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MOS > Systemic Sclerosis > Flashcards

Systemic Sclerosis Flashcards

1
Q

Acquired chronic connective tissue disease; more common in WOMEN; characterized by Autoimmunity, Vascular damage and Tissue Fibrosis

A

Systemic Sclerosis

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2
Q

(Limited/Diffuse) Scleroderma was previously known as CREST syndrome; involvement in hands, arms and face but not internal organs

A

Limited

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3
Q

(Limited/Diffuse) Scleroderma is rapidly progressive and involves the torso, arms and internal organs

A

Diffuse

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4
Q

3 Subtypes of Systemic Sclerosis

A

Limited Cutaneous
Diffuse Cutaneous
Sine Scleroderma

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5
Q

Features of LIMITED Cutaneous Sclerosis

A 
Calcinosis
Raynaud's
Esophageal Dymotility
Sclerodactyly
Telangiectasis

(formerly known as “CREST” syndrome)

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6
Q

Features of DIFFUSE Cutaneous Sclerosis

A 
RAPID involvement
Tendon Friction Rubs
Interstitial Lung Disease
Renal Crisis
Anti-Scl-70 (Topoisomerase) antibody
Anti-RNA polymerase
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7
Q

What differentiates Limited/Diffuse Cutaneous Sclerosis from Sine Scleroderma

A

Sine Scleroderma means “no skin thickening”

many other features are present though

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8
Q

Most common organ systems involved with Systemic Sclerosis

A

Skin
Gastrointestinal
Pulmonary

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9
Q

Vasospastic reaction to cold temperatures or emotional stress; SHARPLY demarcated color changes of skin; can be associated with autoimmune conditions, especially Sclerosis

A

Raynaud’s Phenomenon

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10
Q

Pattern of Color changes seen in Raynaud’s Phenomenon

A

White (ischemia)–> Blue (Cyanosis)–> Red (Re-perfusion)

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11
Q

Conditions that can cause Secondary Raynaud’s Phenomenon

A

Systemic Sclerosis*
SLE
Sjogren Syndrome
Dermatomyositis

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12
Q

Treatment for Raynaud’s Phenomenon

A 
Keep hands warm
Ca+ channel blockers
Nitroglycerin (topical)
PDE inhibitors
Prostacyclines
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13
Q

Leading mortality for Systemic Sclerosis patients

A

Lung Disease (Interstitial Lung Disease and/or PAH)

renal is second most

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14
Q

Interstitial Lung Disease due to Systemic Sclerosis is associated with which antibody?

A

Scl-70 (topoisomerase 1)

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15
Q

Anti-Scl 70 (Anti-Topoisomerase 1) is associated with ____________ Cutaneous Systemic Sclerosis

A

Diffuse (seen with ILD)

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16
Q

Pulmonary Artery Hypertension due to Systemic Sclerosis is associated with which antibody?

A

Anti-Centromere antibody

17
Q

Anti-Centromere Antibody is associated with _______________ Cutaneous Systemic Sclerosis

A

Limited (seen with PAH)

18
Q

How to tell the difference between IF staining for Anti-Centromere vs. Anti-Scl 70 (Topoisomerase) Antibody

A

Anti-Centromere: dots in nuclei (left)

Anti-Scl 70: all of cytoplasm (right)

19
Q

Renal Crisis due to Systemic Sclerosis is associated with which antibody?

A

RNA-Polymerase I and III

20
Q

Signs of Scleroderma Renal Crisis

A 
HTN (high renin)
MAHA
Thrombocytopenia
Proteinuria
Renal insufficiency/failure
21
Q

Treatment for Scleroderma Renal Crisis

A

ACEi (inhibits the high renin state)

22
Q

When should steroids be used for Systemic Sclerosis

A

Active ILD (Mycophenolate or Cyclophosphamide)

23
Q

GI effects of Scleroderma

A 
Persistent GERD
Dysphagia (dec. peristalsis)
Strictures
Gastric Antral Vascular Ectasia (melena or iron def. anemia)
Gastroparesis
Bloating/Nausea
Constipation
24
Q

Lack of gastric motility or emptying of the the stomach/intestines; can be caused by Scleroderma; associated with a Succussion Splash (fluid sloshing around due to dec. emptying)

A

Gastroparesis

25
Q

Why do we use Carbon Monoxide to assess for Diffusion Capacity (DLCO)

A

Has a higher affinity for hemoglobin, so any difficulty in diffusion (dec surface area or inc. thickness) will be apparent

26
Q

Treatment for Sclerotic ILD

A

Cyclophosphamide
Mycophenolate
Rituximab
Stem-cell transplant

27
Q

MOA for Cyclophosphamide

A

Alkylating agent that interferes DNA replication—> dec. B and T cells

28
Q

MOA for Mycophenolate

A

Inhibition of de-novo purine synthesis–> dec. lymphocyte proliferation

29
Q

MOA for Rituximab

A

Anti-CD20 monoclonal antibody against B-cells

30
Q

Numerical definition for Pulmonary Hypertension

A

> 25 mmHg at rest

31
Q

Treatments for Pulmonary HTN

A

Endothelin receptor antagonists
PDE5 inhibitors
Prostacyclins

MOS (7 decks)

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