Systemic Diseases Flashcards

1
Q

what is the most common systemic disease with renal involvement?

A

diabetes

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2
Q

what class of diseases is myeloma part of?

A

dysproteinaemias

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3
Q

what is the pathophysiology of myeloma?

A

overproduction of the plasma cells produced by B cells that then produce antibodies

these then damage the bone marrow

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4
Q

what is myeloma?

A

a cancer of plasma cells

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5
Q

what effect do the collections of abnormal plasma cells have in myeloma?

A

impair production of normal blood cells

results in anaemia

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6
Q

what is a paraprotein?

A

abnormal antibodies

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7
Q

what can cause renal dysfunction in myeloma?

A

paraproteins

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8
Q

what is typical patient with myeloma?

A

60 year old male

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9
Q

what are the symptoms of myeloma?

A
bone pain 
weakness 
fatigue 
weight loss 
recurrent infections
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10
Q

what are the signs of myeloma?

A

anaemia
hypercalcaemia
renal failure
lytic bone lesions

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11
Q

what is the classic presentation of myeloma?

A

back pain and renal failure

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12
Q

what investigation is done for all myeloma patients and what is this looking for?

A

a skeletal survey

looking for areas of bone damage

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13
Q

how is myeloma diagnosed?

A
suspicion 
bloods 
urine 
bone marrow biopsy 
skeletal survey
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14
Q

what blood tests are done for myeloma?

A

serum protein electrophoresis

serum free light chain measurement

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15
Q

what are you looking for in serum protein electrophoresis for myeloma?

A

clonal immunoglobulines

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16
Q

what are you looking for in the urine for myeloma?

A

proteinuria - bence jones protein

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17
Q

what is the general management for renal failure in myeloma?

A

stop nephrotoxic drugs
manage hypercalcaemia
dialysis

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18
Q

what is the specific management for myeloma?

A

chemotherapy

stem cell transplant

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19
Q

what is amyloid?

A

insoluble protein fibrils

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20
Q

what is amyloidosis?

A

the deposition of extracellular amyloid in tissues

21
Q

what causes amyloidosis?

A

abnormal folding of proteins that aggregate and become insoluble

22
Q

what are the four most common forms of amyloidosis?

A

AL
AA
AB2M
ATTR

23
Q

what is AL amyloidosis also called?

A

primary or light chain amyloidosis

24
Q

what is AA amyloidosis also called?

A

secondary/systemic/inflammatory

25
Q

what is AB2M amyloidosis also called?

A

dialysis amyloidosis

26
Q

what is ATTR amyloidosis also called?

A

hereditary and old age amyloidosis

27
Q

what are the two types of amyloidosis that are seen?

A

AA

AL

28
Q

what causes AL amyloidosis?

A

production of abnormal IG light chains that enter the blood and cause deposits

29
Q

what organs are commonly affected by AL amyloidosis?

A
heart 
bowel 
skin 
nerves 
kidneys
30
Q

what is the average age of diagnosis of AL amyloidosis?

A

55-60 years

31
Q

what is AA amyloidosis associated with?

A

systemic inflammation

32
Q

what causes AA amyloidosis?

A

production of the acute phase protein serum amyloid A protein

33
Q

what organs are commonly affected by AA amyloidosis?

A

liver
spleen
kidneys
adrenals

34
Q

how does amyloidosis affecting the kidneys present?

A

proteinuria

impaired renal function

35
Q

what investigations are done for renal amyloidosis?

A

urinalysis
renal function bloods
renal biopsy

36
Q

what staining is used for a renal biopsy in amyloidosis?

A

congo red staining

goes green under polarised light

37
Q

what scan can be used to show the extent of amyloidosis?

A

SAP scan

38
Q

what is vasculitis?

A

inflammation of the blood vessels

39
Q

what are the three types of vasculitis?

A

large
medium
small vessel

40
Q

what type of vasculitis commonly affects the kidneys?

A

small vessel

41
Q

what investigations are done for vasculitis affecting the kidneys?

A

urinalysis
bloods
immunology
renal biopsy

42
Q

what is seen in urinalysis of vasculitis?

A

haematuria

proteinuria

43
Q

what antibodies are seen in vasculitis?

A

ANCA

44
Q

what are the two types of ANCA?

A

p ANCA

c ANCA

45
Q

what is another name for p-ANCA?

A

anti MPO

46
Q

what is another name for c-ANCA?

A

anti PR3

47
Q

how is vasculitis managed?

A

immunosuppression

plasma exchange

48
Q

what is the most frequently observed abnormality in lupus nephritis?

A

proteinuria