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Renal > Kidneys in Systemic Disease > Flashcards

Kidneys in Systemic Disease Flashcards

1
Q

what is dysproteinaemia

A

overproduction of immunoglobulin by clonal expansion of cells from B cell lineage

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2
Q

what is the main role of plasma cells

A

to make antibodies

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3
Q

what is myeloma

A

cancer of the plasma cells

causes excess production of immunoglobulins

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4
Q

what happens in myeloma

A
  • collecting of abnormal plasma cells accumulate in the bone marrow
  • impairment of production of normal blood cells
  • monoclonal production of a paraprotein
  • can cause renal dysfunction
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5
Q

what are the symptoms of myeloma

A 
bone pain 
weakness
fatigue 
weight loss 
recurrent infections

classic presentation= back pain and renal failure (AKI)

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6
Q

what are the signs of myeloma

A

anaemia
hypercalcaemia
renal failure
lytic bone lesions

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7
Q

what are the renal manifestations of myeloma

A

20-40% present with renal impairment

glomerular: AL amyloidosis, monoclonal immunoglobulin deposition (light/heavy chains)
tubular: light chain cast nephropathy
dehydration. hypercalcaemia

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8
Q

what is myeloma kidney

A

cast nephropathy (free light chains form waxy casts within the tubule lumen)

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9
Q

how do you diagnose myeloma

A 
have high index of suspicion 
bloods:
-serum protein electrophoresis (measures globulins)
-serum free light chains 
urine:
-bence jones protein

bone marrow biopsy
skeletal survey
renal biopsy (rarely done)

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10
Q

what is the management for myeloma

A

stop nephrotoxics and manage hypercalcaemia (saline +/- bisphosphonates)

CHEMOTHERAPY (majority of patients get this)
stem cell transplant

plasma exchange (to remove light chains)

supportive= dialysis

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11
Q

what is amyloidosis

A

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

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12
Q

why are amyloid proteins deposited

A

because the are abnormally folded which causes them to aggregate and become insoluble
(normal degradation pathways break down)

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13
Q

what are the 4 most common types of amyloidosis

A

primary/ light chain (AL)
secondary/ systemic/ inflammatory (AA)
dialysis (Abeta2M)
hereditary and old age (ATTR)

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14
Q

what is AL amyloidosis

A

unknown causes causes production of abnormal immunoglobulin light chains from plasma cells
(light chains enter the blood stream and cause amyloid deposits)

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15
Q

what is the usual presentation of AL amyloidosis

A

commonly affects heart, bowel, skin, nerves and kidneys

age at diagnosis 55-60
life expectancy (untreated)= 6 months- 4 years
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16
Q

what is AA amyloidosis

A

associated with systemic inflammation

production of acute phase protein - serum amyloid A protein (SAA)

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17
Q

what is the presentation of AA amyloidosis

A

develops in people with chronic inflammatory conditions/ chronic infections

  • RA, IBD, psoriasis
  • TB, osteomyelitis, bronchiectasis

commonly affects liver, spleen, kidneys and adrenals

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18
Q

what are the possible presentations of amyloid

A

depends on the tissues affected:

  • renal= nephrotic range proteinuria +/- impaired renal function
  • cardiac= cardiomyopathy
  • nerves= peripheral/ autonomic neuropathy
  • hepatomegaly/ splenomegaly
  • GI= malabsorption
19
Q

what investigations for amyloidosis

A

urinalysis + uPCR
bloods- renal function, markers of inflammation, protein electrophoresis, SFLC (serum free light chains)

renal biopsy (congo red staining- apple green under polarised light)

can do abdo fat/ rectal biopsy

SAP scan shows extent of the disease

20
Q

what do you need to diagnose amyloidosis

A

tissue biopsy (congo red stain- apple green bifringence)

21
Q

what is the management for amyloidosis

A

not curative
-reduce further deposition and preserve organ function

AA= treat underlying condition

AL= immunosuppression (steroids, chem, stem cell transplant)

22
Q

what is vasculitis

A

inflammation of the blood vessels

23
Q

what are the ANCA associated vasculitis

A

MPA
GPA
eGPA
drug induced

24
Q

what is small vessel ANCA associated vasculitis

A

necrotising polyangitis that affects capillaries, venules and arterioles

25
Q

what are the systemic symptoms of small vessel ANCA associated vasculitis

A

fever, migratory arthralgia, weight loss, anorexia, malaise

26
Q

what age group is small vessel ANCA vasculitis most common

A

5th, 6th and 7th decade

27
Q

how do you diagnose ANCA vasculitis

A

high index of suspicion
urinalysis- blood ++ protein ++
raised inflammatory markers, AKI, anaemia

ANCA
eGPA + MPA = anti MPO + pANCA
GPA= cANCA + PR3

renal biopsy

28
Q

what is GPA

A

necrotising granulomatous inflammation

-anti- PR3 antibodies

29
Q

where does GPA affect

A

lung (pulmonary/ renal syndrome), nose, nasal symptoms, epistaxis, saddle nose

30
Q

what is MPO

A

small vessel ANCA vasculitis with no granulomas

  • anti MPO antibodies
  • systemic features: renal, lung, skin, GI, nerves
31
Q

what is eGPA

A

associates with late onset asthma and eosinophilia

-2/3rds have skin involvement

32
Q

what is the management for vascultitis

A

immunosuppression

  • steroids
  • cyclophosphamide/ rituximab

plasma exchange

supportive- dialysis, ventilation

33
Q

what are 2 features of the malar rash in SLE

A

butterfly shaped

photosensitive

34
Q

what is SLE

A

chronic autoimmune inflammatory disease (unknown origin)

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

35
Q

who gets SLE

A

women (10:1) in theirs 20-30s

african americans and hispanics better risk

36
Q

how do you diagnose SLE

A

high index of suspicion in young lady with non specific symptoms

bloods:
-raised inflammatory markers
-immunology- ANA +ve, anti-dsDNA
complete (low)

urinalysis

37
Q

what can be used to measure disease activity in SLE

A

anti-dsDNA and complement (low when disease high)

38
Q

what is lupus nephritis

A

renal involvement in SLE

most frequently observed abnormality is proteinuria

39
Q

what are the types of lupus nephritis

A 
classified on how advanced 
class I minimal mesgangial 
class VI advanced sclerosing  
determines management
40
Q

what is the prognosis of SLE

A

varied clinical course
relasping/ remitting

poor prognostic factors for survival

  • renal disease
  • male sex
  • young/ old at presentation
  • deprivation
  • antiphospholipid syndrome
  • high disease activity
41
Q

what are the systemic symptoms of SLE

A 
fever 
weight loss
arthralgia 
rash 
proteinuria 
pleuritis
42
Q

what is the general management for SLE

A

immunosuppression

43
Q

what is required in suspected lupus nephritis

A

renal biopsy

Renal (27 decks)

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