结缔组织病与心力衰竭

结缔组织病,如类风湿关节炎、系统性红斑狼疮、多发性肌炎/皮肌炎和系统性硬化病等常累及心脏,并发心力衰竭是结缔组织病患者死亡的主要原因之一。心脏自身免疫及结缔组织病炎症环境可能是该类人群发生心力衰竭的主要机制。心脏核磁共振用于早期诊断,治疗上与一般人群心力衰竭类似,此外,改善病情的抗风湿药如甲氨蝶呤可能降低心力衰竭的发病率和住院率。     

结蛋白肌病相关的心力衰竭和心律失常一例

结蛋白肌病主要表现为下肢对称性远端肌无力,部分病例累及心肌和心脏传导系统。本文报道1例少见的结蛋白肌病病例,双侧心室心肌受累导致心力衰竭以及传导系统受累导致束支传导阻滞。     

系统性硬化病并发卵巢硬化性间质瘤一例

系统性硬化病（systemic sclerosis，SSc）是一种病因不明的、伴有胶原和其他结缔组织成分在皮肤和多个内脏器官沉积的系统性自身免疫病。通常可累及心脏、肺、肾脏和消化道．合并卵巢硬化性问质瘤罕见。本院最近收治1例，现报告如下。     

系统性红斑狼疮心脏受累的诊治进展

系统性红斑狼疮（SLE）是最常见的系统性自身免疫性疾病之一,合并心脏受累的发生率高达50%以上,而心脏受累常常是SLE患者预后不良和死亡的主要原因。其发生机制包括：心脏和血管的自身免疫性炎症,T细胞激活释放大量细胞因子,长期使用糖皮质激素加速冠状动脉硬化等,上述因素共同作用导致心脏病变和早发的冠状动脉粥样硬化。心脏各层均可受累,心包炎最常见,约见于60%的SLE患者,心肌病变和早发冠状动脉粥样硬化也比较常见,是SLE的常见死因。因此,应常规对SLE的患者进行心脏受累的筛查,早期诊断,并尽早积极治疗可改善SLE患者的预后。     

系统性硬化的临床表现与治疗研究

系统性硬化（SSc）是一种原因不明的、临床上以局限性或弥漫性皮肤增厚和纤维化为特征的结缔组织病.除皮肤受累外,它也可以影响内脏包括心脏、肺脏和消化道等器官.本文旨在系统的讨论和研究SSc的临床表现和治疗,为对疾病的认识提供参考.     

心血管专科大夫如何识别系统性风湿免疫病

系统性风湿免疫病是以多器官受累、慢性、反复发作为特征的自身免疫炎症,心脏和血管是其常见受累器官之一,其所累及的心血管解剖部位是全方位的,如心脏的心外膜、心肌、心内膜和心     

韦格纳肉芽肿与肺结核的鉴别诊断（附6例分析）

韦格纳肉芽肿（Wegener’s Granulomatosis，WG）是一病因未明的系统性、坏死性肉芽肿性血管炎，主要累及上、下呼吸道及肾脏，临床主要表现为鼻及副鼻窦炎、肺病变和进行性肾衰，还可累及关节、眼、耳、皮肤，亦可侵犯心脏及神经系统等；无肾脏受累者称为局限型WG，累及肾脏者称为系统型WG。WG年发病率仅0．4／10万人，较为少见，临床医生对其认识不深，极易误诊。     

超声斑点追踪技术评价系统性红斑狼疮患者左室局部收缩功能

系统性红斑狼疮（systemic lupus erythematosus，SEE）是一种临床上较为常见的自身免疫性疾病．其病因和发病机制目前还不十分清楚。临床表现复杂多样。可发生各个系统和脏器损害。心血管系统是系统性红斑狼疮常累及的重要脏器之一，文献[1]报道52％～89％的SLE患者有心脏受累。大多数心脏受累患者在病程早期无临床症状，但发生冠状动脉明显病变时可危及生命，是SLE患者死亡的主要原因之一。     

硬皮病的心肺表现

硬皮病是一种结缔组织病,临床上并非罕见.除皮肤损害外,心肺易受累.本文重点讨论硬皮病心肺病变的临床特点及其治疗与预后。临床特点硬皮病分局限性和系统性两型.前者主要表现为局限性皮肤硬化;后者除皮损外,并累及内脏器官,尤以心肺病变较为明显。     

系统性红斑狼疮合并急性心肌梗死二例

<正>系统性红斑狼疮(systemic lupus erythematosus,SLE)是一种累及多系统和器官的全身性自身免疫性疾病,其基本病理变化为血管炎。自1942年心脏受累首次报道后,心血管病变逐渐被认识,心脏各个部位均可受累,从心包、心肌、心内膜至心瓣膜,冠状动脉受累并导致心肌梗死者少见。现报道本院收治的2例SLE合并急性心肌梗死患者并结合文献复习,以加深对本病的认识。     

Abnormal cardiac enzymes in systemic sclerosis: a report of four patients and review of the literature

Cardiac involvement in systemic sclerosis (SSc) is heterogeneous and can include primary involvement of the myocardium, pericardium and coronary arteries or be secondary to cardiac complications of pulmonary and renal disease. Primary cardiac involvement in SSc is uncommon but can result in ventricular dysfunction, organ failure, arrhythmias and death. It can remain clinically silent and the prevalence is likely to be under-reported. We report four cases of SSc associated with a raised serum troponin T (TnT), in a proportion of whom cardiac MRI myocardial abnormalities were detected. These cases highlight the heterogeneity of cardiac involvement in SSc, the role of cardiac MRI and promising biochemical responses to immunosuppression. Cardiac biomarkers such as TnT may be useful screening tools to identify subclinical cardiac disease and assess response to therapeutic intervention.     

Imaging Lung Disease in Systemic Sclerosis

Interstitial lung disease and pulmonary hypertension (PH) are the most common cardiopulmonary findings in patients with systemic sclerosis (SSc). About two thirds of patients suffering from SSc develop scleroderma interstitial lung disease. PH is present in about 20% of SSc patients and is typically associated with severe lung disease, although it may be an isolated manifestation of SSc. High-resolution CT scanning is a key method for evaluating chest involvement. There are four roles of imaging in scleroderma interstitial lung disease: 1) detection of lung involvement, 2) identification of patients likely to respond to treatment, 3) assessment of treatment efficacy, and 4) exclusion of other significant diseases to include PH and cardiac and esophageal abnormalities.     

Cardiac manifestations in systemic sclerosis

Primary cardiac involvement, which develops as a direct consequence of systemic sclerosis(SSc), may manifest as myocardial damage, fibrosis of the conduction system, pericardial and, less frequently, as valvular disease. In addition, cardiac complications in SSc may develop as a secondary phenomenon due to pulmonary arterial hypertension and kidney pathology. The prevalence of primary cardiac involvement in SSc is variable and difficult to determine because of the diversity of cardiac manifestations, the presence of subclinical periods, the type of diagnostic tools applied, and the diversity of patient populations. When clinically manifested, cardiac involvement is thought to be an important prognostic factor. Profound microvascular disease is a pathognomonic feature of SSc, as both vasospasm and structural alterations are present. Such alterations are thought to predict macrovascular atherosclerosis over time. There are contradictory reports regarding the prevalence of atherosclerosis in SSc. According to some authors, the prevalence of atherosclerosis of the large epicardial coronary arteries is similar to that of the general population, in contrast with other rheumatic diseases such as rheumatoid arthritis and systemic lupus erythematosus. However, the level of inflammation in SSc is inferior. Thus, the atherosclerotic process may not be as aggressive and not easily detectable in smaller studies. Echocardiography(especially tissue Doppler imaging), single-photon emission computed tomography, magnetic resonance imaging and cardiac computed tomography are sensitive techniques for earlier detection of both structural and functional scleroderma-related cardiac pathologies. Screening for subclinical cardiac involvement via modern, sensitive tools provides an opportunity for early diagnosis and treatment, which is of crucial importance for a positive outcome.     

Heart disease in systemic sclerosis

Primary cardiovascular manifestations of SSc include pericardial disease, myocardial disease, conduction abnormalities, and cardiac arrhythmias. Significant cardiac abnormalities are present in more than half of SSc patients at autopsy. As the frequency of subclinical cardiac involvement is now appreciated and noninvasive cardiac diagnostic modalities continue to improve, the ability to detect early asymptomatic involvement in SSc has improved. Two-dimensional echocardiography, radionucleotide imaging, and ambulatory ECG allow recurrent serial testing with virtually no morbidity. The current treatment of cardiac involvement in SSc is emperic and primarily directed at symptomatology. Large prospective randomized trials are needed to determine if preventive therapy is effective. With the advent of new immunological and cardiotropic agents and a better understanding of the primary disease process, our ability to alter the pathogenesis and final outcome of cardiac involvement in SSc should improve.     

The heart in systemic sclerosis

The heart is one of the major organs involved in scleroderma. Cardiac involvement can be manifested by myocardial disease, conduction system abnormalities, arrhythmias, or pericardial disease. Additionally, scleroderma renal crisis and pulmonary hypertension lead to significant cardiac dysfunction secondary to damage in the kidney and lung. This report summarizes the recent advances to further understand the types and mechanism of abnormalities in the heart in scleroderma. New cardiac technology shows significant frequencies of asymptomatic cardiac abnormalities. Further long-term studies are necessary to determine the outcome and the best approach to treatment of such abnormalities. Diastolic dysfunction has been carefully evaluated in scleroderma in recent years and appears to be more common than once realized. There is controversy as to whether this is a significant finding independent to other cardiopulmonary problems. More extensive evaluation of the conduction and the arrhythmia ablative therapy has helped manage these life-threatening complications.     

Autologous stem cell transplantation in a patient with diffuse systemic sclerosis

Systemic Sclerosis (SSc) is a systemic disease of unknown etiology presenting with disseminated skin thickening and fibrotic impairment of various organs including lung and kidney. According to the rate and degree of skin involvement, SSc can be classified in a limited and a diffuse form, the latter showing a severe and progressive lung involvement, which is responsible for its high related morbidity and mortality along with resistance to standard therapeutic protocols. High dose chemotherapy, followed by autologous stem cell transplantation, is a standard therapeutic regimen for haematological diseases: re-infusion of mobilised peripheral blood progenitor cells overcomes the myeloablative effect of super-maximal eradicative doses of chemotherapeutic agents. Recently, this therapeutic approach has been applied in some cases of resistant SSc and, albeit the low number of cases, it has been proven effective in early diagnosed and rapidly progressive forms of the disease showing a clinical improvement and an instrumentally detectable decrease of fibrosis extent. We report the case of a young woman affected by diffuse SSc with a rapid progression of clinical signs and instrumentally detectable lesions who underwent a conditioning regimen with fludarabine, cyclophosphamide and anti-thymoglobulines followed by re-infusion of autologous peripheral blood stem cells. Two years after transplantation a clinical and instrumental evidence of treatment was observed, with good control of disease evolution. The only sign of disease resumption was a slow worsening of skin involvement.     

Clinical subsets, skin thickness progression rate, and serum antibody levels in systemic sclerosis patients with anti-topoisomerase I antibody

OBJECTIVE: To describe the clinical and laboratory features and natural history of the disease in systemic sclerosis (SSc; scleroderma) patients with anti-topoisomerase I (anti-topo I) antibody who have different skin thickness progression rates (STPRs). METHODS: SSc patients (n = 212) who were anti-topo I antibody positive were divided into 5 subgroups based on STPRs. Skin thickness was measured using the modified Rodnan skin thickness score (MRSS). Anti-topo I IgG antibody levels were determined. RESULTS: Sixty patients who were anti-topo I antibody positive had diffuse cutaneous SSc (dcSSc) with rapid progression, 82 had dcSSC with intermediate progression, and 29 had dcSSc with slow progression, 14 had limited cutaneous SSc (lcSSc) that became dcSSc, and 27 had lcSSc that did not change throughout. Patients beginning with lcSSc were younger at disease onset and had longer disease duration when diagnosed as having SSc. Interstitial lung disease was common and was equally distributed across the subgroups. Renal crisis occurred most often in patients with rapid progression (22%) and was absent in lcSSc patients. Cardiac involvement was most frequent in the dcSSc subgroups. Both kidney and heart disease occurred most often within 3 years after the onset of skin thickening. The 10-year cumulative survival rate was <40% for patients with rapid and intermediate progression. Renal and cardiac causes of death were disproportionately frequent in these 2 subgroups. Anti-topo I antibody levels correlated with the STPR and the MRSS. CONCLUSION: Anti-topo I antibody-positive patients with SSc with a rapid STPR have reduced survival rates, primarily due to early and often fatal renal and cardiac involvement. Anti-topo I antibody levels parallel the MRSS at the first visit and the STPR. This information is important for managing physicians and researchers planning clinical trials involving patients with early dcSSc.     

Early manifestation of myocardial involvement in systemic sclerosis

IntroductionSystemic sclerosis (SSc) is a systemic autoimmune disease involving multiple organs. We present a rare case of SSc in which clinical manifestations of cardiac fibrosis occurred early in the disease course.Case reportWe report the case of a 40-year-old Caucasian man, previously diagnosed with SSc, who presented with decompensated heart failure. Transthoracic echocardiography was remarkable for severe right ventricular systolic dysfunction, abnormal ventricular septal motion, severe functional tricuspid regurgitation and normal pulmonary artery systolic pressure. Left ventricular ejection fraction was 45%. Right heart catheterization revealed no signs of pulmonary hypertension. Cardiac magnetic resonance (CMR) showed diffuse myocardial infiltration, later confirmed as myocardial fibrosis by endomyocardial biopsy.ConclusionsMyocardial fibrosis is an important cause of early heart failure in SSc patients and is associated with poor prognosis. Echocardiography and CMR help establish the diagnosis and enable an appropriate therapeutic strategy to be developed in such cases.     

Cardiac magnetic resonance imaging detects subclinical right ventricular impairment in systemic sclerosis

OBJECTIVE: To assess myocardial involvement in patients with systemic sclerosis (SSc) with no signs or symptoms of cardiac impairment (New York Heart Association functional class I). METHODS: Fifty patients (45 women, 5 men, age 53.3 +/- 12.9 yrs) who did not complain of serious diseases other than SSc were recruited out of 119 consecutive patients with SSc. Thirty-three were found to have limited cutaneous SSc (lSSc) and 17 diffuse SSc (dSSc). All underwent cardiovascular magnetic resonance imaging (MRI) to determine right and left systolic and diastolic volumes and ventricular ejection fractions (RVEF and LVEF). Thirty-one healthy subjects matched for sex, age, and body surface area (BSA) were studied as controls. Diffusion lung capacity test (DLCO) and high resolution computed tomography were performed to evaluate lung involvement. RESULTS: Disease duration between patients with lSSc (14.1 +/- 11.4 yrs) and those with dSSc (6.9 +/-4.4yrs) was found to be significantly different (p < 0.003). lSSc patients were older than those with dSSc (54.8 +/- 13.7 yrs vs 50.4 +/- 9.9 yrs, respectively; p < 0.04). Anticentromere antibodies and Scl-70 were positive in 23 (46%) and 17 patients (34%). Except for the left and right systolic volumes, all unadjusted cardiac MRI measures were significantly reduced in SSc compared to the controls (p < 0.001 and p < 0.009). These differences persisted after adjustment for subjects' height and BSA. Raw RVEF data and RVEF data matched for height and BSA were significantly reduced in dSSc patients in comparison to lSSc (p < 0.03). CONCLUSION: Compromised RVF was found in patients with asymptomatic SSc. Unlike standard diagnostic techniques, cardiac MRI appears to be a rapid and noninvasive means of determining subclinical right myocardial involvement that is otherwise undetected in patients with SSc.     

Impact of cardiac involvement on the risk of mortality among patients with systemic sclerosis: a 5-year follow-up of a single-center cohort

Cardiac involvement is among the leading causes of mortality in patients with systemic sclerosis (SSc). Previously, we demonstrated in a single-center, cross-sectional study the frequent coexistence of different forms of cardiac involvement in systemic sclerosis including pulmonary arterial hypertension (PAH), coronary artery disease (CAD), and microvascular dysfunction (MVD). The aim of the present study was to investigate the prognostic significance of cardiac involvement. One hundred twenty patients with SSc were enrolled. All cases underwent a non-invasive cardiovascular protocol. In 30 patients with suspected cardiac involvement, right heart catheterization and intra-coronary pressure–wire-supplemented coronary angiography were performed. Clinical follow-up was 5 years. Patients with CAD at the baseline showed a trend for higher cardiovascular mortality while in patients with MVD this difference was significant (26.7 % versus 9.5 %, p?=?0.077 and 30 % versus 10.1 %, p?<?0.05, respectively). Cardiovascular mortality of PAH cases was higher but, however, did not reach statistical significance 21.4 % versus 10.4 %, p?=?0.261. Cardiovascular event-free survival was significantly lower among patients with combinations of two or three disorders (p?<?0.05). Multivariate analysis of organ involvements and comorbidities showed that the diffuse cutaneous subset, the presence of kidney involvement, the velocity of the tricuspid regurgitation, as well as diabetes mellitus were independent predictors of overall mortality. MVD and CAD alone or in combination with PAH significantly affected the 5-year cardiovascular mortality. These findings highlight the prognostic importance of coronary disease in patients with SSc [www.clinicaltrials.gov (Reg. Nr.: NCT00843102)].