Multisystem Autoimmune Disease Flashcards

1
Q

List the connective tissue autoimmune diseases

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

List the systemic vasculitis diseases

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

Name the possible mimics

A
  • Drugs: cocaine, minocycline and PTU
  • Infection: HIV, endocarditis, hepatitis and TB
  • Cardiac myxoma
  • Cholesterol emboli
  • Scurvey
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4
Q

Which patient groups are most likely to have SLE?

A
  • Female > male
  • Aged 15-50yrs
  • Afro-caribbean>asian>caucasian
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5
Q

List the classification criteria for SLE (need 4 for diagnosis)

A
  • Malar rash (butterfly)
  • Discoid rash
  • Photosensitivity
  • Oral ulcers
  • Arthritis (at least 2 joints)
  • Serositis (pleurisy or pericarditis)
  • Renal: significant proteinuria or cellular casts in urine
  • Neuro: unexplained seizures or psychosis
  • Haematological: low WCC, platelets, lymphocytes and haemolytic anaemia
  • Immunological: anti ds-DNA, SM, cardiolipin, lupus anticoagulant and low complement
  • ANA (antinuclear antibody)
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6
Q

Which patient groups are most likely to be diagnosed with scleroderma?

A
  • Females > males

- Age: 30-50yrs

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7
Q

What are the features of limited systemic scleroderma?

A
  • Affects skin on the hands, lower arms, feet, lower legs and face
  • Can affect the lungs and GI tract
  • Often starts as Raynaud’s
  • Symptoms: thickening of the skin over the hands, feet and face, red spots on the skin, hard lumps under the skin, heartburn and dysphagia
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8
Q

What are the features of diffuses systemic scleroderma?

A
  • More likely to affect internal organs (can cause SOB, raised BP and pulmonary hypertension)
  • Skin changes can affect the whole body
  • Other symptoms: weight loss, fatigue, joint pain and stiffness
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9
Q

What are the possible complications of systemic scleroderma?

A
  • Limited: pulmonary hypertension

- Diffuse: pulmonary fibrosis, renal crisis and small bowel bacterial overgrowth

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10
Q

Which population groups are more likely to get Sjogren’s syndrome?

A
  • Females > males

- Age: 40-50yrs

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11
Q

What are the clinical features of Sjogren’s syndrome?

A
  • Dry eyes and mouth
  • Parotid gland enlargement
  • 1/3 have systemic upset (fatigue, fever, myalgia and arthralgia)
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12
Q

What are the complications of Sjogren’s syndrome?

A
  • Lymphoma
  • Neuropathy
  • Purpura
  • Interstitial lung disease
  • Renal tubular acidosis
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13
Q

What are the clinical features of auto-immune myositis?

A
  • Muscle weakness: symmetrical, diffuse and proximal
  • Polymyositis
  • Dermatomyositis
  • Gottron’s papsules
  • Heliotrope rash
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14
Q

What are the complications of auto-immune myositis?

A
  • Cancer

- Interstitial lung disease

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15
Q

Which syndromes are mixed connective tissue diseases?

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

List the classification criteria for giant cell arthritis (need 3)

A
  • Age > 50 at onset
  • New headache
  • Temporal artery tenderness/reduced pulsation
  • ESR > 50
  • Abnormal temporal biopsy
17
Q

What are the types of ANCA associated vasculitis?

A
  • Granulomatosis with polyangiitis (Wegener’s)
  • Microscopic polyangiitis
  • Eosiniphilic granulomatosis with polyangiitis
18
Q

What are the features of granulomatosis with polyangiitis?

A
  • Necrotising granulomatous inflammation
  • Usually involves upper and lowe respiratory tract
  • Affects predominantly small to medium vessels
  • Necrotising glomerulonephritis
19
Q

What are the features of microscopic polyangiitis?

A
  • Necrotising vasculitis (small vessels)
  • Necrotising arteritis (small and medium arteries)
  • Necrotising glomerulonephritis
  • Pulmonary capillaritis
  • Granulomatous inflammation is absent
20
Q

What are the features of eosinophilic granulomatosis with polyangiitis?

A
  • Eosinophil rich and necrotising granulomatous inflamm. often involving the respiratory tract
  • Necrotising vasculitis (small to medium vessels)
  • Associated with asthma and eosinophilia
  • ANCA is more frequent with glomerulonephritis is present
21
Q

In which conditions is a positive ANA not helpful?

A
  • RA
  • MS
  • Infection
22
Q

List the classification system for lupus nephritis

A
I: minimal mesangial
II: mesangial proliferative
III: focal
IV: diffuse
V: membranous
VI: advanced sclerosing
23
Q

What are the treatment options for multi-system autoimmune diseases?

A
  • Mild: Hydroxychloroquine
  • Moderate: azathioprine, methotrexate and mycophenolate
  • Severe: cyclophosphamide and rituximab