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Diagnonistic Test

Radionuclide Scan of the Kidneys

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Definition
A radionuclide scan of the kidneys shows a picture of your kidneys while they are at work making urine.A kidney radionuclide scan, also called a kidney scan or renal scan, is a diagnostic imaging test that involves administering a small amount of radionuclide, also called a radioactive tracer, into the body and then imaging the kidneys with a gamma camera. The images obtained can help in the diagnosis and treatment of various kidney diseases and conditions. This test can be useful to evaluate infection, blockages, injury to the kidneys, and some causes of high blood pressure.

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Precautions
A kidney scan requires the use of a radioactive material; therefore, patients who are pregnant or suspect they may be pregnant are cautioned not to have the test unless the benefits outweigh the risks. Women should inform their doctor if they are breast feeding. The doctor will recommend the woman stop breast feeding for a specified period of time, depending on the particular tracer and dose used.

Description
Kidney scans are performed either in a hospital nuclear medicine department or in an outpatient radiology or nuclear medicine facility. The patient is positioned in front of, or under, a gamma camera—a special piece of equipment that detects the radiation emitted from the body and produces an image. An intravenous injection of the radionuclide is administered. Immediately after the injection imaging begins, and, in most studies, the flow of blood to each kidney is evaluated. Serial images of the kidneys are obtained over a specified period of time, depending upon the particular radiopharmaceutical used. Kidney scans may be performed to determine the rate at which the kidneys are filtering a patient’s blood. These studies use a radiopharmaceutical called technetium DTPA (Tc99m DTPA). This radiopharmaceutical also can identify obstruction in the renal collecting system. To establish the function of the renal tubules, the radiopharmaceutical Technetium DMSA (Tc99m DMSA) is used.

A kidney scan ranges from 45 minutes to three hours in length, depending upon the goals of the test, but the test typically takes about an hour to an hour and a half. It is important to understand that kidney scans can reveal an abnormality, but they do not always identify the specific problem. They are very useful in providing information about how the various parts of the kidneys function, which, in turn, can assist in making a diagnosis.

Typically, posterior images are obtained but images are also obtained at oblique angles. If indicated, the patient may be positioned so that mobility of the kidney is demonstrated by sitting up or lying down for the images. If obstruction or renal function is being evaluated, a diuretic (drug to induce urination), such as Lasix, may be injected. If hypertension or renal artery sterosis is being evaluated, Captopril or Enalapril (ACE inhibitors) may be injected.
Preparation
No special preparation is necessary for a kidney scan. In some instances the patient may be required to drink additional liquids and to empty their bladder before the exam. If another nuclear medicine study was recently performed, the patient may have to wait for a specified period to avoid any interference from residual radioactivity in the body. The patient is instructed to remove metal items from the area to be scanned.

Let your doctor know if you could be pregnant or if you are breast-feeding a baby. The medicine used in this test would expose your baby to radiation.

What happens when the test is performed.
You have an IV (intravenous) line placed into a vein. A slightly radioactive version of a substance called sodium pertechnetate is injected through the IV. This substance helps your kidneys and urine show up on pictures.

A camera that is specially designed to detect radioactivity is placed against your back or abdomen. A number of pictures are taken over time. The camera itself does not expose you to any additional radiation, so the number of pictures is not harmful in any way. The test is usually completed within an hour.

Risk Factors:
Many people worry when they hear that the medicine used in this test is slightly radioactive. In truth, this test exposes you to a very small amount of radiation-no greater than that of routine xrays.

Moreover, Nuclear medicine procedures are very safe. Unlike some of the dyes that may be used in x-ray studies, radioactive tracers rarely cause side effects. There are no long-lasting effects of the tracers themselves, because they have no functional effects on the body’s tissues. If pharmaceuticals are injected these can temporarily raise or lower blood pressure, or cause one to urinate.

Aftercare
Patients can resume their normal daily activities immediately after the test. Most radioactive tracers are excreted through the urinary system, so drinking fluids after a kidney scan can help flush the tracer out of the body more quickly.

Results
The scan should reveal normal kidney function for the patient’s age and medical status, as well as show normal relative position, size, configuration, and location of the kidneys. Initial blood flow images should reflect that blood circulation to both kidneys is equal. Patients whose images suggest a space-occupying lesion or obstruction may require other imaging procedures, such as CT or ultrasound, to provide more information. Also, if the kidneys appear to be abnormal in size, have an unusual contour, or are unusually positioned, other imaging procedures may be required.

Resources:
https://www.health.harvard.edu/fhg/diagnostics/radionuclide-scan-of-the-kidneys.shtml
http://www.enotes.com/nursing-encyclopedia/kidney-radionuclide-scan

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The Unfolding Mystery of Scleroderma

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Scleroderma, an autoimmune disease, tends to afflict middle-age women and can affect many parts of the body, inside and out.

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Lung disease, the biggest killer of scleroderma patients, is the main focus of research today..

Doctors have a growing arsenal of proven and potential treatments, some of which are risky and the subjects of current research, including stem cell transplants and powerful but toxic cancer drugs.

Like many autoimmune ailments, scleroderma remains a great unknown. Despite decades of research, the cause of this rare and complicated disease has yet to be discovered. But the good news is that doctors have a pretty clear understanding of how scleroderma progresses — a natural history, they call it — and are better than ever at extending and easing their patients’ lives.

“Lots of patients and lots of doctors used to consider it a ‘black box’ disease, a complete mystery, with little that could be done,” said Dr. Philip J. Clements of the University of California, Los Angeles, who is a scleroderma specialist. “Now there’s a body of evidence that tells us what to watch out for, and when.”

Experts now know, for example, that the gradual hardening of tissues and blood vessels that is a hallmark of scleroderma usually starts on the hands and face, with skin thickening, pitted scars and cool, pale fingertips among the earliest symptoms. Damage can then progress inward to internal organs, though the course varies widely from patient to patient. Of the 10,000 cases diagnosed among Americans each year, mainly women, a small subset will die quickly. But many others are able to manage their condition with a variety of treatments and have normal life expectancies.

Doctors also now know that if a patient’s internal organs are going to be affected as well as the skin, that is likely to happen in the first four or five years of the disease. So early diagnosis and close monitoring of the heart, lungs and kidneys are vitally important.

They have also learned that steroids, once viewed as a cure-all for immune disorders, can worsen the effects of scleroderma, especially in the kidneys, and should be used with caution.

“Learning which drugs to avoid was itself a big step,” said Dr. John Varga, the Gallagher Professor of Medicine at Northwestern University and chairman of the Medical Advisory Board for the Scleroderma Foundation, a nonprofit group that sponsors research and support for patients and families.

Kidney disease used to cause 90 percent of scleroderma-related deaths until the advent of a class of blood pressure drugs called angiotensin-converting enzyme, or ACE, inhibitors in the 1980s. ACE inhibitors prevent kidney damage by slowing down the chemicals that cause the muscles surrounding blood vessels to contract. Complications in the kidneys now account for only 14 percent of scleroderma deaths, Dr. Steen said.

The lungs are still a challenge. About 80 percent of scleroderma patients develop some form of lung problem — either pulmonary hypertension, due to hardening of the veins and arteries in the lung, or pulmonary fibrosis, in which the lung tissue becomes inflamed and then thickened with scarring. Some patients develop both. Either way, breathing becomes more difficult as the lungs become less pliable.

“If you die of a scleroderma-related problem, half of those deaths are from lung disease,” said Dr. Virginia Steen, a professor at Georgetown University and director of the Rheumatology Fellowship Program there. She wrote a seminal 2007 article that documented the shift from kidney disease to pulmonary disease as the biggest cause of death among scleroderma patients.

One successful remedy called Revatio, routinely prescribed since 2005, came from an unexpected source: Viagra. Repackaged from a little blue diamond to a round white tablet and renamed for marketing, dosage and insurance purposes, the drug works by relaxing the blood vessels and improving blood flow, whether for erectile or lung dysfunction.

“No one could understand why all these women were taking it four times a day,” said Frannie Waldron, chief executive of the Scleroderma Foundation.

Doctors also have a growing arsenal of experimental treatments and potential cures, some of which are risky.

Among them is cyclophosphamide, or Cytoxan, a powerful but highly toxic cancer drug that acts on the immune system. The drug decreases the inflammation that causes pulmonary fibrosis and has been used on scleroderma patients for the last 10 years.

But cytoxan has dangerous side effects, including an increased risk of bladder cancer, and usually is not given for more than a year. Moreover, the fibrosis seems to start again once drug treatments stop. Several studies involving the medication are under way, as well as efforts to find alternative treatments, many of them sponsored by drug companies.

Another big push involves stem cell transplant, an extremely risky process in which doctors try to reset the patient’s immune system and bypass the glitch that causes scleroderma. The procedure is the subject of a National Institutes of Health study called the SCOT trial, for Scleroderma: Cyclophosphamides or Transplantation?

Similar to a bone marrow transplant, doctors first draw the patient’s blood and extract the stem cells, the highly malleable building blocks that are thought to be free of the seeds of scleroderma. The patient is then subjected to high doses of radiation or chemotherapy with Cytoxan to kill the bone marrow. The last step is to reinfuse the stem cells, in the hopes that they replicate themselves in a healthy form free of disease.

The study will compare the benefits of the stem cell transplant with giving patients just monthly doses, but high ones, of Cytoxan. Preliminary results have been promising, several experts said.

“You’d think you’d have trouble recruiting for this,” said Dr. Arthur C. Theodore of Boston University, one of the investigators in the project. “But scleroderma patients are desperate.”

Sources: The New York Times

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Herbs & Plants

Eleuthero

Botanical Name:Eleutherococcus senticosus (formerly Acanthopanax senticosus)
Family :Araliaceae
Kingdom: Plantae
Division: Magnoliophyta
Class: Magnoliopsida
Order: Apiales
Genus: Eleutherococcus
Species: E. senticosus

Habitat:It is native to Northeastern Asia. Eleuthero is a shrub that grows in Siberia, China, Korea, and Japan.In Chinese medicine it is known as cì wu jia
Common names: Siberian ginseng, Ci wu jia, Touch-me-not, Devil’s shrub,devil’s shrub, devil’s root, touch-me-not

Description :The herb grows in mixed and coniferous mountain forests, forming low undergrowth or is found in groups in thickets and edges. E. senticosus is sometimes found in oak groves at the foot of cliffs, very rarely in high forest riparian woodland. Its native habitat is East Asia, China, Japan and Russia. E. senticosus is broadly tolerant of soils, growing in sandy, loamy and heavy clay soils with acid, neutral or alkaline chemistry and including soils of low nutritional value. It can tolerate sun or dappled shade and some degree of pollution. E. senticosus is a decidious shrub growing to 2m at a slow rate. It is hardy to zone 3. It flowers in July in most habitats. The flowers are hermaphrodite and are pollinated by insect.

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The dried root and other underground parts of the plant are used in herbal remedies for a variety of conditions. It is a distant relative of true (Panax) ginseng (which includes Asian ginseng and American ginseng), but it does not belong to the Panax group of herbs. Previously sold in the United States as “Siberian ginseng,” a 2002 United States law forbade the “ginseng” label, and the name “eleuthero” is now more commonly used.

Active Constituents:The constituents in eleuthero that have been most studied are the eleutherosides.1 Seven primary eleutherosides have been identified, with most of the research attention focusing on eleutherosides B and E.2 Eleuthero also contains complex polysaccharides (complex sugar molecules).3 These constituents may play a critical role in eleuthero’s ability to support immune function.

Eleuthero is an “adaptogen” (an agent that helps the body adapt to stress). It is thought to help support adrenal gland function when the body is challenged by stress

Medicinal Uses:
E. senticosus is an adaptogen which has a wide range of health benefits attributed to its use. Currently, most of the research to support the medicinal use of E. senticosus is in Russian or Korean. E. senticosus contains eleutherosides, triterpenoid saponins which are lipophilic and which can fit into hormone receptors. Supporters of E. senticosus as medicine claim it possesses a variety of medicinal properties, such as:

*increased endurance

*memory improvement

*anti-inflammatory

*immunogenic

*chemoprotective

*radiological protection

Eleutherococcus senticosis is more tonifying than the true Ginsengs (Panax sp.) It is neutral energetically and so is appropriate for daily use. Taken regularly, it enhances immune function, decreases cortisol levels and inflammatory response, and it promotes improved cognitive and physical performance. In human studies Eleuthero has been successfully used to treat bone marrow suppression caused by chemotherapy or radiation, angina, hypercholesterolemia, and neurasthenia with headache, insomnia, and poor appetite.

The major constituents of E. senticosus are Ciwujianoside A-E, Eleutheroside B (Syringin), Eleutherosides A-M, Friedelin and Isofraxidin.[2][unreliable source?] Most of the active constituents in E. senticosus are triterpenoid saponins. Though all terpenoid compounds have bioactivity in mammals, it is the triterpenes that are most important to the adaptogenic effect. The majority of known triterpenoid compounds in E. senticosus are found as saponin glycosides which refers to the attachment of various sugar molecules to the triterpene unit. These sugars are usually cleaved off in the gut by bacteria, allowing the aglycone (triterpene) to be absorbed. Saponin glycosides have the characteristic of reducing surface tension of water and will strip the lipids. This allows them insert into cell membranes (Attele et al., 1999) and modify the composition, influence membrane fluidity,[8] and potentially affect signaling by many ligands and cofactors.

Eleutherococcus senticosus has been shown to have significant antidepressant effects in rats

Although not as popular as Asian ginseng, eleuthero use dates back 2,000 years, according to Chinese medicine records. Referred to as ci wu jia in Chinese medicine, it was used to prevent respiratory tract infections, colds and flu. It was also believed to provide energy and vitality. In Russia, eleuthero was originally used by people in the Siberian Taiga region to increase performance and quality of life and to decrease infections.

In more modern times, eleuthero has been used to increase stamina and endurance in Soviet Olympic athletes. Russian explorers, divers, sailors, and miners also used eleuthero to prevent stress-related illness. After the Chernobyl accident, many Russian and Ukrainian citizens were given eleuthero to counteract the effects of radiation.

Eleuthero has been shown to enhance mental acuity and physical endurance without the letdown that comes with caffeinated products. Research has shown that eleuthero improves the use of oxygen by the exercising muscle.6 This means that a person is able to maintain aerobic exercise longer and recover from workouts more quickly. Preliminary research from Russia indicates it may be effective for this purpose. Other trials have been inconclusive8 or have shown no beneficial effect.

Eleuthero may also support the body by helping the liver detoxify harmful toxins. It has shown a protective action in animal studies against chemicals such as ethanol, sodium barbital, tetanus toxoid, and chemotherapeutic agents. According to a test tube study eleuthero also helps protect the body during radiation exposure. Preliminary research in Russia has suggested that eleuthero may help alleviate side effects and help the bone marrow recover more quickly in people undergoing chemotherapy and radiation therapy for cancer.

Eleuthero may be useful as a preventive measure during the cold and flu season. However, it has not yet been specifically studied for this purpose. Preliminary evidence also suggests that eleuthero may prove valuable in the long-term management of various diseases of the immune system, including HIV infection and chronic fatigue syndrome. Healthy people taking teaspoons (10 ml) of tincture three times daily have been shown to have increased numbers of the immune cells (T4 lymphocytes) that have been found to decrease during HIV-infection and AIDS.13 Further human clinical trials are needed to confirm that eleuthero may be helpful for this disease.

Dosages: Dried, powdered root and rhizomes, 2–3 grams per day, are commonly used.14 Alternatively, 300–400 mg per day of concentrated solid extract standardized on eleutherosides B and E can be used, as can alcohol-based extracts, 8–10 ml in two to three divided dosages. Historically, eleuthero is taken continuously for six to eight weeks, followed by a one- to two-week break before resuming.

Side Effects:Reported side effects have been minimal with use of eleuthero.15 Mild, transient diarrhea has been reported in a very small number of users. Eleuthero may cause insomnia in some people if taken too close to bedtime. Eleuthero is not recommended for people with uncontrolled high blood pressure. There are no known reasons to avoid eleuthero during pregnancy and breast-feeding. However, pregnant or breast-feeding women should be aware that some products may be adulterated with herbs that should not be taken in pregnancy, such as Asian ginseng. Only eleuthero from a trusted source should be used.

In one case report, a person taking eleuthero with digoxin developed dangerously high serum digoxin levels.16 Although a clear relationship could not be established, it is wise for someone taking digoxin to seek the advise of a doctor before taking eleuthero.

Drug interactions:
Certain medicines may interact with eleuthero. Refer to drug interactions for a list of those medicines.

*People with medicated high blood pressure should consult their doctor before taking E. senticosus as it may reduce their need for medication.

*E. senticosus may cause light sleep in some people, principally those who are “wired”. Users are recommended not to take it in the evening.

*E. senticosus will enhance the effectiveness of mycin class antibiotics.

*E. senticosus when purchased from non-GMP sources has occasionally been adulterated with Periploca graeca which can potentiate digoxin or similar drugs: however this is not an interaction of E. senticosus

You may click to learn more about Eleuthero->………………………….(1)….(2).….(3)

Disclaimer:The information presented herein is intended for educational purposes only. Individual results may vary, and before using any supplements, it is always advisable to consult with your own health care provider

Resources:
http://en.wikipedia.org/wiki/Eleutherococcus_senticosus
http://www.revolutionhealth.com/articles/eleuthero/hn-herb_eleuthero
http://www.cancer.org/docroot/ETO/content/ETO_5_3x_Siberian_Ginseng.asp

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Goodbye to High BP and Obesity

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Can a single pill treat problems of high blood pressure, reduce cholesterol level and act as a blood thinner? Seems like this may be possible soon. In one of the largest clinical trials being conducted for the first time, the impact of the ‘wonder drug’ is being assessed across 20 cities in India.

The trials of the drug ‘polypack’ manufactured by Cadila Pharmaceuticals are being conducted on 2,000 subjects, between the age of 45 and 80 years, who have any one of the risk factors – age, obesity, high blood pressure, high cholesterol or high blood sugar.

The ‘polypack’ contains a combination of five drugs in eight different formulations (three blood pressure lowering agents, an antiplatelet drug, a beta blocker, an ACE inhibitor, a diuretic and a statin) for treating cardio-vascular conditions such as reducing blood pressure and lowering cholesterol levels.

Simply put, the polypack includes blood pressure lowering combinations, aspirin (blood thinner) and a statin, which is a cholesterol lowering agent.

The trials are being conducted by St John Research Institute, Bangalore and Canada-based Population Health Research Institute.

“We need to assess the impact (efficacy) of the different combinations on an individual who has cardio-vascular risk factors, and also the safety of these drugs taken together. The side-effects of the five medicines will also be studied,” says Dr Rajeev Gupta of Fortis Hospital, Jaipur who is involved in the study.

The subjects will be given the combination drug for three months and a follow up will be done in the fourth month. The trials will be completed by July and findings put together by end of the year.

The second phase of trials will assess whether heart attacks and strokes can be prevented with the combination drug, Gupta added.

Cardiovascular diseases are major causes of mortality in the Indian subcontinent, causing over 30% of deaths. It has been predicted that these diseases will increase rapidly and India will contribute to over half the cases of heart diseases in the world within the next 15 years.

Coronary heart disease and stroke have increased in both urban and rural areas. The sharp rise in cardiovascular problems including heart disease and stokes is attributed to increasing number of smokers, obesity with high waist to hip ratio, high blood pressure, high cholesterol, stress and sedentary lifestyle.

 

Sources: The Times Of India

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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

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