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Flashcards in Glomerulonephritis Deck (49):
1

Define glomerulonephritis

"Immune mediated disease of the kidneys affecting the glomeruli"
-Often with secondary interstitial damage"

2

Three main pathogenic mechanisms of GN

-Humoral - antibody mediated
-Cell mediated - (T cells)
-Inflammatory cells, mediators and complement

3

Damage to the selective membrane barrier causes what?

Haematuria &/or proteinuria

4

Damage to what cells causes proliferative lesions and red blood cells in urine

Endothelial
Mesangial

5

Damage to podocytes leads to what kind of lesion?

Non proliferative
Protein in urine

6

How does the mesangium respond to injury?

Proliferate
Released Ang2
Cause chemokine release and attract inflammatory cells

7

Damage to the podocytes causes what?

Atrophy and loss of size/charge of specific barrier

8

Damage to endothelial cells leads to what?

Vasculitis

9

When testing urine proteins, how do you effectively quantify proteinuria?

Creatinine ratio with 24 hour urine

10

What other signs of GN can be seen through urine microscopy?

(Dysmorphic) RBCs
Granular casts
Lipiduria

11

What measurement of proteinuria is definitive of nephrotic syndrome?

>3g/day

12

What reading would be considered asymptomatic proteinuria?

<1g/day

13

What non-renal condition might a patient present with suggestive of renal disease

Hypertension

14

Clinical symptoms/signs of Nephritic Syndrome

Acute renal failure
Oliguria
Oedema and fluid retention
Hypertension
Active urinary sediment
-(RBCs and Granular casts)
Indication of proliferative process affecting ENDOTHELIAL cells

15

Clinical Signs/symptoms of Nephrotic Syndrome

Proteinuria >3g/day
Hypoalbuminaemia <30mg/day
Oedema
Hypercholesterolaemia
Usually normal renal function
Indication of NON proliferative process, affecting PODOCYTES

16

Complications of Nephrotic Syndrome

Infections - due to loss of opsonising antibodies
Renal Vein thrombosis
Pulmonary emboli
Volume depletion
Vit d deficiency
Sub-clinical hypothyroidism

17

Use of what long term drug can cause volume depletion complications in nephrotic syndrome?

Over-use of diuretics

18

Main cause of GN

Primary - Idiopathic

19

Causes of secondary GN

Infection
Drugs
Malignancies
Systemic diseases

20

Examples of systemic diseases causing GN

ANCA associated systemic vasculitis, Good-pastures, HSP, lupus etc

21

Investigative histological tests required for classification of GN

Biopsy
LM
Immunofluorescence
EM

22

What does the term proliferative vs non proliferative refer to?

Presence vs absence of proliferation of mesangial cells

23

Define focal vs diffuse in classification

Focal <50% of glomerulus affected
Diffuse >50% of glomerulus affected

24

What does it mean is biopsy is crescentic?

Presence of crescents noted on histology -
Epithelial cell extra-capillary proliferation
e.g. RPGN in vasculitis

25

What are the principles of treatment in GN?

- Reduce degree of proteinuria
- Induce remission of nephrotic syndrome
- Preserve long-term renal function

26

Non-immunosuppresive treatment for GN

Anti-hypertensives
ACE inhibitors/ARBs
Diuretics
Statins
Consider: anticoagulants, aspirin and anti-platelets
?Omega 3/fish oil

27

Target BP in GN with/without proteinuria

With - <120/75
Without - <130/80

28

Immunosuppresive Treatment for GN

Corticosteroids
Azathioprine
Alkylating Agents
Cyclosporins
MMF
Plasmapharesis
Antibodies

29

General treatment of Nephrotic Patients

Fluid restriction
Salt restriction
Diuretics
ACE/ARB
IV Albumin if volume depleted
Immunosuppression with aim to induce sustained remission

30

Main types of (primary) idiopathic GN

Minimal change
FSGS
Membranous
Membranoproliferative
IgA nephropathy

31

What is minimal change disease?

Most common cause of nephrotic syndrome in children
Normal renal biopsy with foot process fusion on EM
Does NOT cause progressive renal failure
May be caused by IL-13
94% achieve remission on oral steroid alone

32

What is Focal Segmental Glomerulosclerosis
(FSGS)

Commonest cause of nephrotic syndrome in adults
Can be primary or secondary cause (HIV/heroin/obesity/)
Renal biopsy shows what name states on LM and complement deposition on IF
Remission in 60% with prolonged steroids
50% will progress to end stage renal failure in 10years

33

What is Membranous Nephropathy?

2nd commonest cause of nephrotic syndrome in adults
Can be primary or secondary
Renal biopsy shows sub-epithelial immune complex deposition in basement membrane
Treat with steroids etc
30% will progress to ESRF

34

Important secondary causes of membranous nephropathy

Infectious - Hep B and parasites
Connective tissue disease e.g. lupus
Malignancies (carcinoma/lymphoma)
Drugs (gold/penicillamine)

35

What is IgA Nephropathy

Most common GN in the world
Asymptomatic micro haematuria +/- non-nephrotic range proteinuria
Post-infection (GI/Resp) macro haematuria
Can cause AKI/CKD
Associated with Henoch-Schonlein Purpura
Renal Biopsy Shows mesangial cell proliferation nd expansion on LM with IgA deposits in mesangium on IF
25% progress to ESRF

36

What type of GN presents with arthritis/colitis/purpuric skin rash?

IgA nephropathy
These are classic of the Henoch-Schonlein Purpura (HSP)

37

What is Rapidly progressive glomerulonephritis
(RPGN)

A treatable cause of acute renal failure with rapid deterioration in renal function over a few days/weeks which may be part of a systemic disease
It is associated with glomerular crescents on biopsy and active urinary sediment (RBCs and granular casts)

38

ANCA-positive causes of RPGN

Systemic Vasculitis
Wegeners Granulomatosis
Microscopic Polyangitis

39

ANCA-negative causes of RPGN

Goodpastures disease (Anti-GBM)
HSP
SLE

40

Treatment of RPGN

Treatment should be prompt and consist of both supportive and immunosuppression care (and dialysis if needed)
Steroids e.g. pred oral/ IV methylpred
Cytotoxics e.g (Cyclophosphamide/ Mycophenolate)
Plasmapharesis

41

What are the primary causes of Nephrotic Syndrome

Membranous
Minimal Change
FSGS
Mesangiocapillary GN

42

Main Primary causes of Nephritic Syndrome

IgA Nephropathy
Mesangiocapillary GN

43

Secondary causes of Nephrotic Syndrome

Diabetes
SLE - in vascular nephritis
Amyloid
Hep B/C

44

Secondary causes of Nephritic Syndrome

Post-Strep infection
Vasculitis
SLE - in other forms of nephritis
Anti-GBM/ Goodpastures Disease
Cryoglobulinaemia

45

What is a typical patient presentation for IgA Nephropathy?

Young man with episodic macroscopic haematuria
Recovers rapidly between attacks

46

Typical Patient presentation for Henloch-Sconlein Purpura

Purpuric rash on extensors - usually legs, abdo pain, fleeting poly-arthritis

47

Focal Segmental Glomerulosclerosis is the 2nd most common cause of Nephrotic in adults? true/false

False
It is the most common cause

48

2nd Commonest cause of Nephrotic syndrome in adults?

Membranous Nephropathy

49

Most common cause of Glomerulosclerosis worldwide?

IgA nephropathy