硬皮病分型 (Types of Scleroderma, Chinese Version)

硬皮病分型
Types of Scleroderma: Overview

This page was written by Shelley Ensz, translated into Simplified Chinese by Jie Jackson, who is a medical student in China.

针对硬皮病的不同的类型有多种方法来进行分类，由此产生的大量的医学术语很容易让人感到困惑。
下列分类常出现在文献中，但它们的使用和翻译又有不同。

There are many methods used to categorize the various forms of scleroderma, which has brought about a confusing abundance of terms. The following categories are often found in patient literature, but use and interpretation of these categories varies quite a bit.

两种主要类型
Two Main Types
局限性硬皮病
Localized Scleroderma
系统性硬皮病
Systemic
预后
Prognosis
肢端硬化综合症
CREST Syndrome

限制性硬皮病
Limited Scleroderma
弥漫性硬皮病
Diffuse Scleroderma
重叠综合症
Overlap Syndrome
青少年硬皮病
Juvenile Scleroderma

两种主要类型
Two Main Types

硬皮病有两种主要的类型：局限性硬皮病和系统性硬皮病。
There are two maintypes of Scleroderma: Localized and Systemic.

如果儿童患有硬皮病，不管是何种类型，都叫青少年硬皮病，但以局限性硬皮病最常见。
When scleroderma of any type afflicts children, it is called Juvenile Scleroderma. The localized forms of scleroderma (such as Linear and Morphea) are most common in children.

青少年硬皮病是一种少见的小儿疾病。
Scleroderma in children Juvenile systemic scleroderma (jSSc) is a rare childhood disease. Curr Opin Rheumatol 2002 Nov;14(6):699-703.

局限性硬皮病
Localized

局限性硬皮病有两种类型：线形硬皮病和硬斑病。
局限性硬皮病主要是影响到表面皮肤，也可能影响到皮下的肌肉和骨骼。但它不会影响到内部的脏器。
There are two types of Localized Scleroderma: Linear andMorphea . LocalizedScleroderma affects the skin. It may also affect the underlying muscles and bones, but it does not affect internal organs.

总的说来，局限性硬皮病病情相对缓和。相对于系统性硬皮病，
局限性硬皮病只有一些表面的症状，有的只有作皮肤活检并且在显微镜下才能发现。
In general, Localized Scleroderma is relatively mild, and may be related to Systemic Scleroderma only in terms of similar superficial symptoms, such as the appearance of skin biopsy under the microscope.

局限性硬皮病有很大的范围，轻者不严重，重者可致畸。
Localized Scleroderma There is a wide spectrum of localized scleroderma, with types ranging from mild to disabling. eMedicine Dermatology

系统性硬皮病
Systemic

系统性硬皮病有几种类型：CREST综合症，限制性硬皮病和弥漫性硬皮病。
系统性硬皮病也看作系统性硬化症。它也被称进行性系统性硬化症，或家族进行性系统性硬化症。
There are several types of Systemic Scleroderma: CREST, Limited and Diffuse. Systemic Scleroderma is also known as Systemic Sclerosis. It may also be referred to as Progressive Systemic Sclerosis, or Familial Progressive Systemic Sclerosis.

系统性硬皮病影响皮肤、血管和（或）内部脏器。
它主要影响手和（或）脸的皮肤，导致皮肤硬化。影响血管时，
能导致雷诺氏综合症。当它侵犯内脏时，可以导致残废甚至死亡。
Systemic Scleroderma may affect the skin, blood vessels, and/or internal organs. When it affects the skin, it can cause the skin —most commonly on the hands and/or face —to harden. With the blood vessels, it can cause Raynaud's. When it affects the internal organs, it may cause disability or even death.

怎样认识硬皮病。系统性硬皮病是多系统功能失常的疾病，以结缔体素硬化，
动脉和微循环变小的血管异常和自身免疫异常为主要特征。
许多其他的疾病也可以有类似硬皮病的症状。但在这些疾病中甲壁异常，
内脏受影响和自身免疫系统的改变不同于系统性硬皮病的表现。
PubMed. Rev Prat. 2002 Nov 1;52(17):1884-90.
How to recognize scleroderma. Systemic sclerosis is a multisystem disorder characterized by the presence of connective tissue sclerosis, vascular abnormalities concerning small-sized arteries and the microcirculation, and autoimmune changes. Many other diseases may have a scleroderma-like appearance. In these diseases, nail folds abnormalities, visceral involvement and immunological changes are usually different from those observed in systemic scleroderma. PubMed. Rev Prat. 2002 Nov 1;52(17):1884-90.

系统性硬皮病的评估。系统性硬皮病在影响皮肤、血管、内脏的范围程度上有很多的不同，
疾病的发展和预后也存在很多的变性。 G. Valentini. SP0139 EULAR 2003
Assessment of Systemic Sclerosis. Systemic Sclerosis (SSc) presents a great deal of variability in the extent of skin,vascular and internal organ involvement, the pace of the disease and the prognosis. G. Valentini. SP0139 EULAR 2003

系统性硬皮病是多系统的受累的疾病，以结缔组织的硬化、
动脉和微循环的变小相关的血管异常、免疫系统的改变为特征。
在硬斑病，只有皮肤的局部受累，影响美观。 PubMed. Rev Prat 2002 Nov 1;52(17):1884-90
Systemic sclerosis is a multisystem disorder characterized by the presence of connective tissue sclerosis, vascular abnormalities concerning small-sized arteries and the microcirculation, and autoimmune changes. In Morphea, there is only localized sclerosis of the skin with highly variable aesthetic disability.PubMed. Rev Prat 2002 Nov 1;52(17):1884-90

硬皮病的视网膜变化：甲壁毛细血管镜比较
系统性硬皮病的患者常常可以看到视网膜的异常，它们也反映了系统性硬皮病的典型的血管变化
但是，视网膜的变化同甲壁毛细血管的变化有本质的区别。PubMed. Ann Rheum Dis 2003 Mar;62(3):204-7
Retinal findings in systemic sclerosis: a comparison with nailfold capillaroscopic patterns. Retinal abnormalities are often seen in patients with SSc and they may reflect the vascular changes characteristic of SSc. However, retinal changes may differ in quality from the changes of nailfold capillaries. PubMed. Ann Rheum Dis 2003 Mar;62(3):204-7

系统性硬皮病中颈动脉和大腿动脉壁：在系统性硬皮病中颈动脉的弹性出现显著变化
系统性硬皮病的两个主要分型可以通过这进行区分。
这揭示了结缔体素可以在异常地出现在开始未评估的部位
PubMed. Rheumatology (Oxford). 2003 May 30
Carotid and femoral arterial wall mechanics in scleroderma. The elastic properties of the carotid artery are significantly altered in SSc, and the two major subsets of SSc may be distinguished by their carotid artery biomechanics. This suggests that connective tissue abnormality occurs at sites not previously assessed. PubMed. Rheumatology (Oxford). 2003 May 30

预后
Prognosis

以前，只有最严重的硬皮病患者得到诊断，这就导致了广泛的认识，
系统性硬皮病是不断进展的并且是致命的。
现在所有类型的硬皮病都可得到认识和诊断，就增加了另外一种意识，
这种有许多病情发展缓和的类型，预后是长期的。
Years ago, only the worst cases of scleroderma were diagnosed, thus leading to the widespread belief that systemic scleroderma was always progressive and fatal. Now that the full range of types and severity of scleroderma are being recognized and diagnosed, there is increasing awareness that many people have a milder type of illness with a much rosier longterm prognosis.

硬皮病中有三种典型的自我功能对照指标。
A cross-sectional comparison of three self-reported functional indices in scleroderma. This study compares the Disability Index of the Health Assessment Questionnaire (HAQ-DI) with two more recently developed scleroderma-specific tools: scleroderma-visual analogue scales. PubMed. Rheumatology (Oxford) 2003 Apr 16;

CREST综合症
CREST Syndrome

CREST综合症没有皮肤硬化的症状，它是以钙质沉淀、雷诺现象、食管硬化、
指端硬化和毛细血管扩张为特征。CREST综合症可单独出现，也可以伴随其他类型的硬皮病（或其他的免疫系统疾病）出现，
象CREST并限制性硬皮病，CREST并红斑狼疮。
CREST Syndrome, by itself, does not have any skin tightening at all. CREST stands for Calcinosis, Raynaud's, Esophagus, Sclerodactyly, and Telangiectasia. CREST may occur alone, or in combination with any other form of Scleroderma (or even other autoimmune diseases) such as CREST with Limited Scleroderma, or CREST with Lupus. See CREST.

限制性硬皮病
Limited Scleroderma

限制性硬皮病是只连累手部皮肤的硬皮病，尽管有时会牵涉到脸部和颈部。
Limited Scleroderma is when skin involvement is limited to the hands (although the face and neck may also be involved.) See Limited Scleroderma.

弥漫性硬皮病
Diffuse Scleroderma

弥漫性硬皮病是皮肤硬化发生在腕关节以上部位（或肘）。弥漫性硬皮病又可以分，
象Scleroderma sans Scleroderma（它只是内脏硬化，皮肤无影响）和
Familial Progressive Systemic Sclerosis（一种少见的只在家族中出现的疾病）。
Diffuse Scleroderma is when skin tightening also occurs above the wrists (or elbows, see below). There are several subcategories of Diffuse, such as Scleroderma sans Scleroderma where there is internal organ fibrosis, but no skin tightening; and Familial Progressive Systemic Sclerosis, a rare form which runs in families. See Diffuse Scleroderma.

重叠综合症
Overlap Syndrome

如果硬皮病患者还患有其他的免疫系统性疾病，如红斑狼疮，风湿性关节炎等，
就可称为重叠综合症。硬皮病的症状可见于混合性结缔组织病中（MCTD）
或Undifferentiated Connective Tissue Disease (UCTD)中。
If a Scleroderma patient also has any other autoimmune disease (such as lupus, rheumatoid arthritis, etc.) it is referred to as overlap; as in "Diffuse Scleroderma in overlap with lupus." Scleroderma symptoms can also be a part of Mixed Connective Tissue Disease (MCTD), or Undifferentiated Connective Tissue Disease (UCTD). See Scleroderma in Overlap.

See Also

Juvenile Scleroderma by ISN.

Scleroderma Patient and Caregiver Stories by ISN.

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