巨细胞动脉炎(GCA)

什么是巨细胞炎(GCA)?

巨细胞动脉炎(或GCA)是一种疾病,可能导致血管疼痛和肿胀。

血管是管子周围携带血液的管。GCA影响动脉,这是三种血管中最大的动脉。动脉从心脏到身体不同部位吸收血液。

GCA通常会影响头部和颈部的动脉。

这种情况会导致头部侧面的柔软部分疼痛和压痛,在您的眼睛和耳朵之间,被称为寺庙。当该病影响头部的这一部分时,可以称为颞动脉炎。

GCA can also affect other large arteries and their branches that take blood elsewhere around the body.

This condition is treatable, usually with类固醇片。But if it’s left untreated it can be very serious and cause strokes or blindness.

GCA是一组称为血管炎。血管炎一词意味着血管中的炎症。有不同类型的血管炎,因为不同的血管可能受到影响。

谁患有大细胞动脉炎?

GCA is very rare in people younger than 50. It’s more common in women than men, and more common in people of north European descent than in people of other races.

GCA通常与称为多肌痛风湿病(PMR),这会导致肌肉的疼痛和僵硬,尤其是在肩膀,颈部,臀部和大腿上。早晨,PMR的症状可能特别糟糕。人们通常都有PMR和GCA。

症状

People with GCA may have a number of symptoms. Most people will have some, but not all of these. The most common symptoms of GCA are:

  • headaches, often with severe pain and tenderness over the temples and the scalp – it may be painful to brush your hair or to shave. Headaches from GCA can cause pain elsewhere in the head too.
  • thickening or tenderness of the blood vessels at your temples
  • 咀嚼时下巴或舌头的疼痛
  • 严重影响您的生活质量的疲倦,也称为fatigue
  • 类似流感的症状,例如轻度发烧
  • 汗水,白天或黑夜
  • 减肥
  • 双视
  • 很少有视力丧失,可能会突然发生。这可能是部分的,但有时可能是总的。通常在早期阶段是临时的。

如果您的视力有任何变化或问题,或者在进食时颌骨疼痛,则应立即看医生。

如果您无法立即与医生预约,请与医生联系,或者立即去最近医院的事故和急诊室。如果未治疗,这些症状可能会导致永久视力丧失或中风。

通常可以通过迅速的类固醇治疗来避免严重问题。

Less commonly, GCA can affect other large blood vessels that could lead to pain when using the arm muscles or in the calves when walking.

很少有人有GCA或PMRfor a long time, they may develop aneurysms. This is when artery walls increase in size and can bulge and become weak.

原因

我们尚不完全知道人们为什么会发展GCA。

It’s an autoimmune condition. This is when your immune system, which is your body’s defence system that fights infections, gets confused and attacks healthy tissues in the body.

在患有GCA的人中,免疫系统错误地攻击健康的动脉。

您从父母那里继承的基因可能使您更有可能发展GCA。但是,仅仅因为有人拥有GCA,这并不意味着他们的孩子一定会得到它。

诊断

将根据以下方式进行诊断为GCA:

  • 您的考试doctoror a专家,并讨论您的症状
  • blood test results
  • X射线和扫描
  • 进行活检,以查看颞动脉壁的炎症。

我应该看到专家吗?

It’s very important that treatment is started straight away if GCA is suspected. A GP can start this treatment.

但是,还应迅速将您转交给专家以确认诊断和治疗计划。

根据您的症状,该专家可能是:

  • 一种rheumatologist– a doctor who specialises in problems with the immune system
  • 眼科医生 - 眼科专家
  • 一种neurologist- 专业知识与大脑,神经和脊柱有关的医生
  • 一种n acute physician – a doctor within a hospital who sees medical emergencies, for example people who have turned up at an accident and emergency department.

需要快速执行测试,因此您可能会紧急推荐。

我会有什么测试?

Blood tests

可以进行血液检查以检查炎症的迹象。这些测试可用于帮助诊断GCA。

随着时间的流逝,它们还将重复检查炎症是否受到控制。血液检查也可以用于寻找其他症状的可能原因。

扫描

You may be asked to have a chest x-ray to rule out other conditions.

可以建议的另一个测试是CT(计算机断层扫描)扫描。这可以帮助诊断GCA,看看是否有任何并发​​症。CT扫描使用许多X射线创建了体内发生的事情的详细图像。

您的医生可能会要求对寺庙周围和腋下的动脉进行超声检查,以帮助确认诊断。

Ultrasound scans use sound waves to examine and build up pictures of the inside the body. They can show what is happening in tendons, blood vessels, joints and even organs. In the case of GCA, these scans can be helpful because they can detect swelling in blood vessels.

Temporal artery biopsy

有时,可以进行颞动脉活检。

活检是当从身体的一部分中取出一小块皮肤或组织并在显微镜下检查。在这种情况下,它来自头部侧面的颞动脉,但是活检也可以在体内其他地方进行。

A local anaesthetic will be used first to numb the area.

活检是由外科医生进行的,但这不是测试而不是治疗。您的医生将解释涉及的内容,风险和利益。您将被要求签署表格,说您了解会发生什么,并且很乐意继续进行活检。

You can have this biopsy to confirm a diagnosis even if you’ve started treatment. If someone has GCA, biopsies can show how serious their condition is.

所有医院尚未提供这些类型的调查。

After the biopsy is carried out you’ll have a wound about 3–4 cm in length, close to the hairline. The wound will be covered with a dressing until your follow-up appointment about a week later, but you will be able to wash your hair with care. When the anaesthetic wears off you may needpainkillerssuch as paracetamol for a while.

与任何外科手术一样,您应该注意出血和感染的迹象 - 例如发红或连续排出。

很少有人会导致神经的暂时或永久损害,这可能导致麻木或下垂的额头。在颈部动脉狭窄的患者中,被称为颈动脉疾病,中风的风险可能很小。

If you have any concerns about the risks, you should discuss them with your doctor beforehand.

Treatment

Steroid tablets

虽然目前无法治愈GCA,但类固醇片非常有效,通常会在几天内开始工作。泼尼松龙是最常用的类固醇片剂。

Steroid tablets slow down the activity of the immune system, and reduce inflammation in blood vessels.

因为如果没有治疗GCA,就有视力丧失或中风的风险,因此立即开始类固醇治疗很重要。如果您的医生怀疑您患有GCA,则在确认诊断之前,他们可能会开出高剂量的类固醇。

要治疗GCA,通常每天都会给您40毫克至60毫克的类固醇片剂。这种剂量通常持续三到四个星期。

如果您在那之后做得很好,并且您的血液检查表明您的病情有所改善,您的医生将开始减少剂量。在此阶段,专家将希望定期见到您的情况。

如果您在进食时会出现视觉症状,或者下巴或舌头的疼痛,则可能需要紧急去医院,以通过滴入静脉滴注类固醇。

通常,完全脱离类固醇需要一到三年。在大多数情况下,您将处于低剂量。并非总是有可能完全停止完全服用类固醇,有些人将需要长时间或一生的低剂量服用。

You shouldn’t stop taking your steroid tablets suddenly or alter the dose unless advised by your doctor, even if your symptoms have completely cleared up. This is because your body stops producing its own steroids, called cortisol, while you’re taking steroid tablets and needs some time to resume normal production of natural steroids when the medicine is reduced or stopped.

如果血管中的炎症返回,这称为复发,您的类固醇剂量可能必须增加才能处理。在治疗的前18个月内,复发是最常见的。

What are the side effects of steroid treatment?

As with many drugs, there are some possible side effects from steroid treatment. However, GCA is potentially a very serious condition. The benefits of taking steroids for someone with GCA, and therefore successfully treating their condition, far outweigh the risks of taking steroid tablets.

最初的高剂量类固醇片以控制状态,然后持续的低剂量以保持疾病,有时会引起以下副作用:

  • changes in facial and body appearance
  • 面部冲洗
  • 缺乏睡眠
  • 消化不良或胃痛
  • 体重增加
  • 头晕或晕倒
  • 困难集中
  • mood changes.

称为质子泵抑制剂的药物可以降低消化不良的风险。他们通过减少胃中自然产生的酸量来做到这一点。

If you’re on steroids for a long time, other side effects may include:

  • 骨质疏松症- 使骨骼更容易稀薄和骨折的疾病
  • 易于瘀伤,妊娠纹和皮肤变薄
  • 肌肉无力
  • 白内障 - 导致一只眼睛或两只眼睛的镜头具有阴云密布的疾病
  • 青光眼 - 眼睛中的视神经受损的疾病
  • 糖尿病 - 一种使您的血糖水平过高的疾病
  • 高血压。

您的定期检查将有助于确定任何副作用,以便可以及时对待。

如果您使用类固醇超过三个月,则可能需要治疗以防止骨骼稀疏,包括:

  • calcium and vitamin D supplements
  • 双膦酸盐 - 降低骨骼质量损失的药物。

由于类固醇减少了免疫系统的活性,因此您可能更有可能发展感染,并且可能更加严重。例如,服用类固醇的人会很严重。

Contact your doctor if you haven’t had chickenpox, and you come into contact with someone who has either chickenpox or shingles, as you may need antiviral treatment.

Steroid cards

If you’re on steroid treatment, you should always carry a steroid card that says what dose you’re on.

如果你需要看另一个医生出于任何原因,such as if you need an operation or a hospital admission; or if you need to see a healthcare professional such as a dentist, you should tell them what dose of steroids you’re on, or show them your steroid card.

在不太可能的情况下,您必须在紧急情况下被送往医院,对于那里的医生来说,要知道您接受类固醇治疗很重要。类固醇治疗会影响人体对损伤的反应,医生需要知道您正在服用类固醇并给您适当的治疗。

类固醇卡可在大多数药店。

Are any other drugs used to treat giant cell arteritis?

类固醇是使GCA控制并防止任何严重并发症的一线治疗方法。目前,没有其他一线治疗。

有时候,您的医生可能会建议另外的药物来帮助您减少类固醇的剂量,如果以下情况可能会发生这种情况。

  • your symptoms return, otherwise known as a relapse
  • your symptoms do not improve despite steroid treatment
  • 您需要长时间的类固醇治疗。

替代治疗可能包括常规疾病改良的抗风湿药(DMARDS), 包括:

这些药物减慢了免疫系统,这可能会使患有自身免疫性疾病的人失火。

还有一些新的药物可用,称为生物疗法。这些药物靶向免疫系统中的关键细胞,以阻止它们引起炎症。

生物治疗包括药物,例如tocilizumab。Tocilizumab主要用于治疗大船只复发的人血管炎。如果其他治疗方法不起作用,也可以开处方。

At times, clinical trials of possible new treatments are carried out, and you may be offered one of these new drugs as part of a scientific study. Before agreeing to take part in one of these trials, you should make sure you fully understand what it involves by talking to your doctor.

Your doctor may suggest low-strength aspirin as it helps to protect against loss of vision in GCA. You need to discuss this with your doctor to ensure it is safe for you to take aspirin.

管理症状

饮食

There are no particular foods that you should avoid. However, you should eat a healthy, balanced and nutritious diet, that is low in saturated fats, sugar and salt, and contains plenty of calcium and vitamin D.

因为长期服用类固醇片剂可以增加糖尿病的风险,因此保持健康的饮食很重要。如果您有任何疑问,请与您的医生交谈。

钙和维生素D对于降低更大的风险很重要骨质疏松症从类固醇治疗。

最丰富的钙来源是:

  • 乳制品,例如牛奶,奶酪和酸奶
  • 富含钙的豆浆
  • 用骨头吃的鱼,例如沙丁鱼
  • 绿叶蔬菜,豆类,鹰嘴豆,一些坚果和干果也含有钙。

You should have between 700 and 1,200 mg of calcium a day.

一些常见食物的适当钙含量

食物 Calcium content
115 g(4盎司)白饵(用面粉炸) 980 mg
60 g(2盎司)沙丁鱼(包括骨头) 260毫克
0.2升(1/3品脱)半脱脂牛奶 230毫克
0.2 litre (1/3 pint) whole milk 220 mg
3个大片棕色或白面包 215毫克
125 g(4 1/2盎司)低脂酸奶 205毫克
30克(1盎司)硬奶酪 190毫克
0.2升(1/3品脱)钙 - 富含大豆牛奶 180毫克
125 g (4 1/2 oz) calcium-enriched soya yogurt 150 mg
115 g(4盎司)干酪 145毫克
115 g(4盎司)烤豆 60 mg
115 g (4 oz) boiled cabbage 40 mg

Note: measures shown in ounces or pints are approximate conversions only.

维生素D可以从某些食物中获得,尤其是从油性鱼类中获得。维生素D通常被添加到豆浆和蔬菜的人造黄油中。对于许多人来说,获得足够的维生素D的最简单方法是补充剂。

需要维生素D来帮助人体吸收钙。在春季和夏季,可以在皮肤中生产维生素D。

Because we can’t guarantee enough sunlight in the UK all year round, doctors recommend that we all take daily vitamin D supplements in the autumn and winter months to ensure we get enough of this important nutrient. Doctors also recommend that groups at high risk of not getting enough vitamin D take supplements all year round. This includes people:

  • who don’t get out of their house or care home much and so don’t have enough exposure to sunlight
  • 谁穿的衣服覆盖了很多皮肤,因此无法接触到阳光
  • 从非洲加勒比海地区和南亚背景来看,由于皮肤较暗的皮肤无法处理英国阳光的维生素D,而不是皮肤较苍白的皮肤。

休息和运动

You should keep as fit and active as you can. This helps prevent osteoporosis, and may help to avoid weight gain and muscle weakness caused by the steroids. Weight-bearingexercise,例如步行或慢跑,可以增强骨骼并降低骨质疏松症的风险。

疲劳对于某些人来说,即使在治疗时也可能是一个主要问题。这可能是很难管理的症状,休息很重要,但是有些人发现逐渐节奏的运动可以帮助您。

节奏和计划活动以及设定现实,可实现的目标可以改善您的能量水平和福祉。

如果疲劳成为问题,可能会有助于预约专家nurseor an职业治疗师。职业治疗师是医疗保健专业人员,可以帮助人们提高由于医疗状况而可能正在挣扎的日常任务的能力。

生活方式

吸烟会大大损害骨骼健康,并使您更有骨质疏松症的风险。吸烟也会增加中风的风险。GCA患者的中风风险已经比普通人群稍高,因此,如果您患有GCA,请不要吸烟尤其重要。

Drinking too much alcohol is bad for bone health, because it makes it harder for the body to use calcium to make bones strong.

莫琳的故事

我总是非常感谢我在事故和紧急情况下看到的医生发现了巨型细胞动脉炎的症状,并可能拯救了我的视力。

我三天前去过GP手术,因为我的脑海里遇到了这些不良的痛苦。我有一个超声扫描的日期,试图进入正在发生的事情的底部,但是这次约会尚未再过八个星期。

I was at home in the evening three days later and my symptoms got much worse. I had these lumps in my temple, and then all of a sudden I couldn’t open my jaw. I had real pain in my head.

My partner Brian said ‘Right, we’re going to A&E’. I am so glad he was insistent and didn’t take any chances. We went straight to the accident and emergency department at the local hospital.

我看了一位可爱的医生。她听到了我的症状,给了我60毫克类固醇片。她对我说:“立即带他们”。

我问她恐慌是什么,她说:“我认为您患有巨大的细胞动脉炎,这会影响您的视力。”

几天后,我看到了一位专家,他确认这是巨型细胞动脉炎。

After two months of steroid treatment, I was told it had cleared up. I have since developed large vessel vasculitis and I’ve had to go back on the steroid tablets. I’ve not got any symptoms for that.

我非常感谢当晚的A&E医生发现了巨细胞动脉炎的症状并知道该怎么做。