造血干细胞移植治疗系统性硬化病的进展

系统性硬化病(SSc)是一种累及多系统的疾病,属于自身免疫性疾病,病因和发病机制尚不清楚,迄今尚无有效的方法能够阻止SSc疾病进展、逆转皮肤及内脏器官纤维化。传统治疗采用免疫抑制剂,虽然在早期能够改善病情,但对于进展性弥漫性的SSc疗效较差。自体造血干细胞移植术治疗重症SSc患者,Ⅰ和Ⅱ期临床研究证明其能够有效改善皮肤硬化、增厚,且能够稳定内脏器官的功能,延缓疾病的进展,防止复发,疗效较稳定。     

假性硬皮病伴发癌症

1994—2002年间,作者发现71例患者有硬皮病皮肤改变;其中66例诊断为系统性硬化(SSc),5例诊断为假性硬皮病伴发各种恶性疾病。这5例患者的平均病程明显较SSc患者短。抗核抗体阳性、雷诺现象或累及食管的频率显著比SSc患者低。皮肤硬化的分布部位因个体而异。这5例假性硬皮病患者     

系统性硬化病的临床研究

系统性硬化病(systemic sclerosis SSc)曾称硬皮病(scleroclerma)、进行性系统性硬化,是一种原因不明,临床上以局限性或弥漫性皮肤增厚和纤维化为特征,可累及心、肺、肾、消化道等脏器的结缔组织疾病。     

系统性硬化症中医药治疗研究进展

硬皮病又称系统性硬化症（SystemicSclerosis，SSc）是一种原因不明的局限性或弥漫性皮肤增厚为显著特征并以内脏器官结缔组织纤维化、硬化、萎缩为特点的自身免疫性疾病。可将其分为弥漫型、局限型、肢端型、CREST综合征、重叠综合征、混合性结缔组织病及无皮肤硬化的系统性硬化症。     

1例多形性红斑合并系统性硬化症患者的护理

<正>多形性红斑即SSc综合征,是一种少见的炎性反应性皮肤病,可致多个部位皮肤黏膜受累。硬化症俗称硬皮病,是一种以皮肤和内脏的小血管及广泛结缔组织纤维硬化和萎缩为     

114例系统性硬化病临床及免疫学特征分析

目的探讨系统性硬化病(SSc)患者的临床及免疫学特征。方法对宁夏医科大学总医院收治的114例SSc患者的临床资料进行回顾性分析。结果 114例SSc患者的首发症状主要为雷诺现象(44.74%)、皮肤硬肿(38.60%)。最常见的皮肤表现是皮肤硬肿(85.96%)和色素紊乱(23.68%)。累及内脏以肺、心、消化道损害最常见。ANA阳性88例(80%),核型以斑点型、着丝点型、核仁型为主,抗Scl-70抗体阳性29例(26.13%),抗U1RNP抗体阳性29例(26.13%)。结论早期进行自身抗体的检测及相关辅助检查可提高对SSc的诊断率及治疗水平。     

系统性硬化症并发系统性红斑狼疮10例

重叠综合征(OS)常指同一患者先后或同时出现两种或两种以上的鲒缔组织病。OS最常见的组合是系统性红斑狼疮(SLE)、多肌炎、与系统性硬化征(SSc)的两种或两种以上合并。SSc与SLE重叠综合征在国内仅有零星的个案报道。我院近10年共收治此类患者10例,现报告如下。     

系统性硬化症30例分析

系统性硬化症(systemic sclerosis,SSc),又称硬皮病,是一种以小动脉或微血管及广泛结缔组织硬化为特点的自身免疫性疾病,病变累及皮肤、肌肉、关节及内脏,特别是肺、消化系统、心脏、肾等组织,病因不明[1]。最主要的症状是雷诺现象和隐袭性肢端及面部肿胀,并有手指皮肤逐渐增厚,多关节病同样也是突出的早期症状。胃肠道紊乱或呼吸系统症状等,偶尔也是本病的首发表现。累及肺主要是肺纤维化和肺动脉血管病变。本病起病缓,女性多见,男女之比为1∶8。我们收集到资料完整的1例男性与29例女性SSc,对其临床表现及实验室检查作对比分析。现报道如下。     

系统性硬化症相关肺纤维化疾病的治疗现状

系统性硬化症(systemic sclerosis,SSc)是以皮肤和内脏纤维化为特点的结缔组织病,肺是最常受累的内脏器官之一,SSc相关的肺纤维化也是目前导致患者死亡的主要原因。目前,针对SSc肺纤维化的治疗仍是世界性难题,多中心研究表明,环磷酰胺(cyclophosphamide,CYC)是目前唯一对SSc肺纤维化患者有效的药物,但是由于长期应用副作用较大,尚缺乏长期的疗效判定,有待进一步进行验证。     

系统性硬化症发病机制的研究进展

系统性硬化症(SSc)是一种以影响皮肤和内脏为主的结缔组织病,其特点为血管和免疫功能紊乱引起纤维化,临床上可以观察到皮肤局限或弥漫性增厚变硬,并进一步损伤多个器官。其中,免疫系统的紊乱贯穿该病的全过程,在疾病的发生、发展中起重要作用。SSc的发病机制暂不明确,所以目前尚未有针对SSc的有效治疗方案。研究者们进行了大量的临床及基础实验,试图从遗传、环境、免疫细胞、细胞因子、胶原及血管功能紊乱等多个角度进行研究,以为未来新的治疗提供可能。     

系统性硬化患者发生重度肺动脉高压的危险因素分析

目的分析预测系统性硬化患者发生重度肺动脉高压（SPAH）的危险因素。方法收集146例系统性硬化患者的临床资料,包括起病年龄、性别、病程、雷诺现象（RP）、皮肤受累程度、胃食管反流、心脏损害、心功能不全、肾损害、肺动脉压、间质性肺炎、弥散功能、脑梗死、血清抗Scl-70抗体、抗RNP抗体和抗着丝点抗体情况。使用非参数检验方法比较SPAH组和无SPAH组之间临床指标的差异．并使用Cox回归方法分析患者发生SPAH的危险因素。结果SPAH组与无SPAH组患者相比,RP、男性患者、抗Scl-70、抗着丝点抗体、间质性肺炎、弥散功能下降、胃食管反流、冠心病、外周动脉硬化发生率均无显著性差异（P〉0．05）;SPAH组患者中起病年龄〉60岁者、弥漫型皮肤受累（DSSc）者、抗RNP抗体阳性者、心脏损害者、肾脏损害者、脑梗死者均多于无重度肺动脉高压者（分别为44．4％vs．12．5％、55．6％vs．21．9％、44．4％vs．15．6％、44．4％vs．14．1％、33．3％vs．9．4％、11．1％vs．1．6％）。Cox回归分析表明。发病年龄〉60岁（OR=7．091,95％可信区间2．633～19．095,P=0．000）、男性（OR=3．833,95％可信区间1．200～12．246．P=0．023）、RNP抗体阳性（OR=3．031,95％可信区间1．163～7．904,P=0．023）、DSSc（OR=2．635,95％可信区间1．031—6．737,P=0．043）、心脏受累（OR=3．284,95％可信区间1．261～8．553,P=0．015）、肾脏受累（OR=3．089,95％可信区间1．096-8．708,P=0．033）是系统性硬化患者发生SPAH的危险因素。结论对于发病年龄〉60岁、男性、DSSe、RNP抗体阳性、心脏受累、肾脏受累患者应早期监测其肺动脉压,以期早期检出肺动脉高压并积极治疗。     

Expression of MMP-9 and TIMP-1 in Lesions of Systemic Sclerosis and Its Implications

In order to investigate the role of MMP-9 and TIMP-1 in the pathogenesis of systemic sclerosis, the expression of MMP-9 and TIMP-1 was immunohistochemically detected in skin lesions of the patients with diffuse cutaneous systemic sclerosis, skin lesions of the patients with limited cutaneous systemic sclerosis, and skin tissues of normal subjects. The results showed that the expression of MMP-9 in lesions of diffuse cutaneous systemic sclerosis was significantly lower than that of normal skins （P〈0.05）. However, no significant difference in the level of MMP-9 in the limited cutaneous systemic sclerosis and normal skin was found. Meanwhile, the expression of TIMP-1 in lesions of diffuse cutaneous systemic sclerosis and limited cutaneous systemic sclerosis were significantly higher than that of normal skins （both P〈0.05）. It was suggested that the expression of MMP-9 and TIMP-1 might play an important role in the development of systemic sclerosis.     

从太阴论治系统性硬化症的探讨*

系统性硬化症是一种难治性结缔组织病，可归属于中医“皮痹”“肺痹”等范畴。本文基于太阴相关论述，结合系统性硬化症现代医学研究及患者临床表现，系统分析太阴与系统性硬化症的相关性，认为太阴之脏不足引发正虚邪凑，继而影响津液代谢，致使痰浊瘀血内生为该病发生发展的基本病机；治疗遣方用药从太阴入手，增健脾补肺、调和营卫、益气养血之剂多有效验。通过阐述从太阴论治系统性硬化症的理论依据及重要性，为临床提供思路借鉴。     

温阳通络方对硬皮病成纤维细胞Ⅰ、Ⅲ型胶原及MMP-1mRNA的影响

目的：观察温阳通络方对系统性硬皮病（SSc）患者皮肤成纤维细胞Ⅰ、Ⅲ型胶原mRNA及MMP-1mRNA的影响。方法：12例SSc患者分成SSc对照组（初诊未治疗者）、西药组、中药组和中西药联合组,每组3例。除对照组外,其他三组分别给予青霉胺和强的松、温阳通络方煎剂、青霉胺和强的松加温阳通络方煎剂口服,疗程2个月。SSc对照组在治疗前取血分离血清备用;西药组、中药组和中西药联合组治疗2个月后取血分离血清。另取正常及SSc患者皮肤进行成纤维细胞原代培养并以上述血清处理,正常对照组不予处理,然后提取总RNA,以设计的引物逆转录生成cDNA,然后PCR扩增、凝胶电泳后图像分析Ⅰ、Ⅲ型胶原及MMP-1mRNA表达。结果：与正常皮肤比较,SSc皮肤成纤维细胞Ⅰ、Ⅲ型胶原及MMP-1mRNA表达量明显增多（P〈0.01）;与SSc对照组比较,20%含药血清处理后各组Ⅰ、Ⅲ型胶原mRNA表达量明显减少（P均〈0.01）;中药组Ⅰ、Ⅲ型胶原mRNA表达量显著高于西药组和中西药组（P均〈0.01）,中西药组显著低于西药组（P〈0.01）;各组间SSc皮肤成纤维细胞MMP-1mRNA表达量差异无统计学意义（P〉0.05）。结论：SSc患者皮肤成纤维细胞胶原合成量增加,温阳通络方在一定程度上干扰SSc患者皮肤成纤维细胞I、III型胶原mRNA表达,而对MMP-1干预作用不明显。     

Treatment of scleroderma skin ulcers using becaplermin gel and hydrocolloid membrane

Skin ulcers can be very painful and detrimental in patients with systemic sclerosis, or systemic scleroderma. A brief review of scleroderma skin ulcers is presented, as well as a case study that demonstrates the effectiveness of becaplermin gel supplemented by oral immunosuppressive agents in the treatment of ulcers resulting from systemic sclerosis. The time to healing (approximately 3 months) was comparable to that associated with the oral agents and surgical interventions specifically designed to help heal scleroderma ulcers. Except for incisional biopsy, no surgical procedures were performed.     

Exaggerated radiation-induced fibrosis in patients with systemic sclerosis

Four patients with stable systemic sclerosis and limited skin involvement received radiation for the treatment of solid malignant neoplasms. Following localized irradiation, each patient developed an exaggerated cutaneous and internal fibrotic reaction in the irradiated areas. The surface area of fibrosis extended beyond the radiation portals employed, and the fibrotic process was poorly responsive to antifibrotic therapy. Three of the patients died of complications caused by fibrous encasement of internal organs. The extent and severity of postradiation fibrosis in these patients was distinctly unusual. These observations suggest that patients with systemic sclerosis are particularly susceptible to developing excessive radiation-induced fibrosis.     

Progressive systemic sclerosis in the elderly.

There is evidence to suggest that the incidence of progressive systemic sclerosis in the elderly is more common than in younger age groups. As in younger patients, late onset cases may have minimal skin changes and this can cause difficulty in diagnosis. The case histories of 2 patients are presented to illustrate these points.     

进行性系统性硬化症皮损中前胶原基因和SMAD mRNA的表达

目的探讨进行性系统性硬化症（PSS）硬化皮肤组织中Ⅰ型和Ⅲ型前胶原基因以及细胞内信号转导分子SMAD mRNA的表达情况,分析它们在发病机制中的作用。方法用荧光实时定量聚合酶链反应法分别检测11例PSS患者硬化皮损及11例正常对照皮肤组织中Ⅰ型和Ⅲ型前胶原基因以及不同类型SMAD（SMAD2、SMAD3、SMAD4、SMAD7）mRNA的表达水平。结果Ⅰ型前胶原基因α1、Ⅲ型前胶原基因α1、SMAD4及SMAD7的mRNA在硬化皮损中的表达明显高于在正常皮肤组织的表达,差异有统计学意义（均P〈0．05）。SMAD4 mRNA的表达与Ⅰ型、Ⅲ型前胶原基因α1 mRNA的表达成正相关（r值分别为0．728和0．678,均P〈0．05）。SMAD3 mRNA的表达与Ⅰ型前胶原基因α1 mRNA的表达成正相关（r=0．859,P〈0．01）。结论PSS硬化皮肤组织中存在有SMAD4 mRNA的高表达,可能是导致Ⅰ型、Ⅲ型胶原在硬化组织中过度堆积的原因之一。     

Advanced systemic sclerosis complicated by pulmonary hypertension and complete atrioventricular block: a case report.

BACKGROUND: Systemic sclerosis (SSc) is a connective tissue disorder of unknown etiology characterized by fibrosis of the skin and visceral organs, in which the heart is frequently (40-70% of patients) and severely involved. Pulmonary hypertension affects 10-15% of patients with SSc and is one of the most important complications adversely influencing their survival. CASE REPORT: The case report presents a 59-year-old male patient with advanced systemic sclerosis whose initial examination revealed pulmonary hypertension, rhythm and atrioventricular conduction disturbances, and elevated level of NT-proBNP. After six months the patient deteriorated; an increase in NT-proBNP level and progression of pulmonary hypertension were observed. CONCLUSIONS: The described case is followed by a discussion of cardiovascular involvement in systemic sclerosis and emphasizes that heart involvement in SSc may have very serious clinical implications.     

以发热、关节炎、皮肤色素沉着为主要表现的血管免疫母细胞性T细胞淋巴瘤1例

Angioimmunoblastic T-cell lymphoma is a rare T-cell lymphoma. The clinical manifestations are not specific. In addition to the common clinical manifestations of lymphomas such as fever, weight loss, night sweats and lymphadenopathy, it may also have skin rashes, arthritis, multiple serous effusions, eosinophilia and other systemic inflammatory or immune symptoms. The lymphoma cells of angioimmunoblastic T-cell lymphoma originates from follicular helper T cells, and the follicular structure of lymph nodes disappears. In the tumor microenvironment, in addition to tumor cells, there are a large number of over-activated immune cells, such as abnormally activated B cells, which produce a series of systemic inflammation or immune-related symptoms. This disease is rare and difficult to diagnose. This article reports a 36-year-old female. She got fever, joint swelling and pain, skin pigmentation, accompanied by hepatomegaly, splenomegaly, lymphadenopathy, anemia and other multiple-systems manifestations. The clinical manifestations of this patient were similar to autoimmune diseases such as adult onset Still’s disease, rheumatoid arthritis, and systemic sclerosis, which made the diagnosis difficult. At the beginning of the disease course, the patient got arthritis and fever. And her white blood cells were significantly increased. Adult onset Still’s disease should be considered, but her multiple-systems manifestations could not be explained by adult onset Still’s disease. And her arthritis of hands should be distinguished with rheumatoid arthritis. However, the patient’s joint swelling could get better within 3-7 days, and there was no synovitis and bone erosion on joint imaging examination. The rheumatoid factor and anti-CCP antibody were negative. The diagnostic evidence for rheumatoid arthritis was insufficient. The patient’s skin pigmentation and punctate depigmentation were similar to those of systemic sclerosis. But the patient had no Raynaud’s phenomenon, and her sclerosis-related antibody was negative. The diagnostic evidence for systemic sclerosis was also insufficient. After 3 years, she was finally diagnosed with angioimmunoblastic T-cell lymphoma by lymph node biopsy aspiration. This case suggests that the clinical manifestations of angioimmunoblastic T-cell lymphoma are diverse, and some symptoms similar to immune diseases may appear. When the patient’s clinical symptoms are atypical and immune diseases cannot explain the patient’s condition, and further evidence should be sought to confirm the diagnosis.