Female Raynaud's Syndrome

• Dr Muhammad El Hennawy• Ob/gyn Consultant• Rass el barr central hospital and dumyat specialised hospital• Dumyatt – EGYPT• www. mmhennawy.co.nr

Raynaud’s• It is certainly one of the most peculiar of medical conditions afflicting

millions of people worldwide.• It is a circulatory disorder of blood vessels of the extremities (the

fingers, toes, tip of nose, lips , cheeks, ,ears and nipple of breast ) namely caused by an inappropriate response of the peripheral arteries in reaction to environmental stimuli usually to the cold -- results in skin color and Sensation changes

• This disorder is characterized by episodic attacks, called vasospastic attacks

• (Constriction of those vessels is a normal physiological response to low temperatures, helping the body conserve heat ).

• in the hot months the extremities may be red and too hot. The peripheral arteries overdilate in response to the heat

Raynaud's syndrome takes two forms• both primary and secondary Raynaud's conditions are now classified

under the single banner of Raynaud's syndrome

• 1 - About 90 percent of cases are of the type called Raynaud's disease or primary Raynaud's — an isolated condition with no connection to other medical problems. It most often affects women and usually sets in before the age of 40.

NB: Patients who have had Raynaud disease alone for more than 2 years and have not developed any additional manifestations are at low risk for developing an autoimmune disease.

• 2 - The remaining 10 percent of cases are termed Raynaud's phenomenon or secondary Raynaud's. This tends to start later in life, is connected to other medical factors; an underlying disease, for instance, or the long-term use of such vibrating tools as a chain saw or jackhammer

primary Raynaud's Secondary Raynaud's Raynaud's diseaseRaynaud's

phenomenon

milder form 90 % of all cases

much less common 10% of all cases

between 15 and 40 years of age

start later in life

the causeno cause can be identified after appropriate investigation

an underlying disease

usually affects both hands and both feet

usually affects either both hands or both feet

symptoms go completely after each attack

run in some families.

first treat the underlying cause

Names

• Raynaud's disease,• primary Raynaud's syndrome • idiopathic Raynaud's

phenomenon,

• Raynaud's phenomenon,• secondary Raynaud's

syndrome

• More recently this distinction has become less clear cut because some patients may develop collagen disorders several years after the onset of Raynaud's symptoms and patients with apparent primary Raynaud's disease may have low levels of autoantibodies present in their serum

Cold stress caffiene drugs

Heightened sensitivity to the body’s the sympathetic nervous systemrelease of the hormone serotonin

unkown vibration from tools genetic hormones

Cold peripheral Arterioles nicotine autoimmune an exaggerated contraction of the blood vessels

(intermittent vasospasm)

Skin colour changes sensation

CAUSES • There is no known cause for Raynaud syndrome.• variety of hypotheses ==sympathetic overactivity , the sensitivity of the digital arteries to

cold stimuli, a defect in nitric oxide synthesis in the extremities, a deficiency of magnesium results in spasm of blood vessels

• Factors that precipetate Raynaud disease– Cold environments– Mental stressors– Smoking

• ======================================================= • The condition known as Raynaud phenomenon, however, is caused by a specific disease. • Causes of Raynaud phenomenon

– Rheumatologic diseases: 70% of scleroderma cases (or systemic sclerosis) will initially look like Raynaud phenomenon.

– Arterial diseases – Medications--- exposure to certain chemicals and drugs, such as beta-adrenergic blockers (used to

treat high blood pressure) or ergotamine (used to treat migraine headaches); – Certain occupations (vibration from tools)

-- Repetitive physical stress (for example, typing or playing the piano) – Carpal tunnel syndrome – Hypothyroidism – Trauma -- Tissue Injury, from  Frostbite or Surgery

severity• For most sufferers, Raynaud's

syndrome is a mild but maddening condition.

• Ordinarily, its most serious consequence is a loss of sensitivity in the affected extremity.

• Very rarely, a severe case results in tissue death and gangrene

Maurice Raynaud• Raynaud was a French medical student in the late 1800’s

who had to write a thesis as one of the requirements for graduation. He decided to write about the color changes he saw in the hands of women waiting for the streetcar on cold winter days.

• He is first described a group of 25 patients with local asphyxia and symmetrical gangrene of the extremities in 1862.

• He proposed that the observed changes were caused by vasospasm because most of the patients had palpable pulses at the wrist and patent large arteries were observed in those patients who underwent autopsy

INCIDENCE• 70 to 90 % are female• It occurs in 3 - 5% of the population • women are affected 5 times more often than men. • It usually occurs between the ages of 20 and 40 in

women and later in life in men. • the predominance of this disease in women and the

increased severity of symptoms between menarche and menopause suggest that hormonal factors may have an important role in the expression of the disorder

PATHOGENESIS• Cold exposure, emotional stimuli, or even cigarette smoking

produces profound pallor and numbness of the digits due to spasm of the digital arteries.

• The digital microvasculature dilates after a few minutes due to the accumulation of carbon dioxide and the products of hypoxic metabolism.

• As the vasospasm begins to relax a small amount of oxygenated blood enters these dilated vessels where it rapidly becomes desaturated and the pallor changes to cyanosis.

• As the digital vessels relax further, so normal blood flow is re-established and a reactive hyperaemia of the dilated microvasculature ensues as the cyanosis changes to rubor of the digits

Raynaud's attacks• It can also afflict some people’s the nose, lips and ear lobes and tongue. • They are classically described as being

accompanied by color change of the skin (white, blue, and then red) as shown.

Episodes can last anywhere from a minute to several hours.

The period of exposure to the cold

• It is extremely critical. • It only takes about 20 to 30 minutes of

exposure to the cold to cause potentially serious problems, such as tissue damage,

• This can lead to ulcers on the fingertips • Bone damage may also ensue • If left untreated, even gangrene.

• Typically fingers turn white, then blue, then red,

• indicating a progression from total blood deprivation to limited blood flow to sudden infusion of oxygenated blood as blood vessels suddenly dilate. Episodes are brief, usually lasting only a few minutes

• It consists of spasm of the arteries precipitated by cold and relieved by heat.

DIAGNOSIS• To diagnose Raynaud's phenomenon, a doctor may: • perform a thorough general physical examination and ask questions

about symptoms (as already mentioned, Raynaud's phenomenon may occur as a symptom of other illnesses) The cornerstone of diagnosis is a detailed medical history The diagnosis of Raynaud's syndrome depends mainly upon

the history of colour changes induced by cold exposure or occasionally by emotion. The involved extremity turns pale and numb when cold and makes a slow recovery

when warmed, taking 15 to 45 min to pass through the stages of cyanosis and redness back to a normal colour.

Pain in the digits is not a usual feature but may occur, particularly in the secondary varieties where there is digital vessel occlusion and a significant amount of ischaemic tissue damage, leading to ulceration and gangrene. This ischaemic damage may become so severe that the patient may require digital amputation despite the presence of a normal radial pulse

• take blood samples to test for other illnesses that may have similar symptoms

• examine the fingers under a special microscope to look for abnormal blood vessels.

• follow the patient's health status over the course of several visits.

the Allen Brown criteria• Primary Raynaud’s disease is diagnosed following the Allen Brown

criteria: • At least two of the three color changes occurring during attacks which

are provoked by cold and or stress.                                                      

• Episodes have been occurring periodically for at least two years.  • Attacks occurring in both hands.•                                                             • There is no other identifiable cause for the Raynaud’s episodes.• Additional testing which a physician might do to help with the

diagnosis:

• Antinuclear antibody blood test (usually negative in Raynaud’s disease).

• Examine the nailfold capillaries which should be in a normal pattern

laboratory tests

• Usually the attacks of Raynaud syndrome are temporary. A doctor relies on medical history to make a diagnosis.

• If the doctor suspects that patient may have Raynaud phenomenon caused by a specific disease, he may perform laboratory tests to find out.

DIFFERENTIAL DIAGNOSIS

Raynaud's of the nipple • A preventable cause of breastfeeding failure? • Raynaud’s disease can also occur in nipples.• In fact, it is much more common than generally believed. It can occur along with

any cause of sore nipples, but it may also, on occasion, occur without any other kind of nipple pain at all.

• This is a condition which is particularly common in women with Raynaud's disease and which is often overlooked or misdiagnosed by lactation consultants and physicians.

• The resulting pain is intense and could easily cause someone to stop breastfeeding.• Also ,the pain was deep in the breast, and entire chest area, shoulders, and back

(occur in the vascular systems of the breast and nipple )• using calcium and magnesium and herbal supplements. • try always staying warm when patient nursed • heating pad on one breast while nursing on the other• avoided stress and caffeine and exercised regularly • the prescription drug Nifidipine.. One 30-mg tablet of the slow release formulation,

once a day often takes away the pain of Raynaud’s disease • . After two weeks, stop the medication. If pain returns (about 10% of mothers), start

it again. After another two weeks, stop the medication. If pain returns (a very small number of mothers), start it again. Very few mothers took more than three courses. Side effects are uncommon, but headache does occur.

Raynaud's of the lungs? • it is possible that the lungs can be affected by

raynauds. • The blood vessels in the lungs can spasm by

raynaud's the same as those in hands, feet or elsewhere.

• When a person breathes in cold air the vessels in their lungs may constrict and cause significant trouble breathing.

• Some doctors have mentioned that the symptoms of this is very similar to those experienced by someone who is having an asthma attack.

Hand arm vibration syndrome (vibration white finger)

• the commonest cause of secondary Raynaud's. • This is caused by using vibrating tools regularly

over a long time. For example, it occurs in some shipyard workers, mine workers, road diggers, etc.

• It is thought that repeated vibrations over time may damage the small blood vessels or their nerve supply.

Juvenile Raynaud’s Syndrome • Both boys and girls can develop it, although it is

more commonly found in girls.

Raynaud's on fertility .

• It is also unknown

Raynaud's on conception

• It is also unknown

Pregnancy with Raynaud's• The impact of Raynaud's on pregnancy is also unknown,• Some doctors say women with Raynaud's disease may

actually feel better during pregnancy due to increased blood vessel dilation, yet little research has been done to confirm this.

• while pregnancy difficulties have been identified in women with lupus and other autoimmune diseases, less research has been done on Raynaud's phenomenon BUT autoimmune disease increases the risk of poor pregnancy outcome (ie, a small-for-date newborn, miscarriage, preterm delivery, preterm rupture of membranes, and even intrauterine fetal death)

Oral contraceptives with Raynaud's

• adverse reaction also have been reported in patients receiving oral contraceptives

• Women with Raynaud’s disease should not use birth control pills, as this method of contraception affects circulation.

Menopause with Raynaud's• Evening primrose oil inhibits the prostaglandins that

may otherwise promote blood vessel constriction• evening primrose oil reduced the number and

severity of attacks • use 3,000–6,000 mg of evening primrose oil per day

Hormone Replacement therapy with Raynaud's

• Unopposed estrogen therapy was associated with the Raynaud phenomenon in postmenopausal women (more than doubles ).

• This association was not present in women who were receiving combined hormone therapy.

Medications with Raynaud's

• Raynaud's can also be caused by some medications, including beta-blockers used for high blood pressure and heart trouble, and some migraine drugs.

Prevention is the best treatment for Raynaud syndrome

Avoid exposure to cold environments if possible.• Wear warm clothing over hands, feet, and entire

body.• Wear loose-fitting clothing in layers.• Avoid prolonged vibration to fingers.• Stop smoking or never start.

WHAT SHOULD PATIENT DO IF SHE HAVE AN ATTACK ?

• stay calm and follow these steps:• 1. Gently warm her fingers or toes as soon as she can.

Placing her hands under your armpits often helps• .2. Wiggle her fingers or toes. Move or walk around to try

to keep her blood flowing freely• .3. When her hands start feeling cold, hold them above her

head. Then swing them around as if she is throwing a softball

• .4. Run WARM - not hot - water over her fingers or toes until their normal color returns. Do not use a hot water bottle or heating pad

TREATMENT OF RAYNAUD'S SYNDROME • There is no cure for Raynaud's phenomenon, but in

most cases it can be controlled with proper medical care

Relief from Raynaud's is linked to improved circulation

• General measures • Therapy to the underlying disorder• Drug therapy ---Topical Treatment

--systemic ttt--- Nifedipin ,Chemical sympathectomy

• Surgical therapy --- Sympathectomy• Alternative treatments

General measures • No curative treatment is available for Raynaud's syndrome • the aim is therefore to palliate the symptoms by reducing

the frequency and the severity of the attacks • Reassurance that the outlook is generally benign• advice to wear thick woollen gloves or socks in cold weather the use of chemically activated handwarmers electrically heated gloves,• Perhaps less practical might be the advice to move to a

warmer climate. • simple avoidance of tobacco smoking (Smoking a cigarette

may produce a fall in temperature of 2 to 3°C in the fingertips ).

COLD PROTECTION • Dress warmly, especially during coldweather and during changes of season.• Wear layers of loose-fitting clothes.• Wear a hat in cold weather, since much body heat is lost through the scalp.• Cover face and ears with a scarf.• Wear loose-fitting boots and shoes that won't cut off blood supply.• Wear heavy socks or layers of socks.• Wear thick mittens - they're warmer than gloves.• Always keep a sweater or jacket with you, even during the summer. You may need

it in cold, air-conditioned buildings.. Use flannel sheets, layers of blankets, or an electric blanket on a low setting. Before

you get into bed, turn on your electric blanket to warm the sheets. If your hands and feet get cold when you sleep, wear mittens and socks to bed.Keep the rooms you use most often at a comfortable temperature.

. Start running your bath or shower water ahead of time so you don't touch cold water. Keep the bathroom door closed. The steam will warm the room.Enlist help from family and friends.

. In the winter, for example, let someone else get the newspaper from outside or start the car on a cold day.

. Avoid reaching into the freezer or doing tasks that require putting your hands into cold water.Wear thick gloves or mittens to reach into a freezer at home or at the grocery store.Use insulated containers or wear a glove or mitten to hold cold objects such as ice-cold drinks, frozen desserts, or fruit.Rinse and peel vegetables with warm water instead of cold. Use warm, not cold, water when rinsing food or washing dishes

• Mittens are warmer than gloves because the fingers keep each other warm.

• Protect from the cold.• If smoke consider giving it up.• Try cutting back on daily intake of caffeine

beverages, such as coffee.• Make sure to drink at least the recommended

daily amounts of fresh water. • use skin cream to keep your skin supple and

soft • keep the affected skin dry: as water on the

skin evaporates, it cools the skin which makes the circulatory changes worse

• try to avoid sweating too much or make sure you use clothes that wick sweat away from the body, because sweat cools the body

SKIN PROTECTION • Poor blood flow may make skin dry. It also may cause cuts, cracks, or sores to heal

more slowly than normal. Follow these tips to protect skin:• Use lotion with lanolin every day on hands and feet.This keeps skin from chapping

or cracking.• Wash with a mild, creamy soap.• Clean between fingers and toes, but don't soak them.• Examine feet and hands daily to check for ulcers. • If she develop an ulcer, keep it clean and covered. See her doctor right away.• Protect her nails. Use a lotion to keep her cuticles soft. Carefully cut hangnails and

file her nails in a rounded fashion to the tips of her fingers• .Wear rubber gloves while washing dishes• Be aware of activities that put pressure on her fingertips, such as using vibrating

tools or a manual typewriter or playing the guitar or piano. They may cause blood vessels to narrow, thus triggering an attack

• .Wear clothes made of natural fibers, such as cotton and wool. These draw moisture away from skin.

A simple exercise

• swinging the arms around like a windmill — will force blood into the extremities and may be as effective as drug therapy

Biofeedback• people can "teach" their blood vessels to relax. One form of

biofeedback is simply "thinking" her hands warm• Another is more specific • Starting in a warm room, place her hands in a warm bowl of

water for 5 minutes, then move to a cold room or outdoors and again place her hands in warm water, this time for 10 minutes. Repeat the procedure several times a day for as many days as necessary to produce a conditioned reflex that is the opposite of the normal one: when exposed to cold, the blood vessels in the fingers will dilate rather than constrict, without the aid of warm water.

Therapy to the underlying disorder.– Therapy must be tailored to the underlying disorder. – If associated with occupational or toxic exposure, the patient

should avoid the inciting environment. – Patients with hyperviscosity syndromes and cryoglobulinemia

improve with treatments that decrease the viscosity and improve the rheologic properties of their blood (eg, plasmapheresis).

– Unfortunately, patients with autoimmune disorders and associated Raynaud phenomenon usually do not respond well to therapy.

– Infections such as hepatitis B, hepatitis C, and Mycoplasma infections need to be addressed.

– In older patients with new-onset Raynaud and no obvious underlying cause, malignancy must be considered

Drug therapy – Use calcium channel blockers, especially those that cause

vasodilation. The most commonly used drug is nifedipine. Use the lowest dose of a long-acting preparation and titrate up as tolerated. If adverse effects occur, decrease dosage or use another agent such as nicardipine, amlodipine, or diltiazem.

– Angiotensin-converting enzyme inhibitors, angiotensin-receptor antagonists, and intravenous prostaglandins have been advocated, but they are still experimental.

– Therapy with antiplatelet agents has been tried but has not been proven effective, and anticoagulation is not indicated.

– Recently, losartan at 50 mg/d has been found effective in a study by Dziadzio et al of patients with primary Raynaud and scleroderma.

Topical Treatment • topical gels (a gel formulated from sodium nitrate and

ascorbic acid ) could provide effective and convenient treatment for severe Raynaud's syndrome without systemic effects.

The only disadvantages appear to be the messiness of the gel and stinging if applied to broken skin.

• Topical glyceryl trinitrate (nitro- glycerine ointment) has been found to be effective in patients with primary Raynaud's phenomenon and limited cutaneous systemic sclerosis.

Nifedipin One 30-mg tablet of the slow release formulation

sympatholytic agents

• (i.e., drugs that interfere with the effects of certain types of nerve transmission),

• including reserpine, prazosin, doxazosin, terazosin, methyldopa, guanethidine, and phenoxybenzamine.

Sympathectomy• If attacks become extremely frequent and severe and

interfere with her well-being and ability to work or function• Cervical sympathectomy still is considered controversial

and may only offer temporary relief.• Digital sympathectomy (Nerve blocks ) has been gaining

support for patients with severe or tissue-threatening disease. This may be used in patients with either primary or secondary disease, but it is more commonly necessary with the secondary forms.

• This procedure is usually not necessary and may only work for a short period of time.

Alternative treatments • diet, nutrition, • herbs,• exercise• acupuncture,• homeopathy,• mind-body medicine, • electrotherapy, • massage, • postural reeducation,• osteopathy

and chiropractic,• hydrotherapy,• breathing, • aromatherapy,

Nutrition and Vitamins• Nutrition• Vitamin E (400 to 800 IU per day) improves circulation and

helps certain blood cells function well. • Vitamin C (1,000 mg two to three times per day) supports

connective tissue and reduces swelling. • B-complex (50 to 100 mg per day) reduces stress. • Coenzyme Q10 (100 mg two times per day) promotes

healthy tissues. • Calcium (1,500 mg per day) and magnesium (200 mg three

times per day) relieves spasm. • Omega-3 oils (1,500 mg two to three times per day) reduce

swelling and help certain blood cells function well. • Zinc (30 to 50 mg per day) boosts your immune system.

Herbs • Take 20 to 30 drops two times per day.• Hawthorn berries (Crataegus laevigata) strengthens and

mildly dilates blood vessels • Ginkgo (Ginkgo biloba) (120 to 160 mg per day for dried

extracts) keeps blood cells from sticking together • Rosemary (Rosmarinus officinalis) is a gentle relaxant • Ginger root (Zingiber officianale) is a mild soothing agent • Prickly ash bark (Xanthoxylum clava-herculis) enhances

lymph activity and integrity of blood vessels

Acupuncture• that traditional Chinese acupuncture is a reasonable

alternative in treating patients with primary Raynaud's syndrome.

• a significant decrease in the frequency of attacks by 63 %• When attacks did occur, however, duration and

severity did not change significantly. Changes among control subjects were not significant

Prognosis

• Prognosis of primary Raynaud is usually very good, with no mortality and little morbidity.

• Prognosis of secondary Raynaud is related to the underlying disease. Prognosis for the involved digit in these patients is related to the severity of the ischemia and the effectiveness of maneuvers to restore blood flow

Summary of treatment • Most patients with Raynaud's syndrome can be managed

with • advice to stop smoking and to keep the extremity warm. • Failure of these simple measures in patients without digital

ischaemia is an indication for oral drug therapy with nifedipine or thymoxamine.

• The onset of digital ischaemia, especially in the connective tissue disorders, justifies a trial of intravenous iloprost therapy.

• Continued severe symptoms or ischaemia despite full medical treatment warrants consideration for sympathectomy.

• Local digital amputation may be the eventual outcome of severe ischaemic damage