Systemic sclerosis (scleroderma)

全身性硬化症是一种罕见的疾病，会导致人的皮肤和人体内部的结缔组织变硬。

有时，随着皮肤变硬并变厚，您的脸部，手，脚和其他地方的脸部，手，脚和其他地方都会看到系统性硬化症的迹象。

全身性硬化也会导致体内的结缔组织变厚而坚硬。

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.

全身性硬化是一种自身免疫性疾病。这意味着可以保护您免受疾病和感染的免疫系统的困惑并攻击人体自己的细胞。

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.

弥漫性全身性硬化症会影响整个体内的皮肤，并且更有可能影响内部器官。

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.

以下信息是关于系统性硬化症的条件，在该状态下，体内的皮肤和结缔组织受到影响。

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

对寒冷的敏感性（Raynaud的现象）

Most people with systemic sclerosis develop an unusual sensitivity to the cold, known asRaynaud’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 phenomenonis 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.

雷诺（Raynaud）通常是某人有系统性硬化症的第一个迹象。有时可能会在系统性硬化症发作之前出现几年。

Changes in the skin

由于全身性硬化引起的皮肤最常见的变化是：

• thickening and hardening of the skin on the hands, arms and face
• 手和脚肿胀，尤其是在早晨
• shiny skin, without its usual creases
• 面部皮肤僵硬，使张开嘴很难
• small red blood spots on the face, hands and arms.

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

• 嘴唇变薄
• 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.

消化问题

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.

我们有一个管子，将食物从口腔到胃中，称为食道（O-Soff-a-gus）。全身性硬化会导致该管中的肌肉削弱，这可能会使吞咽困难。

由于该食物管中的胃酸过多，全身性硬化会引起胃灼热。这种酸是在胃中自然产生的，以帮助分解食物。

然而，全身性硬化会导致这种酸从胃中渗出到食物管中。这可能是不愉快的，对我们不利。

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.

它也会引起疼痛并在受影响的关节周围肿胀。

肌肉无力有时也是全身性硬化症的症状。

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.

系统性硬化症对每个人的影响都不同，因此很难说它可能如何影响您。

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

大约五分之一的系统性硬化症患者也会出现另一种疾病的症状，例如lupus,Sjogren’s syndromeor 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.

对于某些人来说，症状几年后几乎可以消失。

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
• 肺的血管缩小，导致肺部高血压，进而可能劳累
• 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.

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
• 胃测试（例如内窥镜检查）
• 皮肤活检，其中一小块皮肤被去除并在显微镜下检查
• 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.

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

药物可以帮助解决Raynaud’s phenomenonby 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.

在更严重的雷诺（Raynaud伊洛列伯斯可以作为输液给出。输注是一种通常在医院中滴入静脉的药物的一种方式。

药物通过放松血管并增加手指和脚趾的血液流动来治疗Raynaud的现象。它们还将对体内其他地方的血管产生相同的影响。因此，这些药物会引起副作用，例如皮肤冲洗，头痛和脚踝肿胀。

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.

治疗与胃和消化系统有关的其他问题

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.

有时，肠中会出现额外的细菌，这可以通过抗生素等药物来治疗。

重要的是要注意腹泻和尿失禁等症状，以确保他们没有其他原因。

Treating painful and swollen joints

Painkillers和non-steroidal anti-inflammatory drugs (NSAIDs)应该有助于缓解关节疼痛和肿胀。

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.

这是一个严重的并发症称为系统性sclerosis renal crisis. It can be treated or prevented with drugs that help to control the blood pressure, especially ACE inhibitors.

治疗肺和心脏并发症

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

肺中的高血压（肺动脉高压）是全身性硬化症的罕见并发症，但是您将进行定期检查以检查肺和心脏的工作状况。

如有必要，可以用特定药物（包括波森坦，安布里丹，西地那非或前列环素）治疗肺动脉高压，以改善诸如呼吸困难之类的症状。

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.

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, includemethotrexate,环孢菌素,cyclophosphamide,mycophenolate和azathioprine. It will depend on how systemic sclerosis affects you as to whether you will be prescribed any of these drugs.

The biological therapyrituximab有时用于治疗严重的全身性硬化病例。生物疗法是具有更有针对性方法的新药物。它们降低了可能引起问题的免疫系统中关键细胞的有效性。

对于系统性硬化症的某些症状，您需要服用药物。但是，健康和平衡饮食，定期运动和良好的皮肤护理程序也很重要。

Exercise

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

尽管如果关节肿胀，您可能需要休息，但尽可能多地移动通常会很有帮助。

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

Aphysiotherapist或aspecialist hand therapistwould 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.

饮食和营养

Maintaining a healthy diet is important as it may helpRaynaud’s symptoms和the healing of skin ulcers.

However, digestive problems can make it difficult to keep up a balanced饮食和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.
• 慢慢吃饭，彻底咀嚼，用餐喝大量水。
• 在一天中，吃最大的饭菜可以帮助避免胃灼热。
• 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.

补充医学

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.

尽管缺乏科学证据表明补充医学可以帮助缓解全身性硬化症的症状，但大多数被认为可以安全使用。去著名和注册的供应商总是很重要。如果您想尝试互补的医学，请先先检查GP也很重要。

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和gingeror gingko dietary supplements can also help. If the vitamins don’t help within a three-month period, you should stop taking them.

在尝试补充医学之前，请务必与您的医生交谈。

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 anoccupational therapistfor 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 charityRemapmight 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.

如果您符合资格标准，您可能会获得艾滋病和次要改编费用的帮助。标准包括您患有身体或精神疾病或残疾，无法执行基本任务或活动，例如：

• 烹饪
• eating
• 洗涤
• going to the toilet
• getting dressed
• 打扫
• 在职的
• 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.
• 如果你的皮肤坏了或痛苦,调料可以help to protect it.
• 不要使用强壮的洗涤剂或其他任何会刺激您的皮肤的东西，也不要使用含有天然蜡羊毛蛋白的肥皂。
• 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.
• 吸烟会减少血液流向您的皮肤，很可能会使Raynaud的症状恶化，因此最好不要吸烟。如果您需要帮助停止吸烟，请与GP或药剂师交谈，他们将能够提供支持和建议。他们也许还可以将您推荐给当地的停止吸烟服务。

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.

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情绪上的困难与长期状况有关，皮肤外观的变化可能会令人沮丧。此外，压力可以减少到身体某些部位的血液流动，因此会影响您的病情，尤其是在您有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 withScleroderma 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.

beplay体育怎么下载与关节炎目前正在为我们的几项研究提供资金，以提高我们对系统性硬化症原因的理解。

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.

我们还支持研究，以更好地了解是什么导致系统性硬化症患者的皮肤和内部器官过度疤痕。疤痕组织是由称为肌纤维细胞的细胞制成的，一种称为C-Rel的蛋白质被认为可以调节其产生。这项研究旨在了解C-Rel如何调节疤痕的产生，并发现改变其功能或删除其功能会防止形成疤痕。

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.

我的症状始于2013年7月。我的手指像疯了一样肿胀，脚踝肿胀了很多，我看不到它们。没有人能告诉我发生了什么。我被称为风湿病学家.

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.’

我们去了意大利北部的滑雪假期，因为正处于高空。我生病了，正在努力呼吸。我必须被直升机到一个叫做博尔扎诺的小镇的医院。

我在意大利的这家医院呆了两周半。那里的医生说：“有人告诉过你你有硬皮病吗？”我以前从未听说过。这真是令人震惊。他们使我稳定下来，所以我可以回到英国。

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

他们对我的肾脏进行了活检，并把我带走了。我一直在那里，直到他们确认幸运的是我不需要肾脏透析。当他们看到改善的迹象时，我被允许回家。

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.

我的早期治疗很积极,但它seemed to have the desired effect. I had to give up work though. I was too ill to continue at the time.

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.

这是一个艰难的条件,如果你容易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.

我的丈夫安德鲁和我每天出去散步。如果我坐了很长时间，我就变得更加僵硬。而且只是在新鲜空气中，对心灵有好处。