血管炎

What is vasculitis?

血管炎means inflammation of the blood vessels, the tubes that carry blood around your body. There are three types of blood vessel which can be affected by vasculitis:

  • 动脉 - 从心脏上吸血到身体的不同部位,例如器官(例如肾脏)和组织(例如皮肤)
  • veins – take blood back to the heart
  • capillaries – tiny vessels between the arteries and the veins where oxygen and other materials pass from the blood into the tissues.

The organs and tissues in your body need a regular blood supply to work properly. Inflammation causes swelling of the blood vessel walls, reducing or even blocking the flow of blood to the tissues and organs.

血管炎会引起一系列症状和可能的并发症。损害血管炎的原因取决于身体的哪一部分受到影响。

受影响的血管越大,损害可能就越多。受影响的身体组织越重要,损害就越严重。

The walls of affected blood vessels can swell and bulge (this is called an aneurysm) and may even burst, causing bleeding inside your body. Apart from the damage to the blood vessel itself, this can lead to damage in the tissues or organs which are supplied by the blood vessel.

血管炎可能突然发生在以前完全好的人中 - 当它自己发生时,医生称这种原发性血管炎。血管炎也可能与其他疾病一起发生(包括类风湿关节炎,lupusorSjögren's syndrome), in which case it's known as secondary vasculitis.

Read more about vasculitis in the血管炎路由, 由。。。生产英国血管炎

谁明白了?

血管炎is uncommon. Overall, in every 100,000 people in the UK, only 10–15 will develop vasculitis each year. However, about 22 people per 100,000 aged over 50 years will develop巨细胞动脉炎(GCA)。The different types of vasculitis tend to affect different age groups, for example:

  • GCA (temporal arteritis) is much more common in people over 50, and it's fairly common for it to be associated with a condition calledpolymyalgia rheumatica (PMR)
  • Takayasu arteritis (TA) tends to affect younger Asian women.
  • IGA血管炎在儿童中比成人更为常见。
  • 川崎病只会影响五岁以下的儿童。

症状

当您的身体的任何部位发炎时,它会肿胀并且不舒服或痛苦。由于多种类型的血管炎,肿胀在体内,您看不到外部的任何症状。

血管炎takes different forms according to which blood vessels are affected, and symptoms vary. Many people with vasculitis feel unwell and have fever, sweats, fatigue and weight loss. These can be the first symptoms experienced, so it's important to be seen by your GP.

Other symptoms vary according to which part of the body is affected, for example:

  • 皮肤 - 皮肤中的血管炎会引起可能破裂的斑点,留下开放的疮(溃疡)。当血管炎只会影响皮肤时,长期影响通常不会严重,并且一旦炎症解决,症状通常会清除。
  • 鼻子 - 这里的血管炎会导致鼻子和流鼻血的结层。有时,鼻子的形状会改变。
  • 手指和脚趾 - 一些血管炎的人Raynaud's phenomenon,手指或脚趾变成白色或蓝色的地方,可能在暴露于寒冷条件下刺痛或受伤。
  • 眼睛 - 某些类型的血管炎会突然影响您的视力或导致眼睛变红或痛苦。如果发生这种情况,迅速去看医生非常重要。
  • Nerves – inflammation of the nerves can cause tingling (pins and needles), pain and burning sensations or weakness in the arms and legs.
  • Joints – vasculitis can cause joint pain or swelling.
  • 肌肉 - 这里的炎症会导致肌肉酸痛,最终您的肌肉可能会变得虚弱。
  • Lungs – inflammation of the lungs causes coughing and shortness of breath.
  • Brain – occasionally the blood vessels in the brain can be affected, causing problems like strokes.
  • Kidneys – when vasculitis affects the kidneys there may be problems passing urine or blood in the urine. Vasculitis of the kidneys can be dangerous as symptoms may not appear until the kidneys have been damaged. In severe cases treatment on an artificial kidney (dialysis) machine may be necessary.

Headaches, pain in the jaw and problems with the eyes can be serious symptoms of巨细胞动脉炎(GCA)

原因

没有单一的血管炎原因,在大多数情况下,确切原因是未知的。我们知道,血管炎不是通过我们从父母那里获得的基因直接遗传的,但是遗传因素确实起作用,因为在同一家庭中可能发生了几种情况。您的基因可能使您更有可能发展这种状况,在这种情况下,只需触发小小的触发因素(例如感染或药物)即可开始。

我们还知道,某些类型的血管炎(例如,影响小血管的血管炎)可能与感染有关,尤其是与肝炎相关的感染。某些药物治疗后发生的一些血管炎,例如丙硫酸丙酸甲章(用于治疗甲状腺疾病)和allopurinol(used to treatgout), as well asnon-steroidal anti-inflammatory drugs (NSAIDs)and antibiotics.

人们认为大多数形式的血管炎是一种自身免疫性疾病。这意味着您身体的防御机制并没有在抗击感染方面做正常的工作,而是攻击身体的健康部位,引起炎症。

How will vasculitis affect me?

The most severe types of vasculitis can be life-threatening. Early diagnosis and treatment is essential for the best chances of avoiding permanent damage to tissues and organs.

大多数类型的血管炎对治疗的反应很好,对于许多血管炎,您可能会完全康复,尽管将来可能会复发。

结果取决于血管炎的类型及其对您的影响。总体而言,了解将来可能发生的事情的最佳方法是与您的医生或其他健康专家交谈。

谁诊断和治疗血管炎?

If you have vasculitis it's important you have one consultant who is in overall charge of your care. The management and care of patients with vasculitis is usually undertaken mainly by a风湿病学家, as they specialise in inflammatory diseases, or less frequently by a nephrologist (kidney doctor), especially if the kidneys are affected.

However, vasculitis in some circumstances may affect a variety of different organs. If required, your care should involve a multi-disciplinary team of consultants with different specialities, working together. For example, if your lungs and airways are seriously affected, care may involve a respiratory consultant. In some cases an ear, nose and throat (ENT) consultant, an ophthalmologist,neurologist, gastro-enterologist, immunologist or dermatologist may also be involved.

Diagnosis

如果您认为自己可能正在患有血管炎,则必须尽快去看医生。感染,药物和某些食物有时会引起血管炎,因此您的医生可能会询问您在过去几周中服用的药物以及您的一般健康状况。

有哪些测试?

可能有许多测试可以帮助诊断病情。在本节中,我们将查看最常见的情况,但是您可能需要其他一些。问你的风湿病学家, other specialist or GP if you're not sure about what a certain test will involve.

Blood tests may be used to measure inflammation – for example, the erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP).

A full blood count can help to establish whether you have anaemia and whether you have normal levels of white blood cells (which fight infections) and platelets (which are involved in clotting).

癌细胞质抗体(ANCA)的血液测试对于诊断某些类型的血管炎很重要,特别是:

  • 韦格纳肉芽肿
  • eosinophilic granulomatosis with polyangiitis
  • 微观多血管炎。

以下测试可用于检查肾脏的工作方式:

  • a urea and elecrolytes (U&E) test
  • an estimated glomerular filtration rate test (eGFR)
  • a creatinine test.

Liver function tests may also be carried out to check how your liver is working.

If you have vasculitis along with other conditions, such as类风湿关节炎orlupus, then blood tests might be used to assess how active these other diseases are. Blood tests can measure the level of rheumatoid factor in rheumatoid arthritis, or the levels of complement (an enzyme system or group of proteins in the blood) and antibodies in lupus.

Blood tests may be repeated from time to time to check how your condition is responding to treatment.

Other tests may be carried out to see how the affected body organs are working – for example:

  • Urine tests will show the presence of blood and/or protein, which are often the first signs of an inflamed kidney. People with eosinophilic granulomatosis with polyangiitis, granulomatosis with polyangiitis or microscopic polyangiitis will have regular urine tests for blood and protein.
  • X射线,CT和MRI扫描可用于检查胸部问题。
  • Echocardiograms and electrocardiograms can be used to assess the heart. An echocardiogram is a special ultrasound test and an electrocardiogram (ECG) is an electrical test.
  • A biopsy may be needed to confirm whether the kidneys, muscles, skin or lungs are affected by vasculitis. A small piece of tissue is removed from the organ in question for examination or testing in a laboratory.
  • An ear, nose and throat (ENT) assessment may be needed for people with granulomatosis with polyangiitis who have symptoms in these parts of the body.
  • 在涉及肾脏和肠道等腹部器官的情况下,通常会进行血管造影。这涉及将染料注入动脉,以便它们出现在X射线上。它们也可以在高山动脉炎和巨细胞动脉炎中进行,以查看涉及多少大血管。

有哪种类型的血管炎?

医生们通常定义血管炎acc的类型ording to the size of the blood vessels involved. The most serious types of vasculitis involve both small and medium-sized arteries.

Takayasu arteritis

Takayasu指出动脉炎(TA)是一种炎症性疾病that affects the main artery from the heart (the aorta) and its large branches, usually in younger women. It's rare in the UK (there are only 100 new cases a year) but it's more common in the Far East and Africa.

TA导致动脉变窄,这可以减少血液供应。狭窄的发展缓慢,动脉通常不会完全阻塞,因此通常不会造成臂,腿或任何主要器官的血液供应危险。

Other major arteries can also be affected, including:

  • the carotid arteries in the head and brain
  • 心脏中的冠状动脉
  • renal arteries to the kidneys
  • arteries that take blood to the arm.

巨细胞动脉炎(temporal arteritis)

巨细胞动脉炎或GCA会影响提供头部和颈部的大动脉,尤其是在寺庙上发现的颞动脉。英国每年大约有5,000例新案例,在北欧更为常见。GCA通常不会影响50岁以下的人。

GCA can cause headaches and is often associated with a condition calledpolymyalgia rheumatica (PMR), which causes inflammation and stiffness in the muscles of the shoulders and hips.

GCA偶尔涉及的血液供应eye, where it can cause blindness. If you develop symptoms in your eyes, such as blurring or double vision, you should see your doctor straight away as you'll need to be treated urgently.

其他血管,例如主要动脉,通常不太参与GCA。

多动炎结节

多动炎结节(PAN)can be very serious, but it is very rare – only about one out of every two million people in the UK develop PAN each year. In some cases PAN can be associated with hepatitis B virus infection.

PAN causes inflammation in the medium-sized arteries, particularly those supplying blood to the gut and kidneys. This may only affect part of the wall of the artery, which becomes weak and may bulge, forming an aneurism. If it bursts it can cause serious internal bleeding.

PAN can also involve the whole wall of the artery at a particular point, which causes a blockage.

Kawasaki disease

川崎疾病会影响中小型动脉,最常见于五岁以下的儿童。有时被称为粘膜皮肤淋巴结综合征(因为它涉及粘膜)。

Children with Kawasaki disease will feel unwell – they may have a high temperature, swollen glands in the neck (lymphadenopathy), an inflamed area around the eye and the mouth, and a skin rash similar to measles.

This condition is quite rare but can be serious if the arteries supplying the heart are inflamed (coronary arteritis). Up to 60% of people with Kawasaki disease have coronary arteritis.

韦格纳肉芽肿

The condition granulomatosis with polyangiitis is quite rare – altogether there are only 1,000 new cases of granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis and microscopic polyangiitis a year in the UK. It's slightly more common in men than in women.

It usually develops with ear, nose and throat problems, including nosebleeds and crusting of the nose, and occasionally coughing up blood (haemoptysis). These symptoms can appear a year or two before more general vasculitis starts.

This general vasculitis usually involves the skin, lungs, eyes and kidneys. The kidney problems can sometimes lead to kidney failure if they're not recognised early.

这种疾病的名称最近发生了变化。以前被称为Wegener的肉芽肿病后,他被认为发现了它。新名称描述了该疾病的特征:

  • polyangiitis means that many (poly = many) arteries are involved
  • granulomatosis means that when a biopsy of the inflamed tissue is studied under a microscope you can see swellings called granulomata.

Remember that the terms Wegener's granulomatosis, granulomatiosis with polyangiitis, and GPA describe exactly the same disease.

Behçet's syndrome

Behçet's syndrome, or Behçet's disease (pronounced betchets) is a rare autoimmune condition that can involve mouth ulcers, genital ulcers, skin problems and eye inflammation. It's not an infection and can't be passed from one person to another.

它还可能涉及身体的其他区域,例如胃肠道以及肺部,肌肉骨骼,心血管和神经系统。

Because of the many different parts of the body that can be affected with Behçet's, many different medications can be used to control symptoms.

Eosinophilic granulomatosis with polyangiitis

嗜酸性粒细胞性肉芽肿与聚血管炎引起成人发育,随后由称为肉芽肿的肿胀引起的血管发炎。血液中通常还会有大量的嗜酸性粒细胞(一种特定类型的白细胞)。

由于哮喘,该条件与肉芽肿性有肉芽肿性不同。与肉芽肿性伴有聚血管炎不同,嗜酸性粒细胞性肉芽肿和多血管炎很少会损害您的耳朵和鼻子。

Eosinophilic granulomatosis with polyangiitis can also affect the nerves, causing weakness, pins and needles or numbness.

您的心脏涉及的风险也更高,这有时会对心脏肌肉造成损害,类似于心脏病发作期间的损害。

This disease was previously called Churg-Strauss syndrome after the doctors who were thought to have discovered it. The new name describes the features of the disease – eosinophils, granulomata and involvement of many blood vessels.

微观多血管炎

Almost all people with microscopic polyangiitis have kidney problems that can lead to raised blood pressure and kidney failure.

People may find that they're tired because of anaemia. Anaemia is a shortage of haemoglobin (oxygen-carrying pigment) in the blood which makes it more difficult for the blood to carry oxygen around the body.

Blood tests can show whether the kidney is inflamed. Microscopic polyangiitis can also involve the lungs, with bleeding that can cause breathlessness.

Cryoglobulin-associated vasculitis

In cryoglobulin-associated vasculitis, small-vessel vasculitis is associated with cryoglobulins – these are proteins in the blood that stick together in the cold. Having cryoglobulins can reduce the flow of blood or even block the blood vessels, causing damage to organs or body tissues.

免疫球蛋白A血管炎

免疫球蛋白A血管炎, also known as IgA vasculitis, affects the small blood vessels (capillaries). It often follows a chest infection and may be an allergic reaction to a virus, food or drugs. It mostly affects children aged 2–10 years, and boys are affected more often than girls.

It can also affect adults, but most of the 2,500 new cases a year in the UK are children.

IGA血管炎的症状包括:

  • a skin rash, often over the buttocks (starts red but develops into a bruised purple colour and appears over several days or even weeks)
  • short-lived arthritis, especially of the larger joints
  • 胃痛和/或呕吐或粪便中的血液
  • passing blood in urine (indicating kidney problems)
  • fever, headaches and loss of appetite.

IGA血管炎中看到的皮疹被称为紫紫红色,由Henoch和Schonlein医生描述,解释了该疾病的先前名称。

In most cases the condition doesn't need specific treatment, although relapses are possible for up to a year after the original illness. Kidney problems are quite common, but serious kidney damage is rare.

有时涉及其他血管,很少会发生更严重的并发症,有时会影响肠子或引起癫痫发作。

Treatment

用于血管炎的治疗方法将取决于受影响的血管和器官以及受影响的人体组织受到多少影响。如果血管炎仅影响皮肤,则可能足以治疗任何潜在的感染或去除引发血管炎的药物。但是,在大多数情况下,需要药物治疗来控制疾病及其症状,并阻止或限制血管炎造成的损害。

毒品

用于治疗血管炎的两种主要药物是类固醇和免疫抑制剂。两者都起作用降低免疫系统,以降低其对身体组织的攻击强度。

If you have vasculitis affecting the large blood vessels, then you'll probably be given类固醇片。这些非常有效巨细胞动脉炎and Takayasu arteritis, and often immunosuppressive agents aren't needed for these conditions.

如果您的血管炎影响小和/或中型血管,则可能只需要一小剂量的类固醇即可控制它。

However, you may need a combination of steroids and immunosuppressive drugs, probably over several years, especially if vasculitis affects internal organs. For many types of vasculitis, including those affecting the kidney, lungs or other vital organs (especially if it involves both small and medium-sized blood vessels), your treatment will be given in stages.

如果您患有主要影响中型动脉的血管炎,那么其他疗法可能会有所帮助,具体取决于这种情况:

  • 可以用免疫球蛋白(一种蛋白质)的注射来有效治疗川崎疾病。
  • Hepatitis-associated PAN can be treated with antiviral treatment and plasma exchange.

Plasma exchange (also known as blood washing) involves being connected to a machine that your blood passes through before being returned to you so it can be cleaned of the factors causing the vasculitis. Only a few people with the most severe types of vasculitis – for example who have very severe kidney or lung disease – will need plasma exchange. This will be done in specialist centres.

Possible side-effects of steroids include weight gain, indigestion, diabetes, thinning of the skin and thinning of the bones (osteoporosis). If high doses of steroids are given, then you'll also be given drugs likebisphosphonatesto help prevent osteoporosis.

某些类型的血管炎的治疗阶段:

阶段1:使您缓解。目的是使疾病受到控制。免疫抑制药物(例如cyclophosphamide)可用于抑制正在攻击血管的免疫系统。类固醇片剂或注射剂也将在此阶段使用。

Stage 2 Keeping you in remissions. Once the condition is in remission, the aim is to keep it under control. Drugs (for exampleazathioprine,methotrexateormycophenolate)可以与类固醇一起使用。

Stage 3: Following up and withdrawing therapy. The aim at this stage is to gradually reduce your medications while still keeping the disease under control.

在某些类型的血管炎(例如肉芽肿性炎血管炎)中,感染可能会引发复发。因此,您可能会为您提供抗菌药物,例如辅助药物,以防止这种情况。这些药物还可以帮助防止因免疫抑制药物较强而引起的感染风险增加。

As with all medications, there may sometimes be side-effects.

Cyclophosphamide, for example, can cause bleeding from the bladder, hair thinning and an increased risk of infection. Unfortunately, there's also a significant risk that it can reduce fertility in both men and women.

Because of these risks, cyclophosphamide will be stopped or exchanged for a different immunosuppressive drug as soon as your vasculitis is controlled. This is usually azathioprine, but methotrexate or mycopheonlate might be used instead.利妥昔单抗,生物治疗静脉infusion, can also help to encourage remission in some types of vasculitis.

Managing symptoms

Learn how to manage the symptoms of vasculitis.

Exercise

If you do need treatment then it's very important that you follow your doctor's instructions carefully. It's important to learn and understand as much as you can about your illness and the treatment options; you can discuss alternative treatment options with your medical team.

血管炎从一个人到另一个人,从一种类型的血管炎到另一种类型。重要的是要与您的医生或其他医疗保健专业人员谈谈您可能出现的任何新症状。

血管炎can cause tiredness, and it's important to rest when you need to. However, you should also try to keep muscles and joints healthy by exercising. Start gently and gradually increase the amount of exercise you do. Include some weight-bearing exercise (anything that involves walking or running). Swimming is also recommended. Ask your doctor for advice on how much exercise you should expect to be able to do.

Diet

You won't usually need to keep to any special diet.

A healthy, low-fat, nutritious and balanced diet is important for everyone, but if you're on steroids it's particularly important because these can increase your appetite and cause weight gain. Try not to over eat, and cut down on fatty and sugary foods and others which are particularly high in calories. Instead, eat lots of fresh fruit and vegetables and starchy foods like potatoes and wholemeal bread, pasta and rice.

喝大量水很有帮助。对于每个人来说,不超过建议的每周酒精单位的饮酒是健康的;男女每周14。建议至少有两个无酒精的日子,但这并不意味着您一口气节省所有的单位。

If you're taking steroids then you're more at risk ofosteoporosis, but having plenty ofcalciumin your diet is important to help prevent this from developing. Foods that are good sources of calcium include tinned sardines (with bones), skimmed milk, yoghurt and certain vegetables such as broccoli.

Living with vasculitis

Stop smoking

Smoking makes the blood vessels become narrower inside and can therefore make vasculitis symptoms worse. Smoking can also worsen lung inflammation. It's therefore very important to try to stop smoking. This will also improve any symptoms of Raynaud's phenomenon. If you want to stop smoking see your doctor who will be able to give you advice about quitting.

保持温暖

If your fingers or toes turn blue in response to the cold, this may be due toRaynaud's phenomenon。Wearing warm clothes, including warm socks and gloves, should improve blood circulation to your hands and feet by helping to keep the blood vessels open.