Systemic Sclerosis

Question 1

What is SS?

Answer 1

• Autoimmune inflammatory and fibrotic connective tissue disease

Question 2

What are the two types of SS?

Answer 2

• Limited Cutaneous Systemic Sclerosis (more common)
  
  • Skin affected: face, forearms, lower legs up to knee
• Diffuse Cutaneous Systemic Sclerosis
  
  • Skin affected: upper arms, thighs, trunk

Question 3

What are the features of LcSSc?

*remember CREST syndrome

Answer 3

• Calcinosis
• Raynaud’s disease
• Esophageal dysmotility
• Sclerodactyly
• Telangiectasia

Question 5

What are the features of DC

cSSc?

Answer 5

• CREST + Internal organs
  
  • CVS: HTN, CAD
  • Lungs: Pulmonary HTN & Pulmonary Fibrosis
  • Kidneys: Glomerulonephritis, scleroderma renal crisis

Question 6

What are the cardinal features of SSc

Answer 6

• Excessive collagen production and deposition
• Vascular damage
• Immune system activation via autoantibody production and cell-mediated autoimmune mechanism

Question 7

What are the features of SSc?

Answer 7

• Scleroderma - hardening of skin
• Sclerodactyly - skin tightening around joints
• Telangiectasia
• Calcinosis
• Raynaud’s phenomenon
• Oesophageal dysmotility
• Systemic and pulmonary HTN
• Pulmonary Fibrosis
• Scleroderma renal crisis
• Myalgia, joint pain

*basically all systems are affected. Refer patientinfo for full list

Question 8

What Ix would you order for SSc?

Answer 8

• Bloods
  
  • FBC
  • ESR
  • CRP
  • U&E
• Autoantibodies
  
  • ANA - sensitive but not specific
  • Specific
    
    • Anti-centromere antibodies - LcSSc
    • scl-70 antibodies - DcSSc
• Nailfold capillaroscopy

Question 10

What features will you find in nailfold capillaroscopy?

Answer 10

• Abnormal capillaries
• Avascular areas
• Micro haemorrhages

Question 11

What are the diagnostic crietria for SSc?

*must be equal to or > 9

Answer 11

• Skin thickening extending proximal to the MCP joints (score 9).
• Skin thickening of the fingers (score 2 for puffy fingers, 4 for sclerodactyly).
• Fingertip lesions (score 2 for ulcers, 3 for fingertip pitting scars).
• Telangiectasia (score 2).
• Abnormal nailfold capillaries (score 2).
• Pulmonary arterial hypertension and/or interstitial lung disease (score 2).
• Raynaud’s phenomenon (score 3).
• SSc-related autoantibodies (score 3

Question 12

What are the differential diagnosis for SSc?

Answer 12

• Raynaud’s phenomenon from other causes
• Vibration injury
• RA or SLE
• Amyloidosis
• Chronic graft vs host disease
• Paraneoplastic syndrome

Question 13

How would you mx SSc non-pharmalogically?

Answer 13

• Avoid smoking
• Gentle skin stretching to maintain the range of motion
• Regular emollients
• Avoiding cold triggers for Raynaud’s
• Physiotherapy to maintain healthy joints
• Occupational therapy for adaptations to daily living to cope with limitations

Question 14

How would you mx SSc medically?

Answer 14

*no standardised tx for SSc yet.

• Nifedipine - Raynaud’s phenomenon
• Anti acid medications (e.g. PPIs) and pro-motility medications (e.g. metoclopramide) for gastrointestinal symptoms
• Analgesia - joint pain
• Antibiotics - skin infections
• Antihypertensives can be used to treat hypertension (usually ACE inhibitors)
• Treatment of pulmonary artery hypertension
• Supportive management of pulmonary fibrosis