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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

How does dysproteinaemia affect the kidneys?

A

Abnormal circulating Ig reach the glomerulus and cause a variety of lesions in the kidney

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3
Q

What causes myeloma cast nephropathy?

A

Abnormal circulating Ig passes through basement membrane and forms cysts within distal tubular lamina

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4
Q

What causes light chain Fanconi syndrome?

A

Abnormal Ig forms crystals within the cytoplasm of proximal tubules

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5
Q

What is myeloma?

A

Cancer of plasma cells = collections of abnormal plasma cells accumulate in the bone marrow and impairs production of normal blood cells

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6
Q

What are plasma cells?

A

Type of white blood cells responsible for producing antibodies

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7
Q

Why can myeloma cause renal dysfunction?

A

Monoclonal proliferation of paraprotein (abnormal antibody)

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8
Q

What is the classic presentation of myeloma?

A

Back pain and renal failure

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9
Q

What are some symptoms of myeloma?

A

Bone pain, weakness, fatigue, weight loss, recurrent infections

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10
Q

What are some signs of myeloma?

A

Anaemia, hypercalcaemia, renal failure, lytic bone lesions

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11
Q

What are the renal manifestations of myeloma?

A

20-40% present with renal impairment
Glomerular = AL amyloidosis, monoclonal Ig deposition
Tubular = light chain cast nephropathy

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12
Q

What are some miscellaneous renal manifestations of myeloma?

A

Dehydration, hypercalcaemia, contrast, bisphosphonates, NSAIDs

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13
Q

What are the best two investigations for diagnosing myeloma?

A

Protein electrophoresis and Bence Jones protein urine analysis

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14
Q

What investigations can be done to diagnose myeloma?

A

Bloods = serum protein electrophoresis and serum free light chains
Urine = Bence Jones protein
Bone marrow biopsy, skeletal survey, renal biopsy

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15
Q

What is the management for myeloma?

A

Stop nephrotoxics and supportive dialysis
Manage hypercalcaemia = saline +/- bisphosphonates
Chemotherapy and stem cell transplant
Plasma exchange to remove light chains

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16
Q

What is amyloidosis?

A

Deposition of extracellular amyloid (insoluble protein fibrils)

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17
Q

Why does amyloidosis occur?

A

Due to abnormal folding of proteins which then aggregate and become insoluble = causes breakdown of usual degradation pathway for abnormal proteins

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18
Q

How many forms of amyloidosis are there?

A

Over 30 identified proteins = inherited and acquired forms

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19
Q

What are the 4 most common forms of amyloidosis?

A

Primary/light chain (AL)
Secondary/systemic/inflammatory (AA)
Dialysis (Abeta2M)
Hereditary and old age (ATTR)

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20
Q

What is amyloidosis an important differential for?

A

Nephrotic range proteinuria

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21
Q

What is AL amyloidosis?

A

Deposition of abnormal Ig light chains from plasma cells = light chains enter bloodstream and cause amyloid deposits

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22
Q

What are some features of AL amyloidosis?

A

Affects heart, bowel, skin, nerves and kidneys
Typical age is 55-60
Life expectancy of 6 months - 4 years (untreated)

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23
Q

What is AA amyloidosis associated with?

A

Systemic inflammation = production of acute phase protein (serum amyloid A protein)

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24
Q

How common is AA amyloidosis in patients with chronic inflammatory conditions or infections?

A

Develops in 5% = rheumatoid arthritis, psoriasis, IBD, TB, osteomyelitis, bronchiectasis

25
Q

Where in the body does AA amyloidosis affect?

A

Liver, spleen, kidneys and adrenals

26
Q

What are the renal manifestations of amyloidosis?

A

Nephrotic range proteinuria +/- impaired renal function

27
Q

What are the extra-renal manifestations of amyloidosis?

A

Cardiomyopathy, peripheral or autonomic neuropathy, hepatomegaly, splenomegaly, malabsorption

28
Q

What are some basic investigations that can be done for amyloidosis?

A

Urinalysis and uPCR

Blood tests = renal function, inflammatory markers, protein electrophoresis, SFLC

29
Q

What biopsies can be done for amyloidosis?

A

Renal = congo red staining gives apple green birefringence under polarised light (diagnostic)
Can also do abdominal fat pad or rectal biopsy

30
Q

How are SAP scans used to investigate amyloidosis?

A

Scintigraphy with radiolabelled serum amyloid = shows extent of disease

31
Q

What is the aim of amyloidosis treatment?

A

Not curative = aim to reduce further deposition and preserve organ function

32
Q

What are some of the treatments for amyloidosis?

A 
AA = treat underlying cause
AL = immunosuppression (steroids, chemotherapy, stem cell transplant)
33
Q

What type of vasculitis normally affects the kidneys?

A

Small vessel = usually ANCA positive

34
Q

How does vasculitis affect the kidneys?

A

Causes necrotising polyangiitis that affects capillaries, venules and arterioles

35
Q

How does vasculitis present?

A

Usually age 50-70
Vague symptoms = fever, migratory pain, arthralgia, weight loss, anorexia, malaise, may last for weeks to months before specific organ involvement

36
Q

How is vasculitis diagnosed?

A

Urinalysis = active urine, positive for blood/protein
Bloods = raised inflammatory markers, AKI, anaemia
Immunology = ANCA, anti-MPO, anti-PR3
Renal biopsy

37
Q

What types of ANCA are implicated in vasculitis?

A 
pANCA = peri-nuclear (MPO)
cANCA = cytoplasmic (PR3)
38
Q

What is the immunology of some small vessel vasculitis that affect the kidneys?

A 
GPA = 95% ANCA positive (cANCA), 65% have anti-PR3
MPA = 90% ANCA positive (pANCA), 55% anti-MPO positive
39
Q

How is small vessel vasculitis treated?

A

Immunosuppression = steroids, cyclophosphamide, rituximab
Plasma exchange
Supportive = dialysis, ventilation

40
Q

What is systemic lupus erythematous (SLE)?

A

Chronic auto-immune inflammatory disease of unknow origin = 10 times more common in women, age of onset usually 20-30

41
Q

What areas of the body are affected by SLE?

A

Skin, joints, kidneys, lungs, nervous system and serous membranes

42
Q

What races is SLE more severe in?

A

African Americans and Hispanics

43
Q

What are some of the features of SLE?

A

Malar/discoid rash, oral ulcers, photosensitivity, haemolytic anaemia, thrombocytopenia, fever, weight loss

44
Q

What investigations can be done for SLE?

A

Blood tests = raised inflammatory markers, ANA positive, anti-dsDNA positive, low complement levels
Must do urinalysis and renal biopsy

45
Q

What are the differentials for SLE?

A

Sjogren’s syndrome, fibromyalgia, primary anti-phospholipid syndrome, thrombotic micro-angiopathies

46
Q

How common is lupus nephritis in patients with SLE?

A

Up to 50% will have renal involvement at presentation and up to 60% during the course of their disease

47
Q

What is the most common abnormality seen in lupus nephritis?

A

Proteinuria

48
Q

What are class I and II lupus nephritis?

A 
I = minimal mesangial 
II = mesangial proliferation
49
Q

What are class III and IV lupus nephritis?

A 
III = focal proliferation
IV = diffuse proliferation
50
Q

What are class V and VI lupus nephritis?

A 
V = membranous
VI = advanced sclerosing
51
Q

How are class I and II lupus nephritis treated?

A 
I = treat extra-renal manifestations
II = proteinuria >3gm treated with steroids or CNI
52
Q

How are class III and IV lupus nephritis treated?

A

Steroids + cyclophosphamide

Switch to steroids + MMF in 3 months if first combination not working

53
Q

What is the maintenance treatment for class III and IV lupus nephritis?

A

Azathioprine or MMF + low dose steroids for 1 year

54
Q

How is a patient with class V lupus nephritis but normal kidney function and non-nephrotic range proteinuria treated?

A

Conservative management and treatment dictated by extra-renal manifestations

55
Q

How is a patient with class V lupus nephritis and persistent proteinuria treated?

A

Steroids + cyclophosphamide and/or CNI or MMF or azathioprine

56
Q

What medication should all patients with lupus be taking?

A

Hydroxychloroquine

57
Q

What is the prognosis of SLE?

A

Most have relapsing/remitting disease course

ESRD affects 8-15% of patients with lupus nephritis

58
Q

What are poor prognostic factors for SLE?

A

Renal disease, male sex, young or older age at presentation, poor socio-economic class, anti-phospholipid syndrome, high overall disease activity

Renal (36 decks)

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