Systemic Sclerosis Flashcards
1
Q
Definition of systemic sclerosis
A
- Autoimmune inflammatory and fibrotic connective tissue disease
- Two main patterns of disease:
- Limited cutaneous systemic sclerosis
- Diffuse cutaneous systemics sclerosis
2
Q
Features of limited cutaneous systemic sclerosis (remember CREST)
A
- C – Calcinosis (calcium build up under the skin - usually on fingertips)
- R – Raynaud’s phenomenon (fingertips go blue in response to cold - caused by vasoconstriction)
- E – oEsophageal dysmotility
- S – Sclerodactyly (skin changes in hands, tightening of skin reducing function and ROM in joints)
- T – Telangiectasia (dilated small blood vessels in skin)
3
Q
Features of diffuse cutaneous systemic sclerosis
A
- CV problems (HTN, CAD)
- Lung problems (pulmonary HTN, pulmonary fibrosis)
- Kidney problems (GN, scleroderma renal crisis)
4
Q
Autoantibodies
A
- Antinuclear antibodies (ANA) are positive in most
- Anti-centromere antibodies mostly associated with LCSS
- Anti-Scl-70 antibodies mostly associated with DCSS
5
Q
Nailfold Capillaroscopy
A
- Area where skin meets base of fingernail - magnified and examined
- Abnormal capillaries, avascular areas and micro-haemorrhages indicate systemic sclerosis
- Those wiith primary Raynaud’s without systemic sclerosis will have normal nailfolds
6
Q
Diagnosis of systemic sclerosis
A
- Clinical features
- Antibodies
- Nailfold capillaroscopy
7
Q
Management of systemic sclerosis
A
- Managed by specialist MDT
- Non-medical management
- Acoid smoking
- Gentle skin stretching
- Regular emollients
- Avoiding cold triggers for Raynaud’s
- Physiotherapy for joints
- Occupational therapy
- Medical management
- Nifedipine for Raynaud’s
- Anti-acid and pro-motility medications for GI symptoms
- Analgesia
- Antibiotics for skin infections
- Antihypertensives
- Treatment of pulmonary artery HTN
- Supportive management for pulmonary fibrosis
