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Systems: MSK AB > Multi-System Auto-Immune Disease > Flashcards

Multi-System Auto-Immune Disease Flashcards

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1
Q

What is the diagnosis for someone presenting with:

  • Mylagia/arthralgia
  • Episcleritis
  • Sinusitis
  • Renal failure
A

Wegner’s granulomatosis

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2
Q

What is the diagnosis for someone presenting with:

  • Mylagia/arthralgia
  • Skin rash
  • Pleurisy
  • CVA
A

Lupus

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3
Q

What is the diagnosis for someone presenting with:

  • Mylagia/arthralgia
  • Skin thickening
  • GORD
  • Pulmonary hypertension
A

Scleroderma

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4
Q

What is the diagnosis for someone presenting with:

  • Mylagia/arthralgia
  • Sicca
  • Skin rash
  • Neuropathy
A

Sjorgen’s syndrome

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5
Q

Give examples of autoimmune connective tissue diseases.

A
  • Systemic Lupus Erythematosus
  • Scleroderma
  • Sjogren’s syndrome
  • Auto-immune myositis
  • Mixed connective tissue disease
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6
Q

Give examples of autoimmune systemic vasculitis?

A
  • Giant cell arteritis
  • Granulomatosis polyangiitis (Wegeners)
  • Microscopic polyangiitis
  • Eosinophilic granulomatosis polyangiitis (Churg-Strauss)
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7
Q

How are multisystem autoimmune diseases diagnosed?

A
  • Cardinal clinical features: History & Exam
  • Immunology
  • Imaging
  • Tissue
  • Exclusion of differential diagnosis
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8
Q

What can mimic multisystem autoimmune diseases?

A
  • Drugs such as cocaine, minocycline, PTU
  • Infections including HIV, endocarditis, Hepatitis and TB
  • Malignancy, in particular lymphoma
  • Cardiac myxoma
  • Cholesterol emboli
  • Scurvey
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9
Q

What is the epidemiology of SLE?

A
  • UK Prevalence: 28/100,000
  • UK incidence: 4/100,000
  • F:M 9:1
  • Onset: 15-50 years
  • Significant ethnic diversity: Afro-Caribbean> Asian> Caucasian
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10
Q

How can SLE affect the skin and hair?

A
  • Butterfly rash
  • Photosensitivity
  • Alopecia
  • Vasculitis
  • Purpura
  • Urticaria
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11
Q

How can SLE affect the nervous system?

A
  • Fits
  • Hemiplegia
  • Ataxia
  • Peripheral neuropathy
  • Cranial nerve lesions
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12
Q

How can SLE affect the heart and chest?

A
  • Pericarditis
  • Endocarditis
  • Aortic valve lesions
  • Pleurisy
  • Pleural effusion
  • Lung fibrosis
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13
Q

How can SLE affect the MSK system?

A
  • Myopathy
  • Aseptic necrosis of the hip
  • Arthritis in small joint
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14
Q

How can SLE affect the blood?

A
  • Anaemia (nomochromic normocytic Coombs test positive)
  • Leukopenia
  • Thrombocytopenia
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15
Q

How can SLE affect a person in general?

A
  • Fever
  • Depression
  • Raynaud’s phenomenon
  • Abdominal pain
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16
Q

What is the classification criteria for SLE?

A

Any 4

  • Malar rash (butterfly rash)
  • Discoid rash (raised, scarring, permanent marks, alopecia)
  • Photosensitivity
  • Oral ulcers
  • Arthritis (2 joints at least)
  • Serositis (pleurisy or pericarditis)
  • Renal (significant proteinuria or cellular casts in urine)
  • Neurological (unexplained seizures or psychosis)
  • Haematological (low WCC, platelets, lymphocytes, haemolytic anaemia)
  • Immunological (anti ds-DNA, SM, cardiolipin, lupus anticoagulant, low complement)
  • ANA
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17
Q

What is the earliest sign of kidney involvement in SLE?

A

Blood and protein on urine dipstick

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18
Q

What is scleroderma characterised by?

A

Thickening of the skin

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19
Q

What is the epidemiology of scleroderma?

A
  • UK Prevalence: 24/100,000
  • UK Incidence: 10/1,000,000
  • Onset: 30-50 years
  • F:M 3:1
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20
Q

What is the name for scleroderma limited?

A

Crest syndrome

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21
Q

What are the features of Crest syndrome?

A
  • Calcinosis
  • Raynaud’s
  • Oesophageal dysmotility
  • Sclerodactyly
  • Telangiectasia
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22
Q

What is a possible complication of limited scleroderma?

A

Pulmonary hypertension

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23
Q

What are the possible outcomes of diffuse scleroderma?

A
  • Pulmonary fibrosis
  • Renal crisis
  • Small bowel bacterial overgrowth
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24
Q

What is the epidemiology of Sjogren’s syndrome?

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A
  • Prevalence: 1 in 100
  • Incidence: 4 in 100,000
  • Onset: 40-50yrs
  • F:M 9:1
25
How can sjogren's syndrome affect the GI system?
- Dysphagia | - Abnormal oesophageal motility
26
How can sjogren's syndrome affect the respiratory system?
Interstitial lung disease
27
How can sjogren's syndrome affect the CNS?
- Fits - Hemiplegia - Ataxia - Cranial nerve lesions
28
How can sjogren's syndrome affect the renal system?
Renal tubular acidosis
29
How can sjogren's syndrome affect the skin?
- Palpable purpura | - Raynaud's syndrome
30
How can sjogren's syndrome affect the joints?
Arthralgia
31
How can sjogren's syndrome affect the PNS?
- Sensory neuropathy | - Mononeuritis multiplex
32
How does sjogren's syndrome present?
- Dry eyes and mouth - Parotid gland enlargement - 1/3 have systemic upset including fatigue, fever, myalgia and arthralgia
33
What are the possible complications of sjogren's syndrome?
- Lymphoma - Neuropathy - Purpura - Interstitial lung disease - Renal tubular acidosis
34
What is the epidemiology of auto-immune myositis?
- Rare | - 6/ million incidence
35
What is auto-immune myositis characterised by?
-Symmetrical, diffuse, proximal muscle weakness
36
What are the 2 forms of auto-immune myositis?
- Polymyositis | - Dermatomyositis
37
How do the populations affected by polymyositis and dermatomyositis differ?
- Polymyositis usually affects younger people | - Dermatomyositis usually affects older people
38
Name 2 skin signs associated with auto-immune myositis.
- Gottron's papules (80%) | - Heliotrope rash (30-60%)
39
What are the possible complications of auto-immune myositis?
- Cancer | - Interstitial lung disease
40
What syndromes does auto-immune myositis overlap with?
Mixed Connective Tissue Disorders - Soft tissue swelling - Raynauds - Myositis - Arthalgia
41
Give examples of large vessel vasculitis.
- Takayasu arteritis | - Giant cell arteritis
42
Give examples of medium vessel vasculitis.
- Polyarteritis nodosa | - Kawasaki disease
43
Give examples of ANCA-associated small vessel vasculitis?
- Microscopic polyangitis - Granulomatosis with polyangitis - Eosionophilic granulomatosis with polyangitis
44
Give examples of immune complex small vessel vasculitis
- Anti GBM disease - Cryoglobulinemic vasculitis - IgA vasculitis (Henoch-Schonlein) - Hypocomplementemic urticarial vasculitis (Anti C1q vasculitis)
45
What is the criteria for giant cell arteritis?
3 of the following - Age at onset ≥50 years - New headache - Temporal artery tenderness/reduced pulsation - ESR≥50 - Abnormal temporal biopsy
46
Give examples of ANCA associated vasculitis.
- Granulomatosis with polyangiitis (Wegener’s) - Microscopic polyangiitis - Eosiniphilic granulomatosis with polyangiitis
47
What is the epidemiology of ANCA associated vasculitis?
- Overall incidence 15/million | - Overall Prevalence 150/million
48
What is the presentation of Wegner's granulomatosis?
- Necrotising granulomatous inflammation - Usually involving the upper and lower respiratory tract - Affecting predominantly small to medium vessels - Necrotising glomerulonephritis is common
49
What is the presentation of microscopic polyangiitis?
- Necrotising vasculitis, with few or no immune deposits, predominantly affecting small vessels. - Necrotising arteritis involving small and medium arteries may be present - Necrotising glomerulonephritis is very common - Pulmonary capillaritis often occurs - Granulomatous inflammation is absent
50
What is the presentation of eosinophilic granulomatosis with polyangiitis
- Eosinophil rich and necrotising granulomatous inflammation often involving the respiratory tract - Necrotising vasculitis predominantly affecting small to medium vessels - Associated with asthma and eosinophilia - ANCA is more frequent when glomerulonephritis is present
51
What conditions is a positive ANA unhelpful?
- RA - MS - Infection
52
What is the specific ANA profile associated with SLE?
- dsDNA - Ro - Sm
53
What is the specific ANA profile associated with scleroderma?
- Scl-70 | - Centromere
54
What is the specific ANA profile associated with polymyositis?
Jo-1
55
What is the specific ANA profile associated with Sjorgren's disease?
- Ro | - La
56
What are the different classes of lupus nephritis on biopsy?
- I: minimal mesangial - II: mesangial proliferative - III: focal - IV: diffuse - V: membranous - VI: advanced sclerosing
57
What is the treatment for mild multi-system autoimmune disease?
Hydroxychloroquine
58
What is the treatment for moderate multi-system autoimmune disease?
- Azathioprine - Methotrexate - Mycophenolate
59
What is the treatment for severe multi-system autoimmune disease?
- Cyclophosphamide | - Rituximab

Systems: MSK AB (43 decks)

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