kidneys in systemic disease

Question 1

what is myeloma?

Answer 1

a cancer of plasma cells ( a cell that is usually responsible for producing antibodies)
results in abnormal antibody production

Question 2

who is usually affected by myeloma?

Answer 2

men about 60 years old

Question 3

which protein is found in the urine in myeloma?

Answer 3

bence jones protein

Question 4

what are the symptoms of myeloma?

Answer 4

bone pain
weakness
fatigue
weight loss
recurrent infections

Question 5

what are the signs of myeloma?

Answer 5

anaemia
hypercalcaemia
renal failure
lytic bone lesions

Question 6

what is the classic presentation of myeloma?

Answer 6

middle aged
back/bone pain
renal failure

Question 7

what are the glomerular renal manifestations of myeloma?

Answer 7

AL amyloidosis
monoclonal immunoglobulin deposition (light/heavy chains)

Question 8

what are the tubular renal manifestations of myeloma?

Answer 8

light chain cast nephropathy

Question 9

how is myeloma diagnosed?

Answer 9

bloods
-serum protein electrophoresis (clonal Ig)
- serum free light chains (kappa/lambda)
urine
-bence jones protein

confirmed with a bone marrow biopsy

Question 10

how is myeloma managed?

Answer 10

general measures (stop nephrotoxics and manage hypercalcaemia)
chemo and stem cell transplant
dialysis?

Question 11

what is amyloidosis?

Answer 11

the deposition of extracellular amyloid (insoluble protein fibrils) in tissues or organs

Question 12

what are the 4 “common” forms of amyloidosis?

Answer 12

primary/light chain (AL)
secondary/systemic/inflammatory (AA)
dialysis (AB2M)
hereditary and old age (ATTR)

Question 13

how does primary/light chain amyloidosis occur?

Answer 13

through the production of abnormal immunoglobulin light chains from plasma cells
light chains enter the blood stream and cause amyloid deposits

Question 14

what does primary amyloidosis commonly affect?

Answer 14

heart
bowel
skin
nerves
kidneys

Question 15

what age group is usually affected by primary amyloidosis?

Answer 15

55-60 year olds
life expectancy after diagnosis 6mths-4years (untreated)

Question 16

what causes secondary amyloidosis?

Answer 16

production of acute phase protein SAA
develops in approx 5% of patients with chronic inflammatory conditions/infections

Question 17

what is the presentation of amyloidosis?

Answer 17

depends on organs involved
renal
- proteinuria +/- impaired renal function
cardiac
-cardiomyopathy
nerves
-peripheral or autonomic neuropathy
hepatomegaly/splenomegaly

Question 18

how is amyloidosis investigated?

Answer 18

urinalysis
bloods
renal biopsy
SAP scan

Question 19

what is the management of amyloidosis?

Answer 19

non curative
aim to reduce more deposition and preserve organ function
AA-treat underlying condition
AL-immunosuppression (steroids, chemo, stem cell transplant)

Question 20

what is small vessel vasculitis?

Answer 20

a necrotising polyangiitis
usually presents in 5th, 6th, 7th decade
presentation is very vague

Question 21

how is vasculitis diagnosed?

Answer 21

urinalysis
- blood and protein
immunology
-anti MPO (pANCA)
- anti PR3 (cANCA)

Question 22

what percentage of EGPA is pANCA associated?

Answer 22

40%

Question 23

what is GPA associated with?

Answer 23

lung involvement
saddle nose

Question 24

what is EGPA associated with?

Answer 24

asthma and eosinophilia
2/3 have skin involvement

Question 25

how is ANCA vasculitis treated?

Answer 25

immunosuppression
-steroids
-cyclophosphamide/rituximab
plasma exchange
supportive

Question 26

how is SLE disgnosed?

Answer 26

young lady with vague symptoms
bloods
- raised inflammatory markers
-+ve ANA, anti dsDNA
- low levels of complement (C3 and C4)
urinalysis
- proteinuria+/- microscopic haematuria (very common in renal involvement)