Lecture 24 - The Kidneys in Systemic Disease Flashcards

1
Q

What systemic diseases may affect the kidneys?

A
DM
CV disease
Infection 
Inflammation of the BVs
HUS/TTP
Myeloma
Amyloidosis 
Drugs
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What CV diseases may affect the kidney?

A

Cardiac failure
Artheroembolism
HTN
Atherosclerosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What infections may affect the kidney?

A

Sepsis
Post-infectious GN
IE

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What inflammatory diseases of BVs may affect the kidneys?

A

SLE
Vasculitis
Scleroderma + other connective tissue diseases
Cryoglobulinaemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What drugs may damage the kidneys?

A
Aminoglycosides
NSAIDs
Radiocontrast
ACEi
Penicillamine, gold
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What is the commonest single cause of ESRD?

A

DM

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What is the natural history of diabetic nephropathy?

A
  1. Silent sub-clinical phase with hyperfiltration + increased GFR
  2. microalbuminaemia
  3. Clinical nephropathy
  4. Established renal failure
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Are type I or type II at more risk of diabetic nephropathy?

A

Equal risk

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What is the classification of CKD based on?

A

Kidney function - GFR

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What classification system is used to stage CKD?

A

NKF K/DOQI Classification system -

  1. Kidney damage/normal/high GFR (GFR >90)
  2. Kidney damage/mid deduction in GFR (GFR 60-89)
  3. Moderately impaired (GFR 30-59)
  4. Severely impaired (GFR 15-29)
  5. Advanced or on dialysis (GFR <15)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What does CKD have a relationship with?

A

CV disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What is a very common cause of renal failure in older patients?

A

Renal vascular disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What is vasculitis?

A

Inflammation in the wall of a blood vessel

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What is the vasculitis categorised by?

A

Size of vessel involved

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What are aortic/large artery vasculitides?

A

Takayasu arteritis

Giant cell arteritis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What are e.g.s of medium artery vasculitides?

A

Polyarteritis nodose

Kawasaki disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What are e.g.s of small vessel vasculitides?

A

Wegner’s granulomatosis
Microscopic polyarteritis
Churg-strauss syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What is Wegener’s granulomatosis (granulomatosis with polyangiitis)?

A

Autoimmune condition associated with a necrotizing granulomatous vasculitis, affecting the upper and lower RT and kidneys

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q
What are the features of Wegener's granulomatosis:
URT:
LRT:
Kidneys:
Joints:
Eyes:
Heart:
Systemic:?
A

URT: epistaxis, saddle deformity, sinusitis, deafness
LRT: cough, dyspnoea, haemoptysis, pulmonary haemorrhage
Kidneys: GN
Joints: arthalgia, myalgia
Eyes: scleritis
Heart: pericarditis
Systemic: fever, wt loss, vasculitic skin rash

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What do you see on CXR in someone with Wegener’s?

A

Large cavitating lesions

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What is often +ve in the blood work up of patients with Wegener’s?

A

cANCA (>90%), pANCA (25%)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

How is Wegener’s managed?

A

Steroids
Cyclophosphamide
Plasma exchange

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

What is the median survival with Wegener’s?

A

8-9 years

24
Q

What is microscopic polyangiitis?

A

Small vessel ANCA vasculitis

25
Q

What are the features of microscopic polyangiitis?

A
Renal impairment - raised Cr, haematuria, proteinuria
Fever
Lethargy, myalgia, wt loss
Rash - palpable purpura
Cough, dyspnoea, haemoptysis
Mononeuritis multiplex
26
Q

What ANCA antibodies are usually seen in microscopic polyangiitis?

A

pANCA (against MPO) - positive in 50-75%

cANCA (against PR3) - positive in 40%

27
Q

What investigation results may point towards a vasculitis?

A
Blood/protein in urine
Raised urea/creatinine
Raised alk phosp, CRP, low albumin
Anaemia, thrombocytosis, leucocytosis
Hyperglobinulinaemia
\+ve ANCA
28
Q

What does the c in cANCA stand for?

A

Cytoplasmic

29
Q

What does the p in pANCA stand for?

A

Perinuclear

30
Q

What are ANCA?

A

Anti-neutrophil cytoplasm antibodies are autoantibodies that target neutrophils

31
Q

What two proteins do ANCA specifically bind to?

A

Proteinase 3 (PR3) and myeloperoxidase (MPO_

32
Q

What does binding of ANCA to neutrophils in the blood lead to?

A

Release of toxic substances from neutrophils –> damage to small blood vessel walls + migration of neutrophils through BVs into surrounding tissues –> inflammation there
Release of signalling molecules that attract more neutrophils and perpetuate the response

33
Q

What two ways can ANCA blood tests be performed by?

A

Indirect immunofluorescence and ELISA

34
Q

What does IIF involve?

A

Staining neutrophils
cANCA leads to staining throughout the neutrophil
pANCA leads to staining around the nucleus

35
Q

Why is doing ELISA more beneficial than IIF?

A

It allows the level of PR3/MPO-ANCA to be measured

36
Q

What protein is pANCA usually against?

A

MPO

37
Q

What protein is cANCA usually against?

A

PR3

38
Q

What ANCA is positive in Wegener’s >90% of the time?

A

cANCA

39
Q

What ANCA is positive in microscopic polyarteritis usually?

A

pANCA

40
Q

When might ANCA give a false +ve?

A

If patient has IBD

41
Q

What is infective endocarditis?

A

Bacterial (or fungal) infection of the cardiac valves

42
Q

What are the typical causative organisms in IE?

A

Staph aureus
Viridans strep
Enterococci

43
Q

What renal disease can IE lead to and why?

A

GN +/- small vessel vasculitis due to immune complex formation

44
Q

What things may suggest renal involvement in IE?

A

Abnormal urea/cr
Haematuria/red cell casts
Reduced complement levels

45
Q

How is renal involvement in IE treated?

A

It sound recover when underlying infection treated

46
Q

What is multiple myeloma?

A

Monoclonal proliferation of plasma cells –> excess of Ig and light chains

47
Q

Who is MM most common in?

A

Elderly

48
Q

What are the clinical features of MM?

A
Markedly elevated ESR
Anaemia
Wt loss
Fractures
Infection 
Back pain/cord compression
49
Q

How is MM diagnosed?

A

Bone marrow aspirate >10% clonal plasma cells
Serum paraprotein +/0 immunoparesis
Urinary bence hones protein
Skeletal survey shows lytic lesions

50
Q

How does MM affect the kidney?

A

Abnormal Ig are filtered in the glomerulus and get stuck and block up the renal tubules –> progressive renal failure

51
Q

What are the features of renal failure in myeloma?

A
Cast nephropathy 
Light chain nephropathy
Amyloidosis
HyperCa
Hyperuricaemia
52
Q

What is amyloidosis?

A

Extracellular deposition of an insoluble fibrillar protein called amyloid

53
Q

What is amyloid derived from?

A

Multiple precursor proteins

54
Q

What are the non-fibrillary components of amyloid?

A

Amyloid P component (derived from the acute phase protein serum amyloid P)
Apolipoprotein E
Heparan sulphate proteoglycans

55
Q

What does the accumulation of amyloid fibrils lead to?

A

Tissue/organ dysfunction

56
Q

How do you diagnose amyloidosis?

A

Congo red staining - see apple-green birefringence
Serum amyloid precursor scan
Biopsy of rectal tissue