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msk-derm > Scleroderma > Flashcards

Scleroderma Flashcards

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Dermatology Board Prep flashcards
1
Q

What is scleroderma/systemic sclerosis?

A

An autoimmune disease belonging to the connective tissue disease family with highly variable and heterogenous clinical manifestations

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2
Q

What are the two major subtypes of scleroderma?

A

Limited cutaneous SSc and diffuse cutaneous SSc

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3
Q

What is the triad of clinical features present in scleroderma?

A

Immune dysregulation (autoimmunity/inflammation), vascular damage, and fibrosis

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4
Q

What demographics are most likely to be at risk of developing scleroderma?

A

female predominance

African-American predominance

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5
Q

What scleroderma symptoms predict increased mortality?

A

Skin induration on the trunk, tendon friction rubs at large and small joints, abnormal EKG, reduced diffusion capacity, elevated ESR, and presence of autoantibodies for topoisomerase I and RNA polymerase III

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6
Q

What are scleroderma mimics?

A

Conditions that resemble systemic sclerosis but are not systemic:

localized scleroderma, scleredema, scleromyxedema, eosinophilia-myalgia syndrome, eosinophilic fasciitis

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7
Q

What is the cause of scleroderma?

A

Unknown, but may be related to viral reactivation or occupational exposures

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8
Q

What is the genetic cause of scleroderma?

A

Unclear – low twin contractionrate suggests complicted genetic link. There are SNPs that encode risk alleles for scleroderma

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9
Q

What type of vascular damage is caused by scleroderma?

A

Diffuse vascular damage (microvascular obliterative vasculopathy)

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10
Q

How is vascular damage in scleroderma different from vasculitis or atherosclerosis?

A

There is little to no inflammation or lipid buildup

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11
Q

What are the three phases of scleroderma vascular damage?

A

1) endothelial cell activation with increased ET-1 and decreased prostacyclin release
2) vascular wall remodeling
3) blood vessel obliteration and ceased blood flow

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12
Q

What structures are most affected by vascular damage in scleroderma?

A

Small- and medium-sized vessels of digits, lungs, heart, and GI tract

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13
Q

What changes are associated with phase one of scleroderma vascular damage?

A

Reversible vasoconstriction, upregulation of adhesion molecules, and generation of ROS

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14
Q

What changes are associated with phase two of vascular damage in scleroderma?

A

Intimal proliferation, medial hypertrophy, adventitial fibrosis, platelet aggregation, and in situ thrombosis

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15
Q

What is vascular rarifaction?

A

Paucity of small blood vessels despite high levels of VEGF and angiogenic factors (obliterated blood vessels)

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16
Q

How is fibrotic response exacerbated by vascular damage in scleroderma?

A

Tissue hypoxia and generation of ROS from oxidative stress both lead to increased fibroblast activation

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17
Q

Which T-cell subtypes and cytokines are implicated in the development of fibrosis in scleroderma?

A

Th17 subset, Tregs, and IL-17/IL-23 cytokines

18
Q

What is the role of autoantibodies in scleroderma?

A

Unclear causal role

19
Q

What types of autoantibodies are seen exclusively in scleroderma?

A

topoisomerase-1, RNA polymerase III, and anti-centromere antibodies

20
Q

What is fibrosis?

A

An aberrant tissue repair process where the normal mechanisms that limit repair and promote resolution fail, resulitng in uncontrolled and intractable tissue remodeling

21
Q

What are the key effector cells of fibrosis?

A

Fibroblasts and myofibroblasts

22
Q

What cytokines and growth factors are important for fibrosis in scleroderma?

A

TGF-beta, CTGF, PDGF, Wnt, and endothelin-1

23
Q

What are the components of the ECM that accumulate during fibrosis?

A

Collagens, proteoglycans, fibronectin, fibrillin, tenascin, and elastin

24
Q

What is the role of TGF-beta in fibrosis?

A

It induces differentiation of fibroblasts, endothelial cells, and epithelial cells into smooth muscle cell-like myofibroblasts

25
Q

What accounts for the stiffness of fibrotic skin and other tissues?

A

Crosslinking of secreted collagen into a highly stable, degradation-resistant, and insoluble extracellular matrix

26
Q

What is the effect of fibrosis on capillaries?

A

Fibrosis obliterates capillaries (rarifaction), which creates a viscious cycle of hypoxia and fibrosis

27
Q

What organs are most commonly affected by scleroderma?

A

Skin, lungs, GI tract, kidneys, and heart

28
Q

What is a prominent early vascular feature of SSc?

A

Raynaud phenomenon

29
Q

What is a scleroderma renal crisis?

A

Injury to medium-sized arteries that occurs with malignant hypertension and hyperreninemia, microangiopathic hemolytic anemia, and rapidly progressive renal failure

30
Q

What are risk factors for renal involvement in scleroderma?

A

Extensive and advancing skin involvement, large joint contractures and tendon friction rubs, presence of autoantibodies to RNA polymerase III

31
Q

What drug is important for decreasing mortality of scleroderma with renal involvement?

A

ACE-inhibitors

32
Q

What is the pressure definition of pulmonary artery hypertension?

A

mean pulmonary artery pressure > 25 mmHg via right heart catheterization

33
Q

What is the cardiac involvement of scleroderma like?

A

It is frequent and can be caused by pulmonary hypertension and cardiac fibrosis

34
Q

A 45 y.o. previously healthy woman presents with episodic heartburn, increasing in frequency over the past 6 months. Her symptoms are worse at night. She also had several episodes of near-choking on solid food. On further questioning, she notes that she has had “cold sensitivity” in her fingers for 2-3 years; and for the past year, diffuse aching, and a fine rash on her face. Physical exam shows telangiectasia on her lips and cheeks, and erythema around her nailbed. Her laboratory evaluation is most likely to show which of the following?

a) hematuria
b) serum antibody directed to dsDNA
c) elevated level of serum complement 3
d) serum antibody directed to centromere
e) low serum B12

A

d) serum antibody directed to centromere

35
Q

A patient with long-standing systemic sclerosis is being treated with the endothelin-1 receptor antagonist bosentan. This class of drugs functions by inhibiting which of the following:

a) gastric acid secretion
b) bronchospasm
c) vasoconstriction
d) synovial inflammation
d) calcium deposition
e) collagen synthesis

A

c) vasoconstriction

36
Q

What is a Rondan skin score?

A

A semi-quantitative measure of the extent of skin induration (fibrosis)

37
Q

What is CREST for limited cutaneous SSc?

A

Calcinosis

Raynaud

Esophageal involvement

Sclerodactly

Telangiectasia

38
Q

What antibody is associated with limited cutaneous SSc?

A

Anticentromere antibodies

39
Q

What antibody is diffuse cutaneous SSc associated with?

A

Antitopoisomerase antibody

40
Q
A

msk-derm (41 decks)

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