Glomerulonephritidies Flashcards

1
Q

What are the general features of nephritic syndrome?

A

Haematuria
Hypertension

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2
Q

What are the general features of nephrotic syndrome?

A

Oedema
Proteinuria

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3
Q

What classically causes a nephritic picture?

A

Rapidly progressive glomerulonephritis
IgA nephropathy

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4
Q

What causes a mixed picture?

A

Diffuse proliferative glomerulonephritis
Membranoproliferative glomerulonephritis
Post-streptococcal glomerulonephritis

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5
Q

What classically causes a neurotic picture?

A

Minimal change disease
Membranous glomerulonephritis
Focal segmental glomerulosclerosis
Amyloid
Diabetic nephropathy

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6
Q

What is rapidly progressive glomerulonephritis?

A

Rapid loss of renal function associated with formation of epithelial crescents in majority of glomeruli

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7
Q

What are causes ion rapidly progressive glomerulonephritis?

A

Goodpasture’s syndrome
Granulomatosis with polyangitis
SLE
Microscopic polyarteritis

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8
Q

What are the features of rapidly progressive GN?

A

Nephritic syndrome - haematuria with red cell casts, hypertension, proteinuria, oliguria
Features specific to underlying cause - eg haemoptysis with Goodpasture’s, vasculitis rash if gramulomatosis with polyangitis
Often presents as AKI

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9
Q

What is IgA nephropathy?

A

Mesangial deposition of IgA immune complexes

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10
Q

What is the classic presentation of IgA nephropathy?

A

Classically macroscopic haematuria in young man 1-2 days after URTI

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11
Q

What is IgA nephropathy associated with?

A

Henoch-Schonlein purpura

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12
Q

What is seen on histology with IgA nephropathy?

A

mesangial hypercellularity, positive immunoifluorescence for IgA and C3

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13
Q

What is the management of IgA nephropathy?

A

1st line: ACE inhibitors
2nd line: corticosteroids

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14
Q

What are indications for management of IgA nephropathy?

A

Persistent proteinuria (>500-1000) with normal or slightly reduced GFR –> ACEi
Active disease or failure to respond to ACEi –> steroids

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15
Q

What are markers of poor prognosis in IgA nephropathy?

A

Male, heavy proteinuria >2g, hypertension, smoking, hyperlipidaemia, ACE genotype DD

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16
Q

What are the common causes of diffuse proliferative glomerulonephritis?

A

Post streptococcal GN
Most common renal disease in SLE

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17
Q

What causes post strep glomerulonephritis?

A

Immune complex (IgG, IgM, C3) deposition in glomeruli

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18
Q

What normally provokes post strep GN?

A

Group A strep, usually strep pyogenes

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19
Q

What is the classic presentation of post strep glomerulonephritis?

A

Young children 7-14 days post URTI. Proteinuria and low complement

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20
Q

What are the features of post strep glomerulonephritis?

A

Headache, malaise
Visible haematuria
Proteinuria
HTN
Oliguria

21
Q

What is seen on bloods in post strep GN?

A

Low C3
Raised anti-streptolysin O titre

22
Q

What is seen on biopsy with post strep GN?

A

Acute diffuse proliferative GN
Endothelial proliferation with neutrophils

23
Q

What is seen on electron microscopy with post strep GN?

A

Subepithelial humps caused by lumpy immune complex deposition

24
Q

What is seen on immunofluorescence with post strep GN?

A

Granular or starry sky appearance

25
Q

What are the types of membranoproliferazive glomerulonephritis and causes?

A

type 1 - cryoglobulinaemia, hep C
type 2 - “dense deposit disease” - partial lipodystrophy, factor H deficiency
type 3 - hep B and C

26
Q

What is the most common type of membranoproliferazive GN?

A

Type 1 - cryoglobulinaemia, hep C

27
Q

How does membranoproliferazive glomerulonephritis present?

A

Can be nephrotic syndrome or haematuria

28
Q

What is seen on renal biopsy with type 1 membranoproliferazive GN?

A

Tram track appearance - subendothelial and mesangium immune deposits of electron dense material

29
Q

What is the pathophysiology of type 2 membranoproliferative GN?

A

Persistent activation of alternative complement pathway leads to intramembranous immune complex deposits with dense deposits

30
Q

What is seen on bloods in type 2 membranoproliferative GN?

A

Low circulating C3
C3b nephritic factor - antibody to alternative pathway C3 convertase

31
Q

How is membranoproliferative GN managed and what is prognosis?

A

Steroids may be helpful
Poor prognosis

32
Q

What are the causes of m animal change disease?

A

Idiopathic in 80-90%
NSAIDs, rifampicin
Hodgkin’s, thymoma
Infectious mononucleosis

33
Q

What is the pathophysiology of minimal change disease?

A

T cell and cytokine mediated damage to glomerular basement membrane –> polyanion loss –> reduction in electrostatic charge –> increased glomerular permeability to serum albumin

34
Q

What are the features of minimal change disease?

A

Almost always presents as nephrotic syndrome - hypertension very rare
Selective proteinuria - only intermediate sized protein eg albumin and transferrin leak through glomerulus

35
Q

What is seen on renal biopsy with minimal change disease?

A

Light microscopy: Normal glomeruli
Electron microscopy: fusion of podocytes and effacement of foot processes

36
Q

Who is minimal change disease normally seen in and how common is it?

A

Accounts for 75% of nephrotic syndrome in children and 25% in adults

37
Q

What is the management of minimal change disease?

A
  1. Oral corticosteroids - 80% steroidi responsive
  2. Cyclophosphamide if resistant
38
Q

What is the prognosis of minimal chan ge disease?

A

Overall good
1/3 single episode, 1/3 infrequent relapse, 1/3 frequent relapses which stop before adulthood

39
Q

How prevalent is membranous glomerulonephritis?

A

Most common type of GN in adults and 3rd most common cause of ESRF

40
Q

What are the causes of membranous glomerulonephritis?

A

Idiopathic - anti-phospholipase A2 antibodies
Infection - hep B, malaria, syphilis
Malignancy - prostate, lung, lymphoma, leukaemia
Drugs - NSAIDs, penicillamine
Autoimmune - SLE, rheumatoid, thyroid

41
Q

What is the management of membranous glomerulonephritis?

A

All: ACEi or ARB
Severe/progressive: corticosteroid + cyclophosphamide
Consider anticoagulation

42
Q

What is seen on biopsy with membranous glomerulonephritis?

A

“Spike and dome”
Basement membrane thickened with sub epithelial electron dense deposits

43
Q

What is the prognosis of membranous glomerulonephritis?

A

Rule of thirds
- 1/3: spontaneous remission
- 1/3 remain proteinuria
- 1/3 develop ESRF

44
Q

Who is focal segmental glomerulosclerosis normally seen in?

A

Young adults

45
Q

What are the causes of focal segmental glomerulosclerosis?

A

Idiopathic
Secondary to other renal pathology - eg IgA neohropathy
HIV, heroin
Alpert’s syndrome
Sickle cell

46
Q

What is seen on renal biopsy with focal segmental glomerulosclerosis?

A

Focal and segmental sclerosis and hyalinosis
Effacement of foot processes

47
Q

What is the management of focal segmental glomerulosclerosis?

A

Steroids +/- immunosuppression

48
Q

What is the prognosis of focal segmental glomerulosclerosis?

A

<10% chance spontaneous remission if untreated
High relapse rate post transplant