系统性硬皮病的血管病变

系统性硬皮病患者的血管异常主要表现为小血管（动脉）痉挛及内膜增生。它们可引起肢端病变；并与心、肺、脑、胃肠道胃及有关。其血管异常的发病机制可能是血管紧张调节失衡所致，也可能和补体及抗内皮抗体等自体免疫因子所导致内皮细胞活化和内皮损伤有关，甚若是二者的共同作用。     

系统性硬皮病46例临床分析

为探讨系统性硬皮病(SSc)的皮肤、内脏损害特点和治疗方法,对2001~2005年在上海华山医院住院的46例SSc的临床表现、实验室检查及辅助检查结果、治疗方法进行统计分析。结果:46例均有不同程度皮肤损害,其中肢端型41例,弥漫型5例;45例双手皮肤累及,32例面部出现皮损,内脏损害以肺部最常见,27例检查肺功能有17例发现肺功能障碍,其中仅3例有呼吸道症状。本病目前尚无特效治疗方法。SSc皮肤损害以肢端型为主,双手、面部累及率高;内脏损害以肺部最常出现,应在疾病早期拍胸片、检测肺功能和行肺部高分辨率CT检查,以及早发现肺部病变及时治疗。     

系统性硬皮病105例临床分析

对１０５例系统性硬皮病患的一般情况、临床表现了详细分析。男女患者之比为１：４，发病年龄１５岁－７２岁，２１－５０岁患者占大多数，所有患者均有不同程度的皮肤改变，皮受累范围大小不一。９９％，患者嗓手皮肤受累，８１％患者有雷诺现象，６７例患者内脏器官受累。还对其实验室检查，伴发病、病因、诊断和治疗作了分析和讨论。     

系统性硬化症的心脏病变

系统性硬化症(SSc)除影响皮肤外,还可有内脏器官受累,其中心脏也常受到影响.轻者无临床症状,仅在作心电图及其它心脏检查时发现病变,重者可发生心衰,甚至死亡.国内对SSc的心脏病变仅见个别病例报道.现将我院40年来资料较完整的126例SSc中有心脏病变的48例作回顾性分析.     

γ-干扰素治疗系统性硬皮病

我科自1993年～1994年采用第二军医大学附属长海医院提供的γ干扰素治疗10例难治性系统性硬皮病(ＳＳｃ),疗效较佳,总结如下。一般资料　病员来自上海市硬皮病医疗协作中心。男1例,女9例,年龄27～67岁。弥漫型7例,肢端型3例。10例均符合日本厚生省特殊疾病研究班所订硬皮病诊断标准[1]。均因常规治疗效果不佳而加用γ干扰素。研究方法　自身交叉对照法。按平衡随机法将病员分为2组,每组5例。一组先治疗3月,然后观察3月,另一组反之。治疗期间用药:γ干扰素100万ｕ肌注,隔日1次,共3月,同时服901冲剂(成分:桂枝、茯苓、桃仁、丹参、落得打、乌…     

系统性硬皮病的血管病变发病机制研究进展

系统性硬皮病(SSc)是一种不明病因的自身免疫性疾病，以进行性的皮肤和内脏纤维化、微血管系统改变和多种细胞、体液免疫异常为特征。SSc的临床表现差异较大，从局限的皮肤硬化、轻微的内脏损害到严重的皮肤及多内脏纤维化，形成一个范围较广的病谱．血管病变是SSc多数临床表现的病理学改变基础本文就SSc血管结构及功能的改变、血管活性相关的细胞因子、血管内皮细胞的凋亡、抗内皮细胞抗体等方面的研究进展进行了综述。     

系统性硬皮病治疗的研究进展

系统性硬皮病(SSc)是一种以皮肤及各系统胶原纤维硬化为特征的结缔组织疾病,临床上以皮肤增厚和内脏组织进行性纤维化为特征,常表现为雷诺现象、肺动脉高压、肺组织纤维化及多器官受累。SSc的治疗以免疫抑制剂为基础。随着各相关学科的发展,新型免疫抑制剂、生物制剂和干细胞移植等相继用于治疗SSc,并取得了显著的疗效,且对SSc的治疗也有了更深入的认识。本文就近年来治疗方面的进展作一综述。     

系统性硬皮病治疗的研究进展

硬皮病的发病机制不明,其治疗一直是临床工作中的难点,目前,对系统性硬皮病尚无特效疗法,可应用的治疗方法较多,如血管扩张剂、免疫抑制剂、抗纤维化剂和一些新的疗法等,但疗效均不理想,评价也比较困难.文中将对系统性硬皮病治疗的研究进展作一综述.     

Chromosomal breakage in systemic sclerosis and related disorders.

Chromosome aberrations such as gaps and breaks of one or both chromatids, acentric fragments, dicentrics, ring chromosomes and other abnormal chromosomes are observed in lymphocyte and fibroblast cultures as well as in direct bone marrow preparations from patients with systemic sclerosis. A serum factor producing chromosome breaks in mitoses from healthy donors was observed in 37 of 42 scleroderma patients. The biochemical nature of this breakage factor is still undefined. Increased breakage is also noted in a high percentage of healthy family members of scleroderma patients. It is also a common feature of related disorders such as lupus erythematosus, dermatomyositis, periarteritis nodosa and rheumatoid arthritis. An increase in chromosome breaks and rearrangements is also present in NZB mice developing spontaneously an autoimmune disorder that has been extensively studied by workers interested in lupus erythematosus. The similarity of the cytogenetic findings provides the opportunity to use these mice as an experimental model to investigate relationships between immunological perturbations and chromosomal aberrations.     

Serum adenosine deaminase activity in systemic sclerosis (scleroderma) and related disorders.

BACKGROUND: Adenosine deaminase (ADA) activity in serum is mainly derived from T lymphocytes. OBJECTIVE: Our purpose was to clarify the significance of ADA activity in systemic sclerosis (PSS) and related disorders. METHODS: ADA activity was determined with an enzymatic method in 34 patients with PSS, 4 with mixed connective tissue disease (MCTD), 6 with dermatomyositis (DM), 11 with localized scleroderma (LS), and 13 with other collagen diseases. RESULTS: Serum ADA activity was elevated over the mean (+2 standard deviations) of the control in 85% of the patients with PSS, all with MCTD, 83% of those with DM, and 82% of those with LS. The mean values in 10 PSS patients with anti-topoisomerase I antibodies, 14 patients with anti-RNP antibodies, 12 patients with anticentromere antibodies (ACAs), and 5 patients without antinuclear antibodies (ANAs) were 26.1, 24.9, 22.6, and 16.8 IU/L, respectively. In most cases, except for ACA-positive patients, serum ADA activity changed almost in parallel with ANA titers. CONCLUSION: These results support the notion that T cells are involved in the pathogenesis of PSS and related disorders.     

Mandibular resorption in systemic sclerosis

A patient with long standing systemic sclerosis, extensive mandibular resorption and associated atrophy of the masseter and internal pterygoid muscles is described.     

Iloprost therapy in systemic sclerosis

Systemic sclerosis is an autoimmune disease of unknown origin affecting multiple organ systems. The management of this disease is challenging. Many therapeutic attempts have only been moderately successful. Iloprost, a stable prostacyclin analogue, with its antifibrotic effect can influence an important step in the pathogenesis of systemic sclerosis. In this review we analyze the published data for the optimal dose and duration of treatment. In three different studies, iloprost given for 5 days in the highest tolerated dose of 1-2 ng/kg/min provided a significant improvement of the disease measured by the number and intensity of Raynaud attacks, healing of digital ulcers, and digital perfusion. This improvement lasted for about one month. When the infusions were repeated once a month, treatment effect could be maintained. Although the effect of this treatment regimen should be proven in further long-term studies, we think that an intermittent continuous therapy with iloprost could result in an improvement or stabilization of systemic sclerosis.