Systemic Sclerosis Flashcards
What are the two types of systemic sclerosis?
Limited/Cutaneous= Scleroderma (formerly called CREST) Diffuse= Systemic sclerosis
What are the features of the limited type/cutaneous sclerosis ?
This was previously called crest syndrome
It just affects the hands, feet and face
Calcinosis Raynauds Esophageal dysmotility Sclerodactyly Telangiectasia
What antibody is associated with limited sclerosis?
Anti-centromere
Think mere- it’s merely limited disease
What is an important complication or development of limited scleroderma?
Pulmonary hypertension which can become life threatening
What is the treatment for pulmonary hypertension in limited systemic sclerosis?
Sildenafil
Bosetin
What does sclerodactyly look like?
Inability to fully extend fingers
The ischaemia created due to inflammation can cause digital ulcers to form.
Where does diffuse systemic sclerosis affect?
The whole body- it causes scleroderma and fibrosis of the internal organs
What antibody is systemic sclerosis?
Anti-SCL 70
What is the management for systemic sclerosis?
Immunosuppression
Steroids and steroid sparing regimes
E.g. Methotrexate, Azathioprine, cyclophosphamide, Rituximab (Anti CD20/Anti B Cell)
Anti-fibrotic agents- under investigation for pulmonary fibrosis
Manage symptoms
Raynaud’s- Nifedipine
GORD- Omeprazole
PHTN- Sildenafil, Bosetin