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RENAL > Systemic Disease > Flashcards

Systemic Disease Flashcards

1
Q

What is the most common systemic disease with renal involvement?

A

Diabetes

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2
Q

What is a dysproteinaemia?

A

Overproduction of immunoglobulin

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3
Q

What type of B cell is responsible for the production of antibodies?

A

Plasma cell

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4
Q

Describe how myeloma affects the production of other blood cells?

A

Clonal expansion of B cells (abnormal)
Make abnormal antibodies
These abnormal cells clog the bone marrow
This blocks normal production of other blood cells

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5
Q

What are the main myeloma symptoms?

A 
Bone Pain
Weakness
Fatigue
Weight Loss
Recurrent infections

must be a differential for anyone with back pain + renal failure

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6
Q

What signs can indicate myeloma?

A

Anaemia
Hypercalcaemia
Renal Failure
Lytic bone lesions

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7
Q

What imaging can be carried out if suspicious of lytic lesions?

A

Skeletal Survey

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8
Q

Why does the renal aspect of myeloma have a multifactorial aetiology?

A

Hypercalcaemia makes pt more prone to stones and impaired renal function

Pt may take NSAIDs for their bone pain and damage their kidneys with the drug

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9
Q

What can be seen histologically in the kidney in Myeloma?

A 
Cast Nephropathy (Myeloma Kidney)
- Free light chains form waxy casts within the tubular lumen
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10
Q

What blood tests should you do if there is a high clinical suspicion of myeloma?

A

Serum Protein Electrophoresis

Serum Free light chains

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11
Q

What should you look for in a patients urine if you suspect myeloma?

A

Bence-Jones Protein

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12
Q

Where would you biopsy if you suspect myeloma?

A

Bone Marrow Biopsy

Renal Biopsy

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13
Q

What management is recommended in myeloma?

A
  • Stop nephrotoxics
  • Manage hypercalcaemia

REFER TO HAEMATOLOGY

  • Chemotherapy
  • Stem cell transplant
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14
Q

What supportive measure is an option in myeloma?

A

Supportive - Dialysis

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15
Q

What is the aim of Plasma exchange in conditions such as myeloma?

A

To remove the excess light chains

however this tx is not completely successful

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16
Q

What is amyloidosis?

A

Deposition of extracellular amyloid (insoluble protein)

in tissues or organs

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17
Q

Why is amyloid insoluble?

A

Protein is folded wrong way
Aggregates
Immune system cant break new protein down

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18
Q

How many different types of protein can cause amyloidosis?

A

30

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19
Q

What are the 4 most common types of amyloidosis?

A

Primary / Light chain (AL)
Secondary / Systemic / Inflammatory (AA)
Dialysis (Previously a problem as Aβ2M wasnt removed during dialysis)
Hereditary and old age (ATTR)

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20
Q

AL amyloidosis involves the deposition of light chains where in the body?

A 
heart
bowel
skin
nerves
kidneys
21
Q

When are patients usually diagnosed with AL amyloidosis, and what is the life expectancy from this point if they were untreated?

A

Age at diagnosis 55-60 years

Life expectancy 6 months – 4 years (untreated)

22
Q

What acute phase protein is produced in AA amyloidosis?

A

serum amyloid A protein (SAA)

23
Q

Patients with chronic inflammatory conditions or infections are more likely to develop AA Amyloidosis. TRUE/FALSE?

A

TRUE
Chronic infl. - RA, IBD, psoriasis

Chronic inf. - TB, osteomyelistis, bronchiectasis

24
Q

Where in the body is most commonly affected by AA Amyloidosis?

A

liver
spleen
kidneys
adrenals

25
Q

How does amyloidosis present?

A

Nephrotic range proteinuria +/- impaired renal function

Then dependent on where is affected:

  • Cardiomyopathy (HEART)
  • peripheral /autonomic neuropathy (NERVES)
  • Hepatomegaly / Splenomegaly (LIVER/SPLEEN)
  • malabsorption (BOWEL)
26
Q

What investigations should be completed if you suspect amyloidosis

A

Urinalysis + PCR

Bloods

  • renal function
  • infl. markers
  • protein electrophoresis (to rule out myeloma)

Renal Biopsy
Congo red staining (apple green under polarised light)

27
Q

If patients are not suitable for a renal biopsy, where else can we take the biopsy from?

A

abdominal fat pad

or rectal biopsy

28
Q

What is a SAP scan?

A

Scintigraphy with radiolabelled serum amyloid
=> shows extent of disease
(kinda looks like a PET scan)

29
Q

What are the aims of amyloidosis tx if there is no cure?

A
  • reduce further deposition

- preserve organ function

30
Q

What treatments can be given for AL Amyloidosis?

A

Immunosuppression
Steroids
chemotherapy
stem cell transplant

31
Q

Where are amyloidosis patients referred to?

A

UCL – National Amyloidosis Centre (London)

32
Q

At what age does an ANCA positive vasculitis usually present?

A

5th, 6th and 7th decade

33
Q

What symptoms do patients usually present with in vasculitis?

A 
fever
migratory arthralgia (different joint each day)
weight loss
anorexia 
malaise
34
Q

How is ANCA positive vaculitis diagnosed with investigations?

A

Urinalysis – blood and protein present

Bloods (raised infl. markers, AKI, anaemia)

Immunology:
ANCA
Anti – MPO and Anti – PR3

Renal Biopsy

35
Q

Describe the typical phenotype of Granulomatosis with Polyangiitis (GPA)?

A

Anti-PR3 antibodies
Necrotising granulomatous inflammation
Lung involvement (pulmonary/renal syndrome)

36
Q

Describe the typical phenotype of Microscopic polyangiitis?

A

Anti-MPO antibodies
Small vessel vasculitis with NO granulomas
renal / lung / skin / GI / nerves

37
Q

Describe the phenotype of Eosinophilic Granulomatosis with Polyangiitis?

A

Associated with asthma and eosinophilia

2/3 have skin involvement

38
Q

How is an ANCA associated vasculitis treated?

A

Immunosuppression
Steroids
Cyclophosphamide / Rituximab

Plasma Exchange

Supportive – Dialysis, Ventilation

39
Q

What are the main parts of the body affected by lupus?

A 
skin
joints
kidneys
lungs
nervous system
serous membranes
40
Q

What groups are most likely to develop lupus?

A

Women in their 20s -30s more commonly affected (10:1)

African Americans, Afro-Caribbean’s and Hispanics

41
Q

What investigations should you carry out if you suspect lupus?

A

Bloods:
- Raised infl. markers

Immunology:

  • ANA
  • anti-dsDNA Ab
  • Complement – low levels in flare

Urinalysis

42
Q

What are the main differentials of SLE?

A

Sjogren’s syndrome
Fibromyalgia
Primary anti-phospholipid syndrome
Thrombotic micro-angiopathies

43
Q

What percentage of lupus patients have renal involvement at diagnosis?

A

50%

44
Q

What percentage of patients with lupus will have renal disease during the course of their life?

A

60%

45
Q

What is the main indicator of lupus neprhitis?

A

proteinuria

46
Q

Lupus can cause many different disease processes in the kidney. How do we differentiate which one a patient has developed?

A

Renal biopsy

47
Q

How is Lupus nephritis classified?

A

Class I - minimal mesangial involvement

Class VI - Advanced sclerosing

48
Q

What can predispose to a poorer prognosis in lupus nephritis?

A
  • male sex
  • extremity of age at presentation
  • poor socio-economic status
  • antiphospholipid syndrome
  • high disease activity

RENAL (24 decks)

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