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ICM Infectious Disease > Scleroderma > Flashcards

Flashcards in Scleroderma Deck (51):
1

What is Scleroderma?

Hardening or sclerosis of skin

2

Sclero defined as what?

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

3

Where are abnormalities in Sclero?

Abnormalities of microvasculature and large vessels
Fibrotic changes in muscles, joints, and viscera (esophagus, GI tract, heart, lungs, and kidney)

4

Cause of Sclero?

Unknown, thought to be autoimmune

5

Sclero associated with what autoimmune disease?

Polymyositis and MCTD

6

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

Anti endothelial

7

Microchimerism theory?

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

8

What is important in pathogenesis of sclero?

Potent vasoconstrictors

9

What is primary target tissue of Sclero?

Vascular endothelium

10

How does vascular endothelium present?

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

11

What effects are seen in the skin?

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

12

What happens to epidermis?

Thinned

13

What accumulates in the skin?

Excess collagen bundles and other extracellular matrix constituents

14

Who typically presents with Scleroderma?

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

15

What will a patient be complaining about in Sclero?

Puffy face and hands in the morning

16

What blood vessel sign can be seen in Sclero?

Telangiectasias

17

When does the edematous phase of the skin occur?

Early

18

What happens during the indurated phase?

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

19

What happens during Atrophic phase?

Fingers small and pointed, mouth smaller

20

What happens during late phase?

Skin softens and become pliable

21

Danger of Raynaud's?

Infarcted fingers

22

How common is esophageal dysmotility in patients?

High, 90%

23

Involvment of duodenum and jejunum leads to what in sclero?

Malabsorption

24

What cirrhosis frequently overlaps with sclero?

Biliary cirrhosis

25

What pulmonary problem can occur with sclero?

Diffuse interstitial fibrosis with decreased oxygenation

26

Patients will have dyspnea with what?

Exertion

27

What pulmonary finding can you hear on exam?

Inspiratory crackles that can lead to pulmonary hypertension and death

28

What does chest x-ray show?

Increased vascular markings at bases

29

What does the first type of renal lesion do?

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

30

Second type of renal problem?

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

31

What fixes Sclero renal crisis?

Angiotensin converting enzyme

32

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

No and no

33

Markers for Sclero renal crisis?

Proteinurea
Hypertension
Azotemia
Microangiopathic hemolytic anemia

34

Problems in the heart?

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

35

What is linear sclero?

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

36

What is morphea?

Patch or patches of sclero skin change

37

What is limited sclero formerly CREST?

More limited involvement than PSS

38

What is limited sclero associated with?

Anticentromere pattern ANA on HEP 2 substrate

39

Limited sclero has more what association?

Lung over renal

40

Scleroderma highly associated with what disease?

Diabetes

41

What is eosinophilic fasciitis?

Inflammation and thickening of the deep fascia

42

What is Eosinophilia-Myalgia syndrome

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

43

What is Toxic oil syndrome of Spian/

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

44

Study that shows positive in 90% of cases?

ANA positive

45

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

Anticentromere Antibody

46

Watch what for impending renal failure?

CBC, urine, creatinine

47

Look for what showing pulmonary disease?

Chest x-ray, DLCO

48

Treatment?

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

49

Treatment for pulmonary?

Bosentan (Tracleer)

50

Treatment for Renal?

ACE inhibitors

51

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

D-Penicillamine