结缔组织病相关肺动脉高压79例临床分析

目的探讨结缔组织病相关肺动脉高压的发生率、临床特点及预后,提高对该病的认识。方法收集2005年1月～2008年8月我科收治的1876例弥漫性结缔组织病（CTD）中合并肺动脉高压的79例患者的临床资料及部分患者的随访资料。结果①混合性结缔组织病（MCTD）与系统性硬化症（SSc）肺动脉高压发生率最高（40．0％,30．2％）;②主要的临床表现为雷诺现象（62．0％）,抗SSA抗体与抗U1RNP抗体阳性率最高（51．9％）;③雷诺征发生率、血尿酸水平在轻、中重度肺动脉高压组间比较有统计学差异（P〈0．05）,肺动脉收缩压与雷诺征、心包炎、呼吸困难、血尿酸水平呈正相关（P〈0．05）,与关节炎呈负相关（P〈0．05）;④死亡3例,均为系统性红斑狼疮合并重度肺动脉高压者。结论CTD合并肺动脉高压并不少见,雷诺征与血尿酸水平可作为预测肺动脉高压严重程度的指标。     

结缔组织病并发肺动脉高压110例临床分析

目的：评估结缔组织病并发肺动脉高压的发生率、临床特点、诊断及治疗方法。方法：1278例结缔组织病患者中110例伴有肺动脉高压,对其进行回顾性分析。结果：结缔组织病合并肺动脉高压的发生率约为8.6%（110/1278）。110例患者中,女性95例,男性15例,年龄11～79岁,平均（46±17）岁;病程0.5～20年,平均（7±6）年。系统性硬化症（SSc）和混合性结缔组织病（MCTD）合并肺动脉高压发生率最高,分别为40.0%和25.0%,显著高于其他结缔组织病（P〈0.01）。有雷诺现象或雷诺现象合并肺间质病变者肺动脉压力显著升高（P〈0.01和P〈0.05）。且雷诺现象与肺动脉压力呈正相关（r=0.531,P〈0.01）。肺功能异常发生率较高,以弥散量降低最为多见。轻度肺动脉高压患者临床表现少,治疗可逆转;重度肺动脉高压治疗反应差,病死率高。结论：结缔组织病合并肺动脉高压较为常见,其中以SSc和MCTD合并肺动脉高压发生率最高。雷诺现象与肺动脉压力呈正相关,是预测肺动脉高压的良好指标。早期诊断和治疗,可以改善患者预后。     

系统性红斑狼疮合并肺动脉高压36例临床分析

目的 分析系统性红斑狼疮(SLE)并发肺动脉高压(PAH)的发生率、临床特点及预后影响因素.方法 对312例SLE患者的临床资料进行回顾性分析.结果 本文合并PAH 36例(11.5%),雷诺现象、抗U1RNP阳性率、SLEDAI评分和肺间质病变与PAH严重程度有关.结论 SLE是自身免疫性疾病中合并PAH的较常见疾病,超声心动图及相关检查有利于早期诊断.     

系统性硬化症相关间质性肺疾病的临床特点及危险因素分析

目的探讨系统性硬化症相关间质性肺疾病（SSc-ILD）的临床特点及危险因素。方法收集系统性硬化症（SSc）患者68例,根据有无间质性肺疾病（ILD）分为SSc-ILD组（44例）和单纯SSc组（24例）,分析SSc-ILD患者临床表现、自身抗体、胸部CT、肺功能及超声心动图特点,并与单纯SSc组比较。结果 SSc-ILD组中弥漫性皮肤损害型SSc（dcSSc）21例、局限性皮肤损害型SSc（lcSSc）23例,有呼吸系统表现者34例,其中活动后胸闷/气短30例、干咳18例。抗Scl-70抗体在SSc-ILD和dcSSc中的阳性率分别高于单纯SSc和lcSSc。SSc-ILD最常见的胸部CT表现为磨玻璃影（25例）,其次为网格状影（19例）。28例SSc-ILD行肺功能检测,其中弥散功能减退24例、限制性通气功能障碍13例。8例单纯SSc出现弥散功能减退,其中6例行超声心动图检查,5例有肺动脉高压（PAH）。SSc患者PAH发生率为58.93%（33/56）,ILD同时合并PAH者23例。结论 SSc-ILD以活动后胸闷/气短、磨玻璃影和弥散功能减退常见。单纯SSc若出现弥散功能减退,需警惕PAH可能。dcSSc及抗Scl-70抗体阳性是SSc发生ILD的危险因素。     

自身免疫性疾病并发肺动脉高压83例临床分析

目的分析自身免疫性疾病(AID)合并肺动脉高压的临床特点、诊断以及治疗方法,提高对继发性肺动脉高压的认识。方法对83例诊断明确、资料完整的AID合并肺动脉高压患者进行回顾性分析。结果5年间收治的2016例AID患者中合并肺动脉高压83例(4.1%),年龄10～67岁,平均(33±13)岁,其中男性7例(8.4%),女性76例(91.6%),占同期入院收治的肺动脉高压患者(350例)的31.1%。本组病例中易合并肺动脉高压的AID包括混合性结缔组织病(MCTD)、系统性硬化症(SSc),合并概率最低的为皮肌炎/多发性肌炎(DM/PM)。合并抗磷脂抗体综合征(APS)的系统性红斑狼疮(SLE)患者出现肺动脉高压的概率增加。合并肺动脉高压的AID患者出现雷诺现象的比例很高(P=0),二者存在相关性。出现雷诺现象的患者多有弥散性肺通气障碍,而且病情容易恶化。抗u1RNP阳性的AID患者出现肺动脉高压的可能明显升高(P=0)。结论肺动脉高压是一组以肺小动脉受累的疾病,AID是其常见合并疾病,其中MCTD、SSc合并肺动脉高压的概率最高,DM/PM合并的概率最低。合并APS可以增加SLE出现肺动脉高压的概率。对于出现雷诺现象、抗u1RNP的AID患者应警惕肺动脉高压的可能,早期行超声心动图及相关检查,早期诊断,以改善患者的预后。     

雷诺现象为首发表现的自身免疫性疾病疾病谱及临床特点

目的观察雷诺现象为首发临床表现的自身免疫性疾病疾病谱,以及其临床受累脏器的特点。方法选择2012年10月至2013年3月北京协和医院风湿免疫科门诊以雷诺现象为首发临床表现的自身免疫性疾病患者。除实验室常规检查外,所有患者均行胸部高分辨CT及超声心动图检查。结果共135例患者纳入研究,男9例,女126例,以系统性红斑狼疮（45．9％）、系统性硬化症（20．7％）及未分化结缔组织病（15．6％）最常见。系统受累以皮肤黏膜（97例,71．9％）、关节（86例,63．7％）及肺部受累（72例,53．3％）多见。其中合并肺间质病变者63例（46．7％）,以系统性红斑狼疮（29例,46．8％）、系统性硬化症（17例,60．O％）及混合性结缔组织病（6例,83．3％）多见;合并肺动脉高压者37例（27．4％）,以系统性红斑狼疮（16例,25．8％）、系统性硬化症（10例,35．7％）、未分化结缔组织病（4例,19．7％）及原发性干燥综合征（2例,28．6％）多见。结论雷诺现象可以是多种自身免疫性疾病的早期表现,尤其是系统性红斑狼疮及系统性硬化症。合并雷诺现象的患者更易出现肺部受累,尤其是肺间质病变及肺动脉高压,应重视在发病初期进行筛查。胸部高分辨cT及超声心动图检查分别是早期筛查肺间质病变及肺动脉高压的简便有效的方法。     

抗RNP抗体阳性系统性硬化症患者的临床特点

目的 探讨抗RNP抗体阳性系统性硬化症(systemic sclerosis, SSc)患者的临床表现及实验室特征。方法 纳入中国风湿病数据中心2008年8月至2020年6月在北京协和医院注册的SSc患者。根据抗RNP抗体是否阳性进行分组,比较各组间临床表现及实验室检查的差异。结果 686例SSc患者被纳入研究。抗RNP抗体阳性179例(26.1%),阴性507例(73.9%)。与抗RNP抗体阴性患者相比,抗RNP抗体阳性患者弥漫性SSc少见(27.4%vs. 40.2%,P=0.002),男性少见(6.1%vs. 12.0%,P=0.027),发病年龄更早[(40.1±11.7)岁vs.(44.4±12.6)岁,P<0.001]。临床表现,抗RNP抗体阳性SSc患者肺动脉高压(41.7%vs. 17.3%,P<0.001)、雷诺现象(96.1%vs. 91.1%,P=0.031)、手指肿胀(43.0%vs. 26.4%,P<0.001)、关节炎(19.6%vs.12.4%,P=0.019)、肌炎(25.1%vs. 14.8%,P=0.002)更常见,指端溃疡(21....     

系统性红斑狼疮肺动脉高压63例临床分析

目的 探讨系统性红斑狼疮(SLE)并肺动脉高压(PAH)患者的临床特点、发病机制以及诊治方法.提高对该病的认识.方法 选取本院近5年住院的SLE合并PAH的患者63例,对其临床症状、实验室指标、超声心动图特点、SLE病情活动评分等进行回顾性分析.结果 该组患者抗RNP抗体阳性率较高,肺动脉压力与SLE病情活动评分无关,PAH的轻重与血液中自身抗体的出现无明显关系,PAH重者易有雷诺现象发生,而且与其他系统受累无关联,比较有无肾脏受累的患者在临床以及实验室指标的不同,没有明显差别.结论 SLE患者PAH发生与其他脏器受累无关,发病隐匿,临床上应该重视SLE肺动脉压的筛查,早期发现和治疗以利于病情的控制和改善预后.     

系统性红斑狼疮患者抗u1RNP抗体检测的临床意义

目的探讨系统性红斑狼疮(SLE)患者抗u1RNP抗体阳性的临床意义。方法对2006年6月～2009年6月在我院住院的抗u1RNP抗体阳性的46例SLE患者的临床资料进行分析,并与同期抗u1RNP抗体阴性的SLE患者62例临床资料进行比较。结果抗u1RNP抗体阳性组患者的雷诺现象、关节炎、心肌缺血、肺动脉高压的发生率较高,肾损害程度较轻,抗Sm抗体阳性率较高,两组差异有统计学意义,发热、皮疹、口腔溃疡,血液系统损害,ANA、ENA、ds-DNA阳性率差异无统计学意义。结论抗u1RNP抗体与关节炎、雷诺现象,心肌缺血、肺动脉高压及抗Sm抗体高阳性率密切相关。     

系统性硬化症合并脑血管病变1例

<正>系统性硬化症(systemic sclerosis,SSc)主要病理过程包括纤维化、自身免疫性炎性反应及微血管病变。微血管病变以雷诺现象、指溃疡、肺动脉高压、肾危象等相对常见[1],其病理表现以非炎性血管内皮增生为主[2],称为血管病。不同于系统性红斑狼疮等其他结缔组织病易合并血管炎,SSc合并血管炎并不常见,主要为抗中性粒细胞胞浆抗体相关血管炎,其次是冷球蛋白血症性血管炎,多合并肾脏受累或肺动     

Interstitial lung disease increases mortality in systemic sclerosis patients with pulmonary arterial hypertension without affecting hemodynamics and exercise capacity

Published data suggest that coexisting interstitial lung disease (ILD) has an impact on mortality in patients with systemic sclerosis (SSc) and pulmonary arterial hypertension (PAH), but there is scarce knowledge if this is reflected by hemodynamics, exercise capacity, autoantibody profile, or pulmonary function. In this partially retrospective study, 27 SSc-PAH patients were compared to 24 SSc-PAH patients with coexisting ILD respecting to survival, pulmonary function, hemodynamics, exercise capacity, and laboratory parameters. Survival was significantly worse in SSc-PAH-ILD patients than in SSc patients with isolated PAH (1, 5, and 10-year survival rates 86, 54, and 54% versus 96, 92, and 82%, p = 0.013). Compared to isolated SSc-PAH patients, patients with SSc-PAH-ILD revealed lower forced expiratory volume after 1 s (FEV1) values at the time of PAH diagnosis as well as 1 and 2 years later (p = 0.002) without significant decrease in the PAH course in both groups. At PAH diagnosis, diffusion capacity for carbon monoxide (DLCO) values were lower in the ILD-PAH group. Coexisting ILD was not associated with lower exercise capacity, different FEV1/forced vital capacity (FVC) ratio, higher WHO functional class, or reduced hemodynamics. Higher levels of antibodies against angiotensin and endothelin receptors predict mortality in all SSc-PAH patients but could not differentiate between PAH patients with and without ILD. Our study confirmed an impact of ILD on mortality in SSc-PAH patients. Pulmonary function parameters can be used to distinguish PAH from PAH-ILD. The higher mortality rate cannot be explained by differences in hemodynamics, exercise capacity, or autoantibody levels. Mechanisms of mortality remain to be studied.     

Role of N-terminal brain natriuretic peptide (N-TproBNP) in scleroderma-associated pulmonary arterial hypertension.

AIMS: The aims of this study were to evaluate the diagnostic value and to explore the prognostic value of N-terminal brain natriuretic peptide (N-TproBNP) in patients with systemic sclerosis (SSc) both with and without pulmonary arterial hypertension (PAH). METHODS AND RESULTS: N-TproBNP, six-minute walk distance (SMWD), haemodynamics (at right heart catheterization) or tricuspid gradient (by echocardiography), and survival were assessed in 109 patients with SSc. The study population included 68 individuals with PAH [mean pulmonary artery pressure (PAP) >25 mmHg and pulmonary capillary wedge pressure <15 mmHg] and 41 individuals without PAH. In patients with PAH, the prognostic value of baseline and change in WHO functional class, N-TproBNP levels, and SMWD were compared using Kaplan-Meier survival curves and Cox proportional hazard analysis. The mean duration of follow-up was 10 months (range 1-18 months). One year survival in patients with normal PAP was 100% when compared with 83.5% in those with SSc-PAH (P < 0.05). The patients without PAH had a mean N-TproBNP level of 139 pg/mL (SD 151); those with SSc-PAH had a significantly higher mean N-TproBNP level of 1474 pg/mL (SD 2642) (P = 0.0002). Among patients with PAH for every order of magnitude increase in N-TproBNP level there was a four-fold increased risk of death (P = 0.002 for baseline level and P = 0.006 for follow-up level). Baseline N-TproBNP levels were correlated positively with mean PAP (r = 0.62; P < 0.0001), pulmonary vascular resistance (PVR) (r = 0.81; P < 0.0001), and inversely with SMWD (r = -0.46; P < 0.0001). Among patients with SSc-PAH, 13 patients (19%) were in WHO functional classes II and had mean N-TproBNP levels of 325 pg/mL (SD 388). Fifty-three patients (78%) were in WHO classes III and IV and had significantly higher mean N-TproBNP levels of 1677 pg/mL (SD 2835) (P = 0.02). At an N-TproBNP level of 395 pg/mL, the sensitivity and specificity for predicting the presence of SSc-PAH were 56 and 95% respectively. CONCLUSION: Raised N-TproBNP levels are directly related to the severity of PAH. In screening programs, SSc patients with an N-TproBNP in excess of 395 pg/mL have a very high probability of having pulmonary hypertension. Baseline and serial changes in N-TproBNP levels are highly predictive of survival. A 10-fold increase in N-TproBNP level on therapy is associated with a greater than three-fold increase in mortality, and may indicate therapeutic failure.     

Increased serum levels of adhesion molecules ICAM-1 and VCAM-1 in systemic sclerosis are not specific for pulmonary manifestations

Studies suggest elevated serum intercellular adhesion molecule-1 (ICAM-1) and vascular cell adhesion molecule-1 (VCAM-1) levels may be markers of pulmonary arterial hypertension in systemic sclerosis (SSc-PAH). We sought to evaluate whether ICAM-1 and VCAM-1 levels are useful screening biomarkers for incident SSc-PAH. In this cross-sectional study, four groups were selected from the Australian Scleroderma Cohort Study: group 1 (n?=?15) had definite PAH; group 2 (n?=?19) had interstitial lung disease (ILD); group 3 (n?=?30) were SSc-controls; and group 4 (n?=?34) were healthy controls. Serum ICAM-1 and VCAM-1 levels were measured using the Millipore Milliplex MAP Human 2-Plex Panel. There were no differences in ICAM-1 levels in the PAH versus ILD group (263.0?±?85.4 vs 380.4?±?168.3 ng/mL, p?=?0.136), SSc-controls (263.0?±?85.4 vs 253.1?±?98.0 ng/mL, p?=?1.00), or healthy controls (263.0?±?85.4 vs 201.8?±?57.2 ng/mL, p?=?0.093). Similarly, there were no differences in VCAM-1 level in PAH versus ILD groups (1476.2?±?434.9 vs 1424.8?±?527.6 ng/mL, p?=?1.00) and SSc-controls (1476.2?±?434.9 vs 1409.5?±?341.1 ng/mL, p?=?1.00). SSc subjects had significantly higher levels of ICAM-1 (297.4?±?134.0 vs 201.8?±?57.2 ng/mL, p?<?0.0001) and VCAM-1 compared to healthy controls (1432.7?±?427.4 vs 1125.6?±?273.4 ng/mL, p?<?0.0001). Neither ICAM-1 nor VCAM-1 is a specific screening biomarker of SSc-PAH. Instead, increased levels of these adhesion molecules in SSc, irrespective of pulmonary complications, suggest that they may play a role in SSc pathogenesis.     

An Update on Systemic Sclerosis-Associated Pulmonary Arterial Hypertension: a Review of the Current Literature

Purpose of Review

This review will summarize the most current literature on the clinical impact, epidemiology, risk factors, screening recommendations, predictors of outcomes, and treatment options in patients with pulmonary arterial hypertension (PAH) associated with systemic sclerosis (SSc).

Recent Findings

PAH continues to be a major cause of morbidity and mortality in SSc. Many risk factors and predictors of outcomes have been identified in patients with SSc including clinical, hemodynamic, and laboratory parameters. Screening for PAH in SSc patients is important and screening algorithms have been developed. Despite many available treatment options for PAH, prognosis remains poor.

Summary

Awareness of risk factors, early detection, and up-front combination treatment are important considerations in SSc-PAH and may lead to improved outcomes. Further research to develop better biomarkers and therapies is needed to continue to improve survival and outcomes in patients with SSc-PAH.

     

Pulmonary survival study in 91 patients with systemic sclerosis

In systemic sclerosis (SSc), major determinant of morbidity and mortality is pulmonary complication including pulmonary interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH). In this study, the natural course of pulmonary involvement in SSc patients was investigated. This was a historical cohort study of SSc patients at a referral center for SSc in Iran between February 1998 and December 2007. Patients had a standardized initial evaluation, and interstitial pulmonary involvement was established by high-resolution CT scan (HRCT). Pulmonary hypertension was assessed by tricuspid gradient on echocardiography. Development of abnormal FVC or DLCO was considered as secondary outcome. Analysis of pulmonary survival was performed for primary and secondary outcomes. Ninety-one SSc patients were included in the study with the mean age of 44.1 (14.8). Among these, 65 (71.4%) patients were classified as limited subtype (lcSSc) and 84 (93.3%) were women. PAH was investigated in 8 (8.2%) patients, 1 (6.7%) in dcSSc and 7 (15.9%) in lcSSc subtype of disease. ILD had developed after a median of 107 (SE = 24.4) months after the first symptom of SSc, and 29 patients (31.9%) developed pulmonary fibrosis. Alveolitis and fibrosis had developed after a median of 129.0 (22.9) and 259.0 (74.2) months, respectively. There was a significant difference in Alveolitis-free pulmonary survival between two subgroups of the disease, which showed pulmonary alveolitis developed later in limited SSc (P = 0.03). The difference was not significant in two subtypes when Cox regression model was used to identify the effect of other prognostic factors on pulmonary survival in patients. In the present study, clinical manifestations of two subtypes of disease were divergent at first; however they became convergent in late stages, and this was the same as results in previous studies. Echocardiography for evaluation of pulmonary hypertension and pulmonary function tests for early detection of ILD and PAH is recommended for SSc patients to detect early stages of pulmonary involvement before significant vascular and fibrotic changes occur.     

Screening for pulmonary arterial hypertension in systemic sclerosis: A systematic literature review.

Pulmonary arterial hypertension (PAH) carries a high morbidity and mortality burden in Systemic Sclerosis (SSc). Therefore, PAH screening and early detection are pivotal. A systematic literature review (SLR) to search for all screening tools and modalities for SSc-PAH was performed in reference to right heart catheterization as diagnostic gold standard. Papers from 2 previously published SLRs and derived from a systematic search on Pubmed, EMBASE, Web of Science for papers published from 03/10/2017 to 31/12/2018 were manually included. A total of 199 papers were reviewed and 32 were extracted, with a low bias risk according to QUADAS2. Echocardiography, pulmonary function tests, clinical features and serum biomarkers were the most frequently tools used for screening, with different parameters combined in a variable fashion, as single item or as part of composite algorithms. Among the composite algorithms, the DETECT score, ESC/ERS 2009 or 2015 guidelines, ASIG and ITINER-air algorithms were the most commonly used in a wide range of patients. In different cohorts, DETECT and ASIG showed higher sensitivity and negative predictive value than ESC/ERS 2009. In conclusion, the literature shows echocardiography as the leading screening tool for SSc-PAH. In particular, systolic pulmonary arterial pressure (sPAP) and tricuspid regurgitation velocity (TRV), both as single items or part of composite algorithms, including also serum biomarkers, clinical and functional items, are the most frequent parameters evaluated.     

Determinants of pulmonary arterial hypertension in scleroderma

OBJECTIVE: To define risk factors associated with pulmonary arterial hypertension (PAH) in a large cohort of patients with systemic sclerosis (SSc). METHODS: SSc patients undergoing screening for PAH by means of Doppler echocardiography were identified and their charts were retrospectively reviewed. In all patients, we recorded systolic pulmonary artery pressure along with pulmonary function testing, clinical, and laboratory data. PAH was defined as right ventricular systolic pressure equal or greater than 40 mm Hg. RESULTS: Of 114 SSc patients with echocardiographic measurements, PAH was found in 33 (29%) patients. In a multiple logistic regression analysis, the presence of pulmonary fibrosis on thoracic computed tomography (OR 6.78, CI 1.54 to 29.9), forced vital capacity less than 80% predicted (OR 3.03, CI 1.1 to 8.35), and duration of Raynaud's phenomenon preceding the onset of skin changes for at least 3 years (OR 5.75, CI 1.9 to 17.41) were found to be independent predictors of PAH. Age, disease duration, disease subtype, or autoantibodies were not associated with PAH in our patients. CONCLUSIONS: The present analysis identified pulmonary fibrosis and Raynaud's phenomenon preceding SSc skin manifestations by at least 3 years as risk factors for PAH in our scleroderma cohort. Screening for PAH in these high-risk patients may detect PAH at an earlier stage and guide decisions on therapeutic interventions.     

Red blood cell distribution width as a related factor of pulmonary arterial hypertension in patients with systemic sclerosis

The aim of this study was to investigate the utility of red blood cell distribution width (RDW) as a simple and readily available marker of occurrence of pulmonary arterial hypertension (PAH) in patients with systemic sclerosis (SSc). One hundred and forty-five consecutive patients with SSc were recruited to the single-center cross-sectional study. Demographic characteristics, hematological parameters, Modified Rodnan Skin Score, and World Health Organization functional classification were determined. Diagnosis of PAH was based on screening by echocardiography and was confirmed by right heart catheterization. Interstitial lung disease (ILD) was diagnosed based on chest high-resolution computed tomography findings. There were no significant differences in gender, age, or disease duration between limited and diffused SSc groups. PAH was detected in 28 of lcSSc (33.3%) and 14 of dcSSc (23.0%) subjects. Patients with higher RDW values were more likely to be men with high anti-u1RNP titers and PAH. A significant correlation was found between RDW and high-sensitivity C-reactive protein (p?=?0.375, p?<?0.01) and the diffusing capacity of the lungs for carbon monoxide (ρ?=???0.396, p?<?0.01). The SSc-PAH group had significantly higher RDW values compared to the SSc group without pulmonary disease (15.7?±?2.2 and 13.7?±?1.0, p?<?0.001). The mean RDW in the SSc-PAH-ILD group was significantly higher than that in the SSc-ILD group (16.3?±?2.2% and 14.0?±?1.5%, p?<?0.001). Besides the recognized risk factors, high RDW was an independent predictor of PAH in patients with SSc (OR?=?3.314 [95%CI 1.038–10.580], p?<?0.05). RDW may be a related factor for identifying the pulmonary arterial hypertension in SSc patients.     

Assessment of plasma endothelin level measurement in systemic sclerosis

PURPOSE: According to current knowledge, endothelin (ET)-1 plays an important role in the pathogenesis of systemic sclerosis (SSc). We assessed ET plasma levels in SSc patients according to the clinical presentation and the presence of complications such as pulmonary arterial hypertension (PAH). METHODS: Sixty-three consecutive patients with SSc were included. The control group included 17 healthy patients. ET plasma level was determined for all patients. Pulmonary function test and pulmonary high resolution computed tomography were performed in 44 patients and echocardiography in 51 patients, to screen for PAH, always confirmed by a right heart catheterization. RESULTS: ET plasmatic levels were higher in SSc patients than in healthy group subjects but the difference was not significant (3.72+/-1.13 vs 3.40+/-0.71 pmol/l, p=0.27). ET plasmatic levels were significantly higher in patients with PAH than in patients without PAH (4.28+/-0.65 vs 3.62+/-1.07 pmol/l, p=0.04) and in patients with anticentromere antibodies (3.96+/-1.11 vs 3.19+/-1.12 pmol/l, p=0.03). There was a positive linear correlation between ET plasmatic levels and systolic pulmonary arterial pressure (r=0.34, p=0.013). The best cut-off value for ET plasmatic level to discriminate patients affected by PAH was determined by ROC curve method: 4.1 pmol/l (sensibility 85.7%, specificity 66%). CONCLUSION: ET plasmatic levels were higher in SSc patients affected by PAH and patients with anticentromere antibodies. There was a positive linear correlation between ET plasmatic levels and systolic pulmonary arterial pressure. Assessment of ET plasmatic levels for detection and monitoring of pulmonary hypertension during SSc is warranted in larger prospective studies.