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Mariam MSK > Multi-System Autoimmune Diseases > Flashcards

Multi-System Autoimmune Diseases Flashcards

1
Q

What are examples of connective tissue diseases?

A
  • Systemic lupus erythematous
  • Scleroderma
  • Sjogren’s syndrome
  • Auto-immune myositis
  • Mixed connective tissue disease
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2
Q

What are examples of systremic vasculitis?

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

What is the differential diagnosis for multi-system autoimmune diseases?

A
  • Drugs - cocaine, minocyline, PTU
  • Infection - HIV, endocarditis, Hepatitis, TB
  • Malignancy - lymphoma
  • Cardiac myxoma
  • Cholesterol emboli
  • Scurvey
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4
Q

What does SLE stand for?

A

Systemic lupus erythematous

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5
Q

Describe the epidemiology of SLE in terms of sex, age and ethnicity?

A
  • F:M 9:1
  • Onset 15-50 years (known as disease of the young)
  • Significant ethnic diversity
    • Afro-Caribbean’s > Asian > Caucasian
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6
Q

What organs can be affected by SLE?

A
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7
Q

What is the classification critera for SLE?

A

Classification criteria (any 4):

  • 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
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8
Q

What is scleroderma?

A

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

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9
Q

Describe the epidemiology of scleroderma in terms of sex and age?

A
  • F:M 3:1
  • Onset 30-50 years
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10
Q

What are the different kinds of scleroderma?

A
  • Morphea
    • Isolated patches of hardened skin with no internal organ involvement
  • Limited
    • Skin changes typically occur in lower arms and legs
  • Diffuse
    • More skin involved than limited, such as skin in the trunk is likely to be involved
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11
Q

What are complications of scleroderma?

A
  • Limited
    • Pulmonary hypertension
  • Diffuse
    • Pulmonary fibrosis
    • Renal crises
    • Small bowel bacterial overgrowth
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12
Q

What does Sjogen’s syndrome affect?

A

Glands

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13
Q

Describe the epidemiology of Sjogren’s syndrome in terms of sex and age?

A
  • F:M 9:1
  • Onset 40-50 years
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14
Q

What are the clinical features of Sjogren’s syndrome?

A
  • Two most common symptoms are dry eyes and mouth
  • Parotid gland enlargement
  • Systemic symptoms
    • Fatigue
    • Fever
    • Myalgia
    • Arthalgia
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15
Q

What are potential complications of Sjogen’s syndrome?

A
  • Lymphoma
  • Neuropathy
  • Purpura
  • Interstitial lung disease
  • Renal tubular acidosis
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16
Q

What is acute-immune myositis?

A

Disease involving chronic inflammation that leads to weakening of muscles over time

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17
Q

What are clinical features of auto-immune myositis?

A
  • Muscle weakness
    • Symmetrical, diffuse, proximal
    • Polymyositis or dermatomyositis
      • Gottron’s papules
      • Heliotrope rash
18
Q

What are potential complications of autoimmune myositis?

A
  • Cancer
  • Interstitial lung disease
19
Q

What is overlap syndrome also known as?

A

Known as mixed connective tissue disease (MCTD)

20
Q

What does MCTD stand for?

A

Mixed connective tissue disease

21
Q

What is mixed connective tissue disease characterised by?

A

Characterised by features seen from:

  • SLE
  • Scleroderma
  • Polymyositis
22
Q

What are the clinical features of mixed connective tissue disease?

A
  • Soft tissue swelling
  • Raynauds
  • Myositis
  • Arthalgia
23
Q

What are the different categories of vasculitides?

A
  • Large vessel vasculitis
  • Medium vessel vasculitis
  • Small vessel vasculitis
24
Q

What does GCA stand for?

A

Giant cell arteritis

25
Q

What is giant cell arteritis?

A

Condition where temporal arteries become inflamed

26
Q

What is the classification criteria for giant cell arteritis?

A
  • 3 of the following
    • Age at onset ≥50 years
    • New headache
    • Temporal artery tenderness/reduced pulsation
    • ESR≥50
    • Abnormal temporal biopsy
27
Q

What kind of vasculitis is gian cell arteritis?

A

Large vessel vasculitis

28
Q

What does ANCA stand for?

A

Antineutrophil cytoplasmic antibodies

29
Q

What are antineutrophil cytoplasmic antibodies?

A

Autoantibodies directed against antigens found in the cytoplasmic granules of neutrophils and monocytes

30
Q

ANCA testing is helped to diagnose what conditions?

A
  • Granulomatosis with polyangiitis (Wegener’s)
  • Microscopic polyangiitis
  • Eosiniphilic granulomatosis with polyangiitis
31
Q

What is granulomatosis with polyangiitis also called?

A

Wegener’s disease

32
Q

What is Wegener’s disease?

A

Small artery vasculitis

Necrotising granulomatous inflammation

33
Q

What are clinical features of Wegener’s disease?

A
  • Usually involving upper and lower respiratory tract
  • Affecting predominantly small to medium vessels
  • Necrotising glomerulonephritis is common
34
Q

What does MPA stand for?

A

Microscopic polyangiitis

35
Q

What is microscopic polyangiitis?

A

Disorder causing vasculitis (blood vessel inflammation) leading to organ damage

36
Q

What are clinical features of microscopic polyangiitis?

A
  • Necrotising vasculitis with few or no immune deposits, predominantly affecting small vessels
  • Necrotising arteritis involving small and medium vessels may be present
  • Necrotising glomerulonephritis very common
  • Pulmonary capillaritis
  • Granulomatous inflammation is absent
37
Q

What is eosinophillic granulomatosis with polyangiitis also called?

A

Churg strauss

38
Q

What are the clinical features of Eosinophillic Granulomatosis with Polyangiitis (Churg Strauss)

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
39
Q

What does ANA test detect?

A

ANA test detects antinuclear antibodies (ANA) in blood, immune system makes these to fight infection or in autoimmune disease

40
Q

What are some conditions where ANA test is useful?

A
41
Q

What is the treatment for multi-system autoimmune diseases?

A

Treatment depends on organ threat

Mariam MSK (31 decks)

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