硬皮病及其在不同人群中遗传因素的差异

硬皮病是一种复杂的自身免疫性疾病,其主要特征有微血管受损和组织器官纤维化,根据其发病累及范围不同,主要分为系统性硬皮病和局限性硬皮病两种类型.硬皮病发病症状复杂,发病机制不明,导致治疗效果不甚理想.硬皮病发病有家族聚集现象及种族差异,表明硬皮病具有遗传易感性.近年来许多研究集中于寻找硬皮病发病的遗传因素,并已发现有多种...     

硬皮病患者消化系统动力学的改变

硬皮病患者消化系统动力学的改变中国医科大学第一临床学院风湿免疫科（１１０００１）王晓菲硬皮病是一种以局限性或弥漫性皮肤增厚和纤维化为特点的可累及多脏器的一种结缔组织病。可分为局限性硬皮病和系统性硬化症。后者多伴有多脏器受累，消化系统受累是其主要临床表...     

骨桥蛋白在硬皮病患者皮损中的表达和意义

目的:探讨骨桥蛋白(OPN)在硬皮病患者局部皮损表达和分布情况。方法:采用免疫组化SABC法分别检测硬皮病皮损20例(系统性硬皮病12例,局限性硬皮病8例)和正常皮肤10例中OPN蛋白的表达情况。结果:OPN蛋白在硬皮病患者皮损真皮层的成纤维细胞中表达明显增高,而正常对照组仅少数呈微弱表达(P<0.01),其在系统性硬皮病患者和局限性硬皮病患者之间表达无明显差异(P>0.05)。结论:OPN蛋白在硬皮病患者皮损成纤维细胞内明显高表达,提示OPN可能参与了硬皮病的发病机制,对纤维化的形成发挥一定的作用,并对局限性和系统性硬皮病有相似的作用机制。     

系统性硬皮病19例临床分析

对既往收住院的19例系统性硬皮病患者的病例进行分析。伴有关节痛47.4%，食道症状76.9%。肺部损害55.6%，心脏损害53.3%，肾损害33.3%；发现有5例弥漫性硬皮病发病始于四肢，提示一部分弥漫性硬皮病是由肢端硬皮病演变而来，治疗上加用皮质类固醇激素有利于症状的改善。     

局灶性硬皮病中医药治疗现状

局灶性硬皮病是以皮肤和皮下组织局限性炎症和纤维化,最后发生萎缩为特点的一类疾病。中医将其归属于"皮痹"范畴,中医药在局灶性硬皮病的治疗方面有着广阔的前景。该文总结了近十年来局灶性硬皮病相关文献报道,并对其病因病机、临床治疗及实验研究进行综述,希望对硬皮病医疗工作者有所裨益。     

创造新思维软化硬皮病--记河北省中医肌萎缩治疗中心张庆昌主任医师

硬皮病被人们称作“把人变成石头的疾病”。由于其病因还不十分清楚,临床表现多种多样,人们对这种病允满了神秘感,硬皮病患者更是感到恐惧、紧张。硬皮病     

硬皮病与恶性肿瘤

报告５例硬皮病伴发恶性肿瘤。２例多发性骨髓瘤发生在患硬皮病后半年，分别以血尿和蛋白尿尿毒症为突出表现。２例肺癌及１例胃癌发生于硬皮病后１０－１５年，胸部Ｘ线均有肯定的肺间质纤维化。结合文献对硬皮病伴发恶性肿瘤的发病概况、机理进行讨论。     

系统性硬皮病心电图,心脏超声检查130例分析

目的：探讨无明显心脏病症状的系统性硬皮病患者心脏受损情况。方法：对无明显心脏症状的系统性硬皮病患者行心电图和（或）心脏超声检查，并与局限性硬皮病患者作对照。结果：１３０例心电图和（或）心脏超声检查的异常率为５２．３％，对照组２６例异常率为２６．９％，两组差异有显著性（Ｐ＜０．０５）。结论：无明显心脏病症状的系统性硬皮病患者有较高的心脏病变发生率。肢端硬皮病和弥漫性硬皮病两型的心脏病变发生率无明显差异     

硬皮病伴发MPO-ANCA抗体阳性的Ⅲ型肾小球肾炎

硬皮病是一种自身免疫性疾病，其特征为早期炎症性浸润，进而发展为皮肤及内脏纤维化。CREST是硬皮病的一种相对良性皮肤变异，特点为钙质沉着、雷诺现象、食管功能异常、指端硬化和毛细血管扩张。CREST很少伴发肾小球肾炎。作者报道1例CREST患者发生肾小球肾炎，并在其血清中检出抗着丝点抗体和抗中性粒细胞胞浆抗体（ANCA）。     

食道硬皮病的临床病理及X线50例分析

食道硬皮病的临床病理及Ｘ线５０例分析兰医二院放射科周俊林硬皮病大致可分为两类，一类是局限于皮肤，另一类兼及内脏。其病变特点是结缔组织发生弥漫性纤维化，多先在皮肤，继而累及内脏，特别是食道，因其缓慢进展，故称进行性系统性硬化症（ＰＳＳ）［１］。而有些病...     

硬皮病中西医治疗的研究进展

综述近年来硬皮病的发病机制与中西医治疗方法.硬皮病是一种主要累及皮肤、黏膜组织并以纤维化和硬化为主要特征的结缔组织疾病,自身免疫改变和纤维化是该病的突出特征,其发病机制涉及血管异常、结缔组织异常、免疫改变等.西医治疗硬皮病通常采用药物硝苯地平、泼尼松、波生坦、青霉胺、秋水仙碱、伊洛前列腺素、环磷酰胺、霉酚酸酯等,同时结...     

Cutaneous scleroderma in association with carcinoid syndrome.

A case of scleroderma in a woman with carcinoid syndrome is described and the similarities between our case and those in the literature are reviewed. The carcinoid tumours were all of midgut origin and liver metastases were present. All subsequently developed fibrotic heart disease and none had clinical features or autoantibodies suggestive of systemic sclerosis. The association between carcinoid syndrome and particular features of scleroderma is likely to be more than fortuitous.     

复方丹参联合维生素E治疗新生儿硬肿症的疗效观察

目的：探讨复方丹参联合维生素E治疗新生儿硬肿症的疗效。方法：将60例新生儿硬肿症患儿随机分为治疗组和对照组,两组均给予常规综合治疗,治疗组在常规治疗基础上加用复方丹参和维生素E,对两组疗效进行比较。结果：治疗组反应好转时间、硬肿开始消退时间、硬肿完全消退时间、体温恢复时间、住院时间明显短于对照组,治疗组疗效较优。结论：复方丹参联合维生素E治疗新生儿硬肿症可以提高疗效。     

58例新生儿硬肿症病因探讨

作者对５８例新生儿硬肿症的病因进行了分析，结果表明寒冷地区（乌鲁木齐地区）硬肿症的发生率较温暖地区高，寒冷和感染是诱发本病的的主要原因；低体温与预后呈正相关，肺出血是死亡的主要因素；血气分析可作为判断硬肿症预后的依据。     

云数据库在皮肤病患者临床信息管理中的应用

目的探讨在皮肤科临床工作中建立云数据库的必要性,总结使用云数据库进行数据录入、处理及分析等实际应用经验,并展望皮肤病相关领域的数据管理前景。方法根据临床需求建立以系统性硬皮病和局限性硬皮病为范例的新型网络数据库—云数据库,通过纸质表格搜集患者个人情况、图片资料、标本信息、血生化指标、皮肤受累情况及自评量表计分等,进行云数据库的数据录入、分析、计算;在此过程中探讨并总结云数据库在皮肤科患者信息管理中的应用。结果使用云数据库实际收录并处理了215例系统性硬皮病及522例局限性硬皮病患者个人资料及临床信息,通过统计计算取得多项关于疾病状况、生活质量、预后等的结论性成果。结论云数据库可以快捷有效地存储并处理皮肤病患者的相关数据、图片等,导出与计算快捷灵活,使用方便,且安全性上佳,其实际应用对患者信息管理、临床决策及医学科学研究具有重要意义。     

Scleroderma and repeated spontaneous abortions treated with vitamin E--a case report--

A 33-year-old woman was referred to our hospital due to repeated spontaneous abortions and positive autoantibodies. She had noticed Raynaud's phenomenon 13 years earlier. We diagnosed scleroderma based on the presence of Raynaud's phenomenon, proximal scleroderma, presence of anti-centromere antibodies, and histological findings on skin biopsy. Neither lupus anticoagulant nor anti-cardiolipin-beta2-glycoprotein 1 antibody was detected. We administered tocopherol nicotinate. Five months after the initiation of the treatment, she became pregnant and later delivered a healthy baby.     

Asymptomatic autoimmune liver disease in scleroderma

A case is described of scleroderma with asymptomatic autoimmune liver disease. The patient presented with the features of CRST syndrome (calcinosis, Raynaud''s phenomenon, sclerodactyly and telangiectasia) and a raised plasma alkaline phosphatase was found on routine investigation. It is suggested that this latter biochemical finding is likely to indicate associated autoimmune liver disease in patients with scleroderma.     

Role of cold and emotional stress in Raynaud's disease and scleroderma

Research on the aetiology of Raynaud's disease and phenomenon has been hindered by the difficulty of provoking attacks in the laboratory. A study was therefore conducted in which digital and ambient temperatures, electrocardiograms, and stress ratings were obtained during ambulatory monitoring in patients with idiopathic Raynaud's disease, Raynaud's phenomenon secondary to scleroderma, and in normal subjects. In Raynaud's disease about one third of the vasospastic attacks were associated with tachycardia and increased stress ratings without declines in ambient temperature. In contrast, cold alone was enough to provoke most attacks of Raynaud's phenomenon in scleroderma. Chronically increased stress ratings in patients with scleroderma and increased muscle tension in anticipation of a cold stimulus suggest that these patients have different patterns of stress responses from those with Raynaud's disease.     

SCLEROTIC FASCIITIS A CLINICAL AND PATHOLOGIC ANALYSIS OF 21 CASES

This paper reports 21 cases of eosinophilic fas- ciitis and emphasizes that localized inflammatory and sclerotic changes in the deep tissues characte rize this disease. We propose the name sclerotic fas- ciitis for this condition to reflect its specific patho- logic changes. This disease is an especially deeply situated type of scleroderma, somewhat similar to localized scleroderma. The ultrastructural study of myofibroblasts in this condition indicates that it may be closely related to proliferation of collagen fibers in the fascia. Patients were treated with traditional medicinal herbs in accordance with the traditionaJ Chinese medical principle of stimulating blood circulation to end blood stasis. Therapeutic results were satisfactory.