Systemic sclerosis (scleroderma) (2022)

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What is systemic sclerosis (scleroderma)?

Systemic sclerosis is a rare condition that can cause people’s skin and the connective tissue inside the body to harden.

You can sometimes see signs of systemic sclerosis on the face, hands, feet and elsewhere around the body, as skin hardens and thickens.

Systemic sclerosis can also cause connective tissue inside the body to become thick and hard.

Connective tissue includes tendons, cartilage and blood vessels. Together they form a structure that supports joints and internal organs, and holds them in place.

When skin and connective tissue hardens and thickens it becomes like scar tissue from an injury or a burn. When systemic sclerosis causes connective tissue to harden this can stop muscles, blood vessels, joints and internal organs such as the lungs, working as well as they should.

Most people with systemic sclerosis will only have mild symptoms. Rarely, it can cause serious problems with internal organs. These complications can be treated.

It’s important that potentially serious complications are spotted early. You should therefore have regular check-ups with your doctor, preferably once a year.

Systemic sclerosis is an autoimmune condition. This means that the immune system, which protects you from illness and infection, gets confused and attacks the body’s own cells.

The word systemic means a condition that can affect the whole body. Sclerosis means the hardening of a structure.

Systemic sclerosis affects more women than men. It usually starts between the ages of 25 and 55, although it can occasionally start in children or in older people.

There are different types of systemic sclerosis – diffuse and limited.

Diffuse systemic sclerosis can affect skin all over the body and is more likely to affect the internal organs.

Limited systemic sclerosis is a milder form that only affects the skin on the face, lower arms, hands, lower legs and feet. It normally progresses slowly. Limited systemic sclerosis can affect the lungs and the digestive system too.

Systemic sclerosis is not to be confused with a separate condition called localised scleroderma, which just affects the skin. The word scleroderma, which is an older term, specifically means hard skin.

The following information is about the condition systemic sclerosis, in which skin and connective tissue inside the body are affected.

Symptoms

Systemic sclerosis can cause symptoms affecting different parts of the body.

Systemic sclerosis (scleroderma) (1)

Sensitivity to the cold (Raynaud’s phenomenon)

Most people with systemic sclerosis develop an unusual sensitivity to the cold, known as Raynaud’s phenomenon.

Fingers or toes turn white then blue in the cold. Just walking into a cold room or reaching into a fridge or freezer can make this happen. The colour returns to normal as the hands or feet warm up.

Raynaud’s phenomenon is caused by a narrowing of the blood vessels, which reduces the blood supply to the fingers or toes. Stressful situations can also cause the blood supply to reduce in the hands and bring on an episode of Raynaud’s.

Many people have Raynaud’s without ever having systemic sclerosis. However, most people with systemic sclerosis will have symptoms of Raynaud’s at some time during their illness.

Raynaud’s is often the first sign that someone has systemic sclerosis. It can sometimes appear years before the onset of systemic sclerosis.

Changes in the skin

The most common changes in the skin due to systemic sclerosis are:

  • thickening and hardening of the skin on the hands, arms and face
  • swelling of the hands and feet, especially in the morning
  • shiny skin, without its usual creases
  • stiffening of the facial skin, making it difficult to open the mouth wide
  • small red blood spots on the face, hands and arms.

Less frequently, or later on in the condition, there may be:

  • thinning of the lips
  • thinning of the skin on top of the fingers and the soles of the feet
  • peeling, cracking or open sores (ulcers) in the skin and flesh over the fingertips, caused by poor blood supply
  • white chalky lumps under the skin, caused by deposits of the mineral calcium, often on the fingertips.

Digestive problems

Systemic sclerosis can affect the digestive system, which is responsible for swallowing food, breaking it down, and sending it to different parts of the body.

We have a tube that takes food from the mouth to the stomach, called the oesophagus (o-soff-a-gus). Systemic sclerosis can cause the muscles in this tube to weaken, which can make swallowing difficult.

Systemic sclerosis can cause heartburn, as a result of too much gastric acid in this food tube. This acid is produced naturally in the stomach to help break food down.

However, systemic sclerosis can cause this acid to leak upwards from the stomach into the food tube. This can be unpleasant, and it isn’t good for us.

Most people with systemic sclerosis have some problems with swallowing or heartburn. Less often, other parts of the digestive system may be affected, leading to bloating, diarrhoea or constipation.

Pain or stiffness in the joints or muscles

Systemic sclerosis can cause the tissues around joints to stiffen, which can reduce the range of movement of joints.

It can also cause pain and swelling around affected joints.

Muscle weakness is also sometimes a symptom of systemic sclerosis.

People who have had systemic sclerosis for a long time may find that their joints can tighten into a bent position, because of the tightening of the skin and tissues surrounding the joints.

(Video) Systemic Sclerosis and Scleroderma: Visual Explanation for Students

How is systemic sclerosis likely to affect me?

Systemic sclerosis affects everyone differently, so it’s hard to say how it might affect you.

This is a long-term condition, however the serious problems it can cause are rare.

About one in five people with systemic sclerosis will also have symptoms of another condition, such as lupus, Sjogren’s syndrome or inflammatory arthritis. These conditions will also need treatment.

Most people find that systemic sclerosis affects just a few parts of the body and comes on gradually. It may slowly get worse but usually settles down to become stable after a few years.

Sometimes systemic sclerosis may progress more quickly.

For some people the symptoms can almost disappear after several years.

The skin symptoms can be the worst part of systemic sclerosis for some people. While for other people, the digestive problems will give them the most problems.

Unfortunately, systemic sclerosis can sometimes cause people serious problems. These can include:

  • scarring of the lungs, causing shortness of breath and a dry cough
  • narrowing of the blood vessels of the lungs, leading to high blood pressure in the lungs, which may in turn strain the heart
  • kidney problems resulting in high blood pressure.

These problems are rare. However, it’s important you have regular check-ups with your doctor – preferably once a year – if you have systemic sclerosis.

The body is able to break down or repair extra scar tissue. This means there may be some improvement in scarring – both in the skin and in other organs – once it becomes less active.

Diagnosis

There’s no single test for systemic sclerosis, and often it’s the thickening of the skin that will be a key reason why a doctor has made a diagnosis.

However, your doctor will carry out tests that can be helpful in finding out whether other parts of the body are affected.

What tests are there?

Tests could include:

  • blood tests
  • x-rays and computerised tomography (CT) scans
  • breathing tests
  • a heart scan, for example an echocardiogram
  • stomach tests, (for example an endoscopy)
  • a skin biopsy, where a small piece of skin is removed and examined under a microscope
  • a capillaroscopy, which looks at the small blood vessels (capillaries) around the finger nails that are often affected by systemic sclerosis.

Infrared thermography uses technology to take images of what’s happening inside the body by showing heat spots. This can be used to help make a diagnosis of systemic sclerosis. These tests are often only performed at specialist centres.

(Video) Scleroderma (Systemic Sclerosis)

Treatment

There’s currently no cure for systemic sclerosis. However, there are drugs that can help to control symptoms and treat any complications.

There are many ways systemic sclerosis can affect the body. Therefore, the treatment you’ll need will depend on how the condition affects you.

There’s also a lot you can do to help manage your condition, including regular exercise, a healthy diet and a good skin care routine.

Treating Raynaud’s phenomenon

Drugs can help with the symptoms of Raynaud’s phenomenon by improving circulation to the hands and feet.

There are quite a few drugs available that can have this effect, including nifedipine and sildenafil, which can be taken as tablets. You may need to take more than one drug at the same time.

In more severe cases of Raynaud’s, particularly where there are painful ulcers that won’t heal, drugs such as iloprost can be given as an infusion. An infusion is a way of giving a drug as a drip into a vein, usually in a hospital.

Drugs to treat Raynaud’s phenomenon work by relaxing the blood vessels and increasing blood flow to the fingers and toes. They will also have the same effect on blood vessels elsewhere in the body. Therefore, these drugs can cause side-effects such as skin flushing, headaches and swelling of the ankles.

Treating heartburn and swallowing difficulties

To prevent heartburn and swallowing difficulties, your doctor may recommend antacids, a type of medication that can neutralise the acid produced in the stomach.

These are usually very effective, although you may need to take them for months or years.

You may also be prescribed a drug called a proton pump inhibitor (PPI), which can reduce the amount of acid produced by the stomach.

Treating other problems related to the stomach and digestive system

If you have spells of constipation and diarrhoea, abdominal swelling, increased wind and discomfort, you may need to see a gastroenterologist who specialises in this area.

Some people also experience anal incontinence, meaning that they can’t control their bowel motions properly so that small amounts of stool slip out and can soil their clothes.

This can be uncomfortable or embarrassing, and people may not realise that it’s anything to do with systemic sclerosis. If this happens to you, talk to your doctor and ask for a referral to a gastroenterologist.

Sometimes additional bacteria develop in the bowel, and this can be treated by drugs such as antibiotics.

It’s important to have symptoms like diarrhoea and incontinence looked at, to make sure that they don’t have another cause.

Treating painful and swollen joints

Painkillers and non-steroidal anti-inflammatory drugs (NSAIDs) should help to relieve joint pain and swelling.

You can buy milder forms, such as paracetamol and ibuprofen, over the counter at pharmacies and supermarkets. Your doctor will be able to prescribe stronger painkillers and NSAIDs if needed.

Treating high blood pressure and kidney complications

People with systemic sclerosis can sometimes have high blood pressure, also known as hypertension. Severe cases can lead to kidney damage and strain on the heart.

This is a serious complication known as a systemic sclerosis renal crisis. It can be treated or prevented with drugs that help to control the blood pressure, especially ACE inhibitors.

Treating lung and heart complications

Inflammation in the lungs can be treated with steroids or disease-modifying anti-rheumatic drugs (DMARDs).

High blood pressure in the lungs (pulmonary hypertension) is a rare complication of systemic sclerosis, but you’ll have regular tests to check how well your lungs and heart are working.

If necessary, pulmonary hypertension can be treated with specific drugs (including bosentan, ambrisentan, sildenafil or prostacyclin) that improve symptoms such as breathlessness.

Other drugs that may be used for lung and heart complications include:

  • steroid tablets
  • drugs to suppress an overactive immune system
  • drugs used to treat blood pressure and cholesterol.

Steroid tablets to treat systemic sclerosis

Steroid tablets can be used either in the early stages of systemic sclerosis when the skin is starting to look puffy, or later on if the muscles or lungs are affected.

They’re usually given in low doses because high doses can raise blood pressure and increase the risk of kidney problems.

Steroid tablets are man-made forms of a hormone (cortisone) that occurs naturally in the body. They can be used to reduce swelling.

(Video) Systemic Sclerosis (Scleroderma) - Medical-Surgical (2020 Update) - Immune - @Level Up RN

Immunosuppressive drugs to treat systemic sclerosis

Immunosuppressive drugs target the immune system and may be used in more severe cases of systemic sclerosis, especially where the skin or lung disease is more extensive.

These could include drugs which are known as conventional disease-modifying anti-rheumatic drugs (DMARDs).

These drugs which have the effect of reducing the effectiveness of an overactive and misfiring immune system, include methotrexate, ciclosporin, cyclophosphamide, mycophenolate and azathioprine. It will depend on how systemic sclerosis affects you as to whether you will be prescribed any of these drugs.

The biological therapy rituximab is sometimes used to treat severe cases of systemic sclerosis. Biological therapies are newer drugs that have a more targeted approach. They reduce the effectiveness of key cells in the immune system that may be causing problems.

Living with systemic sclerosis

For some of the symptoms of systemic sclerosis you will need to take drugs. However, a healthy and balanced diet, exercising regularly and a good skin care routine are also important.

Exercise

A regular exercise programme will keep your skin flexible, reduce any tightening in your joints and keep your blood moving freely.

Although you may need to rest if your joints become swollen, it’s generally helpful to keep moving as much as possible.

Regular stretching and range of movement exercises, should help to reduce tightness around the joints, particularly in the hands and wrist.

A physiotherapist or a specialist hand therapist would be able to teach you the best exercises to keep your joints mobile. They may also suggest using splints to wear to help you look after your hands and wrists.

A physiotherapist might be able to give you special exercises to help maintain mobility in the muscles, connective tissue and skin in your face.

Diet and nutrition

Maintaining a healthy diet is important as it may help Raynaud’s symptoms and the healing of skin ulcers.

However, digestive problems can make it difficult to keep up a balanced diet and to keep to your normal weight.

If you have heartburn or difficulty in swallowing, the following tips may help:

  • Eat six small meals a day instead of three larger ones. This helps with digestion, while making sure that your body gets the nutrients it needs.
  • Eat slowly, chew thoroughly and drink plenty of water with meals.
  • Eating your largest meal in the middle of the day can help to avoid heartburn.
  • If you suffer with heartburn, see your doctor, who may prescribe a proton pump inhibitor (PPI) that will help to reduce irritation of the gullet.
  • Don’t eat too much in the evening to make sure you have time for digestion before you go to bed.
  • Raise the head of your bed a few inches to stop acid coming back up from your stomach while you sleep.

Complementary medicine

Massaging the hands using warm paraffin wax can help to keep the skin flexible and reduce discomfort in your joints. However, you must not use a wax bath if you have any open finger ulcers.

While there is a lack of scientific evidence to say that complementary medicine can help ease the symptoms of systemic sclerosis, most are thought to be safe to use. It’s always important to go to a reputable and registered supplier. It’s also very important to check with a GP first if you think you’d like to try a complementary medicine.

In many cases the risks associated with complementary and alternative therapies are more to do with the therapist than the therapy itself. This is why it’s important to go to a reliable and legally registered therapist, or one who has a set ethical code and is fully insured.

The use of high-dose vitamins E and C, fish oil and ginger or gingko dietary supplements can also help. If the vitamins don’t help within a three-month period, you should stop taking them.

Always talk to your doctor before you try a complementary medicine.

Supports, aids and gadgets

If you have trouble with daily activities like dressing or tasks that need you to have good grip strength, there are a number of gadgets available that can help. If in doubt, ask an occupational therapist for advice on how to protect your joints from unnecessary strain.

If you struggle to open child-proof medicine containers, ask your pharmacist to put your drugs in containers you can manage.

Some people find it difficult handling coins when their fingers are sore or swollen. A coin purse that opens out to form a tray for the coins may help with this.

If you think that an aid or gadget might help you with a task you’re struggling with, the charity Remap might be able to help you. This organisation makes bespoke equipment free of charge for people with a medical condition to help them with essential daily tasks.

If you live in England, and have systemic sclerosis, you may also be entitled to free support from your local authority to help with everyday tasks and getting around your home.

You could get help with the costs of aids and minor adaptations if you meet eligibility criteria. The criteria includes that you have a physical or mental illness or disability, and are unable to do basic tasks or activities, such as:

  • cooking
  • eating
  • washing
  • going to the toilet
  • getting dressed
  • cleaning
  • working
  • developing and maintaining family and social relationships

If you’re unable to do two or more of the above tasks or activities, and this has a significant impact on your well-being, you should qualify for aids and minor adaptations.

If you are eligible, you should be able to get aids, and minor adaptations up to the value of £1,000 per item, free of charge. If you need an adaptation which will cost over £1,000 you can apply for a disabled facilities grant. All these aids and adaptations can help with everyday tasks around your home and make it easier to get around.

For more information, visit this website for a needs assessment: www.gov.uk/apply-needs-assessment-social-services

Skin care and keeping warm

You need a good supply of blood flowing to your skin to stop it from cracking, peeling and developing ulcers.

Here are some things you can do to help:

  • Keep warm from top to toe – this will help open the blood vessels to your arms, hands, legs and feet. Wear a hat to help preserve your body heat. Thermal clothes, hand warmers and electrically heated gloves and socks can also help. Remember that layers of clothing will trap heat and keep you warmer than thick clothes.
  • If your skin is broken or painful, dressings can help to protect it.
  • Don’t use strong detergents or anything else that irritates your skin, and avoid soaps that contain the natural wax lanolin.
  • Try soaps, creams and bath oils designed to prevent dry skin until you find the ones that give you the best results in keeping your skin supple.
  • If your hands are prone to dry skin, put cream on them whenever they’ve been in water. You can use either a water-based cream (such as E45 or aqueous cream), which is short-acting, or an oil-based cream (such as emulsifying ointment), which is thicker and longer-lasting.
  • Smoking reduces the blood flow to your skin and is very likely to make Raynaud’s symptoms worse, so it’s best not to smoke. If you need help to stop smoking, talk to a GP or pharmacist, who will be able to offer support and advice. They may also be able to refer you to a local Stop Smoking service.

Managing red blood spots (telangiectasia)

Telangiectasia are small red spots on the skin that often develop on the face, hands, and sometimes the chest and arms. They don’t cause any harm.

(Video) What is Scleroderma? (also known as Systemic Sclerosis)

They are caused by blood vessels becoming wider than normal.

You can use make-up to help cover them up if you want to, which shouldn’t make your skin symptoms worse.

Changing Faces, a charity for people with conditions, marks or scars that affect their appearance, offer a skin camouflage service, giving advice on how to cover the marks with camouflage cream.

Clinics are available throughout the UK and you can find information on the nearest one to you by visiting their website, www.changingfaces.org.uk.

The service is free (though donations are welcome) and is open to anyone. Changing Faces accept self-referrals in some areas, but in others you’ll need to get a healthcare practitioner to refer you online.

Laser treatment can also help in some cases. It’s available in the specialist dermatology units of some hospitals, and you may need several sessions to keep the telangiectasia under control.

Dealing with stress

There may be emotional difficulties connected with having a long-term condition, and the changes in the appearance of your skin can be upsetting. In addition, stress can reduce the blood flow to some parts of your body, so it can affect your condition, particularly if you have Raynaud’s phenomenon.

Talk about any feelings of stress or depression with your family, friends or a healthcare professional. If you need help in handling stress or depression, your doctor may be able to help or can refer you for specialist counselling.

You can also speak to a nurse in the rheumatology clinic. Many clinics have nurses who either specialise in systemic sclerosis or have a special interest in the condition.

Another option could be to get in touch with Scleroderma Raynaud’s UK (SRUK), a charity that provides services to people with systemic sclerosis and Raynaud’s phenomenon. This is a members’ organisation and it provides a range of support services. Talking to people with the same condition can greatly help.

Related information

Research and new developments

Versus Arthritis is currently funding several studies to improve our understanding of the causes of systemic sclerosis.

This includes a long-term study to find out whether a new light-based imaging technique can be used to measure different features of the condition.

This technique has potential to tell us more about what causes blood vessel narrowing and tissue scarring (fibrosis). It may also lead to research into new therapies for systemic sclerosis.

We’re also supporting research to better understand what causes excessive scarring of the skin and internal organs in people with systemic sclerosis. Scar tissue is made by cells called myofibroblasts, and a protein called c-Rel is thought to regulate its production. This research aims to understand how c-Rel regulates scar production and to discover whether altering its function or removing it will prevent scar formation.

We’re also funding research to identify new treatments for systemic sclerosis, including a study which aims to investigate whether treatment with the steroid prednisolone is beneficial, in terms of safety and effectiveness, in patients with the condition.

Barbara’s story

Systemic sclerosis (scleroderma) (2)

My symptoms started in July 2013. My fingers were swelling up like crazy, and my ankles were swelling up so much I couldn’t see them. No-one could tell me what was going on. I was referred to a rheumatologist.

Nothing was showing up in my bloods to indicate anything in particular. But over the following few months, more and more started to go wrong.

I was having trouble walking and I was out of breath all the time. The walk from my car to work was too much, so my husband would drop me off right at the door. After Christmas, I had another blood test and something showed up.

The rheumatologist said: ‘I think we have to put you on some immunosuppressive drugs.’

We went on a skiing holiday in northern Italy, and because it was at high altitude that set things off. I became ill and was struggling to breathe. I had to be helicoptered to a hospital in a town called Bolzano.

I was in this hospital in Italy for two-and-a-half weeks. It was there a doctor said: ‘Has anyone ever told you that you have scleroderma?’ I’d never heard of it before. It was quite a shock. They got me stabilised, so I could come back to England.

I came back to the local hospital and was transferred to a renal unit in Manchester. I had a serious problem with my kidneys.

They did a biopsy on my kidneys and took me off my medication. I was there until they confirmed that fortunately I didn’t need a kidney dialysis. When they saw signs of improvement, I was allowed home.

I have good days and bad days. On a bad day, I’m very tired and don’t want to do anything, and there can be pain. One of the biggest ways it has affected me is my skin. It has made my skin extremely tight, and it gets shiny, itchy and painful. My skin is almost normal again now.

My treatment was quite aggressive early on, but it seemed to have the desired effect. I had to give up work though. I was too ill to continue at the time.

(Video) Rheumatology Pearls in Systemic Sclerosis 9/18/19

It is easy to say, and difficult to do, but you have got to keep a positive mindset that you will get through the worst of it.

This is a tough condition, and if you’re prone to depression, this can make it worse. What really helps, is to talk to people. It helps if you have a good supportive family around you, and I am lucky to have that.

My husband Andrew and I get out for a walk on a daily basis. If I’m sat around for too long, I stiffen up. And just being out in the fresh air, is good for the mind.

FAQs

How accurate is the Scl 70 test? ›

If the ANA screening panel includes Scl 70 (topoisomerase) as the only SSc-specific antibody, the false-negative rate can be as high as 80%,3 potentially delaying a correct diagnosis for years.

What is the normal range for Scl 70? ›

<1.0 U (negative) > or =1.0 U (positive) Reference values apply to all ages. A positive test result for Scl 70 antibodies is consistent with a diagnosis of scleroderma.

Can you be misdiagnosed with scleroderma? ›

About 300,000 Americans suffer from scleroderma, but because scleroderma has symptoms similar to many other autoimmune diseases, diagnosis isn't as easy as it could be. Unfortunately, this leads to several misdiagnosed or undiagnosed cases.

How serious is systemic scleroderma? ›

It is the most fatal of all the rheumatologic diseases. Systemic scleroderma is very unpredictable although most cases can be classified into one of four different general patterns of disease (see Classification).

Can you have a false positive SCL 70? ›

Conclusion: Assays for anti-Scl-70 antibody in commercial laboratories that are commonly utilized in clinical practice can produce false positive results. These results can lead to angst for patients, as well as unnecessary referrals and diagnostic evaluations.

What blood test confirms scleroderma? ›

ANA: Antinuclear Antibody Assay

Antinuclear antibody (ANA) testing is the most important blood test to screen for scleroderma and other connective tissue diseases.

Can you stop scleroderma? ›

There is no treatment that can cure or stop the overproduction of collagen that is characteristic of scleroderma. But a variety of treatments can help control symptoms and prevent complications.

What tests are positive for scleroderma? ›

Specific Tests for Scleroderma
  • Pulmonary function tests or breathing tests to measure how well the lungs are working.
  • CT chest scan may also be ordered to evaluate the extent of lung involvement.
  • Electrocardiogram (EKG or ECG) to see if there are changes in the heart muscle tissue due to scleroderma.

What does it mean if you have antibodies for scleroderma? ›

Scleroderma patients with RNA polymerase III (RNApol3) antibodies are at increased risk of cancer within three years of diagnosis, most notably for breast and lung cancers. New scleroderma-specific antibodies such as eIF2B, RuvBL1/2, and anti-BICD2 are infrequent, but are associated with unique clinical phenotypes.

How fast does systemic scleroderma progress? ›

In our cohort, 56% of observed progression occurred within 5 years since the first non-Raynaud symptom. While progression in skin involvement occurred more frequently in early disease, the proportion of patients with lung, heart or GI progression was relatively stable over time.

Can systemic scleroderma go into remission? ›

Some patients may have a time when their condition stabilizes. During this time, your skin may improve, and the mobility could increase. This could be a short-term change or might even go into long-term remission.

How did your scleroderma start? ›

Scleroderma results from an overproduction and accumulation of collagen in body tissues. Collagen is a fibrous type of protein that makes up your body's connective tissues, including your skin. Doctors don't know exactly what causes this process to begin, but the body's immune system appears to play a role.

How long can you live with systemic sclerosis? ›

Patients with limited cutaneous systemic sclerosis have a 10-year survival estimate of approximately 90%. However, development of interstitial lung disease (ILD) or pulmonary artery hypertension (PAH) dramatically increases the risk of death.

How long can you live with systemic scleroderma? ›

People who have localized scleroderma may live an uninterrupted life with only minor symptom experiences and management. On the other hand, those diagnosed with an advanced and systemic version of the disease have a prognosis of anywhere from three to 15 years.

Is scleroderma worse than lupus? ›

Worse than in rheumatoid arthritis or lupus. Patients with systemic sclerosis (SSc) have worse health-related quality of life than patients with other systemic rheumatic diseases such as rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE), a Korean study found.

Is ANA always positive with scleroderma? ›

Approximately 95% of people with systemic scleroderma have a positive antinuclear antibody (ANA) test, but so do people with other autoimmune diseases (97% of people with lupus, as an example). People with localized scleroderma usually have negative ANA.

What is the skin pinch test for scleroderma? ›

Figure 2 – Tight or thick skin of the fingers is a symptom of systemic sclerosis (SSc). The normal skin fold of a healthy person can be "pinched" over the middle phalanx (left), but in a patient with SSc (right), no skin fold can be pinched over the middle phalanx.

Does everyone have Scl 70 antibodies? ›

Anti-Scl-70 antibodies are found in 30–40% of dcSSc (Spencer-Green et al., 1997; Derk and Jimenez, 2003) and <5% of patients with the limited disease (using highly specific assays).

What is the newest treatment for scleroderma? ›

Two drugs are currently FDA approved to treat lung scarring, or pulmonary fibrosis, that occurs as a result of scleroderma: nintedanib and tocilizumab.

Does scleroderma affect the brain? ›

Conclusion—The neuropathological findings in these two patients suggest that systemic sclerosis may induce primary vascular changes in the brain, of which calcification may be a marker.

What doctor sees scleroderma? ›

Doctors who most commonly diagnose scleroderma are dermatologists and rheumatologists. Dermatologists have expertise in diagnosing diseases that affect the skin, and rheumatologists specialize in diseases that affect the joints, muscles, and bones.

Can you live a normal life with scleroderma? ›

In general, patients with limited scleroderma have a normal life expectancy. Some have problems with their GI tract, especially heartburn; severe Raynaud's and musculoskeletal pain; and a small subset can develop pulmonary hypertension that can be life-threatening.

What foods make scleroderma worse? ›

Avoid foods that may aggra- vate symptoms such as citrus fruits, tomato products, greasy fried foods, coffee, garlic, onions, peppermint, gas-producing foods (such as raw peppers, beans, broccoli or raw onions), spicy foods, carbonated beverages and alcohol.

What virus causes scleroderma? ›

One theory suggests that scleroderma is caused by a virus or other infection. For example, a common virus called CMV (cytomegalovirus) is known to infect cells (endothelial cells) that line our blood vessels, which we know are diseased in scleroderma (3).

What are the stages of scleroderma? ›

Cutaneous involvement has 3 phases: (1) edematous, (2) indurative, and (3) atrophic. Skin becomes thickened and tight.

How do you reverse scleroderma? ›

There is no cure for scleroderma. Medications can treat symptoms and prevent complications. Making changes to your lifestyle and diet can make living with the disease easier.

What vitamins are good for scleroderma? ›

Consider taking an over the counter multivitamin/mineral supplement containing 15 mg zinc, 10-18 mg iron, vitamins A, D, E, and K, folate, and B-12. If specific nutrient deficiencies have been identified, extra supplementation may be required.

What tests confirm systemic sclerosis? ›

Systemic sclerosis is diagnosed based on the presence of various symptoms above and physical examination findings. The diagnosis can be confirmed by the presence of certain autoantibodies in the blood as well as radiographic studies. Particularly, the ANA, or the antinuclear antibody test, is positive, but not always.

Can scleroderma affect the bladder? ›

People with Scleroderma may experience difficulties with their bladder or bowel. They may experience urinary incontinence, faecal incontinence or constipation due to the effects of the condition and/or medications required.

Does scleroderma affect the eyes? ›

In some cases, scleroderma can lead to eye problems, including the following: Dry eyes According to research in the journal Archives of Medical Science, the most common eye complication in people with Scleroderma is dry eyes. Dry eyes occur when you cannot produce enough film of tears to keep the eyes lubricated.

Can you have Scl-70 and not have scleroderma? ›

Serum anti-topoisomerase, also known as SCL-70, is specific to diffuse scleroderma (and the test is positive in 20% of people with scleroderma). Two-thirds of patients with this antibody have diffuse scleroderma.

Does scleroderma show up on ultrasound? ›

Thickening of the A2 pulley was shown on ultrasound in a group of scleroderma patients to be greater compared to the control group [46]. Color Doppler ultrasound is able to prove an increased tissue blood flow which might be a sign of synovial proliferation due to articular inflammation [47].

Does everyone have Scl-70 antibodies? ›

Anti-Scl-70 antibodies are found in 30–40% of dcSSc (Spencer-Green et al., 1997; Derk and Jimenez, 2003) and <5% of patients with the limited disease (using highly specific assays).

What tests are positive for scleroderma? ›

Specific Tests for Scleroderma
  • Pulmonary function tests or breathing tests to measure how well the lungs are working.
  • CT chest scan may also be ordered to evaluate the extent of lung involvement.
  • Electrocardiogram (EKG or ECG) to see if there are changes in the heart muscle tissue due to scleroderma.

Can systemic scleroderma go into remission? ›

Some patients may have a time when their condition stabilizes. During this time, your skin may improve, and the mobility could increase. This could be a short-term change or might even go into long-term remission.

How quickly does systemic sclerosis progress? ›

In our cohort, 56% of observed progression occurred within 5 years since the first non-Raynaud symptom. While progression in skin involvement occurred more frequently in early disease, the proportion of patients with lung, heart or GI progression was relatively stable over time.

Can systemic sclerosis be misdiagnosed? ›

Since the development of the American College of Rheumatology (ACR) 1980 criteria, there have been advances in SSc-specific serologies and nailfold capillaroscopy examination. In addition, the 1980 criteria missed 20% to 66% of patients diagnosed by expert clinicians [7, 8, 9].

Can scleroderma affect the bowel? ›

After Raynaud phenomenon and skin thickening, involvement of the gastrointestinal system is the most common feature of scleroderma. Gastrointestinal involvement occurs only in systemic sclerosis but is a prominent feature of both diffuse scleroderma and limited scleroderma. Dr.

What does scleroderma do to the digestive system? ›

In the gastrointestinal tract, scleroderma can cause progressive atrophy and collagenous fibrous replacement of the muscularis, which may affect any part or all of the gastrointestinal tract but is more prominent in the esophagus. The lower two thirds of the esophagus often develops a rubber hosepipe-like consistency.

Can you see scleroderma on MRI? ›

Conclusion: In patients with systemic scleroderma experiencing musculoskeletal symptoms, whole-body MRI is able to detect involvement of muscles, fasciae, joints and entheses more confidently compared with clinical and laboratory parameters.

Can you stop scleroderma? ›

There is no treatment that can cure or stop the overproduction of collagen that is characteristic of scleroderma. But a variety of treatments can help control symptoms and prevent complications.

Can systemic scleroderma be mild? ›

Like many autoimmune diseases, systemic sclerosis, or scleroderma, can be a challenge to diagnose because its symptoms are varied, can range from mild to debilitating, and often resemble those of other conditions, such as systemic lupus erythematosus and rheumatoid arthritis.

What is the pinch test for scleroderma? ›

“In using a skin scoring system, the physician pinches various parts of the patient's body to test for thickness. The less skin that can be pinched equals a higher score in diagnosing scleroderma,” says Feghali-Bostwick.

How fast does scleroderma progress? ›

The damage of diffuse scleroderma typically occurs over a few years. After the first 3 to 5 years, people with diffuse disease often enter a stable phase lasting for varying lengths of time.

How did your scleroderma start? ›

Scleroderma results from an overproduction and accumulation of collagen in body tissues. Collagen is a fibrous type of protein that makes up your body's connective tissues, including your skin. Doctors don't know exactly what causes this process to begin, but the body's immune system appears to play a role.

What is the newest treatment for scleroderma? ›

Two drugs are currently FDA approved to treat lung scarring, or pulmonary fibrosis, that occurs as a result of scleroderma: nintedanib and tocilizumab.

Videos

1. Rheumatology Patient Education: Systemic Sclerosis (Scleroderma)
(MassGeneralHospital)
2. When Scleroderma Gets Hard on the Stomach | Lisa D. Lin, MD, MS | UCLA Digestive Diseases
(UCLA Health)
3. The Latest Scleroderma Treatment Strategies
(Hospital for Special Surgery)
4. Treatments for Scleroderma | Systemic Sclerosis
(AmeriStaff Nursing Services)
5. Rheumatology - systemic sclerosis
(childrenshospgh)
6. Scleroderma Subtypes (5 Types of Systemic Sclerosis incl. Limited vs Diffuse Scleroderma)
(Medicosis Perfectionalis)

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