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MSK > Systemic Sclerosis > Flashcards

Systemic Sclerosis Flashcards

1
Q

What is the cause of systemic sclerosis?

A

Unknown

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2
Q

What is systemic sclerosis characterised by?

A

Hardened, sclerotic skin and other connective tissues

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3
Q

Who is more commonly affected by systemic sclerosis?

A

Women (4x)

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4
Q

What are the two different patterns of systemic slerosis?

A

Limited cutaneous systemic sclerosis
Diffuse cutaneous systemic sclerosis

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5
Q

What is a subtype of Limited cutaneous systemic sclerosis?

A

CREST syndrome

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6
Q

What does CREST stand for?

A

Calcinosis
Raynaud’s phenomenon
oEsophageal dysmotility
Sclerodactyly
Telangiectasia

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7
Q

What is typically the first sign of Limitied Cutaneous systemic sclerosis?

A

Raynauds syndrome

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8
Q

What is common in people presenting with Limited cutanoeus systmeic sclerosis?

A

Family history

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9
Q

What antibodies are assoicated with limited cutanoeus systemic sclerosis?

A

Anti-centromere antibodies

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10
Q

Where on the body does scleroderma affect in someone with limited cutaneous systemic sclerosis?

A

Face and distal limbs predominately

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11
Q

What is Sclerodactyly?

A

Bright shiny skin of hands and feet
Fingers can be swollen and painful with reduced mobility and function.
Patients are unable to put palm to palm in prayer sign.

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12
Q

How can diffuse cutaneous systemic sclerosis present?

A

With the same symptoms of CREST and internal organ dysfunction

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13
Q

Which internal organs tend to be affected in someone presenting with diffuse cutaneous systemic sclerosis?

A

Kidneys
Lungs
Heart

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14
Q

What cardiovascular problems can diffuse cutaneous systemic scerosis result in?

A

Hypertension
Coronary artery disease
Pericarditis

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15
Q

What respiratory problems can diffuse cutaneous systemic sclerosis result in?

A

Pulmonary hypertension
Pulmonary fibrosis.

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16
Q

What renal problems can diffuse cutnaeous systemic sclerosis result in?

A

Glomerulonephritis
Scleroderma renal crisis.

17
Q

Which antibodies is diffuse cutaneous systemic sclerosis associated with?

A

Anti scl-70 antibodies

18
Q

What is the most common cause of death in someone presenting with diffuse cutanous systemic sclerosis?

A

Respiratory involvement,
–Interstitial lung disease (ILD)
–Pulmonary arterial hypertension (PAH)

19
Q

What other antibodies will be present in someone presenting with systemic sclerosis?

A

ANA positive in 90%
RF positive in 30%

20
Q

What is the prognosis of diffuse cutaneous systemic sclerosis?

A

Poor

21
Q

What medical management can be given for systemic sclerosis?

A

Nifedipine- Raynauds
ACEi- renal function
Antibiotics- skn infection

22
Q

What medication should patients with signs of renal disease be started on?

A

ACEi

MSK (79 decks)

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