Renal manifestations of systemic disease

Question 1

What is the commonest cause of ESRF

Answer 1

Diabetic nephropathy

Question 2

What is the pathophysiology of diabetic nephropathy

Answer 2

hyperglycaemia leads to increase in Bfs -> RAAS activation -> production of glycosylation end products + oxidative stress
There is increased glomerular capillary pressure, podocyte damage and endothelial dysfunction

Question 3

How does diabetic nephropathy present?

Answer 3

1st - albuminuria

later - scarring (GS), nodule formation + fibrosis w loss of renal function

Question 4

What accelerates the course of diabetic nephropathy

Answer 4

raised BP

Question 5

How is a diagnosis of diabetic nephropathy made?

Answer 5

microalbuminuria -> A:CR 3-30mg/mmol

Question 6

What is the treatment of diabetic nephropathy?

Answer 6

1. INTENSIVE DM CONTROL - prevents microalbuminuria and lowers risk of macroalbuminuria (ACR >30)
2. BP <130/80 - ACEi/ARBs, no dual therapy
3. Na+ restriction <5g NaCl <2g Na
4. Statins

Question 7

What is lupus?

Answer 7

Systemic autoimmune disease w abs against nuclear components -> deposition of ab complexes -> inflammation and tissue damage

Question 8

How does lupus present?

Answer 8

Rash
Photosensitivity
ulcers 
Arthritis
Serositis
CNS effects
Cytopenias
Renal disease

Question 9

Does lupus present as nephritis or nephrosis?

Answer 9

EITHER

Question 10

Hows a diagnosis of lupus made?

Answer 10

Clinical
Ab profile - ANA (sensitive not specific)
Anti-dsDNA

Question 11

What is the treatment of lupus?

Answer 11

Depends on histological class
Class I + II
1. ACEi/ARB - renal protection
2. Hydroxychloroquine - for extra renal disease
Class III + IV
Immunosuppression: mycophenolate, glucocorticoids, cyclophosphamide, rituximab

Question 12

What does ANCA stand for?What is it?

Answer 12

Anti-Neutrophil Cytoplasmic Antibodies

Serological marker, presence characterises a group of vasculitides

Question 13

What are the different types of AAV?

Answer 13

1. Wegeners granulomatosis
2. Microscopic polyangiitis
3. Churg-strauss syndrome

Question 14

What does AAV stand for?

Answer 14

ANCA associated vasculitis

Question 15

What are the main types of ANCA?

Answer 15

1. Cytoplasmic - cANCA - Wegeners

2. Perinuclear - pANCA - Churg-Strauss

Question 16

How is a diagnosis of AAV made?

Answer 16

Clinical
ANCA
Biopsy
Rapidly progressive GN w/o immune deposits

Question 17

What is the treatment of AAVs?

Answer 17

High dose glucocorticoids + cyclophosphamide or rituximab

Plasma exchange if presents w RF or pulmonary haemorrhage

Question 18

What is Wegener’s also known as?

Answer 18

Granulamatosis w polyangiitis

Question 19

1. What is Wegener’s?

2. what parts of the body does it affect?

Answer 19

1. Autoimmune condition associated w necrotising granulomatous vasculitis
2. URT + LRT + kidneys

Question 20

What are the clinical features of Wegener’s?

Answer 20

1. URT - epistaxis, sinusitis
2. LRT - SOB, haemoptysis
3. Rapidly progressive GN
4. Saddle shaped nose deformity
5. Vasculitis and rash, proptosis, CN lesions

Question 21

What are the Ix for Wegener’s?

Answer 21

cANCA +ve in 90%, pANCA +ve in >25%
CXR - wide variation including cavitating lesions
Renal biopsy - epithelial crescents

Question 22

What are the features of microscopic polyangiitis?

Answer 22

Renal impairment - raised Cr, haematuria and proteinuria
Fever, lethargy, weight loss
Rash: palpable purpura
Cough SOB haemoptysis

Question 23

What is churg-strauss also known as

Answer 23

Eosiniphilic granulomatosis w polyangiitis

Question 24

What are the features of churg-strauss

Answer 24

asthma
blood eosinophilia
paranasal sinusitis
Mononeuritis multiplex - isolated damage to 2 or more separate nerve areas

Question 25

What are the differences and similarities between wegeners and churg-strauss

Answer 25

``` W: - renal failure - Epistaxis - cANCA CS: - Asthma - Eosinophilia - pANCA Similarities: - vasculitis - sinusitis - SOB ```

Question 26

How does myeloma cause renal disease?

Answer 26

There is tubular obstruction due to light chain casts | There is deposition of Ig/light chains in glomerulus leading to proteinuria

Question 27

How does myeloma w renal disease present?

Answer 27

proteinuria hypercalcaemia renal tract infection due to immunosuppression

Question 28

What is the treatment for the renal disease caused by myeloma?

Answer 28

adequate hydration bisphosphonates for hypercalcaemia anti-myeloma Rx including glucocorticoids

Question 29

How does amyloid cause renal disease?

Answer 29

pathological folding of proteins - extracellular accumulation + organ dysfunction including kidney

Question 30

how is amyloid classified?

Answer 30

AL amyloid - light chains in myeloma | AA amyloid - serum amyloid A in chronic inflammation

Question 31

How is amyloid diagnosed?

Answer 31

Congo red stain gin gon biopsy | SAP scan

Question 32

What are the main features of haemolytic uraemic syndrome (HUS)?

Answer 32

1. AKI 2. Microangiopathic haemolytic anaemia 3. Thrombocytopenia

Question 33

How is HUS classified and what are their causeS?

Answer 33

1. Typical HUS (secondary): - Shiga toxin producing Escherichia Coli (STEC) 0157:H7 - Pneumococcal infection - HIV 2. Atypical HUS: - Dysregulation/uncontrolled activation of complement

Question 34

Which cause of HUS is mostly seen in children

Answer 34

E.Coli shiga toxin producing

Question 35

What is the pathophysiology underlying HUS

Answer 35

Glomerular endothelial cells cause damage to rbcs, platelets | As toxins bind to endothelial cells rbc breakdown -> anaemia

Question 36

What are the investigations for HUS?

Answer 36

``` FBC - anaemia, low platelets Peripheral blood film - schistocytes Stool culture U+E - AKI LDH ```

Question 37

What are the signs and sx of HUS?

Answer 37

Bloody diarrhoea N+V NO fever Colitis

Question 38

What is the treatment of HUS?

Answer 38

good hydration w IV fluids Rx renal failure Atypical - plasma infusion/ exchange, eculizumab

Question 39

How does TTP present?

Answer 39

1. Microangiopathic haemolytic anaemia 2. Thrombocytopenia 3. AKI 4. Neuro sx: headache, palsy, seizure, coma, confusion 5. Fever

Question 40

What is the pathophysiology behind TTP

Answer 40

Congenital deficiency of or acquired abs to ADAMTS13 protease which normally cleaves multimers of vWF Large vWF multimers lead to platelet aggregation and fibrin deposition in small vessels Ultimately leads to: MULTI-SYSTEM THROMBOTIC MICROANGIOPATHY

Question 41

What is the treatment of TTP

Answer 41

Medical emergency!! 1 plasma infusion/exchange - removes abs/ replaces ADAMTS13 2. CS 3. Rituximab for non-responders or relapse

Question 42

What is atherosclerotic renovascular diseasee?

Answer 42

part of systemic atheromatous vascular disease where plaques develop in the main arteries to the kidneys and they can become stenotic

Question 43

How is a diagnosis of atherosclerotic renovascular disease made?

Answer 43

>1.5cm in asymmetry in renal size | CT/MRI angiography

Question 44

How is atherosclerotic renovascular disease treated?

Answer 44

control CV risk factors: ACEi/ARBs Statins Aspirin

Question 45

How is scleroderma renal crisis diagnosed

Answer 45

accelerated HTN >150/85 plus AKI | Biopsy - shows collapsed glomeruli and onion skin thickening of arterioles

Question 46

What is the treatment of scleroderma renal crises?

Answer 46

ACEi/ARBs | IV vasodilators

Question 47

What are the features of sickle cell nephropathy?

Answer 47

hyperfiltration (reduced creatinine) and albuminuria

Question 48

What is the treatment of sickle cell nephropathy?

Answer 48

ACEi/ARB | transplant