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RHEUMATOLOGY > Systemic Sclerosis (SSc) > Flashcards

Systemic Sclerosis (SSc) Flashcards

1
Q

What is systemic sclerosis?

A

autoimmune connective tissue disease involving inflammation and fibrosis (hardening or scarring) of the connective tissues, skin and internal organs

Previously known as scleroderma.

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2
Q

What causes systemic sclerosis?

A

Increased fibroblast activity resulting in abnormal growth of connective tissue leading to vascular damage and fibrosis.

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3
Q

What are the two main subtypes of systemic sclerosis?

A
  • Limited cutaneous SSc
  • Diffuse cutaneous SSc
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4
Q

What is another name for limited scleroderma?

A

CREST syndrome

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5
Q

What are the components of CREST syndrome?

A
  • Calcinosis Cutis
  • Raynaud’s phenomenon
  • Esophageal dysmotility
  • Sclerodactyly
  • Telangiectasia
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6
Q

How long do Raynaud’s symptoms usually precede scleroderma in limited scleroderma?

A

Many years - development of PA HTN after a mean of 10 years of symptoms

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7
Q

What characterizes diffuse scleroderma?

A

Sudden onset of skin involvement, proximal to the elbows and knees.

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8
Q

What is the risk associated with diffuse scleroderma?

A

Higher risk of mortality.

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9
Q

What are the typical inflammatory marker results in systemic sclerosis?

A

Usually normal.

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10
Q

What imaging is used to evaluate calcinosis in systemic sclerosis?

A

X-ray hands

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11
Q

What tests are used to assess pulmonary disease in systemic sclerosis?

A
  • CXR
  • HRCT
  • PFT
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12
Q

What cardiac evaluations are performed for systemic sclerosis?

A
  • ECG
  • ECHO

To look for PA HTN, heart failure, myocarditis and arrhythmias

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13
Q

What percentage of patients with systemic sclerosis have positive ANA?

A

90%

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14
Q

Which antibody is strongly associated with limited systemic sclerosis?

A

Anti-centromere antibody

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15
Q

Which antibodies are associated with diffuse systemic sclerosis?

A
  • Scl-70 (topoisomerase)
  • Anti RNA polymerase III antibodies
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16
Q

Is there a cure for systemic sclerosis?

A

No cure

17
Q

What treatment may reduce Raynaud’s symptoms in systemic sclerosis?

A
  • calcium antagonist
  • sildenafil
  • prostaglandin infusion - iloprost
18
Q

Which medications may reduce skin thickening in systemic sclerosis?

A
  • Methotrexate
  • Mycophenolate mofetil
19
Q

What is the role of ACE inhibitors in systemic sclerosis?

A

Prevent hypertensive crisis & reduce mortality from renal failure.

20
Q

What is morphoea?

A

A localized form of scleroderma.

21
Q

How much more common is scleroderma in women?

A

Five times more common.

22
Q

What vascular involvement occurs in systemic sclerosis?

A
  • Raynaud’s phenomenon
  • Ischemic digital ulcers
  • Hypertensive crisis
  • Pulmonary arterial hypertension
23
Q

True or False: Absence of Raynaud’s phenomenon makes the diagnosis of systemic sclerosis very unlikely.

A

True

24
Q

What is scleroderma renal crisis?

A

A serious condition with features of accelerated hypertension leading to renal failure if not treated promptly.

25
Q

When is scleroderma renal crisis seen in patients with diffuse SSc?

A

Early in the course of the disease.

26
Q

What treatment can be used for SSc flares

A

Short course of prednisolone

27
Q

What treatment can be used for GI symptoms of SSc

A

PPIs

28
Q

what does diffuse cutaneous SSc involve

A

also includes CREST features and affects internal organs:
- CVS: HTN, CAD
- lung: p.HTN, p. fibrosis
- kidney: scleroderma renal crisis

29
Q

what is scleroderma

A

hardening of the skin, giving the appearance of shiny, tight skin without the normal skin folds
- changes are most notable on the hands and face.

30
Q

what is sclerodactyly

A

describes skin changes in the hands:
- skin tightening around the joints which restricts ROM and reduces function
- fat pads on fingers lost
- skin can break and ulcerate

31
Q

what is telangiectasia

A

dilated blood vessels in the skin measuring less than 1mm in diameter

RHEUMATOLOGY (14 decks)

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