Contents
Definition
Raynaud's phenomenon is caused by contractions in blood vessels (vasospasm) which leads to reduced blood circulation in the fingers and toes, often triggered by cold or stress. The purpose is to maintain a sufficient temperature in the internal organs at the expense of the arms and legs. The condition is divided into primary and secondary Raynaud's phenomenon, of which the primary form is the most common, least severe and without an underlying disease. Raynaud's phenomenon is named after the French doctor Maurice Raynaud (1834–1881).
Occurrence
As many as 8 – 10% of the population have Raynaud's phenomenon from time to time and it is nine times more common in women than men, most often among younger people (reference: Maricq HR, 1997). Over 80% are "primary", i.e. not related to an underlying disease (reference: Brand United Nations, 1997).
Symptoms
Three stages. The episodes usually last less than 15 minutes:
- Loss of normal color in the skin (white finger or toes) with reduced feeling
- Blueish discoloration and pain (blood circulation resumed)
- Redness of the skin and increased heat before normalization
Primarily Raynaud's phenomenon. Occurs without any other underlying disease. Most have had symptoms from childhood or early adulthood. It is very unusual that primarily Raynaud's phenomenon causes wounds (ulcers) on the fingers.
Secondary Raynaud's phenomenon. Occurs later in life and can be caused by an underlying disease. The list of possible causes is long (See Wikipedia here ). Among the rheumatic diseases are
Some conditions in which Raynaud's often occurs
- Antisynthetase syndrome (dermatomyositis)
- Beta-blockers (blood pressure/heart medication)
- Cell poison treatment with bleomycin, vinblastine, vincristine og cisplatin (against Lymphoma, testicular cancer and others)
- Caffeine (coffee) (large doses)
- Cold agglutinin disease
- Mixed connective tissue disease (MCTD)
- Nicotine and snuff (large doses)
- Substances (amphetamine, cocaine)
- Systemic lupus erythematosus (SLE)
- Systemic sclerosis
Examinations
Typically primarily Raynaud's phenomenon usually does not require a wide medical examination, but if suspected of underlying disease (secondary Raynaud's phenomenon) the general practitioner will carry out orientation medical examinations.
Disease history may include occupation, age at onset, location, symmetry, ulcers on fingers, triggering causes (temperature drop, stress, new drugs, other illness). Signs of underlying disease. .
Clinical hands and feet are assessed for discoloration, bluing, redness, sores on fingertips, dead tissue and signs of Systemic sclerosis (sclerodactyly, telangiectasias, calcinosis), as well as reduced pulse in the radial artery (vasculitis/vasculitis in large blood vessels, blood clots, atherosclerosis).
Blood tests. Routinely applicable: CRP, SR, Hgb, leukocytes with differential counts, thrombocytes, liver, kidney, thyroid function tests, electrophoresis, ANA, anti-DNA, ANCA. If systemic sclerosis is specifically suspected, a "scleroderma blot" can be requested and the same for a "myositis blot" if (dermato-) myositis is suspected / Antisynthetase syndrome.
Imaging with X-ray of hands if systemic sclerosis is suspected and calcinosis in soft tissue.
Capillaroscopei is performed to distinguish primary Raynaud's phenomenon (normal capillaroscopy) from the secondary type (most often pathological capillaroscopy), this examination is relevant. Capillaroscopy is usually done in specialist healthcare.
Diagnosis
Diagnostic criteria (adapted from Marverakis E, 2014)
Primarily Raynaud's phenomenon when fingers are sensitive to cold and absence of secondary Raynaud's and at least three of the following:
- White and blue (biphasic) episodes
- Can be triggered by stress, anxiety etc.
- Usually on both hands
- Color change observed by doctor
- Patient photo shows typical findings
- Other localization: feet, tip of nose, ears, nipples
- Three-phase form (white, blue, red)
secondary Raynaud's phenomenon with proven underlying disease (see the table under "Symptoms") or at least one of the following:
- Symptoms of the disease capillaroscopy
- Ulcers on fingertips, dead tissue, calluses on fingers (sclerodactyly), calcinosis, fibrosis
- Medical history on systemic connective tissue disease
- ANA in the blood significantly elevated
Similar diseases / differential diagnoses)
- Atherosclerosis (atherosclerosis). Predisposing factors such as smoking, high cholesterol, diabetes, old age.
- Bürger's disease (thrombangitis obliterans). Heavy smokers
- Blood clot (arterial embolism)
- Dysregulation syndrome with acrocyanosis (Flammer J, 2013).
- Erythromelalgia
- Fibromyalgia
- Frostbite
- hypothyroidism (low metabolism)
- Cryoglobulinemia. Often paraproteinemia (electrophoresis)
- Malignant disease / cancer
- Neuropathy
- Polyneuropathy
- Thoracic outlet syndrome. Most often unilateral in one hand
Treatment
Primary Raynaud's phenomenon usually does not require drug treatment. An adapted lifestyle to avoid the cold, prevention with good mittens/gloves and avoiding nicotine is recommended. Heat aids after further assessment, usually by an occupational therapist in some cases.
Calcium blockers (for example nifedipine (Adalat) 30-60mg/day or amlodipine (Norvasc) 2,5-10 mg/day) or lercanidine 10mg (Zanidip) have shown some effect, but also some unpleasant side effects (headache, dizziness , low blood pressure) (reference: Cochrane 2016). Follow-up with control of blood pressure after starting treatment may be relevant (Reference Lee EY, 2014).
Sildenafil tablets (Revatio) are sometimes used after special thorough evaluation, without approved indication
By secondary Raynaud's phenomenon, special measures to avoid wound development (digital ulcers) or severe pain may be appropriate. rheumatic disease is present, a local rheumatologist will help.
