systemic disease and the kidneys

Question 1

why can myeloma cause renal dysfunction

Answer 1

abnormal plasma cells produce excessive amounts of antibodies, which can obstruct filtration in the kidneys

Question 2

classic presentation of myeloma

Answer 2

CRAB
calcium elevated
renal failure
anaemia
bone lesions and bone pain - BACK!!

Question 3

name some investigations done in suspected myeloma and a positive result

Answer 3

serum protein electrophoresis - paraprotein spike
serum bence jones protein !!

Question 4

what is the most common complication of myeloma

Answer 4

anaemia

Question 5

what investigation is used to confirm the diagnosis of myeloma

Answer 5

bone marrow biopsy

Question 6

pepper pot skull on xray might indicate…

Answer 6

multiple lytic lesions in the skull
may indicate myeloma

Question 7

pathophysiology of amyloidosis

Answer 7

depositions of extracellular fibrils (misfolded proteins) in organs and blood vessels - causing dysfunction

Question 8

what are the 2 most common types of amyloidosis

Answer 8

primary - AL amyloidosis
secondary - AA amyloidosis

Question 9

what causes AL amyloidosis

Answer 9

deposition of monoclonal light chains from abnormal plasma cells

Question 10

what condition can commonly cause AL amyloidosis

Answer 10

myeloma

Question 11

what causes AA amyloidosis

Answer 11

systemic chronic inflammation causing the production of amyloid A protein

Question 12

what is a common condition that can lead to AA amyloidosis

Answer 12

rheumatoid arthritis

Question 13

what can periorbital purpura, ‘racoon eyes’ be a sign of

Answer 13

amyloidosis

Question 14

classic renal presentation of amyloidosis

Answer 14

nephrotic syndrome

Question 15

diagnostic investigation for amyloidosis

Answer 15

tissue biopsy shows apple green bifringence when stained with congo red under polarised light

Question 16

what investigation can be used to determine the extent of disease in amyloidosis

Answer 16

SAP scans

Question 17

management of AL amyloidosis

Answer 17

immunosuppression

Question 18

what is small vessel vasculitis

Answer 18

inflammation of the small blood vessels, including capillaries, venules and arterioles

Question 19

what are the 3 main ANCA-associated vasculitis

Answer 19

granulomatosis with polyangiitis
microscopic polyangiitis
eosinophilic granulomatosis with polyangiitis

Question 20

what is the old name for granulomatosis with polyangiitis

Answer 20

wegener’s granulomatosis

Question 21

what is commonly affected in granulomatosis with polyangiitis

Answer 21

respiratory tract and the kidneys

Question 22

classic URT sign of granulomatosis with polyangiitis

Answer 22

saddle shaped nose due to nasal bridge collapse

Question 23

presentation of granulomatosis with polyangiitis

Answer 23

URT: sinusitis, nose bleeds, hearing problems
LRT: cough, wheeze, haemoptysis
renal: rapidly progressing glomerulonephritis

Question 24

antibodies associated granulomatosis with polyangiitis

Answer 24

anti-PR3 (cANCA)

Question 25

antibodies associated with microscopic polyangiitis

Answer 25

anti-MPO (pANCA)

Question 26

classic presentation of microscopic polyangiitis

Answer 26

glomerulonephritis and haemoptysis

Question 27

what causes haemoptysis in microscopic polyangiitis

Answer 27

diffuse alveolar haemorrhage

Question 28

what is another name for eosinophilic granulomatosis with polyangiitis

Answer 28

churg strauss

Question 29

antibody associated with eosinophilic granulomatosis with polyangiitis

Answer 29

anti-MPO (pANCA)

Question 30

classic presentation of eosinophilic granulomatosis with polyangiitis

Answer 30

severe asthma in middle age and raised eosinophils on FBC

Question 31

small vessel vasculitis on renal biopsy

Answer 31

crescentic GN, pauci-immune

Question 32

management of small vessel vasculitis

Answer 32

immunosuppression

Question 33

what is systemic lupus erythematous (SLE)

Answer 33

inflammatory autoimmune conntective tissue disorder

Question 34

what is the usual clinical course of SLE

Answer 34

relapsing-remitting, with intermittent flares of disease

Question 35

most frequent renal abnormality in SLE

Answer 35

proteinuria

Question 36

autoantibodies in a patient with SLE

Answer 36

ANA, anti-dsDNA and anti-sm

Question 37

key diagnostic test for lupus nephritis

Answer 37

renal biopsy

Question 38

first line management of SLE

Answer 38

hydroxychloroquine

Question 39

what is the leading cause of kidney failure in the UK

Answer 39

diabetic nephropathy

Question 40

what is diabetic nephropathy

Answer 40

kidney damage due to long-standing hyperglycaemia in the context of diabetes

Question 41

Kimmelstiel-Wilson lesions

Answer 41

diabetic nephropathy!!

Question 42

what is diabetic nephropathy commonly associated with

Answer 42

retinopathy

Question 43

how do we define diabetic nephropathy

Answer 43

albuminuria and progressive reduction in eGFR in the context of long duration of diabetes

Question 44

main investigation for diabetic nephropathy

Answer 44

biopsy: thickening of the glomerular basement membrane

Question 45

first line management of diabetic nephropathy

Answer 45

ACEi

Question 46

second line management of diabetic nephropathy

Answer 46

SGLT2i

Question 47

what is another name for goodpasture syndrome

Answer 47

anti-GBM

Question 48

what causes goodpasture syndrome

Answer 48

antibodies (IgG) against a3 subunit of type 4 collagen

Question 49

what kind of hypersensitivity reaction is goodpastures syndrome

Answer 49

type 2

Question 50

what are the 2 main clinical presentations of goodpastures

Answer 50

rapidly progressive glomerulonephritis and pulmonary haemorrhage

Question 51

management of goodpastures

Answer 51

plasmapheresis

Question 52

gold standard diagnosis of goodpastures and a positive result

Answer 52

renal biopsy cellular crescents and focal necrosis, linear IgG deposition