Systemic Sclerosis

Question 1

What are other names for systemic sclerosis?

Answer 1

• progressive systemic sclerosis
• scleroderma (old name; implies skin only)

Question 2

What is the 3rd most common autoimmune condition?

Answer 2

systemic sclerosis

Question 3

What is the etiology of systemic sclerosis?

Answer 3

autoimmune

Question 4

Systemic sclerosis is a ____-____ disorder

Answer 4

collagen-vascular
(excess collagen + vasculitis)

Question 5

What demographic is primarily affected by systemic sclerosis?

Answer 5

• onset 40-50 yrs
• F>M (3-8:1)

Question 6

What are the general systems affected by systemic sclerosis?

Answer 6

• GI
• pulmonary, renal & cardiac (fibrosis)
• MSK
• vascular

Question 7

What are the possible clinical manifestations of systemic sclerosis involving the GI tract?

Answer 7

• Xerostomia (“desert mouth”)
• loss of peristalsis (dysphagia)
• severe reflux (GERD)

Question 8

What is xerostomia?

Answer 8

aka “desert mouth”
dry mouth due to lack of saliva
(in systemic sclerosis, caused by fibrosis of mucus glands)

Question 9

What is dysphagia?

Answer 9

difficulty swallowing

Question 10

What is a potential sequela of GI involvement of systemic sclerosis?

Answer 10

severe reflux –> Barrett esoph. –> esoph. carcinoma

Question 11

What is the most common cause of death in patients with systemic sclerosis?

Answer 11

lung disease (COPD)
eg. emphysema w/ no Hx of smoking

Question 12

What are the possible clinical manifestations of systemic sclerosis involving the renal system?

Answer 12

renal insufficiency & malignant HTN

Question 13

What are the possible clinical manifestations of systemic sclerosis involving the MSK system?

Answer 13

• swelling & polyarthralgia
• synovitis
• loss of AROM
• deformity (reversible; swan neck, boutonniere, hitch hiker)
• fatigue
• exacerbation/remission pattern
• “mouse-like” facies (erosion of zygomatic arches)

Question 14

What is systemic sclerosis over-diagnosed as?

Answer 14

fibromyalgia (general fatigue & achiness)

Question 15

How does vasculitis present in patients with systemic sclerosis?

Answer 15

Raynaud phenomenon (finger/toe tips, ears, nose)

Question 16

How do fibroblastic and vascular changes present in patients with systemic sclerosis?

Answer 16

fibrosis of skin (sweat/oil glands dry up) causes leathery/tight skin
- Acro-osteolysis
- osteonecrosis/soft tissue necrosis
- loss of facial expression

Question 17

What is acro-osteolysis and how does it present in patients with systemic sclerosis?

Answer 17

• erosion of distal bone (tips of fingers/toes) due to retraction of tight skin pulling on bone
• presents as ungual tuft resorption radiographically

Question 18

How does soft tissue calcification in systemic sclerosis appear radiographically?

Answer 18

in “working areas” (high contact areas) such as finger pads

Question 19

What are the radiographic characteristics of systemic sclerosis?

Answer 19

• Acro-osteolysis (ungual tuft resorption)
• soft tissue calcification (of necrosed areas)
• possible deformity (swan neck, boutonniere, hitch hiker)

Question 20

What are the relevant lab findings for systemic sclerosis?

Answer 20

• ^ESR/CRP
• anemia of chronic dz
• RF neg.
• ANA pos. (95%)
• ^creatinine & liver enzymes (reduced renal function)

Question 21

Dry mouth and severe GERD are seen mostly in what condition?

Answer 21

progressive systemic sclerosis