Systemic Sclerosis Flashcards
What is Systemic Sclerosis?
Autoimmune inflammatory and fibrotic connective tissue disease, of unknown pathophysiology
What are the two main patterns of disease in systemic sclerosis?
- Limited cutaneous systemic sclerosis
- Diffuse cutaneous systemic sclerosis
Describe the features of limited cutaneous systemic sclerosis.
CREST Calcinosis Raynaud’s phenomenon oEsophageal dysmotility Sclerodactyly Telangectasia
What are the features of diffuse cutaneous systemic sclerosis?
- CREST +
- CVS- HTN and coronary artery disease
- Resp- pulmonary HTN, pulmonary fibrosis
- Renal- glomerulonephritis, scleroderma renal crisis
What is scleroderma?
Hardening of the skin:
- Shiny, tight skin
- Loss of normal folds in the skin
- Most notably on hands and face
What is sclerodactyly?
Skin changes in the hands:
- Restricts ROM of joints and reduces function
- Fat pads on the fingers are lost due to the hardening
- Skin can break and ulcerate
What is telangectasia?
Dilated small blood vessels (veins) that have dilated. They have a fine, threads appearance
What is calcinosis?
Calcium deposits in the skin, most commonly the fingertips
What is Raynaud’s phenomenon?
- Extremities go white and then blue in response to even a mild cold
- Caused by vasoconstriction of vessels supplying the fingers
- Touch hot things with back of forearm
What is oesophageal dysmotility?
- Connective tissue dysfunction of the oesophagus
- Presents as dysphagia, acid reflux and oesophagitis
What is systemic and pulmonary HTN?
High pressure in systemic and pulmonary circulation
What pulmonary fibrosis can be caused by SS?
- Secondary pulmonary fibrosis
- Dry cough and dyspnoea
What is scleroderma renal crisis?
- Acute condition
- severe HTN and renal failure
What autoantibodies are present in systemic sclerosis?
- ANA (but not specific)
- Anti-centromere ABs (limited cutaneous SS)
- Anticoag-Scl 70 ABs (diffuse cutaneous SS, esp. more severe)
What is Nailfold Capillaroscopy?
Technique to magnify and examination the nailfold, to allow examination of the peripheral capillaries. Patients with primary Raynaud’s without SS will have normal capillaries.
