Kidney in systemic disease Flashcards

(48 cards)

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
What is the treatment for AA amyloidosis?
Treat underlying condition
26
What is the treatment for AL amyloidosis?
Immunosuppression Steroids Chemo Stem cell transplant
27
What are the ANCA associated small cell vasculitis?
MPA GPA eGPA Drug induced
28
What are the common immune-complex mediated vasculitis?
``` Henoch-schonlein purpura Anti-GBM Hep B and Hep C Lupus Rheumatoid Behcets ```
29
What are the medium cell vasculitis?
Polyarteritis nodosa | Kawasaki
30
What are the large cell vasculitis?
GCA | Takayasu arteritis
31
When will small cell ANCA associated vasculitis present?
5th 6th and 7th decade
32
How will small ANCA associated vasculitis present?
Constitutional symptoms: fever, migratory arthralgia, weight loss, anorexia, malaise
33
What will be seen on urinalysis of small cell ANCA associated vasculitis?
Active urine: blod ++, protein ++
34
What will be seen in blood of small cell ANCA associated vasculitis?
Raised inflammatory marker AKI Anaemia
35
What immunological markers will GPA show?
c-ANCA | Anti-PR3
36
What immunological markers will MPA show?
p-ANCA | anti-MPO
37
What immunological markers will eGPA show?
p-ANCA | anti-MPO
38
How will GPA present?
Necrotizing granulomatous inflammation resulting in pulmonary haemorrhage and crescentic glomerulonephritis Commonly associated with a saddle nose
39
How will MPA present?
No granulomas | Affects renal, lung, skin, GI and nerves
40
How will eGPA present?
Asthma and eosionphilia
41
How is small cell vasculitis treated?
Immunosuppression: steroids, cyclophosphamide Plasma exchange Supportive - dialysis, ventilation
42
What is SLE?
Chronic auto-immune inflammatory disease | Affects skin, joints, kidneys, lungs, nervous system and serous membranes
43
Who does SLE tend to affect?
Young women | African americans and hispanics
44
What blood tests will confirm SLE?
Raised inflammatory markers High ANA and anti-dsDNA Low complement
45
What is the differential of SLE?
Sjogren's Fibromyalgia Primary antiphospholipid syndrome Thrombotic microangiopathies
46
What is the most frequents observed abnormality in lupus nephritis?
Proteinuria
47
What is required in lupus nephritis?
Biopsy to stage it
48
What is the management of SLE?
ALL on hydroxychloroquine | Immunosuppression depending on severity