雷洛氏综合症

1、什么是雷诺氏病（征）？

目錄 Show

What is Raynaud's phenomenon?
Risk factors
Signs and symptoms
Methods for diagnosis
Types of treatment
Managing Raynaud's attacks
Medications
Stress management
More intensive treatment
Complementary and alternative medicines
Potential complications
FAQ Frequently asked questions
Related topics
About this article

雷诺氏病是一组因血管神经功能紊乱而引起的阵发性末梢动脉痉挛性疾病。

因1862年法国医生Raynaud首先报道了25例因指动脉痉挛引起的发作性手指缺血疾病,将其命名为雷诺氏病（Raynaud`s disease）其后Hutchinson观察到雷诺氏病并非一种独立的疾病,而是可由多种疾病引起的一种共同征象,从而提出“雷诺氏现象(Raynaud`s phenomena）”的概念。目前对该病的命名倾向于统称“雷诺氏综合征(Raynaud`s syndrome）”,包含原发性雷诺氏病和继发于其它全身性疾病的雷诺氏现象两种类型。

2、雷诺氏病有何临床表现？

雷诺氏综合征常由寒冷刺激、或情绪激动等诱发,典型症状表现为肢端皮肤颜色呈阵发性“苍白—紫绀—潮红—正常”的周期性改变，苍白、紫绀、潮红期伴肢端严重刺痛，肤色正常后疼痛亦消失。症状反复发作后期会导致指(趾)端皮肤营养不良性改变如指端溃疡或坏疽形成等。一般以上肢双手常见，,偶见于下肢、口唇及乳头。多见于女性,男女发病率之比约为1:10。发病年龄多在20~30岁之间,寒冷地区和寒冷季节好发。

3、雷诺氏综合征的病因和机制是什么？

雷诺氏综合征的病因目前仍不是十分明确,可能与交感神经功能紊乱、血管内皮损伤、神经激素异常、血液学异常及性腺功能异常等有关。

寒冷刺激、情绪激动、精神紧张、内分泌紊乱等是主要的激发因素。其病理机制主要为肌性小动脉和指(趾)小动脉剧烈痉挛,引起组织缺血(苍白期)、缺氧和代谢产物堆积(紫绀期),继之血管舒张,组织充血再灌注（潮红期)，缺氧改善和代谢产物堆积被带走（恢复正常）。研究显示,血管周围的自主和感觉神经、血管内皮细胞、血管平滑肌等都参与了血管舒缩的调节。雷诺氏综合症病理机制主要有如下三方面:(1)神经源性机制;(2)血液和血管壁的相互作用;(3)炎性异常及免疫反应。研究表明患者常呈交感神经功能亢奋状态,应用交感神经阻滞剂可缓解症状,因此交感神经过度兴奋可能是本征的主要原因，而后二者常与继发雷诺氏现象有关。

4、如何治疗雷诺氏综合症？

A、药物治疗：硝苯地平、利血平、前列腺素、温补类中药。

B、手术治疗：经内科治疗无效并出现严重指端缺血症状或因药物本身的不良反应不能继续用药的患者,可采用手术方法进行治疗。目前常用的手术方法有胸腔镜上胸交感神经阻断术、化学性胸交感神经切断术、星状神经节阻滞术和动脉外膜交感神经末梢切除术等。通过外科治疗可显著改善患者肢体末端缺血症状,促进溃疡愈合,缓解疼痛,降低坏疽及截指(趾)的发生率,提高患者的生活质量。

C、微创介入治疗：针对雷诺氏综合症的主要发病机制为胸交感神经过度兴奋引发的小血管反应性痉挛，我们采用“CT引导下经皮穿刺胸交感神经阻滞”这一微创介入治疗技术来解除胸交感神经的过度兴奋，取得了良好的效果。本技术的优点在于无需手术，创伤小（只从背后扎两根细针），见效快（阻滞注药后5min即见效），病人恢复快（拔针后即可行走），费用低（不及胸腔镜手术费用的1/2），值得临床推广。

D、对于继发性雷诺氏现象,同时治疗原发疾病。

E、雷诺氏综合征的预防：预防雷诺氏综合征的关键在于加强保暖措施、及时缓解精神压力、保持心情愉快。

雷诺氏综合征[6]（英语：Raynaud syndrome），也作雷诺现象（Raynaud phenomenon），是指由于血管痉挛而引起的一系列血管血流减少的情形。[1]一般手指更容易受到影响，脚趾有时也会受到波及。[1]在较为罕见的情形下，鼻、耳、唇也会出现相应症状。[1]雷诺氏综合征会导致肢体变得苍白，随后发绀，[2]并常伴有麻木、疼痛。[2]当血流恢复时，患处会变得潮红并有灼热感。[2]该症状通常持续几分钟，但也可持续数小时。[2]

雷诺氏症候群的发作可能被寒冷或是情绪压力所诱发[2]。可以分成两种主要类型：原发性雷诺氏症候群和续发性雷诺氏症候群。原发性雷诺氏症候群的成因未知，而续发性雷诺氏症候群则伴随著其他症状出现[3]，可能因结缔组织疾病如硬皮病或红斑性狼疮、手部受伤、长时间震动、抽烟、甲状腺问题、一些医疗处方如服用避孕药而发生[7]，通常藉由其症状来诊断[3]。

原发性雷诺氏症候群的处置为避免暴露在寒冷环境[3]，其他处置包含停止使用尼古丁或兴奋剂[3]。对于症状没有改善的案例，可以给予钙离子通道阻滞剂和伊洛前列素[3]。有些许的证据支持替代疗法的效果[3]。罕见的情况下可能并发皮肤溃疡或坏疽[2]。

大约有4%的人口有雷诺氏症候群的现象[3]。原发性雷诺氏症候群好发于15至30岁，且多为女性[3][4]，续发性雷诺氏症候群则较常发生于高龄人口[4]。在寒冷气候下，两种雷诺氏症候群发生率都较高[4]。此症候群是由法国的雷诺医师（Maurice Raynaud）在1862年所描述，并以他的名字命名[3]。

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Fast facts

Raynaud's phenomenon is when the blood flow to the fingers is limited, usually because of cold conditions or stress.
It is most common in young women.
It can occur as an independent condition (primary Raynaud's) or as a complication of an existing medical condition (secondary Raynaud's).
Prevention is the best tool for treating Raynaud's phenomenon. It is best that you identify what triggers your symptoms and take care to avoid these triggers.

What is Raynaud's phenomenon?

The body's normal response to cold exposure is to reduce the blood flow to the extremities. Raynaud's phenomenon is a disorder in which the body over-reacts to cold conditions. Small muscles in the arteries that supply the fingers and other extremities to suddenly constrict. This temporarily cuts off the blood supply to these areas.

In some people, Raynaud's can also be caused by stress or emotional upset.

Causes

In most cases, Raynaud's phenomenon has no underlying cause. This is also known as primary Raynaud's disease.

When it occurs because of an underlying medical condition, this is known as secondary Raynaud's phenomenon. This is more likely to develop after 30 years of age, and can be more serious.

A wide range of factors have been linked to an increased risk of Raynaud's phenomenon.

Underlying medical conditions can include:

Autoimmune conditions such as lupus, scleroderma, rheumatoid arthritis and Sjogren's syndrome;
Diseases of the blood vessels such as atherosclerosis, vasculitis, Buerger's disease and primary pulmonary hypertension;
Migraine;
Carpal tunnel syndrome;
Hypothyroidism;
Some cancers, such as ovarian cancer, and;
Some infections, such as hepatitis B and hepatitis C.

Injuries that can increase the risk include:

Overuse injuries from activities such as typing, piano playing or using crutches;
Previous injuries, such as a fracture, surgery or frostbite, and;
Industrial injuries due to the use of heavy, vibrating machinery such as chainsaws and drills.

Medications and substances that can increase the risk include:

Antihypertensive medications such as beta-blockers (including metaprolol and propanol);
Chemicals such as nicotine in cigarettes;
Some migraine medications such as ergotamine and sumatriptan;
Medications for attention deficit hyperactivity disorder (ADHD), such as methylphenidate;
Some nasal decongestants such as ephedrine and phenylephrine;
The combined oral contraceptive pill and estrogen replacement therapy, and;
Some chemotherapy medications such as bleomycin.

Risk factors

For primary Raynaud's disease, risk factors include:

Being aged between 15-30 years old, although Raynaud's can develop at any point in life;
Being female - women are at an increased risk;
Cold, wet weather and climate, and;
Having a family history of primary Raynaud's disease.

For secondary Raynaud's disease, risk factors include:

Having an underlying medical condition associated with Raynaud's disease;
Smoking;
Some medications (as noted above in 'Causes'), and;
Using heavy vibrating equipment.

Signs and symptoms

Most commonly, Raynaud's phenomenon affects the fingers, with symptoms usually starting in one finger before appearing in the others.

Other parts of the body that are more rarely affected include the toes, ears, nose, nipples, face, lips and tongue.

The skin of the affected areas generally changes color over the course of an 'attack' of Raynaud's phenomenon. Initially, the area goes white as blood flow reduces to the area and then the skin can turn blue or purple. Finally, as blood flow returns, usually after 15-20 minutes, the skin becomes red.

As blood flow returns, other symptoms in the affected areas include:

Discomfort or pain, which can be severe;
Sensations such as tingling or pins and needles, and;
Numbness.

The fingers are commonly affected by Raynaud's phenomenon.

Methods for diagnosis

To diagnose Raynaud's phenomenon, your doctor will perform a physical examination and ask you questions about your symptoms.

It is important to rule out other causes for the symptoms and identify any underlying causes for Raynaud's phenomenon, and tests may be recommended for this reason.

Tests that can help to identify autoimmune disorders include:

An antinuclear antibody test, and;
An erythrocyte sedimentation rate test.

A blood test can be used to identify if an underlying medical condition is causing Raynaud's phenomenon.

Types of treatment

The most effective way to manage Raynaud's phenomenon is to take steps to prevent attacks from occurring.

Learning what triggers your attacks is a good first step. For some people, an attack may occur only during cold weather, while for others it may be exposure to relatively small changes in temperature, such as when entering an air-conditioned building when it is hot outside, or handling frozen items out of the freezer.

Steps to avoid triggers include:

Wearing warm clothing, particularly gloves, mittens and thick socks;
Avoiding situations where temperatures change rapidly;
Taking care when handling cold items;
Managing stress and emotional upset;
Avoiding medications that can trigger an attack;
Avoiding cigarette smoke (both through smoking and passive exposure to smoke), and;
Avoiding activities that can damage the fingers and trigger attacks.

The link between caffeine and Raynaud's phenomenon is not clear. Some experts recommend avoiding coffee, while others do not. If you find that drinking coffee appears to trigger attacks, you may wish to avoid it.

Wearing warm clothing can help to avoid attacks of Raynaud's phenomenon.

Managing Raynaud's attacks

To encourage the blood vessels to open up and blood flow to return to the affected areas, it is best to try to warm them gradually.

You can:

Bathe them in warm water;
Use body heat (such as putting an affected hand in your armpit), and;
Rotate your arms like windmills, or rub your hands together to help encourage blood flow.

Medications

If the symptoms of Raynaud's phenomenon cannot be managed well with the measures above, medications may be recommended.

The most common medications are calcium-channel blockers, such as nifedipine or amlodipine, which help to relax the muscle cells in arteries and open them up.

If these are not effective, other medications may be substituted, or added to help open up the arteries. These can include:

Sildenafil, known commonly as Viagra® or by other trade names;
Nitroglycerin, applied to the skin as an ointment or gel, and;
Losartan, a particular blood pressure medication.

Stress management

It is not clear whether psychological treatment is effective for treating Raynaud's attacks. If stress or emotional upset trigger Raynaud's attacks, counselling or using relaxation techniques may be helpful.

More intensive treatment

For people with severe symptoms, or complications such as ulcers, more intensive treatment may be required. This usually only occurs in people who have serious underlying medical conditions such as scleroderma.

More intensive treatments include:

Intravenous (IV) infusions of a prostanoid such as iloprost;
Sympathectomy - blocking of the nerves that can stimulate a Raynaud's attack with injections such as botulinum toxin (Botox®), or surgery, and;
Other medications such as anticoagulants and antibiotics, if necessary.

Complementary and alternative medicines

A range of complementary therapies such as acupuncture, biofeedback, wearing therapeutic gloves, or various herbs such as Ginkgo biloba, have been suggested for treating Raynaud's phenomenon, but there is currently not enough evidence to support the effectiveness of any of these therapies.

Potential complications

Primary Raynaud's disease is normally a minor complaint; complications are extremely rare.

In severe secondary Raynaud's disease, if blood flow cannot be restored to the affected area (usually the fingers or toes) this can lead to ulceration and gangrene.

If this cannot be managed with more intensive treatments, amputation of the affected area may be necessary.

Prognosis

Most people who have Raynaud's attacks have the less serious primary form that can be managed well by avoiding triggers. In some cases, the symptoms will improve over time, with women often finding that symptoms noticeably reduce with menopause. However, some people have lifelong symptoms.

Secondary Raynaud's disease can be much more serious and may require more intensive treatment. It can lead to ulceration and, in very severe cases, may require amputation.