系统性硬化症研究现状及治疗进展

系统性硬化症（SSc）是涉及炎症,纤维化,血管病变的多系统自身免疫疾病。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、肺组织纤维及肾危象等致死性内脏损害,根据其皮肤病变的程度及病变累及的部位,可分为局限性和弥漫性。该病发病率和死亡率高,以女性为主女：男=（4.6：1）,以45~60岁为发病高峰,一般认为系统性硬化症的发生和遗传易感性及环境因素相互作用相关,研究表明遗传因素占有重要的地位。本文主要讨论目前SSc研究现状及常用药物的使用。     

以肺痹论治系统性硬化症肺间质纤维化

系统性硬化症肺间质纤维化从中医角度认识,多将其归为中医肺痹一证进行论治,本文总结前人对肺痹的认识,认为本病是由痹证日久,肺气损伤,外邪侵袭造成的,以风寒湿等外邪痹阻日久,肺肾两虚,痰瘀毒阻于肺络为基本病机,在治疗上主张以补肺纳肾,祛痰化瘀解毒通络为总原则,以便更明确的认识本病,指导临床。     

系统性硬化症的主要发病因素及治疗研究进展

系统性硬化症在临床上较为罕见,也被称为硬皮病,属于免疫性疾病的一种,临床上一般根据患者发病后皮肤损伤情况将系统性硬化症分为局限性系统性硬化症、弥漫性系统性硬化症、重叠综合征、无硬化系统性硬化症、未分化结缔组织疾病.通过分析系统性硬化症患者的主要发病原因探讨其应对的治疗措施.系统性硬化症的发病率容易受环境因素、遗传因素的...     

系统性硬化症血管病变治疗进展

系统性硬化症(Systemic sclerosis,SSc)是一种累及皮肤及多脏器的自身免疫性疾病,血管病变在SSc发病中起重要作用.本文综述了系统性硬化症血管病变诊治进展.     

尼达尼布治疗间质性肺疾病的作用机制研究进展北大核心CSCD

尼达尼布是德国勃林格殷格翰公司研发的一种酪氨酸激酶抑制剂,目前被获批用来治疗多种间质性肺病,本文主要对尼达尼布治疗间质性肺病的作用机制进行综述,包括特发性肺纤维化、系统性硬化症相关间质性肺病、进行性纤维化性间质性肺病以及类风湿性关节炎相关间质性肺病,总结出尼达尼布通过抑制多种促纤维化信号通路及干预多种炎症相关的表型,从而在持续性肺纤维化和炎症免疫相关间质性肺疾病中发挥治疗作用,以期为尼达尼布在间质性肺疾病的临床用药提供理论基础。     

依地酸钙钠治疗系统性硬化症86例

目的观察依地酸钙钠(EDTA)治疗系统性硬化症(SSc)的疗效及安全性.方法对86例SSc患者进行EDTA治疗,以30mg·kg     

系统性硬化症的治疗进展

系统性硬化症（SSc）病因不明,治疗困难。近年来采用新的血管扩张剂、免疫抑制剂、血浆置换、干细胞移植等新疗法,在治疗SSc方面取得一定疗效。     

系统性硬化症药物治疗的护理及观察要点

系统性硬化症(systemic sclerosis,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.为确保护士在给药过程中予以正确的药物指导及观察,本文就SSc各类药物治疗的护理及观察要点作一综述.     

系统性硬化症靶向治疗进展

靶向治疗旨在根据患者自身的病理生理学特征选择治疗药物.尽管针对系统性硬化症的靶向治疗仍处于初步阶段,但靶向治疗的进展对我们更深入理解、研究系统性硬化症的发病机制有重要意义.本文总结了已开展的系统性硬化症靶向治疗药物的现状,并对已获得或较有可能获得阳性结果的药物利妥昔单抗、fresolimumab、托珠单抗、利奥西呱、尼达尼布等做一综述.     

重组干扰素γ治疗系统性硬化症15例

目的：观察重组干扰素γ对系统性硬化症(SSc)的疗效和安全性。方法：选取SSc患者15例，完成3个月的疗程，每日给予重组干扰素γ 100万U，im，每周5 d。结果：治疗3个月在皮肤硬度积分、关节功能积分、最大齿距、血沉(ESR)、C 反应蛋白等方面差异均有极显著性，一氧化碳弥散率差异有显著性，无严重不良反应发生。结论：重组干扰素γ对SSc有效，且副作用低。     

Long-term N-acetylcysteine therapy in systemic sclerosis interstitial lung disease: a retrospective study

Systemic sclerosis (SSc) is associated with interstitial lung diseases. The primary endpoints of this study were changes between baseline and month 24 in single-breath carbon monoxide diffusing capacity (DLco). The secondary endpoints were: vital capacity (VC), forced expired volume in 1 sec (FEV1), total lung capacity (TLC), scores of high resolution computed tomography (HRCT) of the chest, number of adverse effects. In this study, we retrospectively investigated data from SSc patients who had undergone therapy with high-dose intravenous N-acetylcysteine (NAC) at a dosage of 15 mg/Kg/h for 5 consecutive hours every 14 days. After NAC therapy median values of DLco (69.5 vs 77.7%), VC (99 vs 101.3%) and TLC (93 vs 98.3%) significantly increased. We did not observe any significant changes from baseline in FEV1 value and HRTC score. The improvement in lung function was more evident in SSc patients without radiological signs of pulmonary fibrosis than in patients with pulmonary fibrosis. In SSc patients with mild-moderate pulmonary fibrosis intravenous NAC administration slows the rate of deterioration of DLco, VC and TLC. In conclusion, this retrospective study demonstrates that long-term therapy with intravenous NAC ameliorates pulmonary function tests in SSc patients.     

Targeting vascular disease in systemic sclerosis

Systemic sclerosis (scleroderma, SSc) is a chronic connective tissue disease of unknown etiology characterized by progressive fibrosis of the skin and a distinctive pattern of internal organ involvement. Excessive fibrosis, vascular injury, autoimmunity and inflammation are permanent features of the disease process leading to irreversible organ damage and significant morbidity and mortality in SSc patients. Recent progress in understanding the pathogenesis of SSc as well as diagnostic and therapeutic advances in medicine have made more effective treatment strategies possible. So far, therapies targeting vascular aspects of SSc have been most successful. This underlines the role of vascular injury in the pathogenesis of the disease and raising hopes of significant improvement in the management of SSc patients. The aim of this review is to summarize recent and potential future treatments of SSc-associated vascular disease.     

Interstitial lung disease in systemic sclerosis

Interstitial lung disease is an early and serious complication of systemic sclerosis (SSc). Because it may be asymptomatic for a long period, and only the early flogistic phase is at present susceptible to treatment, early diagnosis and identification of risk are critical to the outcome. However, identifying SSc patients at risk for developing interstitial lung disease is at present difficult; therefore, a strict monitoring of the disease, especially in the first years, is mandatory. Treatment strategy is aimed at suppressing inflammation. Unfortunately, optimal therapy has not yet been established. Combination of corticosteroids and cyclophosphamide is considered the best therapeutic approach available so far, but doses and duration of treatment need to be determined. Future research should focus on new anti-inflammatory or immunosuppressive agents.     

Treatment of complications associated with systemic sclerosis.

PURPOSE: Current and emerging drug therapy options for patients suffering from the complications of systemic sclerosis are presented. SUMMARY: Systemic sclerosis is a devastating and rare, chronic, autoimmune disease and is characterized by various disease complications due to skin thickening, vascular damage, and inflammation affecting numerous organs. There are two major subtypes of systemic sclerosis: limited cutaneous scleroderma and diffuse cutaneous scleroderma. Patients suffer from Raynaud's phenomenon, skin changes, musculoskeletal changes, gastrointestinal complications, pulmonary complications, scleroderma renal crisis, and dryness of the eyes and mouth. Currently, there is no cure for systemic sclerosis, but research is focusing on decreasing the progression and symptoms of this disease. Raynaud's phenomenon is the temporary vasoconstriction of the small vessels of the fingers, toes, tip of the nose, and earlobes. Skin thickening is the cardinal symptom of systemic sclerosis, with as many as 50% of patients developing digital ulcers. Care of these ulcers is crucial in the prevention of osteomyelitis and other infections. Malabsorption syndrome may also occur in patients, many of whom will eventually require parenteral nutrition to maintain their caloric needs. Pulmonary interstitial fibrosis and pulmonary arterial hypertension are additional serious complications of systemic sclerosis. The use of prostacyclin analogues, phosphodiesterase inhibitors, calcium-channel blockers, cyclophosphamide, bosentan, and other agents has been investigated in patients suffering from the complications of systemic sclerosis. CONCLUSION: Systemic sclerosis is characterized by various circulatory, dermatological, gastrointestinal, musculoskeletal, pulmonary, and renal complications. Although there is no cure for systemic sclerosis, management of its associated complications can help improve patients' quality of life.     

Diffuse interstitial pulmonary disease during amiodarone treatment

A case of diffuse interstitial pneumopathy which was observed by photonic microscopy and confirmed by electron microscopy is presented in a patient treated with amiodarone only. The iatrogenic origin of this pneumopathy appears certain as clinical and radiological signs improved after amiodarone treatment was withdrawn.     

Endothelin receptor antagonists as disease modifiers in systemic sclerosis

Systemic sclerosis (SSc) is a multisystem connective tissue disease of unknown etiology that is characterized by inflammation, vascular dysfunction and fibrosis of the skin and visceral organs. SSc is clinically diverse both in terms of the burden of skin and organ involvement and the rate of progression of the disease. Recent studies indicate that the endothelin system, especially ET-1 and the ETA and ETB receptors may play a key role in the pathogenesis of SSc. A new class of drugs, endothelin receptor antagonists has been introduced for treatment of patients with pulmonary arterial hypertension (PAH). Bosentan, a dual endothelin receptor antagonist as well as Sitaxsentan and Ambrisentan, selective blockers of the ETA receptor have proven effective in SSc-PAH. This effect may be mediated through both a vasodilatory and antifibrotic effect, thus making these agents attractive as potential disease modifying agents for SSc.     

Non-invasive diagnostic and functional evaluation of cardiac and pulmonary involvement in systemic sclerosis

BACKGROUND: The aim of the present study was to evaluate the role of non-invasive methods in the early detection of pulmonary and cardiac involvement in Systemic sclerosis (SSc) and to identify clinical and/or instrumental patterns of prognostic value. PATIENTS AND METHODS: Twenty female patients affected by SSc (8 with diffuse cutaneous SSc and 12 with limited cutaneous SSc) were enrolled in our study. Cardiac and pulmonary involvement (respiratory function tests and carbon monoxide lung diffusion [DLCO], chest radiography, high resolution computed tomography [HRCT] and lung perfusion magnetic resonance) were evaluated. RESULTS: All 18 patients studied with respiratory function tests showed a significant reduction of DLCO. HRCT was considerably more sensitive than traditional chest radiography (59% versus 28%; p<0.05). Lung perfusion MRI revealed normal findings in 15 patients. Abnormal lung perfusion MRI results were found only in 3 patients. Angina pectoris with electrocardiographic and scintigraphic ischemic changes, severe regional wall motion abnormalities and complex arrhythmias seemed to be associated with poor prognosis. CONCLUSION: Taken together these results indicate that a pulmonary involvement occurs both in limited and in diffuse cutaneous SSc patients and develops, in 83% of the cases, without any regional lung perfusion abnormality. Furthermore, cardiac involvement is detected in 65% of the cases as a consequence of a range of noxious events including myocardial ischemia, fibrosis and pressure overload which may result in ventricular dysfunction and arrhythmias. Lung perfusion MRI should be considered as a complementary diagnostic method for the functional evaluation of these symptoms in systemic sclerosis.     

系统性硬化症合并肺间质病变患者血清中KL-6及SP-D水平与肺功能参数的关系

目的 通过检测系统性硬化症（SSc）合并间质性肺病（ILD）患者血清中肺表面活性蛋白D（SP-D）和Ⅱ型肺泡细胞表面抗原（KL-6）,分析其与患者临床症状、免疫学指标及肺功能的相关性,探索诊断ILD的新血清学指标。方法 收集安徽省立医院风湿免疫科2013年1～10月住院及门诊确诊为SSc患者35例,其中SSc-ILD组20例,SSc-非ILD组15例,正常对照组选择年龄、性别相匹配的19例我院门诊健康体检者。采用ELISA法检测所有研究对象血清中SP-D和KL-6的水平。比较SSc组、SSc-ILD组、SSc-非ILD组、正常对照组血清样本中SP-D和KL-6的水平,分析上述血清学指标与肺功能指标的相关性。结果 ①血清中KL-6、SP-D水平SSc-ILD组患者高于正常对照组,差异有统计学意义（P<0.05）,SSc-ILD组显著高于SSc-非ILD组（P<0.05）,而SSc-非ILD组与正常对照组比较,差异无统计学意义（P>0.05）;② SSc患者血清中KL-6、SPD与肺功能指标用力肺活量（FVC）、一氧化碳弥散量（DLCO）、肺活量（VC）呈负相关（P<0.05）,其中KL-6与血沉（ESR）、C反应蛋白（CRP）、IgG、IgA、IgM 无相关性,SP-D与IgG、IgM呈负相关（P<0.05）,与ESR、CRP、IgA无相关性。结论 SSc合并ILD患者血清中KL-6、SP-D水平显著升高,SSc患者血清中KL-6、SP-D水平与肺功能指标呈负相关;血清KL-6、SP-D可能作为诊断与评估SSc-ILD病情严重程度的血清学指标。     

Bosentan in systemic sclerosis

Systemic sclerosis (SSc) is a relatively rare chronic connective tissue disease characterized by varying degrees of skin fibrosis and visceral organ involvement. Pulmonary compromise, including pulmonary arterial hypertension and interstitial lung disease, is currently the leading cause of death in patients with SSc. Digital ulcers are common complications which lead to substantial morbidity and functional limitation. Until recently, treatment options for these complications were quite limited. Endothelin-1 (ET-1) is a peptide that has a role in promoting both vascular injury and the fibrotic process in SSc. Bosentan is a dual endothelin receptor antagonist approved for the treatment of pulmonary arterial hypertension. In patients with pulmonary hypertension secondary to SSc, bosentan therapy prevents deterioration in exercise capacity and may improve survival. No beneficial effect was found in one study in patients with interstitial lung disease and SSc. Bosentan is able to reduce the number of new digital ulcers in patients with either a history of previous ulcers or an active ulcer, without expediting the healing of existing ulcers. Bosentan therapy is contraindicated in pregnancy and causes elevated liver transaminases in up to 14% of patients. Hence, monthly pregnancy tests should be performed and hepatic function should be monitored.     

肺间质纤维化34例临床分析

目的 研究慢性阻塞性肺疾病（COPD）合并肺间质纤维化（PIF）的临床特点并探讨其临床意义。方法对34例COPD合并PIF患者的病程、症状、体征、肺功能和血气分析、胸部X线片、胸部高分辨率CT（HRCT）进行回顾性分析,并与单纯COPD患者进行比较。结果 1与单纯COPD相比较,COPD合并PIF患者病程较长,临床多表现为混合型呼吸困难并进行性加重,肺部听诊可闻及Velcro啰音伴有杵状指（趾）（P〈0.01）;2合并PIF组混合性通气功能障碍发生率及肺弥散功能降低显著大于单纯COPD组,单纯COPD组阻塞性通气功能障碍发生率显著高于合并PIF组;3合并PIF组PaO2降低和PaCO2升高显著高于单纯COPD组;4影像学显示合并PIF组胸部X线片显示弥漫点状、结节状影、网格状、毛玻璃状和蜂窝状影较多,HRCT显示以支气管管壁增厚,支气管血管束增粗扭曲、变形、小叶间隔增厚为主。结论 COPD合并PIF患者由于双重的病理损害造成肺通气功能、弥散功能严重障碍和严重低氧血症,并具有独特的临床与影像学特征,影响患者预后,肺功能及HRCT可为临床提供重要的诊断依据。