Systemic sclerosis Flashcards
1
Q
What is systemic sclerosis?
A
- an uncommon connective tissue disease that is characterised by fibrosis of the skin and internal organs, pronounced alterations in the microvasculature and frequent cellular and humoral abnormalitis
2
Q
What group of diseases does systemic sclerosis fit into?
A
Scleroderma
3
Q
Two major categories of scleroderma
A
- localised scleroderma
- systemic sclerosis
4
Q
2 subsets of systemic sclerosis
A
- limited systemic sclerosis (distal to elbows, knees and face)
- diffuse systemic sclerosis (proximal to knees and elbows)
5
Q
Difference in presentation of lcSSc and dcSSc
A
- lcSSc usually starts with Rauynauds for years before progression
- dsSSc usually progresses rapidly
6
Q
Features of limited systemic sclerosis
A
- limited skin involvement
- isolated pulmonary HPT
- no kidney involvement
- anti-centromere antibody
- long duration of Raynaud’s
7
Q
Features of diffuse systemic sclerosis
A
- diffuse skin involvement
- severe interstitial lung disease
- kidney involvement
- anti-topoisomerase antibody
- short duration of Raynaud’s
8
Q
What does CREST stand for?
A
Calcinosis, Raynauds, Esophageal dysmotility, sclerodactyly, Telangiectasia
9
Q
What is SSc sine scleroderma?
A
- small number of patients who demonstrate vascular and serological features of SSc without any skin involvement
10
Q
Pathogenesis of systemic sclerosis
A
- endothelial cell apoptosis and dysfunction and immune activation
- release of pro-fibrotic cytokines resulting in cytokine activation of fibroblasts to increase extracellular matrix production
- results in deposition of excess fibrosis on the extracellular matrix
11
Q
Vascular abnormalities assocaited with systemic sclerosis
A
- Raynaud’s
12
Q
3 stages of skin changes in systemic sclerosis
A
- inflammatory
- progressive skin fibrosis
- skin softening
13
Q
GIT manifestations of systemic sclerosis
A
- microstomia
- xerostomia
- dysphagia
- GORD
- Barrett’s oesophagus
- GI bleeding
- GAVE
- intestinal dysmotility
- malabsorption
- rectal prolapse and rectal incontinence
14
Q
Lung disease associated with systemic sclerosis
A
- interstitial lung disease
- pulmonary hypertension
- aspiration pneumonia
- endobronchial telangiectasia with hemoptysis
15
Q
Cardiac manifestations of systemic sclerosis
A
- pericardial disease
- left or right ventricular systolic dysfunction
- arrhythmias
