Overview of Raynaud’s Syndrome
Raynaud’s syndrome is a vascular neurological dysfunction caused by small arterial spasmodic disease of the extremities appeared after the cold or emotional fluctuations, the skin of the fingers and toes appeared to be pale, bruising, flushing is not completely clear, partly related to autoimmune diseases, thrombosed vasculitis, cold stimulation, etc. Smoking cessation, warmth, medication-based therapy
Definition
Raynaud’s syndrome is a spasmodic disorder of the small arteries of the extremities caused by vascular neurologic dysfunction. Typical symptoms include programmed pallor and chills, bruising and pain in the affected area, flushing, and then recovery.
Classification
Classification according to cause
Raynaud’s disease: also known as arterial spasmodic disease of the extremities, caused solely by vasospasm without underlying disease.
Raynaud’s phenomenon: vasospasm accompanied by other systemic diseases, e.g., autoimmune disease, thromboembolic vasculitis.
Morbidity
The disease is rare and most often occurs in women, especially in neurotics, with a male to female ratio of 1:10.
The age of onset is 20 to 40 years old. It occurs mostly in the cold season.
Causes
Causes
Raynaud’s disease
It is caused solely by vasospasm and may be related to genetic factors, sympathetic nerve dysfunction and other factors.
Genetic factors
Family and twin studies have shown that genetic factors seem to play an important role in the development of Raynaud’s disease, with half of Raynaud’s patients having a positive family history.
Sympathetic Nerve Dysfunction
Raynaud’s disease is thought to be a “localized defect” in vascular thermoregulation. Cold stimuli increase the sensitivity of contractile proteins, leading to vasoconstriction.
Vascular Sensitization Factors
Increased sensitivity of the extremity arteries themselves to cold.
Vascular wall structural factors
Alterations in the organization of the vascular wall can cause normal vasoconstriction or an abnormal response to blood adrenaline.
Other factors
May be endocrine related, with symptoms worsening during menstruation in women.
Raynaud’s phenomenon
Vasospasm accompanied by other systemic diseases such as autoimmune disease, thromboembolic vasculitis.
Autoimmune diseases: systemic sclerosis, rheumatoid arthritis, systemic lupus erythematosus, dermatomyositis, polymyositis, etc.
Obstructive arterial disease: thromboembolic vasculitis, acute arterial obstruction, etc.
Spasmodic arterial disease: primary pulmonary hypertension.
Neurological diseases: intervertebral disc disease, spinal cord cavernous disease, spinal cord tumor, poliomyelitis, carpal tunnel syndrome, etc.
Hematologic disorders: cryoglobulinemia, cryofibrinogenemia, myeloproliferative disorders, macroglobulinemia, etc.
Others: drug damage, such as ergot, birth control pills, beta-blockers.
Predisposing factors
Cold stimulation.
Emotional tension, excitement, exertion.
Smoking.
Symptoms
Main Symptoms
Generally after cold and emotional excitement, the skin color changes intermittently, and the classic changes are divided into ischemic, bruising, and congestive phases, and the specific symptoms are as follows.
Ischemic phase
Usually occurs symmetrically on the ends of both limbs, especially the ends of the fingers, and occasionally on the ears, tip of the nose, cheeks or chin. Pallor, coldness, and decreased skin temperature of the extremities appear.
It is often accompanied by a sensation of ants, numbness or pain.
Symptoms often last from a few minutes to several hours.
Each attack is not necessarily the same finger or toe.
Bruising phase
The localized ischemic phase continues with continued signs of sensory deficits, decreased skin temperature, bruising and pain in the extremities.
Congestive phase
Arterial congestion is manifested by a rise in skin temperature and flushing of the skin, which then returns to normal.
Complications
Occasionally, trophic changes can be seen at the ends of the fingers (toes), such as curved, deformed and brittle nails, atrophy of the digits, and ulcers or gangrene at the tips of the fingers (tissue that is necrotic is painful, and when the tissue is necrotic, it becomes numb and black).
Seek medical attention
Recommendations for medical consultation
Raynaud’s syndrome is diagnosed and treated primarily in a rheumatologist.
If symmetrical skin pallor, bruising, or redness of the skin at the ends of the extremities occurs after a cold stimulus or emotional excitement, it is important to seek medical attention.
Follow medical advice for repeat patients.
Preparation for consultation
Registration
Before the outpatient consultation, you need to register at the hospital site or through official channels (such as the hospital’s official website, official app, 114 platform, etc.).
Preparation of information
Prepare your medical documents such as medical card, social security card (health insurance card), etc.
Bring information from previous medical visits, such as medical records and laboratory test results.
If you are taking medication, prepare a list of medications.
What questions the doctor may ask
When do fingers turn white or purple? Can it return to normal?
What kind of work was done?
Have the fingers or toes been traumatized?
Are there any other systemic diseases?
Is there anyone in the family with similar condition?
Have you had a relevant examination? What are the results?
Have you had any treatment? What kind of treatment? What is the effect?
Any history of drug allergy?
Are there any medications you are taking?
Questions you can ask your doctor
What is the cause of the disease?
What tests are needed?
What treatments are available?
Can it be cured?
What do I need to pay attention to in my daily life?
Diagnosis
Diagnosis
History of the disease
Relatives who have had the disease.
History of autoimmune disease.
Clinical manifestations
When the local cold or emotional excitement, bilateral fingers or toes and other parts of the appearance of pale, cold, extremity skin temperature decreased and so on.
Symptoms may resolve on their own, or after exposure to a warm environment, warm baths, rubbing or waving the affected limbs.
Laboratory Tests
These include routine blood tests, inflammatory markers (erythrocyte sedimentation rate, C-reactive protein), and immunologic tests. In Raynaud’s disease, the results of these tests are normal; if the results of the above tests are abnormal, it is mostly suggestive of Raynaud’s phenomenon.
Routine blood test: it can detect whether the white blood cell count is abnormal; if it is elevated, it suggests the presence of infection.
Erythrocyte sedimentation rate: if increased, it supports Raynaud’s phenomenon.
C-reactive protein: if elevated, it supports Raynaud’s phenomenon.
Immunologic tests: e.g., antinuclear antibody profile, rheumatoid factor, complement, etc., to determine the presence of rheumatoid immune diseases (systemic lupus erythematosus, scleroderma, and rheumatoid arthritis, etc.).
Nailfold microangioscopy
Nailfold microangioscopy is a unique, non-invasive method of visualizing the structure of the microcirculation.
Normal capillaries are uniformly distributed and have a “hairpin” appearance.
Nailfold microangioscopy is usually normal in Raynaud’s disease.
In Raynaud’s phenomenon associated with scleroderma, the early stages show capillary dilatation and small amounts of hemorrhage; as the disease progresses, the hemorrhage increases and “giant” capillaries appear; and in the late stages there is a loss of capillaries, areas of extensive ischemia, and abnormal neovascularization.
Other tests
Cold water test: fingers (toes) are immersed in 4℃ cold water for 1 minute, skin color change is positive.
Fist clenching test: clench both hands for 90 seconds, then bend the upper limbs at the elbow and flatten the waist to release the hands. Some patients may show skin color change during seizure.
Differential diagnosis
Cyanosis of the extremities
Cyanosis of the extremities is not significantly related to mood, the change in skin color is persistent, the entire hand and foot are involved, and the skin is sticky and damp. It can be differentiated by pathologic examination.
Reticular cyanosis
Cyanotic spots appear in the proximal part of both lower limbs, aggravated by cold, and the reticular cyanosis can be reduced or disappeared after elevating the affected limbs. It can be differentiated according to clinical manifestations.
Erythematous limb pain
The main manifestations are intermittent burning pain, erythema and increased skin temperature of both feet, which can be differentiated by skin critical temperature test.
Treatment
Internal medicine treatment
General treatment
If the disease is caused by simple vasospasm, it is necessary to adjust the lifestyle, pay attention to hand warmth, and smokers should quit smoking; if it is accompanied by other systemic diseases, it is necessary to actively treat the primary disease.
Medication
Calcium channel blockers
Calcium channel blockers can dilate blood vessels and increase blood flow.
Commonly used drugs include nifedipine and verapamil.
When using nifedipine, adverse reactions such as facial redness, fever, headache, ankle edema and tachycardia may occur.
Vasodilators
Nafronyl oxalate: has a mild peripheral vasodilator effect, may shorten the duration of attacks and reduce pain. Contraindicated in women during pregnancy.
Inositol nicotinate: may shorten the duration of attacks and reduce the number of attacks. There may be mild nausea, sweating, itching and other adverse reactions.
Tolazoline hydrochloride: has peripheral vasodilatory effect, common adverse reactions such as flushing, cold, abdominal pain, nausea, vomiting.
Prostaglandin.
It has strong vasodilatory and anti-platelet aggregation effects.
Commonly used drugs include prostacyclin and prostaglandin.
Negative pressure therapy of the limb
Negative pressure causes vasodilatation of the limb, which overcomes the contraction of vascular smooth muscle, and the arteries appear to be continuously dilated, which can temporarily improve the state of severe local hypoxia.
Surgical treatment
Surgery can be considered for those whose symptoms are not relieved after internal medicine treatment, such as the duration of the disease is more than 3 years, drug treatment is ineffective, and the symptoms persist for a long time and affect life.
Sympathectomy is commonly used. Thoracic sympathectomy can be performed for upper limb lesions; lumbar sympathectomy and micro sympathectomy around the palm and finger arteries can be performed for lower limb lesions.
Prognosis
Cure
After active treatment, the prognosis of Raynaud’s disease patients is generally good, and most of them are not life-threatening, especially when diagnosed and treated at an early stage, the prognosis is better.
The prognosis of patients with Raynaud’s phenomenon depends on the regression of the primary disease due to the concomitant other systemic diseases.
Hazards
Numbness or pain in the limbs often occurs, which may affect normal work and life.
Some patients may have complications such as fingertip ulcers and gangrene due to recurrent attacks, causing serious psychological burden.
Daily
Daily management
Dietary management
It is advisable to consume more fresh vegetables, fruits, lean meat and other high-protein and high-vitamin foods.
Avoid eating spicy and stimulating foods, such as wine, strong tea, coffee, raw garlic, ginger, chili pepper and curry.
Avoid eating raw and cold foods.
Life Management
Avoid cold stimulation
Observe weather changes and increase clothing appropriately in cold weather.
Minimize outdoor activities or work in cold weather.
Wear warm hats, masks, gloves and warm socks.
Try to stay out of cold water and avoid touching objects that are cold.
Use warm water to wash hands and feet.
Appropriate Exercise
Depending on your health condition, perform exercises such as walking and playing tai chi.
Exercise should be gradual and not too strenuous.
Prevention of infection
Avoid going to places where people gather.
Keep hands and feet clean.
Smoking cessation
Strictly quit smoking and avoid exposure to second-hand smoke.
Psychological care
Face the disease with a positive and optimistic attitude and build up confidence in overcoming the disease.
Talk to your family members, partners and friends about your inner feelings to avoid vasospasm triggered by emotional stress and fatigue.
Actively participate in work or activities within your ability to enrich your life.
If severe anxiety, depression, insomnia, etc. occur, it is recommended to go to the hospital in time for formal psychological treatment.
Prevention
Actively treat autoimmune diseases or other systemic diseases.
If possible, it is best to live or work in a warmer climate.
Ensure a good mood and adequate sleep.
Avoid mood swings.
Do not smoke.
Avoid medications such as ergot alkaloids, beta-blockers and birth control pills.
If any of your relatives suffer from Raynaud’s disease, you should have regular medical checkups for timely detection and early treatment.