Kidneys in Systemic Diseases

Question 1

what is diabetic nephropathy?

Answer 1

small vessel damage due to poor glycaemic control

Question 2

presentation of diabetic nephropathy

Answer 2

proteinuria
other small vessel damage
poor control signs/ history

Question 3

management of diabetic nephropathy

Answer 3

ACEI (watch for dry cough)
good control HbA1c
may need RRT

Question 4

what is myeloma?

Answer 4

dysproteinaemia that is a cancer of plasma cells (produce antibodies)

Question 5

what happens in myeloma?

Answer 5

there is abnormal plasma cells accumulating in bone marrow resulting in reduced production of normal blood cells

Question 6

what antibody produced in myeloma causes renal dysfunction?

Answer 6

paraprotein (Ab)

Question 7

presentation of myeloma

Answer 7

bone pain (lytic bone lesions, hypercalcaemia)
weakness, fatigue (anaemia)
weight loss
recurrent infections
renal failure

Question 8

classic presentation of myeloma

Answer 8

back pain
renal failure (AKI)

Question 9

renal manifestations in myeloma

Answer 9

• glomerular= AL amyloidosis and Ig deposition
• tubular= light chain cast nephropathy
• miscellaneous= dehydration, hypercalcaemia, contrast, bisphosphonates, NSAIDs (important to look at whole history)

Question 10

diagnosis of myeloma

Answer 10

bloods for serum protein electrophoresis and serum light chains
urine for Bence Jones protein (BJP)
bone marrow biopsy
skeletal survey
renal biopsy

Question 11

management of myeloma

Answer 11

chemotherapy
stem cell transplant
stop nephrotoxics
manage hypercalcaemia (saline +/- bisphosphonates)
plasma exchange to remove light chains (supportive= dialysis)

Question 12

what is amyloidosis?

Answer 12

deposition of insoluble, abnormally folded proteins

Question 13

four types of amyloidosis

Answer 13

1. primary/ light chain (AL)
2. secondary/ systemic/ inflammatory (AA)
3. dialysis
4. hereditary and old age

Question 14

what does AL amyloidosis produce?

Answer 14

abnormal immunoglobulin light chains from plasma cells

Question 15

age group of AL amyloidosis

Answer 15

55-60

Question 16

what does AA amyloidosis produce?

Answer 16

acute phase protein (serum amyloid A)

Question 17

what is AA amyloidosis associated with?

Answer 17

inflammatory conditions such as RA, IBD, psoriasis, TB, osteomyelitis, bronchiectasis

Question 18

what organs does AA amyloidosis affect?

Answer 18

liver
spleen
kidneys
adrenals

Question 19

presentation of amyloidosis

Answer 19

nephrotic proteinuria +/- impaired function
cardiomyopathy
peripheral or autonomic neuropathy
hepatomegaly/ splenomegaly
GI absorption

Question 20

diagnosis of amyloidosis

Answer 20

urinalysis and bloods
renal biopsy with Congo Red staining
SAP scan

Question 21

what is Congo red staining?

Answer 21

apple green birefringence under polarised light

Question 22

what is an SAP scan?

Answer 22

scintigraphy with radiolabelled serum amyloid

Question 23

management of AA amyloidosis

Answer 23

treat underlying condition

Question 24

management of AL amyloidosis

Answer 24

immunosuppression such as:

• steroids
• chemotherapy
• stem cell transplant

Question 25

define vasculitis

Answer 25

inflammation of blood vessels

Question 26

what are the three small vessel ANCA vasculitis types

Answer 26

1. GPA 2. MPA 3. eGPA

Question 27

what are the three small vessel ANCA vasculitis types?

Answer 27

1. GPA 2. MPA 3. eGPA

Question 28

antibodies in GPA

Answer 28

anti-PR3 Ab | cANCA

Question 29

what is GPA

Answer 29

necrotising granuloma inflammation

Question 30

presentation of GPA/what does it affect?

Answer 30

pulmonary/ renal syndrome

Question 31

antibodies in MPA

Answer 31

anti-MPO Ab | pANCA

Question 32

what is MPA?

Answer 32

no granulomas

Question 33

what does MPA affect?

Answer 33

``` systemic renal lung skin GI nerves ```

Question 34

what is eGPA?

Answer 34

vasculitis associated with asthma and eosinophilia has skin involvement

Question 35

presentation of vasculitis

Answer 35

``` fever arthralgia weight loss malaise saddle nose in GPA prodomal symptoms can last weeks-months before specific organ involvement ```

Question 36

diagnosis of vasculitis

Answer 36

``` active urinalysis with protein and blood raised inflammatory markers AKI anaemia renal biopsy has crescent GN and pauci-immune immunology ```

Question 37

management of vasculitis

Answer 37

immunosuppression (steroids, cyclophosphamide/ rituximab) plasma exchange dialysis ventilation

Question 38

what is SLE?

Answer 38

chronic AI inflammatory disease of unknown origin

Question 39

diagnosis of SLE in terms of lupus nephritis

Answer 39

urinalysis has proteinuria +/- microscopic haematuria

Question 40

classification of lupus nephritis

Answer 40

ISN classification of I-VI | via renal biopsy

Question 41

management of lupus nephritis

Answer 41

immunosuppressants such as steroids, azathioprine, cyclophosphamide, etc.

Question 42

what age is vasculitis most common?

Answer 42

5-7th decade