Connective Tissue Diseases Part 2 Flashcards

1
Q

What are some general symptoms of connective tissue diseases?

A

Dry eyes and mouth, Sjogren’s (Sicca) syndrome, poor dental health, mouth ulcers, changes in appearance, skin changes/rashes, Raynaud’s, myalgia, arthralgia

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2
Q

What are some symptoms of complications caused by connective tissue diseases?

A

SOB, chest pain, GORD, dysphagia, fatigue, tiredness, pain

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3
Q

What are the symptoms of Sjogren’s syndrome?

A

Dry eyes (gritty feeling), dry mouth, vaginal dryness, bilateral parotid gland enlargement, joint pains, fatigue, unexplained increase in dental caries

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4
Q

What antibodies are involved in Sjogren’s syndrome?

A

Anti-Ro, Anti-Jo

May also have raised IgG and PV/ESR

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5
Q

What are some complications of Sjogren’s syndrome?

A

Increased risk of lymphoma, less commonly associated with organ complications than other CTDs

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6
Q

What is systemic sclerosis (scleroderma)?

A

Multi-system autoimmune disease characterised by vasculopathy, auto-immunity and fibrosis

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7
Q

What are the classic symptoms of systemic sclerosis?

A

Raynaud’s, skin thickening, dysphagia, GORD, telangiectasia, calcinosis, +/- SOB

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8
Q

What are red flag symptoms in systemic sclerosis?

A

Onset of Raynaud’s in mid-adulthood
Raynaud’s and SOB/telangiectasia
Digital ulcers/ischaemia
Skin tightness/loss of dexterity of hands

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9
Q

How is the skin involved in diffuse cutaneous systemic sclerosis?

A

Skin involvement on extremities above and below elbows and knees (plus face and trunk)

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10
Q

How is the skin involved in limited cutaneous systemic sclerosis?

A

Skin involvement on extremities and only below elbows and knees (and face)

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11
Q

What antibodies are involved in systemic sclerosis?

A
Anticentromere = 20-30%
Antitopoisomerase (anti-Scl-70)
Anti-PM-Scl
Anti-To/Th
Anti-RNA polymerase (20%)
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12
Q

What facial changes occur in systemic sclerosis?

A

Small mouth with puckering, beaked nose, telangiectasia, thickened/tight skin (lack of wrinkles)

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13
Q

What are some GI complications of systemic sclerosis?

A

Dysphagia, GORD, gastric antral vascular ectasia (watermelon stomach), small intestinal bacterial overgrowth, malabsorption, fluctuating bowel habit, faecal incontinence

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14
Q

What are some cardio and resp complications of systemic sclerosis?

A

Interstitial lung disease, pulmonary arterial hypertension (probably vasculopathy), myocardial disease

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15
Q

What are some renal complications of systemic sclerosis?

A

Scleroderma renal crisis, non-progressive renal dysfunction

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16
Q

What may systemic sclerosis lead to?

A

Sclerodactyly, digital ischaemia, myositis

17
Q

What is the classic triphasic presentation of Raynaud’s?

A

Blanching (white), acrocyanosis (purple/blue), reactive hyperaemia (redness)

18
Q

What are some treatments for Raynaud’s and recurrent digital ulcers (list from Reynaud’s treatments to treatments for ulcers)?

A

Calcium channel blockers = nifedipine usually first line
PDE-5 inhibitor = sildenafil
Prostacyclin infusion = iloprost
Endothelin receptor antagonist

19
Q

What are some drugs used for general Raynaud’s treatment?

A

Fluoxetine, ARBs, nitrates

20
Q

What is the main symptoms of pulmonary hypertension?

A

Predominantly SOB on exertion

21
Q

What are some risk factors for pulmonary hypertension?

A

Telangiectasia, anticentromere antibody, increased disease duration (e.g systemic sclerosis)

22
Q

What are some features of pulmonary hypertension?

A

Must screen for it yearly in at risk patients, progressive complication that can lead to right heart failure

23
Q

How is pulmonary hypertension treated?

A
Aimed at slowing progression
PDE 5 inhibitor = sildenafil/tadalafil/vardenafil
ERA = bosentan/ambrisentan/macitentan 
Eproprostenol infusions
Oxygen
24
Q

How does pulmonary fibrosis present?

A

Progressive SOB, usually associated with a cough, bilateral crackles on chest examination, due to hardening of lung tissue (less pliable and more inefficient)

25
What are the pulmonary function test results for a patient with pulmonary fibrosis?
Restrictive pattern, reduced forced vital capacity, reduced transfer factor, must be done yearly
26
How is pulmonary fibrosis treated?
Mycophenolate mofetil = causes immunosuppression Rarely cyclophosphamide = for very aggressive disease Ritiximab = second line agent Nintedanib = antifibrotic Rarely lung transplant
27
What are some features of systemic sclerosis renal crisis?
Associated with anti-RNA polymerase III antibody, usually early in disease, high dose of steroids puts people at risk
28
What occurs in systemic sclerosis renal disease?
Uncontrolled hypertension with proteinuria and rapidly worsening renal function = treated with ACE inhibitors (sometimes need dialysis)
29
How is skin fibrosis treated?
Methotrexate, mycophenolate