Kidneys in systemic disease

Question 1

what is myeloma

Answer 1

cancer of plasma B cells which causes dysproteinaemias

Question 2

what does myeloma dysproteinaemia cause

Answer 2

overproduction of immunoglobulin proteins by expansion of B cell lineage and monoclonal production of paraprotein

Question 3

what is the classic presentation of myeloma

Answer 3

back pain, bone pain and AKI/ renal failure

Question 4

what are symptoms of myeloma (5)

Answer 4

bone pain, weakness, fatigue, weight loss, recurrent infections

Question 5

what are signs of myeloma (4)

Answer 5

anaemia, hypercalcaemia, renal failure, lytic bone lesions

Question 6

what renal manifestations of myeloma are there

Answer 6

build up of proteins, glomerular: AL amyloidosis, immunoglobulin deposits // tubular: light chain cast nephropathy

Question 7

how do you diagnose myeloma

Answer 7

bloods: FBC + electrophoresis - free light chains // urine: bence Jones proteins // CT: lytic bone legions // bone marrow and renal biopsy

Question 8

how do you manage myeloma

Answer 8

saline +/- biphosphonates (hypercalcaemia) // dexamethasone, cyclophosphamide (chem), stem cell transplant, dialysis

Question 9

what is amyloidosis

Answer 9

deposition of extracellular amyloid (insoluble proteins) in tissues and organs

Question 10

what are the 2 most common forms of amyloidosis

Answer 10

primary light chains (AL) // secondary, systemic, inflammatory (AA) - both commonly linked to myeloma

Question 11

what abnormal protein is produced in AL amyloidosis

Answer 11

abnormal immunoglobulin light chains from plasma cells

Question 12

where does AL amyloidosis commonly cause deposits (5)

Answer 12

heart, bowel, skin, nerves, kidneys

Question 13

what age does AL amyloidosis commonly present in and what is the life expectancy

Answer 13

55-60, <4 years if untreated

Question 14

how do you treat AL amyloidosis

Answer 14

immunosuppression: steroids, chemo, stem cell transplant

Question 15

what abnormal protein is produced in AA amyloidosis

Answer 15

acute phase protein (SAA)

Question 16

who commonly develops AA amyloidosis

Answer 16

patients with chronic inflam conditions or chronic infections

Question 17

where does AA amyloidosis commonly cause deposits (4)

Answer 17

liver, spleen, kidneys, adrenals

Question 18

what are symptoms of amyoidosis (where it affects)

Answer 18

nephrotic syndrome!!! proteinuria +/- renal function, cardiomyopathy, neuropathy: (numbness, tingling), hepatosplenomegaly, GI malabsorption: (nausea, diarrhoea)

Question 19

how do you diagnose amyloidosis

Answer 19

renal biopsy: congo reed staining (apple green bifrigence)

Question 20

what other investigations can be done for amyloidosis

Answer 20

urinalysis, blood tests (RFT, inflam), SAP scan - extent of disease

Question 21

what types of vasculitis commonly affect the kidneys

Answer 21

small vessel ANCA positive: GPA and MPA

Question 22

how do you diagnose vasculitis

Answer 22

urinalysis, renal biopsy, blood antibodies: anti PR3 cANCA for GPA /// anti MPO pANCA for MPA

Question 23

how do you manage vasculitis

Answer 23

immunosuppression: steroids, cyclophosphamide, plasma exchanged, dialysis

Question 24

what is the most common presentation in SLE affecting the kidneys

Answer 24

proteinuria

Question 25

what is the most common cause of renal failure

Answer 25

T2DM

Question 26

which arteriole is affected more in diabetes and what does this result in

Answer 26

efferent > afferent, results in initial increased GFR which causes hyperfiltration and microalbinuria

Question 27

how does T2DM cause renal failure

Answer 27

glycolysation causes leaky capillaries –> hyperfiltration in the early stages –> type IV collagen deposits

Question 28

how do you treat T2DM kindey failure

Answer 28

control BP with ACEi and ARBs, insulin control, statins, dialysis (bad prognosis)