Systemic Sclerosis (Scleroderma)

Question 1

Main Characteristics of Scleroderma are

Answer 1

Thickening and fibrosis of skin AND internal organs

- Autoantibody production

Question 2

There is a hardcore vascular effect in scleroderma

Answer 2

Dr. P says “ vascular lesions in small blood vessels occur early and progress to obliterative vasculopathy that causes tissue hypoxia, oxidative stress, and vascular complications

Question 3

Peak incidence of Scleroderma is when?

Answer 3

Ages 30-50

Question 4

What sex is more affected?

Answer 4

WOMEN three times more so than MEN!!!

Question 5

What parts of the body does Limited Scleroderma affect?

Answer 5

Below the knees, below the elbows, above the neck. Basically anything that wouldn’t be covered by Shorts and a T shirt. Its like the pt was wearing shorts and a t shirt around.

Question 6

Skin changes in diffuse scleroderma usually begin with what>

Answer 6

Puffy hands

Question 7

After the hands, what is affected next?

Answer 7

The face

Question 8

Thickening progresses proximally from the hands

Answer 8

yes

Question 9

What is Raynaud’s phenomenon

Answer 9

COLD INDUCED…….VASOSPASM

Red, white, and blue hands (Erythema, blanching, cyanosis)

Question 10

In about 3/4 of scleroderma pts, what is the initial complaint>

Answer 10

Raynauds….cold induced vasospasm

THIS is more true for limited sclerosis

Question 11

KNOW CREST…This is for localized, but it works for diffuse as well.

Answer 11

C- Calcinosis (calcium deposits in the skin)
R- Raynaud's 
E- Esophageal dysmotility
S- Sclerodactyly
T- Telangiectasia

Question 12

Two major antibodies seen in systemic scleroderma

Answer 12

ANTI- Scl 70

Anti-RNA polymerase

Question 13

Teleangiectasias

Answer 13

seen mostly in limited scleroderma

Question 14

Calcinosis

Answer 14

Seen mostly in limited scleroderma

Question 15

What are some of the musculoskeletal effects of scleroderma

Answer 15

Arthritis, Arthralgia, Tendonitis, Myopathy

Question 16

What is the most common visceral involvement in systemic sclerosis

Answer 16

The GI tract

Question 17

WHat is the most common GI issue in SSc?

Answer 17

Esophageal dysmotility…..acid reflux. treat with proton pump inhibitors

• Other issues= Blood loss through gastric bleeding

Question 18

Midgut disease=

Answer 18

altered motility in the stomach, bacteria overgrow, need antibiotics to calm it down
- May have recurrent obstruction

Question 19

Large Bowel DZ/ Giant Diverticuli

Answer 19

Cause by fibrosis of the diverticulum

Question 20

Cardiac problems

Answer 20

• Myocardial fibrosis ( EVERYTHING FIBROSIS)
• = Conduction abnormalities
• Arrhythmias
• CHF

Question 21

Renal dz in SSC

Answer 21

Scleroderma renal crisis…you get hypertension….ACEi have made this a less frequent cause of mortalty

Question 22

What does Scleroderma renal crisis look like in clinic

Answer 22

ABRUPT Htn.

- Urinalysis shows hematuria and proteinuria

Question 23

What, according to Dr. P, are the earliest pathological changes in SSc

Answer 23

changes in endothelial cell function featuring:

• Increased apoptosis
• upreg of MHC class II
• upreg of Intracellular Adhesion Molecule (ICAM-1)
• ALSO, platelett aggregation
• Intimal thickening

Question 24

Pulmonary problems

Answer 24

pleurisy, pleural effusins, aspiration pneumonia, malignancy, Interstitial lung dz, Pulmonary Vasculature dz

Question 25

Lung Dz is a frequent cause of death in SSC

Answer 25

true….esp PAH and ILD

Question 26

What kinds of pts have the worst prognosis when it comes to SSc lung dz

Answer 26

Blacks with anti-topoisomerase 1

Question 27

Risk factors for severe fibrosis

Answer 27

Early diffuse scleroderma with Anti-Scl 0 or nucleolar ab

• FVC less than 75%
  FVC that increases by more than 10% per year.

Question 28

What is the prevalence of PAH in SSc

Answer 28

7-15%

Question 29

Risk factors for Scleroderma renal crisis

Answer 29

early diffuse skin dz, use of corticosteroids, presence of anti-RNA polymerase III antibodies