture’s disease and systemic vasculitis

68
Q

what does the cresentic nephritis renal biopsy show

A

acute inflammatory process with crescent formation (cellular proliferation in bowman’s spacce)

69
Q

what is lupus nephritis

A

occurs when lupus autoantibodies affect parts of the kidneys that filter out waste

70
Q

what test is vital in lupus nephritis

A

renal biopsy

71
Q

lupus nephritis biopsy findings (6)

A
  1. endocapillary hypercellularity
  2. neutrophil infiltration/karyorrhexys
  3. fibrinoid necrosis
  4. hyaline deposits
  5. cellular/fibrocellular crescents
  6. interstitial inflammation
72
Q

key inveStigations to consider in glomerular disease pt

A
  1. urine
  2. bloods
  3. imagine
  4. renal biopsy
73
Q

what are “renal screen” blood test components

A
  1. autoantibodies (ANA, ANCA etc.)
  2. serum immunoglobulin and electrophoesis
  3. serum free light chains
  4. cryoglobulin levels
  5. blood cultures
  6. serology

3B week 1 - abnormal urine (5 decks)

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