Raynaud’s PhenomenonDz

Synonyms: Raynaud’s disease.

ICD-9 Code: 443.0.

ICD-10 Code: I73.0

Definition: Raynaud’s phenomenon is paroxysmal, reversible vasospasm of small arteries that may be triggered by cold exposure or stress. When it occurs in the absence of another underlying connective tissue disease, the term primary Raynaud’s syndrome or Raynaud’s disease is used.

Etiology: Structural and functional alterations of blood vessels are probably contributory. Arteries are usually narrowed (see Pathology section), so even normal vasospastic responses may occlude the lumen. Other data suggest changes in mediators of vascular tone, such as endothelin. Underlying causes for these abnormalities are unknown.

Pathology: Digital arteries show proliferation of subintimal tissues and fibrotic change. Small thrombi may form on the altered intimal surfaces. Luminal narrowing or occlusion can occur in these small vessels as well as in larger arteries. Vasculitis is not present.

Demographics: It occurs predominantly in females, accounting for at least 80% of those with Raynaud’s phenomenon. Raynaud’s disease may appear in younger women than the corresponding syndrome occurring in association with other connective tissue disorders. Although nearly all patients with systemic sclerosis will have Raynaud’s phenomenon, very few patients with Raynaud’s phenomenon will progress to scleroderma or CREST.

Associated Conditions: Raynaud’s phenomenon may be associated with diffuse and limited (CREST) systemic sclerosis, SLE, MCTD, antiphospholipid syndrome, RA, polymyositis, cryoglobulinemia, and Sjögren’s syndrome.

Cardinal Findings: The classic “white, blue, and red” changes do not occur in all cases. Initially, severe blanching, or white, results from vasospasm and lack of arterial perfusion. Blanching is the least common of the three and is followed by the frequently observed blueness caused by venous pooling and tissue cyanosis. On rewarming, reactive painful hyperemia causes redness in the fingers or hand. The hands are most commonly affected, but the feet and the tips of the nose and ears also can be involved.

Patients with primary Raynaud’s phenomenon have a normal physical examination and only manifest physical findings in the distal extremities. Such patients usually have normal nailfold capillaries and a low risk of developing other connective tissue diseases. Patients with secondary Raynaud’s phenomenon clearly demonstrate evidence of the underlying connective tissue disorder (e.g., sclerodactyly, malar rash, arthritis). Nailfold capillaroscopy should be abnormal and the prognosis is guarded.

Uncommon Findings: Distal finger pad ulcerations and scarring are uncommon. In extreme cases, gangrene and autoamputation may develop.

Diagnostic Tests: The diagnosis is made largely on clinical data (history, physical examination). In patients without evidence of an associated connective tissue disorder (e.g., scleroderma, SLE), measurement of ANA may be useful in predicting the risk of developing such disorders.

Imaging: Changes in small capillaries can be viewed by nailfold capillaroscopy, which may provide prognostically important information. Radiographs show osteolysis of distal phalanges. Angiography is rarely required to distinguish Raynaud phenomenon from occluding blood clots or cholesterol emboli.

Keys to Diagnosis: Cold-induced pain, pallor, or cyanosis of digits is an important clue.

Differential Diagnosis: Atherosclerotic occlusion of small vessels occurs in older individuals, has a male predominance, and is less likely to be symmetric. Thromboangiitis obliterans (Buerger ’s disease) involves lower extremities more often than the hands and is usually accompanied by claudication. Heavy cigarette smoking can cause symptomatic vasospasm; obtaining a careful tobacco use history is important for all patients. Other causes of blue digits include cryoglobulinemia, hyperviscosity states, and APL syndrome.

Therapy: The hands and feet should be kept warm with gloves and other coverings that may be required both at night and during the day. Ulcerated finger tips may require protective bandages or guards. Tobacco should be discontinued, and particular medications (e.g., beta-blockers, ergotamine, amphetamines) should be avoided. Biofeedback training may be effective. A few pharmacologic agents, such as long-acting forms of nifedipine, are useful and well tolerated; blood pressure should be closely monitored. Antiplatelet therapy (e.g., ASA, dipyridamole) is advisable in some. Topical nitrates may help with digits, but their use may be limited by headaches. Difficult cases may require chronic vasodilator therapy. Although there has been anecdotal use of bosentan (endothelin receptor antagonist) and epoprostenol (prostacyclin) for severe, refractory digital ulcers, this requires further study because these agents are cost prohibitive and potentially toxic.

Surgery: Truncal or digital sympathectomy may be attempted if local measures and drugs are ineffective. Digital ischemic ulcers are best treated with local wound care measures rather than with surgical intervention. Gangrenous fingertips should be allowed to self-demarcate whenever possible.

Prognosis: For patients with secondary Raynaud’s, outcome is related to the underlying disease. Raynaud’s phenomenon has such a high prevalence in scleroderma  that it does not identify a prognostic subset, nor does it distinguish between CREST and diffuse systemic sclerosis. Patients may have an increased chance of developing other connective tissue disorders if Raynaud’s phenomenon is accompanied by abnormal periungual capillaries, puffy fingers, pitting of the nails, or ANA positivity.

BIBLIOGRAPHY

Cutolo M, Grassi W, Matucci et al. Raynaud’s phenomenon and the role of capillaroscopy. Arthritis Rheum 2003;48:3023–3030.PMID:14613262

Hummers LK, Wigley FM. Management of Raynaud’s phenomenon and digital ischemic lesions in scleroderma. Rheum Dis Clin North Am 2003;29:293–313. PMID:12841296