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Clinical Medicine Ortho > Scleroderma > Flashcards

Scleroderma Flashcards

1
Q

What is scleroderma?

A

Acquired rheumatic disease with systemic sclerosis

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2
Q

What is the clinical presentation of scleroderma?

A

Thickening of skin with internal organ involvement

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3
Q

What is the cause of scleroderma?

A

No known cause

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4
Q

What are the 3 cardinal processes of scleroderma?

A
  1. Autoimmunity and inflammation
  2. Vascular injury and obliteration
  3. Fibrosis and matrix deposition
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5
Q

What is the path of scleroderma?

A

Capillary loss

Obliterative vasculopathy
- Intimal proliferation in small and medium vessels

Acellular fibrosis
- Deposition of connective tissue matrix

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6
Q

What tissues will be biopsied in scleroderma?

A

Skin
Lungs
GI

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7
Q

What will be found in a lung tissue biopsy?

A

Thickened alveolar septae

- Honeycombing

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8
Q

What will be found in a GI tissue biopsy?

A
  • Fibrosis of lamina propria and submucosa

- Muscle atrophy

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9
Q

What will be found in a skin tissue biopsy?

A
  • Collagen deposits in hair follicles

- Atrophic epidermis

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10
Q

What are the classifications of scleroderma?

A
  • Systemic Sclerosis
  • Mixed Connective Tissue Disorder
  • Localized Scleroderma
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11
Q

What is involved with systemic scleroderma?

A

Diffuse cutaneous

Limited cutaneous

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12
Q

What is diffuse cutaneous?

A

Involves skin

  • proximal to elbows and knees
  • trunk
  • distal extremities

Rapid progression

Early pulmonary fibrosis and acute renal failure

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13
Q

What is limited cutaneous?

A

Involves

  • fingers
  • toes
  • distal extremities
  • face

Trunk is spared

Raynaud’s phenomenon

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14
Q

What is CREST syndrome?

A 
Calcinosis cutis
Raynaud’s phenomenon
Esophageal dysmotility
Sclerodactyly
Telangiectasia
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15
Q

What is CREST syndrome involved with?

A

Limited cutaneous

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16
Q

What is localized scleroderma?

A
  • Benign skin conditions
  • Commonly affects children
  • Morphea- reddish/purple lesions of the skin
  • Skin induration
  • No Raynaud’s phenomenon
  • No systemic involvement
17
Q

What is scleroderma attributed to?

A

Environmental factors

Genetics

18
Q

What are the environmental factors of scleroderma?

A

Infectious agents

  • Cytomegalovirus
  • Parvovirus B19

Occupational

  • Silica
  • Epoxy resins

Dietary

Medications

  • Bleomycin
  • HRT
  • Cocaine
  • Appetite suppressants

Lifestyle exposures

19
Q

What is involved in genetics of scleroderma?

A

First degree= higher risk

20
Q

What is the initial presentation of diffuse cutaneous?

A 
Soft tissue swelling
Erythema
Pruritus
Fatigue
Stiffness 
Malaise
Raynaud’s will develop later
21
Q

What is the initial presentation of limited cutaneous?

A

History of Raynaud’s

+/- ischemic ulcerations

Indolent and delayed onset of other signs (CREST syndrome)

Vascular manifestations

  • Digital ischemia
  • Telangiectasia
  • Pulmonary HTN
22
Q

What are the organs involved in scleroderma?

A 
Skin
GI tract
Lungs
Kidneys
Cardiac
MSK
23
Q

What are the other manifestations of scleroderma?

A 
Dry eyes
Dry mouth
Hypothyroidism
CNS is spared usually
- Trigeminal nerve fibrosis can occur
24
Q

What labs are used in scleroderma

A 
CBC with diff
- Anemia
Sed Rate 
- Normal 
CRP 
- Normal
RA 
- Negative
ANAs
Vitamin deficiencies
- Folate
- Vit B12
- Vit D
25
Q

What is nailfold capillaroscopy?

A

Differentiates between primary vs secondary raynaud’s

26
Q

What is the diagnosis of scleroderma?

A

Skin induration
Raynaud’s phenomenon
Organ manifestations

27
Q

What are the treatment options of scleroderma?

A
  • No known regime to significantly alter the course
  • Early recognition
  • Accurate classification
  • Risk stratification
  • Early recognition of organ involvement
28
Q

What immunosuppressive agents are used in treatment of scleroderma?

A

Corticosteroids

  • Treats stiffness and aching
  • Use sparingly and at low doses

Cyclophosphamide

  • Decreased progression of symptomatic interstitial lung disease
  • Weigh risks and benefits

Methotrexate
- Skin manifestations

29
Q

What antibiotic theraphy is used in treatment of scleroderma?

A

D-Penicillamine

  • Improves skin induration
  • Prevents new internal organ involvement
  • Improved survival
30
Q

What has the worse prognosis?

A

Diffuse cutaneous systemic sclerosis

31
Q

What is involved 2-4 years after onset of scleroderma?

A

Inflammatory symptoms- fatigue; edema, arthralgia- subside
Skin thickening plateau
Organ involvement- most rapid progression

32
Q

When does scleroderma renal crisis occur?

A

Within 1st 4 years of onset

33
Q

What are late manifestations of scleroderma?

A

PAH

GERD

34
Q

What is involved in 10 year survival of scleroderma?

A

Diffuse cutaneous systemic scleroderma- 55%

Limited cutaneous systemic scleroderma- 75%

35
Q

What are the leading causes of death in scleroderma?

A

Pulmonary fibrosis
PAH
GI involvement
Cardiac disease

36
Q

What are some things that lead to a poor prognosis?

A

Male gender
AA race
Older age of disease onset
Low body mass index
Extensive skin thickening with truncal involvement
Presence of antibodies topoisomerase-I or RNA polymerase III
Absence of anticentromere antibodies

Clinical Medicine Ortho (42 decks)

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