Raynaud's Phenomenon is....
A condition that was first described in 1862 by Maurice Raynaud, hence its name. It is a disorder in which blood vessels are peculiarly sensitive to cold or emotional stress which leads initially to blanching of the extremities (white discoloration), later to stagnation of the venous blood (blueness or cyanosis) and finally to an increase in blood flow during the recovery phase (redness, or suffusion). When all three changes occur in sequence the attacks are called triphasic . Any or all three of these phases may be troublesome causing pain, numbness or burning discomfort.
These symptoms occur when the supply of blood to the extremities is interrupted, the white colour is due to a reduction in the blood supply to the affected part of the body, blue as due to lack of blood the fingers lack oxygen and turn red as the blood comes rushing back again.
This is a normal response for everyone's body on exposure to extreme cold that the circulation to our extremities e.g. finger/toes are shut down to conserve heat but it seems that people with Raynaud's have an overactive response in which the blood vessels narrow or go into spasm more ea sily than they should in response to mild cold or stress.
There is still debate amongst scientists as to whether Raynaud's is due to a generalised abnormality of blood vessel control or to a local alteration in blood vessels of the extremities. Raynauds phenomenon can be described as 'Primary' or 'Secondary'.
Primary Raynaud's:
This is the most common type which occurs spontaneously without any underlying condition. This is often the type that young women get, there is often a family history and onset is often as a teenager.
Secondary Raynaud's:
This type of Raynaud's is associated with an underlying condition such as scleroderma (systemic sclerosis) or systemic lupus erythematosus (SLE) or other forms of arthritis or muscle inflammation.
Autoimmune Raynaud's:
We are now recognising some people who have Raynaud's without any clinical evidence of an underlying condition but with the presence of antibodies in their blood.
Secondary and autoimmune Raynaud's:
Symptoms tend to be more severe than in the primary form.
Who has Raynaud's
It is a very common condition affecting about 10% of the general population. It can appear at any age and 9 out of every 10 people affected will be women.
How is Raynaud's Phenomenon Diagnosed?
The best clue as to whether someone has Raynaud's or not is to ask them to describe the colour changes and symptoms triggered by cold environments. In hospital Raynaud's can be confirmed by thermography and other specialised techniques (laser Doppler or digital plethysmography - these are mainly for research studies) There are other simple tests that may be helpful to further support the diagnosis of Raynaud's and to help determine whether it is Primary or Secondary.
Thermography
An image showing the skin temperature on exposure to cold and the time taken to rewarm again (recovery is slower in Raynaud's)
Nailfold capillaroscopy
Having the nail bed of one of your fingers examined under the microscope to look for damage to small blood vessels that occurs more often in secondary Raynaud's.
Chest radiograph
A few people with Raynaud's will have this due to an extra (cervical) rib present at the base of the neck causing pressure on the blood vessels which supply the arms, if present this will be seen on a chest or neck x-ray.
Blood tests
To check for any underlying rheumatic disease which may give more clues as to which type of Raynaud's you have. The most useful test is an antinuclear antibody (ANA). This is almost always positive if there is an underlying disease (secondary/autoimmune Raynaud's).
Our own research shows that if you have normal nailfold capillaries and negative ANA (on 2 occasions 1 year apart) there is almost no chance of developing a major connective tissue disease such as lupus or scleroderma.
Treatment of Raynaud's Phenomenon
There are several options for the treatment of Raynauds the aim of this is to lessen the symptoms and to improve ability to perform activities but often does not stop them totally. The treatment of all types of Raynaud's phenomenon begins with education about what the condition is and how simple actions can help avoid or control it. Whether you need any other treatment will depend on the severity of your symptoms; how often they come on or last and whether they affect your ability to perform activities. It is sensible to see patients with secondary or autoimmune Raynaud's phenomenon every year to check how they are getting on, particularly in the early stages when things may change more.
Simple advice for everyone
STOP SMOKING!
Smoking definitely damages blood vessel walls.
It can cause more severe Raynaud's
It can cause cancer and heart disease
KEEP WARM
e.g. wear gloves and a hat etc. in cold weather
INFORMATION PAMPHLETS
e.g excellent information from
The Raynaud's & Scleroderma Association
The Arthritis & Research Campaign
The Scleroderma Society
to name a few plus material on the Internet.
Over the counter' and natural supplements
There are a number of supplements which may be of benefit and can be bought from a pharmacy or health shop without requiring a prescription from your doctor. They are listed in different categories with several examples in each and they are safe in combination.
Antioxidant Vitamins - these may prevent blood vessel damage.
Vitamin E ( tocopheryl acetate) 200mg - 400mg daily
Vitamin C (ascorbic acid) 500mg - 1000mg daily
Omega-3 fish oils and Gamolenic Acid (GLA)
These stimulate a natural substance in your body called prostacyclin which dilates blood vessels
Gamolenic Acid (GLA) 320 mg daily
Evening Primrose Oil [this contains GLA]
Maxepa (fish oils) 10,000mg daily [5 caps twice a day]
Omacor (fish oils) 2000mg - 4000mg daily
Other possible supplements
We have some knowledge about the way the above supplements may benefit Raynauds and in addition some patients report benefit from the following:
Gingko Biloba, Ginger, Cayenne, Hawthorn, Prickly Ash, Dong Quai, Magnesium.
We advise that you should always carefully read the labels of supplements to check their contents and that they should be avoided if you are taking warfarin or blood thinning agents.
Medications prescribed by your doctor
There are several different types of medications which may help, however the response of an individual patient to a certain medication is variable i.e. the same medication will not benefit everyone and so sometimes more than one may need to be tried (generally in rotation for about 4 weeks each) until you find the one that works best for you. Like all prescribed medications they can cause side effects in some people. For vasodilators such as nifedipine side-effects may include headache, flushing, nausea, dizziness, low blood pressure, and ankle swelling. Some different types of drug include:
Calcium channel blocking vasodilators .
Relax the muscle in blood vessel walls. e.g. nifedipine , amlodipine , diltiazem
Angiotensin blocking vasodilators .
Block the effects of a substance that makes blood vessels constrict ( angiotensin ). Angiotensin converting enzyme (ACE) inhibitors prevent angiotensin formation (e.g. perindopril , captopril , enalapril , lisinopril , ramipril , quinapril ). Angiotensin II receptor blockers prevent its action directly (e.g. losartan , valsartan , irbesartan ).
Serotonin re-uptake inhibitors .
These reduce levels of serotonin, another vasoconstrictor. They also work in the brain as antidepressants but this is separate from effects on Raynaud's. e.g. fluoxetine ( prozac ®, paroxetine , sertraline . Another drug that blocks serotonin is ketanserin and this was previously used with considerable benefit to treat Raynaud's . Unfortunately it is not easily available and so is now rarely used.
Other agents :
In some patients other drugs may be useful including nitrate patches or other forms of vasodilator. A few Raynaud's sufferers have found anti-platelet drugs useful.
Hospital based treatments
Very severe Raynaud's despite tablet therapies or complications such as painful fingertip ulcers may necessitate a more intensive treatment by intravenous infusion ("drip"). Most often this is for between 3 and 5 days using a drug called iloprost . Sometimes an alternative termed CGRP ( Calcitonin Gene Related Peptide) is used.
Both treatments aim to replace naturally occurring chemicals in blood vessel walls that appear deficient in Raynaud's.
Surgical options
In the past many patients were treated by an operation called cervical sympathectomy . This involves cutting nerves in the neck that control blood vessels in the arm. Unfortunately the operation can damage nerves that control the eyelid and pupil and often only worked for a short while. A similar procedure in the back can help lower limb Raynaud's and is more long-lasting. Temporary treatment can be given first to assess benefit. Finally a digital sympathectomy strips nerves from blood vessels in the fingers. This is good if one finger is severely affected.
The Future
Doctors are a lways striving towards better treatments for Raynaud's by testing their effect in controlled trials. At the Royal Free Hospital Rheumatology department we have the opportunity to allow patients to try some of the newest treatments being tried such as inhaled iloprost thus avoiding the need for a drip and hospital stay and so we really rely on patients themselves to help us develop improved treatments for this condition. Other studies have looked at powerful antioxidants (e.g. probucol ) and injections of heparin under the skin. Both of these approaches seemed to help. Drugs that are used in other blood vessel problems are also being tested for Raynaud's including bosentan , which blocks a substance called endothelin that can cause blood vessel spasm and sildenafil ( viagra ) which blocks an enzyme in blood vessel walls. Both of these drugs are very much still at a stage of clinical trials and it is exciting that a study of bosentan last year suggested that it may prevent the development of painful ulcers on the fingers of scleroderma patients with severe Raynaud's. Tablet forms of iloprost have also been tested but so far have not been found to be particularly useful.