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MSK > Multisystem autoimmune **marty pls review** > Flashcards

Multisystem autoimmune **marty pls review** Flashcards

1
Q

Give 5 examples of connective tissue diseases:

A
  1. Systemic Lupus Erythematosus
  2. Scleroderma
  3. Sjogren’s syndrome
  4. Auto-immune myositis
  5. Mixed connective tissue disease
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2
Q

Give 4 examples of systemic vasculitis:

A
  1. Giant cell arteritis
  2. Granulomatosis polyangiitis (Wegeners)
  3. Microscopic polyangiitis
  4. Eosinophilic granulomatosis polyangiitis (Churg-Strauss)
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3
Q

How is a diagnosis of systemic disease made?

A
  • Cardinal clinical features: History & Exam
  • Immunology
  • Imaging
  • Tissue
  • Exclusion of differential diagnosis
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4
Q

Systemic Lupus Erythematosus [SLE]: What are it’s epidemiological characteristics?

A
  • Disease of the young
  • 9:1, Female:Male
  • UK incidence: 4/100,000
  • Onset: 15-50 years
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5
Q

What is the classification criteria for SLE?

A

Any 4 of the below:

  • 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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6
Q

What are the specific skin manifestations of SLE?

A
  • DDx butterfly: rosacea, mitral stenosis
  • Discoid: Scaly centre, dark rim
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7
Q

What is scleroderma?

A

Uncommon condition that results in hard, thickened areas of skin and sometimes problems with internal organs and blood vessels

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8
Q

Epidemiology of scleroderma?

A
  • UK Prevalence: 24/100,000
  • UK Incidence: 10/1,000,000
  • Onset: 30-50 years
  • Female:Male 3:1
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9
Q

What are the two main subtypes of scleroderma?

A
  • Limited cutaneus scleroderma: presents with raynauds phenomonen
  • Diffuse cutaneus scleroderma: more likely to have internal organ involvement
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10
Q

What are complications with limited and diffuse scleroderma?

A
  • Limited: Pulmonary hypertension
  • Diffuse: Pulmonary fibrosis, Renal crisis, small bowel bacteria overgrowth
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11
Q

What is sjogren’s syndrome? Epidemiology?

A

Long-term autoimmune disease that affects the body’s moisture-producing glands.

  • Prevalence: 1 in 100
  • Incidence: 4 in 100,000
  • Onset: 40-50yrs
  • Female : Male 9:1
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12
Q

What are common symptoms of sjogren’s syndrome?

A
  • Dry eyes and mouth
  • Parotid gland enlargment
  • 1/3 have systemic upset
    • –fatigue
    • –fever
    • –myalgia
    • –arthalgia
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13
Q

Serious complications of sjogrens?

A
  • Lymphoma
  • Neuropathy
  • Purpura
  • Interstitial lung disease
  • Renal tubular acidosis
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14
Q

What is auto-immune myositis?

A

VERY rare disease

  • Muscle Weakness - symmetrical, diffuse, proximal
    • Polymyositis
    • Dermatomyositis
      • –Gottron’s papules (80%)
      • –Heliotrope rash (30-60%)
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15
Q

What are mixed connective tissue diseases normally inclusive of?

A
  • Soft tissue swelling
  • Raynauds
  • Myositis
  • Arthalgia
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16
Q

What is Giant Cell arteritis?

A

Giant cell arteritis (GCA) is a common form of vasculitis in people aged 50 years or older. The extracranial branches of the carotid artery are usually affected.

17
Q

What is GCA classification criteria?

A
  • 3 of the following
    • Age at onset ≥50 years
    • New headache
    • Temporal artery tenderness/reduced pulsation
    • ESR≥50
    • Abnormal temporal biopsy
18
Q

What is a serious complication of GCS?

A

Irreversible blindness is the most common serious consequenc

19
Q

What are ANCA associated vasculitis?

A

Anti-neutrophil cytoplasmic antibody (ANCA)

These are systemic vasculitis associated with the above:

  1. Granulomatosis with polyangiitis (Wegener’s)
  2. Microscopic polyangiitis
  3. Eosiniphilic granulomatosis with polyangiitis

They are very rare

20
Q

What are the characteristcs of Wegeners?

A
  • Necrotising granulomatous inflammation
  • Usually involving the upper and lower respiratory tract
  • Affecting predominantly small to medium vessels
  • Necrotising glomerulonephritis is common
21
Q

What are the characteristics of Microscopic polyangiitis (MPA)?

A
  • 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
22
Q

What are the features of Eosinophilic Granulomatosis with Polyangiitis (Churg Strauss) (EGPA)?

A
  • 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
23
Q

Marty please look at slides 41-51 and see if useful

A

xoxo

MSK (33 decks)

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