What Is Raynaud’s Disease: Causes, Symptoms, Diagnosis, and Treatment

What is Raynaud’s disease?

Raynaud’s disease, also known as Raynaud’s syndrome or Raynaud’s phenomenon, is a rare blood vessel disorder described by a French physician named Maurice Raynaud in 1862. In this condition, the blood vessels that carry blood from the heart to other parts of the body are usually affected. It causes brief vasospasms that can lead to the narrowing of blood vessels and a restricted flow of blood to the extremities, such as the fingers and toes. 

Women are more likely to be affected by this disorder than men. Raynaud's disease is also commonly seen in people who live in cold climates. Although the disease can affect people's quality of life, it is not totally disabling. 

Types of Raynaud's Disease

The two types of Raynaud's disease are based on their symptoms and main causes:

• Primary Raynaud’s - This type is more common but less severe. It usually occurs without any known cause with very mild symptoms. For this reason, people with primary Raynaud's often do not seek medical treatment. 

• Secondary Raynaud’s - This type usually occurs due to other medical conditions, particularly those that affect the connective tissues in the body, such as rheumatoid arthritis and lupus. Although secondary Raynaud's is less common, this type often causes serious health conditions, such as gangrene and skin sores. These conditions happen when there are cell and tissue death due to a lack of blood flow. 

Signs and Symptoms

Primary and secondary Raynaud's have similar symptoms. The symptoms are often triggered by stress or cold temperature exposure. The fingers and toes of people with Raynaud's are usually affected. However, a person's lips, nose, ears, and nipples may also become affected. 

During each attack, the signs and symptoms of the disease may develop in other parts of the body and do not always occur on a person's fingers and toes. The signs and symptoms may initially be noticed in one or two fingers or toes and then spread to other areas of the body. 

The symptoms are due to the thinning of the blood vessels, which limits the flow of blood to the extremities. The most common symptoms of the attack may include:

• Feeling cold and numb
• Pain and tingling sensations
• Changes in skin color (skin turns white before turning blue)
• Skin turning red when the flow of blood returns
• Throbbing or burning sensation when blood flow turns back to normal

The duration of the attacks may last for a minute or several hours. Depending on the main cause of the attacks, people with Raynaud's may experience the symptoms occasionally, several times a day, or every day. 

Complications

Severe secondary Raynaud's is rare, but when it occurs, it could cause tissue damage due to poor blood circulation to the fingers or toes. Blocked arteries can lead to skin ulcers and gangrene, which are conditions that can be quite difficult to treat. In rare, extreme, and untreated cases, amputation or the removal of a limb may be required.  

Causes

The cause of primary Raynaud’s is still unknown, but the following triggers can lead to an attack:

• Cold Temperature - A Raynaud's attack can happen if there is a sudden blood vessel constriction due to cold temperature exposure. The constriction of blood vessels can restrict the normal flow of blood in the extremities. According to research, Raynaud’s attacks can occur more in people who are frequently exposed to cold temperatures and have blood disorders or skin conditions. 

• Stress - People who have high levels of anxiety and stress are more prone to experiencing Raynaud’s attacks even without cold temperature exposure. 

There are many factors or conditions that can cause secondary Raynaud’s. The following are some of the conditions that are commonly associated with Raynaud’s disease:

• Lupus - Lupus is a chronic autoimmune disorder that can cause inflammation in different body systems, including the skin. Poor blood flow in the extremities, which causes Raynaud's attacks, is also one of the common symptoms of lupus. 

• Rheumatoid Arthritis - This is a chronic inflammatory disorder that usually affects the joints, such as those located in the hands. Its common symptoms include muscle stiffness, joint pain, and difficulty moving. 

• Scleroderma - It is a chronic autoimmune connective tissue disease that involves the overproduction of collagen. In this condition, the skin and connective tissues become hard and tight due to collagen excess. Although scleroderma commonly affects the skin, it can also affect the digestive tract, internal organs, and blood vessels of other people. The signs and symptoms of scleroderma tend to vary depending on the affected structures. Women are more likely to be affected by scleroderma than men.

• Atherosclerosis - This disease develops when plaque builds up in the arteries. The artery wall becomes thicker as plaques build up. The buildup can lead to the narrowing of the blood vessel, restricting the normal flow of blood. Since atherosclerosis affects the blood vessels, a vasospasm link has been found, leading to the symptoms of Raynaud’s disease. Sudden numbness in the arms and legs are common symptoms of both Raynaud’s disease and atherosclerosis. 

• Sjögren’s Syndrome - It is an immune system disorder that mainly affects the salivary glands and tear ducts. It can also affect the body's nerves, joints, and soft tissues. According to research studies, people with Sjögren's syndrome also show the symptoms of Raynaud's disease.  

• Buerger’s Disease (thromboangiitis obliterans) - This disease affects the veins and arteries in the legs and arms. It causes the formation of clots and prevents the normal flow of blood. Raynaud's and Buerger’s disease have similar symptoms, which include skin color changes, cold feet and hands, and painful extremities.

• Thyroid Problems - People with hypothyroidism are often sensitive to cold temperatures. Having a poor tolerance to cold temperatures are often caused by poor blood circulation, which is a characteristic feature of Raynaud's disease. 

• Blood Disorders - Certain disorders of the blood that affect the blood vessels are often linked to Raynaud’s disease. An example of a blood disorder associated with Raynaud's disease is cryoglobulinemia, in which large amounts of cryoglobulins are present in the blood. Cryoglobulins are proteins that become insoluble in cold temperatures. 

Other factors that can cause secondary Raynaud’s may include:

• Hand and Foot Injuries - From accidents, trauma, surgical injuries, or frostbite.

• Repetitive Movements - Typing, playing the piano, or using tools that vibrate.

• Alcohol Consumption and Smoking - Nicotine exposure and excessive alcohol intake. 

• Certain Medications - Birth control pills, diet pills, medications for allergy, beta blockers, migraine medications with ergotamine, and chemotherapy drugs, such as vinblastine and cisplatin. 

Risk Factors

Primary Raynaud's

• Age - Primary Raynaud's often develop in people who are 15-30 years old. 

• Sex - Women are more likely to be affected by primary and secondary Raynaud's compared to men. 

• Family History - Having a family member or a first-degree relative with Raynaud's increases a person's risk of developing primary Raynaud's.

• Climate - People who live in colder climates are more prone to developing the disease. 

Secondary Raynaud's

• Associated Diseases - Such as lupus and scleroderma. 

• Occupation - Jobs that cause repetitive stress injuries (using tools that vibrate).

• Certain Substances - Include certain medications, chemicals, and smoking. 

Diagnosis

Certain tests may be done to rule out other conditions that have the same signs and symptoms. 

• Nailfold Capillaroscopy - Helps distinguish between primary and secondary Raynaud's disease. 

• Antinuclear Antibody (ANA) Test - A blood test that can help evaluate autoimmune disorders and connective tissue diseases. 

• Erythrocyte Sedimentation Rate (ESR) - Another blood test that can help show inflammatory activity in the body. 

There is no single blood test that can accurately diagnose Raynaud's disease. However, the above tests can be used to help rule out artery disease and identify other medical conditions that are linked to Raynaud's. 

Treatment

Primary Raynaud's

When it comes to treating the mild symptoms of Raynaud's, avoiding cold temperatures and quitting smoking can help. If a person's symptoms are triggered by stress and anxiety, relaxation techniques and counseling can be great treatment options. Medications can also be used when a person's condition involves behavioral problems. They include:

• Calcium channel blockers
• Alpha-blockers (prazosin)
• Topical nitroglycerine
• Pentoxifylline 

Secondary Raynaud's

The main aim of the treatment is to treat the underlying disorder. Calcium channel blockers or prazosin can also be used as treatments for secondary Raynaud's. For ischemic ulcers, the doctor may prescribe antibiotics. Surgical debridement can also be occasionally suggested.

Thrombosis can be prevented by taking low-dose aspirin. However, prostaglandin inhibition may worse the vasospasm. It has been suggested that intravenous prostaglandin can be beneficial, and an option for patients with ischemic digits. A number of pharmacological and surgical therapies can be useful for those who still experience some symptoms.