Raynaud’s Phenomenon – Diagnosis, Symptoms and Treatment

What is Raynaud's Phenomenon?

Raynaud's phenomenon is a medical condition characterized by an exaggerated response of the blood vessels in the hands to cold temperature or stress. Individuals typically develop color changes involving the fingers' skin, such as white and blue/purple discoloration due to vasoconstriction of the digital arteries. There are primary and secondary forms of the disease. Primary Raynaud's phenomenon occurs when symptoms occur without evidence of any other related condition.

Secondary Raynaud's phenomenon is frequently associated with rheumatologic diseases such as systemic lupus erythematosus and systemic sclerosis. Patients with severe forms of the disease can develop autoamputation of the digits due to ischemia and necrosis of the fingers' tips.

What Causes Raynaud's Phenomenon?

Raynaud's phenomenon is caused by an abnormal, exaggerated arterial response to cold temperature or stress. This leads to vasoconstriction of the arteries in the digits, resulting in pallor or cyanosis. This is most frequently seen in the hands' digits but may also affect the toes and feet. Studies show that the abnormality is related to an increase in alpha-2 adrenergic receptor responses in the arterioles and arteries in the digits.

Secondary Raynaud's phenomenon occurs when an underlying condition interrupts vessel reactivity, leading to inappropriate vasoconstriction in the digits. This is often caused by other diseases such as systemic lupus erythematosus and systemic sclerosis. Substances such as cocaine, amphetamines, and tobacco may also provoke digital vasoconstriction. In advanced untreated cases, patients can develop autoamputation of the digits due to digital ischemia and necrosis.

Individuals with secondary Raynaud's phenomenon tend to have the following features:

•         Onset after age 40
•         A known trigger
•         Male gender
•         Signs of ischemia & ulceration
•         Asymmetric symptoms
•         Signs & symptoms of another disorder
•         Laboratory studies suggesting systemic disease
•         Ischemic signs/symptoms proximal to the digits

How Common is Raynaud's Phenomenon?

Raynaud's phenomenon is a common disorder that is often evaluated in the primary care clinic. Severe cases often require referral to a rheumatology specialist, especially if first-line measures are unsuccessful.

The prevalence of this condition is approximately 3%-20% in women and 3%-14% in men. In individuals over age 60, the prevalence of the disease is about 0.1%-1%. It affects approximately 5% of people in the United States.

Signs and Symptoms

Raynaud's phenomenon is classically described by the abrupt onset of cold digits associated with clearly defined color changes caused by vasoconstriction. The skin initially turns pale (white) then cyanotic (blue/purple). This is followed by a reperfusion phase in which the digits are red. Symptoms most often affect the fingers and hands but may also involve the feet and toes. They usually start with one finger and spread to others in both hands in a symmetric fashion. Occasionally other body regions are involved, including the skin of the ears or nose.

Patients with Raynaud's phenomenon also frequently exhibit livedo reticularis – this is a violaceous mottling of the skin that typically involves the arms and legs. It is entirely benign and reversible in individuals with primary Raynaud's phenomenon.

Diagnosis

The diagnosis of Raynaud's phenomenon is suggested based on symptoms and physical examination. There are no specific laboratory or imaging studies. Your doctor may ask you screening questions regarding whether you have finger sensitivity to cold temperature or cold-induced color changes in the fingers – these are cardinal features of the disease.

Individuals with features that are suggestive of an associated underlying condition may require laboratory studies such as a CMP (comprehensive metabolic panel), CBC (complete blood cell count), thyroid function studies (TSH, free T4), complement levels (C3, C4), and urinalysis. Your doctor may occasionally evaluate for conditions such as peripheral vascular disease or thoracic outlet syndrome with an arterial Doppler ultrasound.

Your doctor may also investigate rheumatologic diseases such as systemic lupus erythematosus. They may order an ANA (anti-nuclear antibody), RF (rheumatoid factor), and DS-DNA (double-stranded DNA).

Raynaud's Phenomenon Treatment

Raynaud's phenomenon is typically treated with vasodilators to prevent vasoconstriction and ischemia in the digits. Primary Raynaud's phenomenon is unlikely to result in digital ischemia and often responds to conservative measures. These measures often include avoiding cold temperatures, staying warm, quitting smoking, and reducing stress levels.

Patients with secondary Raynaud's phenomenon often have more severe disease. Management should include treatment of the underlying disorder as well as specific therapy for Raynaud's phenomenon. First-line medical therapy includes long-acting dihydropyridine calcium channel blockers such as Procardia (Nifedipine) or Norvasc (Amlodipine). For people refractory to calcium channel blockers, phosphodiesterase inhibitors like sildenafil, vardenafil, or tadalafil can be good second-line treatments.

References:

1.     Wigley FM, Flavahan NA. Raynaud's's Phenomenon. N Engl J Med 2016; 375:556. - https://www.ncbi.nlm.nih.gov/pubmed/27509103
2.     Hughes M, Herrick AL. Raynaud's phenomenon. Best Pract Res Clin Rheumatol 2016; 30:112. - https://www.ncbi.nlm.nih.gov/pubmed/27421220