Comparison of primary and secondary Raynaud's phenomenon

• primary RP - generally begins during the teens and early 20's; represents an exaggerated physiological response to cold stimuli (1); not generally associated with any other disease entity
  • symmetrical involvement
  • no tissue necrosis or gangrene
  • ESR normal
  • normal nail fold capillaries
  • negative for anti nuclear antibodies (2,4)
    
    
• secondary RP - the development of RP at an older age (i.e. > 30 years), and particularly in males, suggests the possibility that RP is secondary to some underlying connective tissue disease (CTD) (1). RP occurs in over 90% of patients with systemic sclerosis and in up to 40% of patients with systemic lupus erythematosus and idiopathic inflammatory myositis(1). Features that make a secondary cause of RP more likely include:
  • digital ulceration, or year-round symptoms
  • pain and discomfort is higher in severity
  • abnormal nail fold capillaries (viewed with an ophthalmoscope with the +20 lens)
  • assymetric upper limb pulses or bruits
  • puffiness/tightness of the finger skin
  • a raised ESR
  • positive ANA or other antibodies (Ro/La/Scl-70 etc)
  • clinical features suggestive of an underlying connective tissue disease (3,4)

Reference:

• (1) Raynaud’s & Scleroderma association. Raynaud’s & Scleroderma – An update for GP’s.
• (2) Wigley FM. Raynaud’s phenomenon. NEJM 2002;347:1001-1008
• (3) Sunderkötter C, Riemekasten G. Pathophysiology and clinical consequences of Raynaud's phenomenon related to systemic sclerosis. Rheumatology 2006;45(3):iii33-iii35
• (4) Goundry B, Bell L, Langtree M, Moorthy A. Diagnosis and management of Raynaud's phenomenon. BMJ. 2012;344:e289