Raynaud’s disease


Definition:-

Raynaud’s disease is a condition that causes some areas of your body — such as your fingers, toes, tip of your nose and your ears — to feel numb and cool in response to cold temperatures or stress. In Raynaud’s disease, smaller arteries that supply blood to your skin narrow, limiting blood circulation to affected areas.

Click to see the pictures of  Raynaud’s  diseas

Raynaud’s disease (also known as “Primary Raynaud’s phenomenon” where the phenomenon is idiopathic, and Raynaud’s syndrome (secondary Raynaud’s), where it is caused by some other instigating factor. Measurement of hand-temperature gradients is one tool used to distinguish between the primary and secondary forms.

It is possible for the primary form to progress to the secondary form.

Symptoms:-
Raynaud’s disease is more than simply having cold hands and cold feet, and it’s not the same as frostbite. Signs and symptoms of Raynaud’s depend on the frequency, duration and severity of the blood vessel spasms that underlie the disorder.

The list of signs and symptoms mentioned in various sources for Raynaud’s phenomenon includes the 51 symptoms listed below:

•Symptoms usually affect fingers, toes, nose, lips or earlobes
•Skin color changes
•Skin whiteness then blueness then redness
•Cold sensitivity
•Pallor (whiteness)
•Cyanosis (blueness)
•Redness (rubor)
•Finger symptoms
*Finger color changes
*Finger pallor
*Finger tingling
*Finger redness
*Finger numbness
*Finger sensitivity
*Finger pain

•Toe symptoms

*Toe color changes
*Toe numbness
*Toe redness
*Toe pallor
*Toe sensitivity
*Toe pain

•Nose symptoms
*Nose color changes
*Nose numbness
*Nose redness
*Nose pallor
*Nose sensitivity
*Nose pain

•Earlobe symptoms
*Earlobe color changes
*Earlobe numbness
*Earlobe redness
*Earlobe pallor
*Earlobe pain
•Lip symptoms

*Lip color changes
*Lip numbness
*Lip redness
*Lip pallor
*Lip sensitivity
*Lip pain

•Episodic attacks – lasting minutes or hours
•Small blood vessel constriction (vasospastic attacks)
•Symmetric symptoms – usually both hands or both feet rather than just one
•Both hands and both feet – primary Raynaud’s affects all 4; secondary Raynaud’s typically affects either hands or feet but not both.

•Other areas affected – hands and feet most common but others are possible
*Nose symptoms
*Lips symptoms
*Ear lobes symptoms

Causes:-
Doctors don’t completely understand the cause of Raynaud’s attacks, but blood vessels in the hands and feet appear to overreact to cold temperatures or stress:

*Cold temperatures. When your body is exposed to cold temperatures, your extremities lose heat. Your body slows down blood supply to your fingers and toes to preserve your body’s core temperature. Your body specifically reduces blood flow by narrowing the small arteries under the skin of your extremities. In people with Raynaud’s, this normal response is exaggerated.
*Stress. Stress causes a similar reaction to cold in the body, and likewise the body’s response may be exaggerated in people with Raynaud’s.


Blood vessels in spasm
:
With Raynaud’s, arteries to your fingers and toes go into what’s called vasospasm. This narrows your vessels dramatically and temporarily limits blood supply. Over time, these same small arteries may also thicken slightly, further limiting blood flow. The result is that affected skin turns a pale and dusky color due to the lack of blood flow to the area. Once the spasms go away and blood returns to the area, the tissue may turn red before returning to a normal color.

Cold temperatures are most likely to trigger an attack. Exposure to cold can be as simple as putting your hands under a faucet of running cold water, taking something out of the freezer or exposure to cold air. For some people, exposure to cold temperatures isn’t necessary. Emotional stress alone can cause an episode of Raynaud’s.

Raynaud’s may be partly an inherited disorder.

In extreme cases, the secondary form can progress to necrosis or gangrene of the fingertips.

Raynaud’s phenomenon is an exaggeration of vasomotor responses to cold or emotional stress. More specifically, it is a hyperactivation of the sympathetic system causing extreme vasoconstriction of the peripheral blood vessels, leading to tissue hypoxia. Chronic, recurrent cases of Raynaud phenomenon can result in atrophy of the skin, subcutaneous tissues, and muscle. In rare cases it can cause ulceration and ischemic gangrene.

It is important to distinguish Raynaud’s disease from syndrome. In order to diagnose these two forms of Raynaud’s, a doctor may look for signs of arthritis or vasculitis, and may conduct a number of laboratory tests.

Primary Raynaud’s (disease):
Raynaud’s disease, or “Primary Raynaud’s”, is diagnosed if the symptoms are idiopathic, that is, they occur by themselves and not in association with other diseases. Some refer to Primary Raynaud’s disease as “being allergic to coldness”. It often develops in young women in their teens and early adulthood. Primary Raynaud’s is thought to be at least partly hereditary, although specific genes have not yet been identified.

Smoking worsens frequency and intensity of attacks, and there is a hormonal component. Caffeine also worsens the attacks. Sufferers are more likely to have migraine and angina than controls.

Secondary Raynaud’s (syndrome)
:
Raynaud’s syndrome, or “Secondary Raynaud’s”, occurs secondary to a wide variety of other conditions. Secondary Raynaud’s has a number of associations:

Connective tissue disorders:
*scleroderma
*systemic lupus erythematosus
*rheumatoid arthritis
*Sjögren’s syndrome
*dermatomyositis
*polymyositis
*mixed connective tissue disease

*cold agglutinin disease

*Ehlers-Danlos Syndrome

Eating disorders
*anorexia nervosa

Obstructive disorders :
*atherosclerosis
*Buerger’s disease
*Takayasu’s arteritis
*subclavian aneurysms
*thoracic outlet syndrome


Drugs
:
*Beta-blockers
*cytotoxic drugs – particularly chemotherapeutics and most especially *bleomycin
*ciclosporin
*ergotamine
*sulfasalazine
*anthrax vaccines whose primary ingredient is the Anthrax Protective Antigen


Occupation
:
*jobs involving vibration, particularly drilling
*exposure to vinyl chloride, mercury
*exposure to the cold (e.g. by working packing frozen food)


Others
:
*hypothyroidism
*cryoglobulinemia
*malignancy
*reflex sympathetic dystrophy
*carpal tunnel syndrome
*Magnesium Deficiency
*Erythromelalgia, (the opposite of Raynaud’s, with hot and warm extremities) often co-exists in patients with Raynaud’s)
It is important to realize that Raynaud’s can herald these diseases by periods of more than 20 years in some cases, making it effectively their first presenting symptom. This can be the case in the CREST syndrome, of which Raynaud’s is a part.

Patients with Secondary Raynaud’s can also have symptoms related to their underlying diseases. Raynaud’s phenomenon is the initial symptom that presents for 70% of patients with scleroderma, a skin and joint disease.

Raynaud’s phenomenon which is limited to one hand (or to one foot) is referred to as Unilateral Raynaud’s. This is an uncommon form, and it is always secondary to local or regional vascular disease. It commonly progresses within several years to affect other limbs as the vascular disease progresses.

Risk factors:-
Risk factors for primary Raynaud’s include:

*Your gender.
Primary Raynaud’s affects women more than men.
*Your age. Although anyone can develop the condition, primary Raynaud’s often begins between the ages of 15 and 30.
*Where you live. The disorder is also more common in people who live in colder climates.
*Your family history. Additionally, a family history appears to increase your risk of primary Raynaud’s. About one-third of people with primary Raynaud’s have a first-degree relative — a parent, sibling or child — with the disorder.

Risk factors for secondary Raynaud’s include:


*Associated diseases.
These include conditions such as scleroderma and lupus.

*Certain occupations. People in occupations that cause repetitive trauma, such as workers who operate tools that vibrate, also may be more vulnerable to secondary Raynaud’s.

*Exposure to certain substances.
Smoking, medications that affect the blood vessels and exposure to chemicals such as vinyl chloride are associated with an increased risk of Raynaud’s.
Complications:
If Raynaud’s is severe — which is rare — blood circulation to your fingers or toes could permanently diminish, causing deformities of your fingers or toes.

If an artery to an affected area becomes blocked completely, sores (skin ulcers) or dead tissue (gangrene) may develop. Ulcers and gangrene can be difficult to treat.

Diagnosis:-
Examinations & Tests:
To diagnose Raynaud’s, your doctor will ask detailed questions about your symptoms and medical history and conduct a physical examination. Your doctor may also run tests to rule out other medical problems that may cause similar signs and symptoms, such as a pinched nerve.

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Your doctor may perform a simple test called a cold-stimulation test during your office visit. This test may involve placing your hands in cool water or exposing you to cold air, to trigger an episode of Raynaud’s.

A careful medical history will often reveal whether the condition is primary or secondary. Once this has been established, an examination is largely to identify or exclude possible secondary causes.

Digital artery pressure: pressures are measured in the arteries of the fingers before and after the hands have been cooled. A decrease of at least 15 mmHg is diagnostic (positive).

Doppler ultrasound: to assess blood flow.

Full blood count: this can reveal a normocytic anaemia suggesting the anaemia of chronic disease or renal failure.

Blood test for urea and electrolytes:
this can reveal renal impairment.
Thyroid function tests: this can reveal hypothyroidism.
An autoantibody screen, tests for rheumatoid factor, Erythrocyte sedimentation rate and C-reactive protein, which may reveal specific causative illnesses or a generalised inflammatory process.
Nail fold vasculature: this can be examined under the microscope

Sorting out primary vs. secondary Raynaud’s
:
To distinguish between primary and secondary Raynaud’s, your doctor may perform an in-office test called nail fold capillaroscopy. During the test, the doctor examines your nail fold — the skin at the base of your fingernail — under a microscope. Tiny blood vessels (capillaries) near the nail fold that are enlarged or deformed may indicate an underlying disease. However, some secondary diseases can’t be detected by this test.

If your doctor suspects that another condition, such as an autoimmune or connective tissue disease, underlies Raynaud’s, he or she may order blood tests, such as:

*Antinuclear antibodies test. A positive test for the presence of these antibodies — produced by your immune system — indicates a stimulated immune system and is common in people who have connective tissue diseases or other autoimmune disorders.

*Erythrocyte sedimentation rate. This blood test determines the rate at which red blood cells settle to the bottom of a tube in the space of an hour. A faster than normal rate may signal an underlying inflammatory or autoimmune disease. Autoimmune diseases are commonly associated with secondary Raynaud’s.
There’s no single blood test to diagnose Raynaud’s. Your doctor may order other tests, such as those that rule out diseases of the arteries, to help pinpoint a disease or condition that may be associated with Raynaud’s.

Modern Treatments and drugs:-

Treatment options are dependent on the type of Raynaud’s present. Raynaud’s syndrome is treated primarily by addressing the underlying cause, but includes all options for Raynaud’s disease as well. Treatment of primary Raynaud’s focuses on avoiding triggers.

General care:
*Avoid environmental triggers, e.g. cold, vibration, etc. Emotional stress is another recognized trigger; although the various sources of stress can not all be avoided, it is possible to learn healthier, more effective ways of dealing with them, which will reduce stress and its damaging physical effects overall.

*Keep your hands, feet and head warm—especially your fingers, toes, ears and nose—by wearing mittens, insulated footwear, a ski mask; by using hand and foot warmers, etc.

*Quit smoking.

*Avoid caffeine and other stimulants and vasoconstrictors that have not been prescribed to you by your doctor. Read product labels; caffeine is found not only in coffee and tea, stay-awake pills, many soft drinks and candies, but also in some cosmetics, soaps and shampoos(reference needed).

  • Exercise. Your doctor may encourage you to exercise regularly, particularly if you have primary Raynaud’s. Exercise can increase circulation, among other health benefits.
  • Control stress. Because stress may trigger an attack, learning to recognize and avoid stressful situations may help control the number of attacks.

*Make sure all your doctors know about all the medicines you take and about all the OTC remedies you use, especially hormones and drugs that regulate hormones, such as hormonal contraception, so that these professionals can make an assessment of your chemical regimen and make any changes that may be indicated. Contraception which is low in estrogen is preferable, and the progesterone only pill is often prescribed for women with Raynaud’s.

*If you are diabetic, follow your diabetes treatment plan.

Emergency measures:
*If white finger (Raynaud’s) occurs unexpectedly and a source of warm water is available, allow tepid to slightly warm water to run over the affected digits while you gently massage the area. Continue this process until the white area returns to its normal, healthy color.

*If triggered by exposure in a cold environment, and no warm water is available, place the affected digits in a warm body cavity – arm pit, crotch, or even in the mouth. Keep the affected area warm at least until the whiteness returns to its normal, healthy color. Get out of the cold as soon as possible.

Drug therapy
:
*Treatment for Raynaud’s phenomenon may include prescription medicines that dilate blood vessels, such as calcium channel blockers (nifedipine) or diltiazem.  It has the usual common side effects of headache, flushing, and ankle edema; but these are not typically of sufficient severity to require cessation of treatment.

*There is some evidence that Angiotensin II receptor antagonists (often Losartan) reduce frequency and severity of attacks,and possibly better than nifedipine.

*Alpha-1 adrenergic blockers such as prazosin can be used to control Raynaud’s vasospasms under supervision of a health care provider.

*In a study published in the November 8, 2005 issue of Circulation, sildenafil (Viagra) improved both microcirculation and symptoms in patients with secondary Raynaud’s phenomenon resistant to vasodilatory therapy. The authors, led by Dr Roland Fries (Gotthard-Schettler-Klinik, Bad Schönborn, Germany), report: “In the present study, capillary blood flow was severely impaired and sometimes hardly detectable in patients with Raynaud’s phenomenon. Sildenafil led to a more than 400% increase of flow velocity.”

*Fluoxetine, a selective serotonin reuptake inhibitor, and other antidepressant medications may reduce the frequency and severity of episodes if caused mainly by psychological stress.

Surgical Intervention
:
*In severe cases, a sympathectomy   procedure can be performed. Here, the nerves that signal the blood vessels of the fingertips to constrict are surgically cut. Microvascular surgery of the affected areas is another possible therapy. Infusions of prostaglandins, e.g. prostacyclin, may be tried, with amputation in exceptionally severe cases.

*A more recent treatment for severe Raynaud’s is the use of Botox. The 2009 article studied 19 patients ranging in age from 15 to 72 years with severe Raynaud’s phenomenon of which 16 patients (84%) reported pain reduction at rest. 13 patients reported immediate pain relief, 3 more had gradual pain reduction over 1-2 months. All 13 patients with chronic finger ulcers healed within 60 days. Only 21% of the patients required repeated injections. A 2007 article describes similar improvement in a series of 11 patients. All patients had significant relief of pain.

Sometimes in cases of severe Raynaud’s, approaches other than medications may be a treatment option:

*Nerve surgery
. Nerves called sympathetic nerves in your hands and feet control the opening and narrowing of blood vessels in your skin. Sometimes it’s necessary in cases of severe Raynaud’s to cut these nerves to interrupt their exaggerated response. Through small incisions in the affected hands or feet, a doctor strips away these tiny nerves around the blood vessels. The surgery, called sympathectomy, may reduce the frequency and duration of attacks, but it’s not always successful.
*Chemical injection. Doctors can inject chemicals to block sympathetic nerves in affected hands or feet. You may need to have the procedure repeated if symptoms return or persist.
*Amputation. Sometimes, doctors need to remove tissue damaged from a lack of blood supply. This may include amputating a finger or toe affected by Raynaud’s in which the blood supply has been completely blocked and the tissue has developed gangrene. But this is rare.


Alternative and Experimental (Research) Approaches
:-
Lifestyle changes and supplements that encourage better circulation may be effective alternatives for managing Raynaud’s. If  one is interested, may talk to the doctor about:

*Biofeedback. Biofeedback — using your mind to control body temperature — may help decrease the severity and frequency of attacks. Biofeedback includes guided imagery to increase the temperature of hands and feet, deep breathing and other relaxation exercises. Your doctor may be able to suggest a therapist who can help you learn biofeedback techniques. Books and tapes also are available on the subject.

*Niacin. Niacin, also known as vitamin B-3, causes blood vessels to dilate, increasing blood flow to skin. Niacin supplements may be useful in treating Raynaud’s, although niacin supplements may have side effects.
*The extract of the Ginkgo biloba leaves (Egb 761, 80 mg) may reduce frequency of attacks.

*Two separate gels combined on the fingertip (somewhat like two-part epoxy, they cannot be combined before use because they will react) increased blood flow in the fingertips by about three times. One gel contained 5% sodium nitrite and the other contained 5% ascorbic acid. The milliliter of combined gel covered an area of ~3 cm². The gel was wiped off after a few seconds.

*Piracetam, a nootropic drug, can be useful as a long-term treatment for vasospastic disorders.

*Arginine, which increase nitrous oxide acts as a vasodilator

*Milder cases of Raynaud’s can often be addressed by biofeedback[23] or other techniques to help control involuntary body functions like skin temperature.

*Fish oil supplements which contain long-chain omega-3 fatty acids may help to control symptoms of primary Raynaud’s. There are few studies in the medical literature dealing with this subject. However, in one 1989 controlled, double-blinded study of 32 patients, consumption of roughly 6.5 grams of long chain omega-3 fatty acids in the form of fish oil significantly increased the time to onset or entirely prevented symptoms in response to cold in patients with primary Raynaud’s. Lower doses of fish oil such as may be commonly available from commercial vendors have not been studied and may not be as effective.

Coping with the stress and nuisance of Raynaud’s takes patience and effort. Work with your doctor to manage your condition and maintain a positive attitude. The majority of people with Raynaud’s respond to treatment..

Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose.


Rersources:
http://en.wikipedia.org/wiki/Raynaud’s_phenomenon
http://www.mayoclinic.com/health/raynauds-disease/DS00433/DSECTION=lifestyle-and-home-remedies
http://www.wrongdiagnosis.com/r/raynauds_phenomenon/symptoms.htm
http://www.myfootshop.com/detail.asp?Condition=Raynauds%20Disease.

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