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Kidneys in Systemic Disease Flashcards

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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?

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A

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

25
Where in the body does AA amyloidosis affect?
Liver, spleen, kidneys and adrenals
26
What are the renal manifestations of amyloidosis?
Nephrotic range proteinuria +/- impaired renal function
27
What are the extra-renal manifestations of amyloidosis?
Cardiomyopathy, peripheral or autonomic neuropathy, hepatomegaly, splenomegaly, malabsorption
28
What are some basic investigations that can be done for amyloidosis?
Urinalysis and uPCR | Blood tests = renal function, inflammatory markers, protein electrophoresis, SFLC
29
What biopsies can be done for amyloidosis?
Renal = congo red staining gives apple green birefringence under polarised light (diagnostic) Can also do abdominal fat pad or rectal biopsy
30
How are SAP scans used to investigate amyloidosis?
Scintigraphy with radiolabelled serum amyloid = shows extent of disease
31
What is the aim of amyloidosis treatment?
Not curative = aim to reduce further deposition and preserve organ function
32
What are some of the treatments for amyloidosis?
``` AA = treat underlying cause AL = immunosuppression (steroids, chemotherapy, stem cell transplant) ```
33
What type of vasculitis normally affects the kidneys?
Small vessel = usually ANCA positive
34
How does vasculitis affect the kidneys?
Causes necrotising polyangiitis that affects capillaries, venules and arterioles
35
How does vasculitis present?
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
How is vasculitis diagnosed?
Urinalysis = active urine, positive for blood/protein Bloods = raised inflammatory markers, AKI, anaemia Immunology = ANCA, anti-MPO, anti-PR3 Renal biopsy
37
What types of ANCA are implicated in vasculitis?
``` pANCA = peri-nuclear (MPO) cANCA = cytoplasmic (PR3) ```
38
What is the immunology of some small vessel vasculitis that affect the kidneys?
``` GPA = 95% ANCA positive (cANCA), 65% have anti-PR3 MPA = 90% ANCA positive (pANCA), 55% anti-MPO positive ```
39
How is small vessel vasculitis treated?
Immunosuppression = steroids, cyclophosphamide, rituximab Plasma exchange Supportive = dialysis, ventilation
40
What is systemic lupus erythematous (SLE)?
Chronic auto-immune inflammatory disease of unknow origin = 10 times more common in women, age of onset usually 20-30
41
What areas of the body are affected by SLE?
Skin, joints, kidneys, lungs, nervous system and serous membranes
42
What races is SLE more severe in?
African Americans and Hispanics
43
What are some of the features of SLE?
Malar/discoid rash, oral ulcers, photosensitivity, haemolytic anaemia, thrombocytopenia, fever, weight loss
44
What investigations can be done for SLE?
Blood tests = raised inflammatory markers, ANA positive, anti-dsDNA positive, low complement levels Must do urinalysis and renal biopsy
45
What are the differentials for SLE?
Sjogren's syndrome, fibromyalgia, primary anti-phospholipid syndrome, thrombotic micro-angiopathies
46
How common is lupus nephritis in patients with SLE?
Up to 50% will have renal involvement at presentation and up to 60% during the course of their disease
47
What is the most common abnormality seen in lupus nephritis?
Proteinuria
48
What are class I and II lupus nephritis?
``` I = minimal mesangial II = mesangial proliferation ```
49
What are class III and IV lupus nephritis?
``` III = focal proliferation IV = diffuse proliferation ```
50
What are class V and VI lupus nephritis?
``` V = membranous VI = advanced sclerosing ```
51
How are class I and II lupus nephritis treated?
``` I = treat extra-renal manifestations II = proteinuria >3gm treated with steroids or CNI ```
52
How are class III and IV lupus nephritis treated?
Steroids + cyclophosphamide | Switch to steroids + MMF in 3 months if first combination not working
53
What is the maintenance treatment for class III and IV lupus nephritis?
Azathioprine or MMF + low dose steroids for 1 year
54
How is a patient with class V lupus nephritis but normal kidney function and non-nephrotic range proteinuria treated?
Conservative management and treatment dictated by extra-renal manifestations
55
How is a patient with class V lupus nephritis and persistent proteinuria treated?
Steroids + cyclophosphamide and/or CNI or MMF or azathioprine
56
What medication should all patients with lupus be taking?
Hydroxychloroquine
57
What is the prognosis of SLE?
Most have relapsing/remitting disease course | ESRD affects 8-15% of patients with lupus nephritis
58
What are poor prognostic factors for SLE?
Renal disease, male sex, young or older age at presentation, poor socio-economic class, anti-phospholipid syndrome, high overall disease activity

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