Scleroderma

Question 1

What is Scleroderma?

Answer 1

Multisystem autoimmune disease characterised by skin sclerosis as well as systemic sclerosis

Two main subtypes- limited cutaneous and diffuse

Question 2

What is the pathology?

Answer 2

Perivascular fibrosis causes ischaemic changes in surrounding tissues
Skin most commonly affected (hard and shiny)
Can affect heart, lungs, kidneys and oesophagus

Question 3

What are features of limited cutaneous scleroderma?

Answer 3

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

Question 4

What are the clinical features of diffuse cutaneous scleroderma?

Answer 4

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 (HTN and acute renal failire)

Question 5

What investigations are needed?

Answer 5

Limited
-anti centromere antibodies +ve in 60%

Diffuse

• Anti Scl 70 antibodies +ve in 40%
• RNA polymerase antibodies present

Nailfold capillaroscopy

• looks at capillaries in the nails
• in secondary Raynauds (due to SS)
• -abnormal capillaries
• -avascular areas
• -microhaemorrhages

Question 6

What is the management?

Answer 6

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

Question 7

What is the prognosis?

Answer 7

Highest mortality rate of all autoimmune rheumatic diseases

Limited has higher survival rate due to less organ involvement