Categories
Ailmemts & Remedies

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.

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.

Enhanced by Zemanta
Categories
Health Quaries

Some Health Quaries & Answers


Q.I‘ve suffered with Raynaud’s disease in my hands for two years. There is no underlying cause, such as lupus.

My doctor has prescribed blood pressure medicines that are supposed to work for this condition, but nothing seems to help. Is there a natural remedy that might help? Would acupuncture work? Any suggestions would be most appreciated, as cold weather makes it hard for me to function.

[amazon_link asins=’B06XWJ6XDQ,B01MA4AY6Q,B01N7FOE7Q,B01MSCYNHX’ template=’ProductCarousel’ store=’finmeacur-20′ marketplace=’US’ link_id=’ed171f40-47a9-11e7-8066-2f612f2e0890′]

A.People with Raynaud’s syndrome really suffer in the winter. In this condition, blood vessels in the hands and feet constrict. Fingers may turn white or blue and be painful or numb. The colder the temperature, the worse the symptoms.

Acupuncture may be helpful. One small study found that acupuncture reduced attacks by 63%, compared with a 27% reduction in the control patients (Journal of Internal Medicine, February 1997).

Some one reported that taking cinnamon capsules alleviated her Raynaud’s symptoms. In Chinese medicine, cinnamon and astragalus have been used for circulatory problems of this sort.

[amazon_link asins=’B01NBAOYX3,B01BNW5EFO,B01CRBESJI,B01LX8UHB4′ template=’ProductCarousel’ store=’finmeacur-20′ marketplace=’US’ link_id=’47a4bb79-47aa-11e7-aa50-b531b4da7ebe’]

Q.I was having trouble with my nails. Although I tried numerous remedies, some very expensive, nothing seemed to work. Then my daughter told me to try castor oil.

[amazon_link asins=’B0186U9736,B003CTTZQQ,B00VN79K2I,B01772051W’ template=’ProductCarousel’ store=’finmeacur-20′ marketplace=’US’ link_id=’71e0591c-47aa-11e7-8f16-af7008bae45a’]

A.I put it on my nails and leave it on for 15 or 20 minutes, then rub it in and wipe the residue off. It has made all the difference. I’m glad I found something so effective and yet so simple. I don’t get hangnails anymore either.Others have found almond oil helpful.

Sources: Los Angles Times

Reblog this post [with Zemanta]
Categories
Ailmemts & Remedies

Scleroderma

Definition:
Scleroderma (sklere-o-DER-muh) is a rare, progressive disease that leads to hardening and tightening of the skin and connective tissues    the fibers that provide the framework and support for your body. Scleroderma usually starts with a few dry patches of skin on the hands or face that begin getting thicker and harder. These patches then spread to other areas of the skin. In fact, scleroderma literally means “hard skin.”

Click to see the pictures> ….(1)…….. .(2)..…..

Scleroderma is a chronic disease characterized by excessive deposits of collagen in the skin or other organs. The localized type of the disease, while disabling, tends not to be fatal. The systemic type or systemic sclerosis, the generalized type of the disease, can be fatal as a result of heart, kidney, lung or intestinal damage

In some cases, scleroderma also affects the blood vessels and internal organs. Scleroderma is one of a group of arthritic conditions called connective tissue disorders. In these disorders, a person’s antibodies are directed against his or her own tissues.

Researchers haven’t established a definitive cause for scleroderma. It’s more common in women than in men and more common in adults than in children. Scleroderma can run in families, but in most cases it occurs without any known family tendency for the disease. Scleroderma isn’t considered contagious or cancerous, but this chronic condition can greatly affect self-esteem and the ability to accomplish everyday tasks.

Skin symptoms
Scleroderma affects the skin, and in more serious cases it can affect the blood vessels and internal organs. The most evident symptom is usually the hardening of the skin and associated scarring. The skin may appear tight, reddish or scaly. Blood vessels may also be more visible. Where large areas are affected, fat and muscle wastage may weaken limbs and affect appearance.

The seriousness of the disease varies hugely between cases. The two most important factors to consider are the level of internal involvement (beneath the skin) and the total area covered by the disease. In general, the more skin that is involved, the more severe the case of scleroderma.

For the systemic form of the disease, almost all patients(over 80%) have vascular symptoms and Raynaud’s phenomenon. During an attack, there is discoloration of the hands and feet in response to cold. Raynaud’s normally affects the fingers and toes.

Systemic scleroderma and Raynaud’s can cause painful ulcers on the fingers or toes which are known as digital ulcers.

Calcinosis is also common in systemic scleroderma, and is often seen near the elbows, knees or other joints.

Other organs
Diffuse scleroderma can cause musculoskeletal, pulmonary, gastrointestinal, renal and other complications.Patients with larger amounts of cutaneous involvement are more likely to have involvement of the internal tissues and organs.

Musculoskeletal
The first joint symptoms that patients with scleroderma have are typically non specific joint pains, which can lead to arthritis, or cause discomfort in tendons or muscles. Joint mobility, especially of the small joints of the hand, may be restricted by calcinosis or skin thickening. Patients may develop muscle weakness, or myopathy, either from the disease, or its treatments.

Lungs
Some impairment in lung function is almost universally seen in patients with diffuse scleroderma on pulmonary function testing;[4] however, it does not necessarily cause symptoms, such as shortness of breath. Some patients can develop pulmonary hypertension, or elevation in the pressures of the pulmonary arteries. This can be progressive, and lead to right sided heart failure. The earliest manifestation of this may be a decreased diffusion capacity on pulmonary function testing.

Other pulmonary complications in more advanced disease include aspiration pneumonia, pulmonary hemorrhage and pneumothorax.

Digestive tract
Diffuse scleroderma can affect any part of the gastrointestinal tract. The most common manifestation in the esophagus is reflux esophagitis, which may be complicated by peptic stricturing, or benign narrowing of the esophagus. This is best initially treated with proton pump inhibitors for acid suppression, but may require bougie dilatation in the case of stricture.

click to see..(2)

Scleroderma can decrease motility anywhere in the gastrointestinal tract. The most common source of decreased motility involvement is the esophagus and the lower esophageal sphincter, leading to dysphagia and chest pain. As Scleroderma progresses, esophageal involvement from abnormalities in decreased motility may worsen due to progressive fibrosis (scarring). If this is left untreated, acid from the stomach can back up into the esophagus causing esophagitis, and GERD. Further scarring from acid damage to the lower esophagus many times leads to the development of fibrotic narrowing, also known as strictures which can be treated by dilitation, and Barrett’s esophagus. The small intestine can also become involved, leading to bacterial overgrowth and malabsorption, of bile salts, fats, carbohydrates, proteins, and vitamins. The colon can be involved, and can cause pseudo-obstruction or ischemic colitis.

Rarer complications include pneumatosis cystoides intestinalis, or gas pockets in the bowel wall, wide mouthed diverticula in the colon and esophagus, and liver fibrosis. Patients with severe gastrointestinal involvement can become profoundly malnourished.

Scleroderma may also be associated with gastric antral vascular ectasia (GAVE), also known as watermelon stomach. This is a condition where atypical blood vessels proliferate usually in a radially symmetric pattern around the pylorus of the stomach. GAVE can be a cause of upper gastrointestinal bleeding or iron deficiency anemia in patients with scleroderma.

Kidneys
Renal involvement, in scleroderma, is considered a poor prognostic factor and not infrequently a cause of death in patients with scleroderma…..click & see

The most important clinical complication of scleroderma involving the kidney is scleroderma renal crisis. Symptoms of scleroderma renal crisis are malignant hypertension (high blood pressure with evidence of acute organ damage), hyperreninemia (high renin levels), azotemia (kidney failure with accumulation of waste products in the blood) and microangiopathic hemolytic anemia (destruction of red blood cells). Apart from the high blood pressure, hematuria (blood in the urine) and proteinuria (protein loss in the urine) may be indicative.

In the past scleroderma renal crisis was almost uniformily fatal. While outcomes have improved significantly with the use of ACE inhibitors the prognosis is often guarded, as a significant number of patients are refractory to treatment and develop renal failure. Approximately 10% of all scleroderma patients develop renal crisis at some point in the course of their disease.Patients that have rapid skin involvement have the highest risk of renal complications.

Treatments for scleroderma renal crisis include ACE inhibitors, which are also used for prophylaxis, and renal transplantation. Transplanted kidneys are known to be affected by scleroderma and patients with early onset renal disease (within one year of the scleroderma diagnosis) are thought to have the highest risk for recurrence.

Types
There are three major forms of scleroderma: diffuse, limited (CREST syndrome) and morphea/linear. Diffuse and limited scleroderma are both a systemic disease, whereas the linear/morphea form is localized to the skin. (Some physicians consider CREST and limited scleroderma one and the same, others treat them as two separate forms of scleroderma.) There is also a subset of the systemic form known as “systemic scleroderma sine scleroderma”, meaning the usual skin involvement is not present.

Diffuse scleroderma…..click & see
Diffuse scleroderma (progressive systemic sclerosis) is the most severe form – it has a rapid onset, involves more widespread skin hardening, will generally cause much internal organ damage (specifically the lungs and gastrointestinal tract), and is generally more life threatening.

Limited scleroderma/CREST syndrome……click & see
The limited form is much milder: it has a slow onset and progression, skin hardening is usually confined to the hands and face, internal organ involvement is less severe, and a much better prognosis is expected.

In typical cases of limited scleroderma, Raynaud’s phenom…striction of the small arteries of exposed peripheries – particularly the hands and feet – in the cold. It is classically characterised by a triphasic colour change – first white, then blue and finally red on rewarming. The scleroderma may be limited to the fingers – known as sclerodactyly.

The limited form is often referred to as CREST syndrome. “CREST” is an acronym for the five main features:

1.Calcinosis
2.Raynaud’s syndrome
3.Esophageal dysmotility
4.Sclerodactyly
5.Telangiectasia

CREST is a limited form associated with antibodies against centromeres and usually spares the lungs and kidneys.

Morphea/linear scleroderma….…click & see
Morphea/linear scleroderma involves isolated patches of hardened skin – there generally is no internal organ involvement.

Diagnosis
Diagnosis is by clinical suspicion, presence of autoantibodies (specifically anti-centromere and anti-scl70/anti-topoisomerase antibodies) and occasionally by biopsy. Of the antibodies, 90% have a detectable anti-nuclear antibody. Anti-centromere antibody is more common in the limited form (80-90%) than in the systemic form (10%), and anti-scl70 is more common in the diffuse form (30-40%) and in African-American patients (who are more susceptible to the systemic form).

In 1980 the American College of Rheumatology agreed upon diagnostic criteria for scleroderma

Causes
There is no clear obvious cause for scleroderma and systemic sclerosis. Genetic predisposition appears to be limited: genetic concordance is small; still, there often is a familial predisposition for autoimmune disease. Polymorphisms in COL1A2 and TGF-β1 may influence severity and development of the disease. There is limited evidence implicating cytomegalovirus (CMV) as the original epitope of the immune reaction, and organic solvents and other chemical agents have been linked with scleroderma.

Click to see>Gene clue to fatal skin disease

One of the suspected mechanisms behind the autoimmune phenomenon is the existence of microchimerism, i.e. fetal cells circulating in maternal blood, triggering an immune reaction to what is perceived as “foreign” material.

A distinct form of scleroderma and systemic sclerosis may develop in patients with chronic renal failure. This entity, nephrogenic fibrosing dermopathy or nephrogenic systemic fibrosis, has been linked to the exposure to gadolinium-containing radiocontrast.

Bleomycin (a chemotherapeutic agent) and possibly taxane chemotherapy may cause scleroderma, and occupational exposure to solvents has been linked with an increased risk of systemic sclerosis.

Pathophysiology
The overproduction of collagen is thought to result from an autoimmune dysfunction, in which the immune system would start to attack the kinetochore of the chromosomes. This would lead to genetic malfunction of nearby genes. T cells accumulate in the skin; these are thought to secrete cytokines and other proteins that stimulate collagen deposition. Stimulation of the fibroblast, in particular, seems to be crucial to the disease process, and studies have converged on the potential factors that produce this effect.

A significant player in the process is transforming growth factor (TGFβ). This protein appears to be overproduced, and the fibroblast (possibly in response to other stimuli) also overexpresses the receptor for this mediator. An intracellular pathway (consisting of SMAD2/SMAD3, SMAD4 and the inhibitor SMAD7) is responsible for the secondary messenger system that induces transcription of the proteins and enzymes responsible for collagen deposition. Sp1 is a transcription factor most closely studied in this context. Apart from TGFβ, connective tissue growth factor (CTGF) has a possible role.

Damage to endothelium is an early abnormality in the development of scleroderma, and this too seems to be due to collagen accumulation by fibroblasts, although direct alterations by cytokines, platelet adhesion and a type II hypersensitivity reaction have similarly been implicated. Increased endothelin and decreased vasodilation has been documented.

Jimenez & Derk describe three theories about the development of scleroderma:

The abnormalities are primarily due to a physical agent, and all other changes are secondary or reactive to this direct insult.
The initial event is fetomaternal cell transfer causing microchimerism, with a second summative cause (e.g. environmental) leading to the actual development of the disease.
Physical causes lead to phenotypic alterations in susceptible cells (e.g. due to genetic makeup), which then effectuate DNA changes which alter the cell’s behavior.

Therapy
There is no cure for every patient with scleroderma, though there is treatment for some of the symptoms, including drugs that soften the skin and reduce inflammation. Some patients may benefit from exposure to heat.

A range of NSAIDs (nonsteroidal anti-inflammatory drugs) can be used to ease symptoms, such as naproxen. If there is esophageal dysmotility (in CREST or systemic sclerosis), care must be taken with NSAIDs as they are gastric irritants, and so a proton pump inhibitor (PPI) such as omeprazole can be given in conjunction.[citation needed]

Immunosuppressant drugs, such as mycophenolate mofetil (Cellcept), cyclophosphamide or methotrexate are sometimes used to slow the progress. Digital ulcerations and pulmonary hypertension can be helped by prostacyclin (iloprost) infusion. Iloprost being a drug which increases blood flow by relaxing the arterial wall.

While still experimental (given its high rate of complications), hematopoietic stem cell transplantation is being studied in patients with severe systemic sclerosis; improvement in life expectancy and severity of skin changes has been noted.

Treatment

Scleroderma has no known cure    there’s no treatment to stop the overproduction of collagen. Your doctor may recommend a number of medications to make it easier for you to live with scleroderma by treating its symptoms. Your doctor may also suggest medications to prevent complications of scleroderma that may affect various organs. Here are some of the many treatments prescribed for the symptoms and complications of this condition.

Skin changes
If you have localized scleroderma, your doctor may recommend a topical treatment, such as a moisturizer or corticosteroid medication that you apply to your skin. Corticosteroid medications impede your body’s ability to make substances that can cause inflammation.

If your condition involves a large area of skin, your doctor may recommend additional treatments. Doctors sometimes prescribe minocycline (Minocin, Dynacin) to control the skin-related (cutaneous) symptoms of scleroderma, although no studies have addressed its long-term effectiveness. In preliminary studies, light therapy (phototherapy) also has proved effective in treating the lesions that are associated with scleroderma, but more research is needed.

Cosmetic treatments are another consideration. Some people with scleroderma are discouraged or embarrassed by lesions and marks on the skin, including tiny dilated blood vessels that often appear on the face (telangiectasia). Specialized brands of foundation makeup and pulsed dye laser surgery can help camouflage or eliminate these lesions. Consult a dermatologist about treatments for skin changes.

Circulation problems
Your doctor may also prescribe medications to dilate blood vessels and promote circulation. These medications can prevent high blood pressure and kidney problems and help treat Raynaud’s phenomenon.

Medications that help with blood circulation include:

  • Calcium channel blockers.
  • Alpha blockers
  • Angiotensin-converting enzyme (ACE) inhibitors
  • Angiotensin II receptor blockers
  • Low-dose enteric-coated aspirin

Creams containing nitroglycerin also may help promote circulation.

Joint stiffness, pain and inflammation
Your doctor may prescribe anti-inflammatory medications such as aspirin, nonsteroidal anti-inflammatory drugs (NSAIDs) or low-dose corticosteroids to relieve joint pain and stiffness.

Often, along with NSAIDs, doctors prescribe certain medications called disease-modifying antirheumatic drugs (DMARDs). These medications seem to do their job by having an effect on immune systems that have gone out of control, but doctors don’t understand exactly how DMARDs work. Common DMARDs include:

  • Hydroxychloroquine (Plaquenil). This drug has relatively few side effects, and it’s also effective for the arthritis that can be associated with scleroderma. Apart from hydroxychloroquine’s apparent ability to affect the way immune cells work, scientists don’t completely understand how it helps tame the disease process.
  • Penicillamine (Cuprimine, Depen). Similar to other DMARDs, penicillamine can reduce inflammation. Its full effect may require many months to develop, but its beneficial effects may be longer lasting. However, because of a relatively high incidence of adverse reactions to this drug and studies casting doubt on its effectiveness, its use has declined in recent years.
  • Methotrexate (Rheumatrex, Trexall). This drug does its job by affecting cells that are responsible for some of the pain, inflammation and joint swelling that accompany scleroderma. Trials have shown conflicting results regarding the effectiveness of methotrexate in treating scleroderma.

Immunosuppresents are another class of medications that can help manage out-of-control immune systems. Cyclophosphamide (Cytoxan) is one example. This extremely potent medication works by damaging cells’ genetic information. In particular, it kills white blood cells called lymphocytes that are part of autoimmune disease.

Lung damage
If you have scleroderma that affects your lungs, you may need additional medications. Cyclophosphamide (Cytoxan) is sometimes used to treat pulmonary fibrosis. A 2006 study of people with scleroderma-related lung disease found cyclophosphamide modestly improved lung function and quality of life. The long-term effects of cyclophosphamide treatment in people with scleroderma are unknown. Bosentan (Tracleer) is an oral medication that has been approved for pulmonary hypertension in people with scleroderma.

Digestive difficulties
If scleroderma has affected your esophagus and you’re experiencing heartburn, your doctor may suggest prescription medications that decrease stomach acid production. These medications include H-2-receptor blockers and proton pump inhibitors. Your doctor may also suggest antibiotics, special diets and medications that improve your gut’s ability to contract.

Complications
Having systemic scleroderma may result in a number of other health conditions:

Gastrointestinal complications. In scleroderma, wasting occurs in the muscular walls of your intestine. This can reduce absorption of nutrients and movement within the intestine, resulting in weight loss and malnutrition. When scleroderma affects the muscular lining of your esophagus, heartburn can occur.
Lung complications. Scarring of lung tissue (pulmonary fibrosis) can result in reduced lung function, reduced ability to breathe and reduced tolerance for exercise. You may also develop high blood pressure in the arteries to your lungs (pulmonary hypertension).
Kidney complications. When scleroderma affects your kidneys, you can develop an elevated blood pressure and an increased level of protein in your urine. More serious effects of kidney complications may include renal crisis, which involves a sudden increase in blood pressure and rapid kidney failure.
Heart complications. Scarring of heart tissue increases your risk of heart arrhythmias and congestive heart failure, and can cause inflammation of the membranous sac surrounding your heart (pericarditis).

Self-care

You can take a number of steps to help manage your symptoms of scleroderma:

  • Stay active. Exercise keeps your body flexible, improves circulation and relieves stiffness. Range-of-motion exercises can help keep your skin and joints flexible.
  • Don’t smoke. Nicotine causes blood vessels to contract, making Raynaud’s phenomenon worse. Smoking can also cause permanent narrowing of your blood vessels. Quitting smoking is difficult — ask your doctor for help.
  • Manage heartburn. Avoid foods that give you heartburn or gas. Also avoid late-night meals. Elevate the head of your bed to keep stomach acid from backing up into your esophagus (reflux) as you sleep. Try over-the-counter antacids for relief of symptoms.
  • Protect yourself from the cold. Wear warm mittens for protection when your hands encounter cold temperatures   such as when you reach into a freezer. When you’re outside in the cold, cover your face and head and wear layers of warm clothing.

Coping skills

Depending on how you’re affected by scleroderma, you may benefit from physical therapy and occupational therapy. Therapists can help you manage pain, improve your strength and mobility, and work on performing essential daily tasks to maintain your independence. Ask your doctor to recommend a physical therapist or an occupational therapist.

As is true with other chronic diseases, living with scleroderma can place you on a roller coaster of emotions. Here are some suggestions to help you even out the ups and downs:

  • Maintain normal daily activities as best you can.
  • Pace yourself and be sure to get the rest that you need.
  • Stay connected with friends and family.
  • Continue to pursue hobbies that you enjoy and are able to do.

If scleroderma makes it difficult for you to do things you enjoy, ask your doctor about ways to get around the obstacles.

Keep in mind that your physical health can have a direct impact on your mental health. Denial, anger and frustration are common with chronic illnesses.

At times, you may need additional tools to deal with your emotions. Professionals, such as therapists or behavior psychologists, may be able to help you put things in perspective. They can also help you develop coping skills, including relaxation techniques.

Joining a support group, where you can share experiences and feelings with other people, is often a good approach. Ask your doctor what support groups are available in your community.

In addition, many chronic illnesses place you at an increased risk of depression. This isn’t a failure to cope but may indicate a disruption in your body’s neurochemistry that can be helped with appropriate medical treatment. Talk with your family, friends and doctor if you’re feeling depressed.

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.

Resources:
http://en.wikipedia.org/wiki/Scleroderma
http://www.mayoclinic.com/health/scleroderma/DS00362/DSECTION

Enhanced by Zemanta