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Flashcards in Kidneys in Systemic Disease Deck (36):
1

Give some systemic diseases affecting the kidney

Diabetes mellitus

Cardiovascular disease:
-Cardiac failure
-Atheroembolism
-Hypertension
-Atherosclerosis

Infection:
-Sepsis
-Post-infective GN
-Infective endocarditis

Inflammation in blood vessels:
-SLE
-Vasculitis
-Scleroderma and other connective tissue diseases
-Cryoglobulinaemia

HUS/TTP
Myeloma
Amyloidosis

Drugs
-Aminoglycosides
-ACE inhibitors
-Penicillamine, gold
-NSAIDs
-Radiocontrast

2

What type of damage to drugs cause to the kidney?

Acute interstitial nephritis

3

What is the pathology of diabetic nephropathy?

1. Hyperfiltration
-Silent sub-clinical phase
-Pathological increase of GFR
-Hyperfiltration due to osmotic effect of glucose

2. Microalbuminuria
-[20-200ug/d]
-Check diabetics every year for this

3. Clinical nephropathy
-Proteinuria > 0.5g/d
-Decline in kidney function
-Brisk rise in proteinuria

4. Established renal failure

4

How does diabetic nephropathy in type 1 and type 2 differ in terms of rate of progression?

Doesn't

Nephropathy due to chronic increase in blood glucose.

Progression seems to be the same

5

What is the long term risks of diabetic nephropathy for Type 1 and type 2 diabetic patients?

Type 1
-4% develop nephropathy within 10 years
-5% develop nephropathy within 25 years

Type 2
-10% nephropathy by 5 years
-30% nephropathy by 20 years

6

What percentage of those with diabetic nephropathy will progress to ESRF?

30%

7

What is the commonest single cause of ESRF?

Diabetic nephropathy

8

How does GFR stage Chronic Kidney Disease?

Stage 1 = GFR >90
Stage 2 = GFR 60-89
Stage 3 = GFR 30-59
Stage 4 = GFR 15-29
Stage 5 = GFR

9

What is a common cause of renal failure in older patients?

Renal vascular disease

10

Describe the pathology of atheroembolic diasease

Eosinophilia -> allergic reaction to cholesterol

Peripheral rash in legs

Common if on warfarin

Vascular procedures can precipitate by disturbing cholesterol triggering eosinophilia

11

What is vasculitis?

Inflammatory reaction in the wall of any blood vessel

Defined by size of vessel involved

Can affect single or multiple organs

Wide spectrum of clinical presentations

12

Describe the type of vasculitis and causes

Aorta/ large artery
-Takayasu
-Giant cell arteritis

Medium artery
-Polyarteritis nodosa
-Kawasaki disease

Small vessel
-Wegener's granulomatosis
-Microscopic polyarteritis
-Churg-strauss syndrome

13

What us wegener's granulomatosis also called?

Granulomatous polyarteritis

14

What is Churg-Strauss syndrome?

Autoimmune condition -> vasculitis of medium-sized blood vessels in persons with a history of airway allergic hypersensitivity (atopy).

Usually manifests in three stages.

-Early (prodromal) stage:
---Marked by airway inflammation; almost all patients experience asthma and/or allergic rhinitis.

-Second stage
---Characterized by hypereosinophilia, which causes tissue damage, most commonly to the lungs and the digestive tract.

-Third stage
---Vasculitis, which can eventually lead to cell death and can be life-threatening.

15

What is the most common pANCA target?

By far the most common p-ANCA target is myeloperoxidase (MPO), a neutrophil granule protein whose primary role in normal metabolic processes is generation of oxygen radicals.

When the condition is a vasculitis, the target is usually MPO.

16

What is Wegener's granulomatosis?

Granulomatous inflammation in respiratory tract

Focal necrotising glomerulonephrotos with crescents

Slightly more common in males

Affects all age groups, most common 40-60

17

How does Wegener's granulomatosis effect the respiratory tract?

Upper respiratory tract
-Epistaxis
-Nasal deformity
-Sinusitis
-Deafness

Lower respiratory tract
-Cough
-Dyspnoea
-Haemoptysis
-Pulmonary haemorrhage

18

Apart from the lungs what other organs does wegener's granulomatosis effect?

Kidney - gomerulonephritis

Joints - arthralgia, myalgia

Eyes- scleritis

Heart - pericarditis

Systemic - fever, weight loss, vasculitic skin rash

19

What is microscopic polyarteritis?

Similar clinical spectrum to Wegener's granulomatosis

Can present with systemic disease, renal and pulmonary involvement

More commonly renal limited disease

20

How can you diagnose vasculitis?

Urine
-Blood/protein

Renal function
-Raised urea/creatinine

Biochemistry
-Raised alk phos, CRP, low albumin

Haematology
-Anaemia, thrombocytosis, leukocytosis

Immunology
-Hyperglobulinaemia
-Positive ANCA

Renal biopsy

21

What are the two types of ANCA seen in vasculitis?

c-ANCA
p-ANCA

22

What ELISA matches to immunostaining?

p-ANCA -> myeloperoxidase

c-ANCA -> Proteinase 3

23

What do different ANCA results mean?

Wegener's granulomatosis >90% c-ANCA

Microscopic polyarteritis usually p-ANCA

Churg-Strauss may be neither

ANCA can be false positive
-e.g. inflammatory bowel disease

24

What is infective endocarditis and what causes it typically?

A result of bacterial (or fungal) infection on cardiac valves

Typical infections:
-Staphylococcus aureus
-Viridans streptococci
-Enterococci

25

How can infective endocarditis lead to secondary glomerulonephritis +/- small vessel vasculitis?

Immune complex formation

26

How is glomerulonephritis in infective endocarditis spotted?

Renal involvement suggested by:
-Abnormal urea/creatinine
-Haematuria, red cell casts
-Reduced complement levels

Renal disease shouls recover when underlying infection is treated

27

What is multiple myeloma?

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

Common in the elderly

28

What are the clinical features of multiple myeloma?

-Markedly elevated ESR
-Anaemia
-Weight loss
-Pathological fractures
-Infections
-Back pain/ Cord compression

29

How is multiple myeloma diagnosed?

Bone marrow aspirate > 10% clonal plasma cells

Serum paraprotein +/- immunoparesis

Urinary Bence-Jones protein (BJP)

Skeletal survey
-lytic lesions

30

How can myeloma result in renal failure?

Cast nephropathy
-"Myeloma kidney"

Light chain nephropathy

Amyloidosis

Hypercalcaemia

Hyperuricaemia

31

How is cast nephropathy seen?

Light microscopy
-Tubule will be filled with material

Anti-lambda immunochemistry

32

What can you see for light chain disease on light microscopy and immunoflourescence?

Light chain disease = TBM Ig deposition

LM = PAS stain
IF = anti-kappa

33

What symptoms should make you suspect a systemic disease with renal involvement?

Fever, malaise, weight loss

Arthralgia, myalgia

Skin rash (vasculitic)

Gritty eyes

Breathlessness, haemoptysis, epistaxis

Haematuria, oedema

34

What signs should make you suspect a systemic disease with renal involvement?

Hands
-Spinter haemorrages, purpura, Rayneud's

Face
-Scleritis, uveitis, nasal cartilage deformity, retinal vasculitis, hypertensive retinopathy

Skin
-Vasculitic rash, scleroderma

CVS
-Hypertension, murmur

Chest:
-Crepitations, haemoptysis

Locomotor
-Joint swelling, tenderness

CNS
-Stroke, encephalopathy

35

What investigations would make you suspect a systemic disease with renal involvement?

Urine
-Blood/protein on urinalysis
-microscopy - red cell casts

Blood
-Elevated urea/creatinine
-Raised CRP
-Thrombocytosis, anaemia
-Raised alkaline phosphate

36

If suspecting a systemic disease with real involvement what investigations should you carry out?

Blood:
-ANCA (anti-MPO/anti-PR3 antibodies
-ANA, dsDNA antibodies
-Complement levels C3, C4
-Blood cultures

Radiology
-CXR
-USS abdomen (renal size)
-CT thorax (pulmonary granulomas, interstitial disease)
-Echocardiography

Biopsy
-Kidney, nasal mucous, lung, skin