Renal Disorder in Systemic Disease

Question 1

Diabetic nephropathy pathology

Answer 1

Hyperglycaemia results in increased growth factors, RAS activation, oxidative stress + advanced glycosylation end products
-> Increased glomerular capillary pressure, podocyte damage + endothelial dysfunction
Glomerulosclerosis and nodule formation (Kimmelstiel-Wilson lesions) and fibrosis are late stage changes

Question 2

Diabetic nephropathy diagnosis

Answer 2

Microalbuminuria A:CR 3-30mg/mmol, from this stage regression not possible

Question 3

Diabetic nephropathy management

Answer 3

Keep BP <130/80
Sodium restriction <2g/day (<5g NaCl)
Statins to reduce CV risk
Blood sugar control to prevent development of complications

Question 4

SLE nephropathy presentation

Answer 4

Can present as nephritis or nephrosis

Question 5

SLE nephropathy diagnosis

Answer 5

Clinical
Anti-dsDNA and ANA (not specific) Abs
Biopsy if A:CR >30 or P:CR >50

Question 6

SLE nephropathy treatment

Answer 6

Class I and II show mild changes with little risk of renal progression so anti-RAS drugs and hydroxychloroquine for extra renal disease
Class III-V require immunosuppression

Question 7

Small vessel vasculitis diagnosis

Answer 7

Clinical
ANCA
Biopsy: rapidly progressive GN without immune deposits

Question 8

Small vessel vasculitis treatment

Answer 8

High dose glucocorticoids + cyclophosphamide/ rituximab

Plasma exchange if presents with renal failure/pulmonary haemorrhage

Question 9

Myeloma renal disease treatment

Answer 9

Adequate hydration
Bisphosphonates for hypercalcaemia (beware GFR<30)
Anti-myeloma treatments

Question 10

Amyloid pathology

Answer 10

Pathological folding of proteins causes extracellular accumulation + organ dysfunction
Classified according to protein

Question 11

Amyloid diagnosis

Answer 11

Congo red staining goes apple green on biopsy

SAP scan

Question 12

Haemolytic uraemic syndrome presentation

Answer 12

Microangiopathic haemolytic anaemia and AKI due to thrombosis of glomerular capillaries
In children associated with haemorrhagic colitis due to Shiga toxin producing E. coli (STEC)
Atypical HUS due to complement issues

Question 13

Haemolytic uraemic syndrome diagnosis

Answer 13

Triad of haemolytic anaemia, decreased platelets and AKI with haemat/proteinuria
Evidence of STEC may be present
Complement abnormalities may be present

Question 14

Haemolytic uraemic syndrome treatment

Answer 14

STEC-HUS: supportive

aHUS: plasma exchange, eculizumab (anti-C5)

Question 15

TTP pentad

Answer 15

Fever
AKI
Neuro symptoms
Decreased platelets
Microangiopathic haemolytic anaemia

Question 16

TTP pathology

Answer 16

Congenital deficiency/acquired antibodies to ADAMTS13 protease which normally cleaves vWF multimers, no cleavage so platelet aggregation + fibrin deposition in small vessels

Question 17

TTP diagnosis

Answer 17

Clinical

ADAMTS13 activity

Question 18

TTP treatment

Answer 18

Haem referral emergency
Plasma exchange to remove/replace ADAMTS13 (Abs)
Corticosteroids
Rituximab for refractory/relapse

Question 19

Atherosclerotic renovascular disease diagnosis

Answer 19

> 1.5cm asymmetry in renal size

CT/MR angiography

Question 20

Atherosclerotic renovascular disease treatment

Answer 20

CV RFs controlled
eGFR reduced <25% for longer-term renal and cardiac outcomes
Revascularisation only in flash pulmonary oedema/ rapid oligo/anuric renal failure

Question 21

Scleroderma renal crisis diagnosis

Answer 21

Accelerated HT (new >150/85) and AKI (decrease eGFR >30%)
Biopsy shows collapsed glomeruli + onion skin thickening of arterioles

Question 22

Scleroderma renal crisis treatment

Answer 22

anti-RAS
IV vasodilators to reduce vasc resistance and for digital ischaemia
Beware beta blockers as inc HR compensating for dec SV

Question 23

Scleroderma renal crisis RFs

Answer 23

Diffuse scleroderma
Anti-RNA polymerase III Abs
<2yrs from diagnosis

Question 24

Sickle cell nephropathy pathology

Answer 24

HbSS associated with hyperfiltration and albuminuria

Progression to renal failure usually associated with another trigger e.g. infection

Question 25

Sickle cell nephropathy diagnosis

Answer 25

Clinical

Biopsy only to look for other diagnosis

Question 26

Sickle cell nephropathy treatment

Answer 26

anti-RAS

Aim to transplant as mortality increased on dialysis