Raynaud’s Phenomenon Disease

DESCRIPTION

Bilaterally occurring vasospastic disorder manifested by intermittent attacks of extreme pallor, then cyanosis of the fingers (rarely, of the toes) brought on by cold exposure. With warming, vasodilatation and intense redness develops, followed by swelling, throbbing, paresthesias. Resolves with warming. May accompany emotional upset. Thumbs rarely involved. 13% may progress to atrophy of digital fat pads, ischemic ulcers of fingertips. 50% idiopathic or primary Raynaud's disease, 50% secondary (Raynaud's phenomenon).

Disease: progressive, symmetrical. Involves fingers; rarely toes. Ages 15 to 45. Spasm is more frequent, more severe with time. No gangrene; rarely ulcerates. Diagnose only after 2 years if no underlying associated disease. May be associated with coronary vasospasm or primary pulmonary hypertension. No histologic abnormality found in digital arteries.
Phenomenon: may be unilateral, asymmetric; may affect only one or two fingers. Underlying condition usually identifiable with time. Usually has a worse morbidity and prognosis than primary disease.
System(s) affected: Skin/Exocrine, Musculoskeletal, Hemic/Lymphatic/Immunologic
Genetics: Little information available, but some suggest a dominant inheritance pattern. One study suggests there is a 5-fold increase in Raynaud's in family members of a Raynaud's patient. (Arthritis Rheum 1996; 39:1189-91)
Incidence/Prevalence in USA: 4 to 10% of population (based on reporting of characteristic color changes, cold intolerance)
Predominant age: After 40
Predominant sex:
- Female > Male (4:1 in primary form)
- Male = Female (secondary form)

SIGNS AND SYMPTOMS

Pallor/whiteness of fingertips with cold exposure, followed by cyanosis, then redness and pain with warming
Ulceration of finger pads, progressing to autoamputation in severe, prolonged cases (10-13% of cases)
Normal physical exam in primary; may show signs of underlying vasospastic or autoimmune disease in secondary form

CAUSES

Unknown. May involve increased sensitivity of alpha-2-adrenergic receptors in digital vessels in primary type. Serotonin receptors (5-HT2 type) may be involved in secondary Raynaud's. Platelet and blood viscosity abnormalities also implicated.

RISK FACTORS

Smoking (men only)
Existing autoimmune or connective tissue disorder
Alcohol use (women only)
Unopposed estrogen therapy associated with higher incidence in postmenopausal women; not seen with combined hormone therapy
Helicobacter pylori infection may contribute; some patients improve after eradication

LABORATORY

Tests for underlying secondary causes (CBC, ESR, RA, ANA, immunoelectrophoresis, esophageal motility studies)
Drugs that may alter lab results: N/A
Disorders that may alter lab results: N/A

PATHOLOGICAL FINDINGS

Histology of skin biopsy correlates poorly with clinical presentation. May show edema, necrotizing or non-necrotizing vasculitis, and/or perivasculitis.

SPECIAL TESTS

Cold challenge test – elicits characteristic color changes (pallor → cyanosis → redness) in fingers/hands
Nailfold capillaroscopy – detects enlarged or irregular capillary loops; normal in primary Raynaud's, abnormal in secondary forms associated with connective tissue disease

IMAGING

Rarely, cases may demonstrate osteolysis of distal metaphyseal portions of phalanges, tapering and calcification of soft tissues

DIAGNOSTIC PROCEDURES

Diagnosis largely determined by history, provocative exposure to cold

APPROPRIATE HEALTH CARE

Outpatient management usually sufficient

GENERAL MEASURES

Keep warm: dress warmly, wear gloves, avoid cold exposure
Avoid smoking
Avoid vasoconstrictive drugs: beta-blockers, amphetamines, ergot alkaloids, sumatriptan
Biofeedback techniques: teach patients to increase hand temperature
Protect ulcerated fingertips: use finger guards

SURGICAL MEASURES

Cervical sympathectomy effect is transient - symptoms return in 1 to 2 years

ACTIVITY

Avoidance of situations in which exposure to cold is likely; avoid vibrating tools

DIET

No special diet

PATIENT EDUCATION

Emphasis on smoking cessation. Avoidance of aggravating factors (trauma, vibration, cold, etc.).

PREVENTION/AVOIDANCE

Avoid trauma to fingertips
Avoid exposure to cold
Smoking cessation

POSSIBLE COMPLICATIONS

Gangrene, autoamputation of fingertips

EXPECTED COURSE AND PROGNOSIS

Prolonged course with recurrent ischemia and ulceration
In secondary Raynaud's phenomenon, many patients eventually develop hallmarks of the underlying disease
Between 12–50% of Raynaud's patients develop a secondary disorder, many of which are connective-tissue diseases

ASSOCIATED CONDITIONS

Lupus
Rheumatoid arthritis
Scleroderma
Polymyositis
Sjögren's syndrome
Occlusive vascular disease
Cryoglobulinemia
Use of vibrating tools

AGE-RELATED FACTORS

Pediatric: Associated with systemic lupus erythematosus and scleroderma in children
Geriatric: Appearance of Raynaud's after 40 almost always indicates an underlying disease
Others: N/A

PREGNANCY

N/A

OTHER NOTES

N/A

ABBREVIATIONS

N/A

Clinical Investigations

ROLE OF HOMOEOPATHY

Holistic healing that treats the person, not just the disease. Experience gentle, lasting wellness with expert constitutional care.