kidneys in systemic disease Flashcards

1
Q

systemic causes of CKD

A
  • myeloma
  • amyloidosis
  • vasculitis
  • SLE
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2
Q

what is myeloma

A

cancer of plasma cells

  • collection of abnormal plasma cells can accumulate in the bone marrow and/or soft tissue
  • impairment of production of normal blood cells > anaemia
  • monoclonal production of a paraprotein could cause renal dysfunction
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3
Q

presentation of myeloma

A
  • bone pain
  • weakness
  • fatigue
  • weight loss
  • recurrent infections
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4
Q

signs of myeloma

A
  • anaemia
  • hypercalcaemia
  • renal failure
  • lytic bone lesions
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5
Q

renal manifestations of myeloma

A

Glomerular

  • AL amyloidosis
  • monoclonal immunoglobulin deposition

Tubular
-light chain cast nephropathy

-dehydration/hypercalcaemia,

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6
Q

diagnosis of myeloma

A
bloods (proteins, free light chains)
urine (bence jones protein)
bone marrow biopsy
skeletal survey
renal biopsy

usually protein electrophoresis and BJP

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7
Q

management of myeloma

A
  • stop nephrotoxics
  • manage hypercalcaemia
  • chemotherapy
  • stem cell transplant
  • dialysis
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8
Q

how do you manage hypercalcaemia

A

saline +/- bisphosphonates

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9
Q

what is amyloidosis

A

deposition of extracellular amyloid in tissues or organs

-occurs due to abnormal folding of proteins which then aggregate and become insoluble

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10
Q

what are the four forms of amyloidosis

A
  • primary/light chain (AL)
  • secondary/systemic/inflammatory (AA)
  • dialysis
  • hereditary and old age
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11
Q

what is AL amyloidosis

A
  • production of abnormal immunoglobulin light chains from plasma cells
  • light chains enter bloodstream and cause amyloid deposits
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12
Q

what does AL amyloidosis commonly affect

A
  • heart
  • bowel
  • skin
  • nerves
  • kidneys
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13
Q

who usually gets AL amyloidosis

A

55-60yrs

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14
Q

what is AA amyloidosis

A
  • associated with systemic inflammation

- production of acute phase protein - serum amyloid A protein

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15
Q

what does AA amyloidosis affect

A
  • liver
  • spleen
  • kidneys
  • adrenals
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16
Q

presentation of amyloidosis

A

depends on organ or tissues involved

  • renal - proteinuria +/- impaired renal function
  • cardiomyopathy
  • peripheral or autonomic neuropathy
  • hepatomegaly/splenomegaly
  • malabsorption
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17
Q

renal investigation for amyloidosis

A

-urinalysis + uPCR
-blood tests
-renal biopsy (congo red staining)
-

18
Q

management for amyloidosis

A

no cure

  • preserve organ function and reduce further deposition
  • AA - treat underlying cause
  • AL - immunosuppression - steroids, chemo, stem cell transplant
19
Q

what is ANCA associated vasculitis

A

necrotising polyangiitis that affects capillaries, venules and arterioles

20
Q

symptoms of ANCA vasculiitis

A
  • fever
  • migratory arthralgia
  • weight loss
  • anorexia
  • malaise
21
Q

diagnosis of ANCA vasculiitis

A
  • urinalysis - active urine, blood and protein
  • bloods - raised inflammatory markers, AKI, anaemia
  • immunology - ANCA, anti-MPO, anti-PR3
  • renal biopsy - crescentic GN, pauci-immune
22
Q

what are the three types of ANCA vasculiitis

A
  • granulomatosis with polyangiitis (Wegener’s granulomatosis)
  • Microscopic polyangiitis (MPA)
  • eosinophilic granulomatosis with polyangiitis (churg-strauss)
23
Q

what is GPA

A
  • predominantly anti-PR3 antibodies
  • necrotising granulomatous inflammation
  • lung involvement (pulmonary/renal syndrome)
24
Q

what is microscopic polyangitis

A
  • anti-MPO antibodies
  • small cell vasculitis with no granulomas
  • systemic features/renal/lung/skin/GI/nerves
25
Q

what is EGPA (churg-strauss)

A
  • asthma and eosinophilia

- 2/3 have skin involvement

26
Q

management for vasculitis

A

immunosuppression - steroids/cyclophosphamide/rituximab
plasma exchange
dialysis, ventilation

27
Q

is GPA c-ANCA or p-ANCA

A

c-ANCA

28
Q

is MPA c-ANCA or p-ANCA

A

p-ANCA

29
Q

is EGPA c-ANCA or p-ANCA

A

p-ANCA

30
Q

what is SLE

A

chronic auto-immune inflammatory disease of unknown aetiology

31
Q

what does lupus affect

A
  • skin
  • joints
  • kidneys
  • lungs
  • nervous system
  • serous membranes
32
Q

who gets lupus

A

women in their 20s-30s

african americans and hispanics

33
Q

presentation of lupus

A
  • malar rash
  • discoid rash
  • photosensitivity
  • oral ulcers
  • nonerosive arthritis
  • pleuropericarditis
  • renal disease
  • neurologic disorder
  • positive LE preparation, raised anti-DNA antibody, anti-Sm present
  • positive fluorescent antinuclear antibody test
34
Q

differential diagnosis for lupus

A
  • sjogren’s syndrome
  • fibromyalgia
  • primary anti-phospholipid syndrome
  • thrombotic micro-angiopathies
35
Q

most frequently observed abnormality in lupus nephritis

A

proteinuria

36
Q

what are the different 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 slcerosing
37
Q

what medication should all patients with lupus nephritis be on

A

hydroxychloroquine

38
Q

what are some poor prognosis factors for lupus nephritis

A
  • renal disease
  • male sex
  • young age or older age
  • poor socio-economic status
  • antiphospholipid syndrome
  • high overall disease activity
39
Q

treatment for class II lupus nephritis

A

proteinuria >3gm can be treated with steroids or CNI

40
Q

treatment for class III and IV lupus nephritis

A
  • steroids and either cyclophosphamide or MMF

- maintenance is azathioprine or MMF + low dose steroids for 1 year

41
Q

how do you treat class V lupus nephritis

A
  • if normal kidney function then conservative management

- if proteinuria then > steroids + cyclophosphamide and/or CNI or MMF or azathioprine