kidney systemic dis

Question 1

pathophys of myeloma

Answer 1

b cell prolif
monoclonal production of paraportein (serum protein electropheresis)
(bence jones protein urine)

Question 2

classic presentation

Answer 2

back pain n renal failure

Question 3

myeloma dx

Answer 3

bloods: serum protein electropheresis (clonal Ig) , serum free light chains (kappa/lambda)

urine: bence jones protein

bone marrow biopsy

skeletal survey

renal biopsy

Question 4

management myeloma

Answer 4

general (stop nephrotoxics, manage hypercalcaemia)

disease specific: chemo, stem cell transplant

supportive: dialysis

Question 5

myeloma in a nutshell

Answer 5

cancer of b cells (plasma cells)
excess clonal production of immunoglobulins (proteins)
renal involvement is multifactorial

classic presentation: AKI back pain

1st invest: protein electropheresis + bjp

refer to haem

renal follow up supportive

Question 6

common forms amyloidosis

Answer 6

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

Question 7

primary light chain amyloidosis

Answer 7

commonly affecrs heart bowel skin nerves kidneys

Question 8

secondary inflammatory amyloidosis

Answer 8

develops in approx 5% patients chronic inflammatory conditions or chronic infections (RA, ibd, psoriasis
TB, osteomyelitis, bronchiectasis)

commonly affects liver spleen kidneys adrenals

Question 9

presentation

Answer 9

dependent on organ tissue involved

renal: proteinuria, impaired renal function

cardiac: cardiomyopathy
nerves: peripheral or autonomic neuropathy
hepato/splenomehaly
GI malabsorption

Question 10

renal invest

Answer 10

the usuals
+ SAP scan (scintigraphy w/ radiolabelled serum amyloid shows extent of disease

Question 11

management

Answer 11

not curative so need to reduce further deposition and preserve organ function

AA - treat underlying condition

AL - immunosuppression (steroids, chemo, stem cell transplant)

Question 12

amyloidosis summary

Answer 12

key diagnosis tool is biopsy
congo red stain - apple green bifringence

Question 13

anca phenotypes

Answer 13

GPA - 95% ANCA, mainly 65% antiPR3
microscopic polyangitis - 90% ANCA mainly anti MPO 55%

Question 14

vascul mx

Answer 14

immunosuppression (steroids, cyclophosphamide/rituximab)

plasma exchange

supportive - dialysis ventilation

Question 15

cause sle

Answer 15

unknown aetiology

Question 16

lupus nephritis renal involvement

Answer 16

up to 50% renal presentation and up to 60% through disease course

most frequently observed abnormality proteinuria