Scleroderma Flashcards
What is Scleroderma?
Multisystem autoimmune disease characterised by skin sclerosis as well as systemic sclerosis
Two main subtypes- limited cutaneous and diffuse
What is the pathology?
Perivascular fibrosis causes ischaemic changes in surrounding tissues
Skin most commonly affected (hard and shiny)
Can affect heart, lungs, kidneys and oesophagus
What are features of limited cutaneous scleroderma?
Also known as CREST syndrome
Calcinosis in finger tips
Raynauds phenomenon
Oesophageal dysmotility- swallowing difficulties
Sclerodactyly- skin changes in hand
- skin tightens affecting range of joint movement
- skin can break and ulcerate
Telangiectasia
Fatigue
Late symptoms/complications
- oesophageal strictures
- small bowel malabsorption
- pulmonary fibrosis/HTN
What are the clinical features of diffuse cutaneous scleroderma?
Short history or Raynauds Rapidly progressing skin sclerosis Weight loss Fatigue Anorexia
Affects many internal organs
- CV= HTN and coronary artery disease
- Lung= pulmonary HTN and fibrosis
- Kidney= glomerulonephritis and scleroderma renal crisis
What investigations are needed?
Limited
-anti centromere antibodies +ve in 60%
Diffuse
- Anti Scl 70 antibodies +ve in 40%
- RNA polymerase antibodies present
What is the management?
Limited
- digital sympathectomy- cut small nerves to arteries that supply fingers (Raynauds)
- Vasodilators for symptoms e.g. nifedipine
- removal of calcinoses
- treatment of oesophageal problems e.g. antacids
Diffuse
- immunosuppression to prevent complications
- use treatment for limited also
Conservative
- avoid smoking
- gentle skin stretching
- regular emollients
- avoid cold triggers
- MDT
ACEi for HTN
Analgesia for joint pain
What is the prognosis?
Highest mortality rate of all autoimmune rheumatic diseases
Limited has higher survival rate due to less organ involvement
