Autoimmune connective tissue diseases

Question 1

What is systemic sclerosis?

Answer 1

• Systemic scleroderma, also called diffuse scleroderma or systemic sclerosis, is an autoimmune disease of the connective tissue.
• It is characterized by thickening of the skin caused by accumulation of collagen, and by injuries to small arteries.

Question 2

How is systemic sclerosis investigated?

Answer 2

• ANA antibody positive
• Anti-centromere antibodies

Question 3

Systemic sclerosis skin disease is either limited or diffuse. What are the differences between both?

Answer 3

• Limited
  
  • Face, hands and feet
  • ANCA and ANA positive
  • Pulmonary hypertension
• Diffuse
  
  • Whole body is affected
  • Antitopoisomerase antibodies and anti-RNA polymerase

Question 4

How is limited systemic sclerosis treated?

Answer 4

• Sildenafil
• Bosentan

Question 5

How is diffuse systemic sclerosis treated?

Answer 5

• Control BP meticulously
• Perform annual acho and spirometry

Question 6

What are the complications of systemic sclerosis?

Answer 6

• Organ fibrosis
  
  • Renal
  • GI
  • Lung
  • Heart

Question 7

How is systemic sclerosis managed?

Answer 7

• No cure
• Immunosuppressive regimens
  
  • Cyclophosphamide
• Monitor BP and renal function
  
  • Regular ACEi or ARBs decrease renal crisis

Question 8

What are Reynaud’s?

Answer 8

• Raynaud’s disease causes some areas of your body — such as your fingers and toes — to feel numb and cold in response to cold temperatures or stress.
• In Raynaud’s disease, smaller arteries that supply blood to your skin narrow, limiting blood circulation to affected areas (vasospasm).
• The cold extremities go from white to blue to red

Question 9

How is Reynaud’s treated?

Answer 9

• Stop smoking
• Gloves
• Hand warmers
• Vasodilators
  
  • Calcium channel blockers
    
    • Nifedipine
    • Amlodipine
    • Diltiazem
  • Evening primrose oil