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Renal > Kidneys in systemic disease > Flashcards

Kidneys in systemic disease Flashcards

1
Q

What are some systemic causes of CKD?

A

Myeloma
Amyloidosis
Vasculitis
SLE

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2
Q

What is myeloma?

A

A haematological cancer in which there is clonal expansion of plasma cells causing production of clinical immunoglobulins (M-proteins)

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3
Q

What are some symptoms of myeloma?

A
  • Bone pain
  • Weakness
  • Fatigue
  • Weight loss
  • Recurrent infections
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4
Q

What are some signs of myeloma?

A
  • Anaemia
  • Hypercalcaemia
  • Renal failure
  • Lytic bone lesions
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5
Q

What causes renal impairment in myeloma (20-40%)?

A
  • Glomerular - Monoclonal immunoglobulin deposition
  • Tubular - Light chain cast nephropathy
  • Misc. - Dehydration, hypercalcaemia, contract, bisphosphonates and NSAIDs
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6
Q

What investigations are required in myeloma?

A

Bloods
Urine
Bone marrow biopsy
Skeletal survey
Renal biopsy

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7
Q

What blood tests in particular are required in myeloma?

A

Serum protein electrophoresis
Serum free light chains

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8
Q

What will be seen in urine in myeloma?

A

Bence-Jones proteins (Clonal light chains)

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9
Q

What are some management options of renal impairment in myeloma?

A

Stop nephrotoxic drugs
Manage hypercalcaemia
Chemotherapy or stem-cell transplant
Dialysis

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10
Q

What is amyloidosis?

A

This is the deposition of extracellular amyloid (Insoluble protein fibrils) in the tissues or organs

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11
Q

Describe the pathophysiology of amyloidosis

A

Abnormal folding of proteins which then aggregate and become insoluble

Breakdown of usual degredation pathways for abnormally folded proteins

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12
Q

What re the 4 most common forms of amyloidosis?

A
  • Primary / Light chain (AL)
  • Secondary / Systemic / Inflammatory (AA)
  • Dialysis (Aß2M)
  • Hereditary and old age (ATTR)
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13
Q

Describe the pathway of primary (Light chain) amyloidosis (AL)

A

Unknown factor causes monoclonal B-lymphocyte proliferation
This causes monoclonal plasma cell formation
This causes release of immunoglobulin light chains (Bence-Jones) which is deposited in the kidneys causing damage

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14
Q

Describe the pathway of secondary/systemic/inflammatory amyloidosis (AA)

A

Chronic inflammation causes macrophage activation
Macrophages release IL-1 and IL-6
This causes liver cells to release SAA protein which build up in the kidneys causing damage

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15
Q

Renal presentation of amyloidosis

A

Nephrotic proteinuria +/- Impaired renal function

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16
Q

Cardiac presentation of amyloidosis

A

cardiomyopathy

17
Q

Nervous presentation of amyloidosis

A

Peripheral or autonomic neuropathy

18
Q

Gastrointestinal presentation of amyloidosis

A
  • Hepatomegaly/Splenomegaly
  • Malabsorption
19
Q

What investigations are required in amyloidosis?

A

Urinalysis + PCR
Blood tests - RFT, Inf markers, protein electrophoresis
Renal biopsy
SAP scan

20
Q

What test is performed on renal biopsy in amyloidosis?

A

Congo red staining → Apple green under polarised light

21
Q

What is a SAP scan?

A

Scintigraphy with radiolabelled serum amyloid to show extent of the disease

22
Q

How is AA amyloidosis managed?

A

Treat underlying condition

23
Q

How is AL amyloidosis managed?

A

Immunosuppression - Steroids, chemotherapy, stem cell transplant

24
Q

What is systemic lupus erythematosus (SLE)?

A

This is a chronic auto-immune inflammatory disease of unknown origin

25
Q

What systems can be affected by SLE?

A

skin, joints, kidneys, lungs, nervous system and serous membranes

26
Q

Who is most at risk of SLE?

A

Most commonly affects women aged 20-30 and African Americans and Hispanics

27
Q

What tests are required in SLE?

A
  • Blood tests - Raised inflammatory markers, immunology (Anti-dsDNA) , complement system markers
  • Urinalysis - Proteinuria +/- microscopic haematuria
28
Q

What percentage of SLE patients have renal involvement?

A

50%

29
Q

What is renal impairment due to SLE called?

A

Lupus nephritis

30
Q

What are the 6 ISN classes of lupus nephritis?

A
  • Class I - Minimal mesangial
  • Class II - Mesangial proliferative
  • Class III - Focal proliferative
  • Class IV - Diffuse proliferative
  • Class V - Membranous
  • Class VI - Advanced sclerosing
31
Q

How is lupus nephritis managed?

A

Immunosuppression

32
Q
A

Renal (41 decks)

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