Systemic Sclerosis

Question 1

What is systemic sclerosis?

Answer 1

Autoimmune inflammatory and fibrotic connective tissue disease with unknown cause. Affects skin in all areas and can also internal organs (subtype dependent)

**Passmed “condition of unknown aetiology characterised by h**ardened, sclerotic skin and other connective tissues.”

Question 2

State the three main disease patterns in systemic sclerosis

Answer 2

• Limited cutaneous systemic sclerosis
• Diffuse cutaneous systemic sclerosis
• Scleroderma

Question 3

For limited cutaneous systemic sclerosis, discuss:

• What it used to be called (this is now recognised as a subtype)
• Features

Answer 3

• CREST syndrome
• Features:
  
  • Calcinonsis
  • Raynaud’s phenomenom
  • Oesophageal dysmotility
  • Sclerodactyly
  • Telangiectasia

Question 4

Describe the features of diffuse cutaneous systemic sclerosis/describe how it differs from limited cutaenous systemic sclerosis

Answer 4

Includes features of CREST syndrome plus more likley to have many internal organ problems:

• Cardiovascular: hypertension, coronary artery disease
• Lung problems: pulmonary hypertension, pulmonary fibrosis
• Kideny problems: glomerulonephritis, scleroderma renal crisis

Question 5

Compare which areas of skin are affected in limited cutaneous and diffuse cutaneous scleroderma

Answer 5

Question 6

Describe each of the following:

• Scleroderma
• Slcerodactyly
• Telangiectasia
• Calinosis
• Raynauds
• Oesophageal dismotility

Answer 6

• Scleroderma: hardening of skin; appears shiny, tight, without normal folds
• Slcerodactyly: skin changes in hands; tighten around joints restricting motion and eventually fat pads on fingers are lost
• Telangiectasia: dilated tiny blood vessels in skin
• Calcinosis: calcium deposits under skin- commonly fingertips
• Raynauds: vasospasm in digits (white, blue, red)
• Oesophageal dismotility: swallowing difficulties, acid reflux, oesophagitis

Question 7

What is a scleroderma renal crisis?

Answer 7

Combination of severe hypertension & renal failure

Question 8

There are lots of autoantibodies in systemic slcerosis which are helpful in predicting extent of disease and which organs will be affected. State some of key antibodies to know

Answer 8

• Anti-nuclear antibodies (positive in most pts)
• Anti-centromere antibodies (most associated with limited cutaneous)
• Anti-Scl-70 (most associated with diffuse cutaenous & more severe disease)

Question 9

How is systemic slcerosis diagnosed?

Answer 9

Use ACR and EULAR criteria; must meet a number of criteria including clinical features, antibodies and nail fod capillaroscopy findings

Question 10

What is nailfold capillaroscopy?

What findings support a diagnosis of systemic slcerosis?

Answer 10

• Nailfold (where skin meets nail) is magnified and examined
• Systemic sclerosis findings:
  
  • Abnormal capillaries
  • Avascular areas
  • Micro-haemorrhages

Question 11

Why do we do nail-fold capillaroscopy in Raynaud’s?

Answer 11

To exlcude systemic sclerosis; pts with primary Raynaud’s without systemic sclerosis will have normal nailfold capillaries.

Question 12

Discuss the conservative/non-medical management for systemic sclerosis

Answer 12

• Smoking cessation
• Gentle skin stretching
• Regular emmollients
• Avoiding cold triggers for Raynauds
• Physiotherapy
• Occupational therapy

Question 13

Discuss the medical management of systemic sclerosis, think about management of:

• Diffuse disease with complications
• Raynaud’s
• GI symptoms
• Joint pain
• Skin infections
• Pulmonary fibrosis
• Hypertension

Answer 13

Steroids & immunosupressants for diffuse disease with complications.

Other treatments:

• Nifedipine for Raynaud’s
• Oesophageal dismotility:
  
  • PPIs
  • Pro-motility agents e.g. metoclopramide
• Joint pain
  
  • Analgesia
• Skin infections
  
  • Abx
• Pulmonary fibrosis
  
  • Supportive e.g. oxygen
• Hypertension
  
  • Antihypertensives (usually ACE inhibitors)