The kidneys in systemic disease

Question 1

List 4 reasons why the kidney is a particular target for systemic diseases

Answer 1

receives more blood flow per unit than any other organ
glomeruli may filter proteins absorbed by tubule epithelial cells
Glomeruli may trap proteins or immune complexes
kidney metabolises or excretes certain drugs which may be toxic to the kidney

Question 2

What are the clinical features or diabetic nephropathy

Answer 2

proteinuria and declining GFR

Question 3

4 phases of diabetic nephropathy

Answer 3

silent sub-clinical phase
microalbuminuria
clinical nephropathy
established renal failure

Question 4

5 CKD stages

Answer 4

stage 1 - >90
stage 2 - 60-89
stage 3 - 30-59
stage 4- 15-30
stage 5 <15

Question 5

Who is particularly at risk of reno-vascular disease

Answer 5

the elderly

Question 6

Vasculitis

Answer 6

Inflammatory reaction in the wall of any blood vessel

Question 7

What is vasculitis defined by?

Answer 7

the size of vessel affected

Question 8

Vasculitis in aorta/large artery

Answer 8

giant cell arteritis

takyasu arteritis

Question 9

Medium artery vasculitis

Answer 9

polyarteritis nodosa

Kawasaki disease

Question 10

Small artery vasculitis

Answer 10

wegners granulomatosis
microscopic polyarteritis
churg-strauss syndrome

Question 11

What is wegners granulomatosis?

Answer 11

granulomatous inflammation in respiratory tract

focal necrotising GN with crescents

Question 12

Who does wegners granulomatosis affect?

Answer 12

males

usually 40-60

Question 13

Upper resp tract symptoms of wegners

Answer 13

epistaxis, nasal deformity, sinusitis, deafness

Question 14

Lower resp tract symptoms of wegners

Answer 14

cough, dyspnoea, haemoptysis, pulmonary haemorrhage

Question 15

Systemic symptoms of wegners

Answer 15

kidney - GN
joints - arthralgia and myalgia 
eyes - scleritis 
heart - pericarditis 
systemic - fever, weight loss, vasculitis skin rash

Question 16

How does microscopic polyarteritis present?

Answer 16

usually renal disease but can also have pulmonary and systemic involvement
similar clinical spectrum to wegners

Question 17

Diagnosing vasculitis and what is looked for 
a) urine
b)renal function 
c) biochemistry 
d) haematology 
e)immunology
renal biopsy

Answer 17

a - blood and protein 
b - raised urea/creatinine
c - raised alk phos, CRP, low albumin 
d - anaemia, thrombocytosis, leucocytosis
e - hyperglobulinaemia - positive ANCA

Question 18

2 types of ANCA and what they stand for

Answer 18

p - perinuclear and c - cytoplasmic

Question 19

P-ANCA and C-ANCA in wegners and microscopic polyarteritis

Answer 19

c - wegners

p - M.P

Question 20

When can ANCA be a false positive

Answer 20

IBD

Question 21

What Is infective endocarditis?

Answer 21

A result of bacterial or fungal infection in cardiac valves

Question 22

3 typical organisms of infective endocarditis

Answer 22

staph aureus
virdians streptococci
enterococci

Question 23

What does infective endocarditis lead to?

Answer 23

GN +/- small vessel vasculitis due to immune complex formation

Question 24

3 ways in which renal involvement is suggested in infective endocarditis

Answer 24

raised urea/creatinine
haematuria, red cell clasts
decreased complement levels

Question 25

Will the renal disease recover when infective endocarditis is treated?

Answer 25

yes

Question 26

What is multiple myeloma?

Answer 26

A monoclonal proliferation of plasma cells producing an excess of immunoglobulins and light chains

Question 27

Who is multiple myeloma common in?

Answer 27

elderly

Question 28

6 clinical features of multiple myeloma

Answer 28

``` anaemia infections spinal cord compression fractures weight loss elevated ESR ```

Question 29

5 ways in which multiple myeloma is diagnosed

Answer 29

``` bone marrow aspirate >10% clonal plasma cells serum paraprotein +/- immunoparesis urinary Bence-jones protein skeletal survey - lytic lesions serum free light chains ```

Question 30

5 suggestions of renal failure in multiple myeloma

Answer 30

``` cast nephropathy - myeloma kidney light chain nephropathy amyloidosis hypercalcaemia hyperuricaemia ```

Question 31

How is cast nephropathy seen?

Answer 31

light microscopy | anti-lambda immunochemistry

Question 32

Amyloid

Answer 32

deposition of abnormal fibrillary proteins that persist

Question 33

History of renal disease with systemic involvement

Answer 33

``` fatigue malaise weight loss anaemia arthralgia, myalgia uveitis, scleritis gritty eyes SOB, haemoptysis epistaxis haematuria oedema vasculitic rash ```

Question 34

Hand signs in renal systemic disease

Answer 34

splinter haemorrhages, purpura, raynauds

Question 35

Face signs in renal systemic disease

Answer 35

scleritis, uveitis, nasal cartilage deformities, hypertensive retinopathy

Question 36

Skin signs in renal systemic disease

Answer 36

vasculitic rash and scleroderma

Question 37

CVS signs in renal systemic disease

Answer 37

hypertension, murmur

Question 38

Chest signs in renal systemic disease

Answer 38

crepitations, haemoptysis

Question 39

locomotor signs in renal systemic disease

Answer 39

joint swelling, tenderness

Question 40

CNS signs in renal systemic disease

Answer 40

stroke, encephalopathy

Question 41

Initial urine investigations

Answer 41

blood and protein | microscopy - red cell clasts

Question 42

Initial blood tests

Answer 42

urea/creatinine CRP thrombocytosis, anaemia raised alkaline phosphatase

Question 43

Further blood investigations

Answer 43

``` ANCA blood cultures IG and electrophoresis complement levels C3.4 ANA, dsDNA antibodies ```

Question 44

Radiology

Answer 44

CXR echo USS abdomen CT thorax

Question 45

4 biopsies

Answer 45

kidney lung nasal mucosa skin