kidneys in systemic diseases Flashcards

1
Q

what are some systemic causes of CKD?

A
  • diabetes
  • myeloma
  • amyloidosis
  • vasculitis
  • SLE
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2
Q

what is myeloma?

A
  • a dysproteinaemia
  • overproduction of immunoglobulin (a protein) by clonal expansion of cells from B cell linage
  • a cancer of plasma cells (WBC normally responsible for producing antibodies)
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3
Q

how does myeloma present?

A

classic presentation= back pain and renal failure

Symptoms:

  • bone pain
  • weakness
  • fatigue
  • weight loss
  • recurrent infections

Signs:

  • anaemia
  • hypercalcaemia
  • renal failure
  • lytic bone lesions
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4
Q

what investigation is done for suspected myeloma?

A

Protein electrophoresis + BJP= first line

Skeletal survey (Xray of all skeleton):
-shows lytic lesions

Bloods:

  • serum protein electrophoresis (clonal Ig)
  • serum free light chains (kappa/ lambda)

Urine:
-Bence Jones Protein

  • Bone marrow biopsy
  • skeletal survey
  • renal biopsy
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5
Q

what are the renal manifestations of myeloma?

A

Renal manifestations:
20-40% present with renal impairment

Glomerular:

  • AL amyloidosis
  • monoclonal immunoglobin deposition (light/heavy chains)

Tubular:
-light chain cast nephropathy

Miscellaneous:
-dehydration/hypercalcaemia/ contrast/ bisphosphonates/ NSAIDs

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6
Q

what is amyloidosis?

A

-deposition of extracellular amyloid (insoluble protein fibrils) in tissues or organs

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7
Q

what are the 4 most common types of amyloidosis?

A
  • Primary/ light chain (AL)
  • secondary/systemic/inflammatory (AA)
  • dialysis
  • hereditary and old age (ATTR)
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8
Q

what is AL/ light chain amyloidosis?

A
  • production of abnormal immunoglobulin light chains from plasma cells
  • light chains enter the bloodstream and cause amyloid deposits
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9
Q

what does AL/light chain amyloidosis typically affect?

A

More commonly affects the heart, bowel, skin, nerves, kidneys

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10
Q

who is AL/light chain amyloidosis more common in?

A

55-60 year olds

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11
Q

what is AA amyloidosis?

A
  • amyloidosis associated with systemic inflammation

- production of acute phase protein (serum amyloid A protein

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12
Q

who does AA amyloidosis typically affect?

A

Develops in roughly 5% of patients with chronic inflammatory conditions or chronic infections:

  • RA, IBS, psoriasis
  • TB, osteomyelitis, bronchiectasis
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13
Q

what structures does AA amyloidosis tend to affect?

A

More commonly affects the liver, spleen, kidneys and adrenals

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14
Q

how does amyloidosis present?

A

Renal- proteinuria +/- impaired renal function
Heart- cardiomyopathy
Nerves- peripheral or autonomic neuropathy
Hepatomegaly/splenomegaly
GI- malabsorption

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15
Q

what investigations are done for amyloidosis?

A

Urinanalysis + uPCR
Blood tests- renal function, markers of inflammation, protein electrophoresis, SFLC

Renal Biopsy: congo red staining (apple green under polarised light)

Can also biopsy other areas (easiest is abdominal fat pad or rectal biopsy)

To see extent of disease can do a SAP scan

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16
Q

what is the management for AL/ light chain amyloidosis?

A
  • immunosuppression
  • steroids
  • chemotherapy
  • stem cell transplant
17
Q

what is the management for AA amyloidosis?

A

-treat underlying condition

18
Q

investigations for MPA?

A

Urinalysis:
- ‘active urine’ so leakage of blood and protein

Bloods:
-raised inflammatory markers

Immunology:

  • ANCA
  • Anti MPO (p-ANCA)

Renal Biopsy:

  • crescentic GN
  • pauci immune
19
Q

investigations for EGPA?

A

Urinalysis:
- ‘active urine’ so leakage of blood and protein

Bloods:
-raised inflammatory markers

Immunology:

  • ANCA
  • Anti MPO (p-ANCA)

Renal Biopsy:

  • crescentic GN
  • pauci immune
20
Q

investigations for GPA?

A

Urinalysis:
- ‘active urine’ so leakage of blood and protein

Bloods:
-raised inflammatory markers

Immunology:

  • ANCA
  • Anti PR3 (c-ANCA)

Renal Biopsy:

  • crescentic GN
  • pauci immune
21
Q

how does GPA present?

A
  • fever, weight loss, anorexia, malaise
  • necrotising granulomatous inflammation
  • lung involvement (pulmonary/renal syndrome)
  • saddle shaped nose deformity
22
Q

how does EGPA present?

A
  • fever, weight loss, anorexia, malaise
  • associated with asthma and eosinophilia
  • 2/3 have skin involvement
23
Q

how does MPA present?

A
  • fever, weight loss, anorexia, malaise
  • small cell vasculitis with no granulomas
  • systemic features/ renal/ lung/ skin/ GI/ nerves
24
Q

what is the treatment for small cell vasculitis?

A

Immunosuppression:

  • steroids
  • cyclophosphamide/rituximab
  • plasma exchange

Supportive:

  • dialysis
  • ventilation
25
Q

what can be used to diagnose lupus?

A
26
Q

what is the classification for lupus?

A

ISN classification