Scleroderma

Question 1

What is Scleroderma?

Answer 1

Hardening or sclerosis of skin

Question 2

Sclero defined as what?

Answer 2

Disorder of connective tissue characterized by induration and thickening of the skin

Question 3

Where are abnormalities in Sclero?

Answer 3

Abnormalities of microvasculature and large vessels

Fibrotic changes in muscles, joints, and viscera (esophagus, GI tract, heart, lungs, and kidney)

Question 4

Cause of Sclero?

Answer 4

Unknown, thought to be autoimmune

Question 5

Sclero associated with what autoimmune disease?

Answer 5

Polymyositis and MCTD

Question 6

About 20-30% of Sclero patients have anti what cytotoxic factors?

Answer 6

Anti endothelial

Question 7

Microchimerism theory?

Answer 7

Cells from mother are transported across the placenta, persist and cause a graft versus host type disease

Question 8

What is important in pathogenesis of sclero?

Answer 8

Potent vasoconstrictors

Question 9

What is primary target tissue of Sclero?

Answer 9

Vascular endothelium

Question 10

How does vascular endothelium present?

Answer 10

Intimal sclerosis, fibrinoid change, and necrosis, decrease in vessel diameter, local ischemia, and fibrosis

Question 11

What effects are seen in the skin?

Answer 11

Atrophy of skin appendages and loss of hair follicle and sweat gland

Question 12

What happens to epidermis?

Answer 12

Thinned

Question 13

What accumulates in the skin?

Answer 13

Excess collagen bundles and other extracellular matrix constituents

Question 14

Who typically presents with Scleroderma?

Answer 14

Female aged 30-40 with pallor/cyanosis of fingers and toes on cold exposure or emotional upset

Question 15

What will a patient be complaining about in Sclero?

Answer 15

Puffy face and hands in the morning

Question 16

What blood vessel sign can be seen in Sclero?

Answer 16

Telangiectasias

Question 17

When does the edematous phase of the skin occur?

Answer 17

Early

Question 18

What happens during the indurated phase?

Answer 18

Skin is stiff and bound to underlying structures. Alopecia and hyper pigmentation/depigmentation occur

Question 19

What happens during Atrophic phase?

Answer 19

Fingers small and pointed, mouth smaller

Question 20

What happens during late phase?

Answer 20

Skin softens and become pliable

Question 21

Danger of Raynaud’s?

Answer 21

Infarcted fingers

Question 22

How common is esophageal dysmotility in patients?

Answer 22

High, 90%

Question 23

Involvment of duodenum and jejunum leads to what in sclero?

Answer 23

Malabsorption

Question 24

What cirrhosis frequently overlaps with sclero?

Answer 24

Biliary cirrhosis

Question 25

What pulmonary problem can occur with sclero?

Answer 25

Diffuse interstitial fibrosis with decreased oxygenation

Question 26

Patients will have dyspnea with what?

Answer 26

Exertion

Question 27

What pulmonary finding can you hear on exam?

Answer 27

Inspiratory crackles that can lead to pulmonary hypertension and death

Question 28

What does chest x-ray show?

Answer 28

Increased vascular markings at bases

Question 29

What does the first type of renal lesion do?

Answer 29

Causes lesions that reduce flow of renal artery and impinges glomerular function

Question 30

Second type of renal problem?

Answer 30

Scleromderma renal crisis where sudden collapse of glomerular filtration leads to severe volume depletion

Question 31

What fixes Sclero renal crisis?

Answer 31

Angiotensin converting enzyme

Question 32

Is Sclero renal crisis inflammatory? Does it have immune complexes?

Answer 32

No and no

Question 33

Markers for Sclero renal crisis?

Answer 33

Proteinurea Hypertension Azotemia Microangiopathic hemolytic anemia

Question 34

Problems in the heart?

Answer 34

Pericardial effusion, cor pulmonale secondary to pulmonary hypertension ad microvascular ischemia

Question 35

What is linear sclero?

Answer 35

Band of sclerotic induration and hyper pigmentation occurring on single extremity or face

Question 36

What is morphea?

Answer 36

Patch or patches of sclero skin change

Question 37

What is limited sclero formerly CREST?

Answer 37

More limited involvement than PSS

Question 38

What is limited sclero associated with?

Answer 38

Anticentromere pattern ANA on HEP 2 substrate

Question 39

Limited sclero has more what association?

Answer 39

Lung over renal

Question 40

Scleroderma highly associated with what disease?

Answer 40

Diabetes

Question 41

What is eosinophilic fasciitis?

Answer 41

Inflammation and thickening of the deep fascia

Question 42

What is Eosinophilia-Myalgia syndrome

Answer 42

Abrupt onset of myalgia, fatigue and periphery eosinophilia associated with ingestion of L-tryptophan dietary supplement

Question 43

What is Toxic oil syndrome of Spian/

Answer 43

Epidemic of acute pneumonitis, scleroderma like skin thickening, neromyopathy, and sicca syndrome that affected thousands in Spain in 1982 due to rapeseed oil

Question 44

Study that shows positive in 90% of cases?

Answer 44

ANA positive

Question 45

Hep-2 substrate present in 50-96% of patients?

Answer 45

Anticentromere Antibody

Question 46

Watch what for impending renal failure?

Answer 46

CBC, urine, creatinine

Question 47

Look for what showing pulmonary disease?

Answer 47

Chest x-ray, DLCO

Question 48

Treatment?

Answer 48

No drug, hand protection, moisturizing, keep warm, no smoking

Question 49

Treatment for pulmonary?

Answer 49

Bosentan (Tracleer)

Question 50

Treatment for Renal?

Answer 50

ACE inhibitors

Question 51

Drug used without evidence for many years without proof it works?

Answer 51

D-Penicillamine