多发性硬化症和全身性硬化症之间的差异

关键区别 - 多发性硬化与全身性硬化症

Both multiple sclerosis and systemic sclerosis are自身免疫性疾病其发病机理是由未发现的环境和遗传因素触发的。多发性硬化症是一种慢性自身免疫性，T-cellmediated inflammatory disease affecting thecentral nervous systemwhereas systemic sclerosis, also known asscleroderma, is an autoimmune multisystem disease with a progressively worsening clinical picture. The key difference between multiple sclerosis and systemic sclerosis is that多发性硬化症仅影响中枢神经系统，但全身性硬化是一种多系统疾病，几乎影响体内所有系统。

内容

1.Overview and Key Difference
2.什么是多发性硬化症
3.What is Systemic Sclerosis (Scleroderma)
4.多发性硬化症和全身性硬化症之间的相似之处
5.Side by Side Comparison – Multiple Sclerosis vs Systemic Sclerosis in Tabular Form
6.概括

什么是多发性硬化症?

多发性硬化症是一种慢性自身免疫性，T-cell mediated inflammatory disease affecting the central nervous system. Multiple areas of demyelination are found in the brain and the spinal cord. The incidence of MS is higher among women. MS mostly occurs between 20 and 40 years of age. The prevalence of the disease varies according to the geographical region and ethnic background. The patients with MS are susceptible to other autoimmune disorders. Both genetic and environmental factors influence the pathogenesis of the disease. Three commonest presentations of MS are optic neuropathy, brain stem demyelination, and spinal cord lesions.

Pathogenesis

T细胞介导的炎症过程主要发生在white matterof the brain and spinal cord producing plaques of demyelination. 2-10mm sized plaques are usually found in the optic nerves, periventricular region, corpus callosum,brain stem及其小脑连接和颈绳。

In MS, peripheral myelinated nerves are not directly affected. In the severe form of the disease, permanent axonal destruction occurs resulting in progressive disability.

Types of Multiple Sclerosis

• 复发复发MS
• 次要进步MS
• 主要进行性MS
• Relapsing-progressive MS

Common Signs and Symptoms

• Pain on eye movements
• 中央视觉/颜色去饱和/密集的中央Scotoma的轻度雾气
• Reduced vibration sensation and proprioception in feet
• Clumsy hand or limb
• 走路不稳定
• 尿紧迫性和频率
• Neuropathic pain
• 疲劳
• Spasticity
• Depression
• Sexual dysfunction
• 温度灵敏度

In late MS, severe debilitating symptoms with optic atrophy, nystagmus, spastic tetraparesis, ataxia, brainstem signs, pseudobulbar palsy, urinary incontinence and cognitive impairment can be seen.

Diagnosis

Diagnosis of MS can be made if the patient has had 2 or more attacks affecting different parts of the CNS. MRI is the standard investigation used for the confirmation of clinical diagnosis. CT and CSF examination can be done to provide further supportive evidence for the diagnosis if necessary.

管理

MS没有明确的治疗方法。But several immunomodulatory drugs have been introduced to modify the course of the inflammatory relapsing-remitting phase of MS. These are known as Disease Modifying Drugs (DMDs). Beta-interferon and glatiramer acetate are examples of such drugs. Apart from drug therapy, general measures such as physiotherapy, supporting the patient with the help of a multidisciplinary team and occupational therapy can vastly improve the living standards of the patient.

预后

The prognosis of multiple sclerosis varies in an unpredictable manner. A high MR lesion load at the initial presentation, high relapse rate, male gender and late presentation are usually associated with a poor prognosis. Some patients continue to live a normal life with no apparent disabilities while some can get severely disabled.

What is Systemic Sclerosis?

系统性硬化症，这也已知scleroderma。是一种自身免疫性多系统疾病，具有逐渐恶化的临床情况。

Risk Factors

Exposure to

• Vinyl chloride
• Silica dust
• 掺假的菜籽油
• 三氯乙烯

Clinical Features

• Tight skin over face, small mouth, beak nose and telangiectasia
• Dysmotility or strictures of食管
• Myocardial fibrosis
• 硬皮病和肾脏危机
• Pulmonary hypertension orpulmonary fibrosis
• 肠道和粪便失禁的吸收不良，缺失
• Raynaud’s phenomenon

调查

• Full blood count – a full blood count usually reveals the presence of a normochromic, normocytic anemia.
• 尿素和电解质 - 在硬皮病相关肾脏损伤中可以升高尿素和电解质水平
• Testing for the antibodies such as ACA and ANA that are typically seen in systemic sclerosis
• Urine microscopy
• Imaging
• 胸部X射线 - 可用于排除可能具有类似临床情况的任何其他病理状况。除此之外，这可能有助于识别任何异常心脏或肺部变化。
• X-ray of a hand – this can show the deposits of calcium that have accumulated around the bones of fingers.
• GI endoscopy can reveal any abnormalities in the esophagus
• High-resolution CT can be done to identify any fibrotic lung involvement

管理

没有明确治疗系统性硬化症。相当令人惊讶的是，已证明皮质类固醇和免疫抑制剂在这种情况下的有效性较差。特定于器官治疗该疾病的方法可用于避免与全身性硬化症相关的长期并发症。

• Patient counseling and family support are extremely important
• The use of skin lubricants and skin exercises can limit the development of contractures
• Hand warmers and oral vasodilators can be used to control Raynaud’s effect
• Proton pump inhibitors are usually prescribed to alleviate the esophageal symptoms
• ACE inhibitors are the drug of choice in the management of renal impairment
• Pulmonary hypertension should be treated with oral vasodilators, oxygen, and warfarin

预后

The mild form of the disease has a better prognosis than the diffuse disease. Extensive pulmonary fibrosis is usually the leading cause of death among the scleroderma patients.

What are the Similarities Between Multiple Sclerosis and Systemic Sclerosis?

• Both multiple sclerosis and systemic sclerosis are autoimmune diseases.
• 两种疾病都没有确定的治愈方法。

What is the Difference Between Multiple Sclerosis and Systemic Sclerosis?

多发性硬化与全身性硬化症
多发性硬化症是一种慢性自身免疫性，T-cell mediated inflammatory disease affecting the central nervous system.	全身性硬化症（硬皮病）是一种自身免疫性多系统疾病，临床状况逐渐恶化。
Risk Factors
Female gender and genetic predisposition are the major known factors.	Exposure to Vinyl chloride, silica dust, adulterated rapeseed oil, and trichloroethylene are the risk factors.
Clinical Features
The early disease is characterized by the following clinical features. · Pain on eye movements · Mild fogging of central vision/color desaturation/dense central scotoma · Reduced vibration sensation and proprioception in feet 笨拙的手或林b ·走路不稳定 · Urinary urgency and frequency · Neuropathic pain · Fatigue ·痉挛 · Depression · Sexual dysfunction ·温度灵敏度 In late MS, severe debilitating symptoms can be seen with optic atrophy nystagmus 痉挛性四脂 ataxia brainstem signs pseudobulbar palsy 尿失禁 认知障碍	The thickening of the skin in different regions of the body is the most prominent clinical sign. In addition to that, the following features can also be frequently seen. · Tight skin over face, small mouth, beak nose and telangiectasia ·食道的运动障碍或狭窄 · Myocardial fibrosis ·硬皮病和肾脏危机 · Pulmonary hypertension or pulmonary fibrosis · Malabsorption, hypomotility of intestine and fecal incontinence ·雷诺的现象
Diagnosis
A diagnosis of MS can be made if the patient has had 2 or more attacks affecting different parts of the CNS. MRI is the standard investigation used for the confirmation of clinical diagnosis. 可以进行CT和CSF检查，以提供进一步的支持证据，以便在必要时进行诊断。	进行以下研究是为了诊断多发性硬化症以及疾病进展的评估。 · Full blood count ·尿素和电解质 ·测试全身性硬化症通常可见的抗体（例如ACA和ANA） · Urine microscopy ·胸部X射线 · X-ray of a hand ·GI内窥镜检查可以揭示食道的任何异常 ·可以进行高分辨率CT以识别任何纤维化肺部受累
治疗
MS没有明确的治疗方法。 Several immunomodulatory drugs known as Disease Modifying Drugs (DMDs), have been introduced to modify the course of the inflammatory relapsing-remitting phase of MS. Beta-interferon and glatiramer acetate are examples of such drugs. General measures such as physiotherapy, supporting the patient with the help of a multidisciplinary team and occupational therapy can vastly improve the living standards of the patient.	的管理nt aims at controlling the severity of the symptoms and arresting the disease progression. ·患者咨询和家庭支持非常重要 · The use of skin lubricants and skin exercises can limit the development of contractures · Hand warmers and oral vasodilators can be used to control Raynaud’s effect · Proton pump inhibitors are usually prescribed to alleviate the esophageal symptoms ·ACE抑制剂是肾功能障碍管理的首选药物 · Pulmonary hypertension should be treated with oral vasodilators, oxygen, and warfarin

概括– Multiple Sclerosis vs Systemic Sclerosis

多发性硬化症是一种慢性自身免疫性，T-cell mediated inflammatory disease affecting the central nervous system whereas systemic sclerosis which is also known scleroderma is an autoimmune multisystem disease with a progressively worsening clinical picture. The main difference between multiple sclerosis and systemic sclerosis is that multiple sclerosis only affects the central nervous system while systemic sclerosis extends its effect over almost all the systems of the body.

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References:

1. Kumar, Parveen J., and Michael L. Clark. Kumar & Clark clinical medicine. Edinburgh: W.B. Saunders, 2009. Print.

Image Courtesy:

1. “Multiple Sclerosis” By BruceBlaus – Own work(CC BY-SA 4.0)viaCommons Wikimedia
2. “Radiological evaluation through HRCT” By IPFeditor – Own work(CC BY-SA 3.0)viaCommons Wikimedia