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Renal > Kidney in systemic disease > Flashcards

Kidney in systemic disease Flashcards

1
Q

What is multiple myeloma?

A

Damaged B cells that produce abnormal antibodies
Collections of abnormal plasma cells accumulate in the bone marrow
Impairment of production of normal blood cells

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2
Q

What are the symptoms of multiple myeloma?

A 
Bone pain 
Weakness 
Fatigue 
Weight loss 
Recurrent infections
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3
Q

What are the signs of multiple myeloma?

A

Anaemia
Hypercalcaemia
Renal failure
Lytic bone lesions

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4
Q

Classical presentation of multiple myeloma?

A

50 yr old with back pain and AKI

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5
Q

What are the renal manifestations of myeloma?

A

AL amyloidosis
Monoclonal immunoglobulin deposition
Tubular: light chain cast nephropathy

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6
Q

What is the commonest nephropathy associated with myeloma?

A

Cast nephropathy

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7
Q

What bloods should be taken in suspicion of myeloma?

A

Serum protein electrophoresis

Serum free light chains

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8
Q

What should be analysed in the urine?

A

Bence jones protein

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9
Q

What is the management of myeloma?

A 
Stop nephrotoxics
Manage hypercalcemia (saline +/- bisphosphonates) 
Chemo
Stem cell transplant 
Plasma exchange - remove light chains 
Dialysis
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10
Q

What is amyloidosis?

A

Deposition of extracellular amyloid (insoluble protein fibrils) in tissues or organs
Occurs due to abnormal folding of proteins which then aggregate

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11
Q

What are the 2 commonest types of amyloidosis?

A

Primary/ light chain (AL)

Secondary/ systemic/ inflammatory (AA)

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12
Q

What causes AL amyloidosis?

A

Production of abnormal immunoglobulin light chains from plasma cells
Light chains enter the bloodstream and cause amyloid deposits

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13
Q

Where will AL amyloidosis commonly affect?

A 
Heart
Bowel
Skin
Nerves
Kidneys
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14
Q

What is AA amyloidosis associated with?

A

Systemic inflammation - production of acute phase protein:

RA, IBD, psoriasis, TB, osteomyelitis, bronchiectasis

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15
Q

Where will AA amyloidosis commonly affect?

A

Liver
Spleen
Kidneys
Adrenals

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16
Q

What is the pathogenesis of AL protein amyloidosis?

A

Unknown trigger
B cell proliferation
Immunoglobulin light chains misfolded
Resulting in limited proteolysis and AL protein

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17
Q

What is the pathogenesis of AA protein amyloidosis?

A 
Chronic inflammation 
Macrophage activation
IL-1 and IL-6 
Liver cells
SAA protein 
Limited proteolysis 
AA protein
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18
Q

What is the presentation of renal amyloidosis?

A

Nephrotic syndrome with proteinuria +/- impaired renal function

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19
Q

What is the presentation of cardiac amyloidosis?

A

Cardiomyopathy

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20
Q

What is the presentation of nerve amyloidosis?

A

Peripheral or autonomic neuropathy

21
Q

What is the presentation of GI amyloidosis?

A

Hepatomegaly
Splenomegaly
Malabsorption

22
Q

When should you suspect amyloidosis?

A

Chronic autoimmune condition who presents with new heavy proteinuria

23
Q

How should renal amyloidosis be investigated?

A

Urinalysis + uPCR
Blood tests - renal function, markers of inflammation, protein electrophoresis
Renal biopsy
SAP scan

24
Q

What staining wil show amyloidosis?

A

Congo red staining which shows apple green under polarised light

25
Q

What is the treatment for AA amyloidosis?

A

Treat underlying condition

26
Q

What is the treatment for AL amyloidosis?

A

Immunosuppression
Steroids
Chemo
Stem cell transplant

27
Q

What are the ANCA associated small cell vasculitis?

A

MPA
GPA
eGPA
Drug induced

28
Q

What are the common immune-complex mediated vasculitis?

A 
Henoch-schonlein purpura
Anti-GBM 
Hep B and Hep C 
Lupus 
Rheumatoid
Behcets
29
Q

What are the medium cell vasculitis?

A

Polyarteritis nodosa

Kawasaki

30
Q

What are the large cell vasculitis?

A

GCA

Takayasu arteritis

31
Q

When will small cell ANCA associated vasculitis present?

A

5th 6th and 7th decade

32
Q

How will small ANCA associated vasculitis present?

A

Constitutional symptoms: fever, migratory arthralgia, weight loss, anorexia, malaise

33
Q

What will be seen on urinalysis of small cell ANCA associated vasculitis?

A

Active urine: blod ++, protein ++

34
Q

What will be seen in blood of small cell ANCA associated vasculitis?

A

Raised inflammatory marker
AKI
Anaemia

35
Q

What immunological markers will GPA show?

A

c-ANCA

Anti-PR3

36
Q

What immunological markers will MPA show?

A

p-ANCA

anti-MPO

37
Q

What immunological markers will eGPA show?

A

p-ANCA

anti-MPO

38
Q

How will GPA present?

A

Necrotizing granulomatous inflammation resulting in pulmonary haemorrhage and crescentic glomerulonephritis
Commonly associated with a saddle nose

39
Q

How will MPA present?

A

No granulomas

Affects renal, lung, skin, GI and nerves

40
Q

How will eGPA present?

A

Asthma and eosionphilia

41
Q

How is small cell vasculitis treated?

A

Immunosuppression: steroids, cyclophosphamide
Plasma exchange
Supportive - dialysis, ventilation

42
Q

What is SLE?

A

Chronic auto-immune inflammatory disease

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

43
Q

Who does SLE tend to affect?

A

Young women

African americans and hispanics

44
Q

What blood tests will confirm SLE?

A

Raised inflammatory markers
High ANA and anti-dsDNA
Low complement

45
Q

What is the differential of SLE?

A

Sjogren’s
Fibromyalgia
Primary antiphospholipid syndrome
Thrombotic microangiopathies

46
Q

What is the most frequents observed abnormality in lupus nephritis?

A

Proteinuria

47
Q

What is required in lupus nephritis?

A

Biopsy to stage it

48
Q

What is the management of SLE?

A

ALL on hydroxychloroquine

Immunosuppression depending on severity

Renal (22 decks)

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