systemic lupus erythematous

系统性红斑狼疮
  • 文章类型: Case Reports
    肺收缩综合征(SLS)是一种罕见的肺部并发症,主要与自身免疫性疾病如系统性红斑狼疮(SLE)有关。一名38岁的女性最近被诊断为使用羟氯喹的SLE,泼尼松,甲氨蝶呤有一周的进行性呼吸急促史,非生产性咳嗽,胸膜炎性胸痛.她有足够的氧饱和度。检查发现肺部有几处细细的裂纹。实验室结果显示全血细胞减少。初始治疗包括广谱抗生素和静脉注射甲基强的松龙治疗疑似狼疮发作。感染的培养和测试,包括肺结核,是阴性的。影像学显示双侧空域疾病,无肺栓塞。自身免疫检查显示抗核抗体高,抗心磷脂抗体阳性,核糖核蛋白,和抗史密斯抗体。用SLS诊断,她开始逐渐减少甲基强的松龙和羟氯喹的剂量,还有利妥昔单抗,导致显著改善。肺功能测试(PFTs)显示出限制性模式。SLS,SLE的患病率非常低,也可能发生在系统性硬化症中,干燥综合征,和类风湿性关节炎。典型的症状包括呼吸困难,胸膜炎性胸痛,还有咳嗽.诊断包括胸部X线照相,显示隔膜升高和限制性PFT模式。治疗通常包括皮质类固醇如甲基强的松龙和免疫抑制剂。利妥昔单抗在对常规治疗无反应的病例中显示出改善。
    Shrinking lung syndrome (SLS) is a rare pulmonary complication primarily associated with autoimmune diseases such as systemic lupus erythematosus (SLE). A 38-year-old female recently diagnosed with SLE on hydroxychloroquine, prednisone, and methotrexate presented with a one-week history of progressive shortness of breath, non-productive cough, and pleuritic chest pain. She was afebrile with adequate oxygen saturation. Examination revealed a few fine crackles in the lung fields. Laboratory results showed pancytopenia. Initial treatment included broad-spectrum antibiotics and intravenous methylprednisolone for a suspected lupus flare. Cultures and tests for infections, including tuberculosis, were negative. Imaging revealed bilateral airspace disease with no pulmonary embolism. Autoimmune workup showed high antinuclear antibodies, positive anticardiolipin antibody, ribonucleoprotein, and anti-Smith antibody. Diagnosed with SLS, she was started on a tapering dose of methylprednisolone and hydroxychloroquine, along with rituximab, leading to significant improvement. Pulmonary function tests (PFTs) showed a restrictive pattern. SLS, with a very low prevalence in SLE, can also occur in systemic sclerosis, Sjogren\'s syndrome, and rheumatoid arthritis. Typical symptoms include dyspnea, pleuritic chest pain, and cough. Diagnosis involves chest radiography showing an elevated diaphragm and restrictive PFT pattern. Treatment often includes corticosteroids such as methylprednisolone and immunosuppressive agents. Rituximab has shown improvement in cases unresponsive to conventional therapy.
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  • 文章类型: Case Reports
    临床医生应仔细考虑全身淋巴结病,特别是病毒感染后,作为可能的系统性红斑狼疮(SLE)的首发症状之一,涉及异常关节受累,例如骶髂关节炎。这种自身免疫性炎性疾病的晚期诊断,可能导致不可逆转的发病率和更高的死亡率。
    淋巴结病可以代表各种病因,包括感染,恶性肿瘤,和风湿病。SLE被称为伟大的模仿者,可以以不同的第一表现呈现。我们报道了一名42岁的女性在爱泼斯坦-巴尔感染的急性期,患有多关节周围性关节炎,骶髂关节炎,和全身淋巴结病。她没有类似的病史,也没有服用未经巴氏消毒的乳制品。节点是软的,mobile,柔软,没有皮肤变化。在这个过程中,患者被诊断为SLE,接受泼尼松龙30mg/天和羟氯喹400mg/天治疗后出院.经过2周的随访,所有淋巴结肿大和症状均减轻.此案突显了SLE与生俱来的千张面孔。临床意识将导致准确的诊断和早期干预。
    UNASSIGNED: Clinicians should carefully consider generalized lymphadenopathy, particularly post viral infections, as one of the possible systemic lupus erythematous (SLE) first signs regarding unusual joint involvements such as sacroiliitis. Late diagnosis of this autoimmune inflammatory disease, could lead to irreversible morbidity and higher mortality.
    UNASSIGNED: Lymphadenopathy could represent various etiologies, including infections, malignancies, and rheumatologic diseases. SLE is known as the great mimicker which could be presented with different first manifestations. We report a 42-year-old woman in the acute phase of Epstein-Barr infection, admitted with polyarticular peripheral arthritis, sacroiliitis, and generalized lymphadenopathy. She had no similar history or taken unpasteurized dairy. Nodes were soft, mobile, and tender without skin change on top. During the process, she was diagnosed with SLE and discharged with prednisolone 30 mg/day and hydroxychloroquine 400 mg/day. After 2 weeks of follow-up, all lymphadenopathy and symptoms were diminished. This case underscores the thousand faces innate of SLE. Clinical awareness would lead to an accurate diagnosis and early intervention.
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  • 文章类型: Journal Article
    肾炎是系统性红斑狼疮(SLE)的常见和严重并发症。狼疮性肾炎(LN)的临床过程通常以缓解和恶化的交替阶段为特征。LN的耀斑可导致肾功能恶化,需要及时诊断和治疗。在各种研究中已经报道了SLE患者血清中存在抗C1q(抗C1qAb)的自身抗体。一些研究表明,抗C1qAb滴度的存在和变化可能与LN的发展有关,以及LN活动和肾脏耀斑。然而,抗C1qAb在LN中的确切作用仍是一个争论的话题。尽管已发表的研究结果存在差异,抗C1qAb有望作为评估SLE患者LN活性的非侵入性标志物。测量抗C1qAb水平可以帮助在非活动性疾病和肾耀斑期间诊断和管理LN。然而,需要采用标准化实验室检测的更大的对照试验,以进一步确立抗C1qAb在预测LN的再激活和缓解以及指导治疗策略方面的效用.
    Nephritis is a frequent and severe complication of Systemic Lupus Erythematous (SLE). The clinical course of lupus nephritis (LN) is usually characterized by alternating phases of remission and exacerbation. Flares of LN can lead to deterioration of kidney function, necessitating timely diagnosis and therapy. The presence of autoantibodies against C1q (anti-C1qAb) in the sera of SLE patients has been reported in various studies. Some research suggests that the presence and changes in the titer of anti-C1qAb may be associated with the development of LN, as well as with LN activity and renal flares. However, the exact role of anti-C1qAb in LN remains a subject of debate. Despite variability in the results of published studies, anti-C1qAb hold promise as noninvasive markers for assessing LN activity in SLE patients. Measuring anti-C1qAb levels could aid in diagnosing and managing LN during periods of both inactive disease and renal flares. Nevertheless, larger controlled trials with standardized laboratory assays are necessary to further establish the utility of anti-C1qAb in predicting the reactivation and remission of LN and guiding treatment strategies.
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  • 文章类型: Journal Article
    背景:心血管疾病是系统性红斑狼疮(SLE)患者发病和死亡的主要原因。SLE的心脏受累通常未被发现。三维(3D)斑点追踪超声心动图(STE)是一种无创成像技术,可以以准确且可重复的方式评估心脏心室的功能。这使其成为检测SLE患者心脏病早期体征的有吸引力的选择。通过识别这些亚临床心脏异常,3D-STE可能有助于减少SLE人群中心血管疾病的负面影响。因此,本研究旨在使用二维(2D)和3D-STE比较SLE患者与年龄和性别匹配的对照组之间的左心室(LV)功能.
    结果:当前研究发现左心室射血分数没有显着差异,左心室舒张末期容积,左心室收缩末期容积,左心室舒张末期肿块,两组之间的左心室收缩末期质量。然而,根据所有类型的超声心动图评估,与对照组相比,SLE组的LV整体纵向应变(GLS)均显着降低。包括3D和2D长轴应变,顶端2腔,和心尖4室评估(所有P值<0.05)。此外,关于使用3D-STE进行LVGLS测量,观察到了良好的评分者间可靠性和评分者间可靠性。此外,研究发现LVGLS与SLE病程之间存在显著相关性(r(50)=0.46,P<0.001).还发现使用泼尼松龙和肾病疾病会影响LVGLS测量。
    结论:尽管SLE患者的LVEF正常,LVGLS测量表明,与健康者相比,在SLE患者中更频繁地观察到LV收缩功能障碍。因此,先进的3D-STE技术可能有助于识别SLE患者LV功能的细微异常.
    BACKGROUND: Cardiovascular diseases are leading causes of morbidity and mortality in patients with systemic lupus erythematosus (SLE). Cardiac involvement in SLE can often go undetected. Three-dimensional (3D) speckle tracking echocardiography (STE) is a noninvasive imaging technique that can assess the function of the heart\'s ventricles in an accurate and reproducible way. This makes it an attractive option for detecting early signs of heart disease in SLE patients. By identifying these subclinical cardiac abnormalities, 3D-STE may help reduce the negative impact of cardiovascular diseases in SLE population. Therefore, this study aimed to compare the left ventricular (LV) function between patients with SLE compared to age- and gender-matched controls using two-dimensional (2D) and 3D-STE.
    RESULTS: The current study found no significant differences in left ventricle ejection fraction, left ventricle end-diastolic volume, left ventricle end-systolic volume, left ventricle end-diastolic mass, and left ventricle end-systolic mass between the two groups. However, the SLE group exhibited a significantly lower LV global longitudinal strain (GLS) compared to the control group according to all types of echocardiographic assessments, including 3D and 2D long-axis strain, apical 2-chamber, and apical 4-chamber assessments (all P values < 0.05). Furthermore, a good inter-rater reliability and intra-rater reliability were observed regarding the LVGLS measurement with 3D-STE. Additionally, the study identified a significant correlation between LVGLS and SLE duration (r (50) = 0.46, P < 0.001). The use of prednisolone and nephrology disorders was also found to impact LVGLS measurements.
    CONCLUSIONS: Despite a normal LVEF in patients with SLE, LVGLS measurements indicated that LV systolic dysfunction was observed more frequently in SLE patients compared to their healthy counterparts. Therefore, advanced 3D-STE techniques may be useful in identifying subtle abnormalities in LV function in SLE patients.
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  • 文章类型: Journal Article
    系统性红斑狼疮(SLE),一种自身免疫性疾病,是最常见的风湿性自身免疫性疾病之一。它影响由自身耐受机制的破坏引起的自体结缔组织。在过去的二十年里,干细胞疗法越来越被认为是各种疾病的治疗选择,包括帕金森病,老年痴呆症,中风,脊髓损伤,多发性硬化症,炎症性肠病,肝病,Diabete,心脏病,骨病,肾脏疾病,呼吸系统疾病,以及贫血等血液学异常.这是由于干细胞在受损组织中分裂并分化为特化细胞的独特特性。此外,它们具有免疫调节特性,影响由免疫异常引起的疾病,例如风湿性自身免疫性疾病。在目前的手稿中,两种主要类型的干细胞治疗的疗效,包括间充质干细胞(MSC),和造血干细胞(HSC)在动物模型或人类SLE患者,已被审查。一起来看,MSC和HSC治疗改善了疾病活动,肾脏的严重程度,肺,肝脏,和骨骼(临床表现改善)。此外,发生了免疫学参数的变化(免疫学参数的改善)。自身抗体的水平,包括抗核抗体(ANA),和抗双链脱氧核糖核酸抗体(dsDNAAb)减少。Th1/Th2比率的转换(有利于Th2),并且还检测到Th17/Treg(有利于Treg)。尽管MSC和HSC移植有许多优点,包括功效,安全,提高了SLE患者的生存率,一些并发症,包括疾病的复发,感染的发生,移植后观察到继发性自身免疫性疾病(SAD),应在下一步研究中解决。
    Systemic lupus erythematosus (SLE), an autoimmune disease, is among the most prevalent rheumatic autoimmune disorders. It affects autologous connective tissues caused by the breakdown of self-tolerance mechanisms. During the last two decades, stem cell therapy has been increasingly considered as a therapeutic option in various diseases, including parkinson\'s disease, alzheimer, stroke, spinal cord injury, multiple sclerosis, inflammatory bowel disease, liver disease, diabete, heart disease, bone disease, renal disease, respiratory diseases, and hematological abnormalities such as anemia. This is due to the unique properties of stem cells that divide and differentiate to the specialized cells in the damaged tissues. Moreover, they impose immunomodulatory properties affecting the diseases caused by immunological abnormalities such as rheumatic autoimmune disorders. In the present manuscript, efficacy of stem cell therapy with two main types of stem cells, including mesenchymal stem cell (MSC), and hematopoietic stem cells (HSC) in animal models or human patients of SLE, has been reviewed. Taken together, MSC and HSC therapies improved the disease activity, and severity in kidney, lung, liver, and bone (improvement in the clinical manifestation). In addition, a change in the immunological parameters occurred (improvement in immunological parameters). The level of autoantibodies, including antinuclear antibody (ANA), and anti-double-stranded deoxyribonucleic acid antibodies (dsDNA Abs) reduced. A conversion of Th1/Th2 ratio (in favor of Th2), and Th17/Treg (in favor of Treg) was also detected. In spite of many advantages of MSC and HSC transplantations, including efficacy, safety, and increased survival rate of SLE patients, some complications, including recurrence of the disease, occurrence of infections, and secondary autoimmune diseases (SAD) were observed after transplantation that should be addressed in the next studies.
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  • 文章类型: Journal Article
    过敏性紫癜(HSP)和小儿发作性系统性红斑狼疮(pSLE)与血管炎和血管疾病密切相关。本研究旨在探讨Ang-1、Ang-2和Tie2对HSP和pSLE的临床诊断价值。我们调查了82例HSP患者,34pSLE患者,10个健康的孩子使用酶联免疫吸附试验评估血清和尿液中Ang-1,Ang-2和Tie2的表达水平。使用受试者工作特征曲线分析评估Ang-1,Ang-2和Tie2对HSP和pSLE的诊断价值。结果显示,HSP和pSLE患者血清和尿液中Ang-2和Tie2的表达水平明显升高,而Ang-1/Ang-2值降低。此外,Ang-1在HSP患者的血清和尿液以及pSLE患者的血清中高表达。Ang-1,Ang-2和Tie2在各种类型的HSP和pSLE中显示出差异表达,与健康对照组相比。总之,Ang-1、Ang-2和Tie2可作为HSP和pSLE的生物标志物。此外,在HSP和pSLE患者中Ang-1/Ang-2值降低。Ang-1、Ang-2和Tie2可用作HSP和pSLE的生物标志物。
    Henoch-Schönlein purpura (HSP) and pediatric-onset systemic lupus erythematosus (pSLE) are closely associated with vasculitis and vascular diseases. This study aimed to investigate the clinical diagnostic values of Ang-1, Ang-2, and Tie2 for HSP and pSLE. We surveyed 82 HSP patients, 34 pSLE patients, and 10 healthy children. The expression levels of Ang-1, Ang-2, and Tie2 in the serum and urine were assessed using enzyme-linked immunosorbent assay. The diagnostic values of Ang-1, Ang-2, and Tie2 for HSP and pSLE were evaluated using receiver operating characteristic curve analysis. The results revealed that the serum and urine expression levels of Ang-2 and Tie2 were significantly elevated in HSP and pSLE patients, whereas the Ang-1/Ang-2 values were reduced. Additionally, Ang-1 was highly expressed in the serum and urine of HSP patients and in the serum of pSLE patients. Ang-1, Ang-2, and Tie2 showed differential expression in various types of HSP and pSLE compared with their expression in healthy controls. In summary, Ang-1, Ang-2, and Tie2 can serve as biomarkers for HSP and pSLE. Moreover, Ang-1/Ang-2 values are reduced in HSP and pSLE patients. Ang-1, Ang-2, and Tie2 can be used as biomarkers for HSP and pSLE.
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  • 文章类型: Case Reports
    Systemic lupus erythematosus (SLE) with oral desquamative lesions is one of the rare clinical entities. Periodontal disease and SLE display various mechanisms and possess a wide range of pathological characteristics. The tissue destruction mechanism of periodontitis and autoimmune diseases share similar pathways, and mounting reports studied the association between these two entities. The present case is of a 24-year-old female patient who complained of generalized widening of spaces in between the teeth. Along with it, She suffered from loss of hair, weakness, edema in the legs as well as arthralgia. The patient was identified to be suffering from SLE according to the American Rheumatism Association and European Academy of Dermatology and Venereology criteria 1 year before she reported to the dentist. She suffered from hair loss, weakness, arthralgia as well as edema in the legs. Based on the oral, clinical, and radiographic findings, she was diagnosed with aggressive periodontitis case. After nonsurgical periodontal treatment, the flap was reflected, debridement was done, after root conditioning with tetracycline, bovine osseous xenograft was placed in all the sites where ever there is angular bone loss, later sutured with interrupted direct loop suturing technique with 4-0 silk suture. Clinical and radiographic evaluation was done every 6 weeks to check the progress of the treatment. 6 months and 8-year follow-up revealed satisfactory clinical and radiographic outcomes. Based on the present case report and the previous literature, we recommend the use of xenograft in treating aggressive periodontitis patients.
    Résumé Le lupus érythémateux systémique (LES) avec lésions buccales desquamatives est l\'une des rares entités cliniques. La maladie parodontale et le LED présentent divers mécanismes et possèdent un large éventail de caractéristiques pathologiques. Le mécanisme de destruction des tissus de la parodontite et des maladies auto-immunes partage des voies similaires. partagent des voies similaires, et de nombreux rapports ont étudié l\'association entre ces deux entités. Le cas présent est celui d\'une patiente de 24 ans 24 ans qui se plaignait d\'un élargissement généralisé des espaces entre les dents. En plus de cela, elle a souffert d\'une perte de cheveux, de faiblesse, d\'œdème dans les jambes et d\'arthralgie. La patiente a été identifiée comme souffrant d\'un LED selon les critères de l\'American Rheumatism Association et de l\'Académie européenne de dermatologie et de vénéréologie un an avant de se présenter chez le dentiste. Elle souffrait de de perte de cheveux, de faiblesse, d\'arthralgie et d\'œdèmes dans les jambes. Sur la base des résultats buccaux, cliniques et radiographiques, elle a été diagnostiquée comme souffrant de parodontite agressive. Après un traitement parodontal non chirurgical, le lambeau a été réfléchi, un débridement a été effectué, après un conditionnement radiculaire après conditionnement radiculaire à la tétracycline, une xénogreffe osseuse bovine a été placée dans tous les sites où il y avait une perte osseuse angulaire. technique de suture en boucle directe interrompue avec une suture en soie 4-0. Une évaluation clinique et radiographique a été faite toutes les 6 semaines pour vérifier la progression du traitement. traitement. Le suivi à 6 mois et à 8 ans a révélé des résultats cliniques et radiographiques satisfaisants. Sur la base du présent rapport de cas et de la littérature précédente, nous recommandons l\'utilisation de la xénogreffe dans le traitement des patients atteints de parodontite agressive. Mots-clés: Parodontite, lupus érythémateux systémique, xénogreffe.
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  • 文章类型: Journal Article
    越来越多的证据表明,差异表达的环状RNA(circRNAs)在系统性红斑狼疮(SLE)患者的免疫细胞中起关键作用。Hsa_circ_0000479已在癌症和感染领域进行了研究,而在自身免疫性疾病中很少研究。本研究的目的是探讨中性粒细胞hsa_circ_0000479在SLE中的作用和临床价值。
    通过qPCR检测健康个体和SLE患者中性粒细胞中hsa_circ_0000479的表达水平,并与外周血单核细胞(PBMC)中的表达水平进行比较。此外,还分析了中性粒细胞中hsa_circ_0000479水平与SLE患者临床和免疫学特征的相关性。
    SLE患者中性粒细胞中hsa_circ_0000479的表达水平明显高于PBMC,也显著高于健康对照组(HCs)。此外,中性粒细胞中hsa_circ_0000479的表达水平与中性粒细胞绝对计数和补体3(C3)呈负相关,而在SLE中与抗dsDNA和抗核小体抗体呈正相关。此外,hsa_circ_0000479水平较高的SLE患者表现出更多的临床表现,包括雷诺现象,脱发和白细胞减少症。
    Hsa_circ_0000479在SLE患者的中性粒细胞中上调,并且还与几个重要的实验室指标和临床表现有关,提示中性粒细胞中的hsa_circ_0000479是SLE发病的可能因素之一,具有潜在的临床价值。
    Hsa_circ_0000479在SLE患者的中性粒细胞中表达,明显高于PBMC。中性粒细胞hsa_circ_0000479与实验室参数相关,包括NEUT,C3,抗dsDNA抗体和AnuA,除了与雷诺现象有关,脱发,SLE患者的白细胞减少。中性粒细胞中的Hsa_circ_0000479可能在SLE患者中起影响作用,对了解其发病机制具有重要意义。SLE的分层和治疗。
    Accumulating evidence suggests that differentially expressed circular RNAs (circRNAs) play critical roles in immune cells of systemic lupus erythematosus (SLE) patients. Hsa_circ_0000479 has been studied in the field of cancer and infection, whereas seldom studied in autoimmune diseases. The aim of this study was to investigate the role and clinical value of neutrophil hsa_circ_0000479 in SLE.
    The expression levels of hsa_circ_0000479 in both healthy individuals and SLE patients\' neutrophils were detected by qPCR and compared with those in peripheral blood mononuclear cells (PBMCs) . In addition, the correlation of hsa_circ_0000479 levels in neutrophils with the clinical and immunological features of SLE patients was also analysed.
    The expression levels of hsa_circ_0000479 in the patients with SLE were significantly higher in neutrophils than that of PBMCs, and also significantly higher than that in healthy controls (HCs). Moreover, the expression levels of hsa_circ_0000479 in neutrophils were negatively associated with absolute neutrophil count and complement 3 (C3), whereas positively correlated with anti-dsDNA and anti-nucleosome antibodies in SLE. In addition, SLE patients with higher levels of hsa_circ_0000479 demonstrated more several clinical manifestations, including Raynaud\'s phenomenon, alopecia and leucopenia.
    Hsa_circ_0000479 is up-regulated in neutrophils of SLE patients, and is also associated with several important laboratory indicators and clinical manifestations, suggesting that hsa_circ_0000479 in neutrophils was one of probable factors involved in the pathogenesis of SLE with potential clinical value.
    Hsa_circ_0000479 was expressed in neutrophils and was considerably higher than that of PBMCs in SLE patients.The neutrophil hsa_circ_0000479 was correlated with laboratory parameters, including NEUT, C3, anti-dsDNA antibodies and AnuA, in addition to being associated with Raynaud’s phenomenon, alopecia, and leucopenia in patients with SLE.Hsa_circ_0000479 in neutrophils may play an influential role in SLE patients and will be important to understand the pathogenesis, stratification and treatment in SLE.
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  • 文章类型: Case Reports
    单纯疱疹病毒(HSV)可在免疫功能低下的患者中引起严重的播散性感染。胃肠道受累很少包括结肠。我们介绍了一例罕见的免疫抑制患者播散性皮肤HSV感染并伴有结肠受累的病例。患者的临床表现和计算机断层扫描(CT)检查结果与结肠炎有关。她未能改善抗生素治疗,随后接受了柔性乙状结肠镜检查。总体发现和组织病理学与单纯疱疹病毒性结肠炎一致。必须认识到免疫功能低下患者的这种病理,以评估是否需要进行免疫抑制治疗并确保成功治疗以防止致命后果。
    Herpes simplex virus (HSV) can cause severe disseminated infections in immunocompromised patients. Gastrointestinal tract involvement seldom includes the colon. We present a rare case of disseminated cutaneous HSV infection with concomitant colonic involvement in an immunosuppressed patient. The patient\'s clinical presentation and computerized tomography (CT) findings were concerning for colitis. She failed to improve on antibiotic therapy and subsequently underwent flexible sigmoidoscopy. Gross findings and histopathology were consistent with herpes simplex virus colitis. It is essential to recognize this pathology in immunocompromised patients to evaluate the need to hold immunosuppressive therapy and ensure successful treatment to prevent fatal outcomes.
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  • 文章类型: Case Reports
    肺收缩综合征(SLS)是自身免疫性和结缔组织疾病如系统性红斑狼疮(SLE)的罕见并发症。一个35岁的女性病人,诊断为SLE,来到医院,抱怨严重的呼吸困难和胸痛几个月,劳累加剧。出现时的胸部影像学(X线和CT扫描)显示双侧基底肺不张,隔膜升高。肺功能测试(PFT)显示限制性发现,包括第一秒用力呼气量(FEV1)为37%,肺总容量为40%,在流量环路上采用限制性模式预测32%的肺活量,从而证实了SLS的诊断。治疗集中于甲氨蝶呤和利妥昔单抗。有已知SLE病史的患者开始出现咳嗽和呼吸困难等呼吸道症状,应尽早排除SLS,因为在后期可能致命。
    Shrinking lung syndrome (SLS) is a rare complication of autoimmune and connective tissue diseases like systemic lupus erythematosus (SLE). A 35-year-old female patient, diagnosed with SLE, came to the hospital complaining of severe dyspnea and pleuritic pain for several months that was worsening on exertion. Imaging (X-ray and CT scan) of the chest at the time of presentation showed bilateral basal atelectasis with elevated diaphragm. Pulmonary function test (PFT) showed restrictive findings including forced expiratory volume in the first second (FEV1) of 37%, total lung capacity of 40%, and vital capacity of 32% predicted with a restrictive pattern on flow volume loop confirming the diagnosis of SLS. The treatment focused on methotrexate and rituximab. Patients with a known history of SLE who start respiratory symptoms like cough and dyspnea should be ruled out of SLS at the earliest as it can be deadly in the later stages.
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