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MSK/Derm > SM 239: Scleroderma > Flashcards

SM 239: Scleroderma Flashcards

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Dermatology Board Prep flashcards
1
Q

What is the Rodan skin score?

A

A low tech approach to assess extent of skin duration and fibrosis in SSc

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2
Q

What diffuion suggests Lupus in ANA?

A

ANA 1:60 using fluorescent antibodies

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3
Q

What PFT suggests sclerosis?

A

Lower PFT (Low FVC)

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4
Q

What is systemic sclerosis?

A

A chronic multisysem orphan disease that effects < 200,00 people per year n in the US

Incentivizes pharma companies

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5
Q

What are the two main subsets of Systemic Sclerosis?

A

Limited cutaneous SSc and Diffuse cutaneous SSc an dthey differ by pattern of skin involvement, organ involvement, rate of disease progression, autoantibody profile and long term survival

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6
Q

Which groups are more effected by SSc?

A

Women and african americans, who may also develop more aggressive diesease

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7
Q

What correlates with survival in SSc?

A

Involvement of internal organs, primarily the heart, lungs, kidneys and GI tract

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8
Q

What are the baseline predictors of survival in SSc?

A

Skin induration on the trunk
Tendon friction rubs at large and small joints
Abnormal EKG
Reduced DLCO
Elevaed ESR and teh presence of autoantibodies against Topoisomerase I and RNA Poly III

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9
Q

What must Systemic Sclerosis be differentiated from?

A

Scleroderma mimics, such as localized scleroderma, scleredema, and scleromyxedema

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10
Q

How can SSc be differentiated from mimics?

A

Skin biopsy, clinical presentation, and serologic studies

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11
Q

What causes SSc?

A

Exposure to unknown environmental factors as well as infection with EBV may play a role, but true etiology remains unknown

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12
Q

Describe the genetics of SSc?

A

SSc is a non-mendelian disorder with a complex and mutli-genic background, and an array of relevant genes have been found in SNP’s from GWAS

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13
Q

Which gene’s SNP’s may contribute to SSc?

A

TGF-beta, IL-1, ACE

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14
Q

What is the triad pathophysiology associated with SSc?

A

Triad of inflammation, vascular damage and fibrosis

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15
Q

What causes vascular damage in SSc?

A

Vascular injury, possibly due to virus, autoantibodies against endothelial cells, and ROS buildiup early in SSC

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16
Q

What is Microvascular Obliterative Vasculopathy?

A

A form of diffuse vascular damage that is prominent in most organs

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17
Q

How does Microvascular Obliterative Vasculopathy develop?

A

Endothelial cells produce Endothelin and decrease prostacycllin release, causing vasoconstriction and ROS buildup

Vascular wall remodeling follows with intimal proliferation and medial hypertrophy, leading to in situ thrombosis

Ultimately blood vessels are obliterated and blood flow ceases

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18
Q

How does ROS form a vicious cycle with microvasculature obliterative vasculopathy?

A

Tissue hypoxia leads to fibroblast activation as well as ROS buildup, and ROS buildup further increases fibroblast activation

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19
Q

How is microvasculature obliterave vasculopathy differentiated from vasculitis and atherosclerosis?

A

No inflammation or lipid buildup

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20
Q

How does immunity drive SSc?

A

Immunity can be seen in the activation of Tcells suggesting an antigen driven response, though the antigen is unknown

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21
Q

What can be found uniformly in SSc patients, and what role do they play in pathogenesis?

A

Auto-antibodies are uniformly found in SSc patients, but their casual role in pathogenesis is unproven

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22
Q

What do autoantibodies in SSc target specifically?

A

Autoantibodies are directed against Topoisomerase-1, RNA PolyIII and centromeres - not found in other autoimmune diseases

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23
Q

What are endophenotypes in SSc and how are they identified?

A

Endophenotypes are particular disease subsets in SSc and are determined by the spectrum of antibodies produced in the disease

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24
Q

What is fibrosis?

A

Fibrosis is an aberrant tissue repair process where the normal mechanism that limit repair and promote resolution fail, leading to uncontrolled tissue remodeling

25
Q

If Fibrosis is part of tissue repair, what makes it go hayware?

A

Fibrosis is pathological when the mechanisms that limit repair and promote resolution fail, resulting in uncontrolled tissue remodeling

26
Q

What cells mediate Fibrosis?

A

Fibroblasts and myofibroblasts mediate fibrosis

27
Q

What cytokines and growth factors are important in fibrosis and why?

A

TGF-beta, PDGF, and Endothelin - they promote the synthesis of ECM proteins like collagens and proteoglycans

28
Q

How do ECM proteins effect fibrosis?

A

Buildup of ECM proteins results in fibrosis and blood vessel destruction, and TGF-beta induces the differentiation of fibroblasts into myofibroblasts

29
Q

What organs can be effected by SSc?

A

Skin, lungs, GI tract, kidneys and heart are commonly effected, but SSc can effect any organ

30
Q

How does SSc effect the lungs?

A

SSc promotes fibrosis, leading to ILD and restrictive changes like low FVC on PFT

31
Q

What is Raynaud phenomenon?

A

Intermittent cold-induced vasospasm in the fingers and toes, a prominent early vascular feature of SSc that causes cyanosis and erythema

32
Q

How does SSc effect the kidneys?

A

Injury to the medium-sized arteries can cause scleroderma renal crisis (SRC)

33
Q

What does Scleroderma Renal Crisis lead to?

A

SRC can lead to malignant hypertension and microangiopathic hemolytic anemia

34
Q

What are the risk factors for developing SRC?

A

Extensive skin involvement, presence of autoantibodies to RNA Poly III

35
Q

What class of drugs increases the risk of SRC?

A

Corticosteroids

36
Q

What is the treatment for SRC?

A

Scleroderma Renal Crisis can be treated with ACE inhibitors in an early intervention

37
Q

What is the major lung complication and cause of death in SSc?

A

Pulmonary Arterial Hypertension, due to obliteration of small arterioles in the lung, creating a risk for Right Heart Failure

38
Q

How might right heart failure arise in SSc?

A

SSc destroys small vessels such as the arterioles in the Lungs, leading to Pulmonary Arterial Hypertension and increasing the afterload on the Right Heart Ventricle

39
Q

How can SSc directly effect the heart?

A

Fibrosis of cardiac microvasculature

Indirectly effects the heart due to PH

40
Q

How can cardiac involvement be detected in SSc?

A

Cardiac MRI imaging can detect Cardiac involvement early

41
Q

How can pulmonary hypertension be detected?

A

ECHO doppler

42
Q

What does Limited Cutaneous SSc present with?

A

CREST:

Calcinosis
Raynaud
Esophageal involvement
Sclerodactyly
Telangiectasia
43
Q

Which antibody is limited cutaneous SSc associated with?

A

Anti-centromere antibodies are found in LC SSc

44
Q

Which has better survival and progression, LC SSc or diffuse SSc?

A

LC SSc has better prognosis, slower progression, and long survival

45
Q

How does diffuse cutaneous SSc present?

A

Diffuse skin induration that involves the trunk

46
Q

Which antibody does Diffuse Cutaneous SSc involve?

A

Anti-topoisomerase antibodies are found in diffuse cutaneous SSc

47
Q

What are the four cardinal manifestations of SSc microangiopathy?

A

Mucocutaneous Telangiectasia
Raynaud’s
PAH
SRC

48
Q

What is Telangiectasia?

A

“spider veins” on the skin

49
Q

What are the 2 phases of Raynaud phenomenon?

A

First phase = blanching; second phase = cyanosis; reversible vasospam that leads to ischemic finger ulcers

50
Q

Which group is more like to develop Scleroderma Renal Crisis?

A

Diffuse Cutaneous SSc is more likely to develop SRC than limited cutaneous SSc

51
Q

How does Scleroderma Renal Crisis present?

A

Accelerated/malignant HTN, heart failure, stroke, and blindness due to damage from high blood pressure

52
Q

How does SRC manifest in the kidneys?

A

Renal ischemia from destruction of blood vessels and intra-renal complement activation that leads to onion-skin vascular lesions

53
Q

What do anti-topoisomerase antibodies predict in SSc?

A

Pulmonary Fibrosis

54
Q

What do anti-RNA Polymerase antibodies predict in SSc?

A

SRC

55
Q

Which auto antibodies are protective in SSc?

A

Anti-centromere antibodies

56
Q

Is the multi-organ failure in SSc sequential or simultaneous?

A

Simultaneous multi-organ failure in SSc

57
Q

What lung patterns are found on HRCT for SSc?

A

NSIP or UIP patterns that drive ILD, the leading cause of death in SSc

58
Q

Describe the broad overview of tripartite pathogenesis in SSc?

A

A variety of stressors such as ROS buildup and viral infection leads to impaired vasculogenesis by Endothelial cells

Platelet activation and thrombi formation increase as well as antibodies against dysfunctional Endothelial cells

Fibroblasts are then recruited to fix the ROS environment drives hyperactivity and scarring

MSK/Derm (46 decks)

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