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Renal > Kideny in systemic disease > Flashcards

Kideny in systemic disease Flashcards

1
Q

Myeloma

A

overproduction of Ig by a cloncal expansion of cells from the b-cell lineage

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2
Q

WHat should happen normally in bone marrow?

A

Antibodies against antigens –> priming the cells to produce Ig

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3
Q

What happens in myelomas?

A

One particular cloncal group that produces abnormal antibodies. No differentiation of the cells

Cancer of the plasma cells

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4
Q

Myeloma features:

A

Cancer of plasma cells

Overproduction of protein and antibodies

collection of abnromal plasma cells in the bone marrow

Impairment of production of normal blood cells

Monoclonal production of a paraprotein (abnormal antibody)
Potentially cause renal dysfunction

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5
Q

Clinical symptoms of myeloma

A

Pain, weakness, fatigue, weight loss, recurrent infection

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6
Q

What is the classic presentation for myeloma?

A

back pain and renal failure

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7
Q

Clinical signs of myeloma

A

Anaemia
Hypercalcaemia
Renal Failure
Lytic bone lesions

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8
Q

Why does myeloma cause renal disease?

A

Multifactorial

  1. glomerular immunoglobulin deposition - blocking it
  2. tubular Ig deposition - light chain cast nephropathy
  3. dehydration/ hypercalcaemia. contrast/ bisphosphonates, NSAIDs
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9
Q

How is myeloma diagnosed?

A

Bloods -

  1. serum protein eletrophoresis (measures the Ig in blood)
  2. serum free light chains

Urine:
1.Bence Jones Protein: dip in urine (can have a negative urinalysis)

Bone Marrow biopsy
Skeletal survey
Renal biopsy

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10
Q

What is the management of myeloma?

A 
Stop nephrotoxics
Manage hypercalcaemia (saline +/- bisphosphonates)

Chemotherapy
Stem cell transplant

Plasma exchange
To remove light chains

Supportive - Dialysis

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11
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 and become insoluble.
Breakdown of usual degradation pathways for abnormally folded proteins

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12
Q

How do you get amyloidosis ?

A

Inherited and acquired forms

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13
Q

What are the 4 types of amyloidosis?

A
  1. Primary / Light chain (AL)
  2. Secondary / Systemic / Inflammatory (AA)
  3. Dialysis (Aβ2M)
  4. Hereditary and old age (ATTR)
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14
Q

Why are the features of 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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15
Q

Who and What does AL amyloidosis affect commonly?

A

55-60 years

heart, bowel, skin, nerves, kidneys

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16
Q

What is AAA amyloidosis?

A

Amyloid associated amyloidosis

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17
Q

Causes of AA amyloidosis?

A

Production of acute phase protein – serum amyloid A protein (SAA)

produced normally but then the body is stimulated continuously and it continuously present

18
Q

Who does AAA present in commonly?

A

In those with chronic inflammatory conditions or chronic infection:

RA, inflammatory bowel disease, psoriasis
TB, osteomyelistis, bronchiectasis

19
Q

What does AAA affect commonly?

A

liver, spleen, kidneys, adrenals

20
Q

What are the presentation of amyloidosis?

A

Renal – (nephrotic range) proteinuria
+/- impaired renal function

Cardiac – Cardiomyopathy
Nerves – peripheral or autonomic neuropathy
Hepatomegaly / Splenomegaly
GI - malabsorption

21
Q

What investigation is done in AAA?

A

Urinalysis + uPCR

Renal biopsy - congo red staining (apple green under polarised light)

Abdominal fat pad or **rectal biopsy

SAP scan - Scintigraphy with radiolabelled serum amyloid – shows extent of diseas

22
Q

What is the treatment for amyloidosis?

A

No cure
reduce deposition and preserve organ function

Innumotherapy, steroids, chemotherapy, stem cell transplant

23
Q

What is small vessel ANCA associated vasculitis?

A

Necrotising polyangiitis that affects capillaries, venules and arterioles

24
Q

When does vasculitis present the most?

A

5th, 6th and 7th decade

60 y/o farmer during springtime

25
Q

What are the constitutional symptoms in vasculitis?

A

fever, migratory arthralgia, weight loss, anorexia and malaise

26
Q

Microscopic polyangitis antibodies

A

anti-MPO(p-ANCA)

27
Q

GPA antibodies

A

anti-PR3 (c-ANCA)

28
Q

Eosinophilic GPA

A

asthma and eosinophilia

29
Q

Highest sensitivity for GPA

A

ANCA

30
Q

Is antibody titre a good marker of disease severity?

A

No

31
Q

How is vasculitis managed?

A

Steroids, cyclophosphamides/ Rituximab

Plasma exchange

supportive - dialysis/ ventilation

32
Q

Why is vasculitis more concentrated in kidneys, lungs and skin?

A

concentration of small blood vessels in these areas

33
Q

What is SLE?

A

autoimmune inflammatory condition

affects everything –> skin, joints, kidneysm lungs, nervous system

34
Q

who does SLE affect the most?

A

women in 20s -30s

African Americans and Hispanics

35
Q

What is the diagnosis of lupus?

A

presence of 4 or more of the following criteria gives 96% sensitivity and specificity for the diagnosis for lupus:

Malar rash
discoid rash
photosensitivity
oral ulcers
non-erosive ulcers
pleuropericarditis
renal disease
nerulogic disease
anaemia
positive LE cell prep, raised Anti-DNA antibody
positive ANA
36
Q

How is the diagnosis of SLE made?

A

Blood tests:
Raised inflammatory markers
Immunology – ANA +ve, anti-dsDNA ab (~70%)
Complement – low levels (sensitive marker)

Urinanlysis - renal involvement is better picked up with it, dip stick as well

37
Q

What are the common differential diagnosis for SLE?

A

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

38
Q

What is lupus nephritis?

A

renal involvement in lupus

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

abnormality - proteinuria

39
Q

How to differentiate types of renal nephritis?

A

Renal biopsy - key investigation

40
Q

What are the classifications of Lupus nephritis?

A

Class I: Minimal mesangial
Class II: Mesangial Proliferative (chalk coloured)

Class III: Focal Proliferative
Class IV: Diffuse Proliferative(more proliferation, more stimulation)

Class V: Membranous

Class VI: Advanced sclerosing

Renal (21 decks)

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