Raynaud’s phenomenon

Characterized by cold induced vasospasm that presents as triphasic skin discoloration of digits from initial vasoconstriction, resulting in hypoxemia and re-perfusion. (Pallor, cyanosis, and hyperemic redness).

Precipitated by exposure to cold, emotional upset and cigarette smoking.

Skin discoloration related to abnormal spasm of blood vessels with a diminished blood supply to local tissues.

Initially digits turn white with diminished blood flow and than turn blue because of prolonged lack of oxygen.

Eventually the blood vessels reopen causing a flushing phenomenon turning the digits red.

The three phases of the phenomenon cause the white, blue to red color changes, usually after exposure to cold.

The majority of RP is idiopathic (primary), and benign.

Secondary RP due to an underlying connective tissue disease should be suspected in patients with symptoms onset after the age 30 years, asymmetrical involvement, pain and ischemic skin lesions, abnormally dilated or missing capillaries on nail fold examination, positive specific autoantibody test results, or the clinical features of auto immune process.

Most commonly affects women in the second to fourth decades of life.

An exaggerated vascular response to cold temperature or emotional stress.

The vasoconstrictive response of the fingers and toes is characterized by vasospastic phase that causes pallor, a cyanotic phase caused by deoxygenation of static venous blood and a hyperemic phase when blood flow is restored, resulting in erythema.

The three phases above are present in about 60% of patients.

Primary RP refers to the process with no evidence of an underlying medical illness.

Primary RP is more likely to have symmetrical involvement and affect younger females than secondary disease.

Primary disease patient’s lack a history of potential secondary causes, have normal physical examination, no digital ulceration, necrosis, or gangrene, have normal nail fold capillaries, have negative tests for antinuclear antibodies, and have a normal ESR.

May be an isolated process (Raynaud’s disease) or one related to rheumatic diseases (Raynaud’s phenomenon).

Associated with scleroderma, rheumatoid arthritis, systemic lupus erythematosus, hypothyroidism, carcinoid, medications including propanolol, estrogens, progesterone, nicotine, bleomycin, ergotamine and cancer.

Secondary RP occurs in association with other conditions, such as SLE, scleroderma, and peripheral vascular disease.

RP Is present in 95% of patients with systemic sclerosis and can proceed the development of scleroderma up to 30 years, with mean duration of 10 years.

In patients with RP who have abnormal nail capillaries and a scleroderma specific autoantibody, 80% transition to systemic sclerosis within two years of initial presentation.

The vascular pathology associated with RP in systemic sclerosis is characterized by endothelial dysfunction, intimal fibrosis, ischemia/re-perfusion injury from cyclical vasodpasm and can ultimately progressed to digital ulcers and gangrene.

Occurs in approximately 15-30% of patients with SLE and such cases of classified as secondary RP.

May be an early sign of systemic connective tissue disorders in about 13% of patients.

Secondary RP is more prevalent in patients older than 30 years and in patients with severe prolonged attacks, asymmetrical involvement, and ischemic skin lesions.

The presence of autoantibodies is suggestive of secondary RP.

Diagnostic tools include: nail fold capillaroscopy, thermal imaging, and laser Doppler imaging.

An abnormal nail fold capillary pattern is the best predictor of possible connective tissue disease in patients with RP.

Treatment includes: maintaining core body temperature, stress reduction, calcium channel blockers, angiotensin II-receptor inhibitors, selective serotonin reuptake inhibitors, and botulinum toxin type A injections.

Management begins with lifestyle measures such as wearing gloves, avoiding triggers of cold, eliminating aggravating factors such as smoking.

Calcium-channel blockers, are the first-line therapy.

If long acting calcium channel blockers fail, escalation of vasodilator therapy to oral phosphodiesterase type 5 inhibitors or intravenous prostanoids (epoprosterol, iloprost) are considered.

Topical nitrates can be helpful but are poorly tolerated and should not be combined with PDE5 inhibitors because of the risk of hypotension.

Antiplatelet therapy and statins are used to prevent secondary thrombus formation and improve microcirculation, with limited evidence of benefit.

Bosentan reduces new digital ulcer formation, and digital sympathectomy with botulinum toxin is considered.