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Musculoskeletal > Systemic Sclerosis > Flashcards

Systemic Sclerosis Flashcards

1
Q

Define Systemic Sclerosis?

A

Rare connective tissue disease characterised by widespread small blood vessel damage and fibrosis in skin and internal organs

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2
Q

What is Systemic Sclerosis also known as?

A

Scleroderma

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3
Q

What is the spectrum of diseases in Systemic Sclerosis?

A

Pre-Scleroderma
Diffuse Cutaneous Systemic Sclerosis (40%)
Limited Cutaenous Systemic Sclerosis (60%)
Scleroderma Sine Scleroderma

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4
Q

What are the characteristics of Pre-Scleroderma?

A

Raynaud’s phenomenon
Nail-fold capillary changes
Antinuclear antibodies

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5
Q

What are the characteristics of Diffuse Cutaneous Systemic Sclerosis?

A 
Raynaud's phenomenon
Followed by skin changes with truncal involvement 
Tendon friction
Joint contracture
Early lung disease 
Heart, GI and renal disease 
Nail-fold capillary dilatation
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6
Q

What was Limited Cutaneous Systemic Sclerosis previously known as?

A

CREST Syndrome because of its five characteristic features

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7
Q

What are the 5 characteristic features of Limited Cutaneous Systemic Sclerosis?

A 
CREST
Calcinosis 
Raynaud's phenomenon
(O)esophageal dysmotility 
Sclerodactyly
Telangiectasia
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8
Q

What is Scleroderma sine Scleroderma?

A

Internal organ disease with NO skin changes

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9
Q

What is the aetiology of Systemic Sclerosis?

A

Genetic and environmental factors
Pathogenesis is unclear
Activated monocytes, macrophages and lymphocytes may interact with Endothelial Cells and Fibroblasts

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10
Q

What happens when Activated Monocytes, macrophages and lymphocytes interact with endothelial cells in Systemic Sclerosis?

A

Endothelial Cell Damage
Platelet activation
Narrowing of blood vessels

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11
Q

What happens when Activated Monocytes, macrophages and lymphocytes interact with Fibroblasts in Systemic Sclerosis?

A

Lay down collagen in the dermis

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12
Q

What is the epidemiology of Systemic Sclerosis?

A

Age of onset: 30-60 yrs

3x more common in FEMALE

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13
Q

What are the presenting symptoms and signs of Systemic Sclerosis on the skin?

A

Raynaud’s Phenomenon

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14
Q

What are the presenting symptoms and signs of Systemic Sclerosis on the Hands?

A

Initially swollen painful fingers
Later, they become thickened, tight, shiny and bound to underlying structures
Changes in pigmentation
Finger ulcers

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15
Q

What are the presenting symptoms and signs of Systemic Sclerosis on the Face?

A

Microstomia (puckering of the skin around the mouth)

Telangiectasia

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16
Q

What are the presenting symptoms and signs of Systemic Sclerosis in the Lungs?

A

Pulmonary fibrosis which leads to pulmonary hypertension

17
Q

What are the presenting symptoms and signs of Systemic Sclerosis on the heart?

A

Pericarditis or pericardial effusion
Myocardial fibrosis
Heart failure
Arrhythmias

18
Q

What are the presenting symptoms and signs of Systemic Sclerosis in the GI?

A

Dry mouth
Oesophageal dysmotility
Reflux Oesophagitis
Gastric paresis

19
Q

What are the presenting symptoms and signs of Systemic Sclerosis on the Kidneys?

A

Hypertensive renal crisis

Chronic renal failure

20
Q

What are the neuromuscular presenting symptoms and signs of Systemic Sclerosis?

A

Trigeminal neuralgia
Muscular wasting
Weakness

21
Q

What are the orher presenting symptoms and signs of Systemic Sclerosis?

A

Hypothyroidism

Impotence

22
Q

What investigations do we do for Systemic Sclerosis?

A 
Autoantibodies 
Lung investigations
Heart investigations
GI investigations
Kidney investigations 
Neuromuscular investigations 
Joint investigations 
Skin investigations
23
Q

What autoantibodies do we look for in Systemic Sclerosis?

A

Antinuclear
Anti-centromere (70% of limited cutaneous systemic sclerosis cases)
Anti-topoisomerase II (anti-Scl-70) - 30% of diffuse cutaenous systemic sclerosis cases
Anti-nucleolar
Anti-RNA polymerase

24
Q

What investigations do we do for the Lungs for Systemic Sclerosis?

A

CXR
Pulmonary function tests
CT scan

25
Q

What investigations do we do for the heart in Systemic Sclerosis?

A

ECG

Echocardiography

26
Q

What investigations do we do for the GI in Systemic Sclerosis?

A

Endoscopy

Barium studies

27
Q

What investigations do we do for the Kidneys in Systemic Sclerosis?

A

U&Es

Creatinine clearance

28
Q

What neuromuscular investigations do we do for Systemic Sclerosis?

A

Electromyography

Biopsy

29
Q

What investigations do we do for Joints in Systemic Sclerosis?

A

Radiography

30
Q

What investigations do we do for the Skin in Systemic Sclerosis?

A

Biopsy (rarely needed)

Musculoskeletal (21 decks)

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