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Systemic Sclerosis Flashcards

1
Q

What is the other word for systemic sclerosis?

A

Scleroderma

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2
Q

What are the 3 characteristics of Scleroderma?

A
  1. Vascular endothelial injury
  2. inflammation
  3. extensive activation of fibroblasts
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3
Q

What are the 5 most frequently affected organs in scleroderma?

A
  1. skin
  2. esophagus
  3. lungs
  4. heart
  5. kidneys
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4
Q

CREST?

A
  1. Calcinosis
  2. Raynaud’s Phenomenon
  3. Esophageal dysmotility
  4. Sclerodactyly (a scleroderma in which the fingers become thin and shiny with sclerotic skin at the tip, which is due to subcutaneous and intracutaneous calcinosis and diffused fibrosis of the collagen)
  5. Telangiectasias
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5
Q

Diffuse cutaneous SSC?

A
  • progressive form of SSC with an EARLY onset of RAYNAUD PHENOMENON (usually within 1 year of onset of skin thickening)
  • Characterized by rapid involvement of trunk, face, upper arms, and thighs
  • higher propensity to develop: (CSP)
    - PF (pulmonary fibrosis)
    - Cardiac involvement
    - SRC (scleroderma renal crisis)
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6
Q

antibody associated with diffuse cutaneous SSC?

A

anti-scleroderma 70 (antitopoisomerase-I) or anti-RNA polymerase III antibodies

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7
Q

Limited cutaneous SSc

A

characterized by a LONG pre existing history of RAYNAUD PHENOMENON and
skin changes of the extremities distal to the knee and elbow joints, including facial skin

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8
Q

Limited cutaneous SSC antibody?

A

presents with anti-centromere antibodies (50-70% of cases)

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9
Q

Limited cutaneous SSC is frequently associated with?

A

frequently associated with isolated PAH (Pulmonary arterial hypertension)

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10
Q

What do you call early SSC?

A

It is also known as undifferentiated SSc

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11
Q

early SSC or undifferentiated SSc is defined as?

A

positive RP and
at least 1 additional feature of SSc (positive nailfold capillary alterations, puffy fingers, pulmonary hypertension) and/or detectable scleroderma-associated autoantibodies without fulfilling the ACR criteria.

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12
Q

give the additional features of scleroderma.

A

1) positive nailfold capillary alterations
2)puffy fingers
3)pulmonary hypertension
and/or
detectable scleroderma-associated autoantibodies without fulfilling the ACR criteria

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13
Q

Define SSc sine scleroderma.

A

a very small proportion (1.5%) develop

  1. vascular (RP and/or PAH),
  2. immunologic (most commonly ANTICENTROMERE ANTIBODIES), and
  3. organ based fibrotic features of SSC

DO NOT SHOW SKIN SCLEROSIS

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14
Q

Raynaud phenomenon appears in what percentage of SSc patients?

A

appears in more than 90% of SSc patients

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15
Q

How do you define raynaud phenomenon?

A

it is defined by recurrent attacks of vasospasm of small digital arterioles/arteries at fingers and toes
usually caused by cold and/or other stimuli, for example, emotional stress

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16
Q

How does RP clinically appear?

A

RP clinically appears suddenly
It is clearly restricted and is accompanied by painful pallor/ischemia of single or several digits/toes
Followed by reactive hyperemia after reheating at the end of an attack
In some cases, cyanosis (triphasic RP) also ensues

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17
Q

What is a cardinal feature of SSc?

A

Skin involvement is a cardinal feature of SSc

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18
Q

Where does skin involvement in SSc usually appear?

A

It appears first in the fingers and hands

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19
Q

What kind of edema of the fingers do patients with SSc usually develop?

A

Patient usually develop nonpitting edema of the fingers (puffy fingers), hands and extremities followed by an increasing induration and skin thickening (sclerodactyly)

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20
Q

Depending on the localization of skin thickening, restricted mobility of joints (dermatogeneous contractures), and/or restricted breath excursion may be present

A
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21
Q

Depending on the localization of skin thickening, restricted mobility of joints (dermatogenous contractures), and/or restricted breath excursion may be present.

A
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22
Q

Typical facial features of SSc? (TBR)

A
  1. telangiectasias,
  2. a beak-shaped nose, and r
  3. reduced mouth aperture (microstomia)
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23
Q

typical facial appearance of SSc patients?

A
  1. radial furrowing around the mouth
  2. no expression
  3. a stiff and mask-like facial appearance
  4. sclerosis of the frenulum
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24
Q

Digital ulcers occur in scleroderma because?

A

because of poor blood flow to the fingertips which is in turn related to the narrowing of blood vessels that is the hallmark of all forms of systemic scleroderma.

25
Q

Approximately what percentage of patients with SSc have digital ulceration?

A

50%

26
Q

Define calcinosis cutis

A

It is the abnormal deposition of cutaneous and/or subcutaneous calcium which usually occurs over pressure points (acral, joints)

27
Q

Thibierge-Weissenbach syndrome?

A

Calcinosis cutis next to joints

28
Q

Where does calcinosis cutis in Scleroderma usually occur?

A

It usually occurs over pressure points (acral, joints)

29
Q

What are other skin manifestations of scleroderma?

A
  1. Hypopigmented and hyperpigmented skin (salt-and-pepper)

2. Loss of hair follicles and sweat glands (Hypohidrosis/ anhydrosis)

30
Q

What is the major external feature of structural vessel disease?

A

digital ulcerations

probably attributable to thickened intima and lumen-occluded vessels

31
Q

Tender and painful pitting scars are very frequent and on occasion progress to ulcers

A

These occur on the finger-or-toe tips, over the extensor surfaces of the joints as a result of microtrauma or in association with the abovementioned calcinosis cutis.

32
Q

Digital ulcers are associated with?

A

Strong, local pain

33
Q

What are other complications of digital ulceration?

A
  1. critical digital ischemia
  2. paronychia
  3. infections
  4. gangrene
  5. osteomyelitis
  6. finger pulp loss or amputation
34
Q

What are the cardiopulmonary manifeststaions of SCC?

A

Fibrosis

PAH (pulmonary arterial hypertension)

35
Q

What is currently the most common cause of disease-related death in SSc?

A

PAH (pulmonary arterial hypertension)

36
Q

what subset does PAH currently occur?

A

It occurs in both limited and diffuse cutaneous subsets.

ALTHOUGH, THE MOST TYPICAL CASES ARE THOSE OF LIMITED SSc associated with isolated PAH.

37
Q

What is the most common internal organ involvement in patients suffering from both limited and diffuse SSc?

A

GI involvement (>60%)

38
Q

What are possible gastric manifestations in SSc?

A

atrophy of mucous membrane-associated ulcerations

delayed gastric emptying

39
Q

What is an important complication in some SSc patients?

A

Gastric antral vascular ectasia

40
Q

Scleroderma renal crisis (SRC) occur in what percentage of SSc patients?

A

5% to 10% of patients

41
Q

Anti-platelet derived growth factor receptor

A
  • first functional antibody discovered in systemic sclerosis

- seems to induce SKIN FIBROSIS as a result of activation of fibroblasts into myofibroblasts and fibroblast like cells

42
Q
  • first functional antibody discovered in systemic sclerosis

- seems to induce SKIN FIBROSIS as a result of activation of fibroblasts into myofibroblasts and fibroblast like cells

A

Anti-platelet derived growth factor receptor

43
Q

frequency of anti-platelet derived growth factor receptor in systemic sclerosis?

A

33 to 100%

44
Q

Antiendothelial cell antibody?

A
  1. Mediates endothelial cell damage and activation of fibroblasts resulting from stimulation of proinflammatory and fibrotic cytokines
  2. associated with severe organ manifestation
  3. associated with perivascular, vascular (digital ulcers) and lung involvement (Pulmonary arterial hypertension [PAH])
  4. Also found in other rheumatic diseases
45
Q

functional autoantibody in systemic sclerosis that mediates endothelial cell damage and activation of fibroblasts resulting from stimulation of proinflammatory and fibrotic cytokines

A
  • antiendothelial cell antibodies
46
Q

functional autoantibody in systemic sclerosis that is associated with severe organ manifestation

A

Antiendothelial cell antibodies

47
Q

functional autoantibodies in systemic sclerosis that is associated with perivascular, vascular (digital ulcers), and lung involvement (Pulmonary arterial hypertension)

A

antiendothelial cell antibodies

48
Q

functional autoantibody in systemic sclerosis that is also found in other rheumatic diseases?

A

antiendothelial cell antibodies

49
Q

functional autoantibody in systemic sclerosis that is associated with SCl-70 antibodies and the prevalence of interstitial lung disease and PAH?

A

Antifibroblast antibodies

50
Q

functional autoantibody in systemic sclerosis that is associated with an increased prevalence in DIFFUSE CUTANEOUS SSc compared to limited cutaneous SSc

A

antifibroblast antibodies

51
Q

percentage of antifibroblast antibodies

A

26% to 58%

52
Q

functional autoantibody in systemic sclerosis that activates fibroblasts by stimulation of the reelase of transofrming growht factor-beta beta

A

Antifibrillin-1

53
Q

what is the frequency in systemic sclerosis of anti-fibrillin 1?

A

more than 50%

54
Q

What is the function

A
55
Q

Systemic sclerosis ACR criteria Major? (SPTM)

A
  1. Scleroderma proximal to the MCP or MTP joints
56
Q

Minor criteria for ACR in scleroderma (SDB)

A
  1. Sclerodactyly
  2. Digital ulcerations and/or pitting digital scars
  3. Bibasilar PF
57
Q

Systemic sclerosis ACR/European League Against Rheumatism criteria
*Additional criteria (AFA)

A
  1. Abnormal nailfold capillaries
  2. fingertip lesions
  3. autoantibodies
58
Q

Systemic sclerosis LeRoy and Medsger criteria

*Additional criteria

A
  1. Presence of autoantibodies

2. Nailfold capilloroscopic alterations

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