Kidneys in Systemic Disease Flashcards

1
Q

What is myeloma? What happens?

A

Aggressive cancer of plasma cells > B cells that produce antibodies. The abnormal plasma cells accumulate in the bone marrow and impair function of normal blood cells. Many Ig molecules produced by myeloma cells are incompletely formed and unattached light chains can be released.

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

Describe the structure of an antibody?

A

Two heavy chains form two sides of Y
Two light chains sit along the top bits of the Y
The top half of heavy chains and light chains forms the variable Fab region
The bottom half of the heavy chains forms the constant Fc region

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

Describe how myeloma can damage the kidneys?

A

Due to low molecular weight light chains can pass through glomerular filtrate and cause damage to the epithelial cells as protein precipitates as casts.
The light chains can also pass through capillaries and cause damage to the kidneys and other organs that way.
Myeloma can also cause hypercalcaemia due to metastatic bone destruction which can further damage the kidneys.

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

Myeloma is a disease of young or old people?

A

Old people

Peak age= 70

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

Classic presentation of myeloma?

A

Back pain and AKI

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Punched out skeletal lesions?

A

Myeloma

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

Symptoms and signs of myeloma?

A

Renal failure, Bone pain, weakness, fatigue, weight loss, recurrent infections
Anaemia and hypercalcaemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Describe investigations for myeloma?

A

Test for anaemia and hypercalcaemia
Urine for Bence Jones protein
Bloods for serum protein electrophoresis and serum free light chains
Skeletal survey for punched out lesions
Renal biopsy- congo red staining for apple green birenfringence
Bone marrow biopsy

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

Treatment of myeloma?

A

Renal treatment is supportive

Haematology for cancer treatment

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

Bence Jones protein in urine?

A

Myeloma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What is the most common renal disease associated with myeloma?

A

Light chain nephropathy

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

Describe what amyloidosis is?

A

Disease where you get extracellular amyloid in tissues or organs due to abnormal folding of proteins, these abnormal proteins aggregate and become insoluble

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

What causes amyloidosis?

A

Many causes, over 30 different proteins identified and there are inherited and acquired forms

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

4 most common types of amyloidosis in order from most common?

A

Primary light chain
AA amyloidosis- due to systemic inflammatory disease e.g. RA or IBD
Dialysis
Hereditary and old age

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

Renal presentation of amyloidosis?

A

Usually nephrotic syndrome > proteinuria and impaired function

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

Cardiac presentation of amyloidosis?

Note not in everyone

A

Restrictive cardiomyopathy- heart can’t fill aswell, diastolic dysfunction get breathlessness, fatigue elevated JVP with diastolic collapse

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

Neurological presentation of amyloidosis?

Not in all amyloidosis

A

Neuropathy

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

GI presentation of amyloidosis?

Not in all amyloidosis

A

Hepato and splenomegaly

GI malabsorption

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Investigations for amyloidosis?

A

urinalysis +uPCR
Blood tests- U and Es, inflammatory markers, protein electrophoresis, SFLC
Renal biopsy for congo red staining (apple green birenfrigence of stained fibres under polarised light is indicative of amyloid fibrils)

20
Q

Apple green birenfrigence of congo red fibres?

A

Amyloid fibrils present

21
Q

Management of amyloidosis?

A

Not curative
AA treat underlying condition
AL give immunosuppression

22
Q

Why do small vessel vasculitis diseases commonly affect the kidneys skin and lungs?

A

There is a large concentration of small blood vessels here

23
Q

What type of vasculitis diseases usually affect the kidneys?

A

Small vessel vasculitis

24
Q

ANCA positive vasculitis can cause _________

It clinically presents as ____________

A

focal segmental proliferative glomerulonephritis with necrosis and crescent formation
Rapidly progressive glomerulonephritis

25
Q

How do ANCA positive vasculitis cause kidney disease?

A

ANCA causes inflammation of the vessel wall by stimulating neutrophil infiltration

26
Q

Kidney disease with saddle nose deformity?

A

GPA

27
Q

As well as kidney disease characteristics of GPA are?

A

Saddle nose deformity

Cavitating nodules in lungs, haemorrhage, SOB, haemoptysis and cough

28
Q

As well as kidney disease characteristics of EGPA are?

A

Late onset asthma and 2/3 have skin involvement

29
Q

Difference between MPA vs EGPA and GPA?

A

No granuloma and lots of systemic symptoms

30
Q

Who should vasculitic disease be suspected in?

A

Those with proteinuria, haematuria, raised inflammatory markers and positive immunology

31
Q

Management of kidney vasculitis disease?

A

Immunosuppression

32
Q

Who does HSP occur in?

A

Children aged 2-11 more than 75% have had a preceding URTI or GI infection 1-3 weeks before. Commonly an infection with group A strep.

33
Q

Signs and symptoms of HSP?

A

Purpuric rash over buttocks and lower limbs, colicky abdo pain, bloody diarrhoea, joint pain and swelling, renal involvement- haematuria, proteinuria, hypertension, fluid retention occasionally nephrotic syndrome- there may be no renal involvement or it may be asymptomatic

34
Q

Management of HSP?

A

Usually self limiting lasting around 8 weeks so just need supportive treatment. No proven treatment of benefit.
Must do urinalysis to screen for renal involvement though.

35
Q

Malar rash and renal symptoms?

A

SLE

36
Q

Investigations in SLE renal disease?

A

Raised inflammatory markers, ANA positive, Anti DsDNA positive, low complement, renal biopsy

37
Q

Up to____ of SLE patients have renal involvement at the time of presentation

A

50%

38
Q

Most frequently observed renal abnormality in SLE is?

A

proteinuria

39
Q

In diabetic nephropathy there is initially an ______ in GFR

A

Increase

40
Q

Is there haematuria in diabetic nephropathy?

A

No

41
Q

Why do you give ACEi to diabetics?

A

In diabetes there is a constricted efferent due to glycation and a more dilated afferent which is putting pressure on the glomerulus. ACEi will dilate the efferent so there is less pressure on the glomerulus.

42
Q

Diabetics have a _______ survival on dialysis compared to other renal patients

A

poorer

43
Q

Risk factors for renovascular disease are

A

the same as risk factors for general atherosclerosis e.g. smoking, fatty foods, obesity, high cholesterol, lack of exercise

44
Q

What may be ausculated on examination of someone with renovascular disease?

A

Abdominal bruits

45
Q

Treatment of renovascular disease?

A

Lifestyle changes

Angioplasty and or stenting

46
Q

Why is calcium increased in myeloma?

A

Bone lysis