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ICM Infectious Disease > Scleroderma > Flashcards

Scleroderma Flashcards

1
Q

What is Scleroderma?

A

Hardening or sclerosis of skin

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2
Q

Sclero defined as what?

A

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

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3
Q

Where are abnormalities in Sclero?

A

Abnormalities of microvasculature and large vessels

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

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4
Q

Cause of Sclero?

A

Unknown, thought to be autoimmune

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5
Q

Sclero associated with what autoimmune disease?

A

Polymyositis and MCTD

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6
Q

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

A

Anti endothelial

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7
Q

Microchimerism theory?

A

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

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8
Q

What is important in pathogenesis of sclero?

A

Potent vasoconstrictors

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9
Q

What is primary target tissue of Sclero?

A

Vascular endothelium

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10
Q

How does vascular endothelium present?

A

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

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11
Q

What effects are seen in the skin?

A

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

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12
Q

What happens to epidermis?

A

Thinned

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13
Q

What accumulates in the skin?

A

Excess collagen bundles and other extracellular matrix constituents

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14
Q

Who typically presents with Scleroderma?

A

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

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15
Q

What will a patient be complaining about in Sclero?

A

Puffy face and hands in the morning

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16
Q

What blood vessel sign can be seen in Sclero?

A

Telangiectasias

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17
Q

When does the edematous phase of the skin occur?

A

Early

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18
Q

What happens during the indurated phase?

A

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

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19
Q

What happens during Atrophic phase?

A

Fingers small and pointed, mouth smaller

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20
Q

What happens during late phase?

A

Skin softens and become pliable

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21
Q

Danger of Raynaud’s?

A

Infarcted fingers

22
Q

How common is esophageal dysmotility in patients?

A

High, 90%

23
Q

Involvment of duodenum and jejunum leads to what in sclero?

A

Malabsorption

24
Q

What cirrhosis frequently overlaps with sclero?

A

Biliary cirrhosis

25
Q

What pulmonary problem can occur with sclero?

A

Diffuse interstitial fibrosis with decreased oxygenation

26
Q

Patients will have dyspnea with what?

A

Exertion

27
Q

What pulmonary finding can you hear on exam?

A

Inspiratory crackles that can lead to pulmonary hypertension and death

28
Q

What does chest x-ray show?

A

Increased vascular markings at bases

29
Q

What does the first type of renal lesion do?

A

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

30
Q

Second type of renal problem?

A

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

31
Q

What fixes Sclero renal crisis?

A

Angiotensin converting enzyme

32
Q

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

A

No and no

33
Q

Markers for Sclero renal crisis?

A

Proteinurea
Hypertension
Azotemia
Microangiopathic hemolytic anemia

34
Q

Problems in the heart?

A

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

35
Q

What is linear sclero?

A

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

36
Q

What is morphea?

A

Patch or patches of sclero skin change

37
Q

What is limited sclero formerly CREST?

A

More limited involvement than PSS

38
Q

What is limited sclero associated with?

A

Anticentromere pattern ANA on HEP 2 substrate

39
Q

Limited sclero has more what association?

A

Lung over renal

40
Q

Scleroderma highly associated with what disease?

A

Diabetes

41
Q

What is eosinophilic fasciitis?

A

Inflammation and thickening of the deep fascia

42
Q

What is Eosinophilia-Myalgia syndrome

A

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

43
Q

What is Toxic oil syndrome of Spian/

A

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

44
Q

Study that shows positive in 90% of cases?

A

ANA positive

45
Q

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

A

Anticentromere Antibody

46
Q

Watch what for impending renal failure?

A

CBC, urine, creatinine

47
Q

Look for what showing pulmonary disease?

A

Chest x-ray, DLCO

48
Q

Treatment?

A

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

49
Q

Treatment for pulmonary?

A

Bosentan (Tracleer)

50
Q

Treatment for Renal?

A

ACE inhibitors

51
Q

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

A

D-Penicillamine

ICM Infectious Disease (19 decks)

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