系统性硬化症合并肺动脉高压研究进展

以前系统性硬化症主要的死亡原因是肾危象，但随着血液净化治疗的进步，处理肾危象已经不再是那么高不可攀。目前系统性硬化症最严重的并发症和死亡原因是肺动脉高压，其在系统性硬化症中的发病率高达10％～30％，在局限性系统性硬化症中甚至更高，系统性硬化症合并肺动脉高压患者2年生存率仅为40％～55％。这使得越来越多的学者对系统性硬化症合并肺动脉高压的机制、临床评价、治疗等进行研究，取得了一些有开创意义的成果。     

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.     

结缔组织疾病合并肺动脉高压

结缔组织疾病合并肺动脉高压并非少见,近年来报道日趋增多.早期诊断较为困难,早期肺动脉高压服用血管扩张剂和氧疗有效,重度则无效,预后较差.本文对其发病机理、临床表现、诊断及治疗等方面的进展作一综述。     

老年性系统性硬化病合并肺动脉高压的超声特点

<正>系统性硬化病(SSc)是老年风湿性疾病中较少见但较为严重的一种疾病。肺部病变常表现为肺动脉高压(PAH)及肺间质纤维化。因PAH早期大多数患者无相应临床表现,故早期发现SSc患者PAH,对治疗和预后有重要价值。超声心动图是目前我国检查老年性SSc患者最简便及最常用的方法之一,本研究旨在探讨超声心动图检查在SSc患者中的表现及价值。     

胶原性疾病合并肺动脉高压

肺动脉高压的病因极其复杂,本文重点讨论胶原性疾病合并肺动脉高压的临床特征及实验室结果,报道三例系统性红斑狼疮、类风湿性关节炎、混合性结缔组织病等并发肺动脉高压患者     

系统性硬化症相关性肺动脉高压的临床特点

目的 探讨系统性硬化症（SSc）合并肺动脉高压（PAH）的发病率、临床特点及评估指标.方法 分析本院确诊的18例系统性硬化症合并肺动脉高压的患者临床资料和诊治过程,并以同期确诊的系统性硬化症（未合并肺动脉高压）患者作为对照.结果 两组患者在性别比和年龄上并无统计学差异.但SSc合并PAH组（SSc-PAH）患者在肺动脉收缩压（sPAP）、雷诺现象发生率、肺间质性改变发生率等方面明显高于SSc组.此外,SSc-PAH组患者的RNP抗体阳性率、IgG水平显著高于对照组.结论 雷诺现象、肺间质性改变、RNP抗体、血清IgG水平与SSc患者合并PAH密切相关.这类患者应及早行心超检查,以求早期诊治,改善预后.     

系统性硬化病合并肺动脉高压临床特点及易患因素分析

目的 分析系统性硬化病合并肺动脉高压(PHT)发生率及其危险因素。方法 对1997—2002年合并PHT的28例系统性硬化病患者临床特点、实验室检查以及治疗后病情变化资料进行回顾性研究，并对照同期住院的未合并PHT的73例系统性硬化病患者资料相比较。结果共有28例系统性硬化病患者合并PHT(27．7％)，其中20例患者为血管源性PHT，8例患者为由于严重的肺纤维化导致的肺源性PHT。PHT的发生多起病隐匿，与病程无相关性。与未合并PHT患者相比，合并PHT患者血清中白蛋白、γ-球蛋白、IgA、IgG以及C反应蛋白(CRP)水平均明显高于前者。并且肢端溃疡／指端凹陷性瘢痕、肌酶升高、肾脏受累、食道钡餐及ECG检查异常、重度以上的甲襞微循环异常以及实验室检查有高滴度ANA、RNP阳性结果发生率也较前者明显增多。结论 肺纤维化并非PHT发生的决定因素，但同时合并有肺纤维化的患者预后不良。对于临床上有肢端溃疡／指端凹陷性瘢痕、肌酶升高、肾脏受累、食管钡餐及ECG异常、重度以上的甲襞微循环异常等临床表现的系统性硬化病患者，建议早期行心脏超声检查，以求早期诊断、早期治疗，从而改善患者预后。     

系统性硬化症相关肺动脉高压兔的目征微循环改变

目的观察系统性硬化症相关性肺动脉高压(SSc-PAH)兔的目征改变及特性。方法将30只新西兰幼兔按随机分组法分为溶剂对照组、造模组、激素治疗组,3 w后分别观察每组的目征微循环变化。结果存活兔25只,死亡5只,其中溶剂对照组1只,造模组2只,激素组2只,且造模组肺动脉压力值均高于30 mm Hg。溶剂对照组、造模组与激素组3组两两之间目征微循环总分差异均有统计学意义(P<0. 05),且造模组与激素组均高于溶剂对照组,造模组显著高于激素组;在具体分项积分中,造模组与溶剂对照组差异有统计学意义(P<0. 05),而造模组与激素组、激素组与溶剂对照组的目征微循环亚组间差异无统计学意义(P>0. 05);目征形态学改变,造模组和激素组可见血管增粗、血管扭曲、血管网状畸形、血管瘤或血管怒张,此外,造模组还可见血管色调改变。结论 SSc-PAH模型兔的目征微循环相较于正常兔有明显的改变,提示为其可作为早期预测SSc-PAH的手段之一。     

妊娠合并肺动脉高压的诊治

妊娠合并心脏病是产科的严重合并症,是孕产妇四大死亡原因之一,居非产科因素死亡第一位。重度肺动脉高压（pulmonary artery hypertension,PAH）,尤其是艾森曼格氏综合征是重症心脏病,母婴病死率极高,产妇病死率可高达50%,治疗性流产、早产明显高于正常孕妇,低体质量儿、畸形率均高于正常。     

系统性红斑狼疮相关肺动脉高压的诊治

系统性红斑狼疮(systemic lupus erythematous,SLE)是一种以多器官系统受累和多种自身抗体阳性为主要特征的系统性自身免疫性疾病。肺动脉高压(pulmonary arterial hypertension,PAH)是指静息状态下,右心导管检查发现平均肺动脉压(mPAP)≥3.3 kPa,肺血管阻力(PVR)≥3个Wood单位[1个Wood单位=8 kPa/(L·s)],且毛细血管楔压≤2 kPa     

Pulmonary hypertension in collagen vascular disease.

Pulmonary hypertension is a serious but often overlooked complication in collagen vascular disease. The understanding of the development of pulmonary hypertension has increased substantially during the last years. Abnormal proliferation of pulmonary vascular cells is now being regarded as a predominant process leading to pulmonary vascular obliteration. Medical therapy focuses on prostacyclin treatment, which has been shown to improve exercise capacity and haemodynamic variables in patients with several collagen vascular diseases and pulmonary arterial hypertension. Continuous intravenous prostacyclin remains the standard treatment of associated pulmonary hypertension but less invasive alternatives such as subcutaneous treprostinil, oral beraprost or aerosolized iloprost, as well as, novel substances such as endothelin receptor antagonists may be appropriate for selected patients.     

Pulmonary hypertension in the collagen vascular diseases

Pulmonary hypertension is increasingly recognized as a complication of the collagen vascular diseases. Both isolated and secondary forms of the disease may be appreciated. Pulmonary hypertension is observed with high prevalence in scleroderma and mixed connective tissue disease. It is less commonly seen in systemic lupus erythematosus, and is a rare clinical finding in rheumatoid arthritis. The diagnosis and treatment of pulmonary hypertension associated with the collagen vascular diseases mirror diagnosis and treatment for pulmonary hypertension of any etiology. Vasodilator therapy may play a particularly important role in patients with the isolated form of disease, which is most frequently associated with scleroderma.     

Pulmonary vascular disease in secundum atrial septal defect with pulmonary hypertension

Histopathologic and morphometric studies of small pulmonary arteries were performed in 16 cases of secundum atrial septal defect (ASD) and severe pulmonary hypertension. Besides typical plexogenic arteriopathies found in six cases, organized microthrombi and what we call "musculoelastosis," that is, proliferation of longitudinal smooth muscle bundles and elastic fibers, were seen in small pulmonary arteries in three and four cases, respectively. These changes were observed co-existing in the remaining three cases. Thrombi of the small pulmonary arteries and musculoelastosis were the forms of pulmonary vascular disease characteristically found in the older patients with both ASD and pulmonary hypertension. Yamaki's index of pulmonary vascular disease, though effective in describing severity of plexogenic arteriopathy, proved to be less so for the intimal lesions in old microthrombi and for musculoelastosis. Among the cases with plexogenic arteriopathy, there was a positive significant correlation between the medial thickness of small pulmonary arteries and peak arterial pressure, which, however, was not demonstrated when all the cases of ASD were included.