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Year 3: Block 1: MSK system > Multi-system autoimmune disease > Flashcards

Multi-system autoimmune disease Flashcards

1
Q

Examples of connective tissue disease (4)

A

systemic lupus erythematosus
systemic sclerosis
sjogre’s syndrome
auto-immune myositis

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2
Q

Examples of systemic vasculitides

A
  • giant cell arteritis
  • granulomatosis with polyangiitis
  • microscopic polyangitis
  • eosinphillic granulomatosis
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3
Q

Common presenting symptoms

A

myalgia/arthalgia

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4
Q

Approach to multi system inflammatory disease (6)

A
  • Cardinal clinical features:
  • bedside investigations - o2, bp, temperature, tracheal analysis
  • History & Exam
  • Immunology - lab tests
  • imaging
  • tissue diagnosis - biopsy , skin, renal, lung, temporal artery
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5
Q

possible systemic autoimmune rheumatic disease - mimics/differential diagnosis

A

Drugs - cocaine, minocyline, PTU
Infection - HIV, endocarditis, Hepatitis B and C, TB
Malignancy - lymphoma

Cardiac myxoma
Cholesterol emboli
Scurvey

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6
Q

Minocyline can induce

A

lupus

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7
Q

Systemic lupus erythematosus (SLE) - what is this

A

multi system disorder - encompasses almost all organs and tissues

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8
Q

mallar rash in SLE?

A

across the nasal bridge and cheeks, spares the nasal folds

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9
Q

SLE is more common in?

when is the onset?

A

male

- 15-50 years

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10
Q

Aetiology of SLE (3)

A

genetic and environmental components

- hormonal components (prolactin) - modulate incidence of SLE

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11
Q

Examples of environmental factors for SLE

A

UV light
drugs
infections

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12
Q

Examples of genetic factors for SLE (3)

A

high rate in monozygotic twins

  • sibling risk
  • polygenic mode of inheritance
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13
Q

SLE: pathogenesis (4)

  • describe the process from autoantigens to cytokines
A
  • immune response against endogenous nuclear antigens

(- autoantigenes - related by apoptotic cells - T cell
- Activated T cells help B cells produce autoantibodies - Cytokine)

  • immune complex formation
  • complement activation
  • tissue injury
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14
Q

SLE: clinical presentation (many examples)

A

varied

  • alopecia
  • neuro involvement - Pn, CNL, ataxia, fits
  • rash
  • photosensitivity
  • pleurisy, pleural effusion, fibrosis
  • pericarditis, endocarditis,
  • abdo pain
  • Raynaud’s
  • arthritis in small joints, aseptic necrosis of the hip and the knee
  • myopathy
  • blood - leukopenia, anaemia
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15
Q 
Classification criteria (any 4 of 11) 
- what would you look for
A

1) Malar rash (butterfly rash)
2) Discoid rash (raised, scarring, permanent marks, alopecia) - keratotic scaling in centre
3) Photosensitivity
4) Oral ulcers
5) Arthritis (2 joints at least)
6) Serositis (pleurisy or pericarditis)
7) Renal (significant proteinuria or cellular casts in urine) 0.5g per 24hr
8) Neurological (unexplained seizures or psychosis)
9) Haematological (low WCC, platelets, lymphocytes, haemolytic anaemia)
10) Immunological (anti ds-DNA, SM, cardiolipin, lupus anticoagulant, low complement)
11) ANA

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16
Q

arthritis with SLE

A

must involve 2 joints
(charcot’s arthroy)

bilateral symmetrical involvement
non-erosive

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17
Q

Kidney with SE - should check (lupus nephritis)

A

Urinalysis for proteunira and haematuria

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18
Q

classes of lupus nephritis

A 
I- minimal mesangial
II - mesnagial proliferation 
III- focal
IV - diffuse 
V - membranous
VI - advanced sclerosing
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19
Q

When to consider diagnosis of SLE (women)

A

women of childbearing age - present with fever, weight loss fever, severe fatigue, malaria rash, stomatitis photosensitivity

  • mouth ulcers
  • arthritis (joint pain)
  • pleuritic chest pain
  • dip stick for protein and blood
  • cytopenia
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20
Q

Autoantibodies in SLE

A

ANA (antinuclear antibodies)

  • high specificity 99% in SLE
  • seen in inflammatory, infectious and neoplastic diseases
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21
Q

Specific Autoantibodies in SLE

A

Anti- D’s DNA

Anti- Sm

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22
Q

Autoantibody with strongest association with nephritis

A

anti-d’s DNA

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23
Q

other antibodies in lupus (2) - what are they associated with?

A

ANTI- Ro

  • fetal congenital heart block (monitoring in pregnancy)
  • neonatal lupus

antiphospholoipid antibodies

  • associated with miscarriages
  • venous/areterial thrombus
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24
Q

antiphospholoipid antibodies associated with arterial/venous thrombosis?

A

anti-cardiolipin, lupus anticoagulant

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25
Q

Scleroderma - defintion

onset??

A

fibrosis of the skin and internal organs, alterations in microvasculature and cellar immunity

30-50 years

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26
Q

Classification of scleroderma (2)

what is the difference?

A
  • localised scleroderma
  • systemic sclerosis
  • CREST, diffuse cutaneous
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27
Q

what is the difference between localised scleroderma and systemic sclerosis ?

A

localised scleroderma - restricted to fibrotic skin and subcutaneous tissue

  • systemic = ALSO affects the internal organs
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28
Q

Systemic sclerosis affects

A

internal organs

29
Q

CREST - limited cutaneous SS is defined by?

A

skin involvement distal to the elbows and knees

30
Q

Diffuse Cutaneous SS is denied by?

A

skin thickening proximal to knees and elbows

31
Q

Aetiology of scleroderma

A

Environmental
- silica, solvents, viral infections

  • genetic predisposition
  • pathogenesis
    (vascular damage, inflammation, fibrosis)
32
Q

Scleroderma: limited - management/ clinical signs

A

long and careful follow up
- whitening of the fingers, rash, blood vessels

gangrene changes , can trigger soft tissue infections

33
Q

Scleroderma: limited - management/ clinical signs

Complications?

A

long and careful follow up
- whitening of the fingers, rash, blood vessels

gangrene changes , can trigger soft tissue infections

  • pulmonary hypertension
  • GI involvement
34
Q

Complications of Scleroderma: Diffuse

A

fast progression of skin sclerosis in weeks/months with int organ involvement - lungs

  • rapid interstitial lung disease

Pulmonary fibrosis
Renal crisis
Small bowel bacterial overgrowth

35
Q

Scleroderma: limited - autoantibody?

A

anti- centromere antibodies

- present with late complications

36
Q

Scleroderma: diffuse - autoantibody - what does it lead to clinically (3)

A 
anti - Scl70
- pul fibrosis
- renal crisis 
- small bowel bacterial overgrowth (inflammation and fibrosis - malabsorption)
(abdo bloating, weight loss)
  • GAVE - dilation of small BV in mucosa
37
Q

Scleroderma: diffuse -treatment

A

ace inhibitors
- should not get high doses of steroids

abrupt onset of mod /severe hypertension - AKI
- bland urinalysis

38
Q

Sjogren’s syndrome is characterised by?

A

lymphocytic infiltrates in the salivary and tear glands

  • oral dryness
39
Q

Sjogren’s syndrome more common in

A

females (age peak after menopause)

40
Q

Sjogren’s syndrome symptoms (6/7)

A 
Dry eyes and mouth
Parotid gland enlargment
1/3 have systemic upset
fatigue
fever
myalgia
arthalgia
41
Q

Sjogren’s syndrome - antibodies?

A

Anti RO anti La antibodies

42
Q

Classification of Sjogren’s syndrome (3)

A
  • ocular dryness for at least 3 months (sensation of sand in the eye)
  • oral dryness
  • swollen glands in the neck
43
Q

Sjogren’s syndrome : complications (5)

A
  • Lymphoma
  • Neuropathy
  • Purpura
  • Interstitial lung disease
  • Renal tubular acidosis - monitor electrolytes
44
Q

Auto-immune myositis are?

A

rare diseases

45
Q

Auto-immune myositis includes polymyositis and dermatomyositis - describe the epidemiology (4)

A

Rare
more common in females
peal incidence in 50-60 yo
- increased risk of malignancy

46
Q

Auto-immune myositis - features

  • what is the predominant symptom and what is its distribution?
A

Muscle Weakness - symmetrical, diffuse, proximal

Polymyositis
Dermatomyositis
Gottron’s papules (80%) - purple link - over hands
Heliotrope rash (30-60%)

47
Q

antibodies associated with auto-immune myositis?

what condition are patients at risk of getting with this antibody

A

anti Jo1 antibodies

interstitial lung disease

48
Q

overlap syndrome of missed connective tissue disease - what do they present with (4)

A
  • raynaud’s
    soft tissue swelling/sclerodactyly
  • myositis
  • arthralgia
49
Q

The vasculitides - large vessel - - what are the 2 types

A

Takayasu arteritis - asian popualtions

  • GCA- older patients
50
Q

The vasculitides - medium vessel - - what are the 2 types

A

in children

Polyarteritis Nodosa
Kawasaki disease

51
Q

small vessel vasculitis - divided into?

A

small vessel involvement with immune complex deposition

  • hyeprsensitivity
  • IgA vascualtis
52
Q

Giant cell arteritis is common in?

A

patients above 70 year of age
(50-70)
more commonly in women

53
Q

Giant cell arteritis - classification criteria (5)

A

Age at onset ≥50 years

New headache (can occur in all areas) - continuous pain, especially at night

Temporal artery tenderness/reduced pulsation

ESR≥50

Abnormal temporal biopsy

– jaw claudication - ischemia of master muscles

  • pian brushing hair/ scalp tenderness
  • other systemic symptoms
54
Q

what will a Abnormal temporal biopsy show? (3)

A

inflammatory infiltrate in artery wall

  • intermal hyperplasia
  • luminal occlusion
55
Q

Some patients in GTA will present with?

A

large vessel involvement

- systemic symptoms

56
Q

Investigation of GCA? (5)

A
  • temporal artery biopsy
  • ultrasound doppler (halo sign)
  • CT angiogram - identify evidence of stenosis or aneurysm
  • MR angiogram
  • FDG PET
57
Q

GCA - COMPLICATIONS

A
  • irreversible visual loss
  • aortic aneurysms
  • arterial stenosis and limb ischemia
  • stroke
58
Q

GCA - treatment

A

high dose steroids straight away - prednisolone

  • 40-60mg per day
  • PPI
  • bone protection
  • steroid sparing medication
59
Q

ANCA associated vasculitis - why is it important (small vessel) - give 3 main examples

A

used to very high mortality, is now a chronic disease = good survival rate

Granulomatosis with polyangiitis (Wegener’s)
Microscopic polyangiitis
Eosiniphilic granulomatosis with polyangiitis

60
Q

Granulomatosis with polyangiitis (GPA) (Wegener’s) - what is this?

what does it typically involve?
what vessels does it effect?

A

Necrotising granulomatous inflammation
Usually involving the upper and lower respiratory tract
Affecting predominantly small to medium vessels
Necrotising glomerulonephritis is common

61
Q

Symptoms of Granulomatosis with Polyangiitis (Wegener’s) (GPA)

A 
otitis medium
hearing loss
sinusitis 
nasal crusting
epistaxis, hemoptysis  
- collapsing of nasal bridge
62
Q

What us common with GPA

- what antibodies will they have?

A

Necrotising glomerulonephritis is common

ENT symtoms - cANCA, anti PR3, urine protein

63
Q 
Microscopic polyangiitis (MPA) - what is it ?
affects?

What is often absent?

A

Necrotising vasculitis, with few or no immune deposits, predominantly affecting small vessels.

  • Necrotising glomerulonephritis is very common
    Pulmonary capillaritis often occurs
    Granulomatous inflammation is absent
64
Q

Microscopic polyangiitis (MPA) - patients present with?

what antibodies? (2)

A

pulmonary renal syndrome, renal failure, positive line dipstick

pANCA, anti MPO

65
Q

Eosinophilic Granulomatosis with Polyangiitis (Churg Strauss) (EGPA) - patients will have? late onset of what conditons?

A
  • Eosinophil rich and necrotising granulomatous inflammation often involving the respiratory tract
  • late onset asthma, nasal polyps, eosinophilia
66
Q

ANCA is more frequent when

A

glomerulonephritis is present

67
Q

Eosinophilic Granulomatosis with Polyangiitis - involvement of other systems ?

A

neurological

cardiac, gastrointestinal - poor prognosis

68
Q

You should avoid this drug in treating SLE

A

steroids

Year 3: Block 1: MSK system (22 decks)

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