Fatal post-partum pulmonary vasculitis in systemic lupus erythematosus

Summary A patient with fatal acute pulmonary vasculitis complicating systemic lupus erythematosus (SLE) of ten years duration is described. The patient died seven days after an uneventful pregnancy and delivery. Pathologic examination demonstrated acute necrotizing changes as well as organized lesions in pulmonary arteries. Acute pulmonary arteritis is rare in SLE and may develop as one of the major complications of the disease in the potentially dangerous post-partum period.     

Catastrophic transverse myelitis in a patient with systemic lupus erythematosus

A 24-year-old Japanese woman was admitted to our hospital suffering from high fever and progressive paralysis in both legs. Magnetic resonance imaging of the spinal cord showed high-intensity signals from C5 to Th4 and from Th7 to L1 on T2-weighted images. The patient was diagnosed as having acute transverse myelitis , which was a complication of systemic lupus erythematosus based on the serological findings. Despite aggressive immunosuppressive treatments including corticosteroid pulse therapy, plasmapheresis, and intravenous cyclophosphamide, the paralysis of her lower extremities did not improve. In the catastrophic type of lupus-associated TM, which develops extensively and longitudinally along the spinal cord, the prognosis still seems to be poor despite intensive treatments.     

系统性红斑狼疮合并横贯性脊髓炎八例临床分析

目的横贯性脊髓炎(transversemyelitis,TM)是系统性红斑狼疮(systemiclupuserythe-matosus,SLE)少见的严重合并症。本文报道8例并结合文献复习,对其临床表现、治疗方法、预后进行分析。方法回顾了本院1998—2005年的住院和门诊随访病例,报道8例SLE合并TM,并文献复习。结果8例(7例女性,1例男性)SLE发病年龄中位数为19岁(15￣32岁),SLE距TM发病的中位数时间为8个月(0￣12年),就诊时间距TM发病的中位数为3.3个月(0￣7年)。脊髓病变1例发生在颈髓,余7例均定位在胸髓(T1￣T9)。脊髓磁共振成像(MRI)有助于诊断,其中3/7例呈T2加权长条形病变,4/7例呈斑点状病变。其他临床表现、血清学(其中2例存在抗磷脂抗体)、脑脊液均无特征性提示。4/8例遗留明显的神经系统后遗症。其中3例进行了地塞米松鞘内注射治疗,仅1例有短暂疗效。另外4例完全恢复或基本恢复,其中3例在TM发生早期(<10d)接受了大剂量激素治疗,1例有自愈倾向。结论TM是SLE少见的严重合并症,倾向于早年、早期出现,多累及胸髓。早期积极治疗可能改善预后,肌力Ⅲ级以上者预后较好。     

Two UV-induced episodes of myelitis in a patient with systemic lupus erythematosus.

Two flares of acute myelitis were observed in a 31-year-old woman with previously known mild SLE. The two myelitic episodes both occurred within 1 week after intense sun exposure, and for this reason photobiological induction of the manifestation is considered likely. The diagnostic utility of MRI in the acute situation and the possible influence of sex-steroids on SLE manifestations are also illustrated by this case.     

Pulmonary hypertension in systemic lupus erythematosus

Systemic lupus erythematosus is associated with several forms of pulmonary hypertension. It can cause pulmonary hypertension through pulmonary thromboembolic disease, left heart disease and lung disease as well as causing an isolated pulmonary vasculopathy called pulmonary arterial hypertension. The true prevalence of pulmonary arterial hypertension in patients with lupus is not known but probably is no more than 1%. Currently, treatment for lupus-associated pulmonary arterial hypertension is with pulmonary vasodilators including phosphodiesterase-5 inhibitors, endothelin receptor antagonists and prostacyclin analogues, as it is for other causes of pulmonary arterial hypertension. Case series suggest there may be a special role for immunosuppression in this rare group of patients. We present two brief case histories and summarise our experience over 15 years. Prognosis is better in lupus-associated pulmonary arterial hypertension than in systemic sclerosis-associated pulmonary arterial hypertension, but unfortunately it remains a fatal condition in most patients.     

Anti-Ro antibody and cutaneous vasculitis in systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is a complex systemic autoimmune disease with various clinical and serological manifestations. Previous studies have shown the association of SLE and anti-Ro antibody with a series of clinical manifestations. We investigated this association in Brazilian patients with SLE. Five hundred and nine consecutive patients who fulfilled the revised American College of Rheumatology criteria for the SLE were enrolled in the study from June to December 2007. All patients were from our Service of Rheumatology, School of Medicine, University of São Paulo, Brazil. Frequencies of a series of laboratorial and clinical manifestations were calculated. Anti-Ro antibody was associated to anti-La antibody, female, and cutaneous vasculitis. In multivariate analysis, patients with anti-Ro antibody has 1.63 (95% CI 1.07–2.50) more risk to develop cutaneous vasculitis than patients without this antibody. Our data have demonstrated that anti-Ro antibody is an independent useful serologic marker for cutaneous vasculitis.     

Vasculitis of the gall bladder in rheumatoid arthritis and systemic lupus erythematosus

Summary Three cases of acute cholecystitis due to vasculitis of the gall bladder are described; one in a patient with rheumatoid arthritis, and two in association with systemic lupus erythematosus.     

Ischemic colitis associated with intestinal vasculitis： Histological proof in systemic lupus erythematosus

I schemic colitis is an uncommon complication in patients with systemic lupus erythematosus （SLE）. In previously reported cases of colitis caused by SLE, intestinal vasculitis is implicated as the causative process, but is rarely confirmed histologically. We described a case of a 32-year-old man with increased activity of SLE, who presented with hematochezia and abdominal pain due to ischemic colitis with small vessel vasculitis which was proven by sigmoidoscopic biopsy. The clinical course of the patient was improved after steroid and conservative management.     

Pulmonary hypertension in systemic lupus erythematosus

A prospective echocardiographic and clinical study was performed on 84 Chinese patients with systemic lupus erythematosus (SLE) and 99 controls to investigate the prevalence and the mechanism of pulmonary hypertension (PH) in SLE. Comparison between Doppler estimation and catheterization measurement was made in 12 cases to validate the predictive method. Compared to normal subjects, lupus patients had significantly increased sys-tolic pulmonary artery pressure (SPAP) (29.59±12.52 vs 19.64±5.82, P<0.001), mean pulmonary artery pressure (MPAP) (15.11±7.36 vs 10.21±4.72, P<0.001) and total pulmonary resistance (TPR) (315.85±190.65 vs 220.37± 55.92, P<0.001). Nine of the 84 patients presented PH, defined as SPAP >30 mmHg and MPAP >20 mmHg. Pulmonary hypertensive patients had higher serum endothelin (ET) than non-pulmonary hypertensive patients, were more commonly in active stages, and presented Raynaud's phenomenon and rheumatoid factors. ET level was correlated with echocardiographic pulmonary pressure. Pulmonary hypertension commonly occurs in Chinese patients with SLE (11%), and it correlates with the lupus activity and the elevation of serum endothelin. Received: 29 April 1998 / Accepted: 30 September 1998     

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.     

Transverse myelitis and optic neuritis in systemic lupus erythematosus: a case report with magnetic resonance imaging findings

We describe a patient with systemic lupus erythematosus who developed transverse myelitis and optic neuritis. Magnetic resonance imaging showed the presence of an abnormal signal in a normal-sized spinal cord which corresponded to the patient's neurologic deficit. No abnormality was recognized in either optic nerve. Magnetic resonance may prove to be a useful imaging modality for the diagnosis of a transverse myelopathy in systemic lupus erythematosus.     

Gastrointestinal involvement in systemic lupus erythematosus:Insight into pathogenesis, diagnosis and treatment

Systemic lupus erythematosus (SLE) is an autoimmune inflammatory disease characterized by the presence of a plethora of autoantibodies and immune complex formation. Virtually every system and organ can be affected by SLE. Gastrointestinal symptoms are com-mon in SLE patients, and more than half of them are caused by adverse reactions to medications and viral or bacterial infections. Though not as common as lu-pus nephritis, SLE-related gastrointestinal involvement is clinically important because most cases ...     

系统性红斑狼疮合并肺动脉高压的临床相关因素分析

目的探讨系统性红斑狼疮（SLE）患者易合并肺动脉高压的相关临床因素。方法37例SLE合并肺动脉高压的患者作为病例组,78例诊断为SLE而无合并肺动脉高压的患者为对照组。采用病例1对照研究的方法,对各个研究因素采用逐步引入一剔除法,建立Logistic回归模型。结果多因素Lo—gistic回归分析显示,SLE患者易发生肺动脉高压的独立危险因素包括雷诺现象、疾病活动、心悸气促症状、心包积液。结论雷诺现象、疾病活动、心悸气促症状、心包积液是SLE患者易并发肺动脉高压的独立危险因素,对于这4个指标阳性的患者要尽早进行心脏彩色多普勒检查,以进行早期诊断。     

Acute respiratory distress syndrome due to systemic lupus erythematosus with hemophagocytic syndrome: an autopsy report

This report concerns a patient with systemic lupus erythematosus (SLE) who died of acute respiratory distress syndrome (ARDS) 1 day after the onset of pulmonary symptoms. Autopsy demonstrated severe hemophagocytosis in the bone marrow and histopathology indicating a marked increase in vascular permeability in both lungs and kidneys. In this patient, active SLE and associated hemophagocytic syndrome may have induced an increase in the production of inflammatory cytokines, which immediately induced ARDS. Since fatal ARDS can occur as a life-threatening complication of SLE, careful observation is necessary, particularly when there are clinical findings suggestive of associated hemophagocytic syndrome.     

心率恢复评估系统性红斑狼疮相关肺动脉高压女性患者疾病严重程度的价值

目的 探讨心率恢复评估系统性红斑狼疮相关肺动脉高压(SLE-PAH)女性患者疾病严重程度的价值.方法 回顾性纳入2009年11月到2015年9月在同济大学附属上海市肺科医院住院治疗的21例SLE-PAH女性患者,对所有患者进行右心导管检查、肺功能测试及心肺运动试验,并以同期年龄、性别、体质量指数上匹配的32名女性健康志愿者为正常对照组进行对比分析.结果 与正常组相比,SLE-PAH患者的峰值公斤摄氧量(Peak VO2/kg)、峰值摄氧量占预计值百分比(PeakVO2％ pred)、峰值心率(Peak HR)、摄氧效率平台(OUEP)及OUEP％ pred显著下降(t=-8.59～-2.49,P＜0.05),最低通气效率(Lowest VE/VCO2)及Lowest VE/VCO2％ pred明显升高(t=4.85、5.48,P＜0.01),2组间基础心率差异无统计学意义(t=0.34,P＞0.05),而SLE-PAH患者的HRR1、HRR2[(19.1±7.4)次/min、(34.7±11.1)次/min]却显著低于正常对照组[(28.0±6.3)次/min、(42.0±8.3)次/min](t=-4.73、-2.73,P＜0.05).Pearson相关性分析发现SLE-PAH患者HRR1及HRR2与Peak VO2％ pred、OUEP％ pred、心输出量(CO)、心指数(CI)均呈中度正相关(r =0.47～0.69,P＜0.05),与Lowest VE/VCO2％ pred呈中度负相关(r=-0.62、-0.55,P＜0.05),与mPAP无相关性(r=-0.35、-0.11,P＞0.05).此外,HRR1还与肺血管阻力(PVR)呈弱正相关(r=-0.45,P＜0.05),而PVR与HRR2无相关性(r=-0.31,P＞0.05).ROC曲线显示HRR1及HRR2曲线下面积分别为0.807(敏感度84.4％,特异度71.4％)及0.676(敏感度68.8％,特异度71.4％)(P＜0.05),HRR1及HRR2在ROC曲线上的分界点分别为23次/min及40次/min.结论 HRR1及HRR2均可以用来评估SLE-PAH女性患者疾病严重程度.因此,我们可以把HRR作为早期发现SLE-PAH的一个重要参数,并对疾病严重程度进行评估从而判断预后.     

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.     

Circulating activated endothelial cells in systemic lupus erythematosus: further evidence for diffuse vasculopathy

OBJECTIVE: In flares of systemic lupus erythematosus (SLE), endothelial cells (EC; activated by immune stimuli) are potential participants in the inflammatory processes that contribute to tissue damage. Accordingly, elevated levels of circulating endothelial cells (CEC) may be a marker for vascular injury. This study was undertaken to examine the possibility that stimulated EC are found in the circulation in patients with active SLE. METHODS: The study cohort included 38 patients with SLE and 16 healthy controls. Immunostaining was performed on mononuclear isolates, using mouse P1H12 (endothelial-specific antibody) and rabbit antinitrotyrosine (a "footprint" of a reactive form of nitric oxide [peroxynitritel). RESULTS: Levels of CEC were significantly higher in patients with active SLE compared with those in healthy controls (mean +/- SEM 32+/-7/ml versus 5+/-2/ml; P = 0.0028) and were correlated positively with plasma C3a in these patients (r = 0.81, P = 0.0008). Furthermore, CEC from these patients expressed an activated phenotype, as indicated by staining for nitrotyrosine. CONCLUSION: Elevated levels of CEC observed in patients with active SLE may represent a marker of endothelial injury. The activated phenotype of these cells suggests that they may be capable of further potentiating vascular injury by the production of inflammatory and prothrombotic mediators and engaging in heterotypic aggregation with neutrophils or platelets.     

Tuberculosis infection in patients with systemic lupus erythematosus: pulmonary and extra-pulmonary infection compared

Systemic lupus erythematosus (SLE) patients had an increased susceptibility to tuberculosis (TB). The aim of this study was to investigate the prevalence and clinical characteristics of TB in SLE patients, with focus on the differences between pulmonary and extra-pulmonary TB. This is a retrospective study that reviewed the medical records of 3,179 SLE patients from 1985 to 2004. The diagnosis of TB was confirmed by one of the following: positive acid-fast bacillus (AFB) smear, positive culture of Mycobacterium tuberculosis from appropriate specimens, or a histopathologic finding of caseating granuloma on specimen. During the 20-year review period, TB was documented in 19 SLE patients, with 21 episodes. Ten of 21 episodes (47.6%) were pulmonary TB while the other 11 episodes (52.4%) were extra-pulmonary TB. Among extra-pulmonary TB, there were joint and cutaneous involvements in five, miliary in two, Pott’s disease in two, peritoneum in one, and spleen in one. The most common manifestations of TB were fever and cough. Delayed diagnosis and adverse effects of anti-TB therapy were observed in the extra-pulmonary TB group. While SLE patients commonly present with prolonged fever or chronic cough, tuberculosis infection should be taken into consideration.     

Pulmonary hypertension in systemic lupus erythematosus: Hemodynamics and effects of vasodilator therapy

A case is reported of a 29-year-old woman with systemic lupus erythematosus (SLE) who developed clinical manifestations of pulmonary hypertension at a time when other manifestations of SLE were quiescent. She had a restrictive ventilatory defect but clear lung fields on chest x ray. Cardiac catheterization revealed severe pulmonary hypertension. Calculated pulmonary vascular resistance fell slightly after administration of oxygen and during infusion of vasodilators. Symptomatic improvement and a modest increase in right ventricular ejection fraction, as measured by radionuclide ventriculography, were noted following 1 week of oral hydralazine therapy. Clinically significant pulmonary hypertension is a rare complication of SLE and the increased pulmonary vascular resistance may not be entirely fixed. Cardiac catheterization and radionuclide ventriculography may be useful in assessing response to medical therapy.