以肺动脉高压为首发症状的系统性红斑狼疮1例报告并文献复习

<正> 1 病例介绍患者,女,42岁。因"活动时气急三月余"于2009-02-25入我院,3个月前开始出现活动时气急,且伴有胸闷,常感倦怠、乏力,但休息5～6 min后病情可自行缓解,当时患者未给予重视,未经诊治,后病情有所发展,且夜间无明显诱因下出现胸闷、气急,遂于2009-02-24来我院就诊,拟"肺动脉高压"收住心内科,后转血液风湿科。患者发病以来除经常"感冒"外,无畏寒、发热、咳嗽、咳痰,无头晕、头痛、恶心、呕吐、胸痛,有脱发、雷诺现象。否认高血压、糖尿病、肝炎、结核史,否认遗传性家族疾病史。入院查体:神志清楚,自主体位,全身皮肤黏膜无黄染、瘀点、瘀斑,浅表淋巴结未及肿大,咽无充血,扁桃体无肿大,两肺呼吸音粗糙,左下肺可闻及少许湿啰音,心率90次/min,律整,肺动脉瓣区第二心音亢进伴分裂,余瓣膜区未闻及杂音,腹平软,肝脾肋下未触及,移动性     

系统性红斑狼疮并发肺动脉高压的研究进展

近年来对系统性红斑狼疮并发肺动脉高压越来越加以重视，本文就其诊断与治疗进展作一详细介绍。     

系统性红斑狼疮致重度肺动脉高压1例

正临床资料患者28岁,女性,主因:"发作性胸闷憋气6个月,加重1天"入院。患者自6个月前间断出现活动时胸闷憋气,多于爬楼、上坡时出现,伴心悸,无胸痛,每次持续数分钟可缓解,休息时无不适,无夜间阵发性呼吸困难,未予以重视及诊治,活动耐量逐渐减低,1 d前胸闷憋气加重来我院急诊,行超声心动图示:右心增大,三尖瓣重度反流,肺动脉高压(pulmonary hypertension,PH)(重度),肺动脉平均压     

系统性红斑狼疮并肺动脉高压的研究进展

系统性红斑狼疮 (SLE)是一种累及全身各器官系统的自身免疫性疾病 ,其合并的肺动脉高压 (PAH )起病隐袭 ,进展较快 ,预后较差 ,死亡率高 ,已渐引起关注。本文就SLE并肺动脉高压的发生率、可能的发病机制、常见临床表现、辅助检查发现、诊断治疗及预后诸方面的国内外研究现状作一总结分析。     

系统性红斑狼疮并发肺动脉高压44例临床分析

系统性红斑狼疮(SLE)作为一种多系统自身免疫性疾病,具有不同的临床表现形式,可影响机体器官和系统,其中肾脏、心脏和肺是受累最多的器官[1]。有研究报道显示,SLE并发肺动脉高压(PAH)患者自发现PAH起2年内死亡率高达25%~50%[2]。关于SLE并发PAH的比例各国学者报道结果不一,但均认为PAH是SLE患者死亡率的独立危险因素,并且是导致SLE患者死亡率增加的最主要原因[3-4]。导致SLE患者出现PAH的具体原因目前尚未清楚。早期发现及治疗对SLE并发PAH患者的预后具有重要意义[3]。我们对本院收治的114例SLE患者进行回顾性研究分析,探讨SLE并发PAH的相关危险因素,以期能够为早期识别和治疗SLE并发PAH患者提供依据。     

系统性红斑狼疮相关肺动脉高压

系统性红斑狼疮(SLE) 是一种以多器官系统受累为主要特征的自身免疫性疾病,肺动脉高压(PAH) 是其常见 和严重并发症。中国导致PAH 最常见的结缔组织病是SLE,自身免疫反应及炎症是其发生和进展的主要因素。临 床表现包括SLE 本身和PAH 两个方面。SLE 相关PAH 患者的预后较其他结缔组织病相关PAH 患者更好,因为针 对原发病SLE 的免疫抑制治疗有效。针对PAH 的靶向药物对于SLE 相关PAH 的治疗有效,尤其用于免疫制剂治 疗无效的患者。早期发现和恰当治疗是改善患者预后的关键。     

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     

以肺动脉高压为主要表现的系统性红斑狼疮一例

患者，女，34岁，已婚，农民。因活动时气促4年，加重1年于2004年2月17日入院。患者4年前元明显诱因逐渐出现活动时气促，经休息可缓解，体力明显下降。在家未作治疗，患者气促呈逐渐加重趋势，特别是近1年来气促明显加重，稍活动即感气促，并出现下午双下肢浮肿，脱发，反复咳嗽、咳少许白色黏液样痰。无发热、皮疹、关节肿痛，无少尿及肉眼血尿，无口腔溃疡。门诊做心脏B超检查后以“心脏扩大原因待查”收入我院心内科。     

以肺动脉高压首发的系统性红斑狼疮1例

患者：女,21岁,未婚,学生。因2周内反复发作晕厥3次入院。患者于入院前2周在乘地铁时无明显诱因出现黑噱,继之摔倒,1min左右清醒,1周后在家中再次晕厥,发作前有胸闷、乏力,无心悸,发作时伴牙关紧闭,双上肢抽搐,无大、小便失禁。     

系统性红斑狼疮相关肺动脉高压的诊治进展

肺动脉高压(pulmonary arterial hypertension,PAH)是一类病因多种、发病机制复杂而预后极差的疾病。PAH定义为:休息状态下平均肺动脉压(mPAP)>25mmHg,肺动脉楔压(pulmonary arterial wedge pressure,PAWP)或左心室舒张末压<15mm-Hg和肺动脉阻力增加[1]。2008年Dana Point会议对PAH进行了新的分类,其中危险因素相关性肺动脉高压包括:结缔组织疾病等,见表1(1～4)。PAH     

Systemic lupus erythematosus and thrombotic thrombocytopenic purpura: a case report and literature review

We describe a patient with systemic lupus erythematosus (SLE) who developed severe and acute thrombotic thrombocytopenic purpura (TTP). Detection of the fragmentation of peripheral red blood cells (RBC) helped the early diagnosis of TTP and the patient was rescued by extensive plasma exchange started promptly after the diagnosis. Because manifestations of TTP are similar to those in SLE, it is sometimes difficult to make an accurate diagnosis of TTP in SLE patients. We emphasise here the significance of the early diagnosis of TTP by the observation of fragmented RBC and the intensive therapy, including plasma exchange, for this very severe condition.     

Primary pulmonary hypertension associated with systemic lupus erythematosus

Summary A 24-year-old West Indian woman with a four-year history of systemic lupus erythematosus presented with progressive dypsnoea due to primary pulmonary hypertension. Despite showing a response to vasodilators, these failed to control the condition. Her pulmonary hypertension increased in severity, eventually resulting in her death. We believe primary pulmonary hypertension to be an unusual complication of systemic lupus erythematosus. We suggest that this diagnosis should be considered in all patients with systemic lupus erythematosus and progressive dypsnoea, as optimum benefit can only be obtained by early institution of vasodilator therapy.     

Corticosteroid responsive pulmonary hypertension in systemic lupus erythematosus

Summary Primary pulmonary hypertension is an irreversible and fatal disorder. Every effort should therefore be made to discover all the other treatable diseases which may be associated with pulmonary hypertension. The association of systemic lupus erythematosus and pulmonary hypertension was rarely reported in the past. We add another case in which pulmonary hypertension was the presenting symptom of systemic lupus erythematosus (SLE). In contrast to the previously reported cases, our patient responded well to corticosteroids. It is assumed that this favorable response was due to the relatively early stage of the disease, when the histopathologic pulmonary changes were still in the reversible inflammatory stage.     

Silica-associated systemic lupus erythematosus with lupus nephritis and lupus pneumonitis: A case report and a systematic review of the literature

IntroductionSeveral epidemiological studies have shown that silica exposure triggers the onset of systemic lupus erythematosus (SLE); however, the clinical characteristics of silica-associated SLE have not been well studied.Patient concernsA 67-year-old man with silicosis visited a primary hospital because of a fever and cough. His respiratory condition worsened, regardless of antibiotic medication, and he was referred to our hospital.DiagnosisThe patient showed leukopenia, lymphopenia, serum creatinine elevation with proteinuria and hematuria, decreased serum C3 level, and was positive for anti-double stranded DNA antibody, anti-nuclear antibody, and direct Coombs test. He was diagnosed with SLE. Renal biopsy was performed, and the patient was diagnosed with lupus nephritis (class IV-G(A/C) + V defined by the International Society of Nephrology/Renal Pathology Society classification). Computed tomography revealed acute interstitial pneumonitis, bronchoalveolar lavage fluid showed elevation of the lymphocyte fraction, and he was diagnosed with lupus pneumonitis.InterventionsPrednisolone (50 mg/day) with intravenous cyclophosphamide (500 mg/body) were initiated.OutcomesThe patient showed a favorable response to these therapies. He was discharged from our hospital and received outpatient care with prednisolone slowly tapered off. He had cytomegalovirus and herpes zoster virus infections during treatment, which healed with antiviral therapy.Review:We searched for the literature on sSLE, and selected 11 case reports and 2 population-based studies. The prevalence of SLE manifestations in sSLE patients were comparative to that of general SLE, particularly that of elderly-onset SLE. Our renal biopsy report and previous reports indicate that lupus nephritis of sSLE patients show as various histological patterns as those of general SLE patients. Among the twenty sSLE patients reported in the case articles, three patients developed lupus pneumonitis and two of them died of it. Moreover, two patients died of bacterial pneumonia, one developed aspergillus abscesses, one got pulmonary tuberculosis, and one developed lung cancer.ConclusionClose attention is needed, particularly for respiratory system events and infectious diseases, when treating patients with silica-associated SLE using immunosuppressive therapies.     

Clinical aspects of pulmonary hypertension in patients with systemic lupus erythematosus and in patients with idiopathic pulmonary arterial hypertension

Clinical aspects and pathology of pulmonary hypertension (PH) in patients with systemic lupus erythematosus (SLE) have been reported to be similar to those in patients with idiopathic pulmonary arterial hypertension (IPAH). To determine whether PH in these patients is similar, we compared the clinical characteristics, hemodynamics at diagnosis, and survival in groups of patients with SLE–PH and IPAH. We reviewed the case records of 20 patients with SLE–PH and 34 patients with IPAH, who had been assessed by echocardiography or right cardiac catheterization at Asan Medical Center, Seoul, Korea, from January 1995 to October 2003. Clinical features, laboratory data, chest X-rays, electrocardiogram results, pulmonary function tests, pulmonary perfusion scans, echocardiographic findings, serologic profiles, and survival were compared in the two groups of patients. The mean follow-up period was 18.1±20.6 months for patients with SLE–PH and 33.0±23.4 months for patients with IPAH. During follow-up, 12 SLE–PH (60%) and 11 IPAH (32%) patients died. For SLE–PH, the 3-year survival rate was 44.9% and the 5-year survival rate was 16.8%. For IPAH, the 3-year survival rate was 73.4% and the 5-year survival rate was 68.2% (p=0.02). There were no other significant differences in clinical characteristics and laboratory data between the two groups. In contrast to previous reports that the prognosis of patients with pulmonary arterial hypertension associated with collagen vascular disease was better than that of patients with IPAH, we found that the prognosis of patients with SLE–PH was much worse than that of patients with IPAH.     

Systemic lupus erythematosus in identical twins: a case report

In this report we describe the case of identical twin sisters that developed systemic lupus erythematosus (SLE). These patients have in common major histocompatibility complex class I and class II alleles and identical red blood cell antigens, which is a clear indication of monozygotic twins. Both twins showed high titers of anti-dsDNA antibody. However, only one of them manifested signs of lupus psychosis and was positive for the LE test, rheumatoid factor, anti-Scl 70, anti-SSA, and antiribosomal P antibodies. Both sisters lived together; therefore, the environmental factors were considered to be the same. Interestingly, these patients expressed different types of autoantibodies and the manifestation of disease was also quite different. When one of the twins was diagnosed with SLE, we began to closely follow up signs of the disease in the other twin periodically. This enabled us to promptly diagnose the second twin with SLE and she was successfully treated without progression of the disease. It is important to mention that following up the subsequent history of an identical twin diagnosed with SLE allowed early detection of the disease in the other twin.     

Systemic lupus erythematosus presenting as cardiac tamponade-a case report

Although pericarditis and pericardial effusion (PE) are some of the common manifestations of systemic lupus erythematosus (SLE), the occurrence of cardiac tamponade is quite rare. We present herewith a young girl with cardiac tamponade presenting as initial manifestation of SLE.     

Low-dose epoprostenol improved pulmonary hypertension in a patient with systemic lupus erythematosus

A 43-year-old Japanese woman was referred to our hospital in 1997 because of Raynaud’s phenomenon. Systemic lupus erythematosus was diagnosed on the basis of the presence of antinuclear antibody (1:1,280), anti-DNA antibody (1:640), anti-Sm antibody, antiphospholipid antibody, lymphopenia, and proteinuria. She developed pulmonary fibrosis in 1999 and pulmonary hypertension in 2001. In October 2002, a 24-hr continuous infusion of epoprostenol was started. Dyspnea, Raynaud’s phenomenon, and pulmonary hypertension improved with low-dose epoprostenol (3.0 to 4.0 ng kg⁻¹ min⁻¹). The patient could not tolerate larger doses of epoprostenol so 4.0 ng kg⁻¹ min⁻¹ was selected as the maintenance dose. The clinical course was uneventful at this dosage. It appears that pulmonary hypertension can be controlled with low-dose epoprostenol such as 3.0 to 4.0 ng kg⁻¹ min⁻¹ in some rheumatic patients.     

单纯性左侧肺动脉缺如一例报道及文献复习

目的 提高对单纯性单侧肺动脉缺如(UAPA)的临床表现、病理生理、诊断和治疗的认识.方法 报道上海市静安区中心医院呼吸科诊治的1例单纯性左侧肺动脉缺如患者的相关资料,并结合从多个中文期刊数据库检索到的关于单纯性UAPA的病例资料进行回顾性研究.结果 44例患者平均31.5岁(3个月～69岁),主要症状有反复肺部感染(47.4％)、咯血(52.6％)、气喘或活动后气促(57.9％)、胸闷(31.6％)、心悸(18.4％)、胸痛(7.9％)等,有1例患者没有症状.43.2％(19/44)的患者存在肺动脉高压.CT肺动脉造影(CTPA)、MRI、电子束CT (EBCT)、血管造影(DSA)均有确诊价值.20.5％的患者施行了手术治疗.结论 单纯性UAPA是一种非常罕见的先天性畸形,其症状无明显特异性,易漏诊、误诊.可通过CTPA、MRI、EBCT、DSA等确诊.最理想的手术方法是患侧肺动脉重建术,但在我国还无法实行.