Description

• Idiopathic intermittent episodes of vasoconstriction of digital arteries, precapillary arterioles, and cutaneous arteriovenous shunts in response to cold, emotional stress, or blunt trauma
  • A triphasic color change of the fingers (occasionally the toes, rarely nipples) is the principal physical manifestation.
    • The initial color is white from extreme pallor, then blue from cyanosis, and finally with warming/vasodilatation, the skin appears red.
    • Thumbs are rarely involved.
  • Swelling, throbbing, and paresthesias are associated symptoms.
  • Primary
    • 80% of patients have primary disease.
    • Episodes are bilateral and nonprogressive.
    • Diagnosis confirmed if after 2 years of symptoms, no underlying connective tissue disease develops
  • Secondary
    • Progressive and asymmetric
    • Vascular spasm is more frequent and more severe over time. Ulceration is rare; gangrene does not develop; 13% progress to digital fat pad atrophy and ischemic fingertip changes.
    • Typically associated with an underlying connective tissue disorder
• System(s) affected: hematologic, lymphatic, immunologic, musculoskeletal, dermatologic, exocrine

Pregnancy Considerations

• Raynaud phenomenon can appear as breast pain in lactating women.
• Positive breast milk bacterial culture distinguishes mastitis from Raynaud phenomenon.

Geriatric Considerations
Initial appearance of Raynaud phenomenon after age 40 years suggests underlying connective tissue disease.

Pediatric Considerations
Associated with systemic lupus erythematosus (SLE) and scleroderma

Epidemiology

Incidence

• Primary
  • Predominant age: 14 years; ~1/4 begin >40 years
  • Predominant sex: female > male (4:1)
• Secondary
  • Predominant age: >40 years
  • Predominant sex: no gender predilection

Prevalence

• Primary: 3–12% of men; 6–20% of women (based on clinical history)
• Secondary: ~1% of population

Etiology and Pathophysiology

Unknown. Dysregulation of vascular control mechanisms leads to imbalance between vasodilation and vasoconstriction. There is a reduced endothelin-dependent vasodilation activity and an increased vasoconstriction in peripheral vessels by overproduction of endothelin-1. 5-HT₂ serotonin receptors may be involved in secondary Raynaud phenomenon. Platelet and blood viscosity abnormalities in secondary disease contribute to ischemic pathology.

Genetics
Some studies suggest dominant inheritance pattern. ~1/4 of patients with primary condition also have a first-degree relative with Raynaud phenomenon.

Risk Factors

• Existing autoimmune or connective tissue disorder
• End-stage renal disease with hemodialysis may increase risk if a steal phenomenon develops in association with the arteriovenous shunt.
• Primary and secondary disease associated with elevated homocysteine levels
• Smoking is not associated with increased risk of Raynaud phenomenon but may worsen symptoms.

General Prevention

• Avoid cold exposure.
• Tobacco cessation
• No relationship has been established between Raynaud phenomenon and vibratory tool use.
• Stress and anxiety can trigger attacks.

Commonly Associated Conditions

Secondary Raynaud

• Scleroderma; SLE; polymyositis
• Sjögren syndrome; occlusive vascular disease
• Cryoglobulinemia