Systemic Sclerosis Scleroderma Symptoms and Signs

Systemic Sclerosis Scleroderma Symptoms and Signs

Mentions getting an autopsy. I had stated they wouldn't find out what is wrong with me without an autopsy.

Scleroderma: eMedicine Rheumatology

Scleroderma: eMedicine Rheumatology

This link contains a VERY GOOD explanation of the symptom breakdown.

Systemic sclerosis. DermNet NZ

Systemic sclerosis. DermNet NZ


This link is very good. Here is the part I needed most.

Other skin changes include:

Itchy skin
Thickening of the skin of the fingers, then atrophy (thinned) and sclerosis (scarring). The fingers become spindle-shaped (sclerodactyly) from resorption of the fingertips.
Fragile nails become smaller with ragged cuticles
Taut, shiny skin that may have dark or pale patches (hyper- or hypopigmentation). The tight skin may affect most parts of the body, including the face, resulting in loss of expression and difficulty opening the mouth properly.
Visibly dilated blood vessels (telangiectases) appear on the fingers, palms, face, lips, tongue and chest.
Calcinosis (calcium deposits) develops in the skin, particularly the fingers, hands and other bony areas. These can breakdown and discharge chalky material.
Ulcers may follow minor injuries over the joints, or on the tips of fingers and toes where the circulation is poor. Ulceration can lead to dry gangrene and eventual loss of the tips of the fingers (like frost bite).
Ulcers may also arise over calcinosis and on the lower legs.
Sicca symptoms (dry eyes, dry mouth) and Sjogren syndrome
In addition to the skin changes, the disease affects many other organs. Problems that may occur include:

Friction rubs over the joints and tendons, particularly the knees.
Eye changes with tightness of lids, reduced tear secretion, retinopathy
Joint pain, muscle pain and weakness and limited movement resulting in contractures.
The digestive tract may be affected throughout its length. Oesophageal reflux is common causing difficulty in swallowing solid and liquid food. This can lead to nausea, vomiting, weight loss, stomach cramps, diarrhoea, constipation and bleeding.
Lung and heart involvement may manifest as shortness of breath, high blood pressure, chest pain, pleurisy, pneumothorax, pericarditis arrhythmias, general heart enlargement and heart failure.
Progressive kidney disease resulting in proteinuria, high blood pressure and eventually renal failure.

Scleroderma - Symptoms, Treatment and Prevention

Scleroderma - Symptoms, Treatment and Prevention


Definition of Scleroderma
Description of Scleroderma
Causes and Risk Factors of Scleroderma
Symptoms of Scleroderma
Diagnosis of Scleroderma
Treatment of Scleroderma
Questions To Ask Your Doctor About Scleroderma
Definition of Scleroderma
Scleroderma is a chronic, degenerative, autoimmune disorder that leads to the over-production of collagen in the body's connective tissue. The word "scleroderma" means "hardening of the skin" and refers to one of the possible physical effects of the disease.
If systemic (throughout the body), scleroderma is known as progressive systemic sclerosis.
Description of Scleroderma
Approximately 300,000 people in the U.S. have scleroderma. Women are affected three times as often as men. Although the disease can develop at any age, it most frequently appears in the third to fifth decades of life. It is most frequently a chronic and often progressive illness.
Collagen, a protein manufactured by the connective tissues of the body, is produced in excess in someone suffering with scleroderma. This over-production of collagen can be likened to the process of "scarring," which is the way the body heals a wound.
For persons with scleroderma, the production of collagen is abnormal, depositing it in various organs and/or tissues of the body, especially in layers of the skin.
It is important to stress that the symptoms of scleroderma vary greatly from person to person, as though each person with scleroderma has his/her own version of the disease. Although scleroderma can indeed be serious, most people are able to live a normal life span with varying degrees of discomfort and/or disability.
There are two forms of scleroderma: localized and generalized (also called systemic sclerosis).
Localized Scleroderma
affects mainly the skin in different areas of the body
may affect muscles and bone
does not affect internal organs
is not usually as severe as generalized scleroderma
does not usually develop into generalized scleroderma
Generalized Scleroderma
Generalized scleroderma affects the skin and/or internal body parts, such as blood vessels, the digestive system (esophagus, stomach, and bowel), the heart, lungs, kidneys, muscles, and joints.
Generalized scleroderma most often presents as either CREST syndrome or diffuse scleroderma.
CREST is an acronym derived from the syndrome's five most prominent symptoms:
C - calcinosis, painful calcium deposits in the skin
R - Raynaud's phenomenon, abnormal blood flow in response to cold or stress, often in the fingers
E - esophageal dysfunction, difficulty swallowing caused by internal scarring
S - sclerodactyly, tightening of the skin on the fingers and toes
T - telangiectasia, red spots on the hands, palms, forearms, face and lips
Diffuse scleroderma appears as thickening of the skin that often spreads from the fingers and hands to the face, trunk or major organs.
Other less common forms of scleroderma include chemically induced localized scleroderma, eosinophilic myalgia syndrome (a disorder caused by the ingestion of L-tryptophan), toxic oil syndrome (associated with contaminated oil), and graft-versus-host disease.
Effects Of Scleroderma On The Body
Scleroderma in the skin may cause the cessation of hair growth and sweating; and the tightening and thinning of skin over the knuckles or finger joints, resulting in ulcers (open sores) and curving of the digits.
Scleroderma in the kidney may cause severe hypertension (high blood pressure) and kidney failure.
Scleroderma in the gastrointestinal tract hampers the action of the smooth muscle that lines the esophagus, small intestine, and colon; resulting in swallowing difficulties, malabsorption of digested food into the circulation, severe constipation and/or possible intestinal obstruction.
Scleroderma in the lung makes the lungs less flexible and reduces their blood supply resulting in shortness of breath, susceptibility to bronchial problems, and pulmonary fibrosis (scarring and thickening of lung tissue).
Scleroderma in the heart and pericardium (fibrous sac that lines the heart) restricts the normal pulsation and pumping of the blood by encasing the heart in a shell of sclerosed tissue. This may lead to heart failure.
Scleroderma in the joints causes arthritic symptoms such as inflammation, pain, swelling, stiffness, redness, and, in some cases, joint deformity.

Causes and Risk Factors of Scleroderma
The cause of scleroderma is unknown. It is not contagious. It is not passed on from one generation to the next, except in rare circumstances.
Symptoms of Scleroderma
The following are some of the symptoms of scleroderma:
Gradual hardening, thickening, and tightening of the skin, usually in extremities such as hands, face, and feet
Skin discoloration
Numbness of extremities
Shiny skin
Small white lumps under the surface of the skin that erupt into a chalky white fluid
Raynaud's phenomenon (pain, numbness, and/or color changes in the hands caused by spasm of the blood vessels upon exposure to cold or emotional stress)
Telangiectasia (red spots on the hands, palms, forearms, face, and lips)
Pain and/or stiffness of the joints
Swelling of the hands and feet
Itching of the skin
Stiffening and curling of the fingers
Ulcers (sores) on the outside of certain joints, often the knuckles and elbows
Digestive problems such as heartburn, difficulty in swallowing, diarrhea, and constipation
Fatigue and weakness
Shortness of breath
Arthritis
Weight loss
Hair loss
Internal organ problems
Diagnosis of Scleroderma
The diagnosis of scleroderma is made by a medical history, physical examination and diagnostic tests.
The diagnostic tests may include skin biopsy (removal of a sample of skin for examination) and blood tests.
Other tests, such as pulmonary function studies for the lungs, chest X-rays, studies of gastrointestinal function, and electrocardiography (EKG of the heart) may be performed to determine the disease's severity and effect on the internal organs.
Treatment of Scleroderma
Currently, there is no cure for scleroderma and treatment involves alleviating specific symptoms associated with scleroderma.
To treat skin thickening, medications may include penicillamine.
To treat kidney problems, medications to lower blood pressure such as vasodilators, angiotensin-converting enzyme (ACE) inhibitors, and calcium channel blockers may help.
To treat gastrointestinal discomfort and infection, medications such as antacids, H2 blockers (e.g., Tagamet), omeprazole, and antibiotics may help.
To treat esophageal disease, any medication recommended by a physician should be taken in liquid or crushed form. Patients with esophageal reflux should avoid late-night meals and elevate the head of their bed 4 to 6 inches.
To treat joint pain and swelling, medications such as aspirin or nonsteroidal anti-inflammatory drugs (NSAIDs) may help.
To treat joint and muscle stiffness, steroid medications, as well as heat therapy, physical therapy, and exercise may help.
To treat Raynaud's phenomenon, calcium channel blockers may help.
Protection
One way to reduce the discomfort that comes with scleroderma is to take an active role in protection.
Keep warm - exposure to cold can trigger Raynaud's phenomenon
Develop a physical therapy program with your physician to keep joints flexible
Wear protective joint equipment to avoid sprains and strains of joints
Protect feet and hands from injury and infection
Moisturize the skin to keep it flexible
Avoid using strong detergents, cleansers, and solvents
Get enough sleep
Reduce stress
Do not smoke
Questions To Ask Your Doctor About Scleroderma
Do any tests need to be done for diagnosis?

What type of scleroderma is it?

What areas or organs have been affected?

How serious is the condition?

What treatment do you recommend?

How successful is the treatment?

Can the disease progression be stopped?

What is the prognosis of this disease?

Is there a support group for scleroderma?

Living with Scleroderma

Living with Scleroderma


by Charlene Collins, Staff Writer (Ranked #2 expert in Immune System Diseases)

Scleroderma is a disorder caused by inappropriate responses from the immune system. The disorder causes thickening of the skin and connective tissue of the body. Scleroderma can also affect the blood vessels, lungs and other internal organs. The thickening of the tissues occurs because the body manufactures too much collagen. It’s not known why the body’s immune system attacks the skin and connective tissue. Scleroderma can occur in families, though, to date, no gene has been identified as the causative factor.

There are two different forms of scleroderma, which are localized and diffuse scleroderma. Localized scleroderma occurs in certain parts of the body; diffuse scleroderma is systemic. In other words, the diffuse form of scleroderma can affect the organs of the body, including the kidneys, lungs, bowels and esophagus.

Scleroderma of the skin and connective tissue can make movement very difficult. The skin and connective tissue becomes stiff and hard from the excess collagen being produced by the body. The overall health of an individual can suffer when her/his esophagus, lungs or kidneys become impaired. If the esophagus becomes stiffened, swallowing may become difficult. If the lungs are affected, the individual may suffer from respiratory insufficiency. When the kidneys are affected by scleroderma, filtration problems may occur. An individual may also have blood pressure problems if the kidneys and/or blood vessels are affected.

With scleroderma, an individual is likely to experience different complications associated with the disorder. The complications that could arise are related to hardening of the skin and circulatory problems.

Calcinosis

The medical term for tissue hardening is called calcinosis. Calcinosis is more likely to occur in the bend of the knees, fingers, elbows, toes, and other joints of the body. Calcinosis of the tissues can be painful, especially if the calcified pieces of skin break off. The areas which are calcified can become infected.

Raynaud’s phenomenon

Aside from hardening of the skin, an individual with scleroderma may be very sensitive to heat and cold. This sensitivity is called Raynaud’s phenomenon. Raynaud’s phenomenon occurs when the tiny blood vessels go through periods of spasm and relaxation. The areas on the body most often affected by Raynaud’s phenomenon are the fingers, toes, ears, nose, and tongue. These areas may experience burning like pain due to ischemia (lack of oxygen rich blood flow to the tissues).

Sclerodactyly

Like calcinosis, sclerodactyly occurs when the skin thickens and hardens so much that movement is difficult. The skin becomes shiny with calcified particles on the skin.

Conclusion

Individuals with scleroderma may have reddened areas on the hands, ears, lips and face. These reddened areas are caused by dilated capillaries in the affected areas. People who have diffuse scleroderma are more likely to have other serious medical conditions which are secondary to the disorder. For instance, acid reflux is quite common in people who have scleroderma in their esophagus. The stomach acid can splash up from the stomach into the esophagus, if the bottom third of the esophagus is affected.

People can lead relatively healthy lives if their internal organs are not affected. When people die from scleroderma it is usually due to the secondary conditions caused by the systemic form of the disease. However, most people live relatively normal life spans. Eating can become difficult if the jaw becomes stiff and difficult to move. If the bowel becomes stiff, an individual may be prone to having fecal impactions.

The disease is chronic and usually gets worse as time goes on. The degree of disability will vary from person to person; it will depend on the type of scleroderma and which parts of the body are affected.

Family support is important for people who are diagnosed with scleroderma. Living day to day with this disease can be challenging. Your skin can be painful and itchy all at the same time. It is easy to get depressed when your body slowly becomes immobile. One of my aunts had scleroderma. She lived a long life; she had good days and bad days, but she got progressively worse until she died. The main thing I remember about her was that her hands looked hard and shiny.

Sources:

http://www.medicinenet.com/scleroderma/article.htm

http://www.healthscout.com/ency/68/311/main.html

http://www.mayoclinic.com/health/scleroderma/DS00362/DSECTION=tests-and-diagnosis

About Charlene Collins
Charlene specializes in health/wellness writing and also in diseases and conditions writing. Charlene wears many hats, she is Christian, mother, friend, cook, writer and ghostwriter.

Scleroderma - What Is It?

Scleroderma - What Is It?

http://sparkpeople.com/resource/health_a-z_detail.asp?AZ=412

What Is It?
Scleroderma is a poorly understood illness that causes widespread hardening of the skin, especially on the hands and face. It also can damage the lungs, heart, kidneys, digestive tract, muscles and joints. It is a long-lasting (chronic) autoimmune disorder, an illness in which the body's immune defenses mistakenly attack the body's own cells rather than protecting them from outside invaders. Scleroderma also is called progressive systemic sclerosis.

There are two types of scleroderma. In the limited form, also called limited systemic sclerosis, the skin is the primary target. In the diffuse form (diffuse systemic sclerosis), the damage not only affects the skin, but also can affect the lungs, kidneys and other internal organs.

In people with scleroderma, scientists have identified abnormal immune proteins called autoantibodies, which are programmed to attack specific components of body cells. They also have found abnormal accumulations of protective T cells (white blood cells that are part of the immune system) in the skin and elsewhere. Although scientists don't understand exactly what happens, they believe that the immune system, perhaps involving these autoantibodies or T cells, somehow damages the body's smallest arteries, called arterioles. These damaged arterioles leak fluid, which causes swelling. They also release chemical factors that stimulate cells called fibroblasts to produce too much collagen, a fibrous protein. In the skin, this leads to thickening, hardening and tightness. Elsewhere in the body, the autoimmune attack of scleroderma can damage the digestive tract, the linings of joints, the outside sheaths of tendons, muscles (including the heart muscle), portions of the heart that regulate heart rhythm, the small blood vessels and the kidney.

Scleroderma is rare, affecting about 14 in every 1 million people worldwide, and it is most common in women aged 35 to 54. The cause is unknown. For some reason, cells called fibroblasts make too much scar-type tissue in the skin and in organs throughout the body. A number of theories have been proposed to explain this, including abnormalities in blood vessel function, abnormal proteins and antibodies in the circulation, and abnormal amounts of chemical messengers instructing fibroblasts to become overly active. Because scleroderma is more common in women during the childbearing years, researchers have looked for a pregnancy-related factor to explain why scleroderma develops. One theory suggests that leftover fetal cells can still be circulating in the mother's bloodstream decades after pregnancy, and may play some role in triggering the autoimmune changes behind scleroderma. Genetic factors and infectious triggers have also been proposed.

Older studies have linked scleroderma to exposure to certain chemicals, specifically vinyl chloride, epoxy resins and aromatic hydrocarbons. Some people who took tryptophan, an amino acid that used to be sold as a dietary supplement, developed a condition similar to scleroderma called eosinophilia myalgia syndrome. Since tryptophan was removed from the market, no further cases of eosinophilia myalgia syndrome have been reported, but the clear link between tryptophan and eosinophilia myalgia syndrome raises the possibility that exposure to something in the environment could trigger scleroderma.

Very Good Article on Symptoms of Scleroderma

Arthritis & Other Rheumatic Diseases

http://www.rush.edu/rumc/page-1098987311761.html


Scleroderma

What is scleroderma?

Scleroderma, also called systemic sclerosis, is a chronic, degenerative disease that affects the joints, skin, and internal organs. Scleroderma is also associated with blood vessel abnormalities.

Scleroderma is considered to be a multifactorial condition. Multifactorial inheritance means that "many factors" are involved in causing a health problem. The factors are usually both genetic and environmental, where a combination of genes from both parents, in addition to unknown environmental factors, produce the trait or condition. Often one gender (either males or females) is affected more frequently than the other in multifactorial traits. Females are affected with scleroderma three to four times more often than males.

What are the symptoms of scleroderma?

Scleroderma can lead to scarring of the skin, joints, and other internal organs. The following are the most common symptoms of scleroderma. However each individual may experience symptoms differently. Symptoms may include:

thickening and swelling of the tips of the fingers

pale and tingly fingers that may become numb when exposed to cold or when emotionally upset (called Raynaud's phenomenon)

joint pain

taut, shiny, darker skin on large areas such as the face, that may hinder movement
appearance of spider veins

calcium bumps on the fingers or other bony areas

grating noise as inflamed tissues move

frozen (immobile) fingers, wrists, or elbows due to scarring of the skin

sores on fingertips and knuckles

scarring of the esophagus, leading to heartburn and difficulty swallowing

scarring of the lungs, leading to shortness of breath

heart failure and abnormal heart rhythms

kidney disease
The symptoms of scleroderma may resemble other medical conditions or problems. Always consult your physician for a diagnosis.

How is scleroderma diagnosed?

In addition to a complete medical history and physical examination, a diagnosis of scleroderma is usually based on the changes in the skin and internal organs. An antibody test may help distinguish the type of scleroderma present.

Treatment for scleroderma:

Specific treatment for scleroderma will be determined by your physician based on:

your age, overall health, and medical history
extent of the condition
your tolerance for specific medications, procedures, and therapies
expectation for the course of the disease
your opinion or preference
Treatment may include:

nonsteroidal anti-inflammatory medications or corticosteroids (to relieve pain)
penicillamine (to slow the skin thickening process and delay damage to internal organs)
immunosuppressive medications
treating specific symptoms, such as heartburn and Raynaud's phenomenon
physical therapy and exercise (to maintain muscle strength)
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Arthritis Disease Center l Disease Definitions l Arthritis Disease and Related Conditions

Arthritis Disease Center l Disease Definitions l Arthritis Disease and Related Conditions

http://www.arthritis.org/disease-center.php?disease_id=26&df=effects

SCLERODERMA

What are the effects?
Localized Scleroderma
The symptoms of localized scleroderma are isolated to the skin and underlying tissues. Two types are recognized: morphea and linear scleroderma.
Morphea: These are local patches of hardened skin. Red patches of skin develop white centers with purplish borders. Lesions remain active for weeks to several years. Spontaneous softening that leaves a darkened area of skin often occurs. In localized morphea, you will have one or a few patches, whereas in generalized morphea, the patches may grow over large areas of the body. Morphea can occur at any site. Occasionally the lesions can be extensive and gradually evolve into generalized morphea in which the patches extend over most or all of the body surface.
Linear: A single line or band of thickened and discolored skin develops. The line usually runs down an arm or leg, but sometimes it runs down the forehead. When the band runs down the forehead, it may be called en coup de sabre (“sword stroke” in French).
Systemic Sclerosis (Systemic Scleroderma)
This disease affects not only the skin and underlying tissues, but also affects the blood vessels and major organs of the body. Two types of systemic disease are recognized: limited and diffuse.
Limited: In this form, skin thickening is generally limited to the fingers, forearms, legs, face and neck. Raynaud’s phenomenon may be present for years before any other symptoms develop. People with this form are less likely than people with diffuse disease to develop severe organ involvement. CREST syndrome falls within the limited form of the disease and is named after its primary features: calcinosis, Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia. These symptoms will be discussed below.
Diffuse: In this form, skin thickening may occur anywhere on the body, including the trunk. Only a short interval of time will elapse between the onset of Raynaud’s phenomenon and significant organ involvement. Damage typically occurs over the first three to five years, after which most patients enter a stable phase that varies in length. During this phase, your skin will stay about the same and the rate of damage to internal organs slows or stops. After the stable phase is over, your skin will start to soften and more serious damage to internal organs is unlikely to occur.
Depending on the form and severity of the disease, you may experience these different symptoms:
Raynaud’s phenomenon: Cold temperatures or emotional distress cause the blood vessels feeding the hands and/or feet to constrict. This constriction will cause the hands and feet to feel cold, turn white then turn blue. Once the vessels open back up (after approximately 10 to15 minutes), the hands turn red or mottled. More than 90 percent of people with scleroderma have Raynaud’s phenomenon; it is often the first symptom of the disease. Skin ulcers on the fingertips can result from Raynaud’s phenomenon.
Skin changes: In the earliest stage of disease, your skin may appear mildly inflamed and swollen. The skin will progressively tighten and flexibility will decrease. As the disease progresses, the skin will become more thickened, sweat and oil glands will stop functioning, and the skin surface will become very dry and itchy. This stage will persist for one to three years, at which time the skin will begin to soften and thin.
Sclerodactyly: This is a term for the thickening of the skin of the fingers or toes.
Calcinosis: Calcium deposits can form under the skin or in the muscles. These deposits may cause recurrent inflammation or ulcers on the overlying skin.
Telangiectasias: Small blood vessels called capillaries near the surface of the skin can widen and be seen through the skin. These are not dangerous, but can be unsightly.
Arthralgias and myalgias: Pain and stiffness of the joints and muscles are common symptoms early in the course of systemic sclerosis. Later in the disease course, muscle atrophy and weakness are common.
Dental disease: If the skin around your mouth tightens, you may not be able to open your mouth very wide. You may also have a dry mouth due to secondary Sjögren’s syndrome. [link] Damage to connective tissues in the mouth can lead to loose teeth. These problems can lead to periodontal disease, tooth loss and difficulty chewing, which can compromise your nutritional status.
Esophageal dysmotility: Smooth muscles in the esophagus lose normal movement, causing swallowing difficulties and chronic heartburn.
Gastrointestinal dysfunction: Dysmotility along the whole gastrointestinal tract can cause reflux, early satiety, bloating, nausea, vomiting, cramping abdominal pain, diarrhea, weight loss and malnutrition.
Lung disease: Shortness of breath or difficulty breathing with exertion are the most common initial symptoms when your lungs have become involved with systemic sclerosis. The disease can cause both pulmonary fibrosis (hardening of the lung tissue due to collagen excess) and pulmonary hypertension (high blood pressure in the artery that carries blood from the heart to the lungs). Lung impairment is the leading cause of death in systemic sclerosis. Getting your lung function tested regularly is important for detecting lung disease early.
Kidney disease: Renal crisis is characterized by accelerated hypertension (high blood pressure) and rapidly progressive kidney failure. About 80 percent of cases of renal crisis occur within the first four or five years of disease, usually in people with diffuse disease.
Heart disease: Symptoms of heart disease are not seen until late in the course of systemic sclerosis. Symptoms include breathlessness on exertion, palpitations and, less frequently, chest pain.
Other signs and symptoms:
Fatigue
Appetite and weight loss
Depression
Sexual dysfunction
Impotence
Sjögren’s syndrome
Carpal tunnel syndrome
Thyroid disorders

collagen lung disease

collagen lung disease



Collagen lung disease



by Nathan Wei, MD, FACP, FACR

Nathan Wei is a nationally known board-certified rheumatologist and author of the Second Opinion Arthritis Treatment Kit. It's available exclusively at this website... not available in stores.

Click here: Second Opinion Arthritis Treatment Kit



Collagen lung diseases or as they are better known, interstitial lung disease (ILD) includes more than 180 chronic lung disorders, which are:




• chronic
• nonmalignant (non-cancerous)
• noninfectious


Interstitial lung diseases are named for the tissue between the air sacs of the lungs called the interstitium -- the tissue affected by fibrosis (scarring).

Interstitial lung diseases may also be called interstitial pulmonary fibrosis.

The symptoms and course of these diseases may vary from person to person, but the common link between the many forms of ILD is that they all begin with an inflammation:


bronchiolitis - inflammation that involves the bronchioles (small airways)

alveolitis - inflammation that involves the alveoli (air sacs)

vasculitis - inflammation that involves the small blood vessels (capillaries)



While more than 90 percent of interstitial lung diseases are due to hypersensitivity pneumonitis or other causes, a significant percentage of patients with ILD will have an autoimmune disease responsible for their pulmonary problem.

In interstitial lung disease, the lung is affected in three ways:

• Lung tissue is damaged in some known or unknown way.
• The walls of the air sacs in the lungs become inflamed.
• Scarring (fibrosis) begins in the interstitium.


Fibrosis results in permanent loss of that tissue's ability to breathe and carry oxygen. Air sacs, as well as the lung tissue between and surrounding the air sacs, and the lung capillaries, are destroyed by the formation of scar tissue.

The diseases may run a gradual or rapid course. The development of lung problems closely approximates the progression of the autoimmune disease elsewhere in the body. People with ILD may have varied symptoms -- from very mild to very severe. The condition may remain the same for long periods of time or it may change quickly. The course of ILD is unpredictable. If there is progression, the lung tissue thickens and becomes stiff. The work of breathing then becomes more difficult and demanding. Some of the diseases improve with medication if treated when inflammation occurs. Some people may need oxygen therapy as part of their treatment.

The following are the most common symptoms for interstitial lung diseases; however, each person may experience symptoms differently. Symptoms may include:

• shortness of breath, especially with exertion
• fatigue and weakness
• loss of appetite
• loss of weight
• dry cough that does not produce phlegm
• discomfort in chest
• labored breathing
• hemorrhage in lungs


The symptoms of interstitial lung diseases may resemble other lung conditions or medical problems. Consult your doctor for a diagnosis.

The cause of interstitial lung disease in collagen vascular disease is related to the underlying disorder. In addition to a complete medical history and physical examination, the doctor may also request the following tests:



pulmonary function tests - to determine characteristics and capabilities of the lungs

spirometry - to measure the amount of air that can be forced out

peak flow meter - to evaluate changes in breathing and response to medications

blood tests - to analyze the amount of carbon dioxide and oxygen in the blood

x-ray

computerized axial tomography (CAT) scan

bronchoscopy - to examine the lung using a long, narrow tube called a bronchoscope

bronchoalveolar lavage - to remove cells from lower respiratory tract to help identify inflammation and exclude certain causes

lung biopsy - to remove tissue from the lung for examination in the pathology laboratory



Specific treatment will be determined by your doctor(s) based on:

• age
• overall health and medical history
• extent of the disease
• tolerance for specific medications, procedures or therapies
• expectations for the course of the disease
• your opinion or preference


Treatments may include:

• oral medications, including corticosteroids
• intravenous medications, including immunosuppressive agents or biologic therapies
• influenza vaccine
• pneumococcal pneumonia vaccine
• oxygen therapy from portable tanks
• lung transplantation






An excellent review was published by the Society of Thoracic Radiology…

Pleuropulmonary Manifestations of Collagen Vascular Disease

Gordon L. Weisbrod, M.D., F.R.C.P.C. The University of Toronto



The collagen vascular diseases (CVD) are a heterogenous group of chronic inflammatory and immune-mediated disorders that share certain clinical characteristics, including inflammation of joints and serosal membranes, connective tissues, and blood vessels in various organs. The frequency of thoracic involvement is considerable: Two thirds of 109 patients in one study showed radiological changes in lungs, pleura or heart; pathologic lesions were found at autopsy in 28 of 34 cases in another study.

These disorders frequently manifest distinctive clinical, radiological, and pathological features that permit classification into one disease process. However, considerable overlap may occur, combining features of more than one disease process.



Rheumatoid Arthritis (RA)

In general, most of the pleuro-pulmonary lesions are commoner in males even though rheumatoid arthritis is largely a female disease. The lesions are also commoner in patients with subcutaneous nodules and highly positive tests for rheumatoid factor. The pleuro-pulmonary manifestations are traditionally considered under six headings.

1) Pleuritis and Pleural Effusion
This is the most frequent manifestation; in one series, 21% of 516 patients gave a history of pleurisy and 3.3% had effusion. Low glucose levels are characteristic (under 25mm%) and may be due to defective transport of glucose across the pleural surface membrane. Up to 1/3 of effusions are associated with no respiratory symptoms. They are more commonly unilateral, can be large, and associated parenchymal disease is uncommon.

2) Diffuse Interstitial Disease
In one large series, 1.6% of patients had diffuse interstitial disease radiologically although many more have pulmonary function test abnormalities. A reticulonodular infiltrate is seen, usually worse in the mid and lower lungs. Later, with progressive fibrosis, a course reticular pattern predominates often with honeycombing. The course varies from a few months to many years, ending for some in respiratory failure or cor pulmonale.

3) Necrobiotic Nodules
This is a relatively rare manifestation, appearing as single or multiple peripheral well defined nodules or masses ranging from a few millimeters to several centimeters in diameter, and often resembling metastases. Cavitation occurs in 1/3 to 1/2. The cavities may become infected to form an abscess.
Because the nodules are commonly situated in the periphery of the lung subpleurally and because cavitation is common, rupture into the pleural space may occur with the development of an effusion, empyema, pneumothorax, or bronchopleural fistula.

4) Caplan's Syndrome
This was originally described in 1953 in coal miners in South Wales. The syndrome consists of single or multiple nodules, usually developing rapidly, most often on a background interstitial pattern of coal-workers pneumoconiosis. The nodules often occur concomitantly with the joint changes of rheumatoid arthritis. They may cavitate, increase in size and number, remain unchanged, calcify, or disappear.

5) Pulmonary Arteritis and Pulmonary Hypertension
Narrowing of the pulmonary arteries and arterioles may lead to pulmonary hypertension and cor pulmonale.

6) Bronchiolitis Obliterans
This rare complication of rheumatoid arthritis should be considered in patients with shortness of breath with clear or hyperinflated lungs. No treatment is available and the course is progressively downhill resulting in death. Drug effects must be considered in the differential diagnosis of pulmonary infiltrates in these patients. Gold sodium thiomalate can cause a chronic interstitial pneumonitis, and sometime fibrosis. Penicillamine has also been related to changes in the lungs.



Systemic Lupus Erythematosis (SLE)

The lungs and pleura are involved more frequently in SLE than in any other collagen vascular disease, the incidence varying from 30 to 70%. The radiology can be considered under several categories, one or more being present at any one time.

1) Pleuritis With or Without Effusion
This is the commonest abnormality, being present at some stage in 50 to 75% of patients. The pleural disease is usually painful (as compared with rheumatoid arthritis) and associated with exacerbation of SLE. Effusions are usually bilateral and small.

2) Atelectasis
Areas of discoid atelectasis, usually in the lung bases, and often migratory, are commonly seen. They are frequently associated with pleuritis, effusion, and sometimes diaphragmatic dysfunction.

3) Acute Lupus Pneumonitis
This is a well recognized but very uncommon manifestation of SLE. Radiologically areas of consolidation are seen, usually in the lower lungs, simulating those of infection. The consolidation may become diffuse and extensive and lead to respiratory failure and death in spite of immunosuppressive therapy. The main differential diagnosis clinically and radiologically is that of infection.

4) Interstitial Disease
This is very uncommon in SLE. Radiologically the pattern is similar to that occurring in other collagen diseases such as rheumatoid arthritis and scleroderma. Honeycombing may occur.

5) Diaphragmatic Dysfunction With Loss of Lung Volume
Elevation of the diaphragm with "sluggish movement" and secondary loss of lung volume unshrinking lungs") has been noted in SLE. The inability of the diaphragm to generate normal pressures may be due to a myopathy or immobility and consequent disuse atrophy following extensive pleural adhesions.

7) Infection
This is the commonest abnormality and accounts for about 50% of the pleuropulmonary changes in SLE. Patients on high dose steroid therapy or with renal disease are especially susceptible. Fever and pulmonary involvement in such a patient with SLE should be regarded as presumptive evidence of life-threatening infection until proven otherwise.
The infections may be due to common community-acquired organisms or may be more esoteric reflecting the immunocompromised state.

8) Vasculitis
Vasculitis and pulmonary arterial hypertension are rare manifestations of SLE in the lungs.



Progressive Systemic Sclerosis (PSS)

This is a multisystem disease characterized by varying degrees of vascular change, inflammation and fibrosis of skin and internal organs. In one study, 90% of patients showed morphologic evidence of pulmonary fibrosis at autopsy. The roentgen pattern is one of diffuse reticular interstitial disease, affecting predominantly the lower lobes. Loss of lung volume is a prominent finding. One should also look for bone resorption of the distal clavicles, erosion of the superior cortex of the ribs, and air in the esophagus. The disease progresses at a variable rate and may eventually lead to respiratory failure and Oar pulmonale. Vascular lesions are common in PSS. They consist of intimal thickening of the small and medium sized pulmonary arteries and arterioles with narrowing of the lumina, resulting in pulmonary arterial hypertension. The disturbances in esophageal motility may lead to aspiration pneumonia. There is an increased incidence of bronchogenic carcinoma, especially bronchioloalveolar cell carcinoma in PSS. When respiratory symptoms suddenly worsen or change in PSS, a search should be made for carcinoma.



Polymyositis and Dermatomyositis (PM/DM)

The reported incidence of interstitial lung disease is around 5%. Radiologically, a diffuse reticular or reticulo-nodular infiltrate is seen identical to the other collagen vascular diseases. When polymyositis involves the muscles of respiration, especially the diaphragm, diaphragmatic elevation and small lung volumes are apparent, often in conjunction with atelectasis at the lung bases. This may be severe enough to produce acute respiratory failure. Pharyngeal muscle paralysis may lead to aspiration pneumonia, a frequent terminal event in these patients. The incidence of coexistent carcinoma is probably not as great as is often assumed. Only 16% in one series and 6% in another series had carcinoma. Therefore exhaustive search for malignancy is seldom warranted.



Sjogren's Syndrome (SS)

This is a chronic inflammatory disorder characterized by xerostomia, xerophthalmia, and enlargement of the salivary glands. Pulmonary abnormalities are common in Sjogren's syndrome, varying from 9 to 29%. Lymphocytic infiltration of the mucous glands of the tracheo-bronchial tree with resulting atrophy causes dryness of the mucosa, inspissation of secretions, followed by tracheobronchitis, recurrent infections, and atelectasis. Pneumonia is a common cause of death. Diffuse interstitial fibrosis identical to that in other collagen vascular diseases may occur. A variety of Iymphoproliferative disorders ranging from the benign Iymphocytic interstitial pneumonitis to malignant Iymphoma occur but are very uncommon. Lymphoma usually causes areas of consolidation, nodular or mass-like lesions, and Iymphadenopathy and pleural effusion.



Overlap Syndromes and Mixed Connective Tissue Disease

Overlap syndromes combining features of RA, SLE, PSS, PM-DM have been described. A subgroup of the overlap syndrome has been identified in which antibodies to the ribonucleoprotein component of extractable nuclear antigens are found. This is called mixed connective tissue disease. The pulmonary abnormalities resemble those of SLE, PSS, and PM-DM. Pleural effusion and interstitial lung disease are the commonest. In general, the disease is characterized by an excellent response to steroid therapy.



Ankylosing Spondylitis (AS)

The pulmonary manifestations are principally of two types: chest wall restriction and upper lobe fibrobullous disease. The chest wall restriction is the result of fusion of the costovertebral joints. The resulting impairment of lung function is usually mild. Fibrobullous disease has a reported incidence of 1.3%. The onset ranges from 2 to 38 years after the onset of ankylosing spondylitis. Usually, the disease begins in one upper lobe, but eventually becomes bilateral. The earliest signs are apical pleural thickening and interstitial infiltrate in the lung apex. Cavities or bullae may develop in the areas of fibrosis. The disease may steadily progress or remain stable for years. The fibrosis may become quite extensive, with considerable volume loss and bronchiectasis, simulating tuberculosis. Mycetoma usually of aspergillus, and secondary infection (atypical tuberculosis) may develop in these cavities leading to haemoptysis.



References

1. Subclinical pulmonary involvement in CVD assessed by BAL. Am Rev Resp Dis 1986; 133:574.
2. The interstitial lung diseases associated with the collagen-vascular disorders. Clin Chest Med 1982;3:565-578.
3. Lung biopsy in RA. Am Rev Resp Dis 1985;131:770.
4. Bronchiolitis in RA. Chest 1984;85:705.
5. Pleuro-pulmonary manifestations in RA. Seminars Arth Rheumat 1984;13:235.
6. Necrobiotic nodules presenting in RA. Ann Rheum Dis 1982;41:21.
7. CT of rheumatoid lung disease. J Comp Ass Tomog 1984;8:881.
8. Progressive airway obliteration in adults and its association with rheumatoid disease. Quart J of Med 1977;184:427-444.
9. Pulmonary hypertension in SLE. J Rheumatol 1989;16:918-925.
10. Cardiopulmonary manifestations of SLE. Rheum Dis Clin North Am 1988;14:135 147.
11. Pulmonary manifestation of SLE. Seminars Arthritis Rheum 1985;14:202.
12. Massive pulmonary haemorrhage in SLE. J Rheumotol 1985;12:186.
13. Shrinking lungs, diaphragmatic dysfunction, and SLE. Am Rev Respir Dis 1985; 132:926-928.
14. Diffuse interstitial lung disease in SLE. Ann Intern Med 1973;79:37-45.
15. Proper interpretation of pulmonary roentgen changes in SLE. AJR 1971;111:510 517.
16. Lung involvement in PSS. Rheum Dis Clin North Am 1990; 16:199-216.
17. Interstitial lung disease in PSS: HRCT versus radiography. Radiology 1990; 176:755-759.
18. Pulmonary involvement in PSS: the detection of early change by thin-section CT, BAL and 99mTc-DTPA clearance. Respir Med 1989;83:403-414.
19. The lungs in systemic sclerosis (scleroderma): a review and new information. Seminars Arthritis Rheum 1988; 17:271 -283.
20. PSS subgroups. AJR 1981;137:277.
21. Pulmonary arterial hypertension in PSS. AJR 1975;124:461.
22. Interstitial lung disease in PM & DM. Clinical features and prognosis as correlated with histologic findings. Am Rev Respir Dis 1990;141 :727-733.
23. DM and malignancy. Med Clin North Am 1989;73:1211-1220.
24. Pulmonary disease in DM/PM. Seminars Arth Rheum 1984;14:60.
25. Interstitial lung disease in PM and DM: Analysis of six cases and review of the literature. Medicine 1976;55:89-104.
26. Respiratory manifestations of SS. Chest 1985;88:226.
27. Upper lobe fibrosis in AS. AJR 1975;124:466.
28. Pulmonary manifestations of the collagen vascular diseases. Clin Chest Med 1989; 10:677-722


librarian@vh.radiology.uiowa.edu
Electric Differential Multimedia Lab

75 Photos of Scleroderma

http://images.rheumatology.org/viewphoto.php?albumId=75672&imageId=2861481

http://medical-dictionary.thefreedictionary.com/Scleroderma

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Scleroderma
Definition
Scleroderma is a progressive disease that affects the skin and connective tissue (including cartilage, bone, fat, and the tissue that supports the nerves and blood vessels throughout the body). There are two major forms of the disorder. The type known as localized scleroderma mainly affects the skin. Systemic scleroderma, which is also called systemic sclerosis, affects the smaller blood vessels and internal organs of the body.
Description
Scleroderma is an autoimmune disorder, which means that the body's immune system turns against itself. In scleroderma, there is an overproduction of abnormal collagen (a type of protein fiber present in connective tissue). This collagen accumulates throughout the body, causing hardening (sclerosis), scarring (fibrosis), and other damage. The damage may affect the appearance of the skin, or it may involve only the internal organs. The symptoms and severity of scleroderma vary from person to person.
Scleroderma occurs in all races of people all over the world, but it affects about four females for every male. Among children, localized scleroderma is more common, and systemic sclerosis is comparatively rare. Most patients with systemic sclerosis are diagnosed between ages 30 and 50. In the United States, about 300,000 people have scleroderma. Young African-American women and Native Americans of the Choctaw tribe have especially high rates of the disease. In 2003, researchers reported that they had identified 12 different genetic markers associated with scleroderma in the Choctaw population.
Causes and symptoms
The cause of scleroderma is still a puzzle. Although the accumulation of collagen appears to be a hallmark of the disease, researchers do not know why it occurs. Some theories suggest that damage to blood vessels may cause the tissues of the body to receive an inadequate amount of oxygen—a condition called ischemia. Some researchers believe that the resulting damage causes the immune system to overreact, producing an autoimmune disorder. According to this theory of scleroderma, the immune system gears up to fight an invader, but no invader is actually present. Cells in the immune system called antibodies react to the body's own tissues as if they were foreign. The antibodies turn against the already damaged blood vessels and the vessels' supporting tissues. These immune cells are designed to deliver potent chemicals in order to kill foreign invaders. Some of these cells dump these chemicals on the body's own tissues instead, causing inflammation, swelling, damage, and scarring.
Most cases of scleroderma have no recognizable triggering event. Some cases, however, have been traced to exposure to toxic (poisonous) substances. For example, coal miners and gold miners, who are exposed to high levels of silica dust, have above-average rates of scleroderma. Other chemicals associated with the disease include polyvinyl chloride, benzine, toluene, and epoxy resins. In 1981, 20,000 people in Spain were stricken with a syndrome similar to scleroderma when their cooking oil was accidentally contaminated. Certain medications, especially a drug used in cancer treatment called bleomycin (Blenoxane), may lead to scleroderma. Some claims of a scleroderma-like illness have been made by women with silicone breast implants, but a link has not been proven in numerous studies.
Symptoms of systemic scleroderma
A condition called Raynaud's phenomenon is the first symptom in about 95% of all patients with systemic scleroderma. In Raynaud's phenomenon, the blood vessels of the fingers and/or toes (the digits) react to cold in an abnormal way. The vessels clamp down, preventing blood flow to the tip of the digit. Eventually, the flow is cut off to the entire finger or toe. Over time, oxygen deprivation may result in open ulcers on the skin surface. These ulcers can lead to tissue death (gangrene) and loss of the digit. When Raynaud's phenomenon is the first sign of scleroderma, the next symptoms usually appear within two years.
SKIN AND EXTREMITIES. Involvement of the skin leads to swelling underneath the skin of the hands, feet, legs, arms, and face. Swelling is followed by thickening and tightening of the skin, which becomes taut and shiny. Severe tightening may lead to abnormalities. For example, tightening of the skin on the hands may cause the fingers to become permanently curled (flexed). Structures within the skin are damaged (including those producing hair, oil, and sweat), and the skin becomes dry and scaly. Ulcers may form, with the danger of infection. Calcium deposits often appear under the skin.
In systemic scleroderma, the mouth and nose may become smaller as the skin on the face tightens. The small mouth may interfere with eating and dental hygiene. Blood vessels under the skin may become enlarged and show through the skin, appearing as purplish marks or red spots. This chronic dilation of the small blood vessels is called telangiectasis.
Muscle weakness, joint pain and stiffness, and carpal tunnel syndrome are common in scleroderma. Carpal tunnel syndrome involves scarring in the wrist, which puts pressure on the median nerve running through that area. Pressure on the nerve causes numbness, tingling, and weakness in some of the fingers.
DIGESTIVE TRACT. The tube leading from the mouth to the stomach (the esophagus) becomes stiff and scarred. Patients may have trouble swallowing food. The acid contents of the stomach may start to flow backward into the esophagus (esophageal reflux), causing a very uncomfortable condition known as heartburn. The esophagus may also become inflamed.
The intestine becomes sluggish in processing food, causing bloating and pain. Foods are not digested properly, resulting in diarrhea, weight loss, and anemia. Telangiectasis in the stomach or intestine may cause rupture and bleeding.
RESPIRATORY AND CIRCULATORY SYSTEMS. The lungs are affected in about 66% of all people with systemic scleroderma. Complications include shortness of breath, coughing, difficulty breathing due to tightening of the tissue around the chest, inflammation of the air sacs in the lungs (alveolitis), increased risk of pneumonia, and an increased risk of cancer. For these reasons, lung disease is the most likely cause of death associated with scleroderma.
The lining around the heart (pericardium) may become inflamed. The heart may have greater difficulty pumping blood effectively (heart failure). Irregular heart rhythms and enlargement of the heart also occur in scleroderma.
Kidney disease is another common complication. Damage to blood vessels in the kidneys often causes a major rise in the person's blood pressure. The blood pressure may be so high that there is swelling of the brain, causing severe headaches, damage to the retinas of the eyes, seizures, and failure of the heart to pump blood into the body's circulatory system. The kidneys may also stop filtering blood and go into failure. Treatments for high blood pressure have greatly improved these kidney complications. Before these treatments were available, kidney problems were the most common cause of death for people with scleroderma.
Other problems associated with scleroderma include painful dryness of the eyes and mouth, enlargement and destruction of the liver, and a low-functioning thyroid gland.
Diagnosis
Diagnosis of scleroderma is complicated by the fact that some of its symptoms can accompany other connective-tissue diseases. The most important symptom is thickened or hardened skin on the fingers, hands, forearms, or face. This symptom is found in 98% of people with scleroderma. It can be detected in the course of a physical examination. The person's medical history may also contain important clues, such as exposure to toxic substances on the job. There are a number of nonspecific laboratory tests on blood samples that may indicate the presence of an inflammatory disorder (but not specifically scleroderma). The antinuclear antibody (ANA) test is positive in more than 95% of people with scleroderma.
Other tests can be performed to evaluate the extent of the disease. These include a test of the electrical system of the heart (an electrocardiogram), lung-function tests, and x-ray studies of the gastrointestinal tract. Various blood tests can be given to study kidney function.
Treatment
Mainstream treatments
As of early 2004 there is no cure for scleroderma. A drug called D-penicillamine has been used to interfere with the abnormal collagen. It is believed to help decrease the degree of skin thickening and tightening, and to slow the progress of the disease in other organs. Taking vitamin D and using ultraviolet light may be helpful for localized scleroderma. One group of British researchers reported in 2003 that long-wavelength ultraviolet A light is particularly effective in treating localized scleroderma. Corticosteroids have been used to treat joint pain, muscle cramps, and other symptoms of inflammation. Other drugs have been studied that reduce the activity of the immune system (immunosuppressants). Because these medications can have serious side effects, they are used in only the most severe cases of scleroderma.
The various complications of scleroderma are treated individually. Raynaud's phenomenon requires that people try to keep their hands and feet warm constantly. Nifedipine is a medication that is sometimes given to help control Raynaud's. Thick ointments and creams are used to treat dry skin. Exercise and massage may help joint involvement; they may also help people retain more movement despite skin tightening. An exercise regimen for stretching the mouth opening has been reported to be a helpful alternative to surgery in managing this condition. Skin ulcers need prompt attention and may require antibiotics. People with esophageal reflux will be advised to eat small amounts more often, rather than several large meals a day. They should also avoid spicy foods and items containing caffeine. Some patients with esophageal reflux have been successfully treated with surgery. Acid-reducing medications may be given for heartburn. People must be monitored for the development of high blood pressure. If found, they should be promptly treated with appropriate medications, usually ACE inhibitors or other vasodilators. When fluid accumulates due to heart failure, diuretics can be given to get rid of the excess fluid.
Patients with scleroderma may also benefit from some form of counseling or psychotherapy, as they are at increased risk of depression. One study found that 46% of the patients in its sample met the criteria for a depressive disorder.
Alternative treatments
One alternative therapy that some naturopaths have used in treating patients with scleroderma is superoxide dismutase (SOD), an antioxidant enzyme used in its injectable form. More research, however, needs to be done on the benefits of this treatment.
Prognosis
The prognosis for people with scleroderma varies. Some have a very limited form of the disease called morphea, which affects only the skin. These individuals have a very good prognosis. Other people have a subtype of systemic scleroderma called limited scleroderma. For them, the prognosis is relatively good. Limited scleroderma is characterized by limited involvement of the patient's skin and a cluster of five symptoms called the CREST syndrome. CREST stands for:
C = Calcinosis
R = Raynaud's disease (phenomenon)
E = Esophageal dysmotility (stiffness and malfunctioning of the esophagus)
S = Sclerodactyly (thick, hard, rigid skin over the fingers)
T = Telangiectasias
In general, people with very widespread skin involvement have the worst prognosis. This level of disease is usually accompanied by involvement of other organs and the most severe complications. Although women are more commonly stricken with scleroderma, men more often die of the disease. The two factors that negatively affect survival are male sex and older age at diagnosis. The most common causes of death include heart, kidney, and lung diseases. About 65% of all patients survive 11 years or more following a diagnosis of scleroderma.
As of early 2004 there are no known ways to prevent scleroderma. People can try to decrease occupational exposure to high-risk substances.
Key terms
Autoimmune disorder — A disorder in which the body's immune cells mistake the body's own tissues as foreign invaders; the immune cells then work to destroy tissues in the body.
Collagen — The main supportive protein of cartilage, connective tissue, tendon, skin, and bone.
Connective tissue — A group of tissues responsible for support throughout the body; includes cartilage, bone, fat, tissue underlying skin, and tissues that support organs, blood vessels, and nerves throughout the body.
Fibrosis — The abnormal development of fibrous tissue; scarring.
Limited scleroderma — A subtype of systemic scleroderma with limited skin involvement. It is somestimes called the CREST form of scleroderma, after the initials of its five major symptoms.
Localized scleroderma — Thickening of the skin from overproduction of collagen.
Morphea — The most common form of localized scleroderma.
Raynaud phenomenon/Raynaud disease — A condition in which blood flow to the body's tissues is reduced by a malfunction of the nerves that regulate the constriction of blood vessels. When attacks of Raynaud's occur in the absence of other medical conditions, it is called Raynaud disease. When attacks occur as part of a disease (as in scleroderma), it is called Raynaud phenomenon.
Sclerosis — Hardening.
Systemic sclerosis — A rare disorder that causes thickening and scarring of multiple organ systems.
Telangiectasias — Very small arteriovenous malformations, or connections between the arteries and veins. The result is small red spots on the skin known as "spider veins."

Extremely Informative Journal Article on Sclerodrema

http://webcache.googleusercontent.com/search?q=cache:8W7enzOEs44J:dermatology.cdlib.org/DOJvol8num1/reviews/scleroderma/haustein.html+systemic+sclerosis+scleroderma&cd=2&hl=en&ct=clnk&gl=us

Preparing a Gluten-Free Thanksgiving Dinner

Many of you will be preparing a Thanksgiving Dinner soon. I will be taking some shortcuts this year to make it easier, and have to make modifications to make it gluten-free, so here is my intended menu:

Sangria (I will probably make it this time with alcohol-free red wine and brandy extract instead of brandy, in order to spare my liver)

Roast turkey breast

Mashed potatoes (Readymade) with gluten-free turkey gravy (canned).

Cranberry relish (canned)

Slow-cooked Green beans with toasted sliced almonds

Instead of my traditional cornbread dressing this time I will have a pilaf of quinoa (gluten-free) cooked with cranberries, celery, onion and orange zest, all cooked in chicken broth.

Tomato aspic with celery, onion, and sliced pimento olives in it.

Dessert: Grandma's pumpkin pie, made with a gluten-free pecan shortbread cookie crumb crust. Served with heaps of light whipped cream.

Questions for a Possible Scleroderma Patient

http://rustyferguson.com/life/health/lupus/the-finger

1. Do you have any red or purplish marks on your face or fingers
2. Do you get color changes in your feet or fingers with cold or emotion
3. Do you have any hard bumps under the skin that could be subcutaneous calcinosis?
4. Does dry bread or pills linger in your lower esophagus?
5. Do you have episodes of heartburn or gastric reflux?
6. have your fingers swollen any? – any ring size changes?
7. Do you find your arms are falling asleep at night a lot more than they used to?
8. Having problem with constipation?
9. Any chronic cough?
10. Any problem with burning, aching hands and feet? Pricking pains in them? numbness? paresthesias?
11. Tender lower legs?
12. Tendonitis?
13. Burning, aching spine?
14 Aching all over? Pressure points at night?
15. Sleep apnea?
16. Thyroid problems?
17. Dry eyes? Dry mouth?

Symptoms of Scleroderma

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."

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.
Signs and symptoms

In addition to thickening and hardening of your skin, scleroderma can cause your skin to lose its elasticity and become shiny as it stretches across underlying bone. Other signs and symptoms may include:

Numbness, pain or color changes in your fingers, toes, cheeks, nose and ears, often brought on by cold or emotional distress (Raynaud's phenomenon)
Stiffness or pain in your joints and curling of your fingers
Digestive problems ranging from poor absorption of nutrients to delayed movement of food due to impaired muscular activity in your intestine
Sores over joints, such as your elbows and knuckles
Puffy hands and feet, particularly in the morning
Causes
Collagen is a fibrous type of protein that makes up your body's connective tissues, including your skin. Scleroderma results from an overproduction and accumulation of collagen in body tissues.

Although doctors aren't sure what prompts this abnormal collagen production, the body's immune system appears to play a role. For unknown reasons, the immune system turns against the body, producing inflammation and the overproduction of collagen. In addition to its effects on your skin, some types of scleroderma affect tiny blood vessels and can affect almost every organ.

Types of scleroderma
Doctors classify scleroderma into different subsets:

Localized scleroderma
This type of scleroderma is limited to your skin and the deep tissues below your skin. It includes the following subclassifications:

Morphea. In this form, oval-shaped thick patches appear on your skin — white in the middle, with a purple border. These patches are most likely to occur on your torso, but they can also appear on your arms, legs or forehead.
Linear scleroderma. This form results in bands or streaks of hardened skin on one or both of your arms or legs, or on your forehead.
Systemic scleroderma
This type of scleroderma affects not only your skin but also your blood vessels and major organs. It's also called systemic sclerosis and includes the following subclassifications:

Diffuse cutaneous systemic sclerosis. This type affects the skin of your fingers, hands, arms, legs, face, neck and trunk. It can also affect internal organs, such as your lungs, heart, kidneys and gastrointestinal tract — including your esophagus. It can hinder the functions of your digestive system, create respiratory problems and cause kidney failure. When left untreated, systemic scleroderma may be fatal within several years of onset.
Limited cutaneous systemic sclerosis. This type involves the skin of your fingers, lower arms and legs, face, and neck. A variation is called CREST syndrome.
Sine scleroderma. Some doctors may describe one variation of systemic scleroderma as sine scleroderma, which can be similar to either limited or diffuse scleroderma, the difference being that this form doesn't affect your skin.
Overlap syndrome
This is diffuse or limited systemic sclerosis with features of one or more of the other connective tissue diseases.

Undifferentiated connective tissue disease
This has features of systemic sclerosis, but there are no clinical or specific laboratory findings to make a definite diagnosis.

Systemic sclerosis is commonly called "scleroderma"

because the most obvious symptom is "hard skin." Being a systemic disease, however, it's more than skin deep. The fibrosis, or scarring, it causes can occur anywhere there's connective tissue in the body, and that's almost everywhere. Add to that the malfunction in and damage to blood vessels it can cause, and it seems like a sure recipe for disaster.

Even though most systemic scleroderma patients will not die from complications of their disease, most of us struggle to live with the affects. Profound fatigue is quite common, and you can imagine how that impacts someone's life. We frequently have the joint and muscle pain of secondary fibromyalgia, and almost all have some gastrointestinal involvement - at the very least, GI reflux. Raynaud's phenomenon is constant in about 97% of us - cold and stress cause blood vessels to spasm shut, cutting blood flow to hands, feet, nose, ears, and tongue. And these are the "minor" symptoms.

When it gets dangerous, scleroderma can cause fibrosis in the lungs and heart, further into the GI tract where it can cause malabsorption of nutrients, in the kidneys and it can affect the pulmonary artery. Treatment is getting better - kidney complications used to be the number one killer in scleroderma and are now very treatable with ACE inhibitors. But not all treatments work for everyone, and some just have limited usefulness.

You can find extensive information about all the forms of scleroderma at the Scleroderma Foundation website - http://www.scleroderma.org. I'd also recommend calling them at 1-800-722-HOPE for their free booklet, "Understanding and Managing Scleroderma," written in part by Dr Maureen Mayes, author of "The Scleroderma Book," another good source of info. The Scleroderma Foundation has numerous live support groups in the US where you and your mom could meet and talk to other patients and families about coping. They also directly or through their chapters sponsor local and regional educational meetings to not only benefit patients, but to spread information to health care providers on the latest research and treatments. Also, the SF funds over $1 million each year in peer-reviewed scleroderma research.

Symptoms of scleroderma may include one or more of the following:

Raynaud's phenomenon (numbness, color changes and pain in cheeks, nose, ears, fingers and toes due to abnormal sensitivity to cold)

Joint pain, swelling, and stiffness of the fingers, hands, forearms, feet, lower legs, and face, (especially in the fingers and knees)

Thickening, hardening, and discoloration of the skin

Ulcers or lesions on fingers, face, tongue, and inner lining of the cheek

Shortness of breath and coughing

Digestive and gastrointestinal problems including difficulty swallowing, bloating, and abdominal pain

Sexual dysfunction

Dry eyes

Carpal tunnel syndrome

Five particular symptoms occasionally occur together and are clinically recognized as a variation of scleroderma called CREST syndrome. The term CREST stands for Calcinosis (painful calcium deposits under the skin), Raynaud's phenomenon (abnormal sensitivity to cold in the hands and feet), Esophageal dysfunction (problems with swallowing caused by internal scarring), Sclerodactyly (tightening of the skin on the fingers or toes) and Telangiectasia (lesions on the hands, palms, forearms, face, and lips).

Scleroderma is called both a rheumatic disease and a connective tissue disease. The term rheumatic disease refers to a group of conditions characterized by inflammation and/or pain in the muscles, joints, or fibrous tissue. A connective tissue disease is one that affects tissues such as skin, tendons, and cartilage.

An individual with scleroderma may develop either a localized or a systemic form of the disease. Localized scleroderma usually affects only the skin on the hands and face. Systemic scleroderma, however, affects the connective tissue in many parts of the body, including the skin, the esophagus, gastrointestinal tract, lungs, kidneys, heart, and other internal organs. It is unusual for localized scleroderma to progress to the systemic form.