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Y4: Rheumatology > Autoimmune connective tissue disease > Flashcards

Autoimmune connective tissue disease Flashcards

1
Q

What antibodies are associated with polymyositis and dermatomyositis?

A
  • Anti Jo-1
  • Anti Mi-2
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2
Q

What antibody is associated with systemic sclerosis?

A

Anti-Scl70

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3
Q

Describe Polymyositis and dermatomyositis?

A
  • Proximal skeletal and smooth muscle inflammation
    • Autoimmune-mediated
  • Myalgia -> dysphagia, dysphonia, respiratory weakness
  • Skin involvement in dermatomyositis
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4
Q

Clinical features of PM and DM?

A
  • Symmetrical proximal muscle weakness / pain
    • Affect LLs more than ULs
  • Difficulty rising from chair, climbing stairs and lifting
  • Fever, weight loss, fatigue
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5
Q

What should always be done in polymyositis and dermatomyositis?

A

Screen for cancer

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6
Q

Describe Dermatomyositis?

A
  • Myositis plus skin signs:
    • Rash over back and shoulders
      • Discolouration of the eyelid +/- periorbital oedema
    • Nail-fold capillary dilation
    • Gottron’s papules (rough red papules over knuckles)
    • Raynauds
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7
Q

What are the extra-muscular signs in both polymyositis and dermatomyositis?

A
  • Fever
  • Arthralgia
  • Raynaud’s
  • Interstitial lung fibrosis
  • Myocardial involvement
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8
Q

Describe the tests for polymyositis and dermatomyositis?

A
  • Creatine kinase can be raised
  • Muscle biopsy (use MRI to identify abnormal muscle for biopsy)
    • Confirms diagnosis: Fibre necrosis and inflammatory infiltrate
  • Autoantibody associations
    • Anti-Mi2, Anti-Jo1
  • Electromyography (EMG)
    • Can identify non-AI/inflammatory myopathies
  • Screen for malignancy
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9
Q

Namr some differentials for polymyositis and dermatomyositis?

A
  • Drugs
  • Infection (HIV)
  • Rhabdomyolysis
  • Muscular dystrophy
  • Inclusion-body myositis
  • Carcinomatous myopathy
  • Endocrine/metabolic myopathy
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10
Q

What drugs can cause polymyositis and dermatomyositis?

A
  • Statins
  • Colchicine
  • Chloroquine
  • Penicillamine
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11
Q

Describe the mangagement of polymyositis and dermatomyositis?

A
  • Prednisolone PO
  • Methylprednisolone IV
    • If respirsatory/pharyngeal weakness
  • Resistant cases:
    • Methotrexate + mycophenolate
  • Skin predominant disease
    • Hydroxychloroquine
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12
Q

Describe systemic sclerosis?

A
  • AI disorder of connective tissue causing:
    • Fibrosis of skin, organs and vasculature
  • Autoantibodies:
    • ANA, anti-centromere
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13
Q

Clinical features of Systemic sclerosis?

A
  • Skin
    • Non-pitting oedema of fingers, shiny, taut skin
  • Raynaud’s phenomenon
  • Arthralgia, tenosynovitis, muscle weakness
  • Dysphagia, odynophagia
  • Pulmonary hypertension
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14
Q

What is scleroderma?

A
  • Systemic sclerosis without internal organ invovlement
  • Tightening and fibrosis of skin
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15
Q

Describe Limited cutaneous systemic sclerosis?

A
  • Raynauds
  • Scleroderma of face and distal limbs
  • Anti-centromere antibodies
  • A subtype of limited systemic sclerosis is CREST syndrome
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16
Q

Treatment for limited systemic sclerosis?

A
  • Sildenafil
  • Bosentan
17
Q

Describe diffuse skin involvement of systemic sclerosis?

A
  • Can involve the whole body
  • Anti-Scl70 anitbodies in 70%
  • Anti-RNA polymerase in 20%
  • Monitor BP and perform regular echos and spirometry
18
Q

What are the indicators of a poor prognosis in systemic sclerosis?

A
  • Older age
  • High ESR
  • Proteinuria
  • Pumonary HTN
  • Diffuse disease
19
Q

Describe the management of systemic sclerosis?

A
  • No cure
  • Raynaud’s
    • Avoid cold, thick socks/gloves, sildenafil
  • Interstitial lung disease
    • Glucocorticoids + cyclophosphamide
  • Hypertension
    • Regular ACE-i/ARBs
  • Pulmonary HTN
    • Bosentan, heart-lung transplant
20
Q

Describe Primary Sjogren’s syndrome?

A
  • Lymphocytic infiltration of salivary and lacrimal glands
    • => Glandular fibrosis and exocrine failure
21
Q

Risk factors for Primary Sjogren’s syndrome?

A
  • 40-60 years
  • Female
  • HLA-B8/ HLA-DR3
22
Q

Clinical features of Primary Sjogren’s syndrome?

A
  • Keratoconjunctivitis
  • Non-erosive arthralgia
  • Enlarged salivary glands
  • Raynaud’s phenomenon
23
Q

Autoantibodies detected in Primary Sjogren’s syndrome?

A
  • Rheumatoid factor
  • Antinuclear antibody (ANA)
  • Anti-Ro, Anti-La
  • Thyroid autoantibody
24
Q

Describe the diagnosis of Primary sjogren’s syndrome?

A
  • Schirmer tear test
    • Normal is >6mm of wetting
  • Elevated ESR and hypogammaglobulinaemia
  • Positive autoantibody
25
Q

Management of Primary sjogren’s syndrome?

A
  • No disease modifying treatment
  • Tear substitiutes
  • Lubrication for vaginal dryness
  • Hydroxychloroquine for skin and MSK features
26
Q

Read the presentation of Sjogrens syndrome

A
27
Q

Read the presentation of systemic sclerosis

A
28
Q

Presentation of idiopathic inflammatory myopathies?

Polymyositis, dermatomyositis

A
29
Q

Read the case presentation of SLE

A
30
Q

Investigations into dermatomyositis?

A
  • ANA positive
  • Anti-Mi-2
  • Anti-Jo-1
  • SRP antibodies
31
Q

Describe CREST syndrome?

A
  • Subtype of limited cutaneous systemic sclerosis
  • Calcinosis
  • Raynauds
  • oEsophageal dysmotility
  • Sclerodactyly
  • Telangiectasia
32
Q
A

Y4: Rheumatology (19 decks)

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