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Mini Symposium: Multisystem Autoimmune Disease Flashcards

1
Q

Name some connective tissue diseases?

A
  • Systemic Lupus Erythematosus
  • Systemic sclerosis
  • Sjogren’s syndrome
  • Auto-immune myositis
  • Mixed connective tissue disease
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2
Q

Name some systemic vasculitides?

A
  • Giant cell arteritis
  • Granulomatosis with polyangiitis (Wegeners)
  • Microscopic polyangiitis
  • Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)
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3
Q

What is the approach to diagnosing a patient with multisystem inflammatory disease?

A 
•Cardinal clinical features: history & examination
•Bedside investigations 
•Immunology
•Imaging
•Tissue diagnosis
Exclusion of differential diagnoses
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4
Q

What are some mimics of multi system inflammatory disease?

A
  • Malignancy - lymphoma
  • Infections - endocarditis, hepatitis B and C, TB, HIV
  • Drugs - cocaine, Minocyline, Propylthiouracil

Rarer:
•Cardiac myxoma
•Cholesterol emboli
•Scurvey

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5
Q

Who gets SLE?

A
  • Female:Male 9:1
  • Onset - 15-50 years
  • Afro-Caribbean>Asian>Caucasian
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6
Q

What are the genetic factors that cause SLE?

A
  • High concordance in monozygotic twins
  • Sibling risk for developing SLE is 30-fold higher than in the general population
  • Polygenic mode of inheritance
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7
Q

What are the environmental factors that cause SLE?

A
  • UV light
  • Drugs
  • Infections
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8
Q

What other factors may cause SLE?

A

Hormonal

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9
Q

Describe the pathogenesis if SLE

A
  1. Immune response against endogenous nuclear antigens (break in immunological tolerance)
  2. Immune complex formation
  3. Complement activation
  4. Tissue injury
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10
Q

What is the classification criteria for lupus?

A

4 of the following:

1) Malar rash (butterfly rash)
2) Discoid rash (raised, scarring, permanent marks, alopecia)
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)
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 (anti-nuclear antibody)

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11
Q

How is kidney involvement classed in SLE?

A 
•Lupus nephritis important cause of morbidity and mortality
•Renal involvement can be single organ involvement 
I - minimal mesanginal
II - mesanginal proliferative
III - focal
IV - diffuse
V - membranous
VI - advanced sclerosing
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12
Q

When to consider a diagnosis of SLE?

A
  • Usually seen in women of childbearing age with
  • Constitutional symptoms of fever, weight loss, malaise, and severe fatigue
  • Skin rash and/or stomatitis (mouth and lips)
  • Arthritis
  • Pleuritic chest pain
  • Renal disease
  • Cytopenia
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13
Q

What are the autoantibodies seen in SLE?

A
  • ANA
  • Anti-ds DNA
  • Anti-Sm (Smith)
  • Anti-Ro
  • Antiphospholipid antibodies
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14
Q

What percentage of SLE cases have a positive ANA?

A

95%

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15
Q

What makes ANA testing less useful in diagnosing SLE?

A
  • Not specific for SLE
  • Seen in many inflammatory, infectious, and neoplastic diseases
  • Seen in 5% to 15% of healthy population
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16
Q

What percentage of SLE cases are positive for anti-ds (double-stranded) DNA?

A
  • 60%

* Highly specific for SLE

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17
Q

What is anti-ds DNA closely associated with?

A

Nephritis

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18
Q

What percentage of SLE cases are positive for anti-sm (Smith)?

A
  • 10-30%

* Highly specific for SLE

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19
Q

What is the clinical significance of being positive for anti-Ro autoantibody?

A
  • Risk of foetal congenital heart block
  • Neonatal lupus
  • Foetal cardiac monitoring from 16weeks onwards
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20
Q

What is the clinical significance of being positive for antiphospholipid antibodies?

A
  • Arterial/venous thrombosis

* Miscarriages

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21
Q

What types of antiphospholipid antibodies are relevant to SLE?

A
  • Anti-cardiolipin

* Lupus anticoagulant

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22
Q

What is systemic sclerosis also known as?

A

Scleroderma (fibrosis of skin and internal organs)

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23
Q

How can scleroderma be classified?

A

•Localised scleroderma
•Systemic sclerosis (SSc):
-Limited cutaneous systemic sclerosis (CREST)
-Diffuse cutaneous systemic sclerosis

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24
Q

What is localised scleroderma limited to?

A

Fibrotic involvement of the skin and subcutaneous tissue - should be reassured no risk of systemic involvement

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25
Q

How are limited cutaneous systemic sclerosis (CREST)

and diffuse cutaneous systemic sclerosis defined?

A
  • Limited - skin involvement distal to knees and elbows (slow progression)
  • Diffuse - skin thickening proximal to knees and elbows and trunkal involvement (fast progression with organ involvement - usually lungs)
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26
Q

Who gets scleroderma?

A
  • UK Prevalence: 24/100,000
  • UK Incidence: 10/1,000,000
  • Onset: 30-50 years
  • Female : Male 3:1
27
Q

What is the aetiology of scleroderma?

A 
•Unknown
•Possible environmental factors:
-Silica
-Solvents
-Viral infections
•Genetic predisposition
28
Q

What is the pathogenesis of scleroderma?

A
  • Vascular damage (microcirculation)
  • Immune system activation/Inflammation
  • Fibrosis
29
Q

What are the antibodies associated with limited cutaneous systemic scerosis?

A

•Anti centromere antibodies

30
Q

What are the antibodies associated with diffuse cutaneous systemic scerosis?

A

•Anti Scl70 antibodies

31
Q

What are the systemic involvements associated with limited cutaneous systemic scerosis?

A
  • Pulmonary hypertension

* Gastrointestinal

32
Q

What are the systemic involvements associated with diffuse cutaneous systemic scerosis?

A
  • Pulmonary fibrosis - leading cause for mortality and morbidity
  • Renal crisis
  • Small bowel bacterial overgrowth
33
Q

How should scleroderma not be treated?

A

•Not high doses of steroids (max 20mg prednisolone/day) due to potential renal crisis

34
Q

Who gets Sjorgen’s syndrome?

A
  • Prevalence: 1 in 100
  • Incidence: 4 in 100,000
  • Onset: 40-50yrs
  • Female : Male 9:1
35
Q

What are the symptoms of Sjorgen’s?

A
  • Dry eyes and mouth
  • Systemic autoimmune disease characterised my lymphocytic infitrate or salivary and tear glands
  • Can be primary or secondary to other autoimmune disease
36
Q

What are the investigations used to diagnose Sjorgen’s? (what antibodies are tested for?)

A

•Antibody testing:
-Anti Ro (SSA)
-Anti La (SSB) antibodies
Salivary gland biopsy (taken from lower lip) - should show inflammation

37
Q

What else can cause dry eyes and mouth?

A
  • Menopause
  • Radiotherapy
  • Infections
  • Contact lenses
38
Q

How long do dry eyes need to be present for to diagnose with Sjorgen’s?

39
Q

What are the clinical signs of Sjogren’s?

A 
•Parotid gland enlargement
•1/3 have systemic upset:
-fatigue
-fever
-myalgia
-arthralgia
-dry skin
40
Q

What are the complications of Sjorgen’s (5)?

A
  • Lymphoma
  • Neuropathy
  • Cutaneous vasculitis
  • Interstitial lung disease
  • Renal tubular acidosis
41
Q

What are the complications of Sjorgen’s (5)?

A
  • Lymphoma
  • Neuropathy
  • Cutaneous vasculitis
  • Interstitial lung disease
  • Renal tubular acidosis
42
Q

How common are autoimmune myopathies?

43
Q

What is the epidemiology of polymyositis and dermatomyositis?

A
  • Rare: 6/million incidence
  • More common in females (ratio 3:1)
  • Peak incidence is in 50– 60 year olds
  • Increased risk of malignancy
44
Q

What are the signs and symptoms of myositis?

A

•Muscle weakness - proximal and symmetrical (predominant symptom)
•Cutatneous involvement:
-Gottron’s papules (80%)
-Heliotrope rash (30-60%)

45
Q

What investigations are used to diagnose myositis?

A
  • CK test - should be raised
  • EMG (electromyogram)
  • MRI
  • Muscle biopsy - gold standard
46
Q

What will increase risk of interstitial lung disease in myositis?

A

•Anti Jo1 antibodies

47
Q

What is mixed connective tissue disease?

A

A chronic disorder that has overlapping features of two or more systemic rheumatic disorders

48
Q

How may mixed connective tissue disease present?

A
  • Raynaud’s
  • Soft tissue swelling/sclerodactyly
  • Myositis
  • Arthralgia
49
Q

How are vasculitides classified?

A

Large, medium and small

50
Q

What are the types of large vessel vasculitis (2)?

A
  • Takayasu arteritis - Far East, young women <50

* Giant arteritis - 50+

51
Q

What are the types of medium vessel vasculitis (2)?

A
  • Polyarteritis nodosa - associated with hep B

* Kawasaki disease - children

52
Q

What are the types of small vessel vasculitis (2)?

A

•ANCA-associated vasculitis (without immune complex deposition)
•Immune complex SVV
THE VACULITIDES

53
Q

What is the GCA classification criteria?

A
  • 3 of the following (classic temporal arteritis):
  • Age at onset ≥50 years
  • New headache
  • Temporal artery tenderness/reduced pulsation
  • Jaw claudication - pain on chewing
  • ESR≥50
  • Abnormal temporal biopsy
54
Q

What are the investigations used to diagnose GCA?

A
  • Temporal artery biopsy
  • Ultrasound doppler - halo sign
  • CT and MR angiograms (for large vessel involvement)
  • FDG PET - aortic inflammation
55
Q

What are the complications of GCA?

A
  • Irreversible visual loss
  • Aortic aneurysms
  • Arterial stenosis and limb ischaemia
  • Stroke
56
Q

How is GCA treated?

A
  • Urgent initiation of high dose Prednisolone
  • 40-60mg per day, gradually tapered
  • PPI - proton pump inhibitors
  • Bone protection
  • Steroid sparing medication
57
Q

What are the subtypes of ANCA-associated vasculitis?

A
  • Granulomatosis with polyangiitis (Wegener’s) (62.5%)
  • Microscopic polyangiitis (16.7%)
  • Eosiniphilic granulomatosis with polyangiitis (20.8%)
58
Q

What is the epidemiology of ANCA-associated vasculitis?

A
  • Overall incidence 15/million

* Overall prevalence 150/million

59
Q

How is Granulomatosis with polyangiitis (Wegener’s) characterised?

A
  • Necrotising granulomatous inflammation
  • Usually involving the upper and lower respiratory tract
  • Hearing loss, sinusitis, hemoptysis
  • Necrotising glomerulonephritis is common
  • Subglottic stenosis - silent or strider and voice hoarseness
60
Q

What antibodies are associated with Granulomatosis with polyangiitis (Wegener’s)?

A
  • cANCA

* Anti PR3 antibodies

61
Q

How is Microscopic polyangiitis (MPA) characterised?

A
  • Necrotising vasculitis, with few or no immune deposits, predominantly affecting small vessels
  • Granulomatous inflammation is absent
  • Renal and pulmonary involvement common
  • Seronegative inflammatory arthritis for years before renal involvement
62
Q

What antibodies are associated with Microscopic polyangiitis (MPA)?

A
  • pANCA

* Anti MPO antibodies

63
Q

How is Eosiniphilic granulomatosis with polyangiitis characterised?

A
  • Eosinophil rich and necrotising granulomatous inflammation often involving the respiratory tract
  • Late onset asthma, nasal polyps and eosinophilia
  • Necrotising vasculitis predominantly affecting small to medium vessels
  • Neurological involvement
  • Cardiac, gastrointestinal – poor prognosis
64
Q

What antibodies are associated with Eosiniphilic granulomatosis with polyangiitis?

A

•Anti MPO antibodies positive - 40-60% positive

MSK (28 decks)

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