About Scleroderma

Scleroderma is classified as an autoimmune disease. This means that a person’s immune system works against itself. The normal immune system protects the body by fighting off foreign invaders, such as viruses and bacteria, and developing cancers. In an autoimmune disease, the immune system mistakes a person’s own tissues as foreign invaders and attacks the wrong things. 

In scleroderma, cells start making collagen as if there were an injury that needs repairing. The cells do not turn off as they should and end up making too much collagen. The extra collagen in the tissues can prevent the body’s organs from functioning normally. This is what is often referred to as fibrosis or “scar tissue”. 

Scleroderma has few rules about who gets it and why.  It’s a rare disease and fewer than half a million people in the United States are affected.  The disease is significantly more common in women than men and the most common age to develop scleroderma is between 35 and 50 years of age.  Young children and older adults can also develop scleroderma but this is less common. 

Adapting to a chronic illness can be challenging. Individuals are influenced by an illness but do not have to be defined by it.  While this is a hard goal to achieve, it may help you maintain your sense of self while coping with uncertainty. Your family, your doctor, your religious or community leader, or a counselor can help you with this goal. There are also several ways to be proactive about your health, including exercise, maintaining a healthy diet, and physical and occupational therapy. Your doctor can help you explore the path that is best for you. 

The earliest symptoms of Scleroderma are often fingers that become very sensitive to cold and/or stress and fingers that sometimes change color.  These changes in your fingers are known as Raynaud’s . However,  Raynaud’s phenomenon alone is fairly common in the general population but typically occurs in women under the age of 30. People who develop new onset of Raynaud’s phenomenon after the age of 30, autoimmune disease such as scleroderma should be considered. Sometimes other symptoms are present early in the disease such as puffy fingers, shortness of breath or kidney problems.  

Limited Scleroderma is often a milder form of scleroderma and is more common among patients who self-identify as White.  Patients with limited scleroderma, also formerly known as CREST (calcinosis, Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia) syndrome, often have only thickening of the skin on the fingers.  Patients with limited scleroderma may have a lower risk of internal organ complications, such as lung fibrosis and renal crisis, but it is important to perform routine screening tests as each patient may have a different clinical course.  

Diffuse scleroderma is the most involved form of scleroderma. Thickness of the skin on the arms, legs and trunk can occur, and internal organs are more likely to be involved.  Tightened skin makes movement of some joints difficult.  People may experience trouble bending fingers, hands and joints. Also, they may have a smaller mouth opening caused by tightness on the face.  This makes it difficult to open the mouth wide and makes it hard to close the mouth completely over the teeth. 

A person who has diffuse scleroderma may have areas of the skin that are lighter and darker in pigment.  Some will lose hair, have dry skin and sweat less all due to changes in the skin.  Diffuse scleroderma can cause changes to the gastrointestinal tract, heart, lung or kidneys. 

Increasingly, physicians use markers in the blood called autoantibodies to help determine the course of scleroderma and overall prognosis. Not every patient will produce an autoantibody, but the three most common include centromere, topoisomerase (Scl-70), and RNA polymerase 3. Typically, a patient will only produce one scleroderma autoantibody, and not multiple. Knowledge of the autoantibody helps determine how a patient should be monitored over time. For example, a patient with anti-RNA polymerase III antibodies may have to monitor their blood pressure more at home, whereas a patient with anti-topoisomerase I may need more frequent lung evaluations. 

ou have a chronic illness.  Depending on the severity of your Scleroderma, you may see  other subspecialists. To make the most out of each visit: 

• Come to appointments prepared 
• Ask questions – Don’t wait for the doctor to ask 
• Be very clear about what you want 
• Go to appointments with a written list of questions and concerns that require answers 
• Discuss problems 
• If you don’t understand something, don’t be afraid to speak up 
• If you can’t follow certain advice, tell the doctor and develop an alternative plan 
• Don’t be afraid to ask questions about why a treatment is recommended 
• If a treatment is too expensive, ask about alternatives 

• Exactly for what reason is the medication being prescribed?
• Do I really need the medication?
• What will it do?
• How and when do I take the medication and for how long?
• What foods, medications or activities should I avoid while taking the medication?
• What are the possible side effects?  What should I do if they occur?
• Are any tests required to monitor the medication?
• Can I use an alternative or generic medication that is less expensive?

By taking charge of your illness, you will feel more in charge of your life.  Having a sense of control and having positive things in your life can help you to cope better with your illness and have a better quality of life.  Your illness is only one part of your life. To really take care of yourself, you need to balance the more difficult parts of your life with more positive things such as fun activities and physical exercise.  Some patients find that mindfulness or meditation practices, participating in support groups, or counseling are helpful.  

Unfortunately the less active you are, the more your body becomes weak, stiff and out of shape. This can make pain and fatigue even worse.  Ask your doctor about what exercises or flexibility and strengthening programs might be good for you.  He or she can tell you what level of activity is right for you. Even if your doctor recommends against exercise, think about how you can spend time out of bed doing activities you enjoy.

When the disease is in the active phase, constitutional symptoms of fatigue and low energy are common. These are often coupled with inability to sleep normally. Sleep is disrupted by fear of the illness, depression, pain, skin itching and/or specific internal organ dysfunction (e.g. heartburn from gastrointestinal reflux or shortness of breath from heart or lung failure). The lack of sleep amplifies daytime symptoms; especially diffuse soft tissue pain and fatigue. Disordered sleep is treated by both non-drug and drug therapies. Other problems from the disease can cause fatigue as well, such as anemia from GI issues, muscle inflammation, lung problems. It is important to discuss your fatigue with your doctor so the causes and possible treatments can be identified.  

If your doctor has approved an exercise program for you, you should set aside time for it and exercise several times a week.  Start slowly.  For the first few times, you might limit your activity to warm-up exercises only.

Yes, there is. Start slowly with flexibility and strengthening warm-up to get ready for more intense exercise later on.

No, you should not.  You should try to be more physical in your everyday life. 

• Get out of the house every day.  Spending too much time in bed will make you feel more tired
• Take a stroll after dinner 
• If you can, use stairs rather than an elevator 
• Work in the garden or play with your children or grandchildren 

Several patient reports groups exist on various social media sites and through patient organizations such as the National Scleroderma Foundation and Scleroderma Research Foundation. Many patients find these groups and support helpful in addition to their family support and loved ones. However, please keep in mind that there is tremendous variability in patients’ experiences with scleroderma. The experiences of people you meet may not reflect your own now or in the future.  

The GI tract is a system of digestive organs that includes: 

• Esophagus 
• Stomach 
• Small bowel (intestine) 
• Large bowel (colon) 
• Rectum/anus 

These organs all contain smooth muscle, and its purpose is to move what we eat and drink through the system from the mouth to the rectum/anus.  We do not have voluntary control over these types of muscles, like we do for other muscles in our body.  The GI tract muscles work by reflex and should work to keep food from backing up. 

In some scleroderma patients, the smooth muscle loses its strength and becomes thin and less functional.  When this occurs, you may experience problems with your gut anywhere along the pathway.  

The normally working esophagus moves food and drink down through the esophagus through the lower esophageal sphincter into the stomach.  The lower esophageal sphincter is a muscle that stops the stomach contents from going backward up to the esophagus. 

In the stomach, food and drink mix with stomach acid which helps to break the food down into small pieces that can be absorbed.  The stomach contents are then passed into the small bowel near the common bile duct. 

The bile duct adds salts and enzymes that break the food down further. As the food continues to move downward, nutrients (good stuff) are absorbed and waste products that haven’t been digested pass through the small bowel to the large bowel.  The large bowel soaks up water.  The rectum holds the remaining contents until you have a bowel movement. 

GI disease can begin with any number of symptoms including heartburn, feeling full or bloated, nausea, losing weight, constipation or diarrhea.   

Scleroderma can affect the skin around the mouth making it tighter than usual. This can make chewing and opening the mouth fully more difficult. You may also experience trouble chewing food because of dry membranes in the mouth.  These dry membranes may be caused by Sjogren’s Syndrome (inflammation in the glands that produce saliva).   

Difficulty swallowing: 

Difficulty swallowing food is called dysphagia and occurs when food gets stuck somewhere in the passage between the mouth and the stomach. There isn’t enough muscle power in the esophagus to move the food to the stomach. Drinking a glass of water can help wash the food down.  Taking smaller bites of food and chewing food longer than usual can also help. 

Normally dysphagia is not painful, but it can be uncomfortable.  If it does hurt, it’s possible you have developed an ulcer in the esophagus or an infection.  Ulcers or irritation in the esophagus can occur from repeated exposure to stomach acid caused by reflux.  

Reflux and Heartburn: 

Reflux is a “backwards flow” of stomach contents moving back up into the esophagus and is often accompanied by acid.  Normally stomach acid stays in your stomach because it is blocked from going up by the lower esophageal sphincter. When a person has a reflux problem, the sphincter is too weak to hold the acid and stomach contents back (called GERD, gastroesophageal reflux disease). 

Heartburn is a burning sensation in the chest that is caused by stomach acid moving backwards into the esophagus. It can also cause a bad taste in your mouth, particularly in the morning. 

Stomach acid can also cause sore throat, atypical chest pain, coughing, asthma-like wheezing, hoarse voice or a feeling of gagging. 

It can be painful and uncomfortable.  The stomach’s lining is strong enough to stand the stomach acid but the esophagus is not.  Over time the esophagus can be irritated and damaged by acid reflux. Simple changes in daily living and eating habits can help to prevent some of the damage.  There are also medications that have been proven effective to reduce the acid exposure in the esophagus. 

• Don’t eat within two hours of lying down at night 
• Move the main meal towards midday 
• Eat more frequent small meals instead of one or two large meals 
• Eat sitting up fully 
• Eat slowly 
• Chew food thoroughly 
• Drink sips of water between bites and make sure each mouthful is fully swallowed before taking the next bite 
• Avoid carbonated drinks.  They will fill your stomach with air and push the stomach contents up 
• Don’t exercise after eating; but walking is helpful 
• Elevate the head of the bed at least four inches by placing it on wooden blocks.  Just propping your head up on pillows won’t work because it doesn’t lift your esophagus above your stomach. Wedge pillows which angle the body upward from the waist can be helpful  
• Limit actions that increase pressure on your stomach such as bending, vigorous exercise or tight clothing 
• Try to keep your body weight within a healthy range.  An overweight abdomen can put pressure on your stomach. 

Yes. However, not all foods worsen reflux for all patients. Some exploration and experimentation may be needed to determine which foods trigger worse symptoms.

• Alcohol
• Caffeine, coffee (regular and decaf)
• Chocolate
• Acidic foods ( citrus fruits like oranges, tomato sauce)
• Fried foods
• Raw vegetables
• Foods with high fat content ( fast foods, nuts, dairy products)
• Spicy foods
• Onions

Smoking will also aggravate reflux.

• Antacids (Tums, Maalox, Mylanta, Gaviscon) 
• H-2 Blockers (Pepcid, Tagamet) 
• Proton Pump Inhibitors (Prilosec, Protonix, Prevacid, Aciphex, Nexium, Dexilant, Zegerid) 
• Potassium-competitive acid blockers (Voquezna) 

• Getting full more easily 
  • This happens because the stomach does not empty as quickly as normal.  This may lead to weight loss due to inability to eat sufficient calories. 
• Nausea or Indigestion(burping) 
  • These symptoms could be due to the stomach not emptying quickly.  In extreme cases this can lead to regurgitation or vomiting of food.  
• Erosions or Ulcer Formation 
  • These can result from the buildup of acids or from the use of certain medications.  They cause pain, indigestion or bleeding.  There are medications to treat erosions and ulcers but this may require an endoscopy to diagnose. 
• Gastric antral vascular ectasia (GAVE or watermelon stomach) 
  • Telangiectasias are dilated blood vessels due to scleroderma and sometimes they can bleed. They can occur on the wall on the inside of the stomach. It is called a watermelon stomach or GAVE because the vessels make the stomach wall look like the stripes on the surface of a watermelon. It can be treated with procedures that cauterize the vessels during an endoscopy.   

• Gas, Cramps and Bloating 
  • The first symptom of bowel problems may be bloating after eating.  People also complain of feeling gassy or cramping.  Cramping is caused by the unusually hard muscle contractions as the muscles try to move something through. Sometimes this is caused by an overgrowth of the bacteria in the gut and this is treated with antibiotic therapy.   
• Constipation 
  • Constipation in scleroderma is caused by things moving slowly through the lower bowels. Some people find a high fiber diet can help to reduce constipation.  Others find a high fiber diet worsens their gassiness.  Exercise helps promote motion in the bowels, and it is very important to stay hydrated.  A stool softener can help, but stimulating laxatives should be avoided. Miralax is an over-the-counter product that works by adding more water to the stools and this is safer to use.  
• Diarrhea 
  • Diarrhea may occur when the normal bacteria in bowels overgrow. Diarrhea can also be caused by medications.  Antibiotics and medications are available to treat diarrhea. 
• Obstruction 
  • A bowel obstruction is a serious GI problem that can occur.  It can occur if something is blocking the passage of waste or when the bowels “freeze” and stop moving.  When this happens, the abdomen can be bloated can cause a lot of pain. This situation can be an emergency and needs immediate medical attention.  
• Bowel Incontinence 
  • When this happens, the person has difficulty holding their bowels and may have accidents. This occurs because the rectum has two sphincters (muscles that block the stool) that weaken and thus stool can slip out. Treatment may include management of the other motility issues in the gut or pelvic floor physical therapy.  

Dry membranes of the mouth and eye are very common complaints. Dysfunction of tear or salivary glands secondary to an autoimmune process or tissue fibrosis is thought to be the cause, although many other things can cause this as well (like some medications). Tooth decay and periodontal disease need to be prevented. These problems are due to a decrease in normal saliva and difficulties performing dental care, particularly in patients with a decreased oral aperture (mouth opening). Identifying this problem is very important and instructing the patient to have frequent dental care is essential.

Lung tissue and the blood vessels in the lung can be affected by the scleroderma disease process. Therefore, the lung and heart need to be carefully evaluated in every patient both at the onset of the disease and periodically thereafter. Scleroderma causes a restrictive ventilatory defect (small lungs) secondary to an inflammatory process in the lung alveoli (air sacs). This process, left untreated, leads to fibrosis of the lung tissue (scar tissue) that interferes with normal gas exchange from the air to the blood and with the movement of the lung (breathing in and out).  

The right heart pumps blood into the lung to get oxygen to the body. Usually, this is a very low-pressure system because the lung and its blood vessel are a system with very low resistance. If the blood vessels or lung tissue becomes diseased, then the pressure in the pulmonary circulation and the right heart rises (pulmonary hypertension). Pulmonary hypertension generally occurs later in scleroderma and occurs in about 15% or patients.  

It is challenging to detect lung involvement in scleroderma because active disease may be present in the absence of symptoms. Ideally, we would detect a problem before a patient has symptoms of shortness of breath. The physical examination and traditional chest x-ray are poor methods to detect early lung disease (they may miss problems). Pulmonary function testing is very sensitive and reliable but defines function of the lung and not the activity of the disease. Serial pulmonary function testing can define activity, but several months must pass before changes occur. We also visually examine the lungs by doing a high-resolution computed tomography (CT) scan. This can detect early lung fibrosis and inflammatory changes. A 2D-echocardiography (ultrasound test of the heart) study is a simple, non-invasive method of assessing heart function and estimating right ventricular systolic pressure (an indirect method of estimating pulmonary artery pressure).  

All patients with any type of scleroderma should have a pulmonary function test and a 2D echocardiogram at least once per year.  

Kidney disease is one of the least common problems in scleroderma, but it can damage the kidneys quickly when it happens. It is important to talk to your doctor about your risk for kidney problems. Patients with early scleroderma with diffuse type of skin disease are at greater risk for kidney disease (known as scleroderma renal crisis). Scleroderma kidney disease usually presents as new elevation in blood pressure that may be asymptomatic. Patients with scleroderma should monitor their blood pressure regularly. Particularly in patients with early diffuse scleroderma, this should be done frequently (daily) using a well-calibrated home blood pressure device. Evidence suggests that renal blood vessels suddenly constrict (Raynaud’s phenomenon of the kidney), dropping blood flow to the body or cortex of the kidney. Left untreated, the low blood flow may lead to tissue damage and kidney failure.  Patients who are high risk with new onset of hypertension should be assessed urgently (within 24 hours)  with rapid assessment of renal function (urinalysis and blood testing) and control of any blood pressure elevation with an angiotensin converting enzyme (ACE) inhibitor. Patients with evidence of renal crisis often need to be hospitalized to order to monitor blood pressure and renal function closely and to titrate medications. 

All patients with scleroderma will typically get a 2D echocardiogram of the heart every year. This is typically to try and estimate the pressures between the heart and the lung (ie to look for pulmonary hypertension), but can also reveal other potential problemsin the heart such as reduced heart pump function. Other testing may be ordered if patients are having symptoms such as chest pain, shortness of breath, swelling in the feet or heart palpitations. This testing may include bloot tests, ECGs (electrial testing of the heart) or a heart MRI study to look for heart inflammation.  

This is a result of Raynaud’s Phenomenon.  Raynaud’s phenomenon is oftentimes the earliest symptom of scleroderma. Raynaud’s phenomenon in scleroderma is caused by both spasm of small blood vessels and from thickening and narrowing of blood vessels.  

The color change is an exaggeration of what normally happens when hands are exposed to the cold.  However, the normal cold response is a blotchy red and white pattern while the color changes of a Raynaud’s attack— white, then blue or purple are very different.  At the end of the attack, the fingers turn red which is the result of blood flowing back to the fingers in a rush. This may result in numbness or pain and can be associated with a ‘pins and needles’ sensation. 

Yes, many people have Raynaud’s attacks in their toes.

Yes they can.

• Keep your entire body warm, not just your hands and feet 
• Bring extra layers of clothing when you’re going somewhere that might be cold 
• Wear hats in cold weather 
• Use a space heater in the bathroom or at your office at work if it gets too cold 
• Warm up your bed with an electric blanket or hot water bottle; Use flannel sheets 
• Warm up your car before you leave the house; Use a remote car starter; Cover your steering wheel with a lambskin wheel cover 
• Wear mittens (not gloves) and warm socks when the temperature drops below 65 degrees.  Use chemical or battery operated hand and foot warmers 
• Take extra precautions to protect your fingers and toes from the cold 
• Keep gloves everywhere 
  • In kitchen for reaching into the refrigerator or freezer 
  • In office desk for when air conditioning is high 
  • In purse or pockets to be used in frozen food areas in grocery store 
• Try to avoid sudden changes in temperature 
• Wear comfortable shoes and socks that allow for good circulation 
• Avoid stressful situations which can also trigger a Raynaud’s attack 
• Pay attention to things that may trigger a Raynaud’s attack, such as: 
  • Air conditioning 
  • Holding a cold drink 
  • Washing something in cold water 

The most common medications are called vasodilators.  Calcium channel blockers are the most effective.  They stop the spasm of arteries in Raynaud’s phenomenon.  Other vasodilators include Viagra/Revatio (sildenafil) or other types of blood pressure medications.  

Other medications can sometimes be helpful that are not vasodilators, such as statin medications (typically prescribed to lower cholesterol) and SSRIs (typically prescribed for depression or anxiety). A low dose aspirin could also be prescribed, particularly for those patients with digital ulcers. 

For more severe Raynaud’s phenomenon with digital ulcers combination or intravenous medications may be given.  

Other interventions such as botox injections or surgeries are less proven strategies and may be considered in some cases.  

Biofeedback has been shown to be helpful.  Biofeedback uses a machine to give you information on the temperature of your hands.  A biofeedback therapist can teach you skills for increasing the blood flow and warming the temperature of your hands.

Your body has produced extra collagen in your skin.  Collagen is what makes your skin firm and also what your body makes to help heal wounds. In scleroderma, the cells start making collagen as if there were an injury that needs to be fixed.  The excess collagen gets into the dermis layer of the skin and causes it to thicken and feel tight and hard. 

The degree of skin involvement varies from patient to patient a great deal. Most have “limited scleroderma” with only the fingers and mild face involvement. Others have “diffuse scleroderma” with many areas of the body affected. Skin thickening usually begins on the fingers.  In the diffuse form it can, over time, be on the back of the hands, the forearms, the upper arms, the face, the neck, the trunk, the legs or the feet.  Similar to thickening of the skin, fibrosis or excess collagen can also occur in the internal organs in some patients. 

At each visit, the physician should assess the level of skin involvement by performing a skin score. This is done by palpation of the skin to determine the degree of thickening. Seventeen areas (fingers, hands, forearms, upper arms, face, chest, abdomen, upper leg, lower leg and feet) are scored from normal (zero) to severe (3) thickness. The maximum score is 51. The skin score provides a semi-quantitative method to characterize the degree of skin involvement and classify the patient as having limited or diffuse scleroderma. Palpation of the skin can also stage the disease and give some sense of disease activity. Active disease is associated with inflammatory signs (e.g. swollen skin) while inactive skin disease is manifest by thickened sclerotic skin typical of mature scar tissue. Usually, physical features can determine the subgroup of scleroderma at first visit. However, if the patient has new onset disease then serial observations at short intervals (approximately once per month) are necessary to fully appreciate the disease type. The degree of skin disease and the level of disease activity provide clues about the risk for internal organ involvement. The higher the skin score, the higher the risk for internal organ involvement. Active skin disease may correlate with active disease in other organs (like the lung or kidney).  

In general, without treatment, the skin thickening will reach its peak within 1-2 years after it starts and will then begin to loosen.  How much it will loosen will vary from person to person. However, in patients with early, diffuse scleroderma, we think that medications can modify this normal course so things can improve more quickly.  

The thickened skin can look shiny and scaly from being dry. Some areas may get darker (hyperpigmentation) while other areas may develop a patchy loss of skin color (hypopigmentation). Large patches of pigment loss can also happen. y. The pigment changes usually get better over time and the skin tone may return to its normal appearance after several years. You may also develop red spots caused by the widening of small blood vessels in the surface of the skin (called ‘telangiectasias’). These changes are not painful but may be a cosmetic concern.  

Generally it is best not to try to change the color with drugs or creams but cosmetics that cover better are helpful. Avoidance of sun will prevent increased pigment and decreasing scratching of the skin will reduce damage and prevent further changes in pigment. Creams and lotions that moisten the skin can prevent further skin damage. Prescription creams with vitamin A are sometimes given. 

The most common visible change is tight skin on the fingers causing bending of the fingertips toward the palm, termed ‘flexion contractures’. Tight skin on the face may reduce the size of a person’s mouth opening, create a few small vertical lines around the lips and in some cases interfere with drawing the lips over the teeth. You may experience hair loss on the arms and legs, sweat less or develop skin sores where the skin is injured by trauma. Some patients will develop small calcium deposits in the tissues of the fingers, in the bursa (sac) over the tip of the elbows, rarely over the kneecaps or in other pressure areas. It is important to note that these complications do not happen to all patients with scleroderma.

Typically this is a result of either active scleroderma skin disease, or more commonly, dry skin. Dry skin is caused by several things. First, the extra layer of collagen in the inner layer of skin destroys normal sweat and oil glands.  Second, the outer layer of the skin usually has fatty substances that moisten the skin. In scleroderma, this outer layer often gets thinner so there is less oil. Finally, in the early phase of scleroderma inflammation in the skin may cause the nerves in the skin to create an itch signal.   

Without treatment, itching from inflammation normally occurs in the first 6 months to 2 years of the disease and then gets better as the inflammation disappears.  However, when the skin is thickened and damaged it may have the problem of itching for many years. There are medications that can help itching in addition to the other medications being used to treat your scleroderma.  

Avoid:

• Scratchy fabrics such as wool
• Rubbing alcohol or other products containing alcohol (perfume)
• Harsh soaps, detergents and household cleaners (avoid Ivory, Dial, Zest, Safeguard, Jergens, Lava, Camay, Coast or Irish Spring soap) 
• All fabric softeners
• Hot baths
• Excess cold exposure
• Active or passive cigarette smoke
• Topical numbing agents or antihistamines since they tend to be drying. 
• Electric blankets except to warm the bed. Their constant heat will cause you to lose body moisture. 

Use:

• Moisturizers with higher emollient content (in jars rather than pump bottles) 
• Fragrance free products 
• Soaps that have added moisturizers (Dove, Aveeno, Neutraena) 
• Warm water 
• Take soothing baths. Add ½ cup baby oil to your bath. Bathe with Aveeno Oatmeal, Oilated Aveeno, Cornstarch, Mineral Oil or Cottonseed Oil 
• Moisturize often. 
• Use cooling agents such as menthol or camphor (Sarna Lotions). 
• Use gloves when using any substances that are rough on the hands. 

These sores are called digital ulcers and are caused due to severe Raynaud’s and when excess collagen collects on the inside of blood vessels causing them to narrow and restrict blood flow.  They occur most often on the fingertips but can develop anywhere on the finger or toe when the skin in stretched too tight.  Sores can also appear at pressure points, like elbows. Sores may be very painful and can make it difficult to use your hands or other parts of your body. 

• Avoid stress and cold
• Keep as warm as possible
• Wear layers of clothing
• Wear hats in cold weather
• Wear shoes and socks that provide room for circulation
• Use Band-Aids to protect from damage when using your hands

• See your doctor about specific recommendations for your situation 
• Keep ulcers clean
• Use medication if infection develops

Musculoskeletal complaints are common in the rheumatic diseases and scleroderma is no exception. Arthritis can be detected by physical examination but muscle disease may be asymptomatic until weakness occurs. Muscle weakness, secondary to an autoimmune mediated inflammatory myositis or a non-inflammatory fibrotic myopathy, can be detected by measuring muscle enzymes (CPK, aldolase and transaminases). If the enzymes are abnormal, then further specialized testing (EMG or muscle biopsy) may be necessary. It is equally important to carefully determine if other causes of muscle weakness exist. These include fibrosis of the muscle secondary to the scleroderma process, weakness from disuse or wasting from deconditioning and malnutrition. Patients also can have a non-scleroderma cause of muscle weakness including metabolic disease (e.g. hypothyroidism), side effects from medications (e.g. lipid lowering agents and corticosteroids can induce myopathy) or another disease process (e.g. tumor associated myopathy). When weakness is present, all of these causes must be considered and investigated. Once a cause is determined, effective therapy can be instituted. 

Fatigue is a frequent problem and can seriously alter your sex life.  Like any activity that is important to you, you may need to pace yourself and rest up to continue your sex life. 

The vagina can become dry with less lubrication during arousal. This can make sex uncomfortable or even painful.  Vaginal lubricants can be bought in most drugstores and are very helpful.  It’s important to look at other causes of vaginal dryness as well.  Menopause and the drop in female hormones that goes with it can cause vaginal dryness.  If this is the case, an estrogen replacement in pill form or in a vaginal cream may be helpful. 

Some women find sex painful because they can’t find a comfortable position.  Symptoms of reflux (such as heartburn) can be made worse when laying down or when there is pressure on the abdomen (belly).  Joints may feel stiff and not move as easily.  A warm bath may help.  Being open to new positions or activities are also important. 

The penis becomes erect when more blood is directed to it than drains from it.  The part of the nervous system that is responsible for this is called the parasympathetic nervous system.  There is evidence to show this part of the nervous system is affected in scleroderma. 

Vascular problems may also be responsible.  The blood supply to the penis can also be changed. 

Once damage to nerves or vessels happens, it may not be possible to reverse it. 

Problems with erections can also be caused by medication side effects.  Calcium channel blockers are often used to treat Raynaud’s phenomenon.  These medications improve the circulation to the fingers but decrease circulation to the penis.  Discuss this issue with your doctor before you stop the medication. 

Medications such as Viagra/Cialis can improve the circulation and improve erectile dysfunction.  Other treatments such as penile implants may also be helpful.  You may want to discuss the alternatives with your doctor or be referred to a urologist. 

• Try to set up a calm and relaxed atmosphere 
• Find positions that are comfortable for both of you 
• Avoid sexual activity when you are really tired 
• Avoid sexual activity after a big meal 
• Avoid drinking alcohol before sex 
• Check with your doctor to see if you’re taking medications which may be interfering 
• Rest up and plan for sexual activity 
• Stay as active as possible 
• If you are having problems with arousal, or loss of interest in sex, it may be due to depression.  If treatment for depression doesn’t help, you may want to consult with a professional sex therapist.