Systemic Sclerosis Flashcards
What is systemic sclerosis?
Multi system autoimmune connective tissue disease
Involving inflammation and + increased fibroblast activity > fibrosis of connective tissue, skin + internal organs
Patterns of systemic sclerosis
Limited cutaneous SSc
Diffuse cutaneous SSc
What is limited cutaneous systemic sclerosis
CREST syndrome
- Calcinosis cutis
- Raynaud’s phenomenon
- Eosphageal dysmobility
- Sclerodactyly
- Telangiectasia
What is calcinosis cutis?
Calcium deposits under the skin
Commonly on fingertips
What is oesophageal dysmobility?
- Caused by atrophy + dysfunction of smooth muscle + fibrosis of oesophagus
- causing swallowing difficulties, chest pain, acid reflux + oesophagitis
What is sclerodactyly?
- Skin changes in the hands
- Skin tightening around the joints restricts the ROM + reduces function
- Fat pads on the fingers are lost
- skin can break and ulcerate
What is telangiectasia?
Dilated blood vessels in skin <1mm diameter
What is diffuse systemic sclerosis?
Includes CREST features + also affects internal organs:
- CV problems e.g. hypertension + contrary artery disease
- lung problems e.g. pulmonary hypertension + fibrosis
- kidney problems e.g. scleroderma adrenal crisis
What is scleroderma renal crisis?
Medical emergency with severe hypertension + renal failure
What autoantibodies are associated with systemic sclerosis?
- ANA
- anti-centromere antibodies in limited SSc
- anti-Scl-70 antibodies in diffuse SSc
Investigations of systemic sclerosis
- X-ray hands
- ECG + ECHO
- antibodies: ANA, anti-centromere, anti-scl-70
- nailfold capillaroscopy
Non medical managment of systemic sclerosis
- avoid smoking
- regular emollients
- gentle skin stretching to maintain ROM
- avoid cold triggers for Raynaud’s
- physio
Medical management of systemic sclerosis
- nifedipine for raynaud’s
- methotrexate to reduce skin thickening
- ACEi to prevent hypertensive crisis + reduced mortality from renal failure
- analgesia
- PPI for gastro symptoms
- prednisolone short course for flares
