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Renal > Kidney in Systemic Disease > Flashcards

Kidney in Systemic Disease Flashcards

1
Q

What is dysproteinaemia

A

Abnormal proteins
Dysregulation leads to overproduction
usually from the B cell line

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2
Q

What is myeloma

A

Cancer of the plasma cells
The abnormal cells accumulate in the marrow and impair production of normal blood cells
They also overproduce abnormal antibodies - these block the kidneys

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3
Q

How does myeloma present

A 
Bone Pain - particularly back
Renal failure 
Weakness
Fatigue
Weight Loss
Recurrent infections
Anaemia
Hypercalcemia
Lytic bone lesions
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4
Q

How does myeloma affect the kidneys

A

Deposits of protein in the glomerulus

Can get light chain cast nephropathy - lumps of protein visible in tubules

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5
Q

Which tests should you do if myeloma is suspected

A 
Serum protein electrophoresis 
Serum free light chains 
Urine - BJ proteins 
Bone marrow biopsy 
Skeletal survey 
Renal biopsy
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6
Q

How do you treat myeloma

A 
Stop nephrotoxics
Manage hypercalcaemia (saline +/- bisphosphonates)
Chemo 
Stem cell transplant is curative 
May use plasma exchange or dialysis
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7
Q

What is amyloidosis

A

Deposition of extracellular amyloid in tissue/organs

Due to abnormal folding of proteins which makes them insoluble and they aggregate

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8
Q

What are the 4 most common types of amyloidosis

A

Primary/light chain (AL)
Secondary/inflammatory (AA)
Dialysis
Hereditary and old age

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9
Q

Describe AL (primary light chain) amyloidosis

A

Caused by overproduction of light chains – small bits of antibody
Get into blood and form deposits

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10
Q

Describe AA (secondary) amyloidosis

A

Associated with systemic inflammation
Production of acute phase protein - SAA
Seen in chronic inflammatory conditions - RA, IBD

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11
Q

Where does AL (primary) amyloidosis affect

A 
Heart 
Bowel 
Skin 
Nerves 
Kidneys
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12
Q

Where does AA (secondary) amyloidosis affect

A

Liver
Spleen
Kidneys
Adrenals

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13
Q

How does amyloidosis present

A 
depends on the organs involved 
Get proteinuria and impaired renal function 
Cardiomyopathy 
Neuropathy 
Hepato/splenomegaly 
Malabsorption
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14
Q

How do you diagnose amyloidosis

A

Urinalysis and urine PCR
Bloods - renal function, inflammatory markers, electrophoresis
Renal biopsy - need a tissue diagnosis to confirm
SAP scan

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15
Q

How do you manage AA amyloidosis

A

treat the underlying inflammatory condition

refer to national centre at UCL

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16
Q

How do you treat AL amyloidosis

A

Immunosuppression
Steroids, chemotherapy, stem cell transplant
Refer to national centre at UCL

17
Q

How do you stain a renal biopsy for amyloidosis

A

Use Congo red stain

Will give apple green bifringence

18
Q

How does small vessel vasculitis present

A

Fever, migratory arthralgia, weight loss, anorexia and malaise

19
Q

How do you diagnose ANCA vasculitis

A

Urinalysis - blood and protein
Antibody tests - ANCA etc
Bloods - inflam markers, AKI etc

20
Q

How do you manage vasculitis

A

Immunosuppression:
Steroids
Cyclophosphamide or rituximab

Plasma exchange
Supportive - dialysis etc

21
Q

Describe GPA

A

Predominantly anti-PR3
Necrotising granulomatous inflammation
Lung involvement as well
as kidney

22
Q

Describe microscopic polyangiitis

A

Anti-MPO
Small vessels with no granulomas
Systemic symptoms - renal, lung, skin, GI etc

23
Q

Describe eosinophilic GPA

A

Associated with asthma and eosinophilia

2/3 have skin involvement

24
Q

How does lupus nephritis present

A

Occurs in 50% of lupus patients
Proteinuria
Many different classifications

25
Q

How do you differentiate between the classes of lupus nephritis

A

Renal biopsy is the only way

Renal (18 decks)

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