Systemic Diseases

Question 1

what is the most common systemic disease with renal involvement?

Answer 1

diabetes

Question 2

what class of diseases is myeloma part of?

Answer 2

dysproteinaemias

Question 3

what is the pathophysiology of myeloma?

Answer 3

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

these then damage the bone marrow

Question 4

what is myeloma?

Answer 4

a cancer of plasma cells

Question 5

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

Answer 5

impair production of normal blood cells

results in anaemia

Question 6

what is a paraprotein?

Answer 6

abnormal antibodies

Question 7

what can cause renal dysfunction in myeloma?

Answer 7

paraproteins

Question 8

what is typical patient with myeloma?

Answer 8

60 year old male

Question 9

what are the symptoms of myeloma?

Answer 9

bone pain 
weakness 
fatigue 
weight loss 
recurrent infections

Question 10

what are the signs of myeloma?

Answer 10

anaemia
hypercalcaemia
renal failure
lytic bone lesions

Question 11

what is the classic presentation of myeloma?

Answer 11

back pain and renal failure

Question 12

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

Answer 12

a skeletal survey

looking for areas of bone damage

Question 13

how is myeloma diagnosed?

Answer 13

suspicion 
bloods 
urine 
bone marrow biopsy 
skeletal survey

Question 14

what blood tests are done for myeloma?

Answer 14

serum protein electrophoresis

serum free light chain measurement

Question 15

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

Answer 15

clonal immunoglobulines

Question 16

what are you looking for in the urine for myeloma?

Answer 16

proteinuria - bence jones protein

Question 17

what is the general management for renal failure in myeloma?

Answer 17

stop nephrotoxic drugs
manage hypercalcaemia
dialysis

Question 18

what is the specific management for myeloma?

Answer 18

chemotherapy

stem cell transplant

Question 19

what is amyloid?

Answer 19

insoluble protein fibrils

Question 20

what is amyloidosis?

Answer 20

the deposition of extracellular amyloid in tissues

Question 21

what causes amyloidosis?

Answer 21

abnormal folding of proteins that aggregate and become insoluble

Question 22

what are the four most common forms of amyloidosis?

Answer 22

AL
AA
AB2M
ATTR

Question 23

what is AL amyloidosis also called?

Answer 23

primary or light chain amyloidosis

Question 24

what is AA amyloidosis also called?

Answer 24

secondary/systemic/inflammatory

Question 25

what is AB2M amyloidosis also called?

Answer 25

dialysis amyloidosis

Question 26

what is ATTR amyloidosis also called?

Answer 26

hereditary and old age amyloidosis

Question 27

what are the two types of amyloidosis that are seen?

Answer 27

AA | AL

Question 28

what causes AL amyloidosis?

Answer 28

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

Question 29

what organs are commonly affected by AL amyloidosis?

Answer 29

``` heart bowel skin nerves kidneys ```

Question 30

what is the average age of diagnosis of AL amyloidosis?

Answer 30

55-60 years

Question 31

what is AA amyloidosis associated with?

Answer 31

systemic inflammation

Question 32

what causes AA amyloidosis?

Answer 32

production of the acute phase protein serum amyloid A protein

Question 33

what organs are commonly affected by AA amyloidosis?

Answer 33

liver spleen kidneys adrenals

Question 34

how does amyloidosis affecting the kidneys present?

Answer 34

proteinuria | impaired renal function

Question 35

what investigations are done for renal amyloidosis?

Answer 35

urinalysis renal function bloods renal biopsy

Question 36

what staining is used for a renal biopsy in amyloidosis?

Answer 36

congo red staining goes green under polarised light

Question 37

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

Answer 37

SAP scan

Question 38

what is vasculitis?

Answer 38

inflammation of the blood vessels

Question 39

what are the three types of vasculitis?

Answer 39

large medium small vessel

Question 40

what type of vasculitis commonly affects the kidneys?

Answer 40

small vessel

Question 41

what investigations are done for vasculitis affecting the kidneys?

Answer 41

urinalysis bloods immunology renal biopsy

Question 42

what is seen in urinalysis of vasculitis?

Answer 42

haematuria | proteinuria

Question 43

what antibodies are seen in vasculitis?

Answer 43

ANCA

Question 44

what are the two types of ANCA?

Answer 44

p ANCA | c ANCA

Question 45

what is another name for p-ANCA?

Answer 45

anti MPO

Question 46

what is another name for c-ANCA?

Answer 46

anti PR3

Question 47

how is vasculitis managed?

Answer 47

immunosuppression | plasma exchange

Question 48

what is the most frequently observed abnormality in lupus nephritis?

Answer 48

proteinuria