Raynaud’s Phenomenon in Cold Agglutinin Disease

Cold agglutinin disease (CAD) is an autoimmune condition in which the immune system produces antibodies called cold agglutinins that mistakenly attack red blood cells at low temperatures.

Raynaud’s phenomenon is one of the cold-induced symptoms of CAD.

What is Raynaud’s phenomenon?

Raynaud’s phenomenon is a condition in which blood vessels, most commonly in the toes and fingers, constrict under cold or stressful conditions. This narrowing of blood vessels limits blood flow to these areas, which causes a drastic change in skin color and sensation.

The two types of Raynaud’s phenomenon are primary and secondary Raynaud’s.

Primary Raynaud’s occurs in patients with no underlying condition. It mainly affects women under 30, especially during the teen years, but others may also be affected.

Secondary Raynaud’s occurs secondary to an existing autoimmune condition such as CAD. This type of Raynaud’s usually manifests later in life.

Symptoms of Raynaud’s phenomenon

Raynaud’s phenomenon commonly affects the fingertips and toes, but can also happen in the nipples, ears, nose, and lips.

Common symptoms include:

  • A cold sensation in the fingertips and toes.
  • Change in skin color to white initially and then to blue as the cold sets in.
  • Numbness due to insufficient blood flow.
  • Once warmth is restored, and blood circulation improves, sensation returns and the affected areas may turn red, swell, and cause a sharp tingling pain.

Following the warming process, it may take up to 15 minutes for normal blood flow to return.

Diagnosis of Raynaud’s phenomenon

A detailed review of a patient’s medical history and symptoms, and a physical examination are useful in diagnosing Raynaud’s phenomenon.

A general cold stress test may be used to find out how the patient responds to cold. The test involves using cold water and determining the time it takes for hands and feet to return to normal temperature.

No specific test is available for Raynaud’s phenomenon but determining whether it’s caused by CAD is important. Therefore, doctors may perform tests to find out if the condition is primary or secondary. The tests include:

  • A nailfold capillaroscopy in which the skin under the fingernails is examined under a microscope to identify any deformities or narrowing of blood vessels.
  • An antinuclear antibody test to determine the presence of antibodies in the blood, which is characteristic of autoimmune conditions. This can help rule out other conditions that may cause secondary Raynaud’s phenomenon.
  • An erythrocyte sedimentation rate test to calculate the rate at which red blood cells clump and settle to the bottom of a test tube (in autoimmune or inflammatory conditions, the rate is faster than normal).

Managing and treating Raynaud’s phenomenon

Improving blood circulation is vital to managing Raynaud’s phenomenon. Most of the time, symptoms can be controlled by lifestyle changes that help improve blood flow. These include:

  • wearing warm clothes, especially gloves and socks, during cold conditions.
  • soaking hands and feet in warm water after an attack.
  • keeping living conditions warm.
  • exercising regularly and including practices such as yoga.
  • performing breathing exercises and meditation for relaxation.
  • eating a balanced and healthy diet.
  • limiting the consumption of caffeine.
  • avoiding smoking.

Treating the underlying CAD can help alleviate the symptoms of Raynaud’s phenomenon. In some cases, medications may be required. Available options are:

Additional information

Other conditions that cause secondary Raynaud’s phenomenon include scleroderma, lupus, rheumatoid arthritis, and Sjogren’s syndrome.


Last updated: August 2,  2019


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