The Kidneys in Systemic Disease Flashcards

1
Q

What organ receives more blood flow per unit volume than any other organ in the body?

A

The kidney

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2
Q

What may the glomeruli trap?

A

Proteins

Immune complexes

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3
Q

What systemic diseases affect the kidneys?

A
DM
Cardiac failure
Atheroembolisms
HTN
Atherosclerosis
Sepsis
Post infectious glomerulonephritis
Infective endocarditis
SLE
Vasculitis
Scleroderma / other connective tissue diseases
Cryoglobulinaemia
HUS/TTP
Myeloma
Amyloidosis
Drugs
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4
Q

What drugs affect the kidneys?

A
Aminoglycosides
NSAIDs
ACE inihibtors
Penicillamine
Gold
Radiocontrast
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5
Q

Stages of diabetic nephropathy

A
  1. Silent subclinical phase
    - hyperfiltration
    - increased GFR
  2. Microalbuminamia (20-200ug/d)
  3. Clinical nephropathy (proteinuria >0.5g/d)
  4. Established renal failure
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6
Q

What is the triad of nephropathy?

A

Proteinuria
Hypoalbuminaemia
Oedema

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7
Q

What are the risks of a T1DM patient developing nephropathy at 10 and 25 years?

A

10 - 4%

25 - 25%

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8
Q

What are the risks of a patient with T2DM developing nephropathy within 5 and 20 years?

A

5 - 10%

20 - 30%

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9
Q

What % of those with diabetic nephropathy will progress to ESRF?

A

30%

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10
Q

What does ESRF stand for?

A

End stage renal failure

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11
Q

What is the commonest cause of ESRF?

A

Diabetic nephropathy

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12
Q

What is increasing proteinuria usually assosiated with?

A

Declining GFR

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13
Q

What is the classification of CKD based on?

A

Kidney function

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14
Q

What does CKD stand for?

A

Chronic kidney disease

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15
Q

What does GFR stand for?

A

Glomerular filtration rate

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16
Q

How many stages does CKD have?

A

5

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17
Q

What are the 5 stages of CKD?

A

Stage 1 = Kidney damage -normal or high GFR - GFR > 90
Stage 2 = kidney damage - mild reduction in GFR - GFR = 60-89
Stage 3 = moderately impaired, GFR = 30-59
Stage 4 - severely impaired. GFR = 15-29
Stage 5 - advanced or on dialysis. GFR = < 15

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18
Q

What classification system relates CKD to CVS disease?

A

NKF K/DOQI classification system

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19
Q

What is the commonest cause of renal fialure in older patients?

A

Reno-vascular disease / atheroembolic disease

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20
Q

Features of atheroembolic disease affecting the kidneys

A

Eosinophilia

Peripheral skin lesion

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21
Q

Treatment of atheroembolic disease affecting the kidneys

A

Warfarin

Vascular procedures

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22
Q

Definition of vasculitis

A

Inflammatory reaction in the wall of any blood vessel

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23
Q

What is vasculitis defined by?

A

The size of the vessel involved

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24
Q

What vasculitis conditions affect aorta / large arteries?

A

Takayasu arteritis

Giant cell arteritis

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25
Q

What vasculitis conditions affect the medium sized arteries?

A

Polyarteritis nodosa

Kawasaki disease

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26
Q

What vasculitis conditions affect the small vessel arteries?

A

Wegeners granulomatosis
Microscopic polyarteritis
Churg-strauss syndrome

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27
Q

What is wegeners granulomatosis?

A

Granulomatous inflammation in the respiratory tract

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28
Q

How does wegeners granulomatosis affect the kidneys?

A

Focal necrotising GN with crescents

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29
Q

Who does wegners more commonly affect?

A

Males

40-60 y/o

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30
Q

Presentation of wegeners

A
Epistaxis
Nasal deformity
Sinusitis
Deafness
Cough 
Dyspnoea
Haemopytsis
Pulmonary haemorrhage 
GN
Arthralgia
Myalgia
Scleritis
Pericarditis
Fever
Weight loss
Vasculitic skin rash
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31
Q

What can microscopic polyarteritis present with?

A

Systemic disease
Renal involvement
Pulmonary involvement

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32
Q

Investigations for vasculitis

A
Urine - blood/protein 
Renal function - raised creatinine/urea
Biochemistry - raised ALP, CRP, low albumin 
Anaemia
Thrombocytosis
Leucocytosis
Hyperglobulinaemia
Positive ANCA 
Renal biopsy
33
Q

What immunological molecule is seen in wegeners?

A

> 90% C-ANCA

34
Q

What immunological molecule is usually seen in microscopic polyarteritis?

A

P-ANCA

35
Q

Can ANCA be a false positive? Give an example

A

Yes

IBD

36
Q

What is infective endocarditis?

A

Endocarditis as a result of bacterial or fungal infection on the cardiac valves

37
Q

Causative organisms of infective endocarditis

A

Staph aureus
Viridans streptococci
Enterococci

38
Q

What can infective endocarditis lead to in the kidneys?

A

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

39
Q

What does GN stand for?

A

Glomerulonephritis

40
Q

What is myeloma?

A

A monoclonal proliferation of plasma cells producing an excess of immunoglobulins and light chains

41
Q

Who is myeloma common in?

A

Elderly

42
Q

Presentation of myeloma

A
Marked elevation of ESR
Anaemia
Weight loss
Fractures
Infections
Back pain / cord compression
43
Q

Diagnosis of myeloma

A

Bone marrow aspirate >10% clonal plasma cells
Serum paraprotein +/- immunoparesis
Urinary Bence-Jones protein
Skeletal survery for lytic lesions

44
Q

What does myeloma cause in the kidneys?

A
Cast nephropathy - 'myeloma kidney'
Light chain nephropathy 
Amyloidosis
Hypercalcaemia
Hyperuricaemia
45
Q

What does light chain disease lead to in the kidneys?

A

TBM IgM deposition

46
Q

What is amyloidosis?

A

Deposition of abnormal fibrillary proteins that persist

47
Q

What presentation would indicate there is renal involvement in the systemic disease?

A
Fever
Malaise
Weight loss
Arthralgia
Myalgia
Vasculitic skin rash 
Gritty eyes
Breathlessness
Haemoptysis 
Epistaxis
Haematuria
Oedema
Hands
- splinter haemorrhages
- purpura
- raynauds
Face
- scleritis
- uveitis
- nasal cartilage deformity
- retinal vasculitis
- HTN retinopathy 
- scleroderma
- HTN
- murmur
Haemoptysis
Crepitations
Joint Swelling / tenderness
Stroke
Encephalopathy
48
Q

Investigations if suspect renal involvement in systemic disease

A
Urine dipstick 
Bloods
- urea creatinine
- CRP
- anaemia
- thrombocytosis 
- raised ALP
- ANCA
- Complement levels
- blood cultures
- CXR
- USS abdo 
- CT thorax
- ECHO
Biopsy of
- kidney 
- nasal mucosa
- lung
- skin
49
Q

Who is HUS usually seen in?

A

Children

50
Q

Triad of HUS

A

AKI
Microangiopathic haemolytic anaemia
Thrombocytopenia

51
Q

Causes of HUS (secondary)

A

E coli 0157
Pneumococcal infection
HIV
Rare; SLE, cancer, drugs

52
Q

Who is Ecoli the most common cause of HUS in?

A

Children

53
Q

What is primary HUS due to?

A

Complement dysregulation

54
Q

Investigations of HUS

A
FBC
- anaemia
- thrombocytopenia
- fragmented blood film 
U + Es
- AKI
Stool culture
55
Q

Management of HUS

A

Supportive

56
Q

What does ADPKD stand for?

A

Autosomal dominant polycystic kidney disease

57
Q

Presentation of ADPKD

A
HTN
Recurrent UTIs
Abdo pain 
Renal stones
Haematuria
CKD
Extra renal manifestations
58
Q

What are the extra renal manifestations of ADPKD

A

Liver cysts (70%) which manifests as hepatomegaly
Berry aneurysms (8%)
MVP, Aortic root dilatation, aortic dissection, mitral/tricuspid incompetence
Diverticulosis
Ovarian cysts

59
Q

If a berry aneurysm ruptures, what may this cause?

A

SAH

60
Q

What is the screening test for adult polycystic kidney disease?

A

USS

61
Q

Osmolalities in Diabetes insipidus

A

High plasma osmolality

Low urine osmolality

62
Q

Treatment of ascites

A

Spironolactone

63
Q

What can the accumulation of amyloid fibrils lead to?

A

Tissue/organ dysfunction

64
Q

Diagnosis of amyloidosis

A

Congo red staining; apple-green birefringence
Serum amyloid precursor (SAP) scan
Biopsy of rectal tissue

65
Q

Presentation of amyloidosis

A
SOB
Weakness
Hepatomegaly
Proteinuria
Worsening renal function
66
Q

What age does amyloidosis typically present in?

A

50 - 65 y/o

67
Q

What does HSP stand for?

A

Henoch-Schonlein Purpura

68
Q

What is HSP?

A

IgA mediated small vessel vasculitis

69
Q

Who is HSP usually seen in?

A

Children following an infection

70
Q

What does HSP have a degree of overlap with?

A

IgA nephropathy (Bergers disease)

71
Q

Presentation of HSP

A

Palpable purpuric rash (with localised oedema) over buttocks and extensor surfaces of arms and legs
Abdominal pain
Polyarthritis
Features of IgA nephropathy may occur e.g.
- haematuria
- renal failure

72
Q

Treatment of HSP

A

Supportive

Analgesia for arthralgia

73
Q

Prognosis of HSP

A

Excellent
Self limiting
Especially in children with no renal involvement

74
Q

How many patients with HSP have a relapse?

A

1/3rd

75
Q

What screening is done for diabetic nephropathy?

A

Albumin:Creatinine Ratio (ACR) - early morning specimen

76
Q

What ACR is indicative of microalbuminuria?

A

> 2.5

77
Q

Size of diabetic nephropathy kidneys on USS

A

Large / normal sized

78
Q

Size of kidneys of most patients with CKD

A

Small kidneys

79
Q

What is the earliest clinical detection manifestation of diabetic nephropathy?

A

Microalbuminuria