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Raynaud Phenomenon

BASICS

  • Definition: Idiopathic, intermittent vasoconstriction of digital arteries, arterioles, and cutaneous shunts in response to cold, stress, or trauma, producing triphasic color changes (white → blue → red).
  • Types:
  • Primary: Bilateral, nonprogressive; 80% of cases; no underlying disease after 2 years.
  • Secondary: Associated with connective tissue diseases; progressive/asymmetric, more severe, may lead to ischemic changes.

EPIDEMIOLOGY

  • Primary: Age of onset ~14 years, more common in females (4:1); prevalence 6–20% in women, 3–12% in men.
  • Secondary: Onset >40 years, no sex predilection; ~1% prevalence.

ETIOLOGY AND PATHOPHYSIOLOGY

  • Imbalance in vasodilation/vasoconstriction: ↑ Endothelin-1, ↓ endothelin-dependent vasodilation.
  • Secondary: platelet/blood viscosity abnormalities, 5-HT2 serotonin receptor involvement.
  • Genetics: Dominant inheritance possible; ¼ of primary cases have affected 1st-degree relative.

RISK FACTORS

  • Autoimmune/connective tissue disease
  • ESRD with AV shunt (steal phenomenon)
  • Elevated homocysteine (both types)
  • Smoking (worsens symptoms, not a cause)
  • Stress, cold, anxiety as triggers

ASSOCIATED CONDITIONS

  • Secondary Raynaud: Scleroderma, SLE, polymyositis, Sjögren syndrome, occlusive vascular disease, cryoglobulinemia

CLINICAL PRESENTATION

HISTORY

  • Primary: Symmetric finger attacks, FHx of connective tissue disease, no ulcer/gangrene, normal after ≥2 yrs.
  • Secondary: Onset >40, asymmetric/severe attacks, arthritis, myalgias, systemic symptoms, medication/toxin exposure.

PHYSICAL EXAM

  • Triphasic color change: White (pallor), blue (cyanosis), red (hyperemia).
  • Thumbs typically spared.
  • Primary: Normal exam, normal nailbed capillaries.
  • Secondary: Skin changes, digital ulcers, abnormal capillaries (giant loops, avascular areas), ischemic changes.

PEDIATRICS/GERIATRICS

  • Children: associated with SLE, scleroderma
  • Onset after age 40: suspect underlying disease

DIAGNOSTIC TESTS & INTERPRETATION

  • Primary: ANA negative, ESR normal.
  • Secondary: CBC, ESR, autoantibodies (anticentromere, antitopoisomerase), nailfold videocapillaroscopy (gold standard).
  • Diagnosis is clinical; provocative (cold) testing not needed.
  • Rule out secondary causes with appropriate labs.

DIFFERENTIAL DIAGNOSIS

  • Thromboangiitis obliterans (Buerger)
  • Rheumatoid arthritis, scleroderma, SLE, CREST syndrome
  • Carpal tunnel, thoracic outlet syndrome
  • Hypothyroidism, acrocyanosis, polycythemia
  • Drug/vibration/occupational induced

TREATMENT

GENERAL MEASURES

  • Dress warmly, avoid cold, wear gloves/mittens
  • Avoid triggers (cold, trauma, vibration, stress, smoking)
  • Windmill arm movements, warm water for relief
  • Tobacco cessation
  • Avoid β-blockers, amphetamines, ergot alkaloids, OTC pseudoephedrine, sumatriptan

MEDICATION

  • First Line: Calcium channel blockers (nifedipine SR 30–180 mg/day); effective for 75% (seasonal use ok)
  • Alternatives: Amlodipine, nicardipine, losartan, fluoxetine, PDE5 inhibitors (sildenafil), parenteral iloprost
  • Topical: Nitroglycerin gel/patch, topical sildenafil (secondary cases)
  • Others: Prazosin, botulinum toxin (evidence for severe/refractory), aspirin for digital ischemia

SECOND LINE & ADDITIONAL

  • Finger guards for ulcers
  • Anticoagulation (short term) for critical ischemia
  • Digital/wrist block for pain control
  • Surgical sympathectomy (rare, temporary)
  • Digital fat grafting (experimental for symptom relief)
  • Complementary: Ginkgo, fish oil, vitamin D (if deficient), evening primrose oil, acupuncture (evidence weak)

REFERRAL

  • Suspect/confirm secondary disease: refer to rheumatology

ONGOING CARE

  • Prevent cold exposure, manage stress
  • Monitor for progression to secondary disease
  • Manage/monitor digital ulcers, infection risk

PROGNOSIS

  • Most attacks brief and self-limited; ~13% develop secondary disease
  • Severe complications (gangrene, autoamputation) rare, seen in secondary disease

CLINICAL PEARLS

  • Raynaud is a clinical diagnosis; provocative testing is unnecessary
  • First presentation >40 years old or digital ulcers: investigate for secondary cause
  • Ulcers or digital ischemia are always abnormal and need workup
  • Acute digital ischemia is a vascular emergency

ICD-10 Codes

  • I73.00: Raynaud's syndrome without gangrene
  • I73.01: Raynaud's syndrome with gangrene