Scleroderma

Question 1

What is scleroderma?

Answer 1

Acquired rheumatic disease with systemic sclerosis

Question 2

What is the clinical presentation of scleroderma?

Answer 2

Thickening of skin with internal organ involvement

Question 3

What is the cause of scleroderma?

Answer 3

No known cause

Question 4

What are the 3 cardinal processes of scleroderma?

Answer 4

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

Question 5

What is the path of scleroderma?

Answer 5

Capillary loss

Obliterative vasculopathy
- Intimal proliferation in small and medium vessels

Acellular fibrosis
- Deposition of connective tissue matrix

Question 6

What tissues will be biopsied in scleroderma?

Answer 6

Skin
Lungs
GI

Question 7

What will be found in a lung tissue biopsy?

Answer 7

Thickened alveolar septae

- Honeycombing

Question 8

What will be found in a GI tissue biopsy?

Answer 8

• Fibrosis of lamina propria and submucosa

- Muscle atrophy

Question 9

What will be found in a skin tissue biopsy?

Answer 9

• Collagen deposits in hair follicles

- Atrophic epidermis

Question 10

What are the classifications of scleroderma?

Answer 10

• Systemic Sclerosis
• Mixed Connective Tissue Disorder
• Localized Scleroderma

Question 11

What is involved with systemic scleroderma?

Answer 11

Diffuse cutaneous

Limited cutaneous

Question 12

What is diffuse cutaneous?

Answer 12

Involves skin

• proximal to elbows and knees
• trunk
• distal extremities

Rapid progression

Early pulmonary fibrosis and acute renal failure

Question 13

What is limited cutaneous?

Answer 13

Involves

• fingers
• toes
• distal extremities
• face

Trunk is spared

Raynaud’s phenomenon

Question 14

What is CREST syndrome?

Answer 14

Calcinosis cutis
Raynaud’s phenomenon
Esophageal dysmotility
Sclerodactyly
Telangiectasia

Question 15

What is CREST syndrome involved with?

Answer 15

Limited cutaneous

Question 16

What is localized scleroderma?

Answer 16

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

Question 17

What is scleroderma attributed to?

Answer 17

Environmental factors

Genetics

Question 18

What are the environmental factors of scleroderma?

Answer 18

Infectious agents

• Cytomegalovirus
• Parvovirus B19

Occupational

• Silica
• Epoxy resins

Dietary

Medications

• Bleomycin
• HRT
• Cocaine
• Appetite suppressants

Lifestyle exposures

Question 19

What is involved in genetics of scleroderma?

Answer 19

First degree= higher risk

Question 20

What is the initial presentation of diffuse cutaneous?

Answer 20

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

Question 21

What is the initial presentation of limited cutaneous?

Answer 21

History of Raynaud’s

+/- ischemic ulcerations

Indolent and delayed onset of other signs (CREST syndrome)

Vascular manifestations

• Digital ischemia
• Telangiectasia
• Pulmonary HTN

Question 22

What are the organs involved in scleroderma?

Answer 22

Skin
GI tract
Lungs
Kidneys
Cardiac
MSK

Question 23

What are the other manifestations of scleroderma?

Answer 23

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

Question 24

What labs are used in scleroderma

Answer 24

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

Question 25

What is nailfold capillaroscopy?

Answer 25

Differentiates between primary vs secondary raynaud’s

Question 26

What is the diagnosis of scleroderma?

Answer 26

Skin induration Raynaud’s phenomenon Organ manifestations

Question 27

What are the treatment options of scleroderma?

Answer 27

- No known regime to significantly alter the course - Early recognition - Accurate classification - Risk stratification - Early recognition of organ involvement

Question 28

What immunosuppressive agents are used in treatment of scleroderma?

Answer 28

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

Question 29

What antibiotic theraphy is used in treatment of scleroderma?

Answer 29

D-Penicillamine - Improves skin induration - Prevents new internal organ involvement - Improved survival

Question 30

What has the worse prognosis?

Answer 30

Diffuse cutaneous systemic sclerosis

Question 31

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

Answer 31

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

Question 32

When does scleroderma renal crisis occur?

Answer 32

Within 1st 4 years of onset

Question 33

What are late manifestations of scleroderma?

Answer 33

PAH | GERD

Question 34

What is involved in 10 year survival of scleroderma?

Answer 34

Diffuse cutaneous systemic scleroderma- 55% Limited cutaneous systemic scleroderma- 75%

Question 35

What are the leading causes of death in scleroderma?

Answer 35

Pulmonary fibrosis PAH GI involvement Cardiac disease

Question 36

What are some things that lead to a poor prognosis?

Answer 36

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