• 文章类型: Case Reports
    系统性红斑狼疮(SLE)是一种复杂的自身免疫性疾病,身体对自身抗原失去耐受性,特别是核抗原。T细胞和B细胞的异常反应导致自身抗体的产生和组织中免疫复合物的形成,触发补体激活,炎症,和不可逆的器官损伤。SLE可以影响身体的任何部位,导致不同的临床症状。SLE的一种罕见表现是狼疮肠系膜血管炎(LMV),表现出模糊的症状,异常的实验室发现,和特定的成像特征。LMV,虽然不常见,可以发展为严重的并发症,如肠穿孔,出血,甚至死亡率。这里,我们报告了一例累及多器官系统的LMV(包括皮肤粘膜,肌肉骨骼,浆膜腔,和血液系统),最初出现危及生命的顽固性胃肠道出血,并发严重的肺部感染.通过分享这个案例,我们的目标是增强临床医生对严重SLE病例的管理信心,并提高对疾病监测的认识.
    Systemic lupus erythematosus (SLE) is a complex autoimmune disease where the body loses tolerance to its own antigens, particularly nuclear antigens. Abnormal responses from T and B cells lead to the production of autoantibodies and the formation of immune complexes in tissues, triggering complement activation, inflammation, and irreversible organ damage. SLE can affect any part of the body, resulting in diverse clinical symptoms. One rare manifestation of SLE is lupus mesenteric vasculitis (LMV), which presents with vague symptoms, abnormal laboratory findings, and specific imaging features. LMV, although uncommon, can progress to severe complications such as bowel perforation, haemorrhage, and even mortality. Here, we report a case of LMV with the involvement of multiple organ systems (including mucocutaneous, musculoskeletal, serosal cavities, and haematological systems), presenting initially with life-threatening intractable gastrointestinal bleeding, and complicated by severe pulmonary infection. By sharing this case, we aim to enhance clinicians\' confidence in managing critical SLE cases and raise awareness about disease surveillance.
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  • 文章类型: Case Reports
    Rhupus综合征是一种自身免疫性疾病,结合了狼疮和类风湿性关节炎的症状。这是一种罕见的疾病,会影响身体的结缔组织,如关节,肌肉,和皮肤。rhupus综合征的症状可能与狼疮相似,包括关节痛,疲劳,还有皮疹.然而,rhupus综合征也可以引起类风湿性关节炎的症状,如关节僵硬和肿胀。rhupus综合征的治疗通常涉及药物和生活方式改变的组合,以控制症状并改善整体生活质量。一名24岁的女性患者由当地医生转诊,以评估全血细胞减少症。她的病史可以追溯到六个月,当时她出现了进行性疲劳,轻度劳累时呼吸困难,和多关节痛。初步实验室检查显示全血细胞减少症,抗核抗体(ANA)阳性,抗双链DNA(抗dsDNA),和抗环瓜氨酸肽(抗CCP)抗体。骨髓检查证实诊断为再生障碍性贫血。她开始服用环孢菌素,目的是保持200至250ng/mL的波谷水平。在三到四个月内,她的血液学恢复反应良好。该病例强调了在rhupus综合征并发再生障碍性贫血中对环孢素的血液学和临床反应良好。需要进一步的研究来确定环孢素在该患者人群中的长期疗效。
    Rhupus syndrome is an autoimmune disorder that combines the symptoms of lupus and rheumatoid arthritis. It is a rare condition that affects the connective tissues of the body such as the joints, muscles, and skin. The symptoms of rhupus syndrome can be similar to those of lupus, including joint pain, fatigue, and skin rashes. However, rhupus syndrome can also cause symptoms of rheumatoid arthritis, such as joint stiffness and swelling. Treatment for rhupus syndrome usually involves a combination of medications and lifestyle changes to manage symptoms and improve the overall quality of life. A 24-year-old female patient was referred by a local physician for evaluation of pancytopenia. Her history dates back to six months when she developed progressive fatigue, dyspnea on mild exertion, and polyarthralgia. Initial laboratory investigations revealed pancytopenia, positive antinuclear antibodies (ANA), anti-double-stranded DNA (anti-dsDNA), and anti-cyclic citrullinated peptide (anti-CCP) antibodies. Bone marrow examination confirmed the diagnosis of aplastic anemia. She was started on cyclosporine with an aim to maintain a trough level between 200 and 250 ng/mL. She responded well with hematological recovery in three to four months. This case highlighted the excellent response to cyclosporine hematologically and clinically in rhupus syndrome complicated with aplastic anemia. Further studies are required to establish the long-term efficacy of cyclosporine in this patient population.
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    文章类型: Case Reports
    目的:介绍一例罕见的新生儿红斑狼疮(NLE)伴疑似噬血细胞性淋巴组织细胞增多症(HLH)或巨噬细胞活化综合征(MAS)。
    方法:一个体重为2,995g的女婴,母亲没有任何疾病病史。出生时,患者面部和躯干有红斑丘疹。她在1日龄时入院,C反应蛋白水平升高。基于抗Ro/SSA和抗La/SSB抗体的存在,患者被诊断为NLE。此后,很明显,她母亲的抗体水平也升高了。在20天大的时候,婴儿转氨酶升高,铁蛋白,甘油三酯,和可溶性白细胞介素-2受体水平。尽管怀疑是HLH或MAS,她不符合诊断标准.此后,这些异常值自发改善,使用局部类固醇后皮疹有所改善。患者在39日龄时出院。一岁时,患者生长发育正常。
    结论:出生时出现不明原因皮疹的婴儿应考虑NLE。当做出诊断时,需要密切观察婴儿的临床特征,以确定他们是否会发展为HLH或MAS。
    OBJECTIVE: To present a rare case of neonatal lupus erythematosus (NLE) associated with suspected hemophagocytic lymphohistiocytosis (HLH) or macrophage activation syndrome (MAS).
    METHODS: A female infant weighing 2,995 g was born to a mother without medical history of any disease. At birth, the patient had erythematous papules on her face and trunk. She was admitted at 1 day of age with elevated C-reactive protein levels. The patient was diagnosed with NLE based on the presence of anti-Ro/SSA and anti-La/SSB antibodies. Thereafter, it became clear that the antibody levels in her mother were also elevated. At 20 days of age, the infant showed elevated transaminases, ferritin, triglyceride, and soluble interleukin-2 receptor levels. Although HLH or MAS was suspected, she did not fulfill the diagnostic criteria. Thereafter, these abnormal values spontaneously improved, and the skin rash improved with the use of topical steroids. The patient was discharged at 39 days of age. At 1 year of age, the patient\'s growth and development were normal.
    CONCLUSIONS: NLE should be considered in infants with an unexplained skin rash at birth. When a diagnosis is made, close observation of the infant\'s clinical features is needed to determine whether they will develop HLH or MAS.
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  • 文章类型: Case Reports
    背景:2019年冠状病毒病患者的血栓形成发生率很高,康复后血栓形成发生率降低。当冠状病毒疾病2019伴有容易血栓形成的疾病时,感染后血栓事件的风险可能增加.
    方法:我们报告了一名24岁的中国女性,患有系统性红斑狼疮,2019年从冠状病毒疾病中恢复后出现数字缺血性坏疽。其发病机制与系统性红斑狼疮的临床特点有关,2019年冠状病毒病引起的高凝状态,以及病毒感染引起的二次打击。
    结论:自身免疫性疾病患者应警惕由严重急性呼吸道综合征冠状病毒2和其他病毒引起的自身免疫性疾病。对这些患者的治疗应严格规范,应选择适当的抗凝方法以防止血栓形成。
    BACKGROUND: Patients with coronavirus disease 2019 have a high incidence of thrombosis that decreases after recovery. When coronavirus disease 2019 is accompanied by diseases prone to thrombosis, risk of post-infection thrombotic events may increase.
    METHODS: We report a case of digital ischemic gangrene in a 24-year-old Chinese female with systemic lupus erythematosus after recovery from coronavirus disease 2019. The pathogenesis was related to clinical characteristics of systemic lupus erythematosus, hypercoagulability caused by coronavirus disease 2019, and second-hit due to viral infection.
    CONCLUSIONS: Patients with autoimmune diseases should remain alert to autoimmune system disorders induced by severe acute respiratory syndrome coronavirus 2 and other viruses. Treatment for these patients should be strictly standardized, and appropriate anticoagulation methods should be selected to prevent thrombosis.
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  • 文章类型: Case Reports
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  • 文章类型: Case Reports
    狼疮肠炎是系统性红斑狼疮(SLE)的一种罕见胃肠道并发症,具有显着的发病率和死亡率。利妥昔单抗,针对CD20阳性B细胞的单克隆抗体,在难治性SLE病例中显示出希望。我们介绍了一例45岁女性SLE患者,该患者发展为狼疮肠炎,对利妥昔单抗的反应异常迅速和显着。患者出现严重的腹痛和腹胀。在利妥昔单抗治疗的两天内,病人的腹痛,扩张,和相关的并发症完全解决。这种特殊的反应挑战了利妥昔单抗在SLE中疗效的典型时间表,并强调需要进一步研究影响治疗反应的因素。了解这种快速改善的潜在机制可能会提供对SLE发病机制的见解,并指导最佳结果的治疗策略。
    Lupus enteritis is a rare gastrointestinal complication of systemic lupus erythematosus (SLE) associated with significant morbidity and mortality. Rituximab, a monoclonal antibody targeting CD20-positive B cells, has shown promise in refractory SLE cases. We present a case of a 45-year-old female with SLE who developed lupus enteritis and experienced an unusually rapid and remarkable response to Rituximab. The patient presented with severe abdominal pain and distension. Within two days of Rituximab treatment, the patient\'s abdominal pain, distension, and associated complications resolved completely. This exceptional response challenges the typical timeline of Rituximab efficacy in SLE and highlights the need for further investigation into the factors influencing treatment response. Understanding the mechanisms underlying such rapid improvement may provide insights into SLE pathogenesis and guide therapeutic strategies for optimal outcomes.
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  • 文章类型: Case Reports
    后巩膜炎是一种罕见的炎症性眼部疾病,影响巩膜的后段,在女性中更为普遍。其临床表现,通常是非特异性的,包括眼痛,头痛,和视力丧失。由于缺乏对视力构成潜在威胁的特定症状,误诊很常见。病因通常与风湿病有关,如类风湿性关节炎(RA),系统性红斑狼疮(SLE),和抗中性粒细胞胞浆抗体(ANCA)相关血管炎。后巩膜炎提出了诊断挑战,模仿许多其他眼部疾病,因此有必要进行全面的临床眼科检查。实验室研究,包括炎症标志物和风湿性疾病标志物,可以识别潜在的全身性疾病。成像,包括B超和磁共振成像(MRI),有助于准确诊断。治疗包括非甾体抗炎药(NSAID),以及用于轻度疾病的局部皮质类固醇和用于严重疾病的全身性皮质类固醇。对于难治性病例,生物治疗变得越来越重要。涉及眼科和风湿病的多学科方法对于这种潜在的视力威胁疾病的管理至关重要。该病例报告重点介绍了一名46岁的女性,有RA相关的后巩膜炎病史。
    Posterior scleritis is a rare inflammatory eye condition affecting the posterior segments of the sclera and is more prevalent in females. Its clinical presentation, often nonspecific, includes ocular pain, headache, and vision loss. Misdiagnosis is common due to a lack of specific symptoms posing a potential threat to vision. The etiology is often tied to rheumatic diseases, such as rheumatoid arthritis (RA), systemic erythematous lupus (SLE), and anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis. Posterior scleritis poses diagnostic challenges, mimicking many other ocular conditions, hence necessitating a thorough clinical eye exam. Laboratory studies, including inflammatory markers and markers of rheumatic diseases, may identify underlying systemic diseases. Imaging, including B-scan ultrasound and magnetic resonance imaging (MRI), aids in accurate diagnosis. Treatment involves non-steroidal anti-inflammatory drugs (NSAID), as well as topical corticosteroids for mild disease and systemic corticosteroids for severe disease. Biologic therapy has become increasingly significant for refractory cases. A multidisciplinary approach involving ophthalmology and rheumatology is crucial in the management of this potential sight-threatening disease. This case report highlights a 46-year-old woman with a history of RA-associated posterior scleritis.
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  • 文章类型: Case Reports
    光疗的使用在治疗各种皮肤病中是非常有效的。在这项研究中,目的是在接受UVB治疗的银屑病患者中呈现水泡和水疱性病变。建议在光疗后出现囊泡状病变的情况下考虑BSLE的可能性,以及其他潜在的诊断。
    大疱性系统性红斑狼疮(BSLE)是皮肤红斑狼疮的一种罕见形式,在身体各个部位表现为囊泡和水疱。这些病变的病理外观通常显示有IgG沉积物的表皮下囊泡,IgM,IgA,在直接免疫荧光(DIF)检查下,以颗粒或线性形式的补体C3。临床研究证明了光疗在治疗各种皮肤状况中的有效性。虽然一些研究表明光疗与囊泡状病变的发展之间存在相关性,这些报告大多与大疱性类天疱疮有关,对光疗后BSLE发生的研究有限。在这个案例报告中,描述了一名接受UVB治疗的70岁男性牛皮癣的水泡和起泡性病变。病理检查证实诊断为大疱性系统性红斑狼疮,患者在使用氨苯砜片剂治疗后经历了显著的改善。对光疗后囊泡状病变的发展进行了文献综述,比较以前研究中提出的不同方法。我们的结论强调了将BSLE视为光疗后膀胱小斑病变的可能诊断的重要性,其他潜在条件。
    UNASSIGNED: The use of phototherapy is highly effective in treating various skin diseases. In this study, the aim is to present vesicular and blister lesions in patients treated with UVB for psoriasis. It is advisable to consider the possibility of BSLE in cases of vesiculobullous lesions following phototherapy, along with other potential diagnoses.
    UNASSIGNED: Bullous systemic lupus erythematosus (BSLE) is a rare form of cutaneous lupus erythematosus that presents as vesicles and blisters on various parts of the body. The pathological appearance of these lesions often shows subepidermal vesicles with deposits of IgG, IgM, IgA, and complement C3 in granular or linear forms under direct immunofluorescence (DIF) examination. Clinical studies demonstrate the effectiveness of phototherapy in treating various skin conditions. While several studies suggest a correlation between phototherapy and the development of vesiculobullous lesions, most of these reports are related to bullous pemphigoid, with limited research on the occurrence of BSLE following phototherapy. In this case report, vesicular and blistering lesions in a 70-year-old man undergoing UVB treatment for psoriasis are described. Pathological examination confirmed the diagnosis of bullous systemic lupus erythematosus, and the patient experienced significant improvement after treatment with dapsone tablets. A literature review was conducted on the development of vesiculobullous lesions after phototherapy, comparing different approaches presented in previous studies. Our conclusion highlights the importance of considering BSLE as a possible diagnosis in cases of vesiculobullous lesions post-phototherapy, alongside other potential conditions.
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  • 文章类型: Journal Article
    系统性红斑狼疮(SLE)影响多器官系统,最近有越来越多的证据表明癌症风险显著上升。尽管有越来越多的证据,SLE与多发性骨髓瘤(MM)之间的关系仍未得到重视。这篇综述综合了2012年至2023年发表的病例报告的结果,以探讨这种关系。我们使用PubMed进行了全面搜索,Embase,和谷歌学者用关键词“SLE”和“多发性骨髓瘤”描述了SLE患者MM的临床特点。回顾了7例病例报告。五个病例报告包括女性参与者,两个人同时诊断为SLE和MM,而在其他方面,MM随访SLE7个月至30年。2例报告MM有所改善。四例报告因并发症死亡,其中包括震惊,心肌梗塞,和肺炎。2例狼疮性肾炎合并MM和SLE综合征。较大,专注于临床表现的成熟研究,诊断策略,治疗,和结果需要更好地了解SLE和MM之间的关联。医护人员应该意识到SLE中恶性肿瘤的风险增加,并相应地定制筛查。
    Systemic lupus erythematosus (SLE) affects multiple organ systems, and there has recently been increasing evidence that suggests a considerable rise in cancer risk. Despite growing evidence, the relationship between SLE and multiple myeloma (MM) remains underlooked. This review synthesizes findings from case reports published between 2012 and 2023 to explore this relationship. We conducted a comprehensive search using PubMed, Embase, and Google Scholar with the keywords \'SLE\' and \'multiple myeloma\' and described the clinical profile of MM in patients with SLE. Seven case reports were reviewed. Five case reports included female participants, two had a simultaneous diagnosis of SLE and MM, and in others, MM followed SLE varying from 7 months to 30 years. Two cases reported an improvement in MM. Four cases reported death due to complications, which included shock, myocardial infarction, and pneumonia. Lupus nephritis was seen to complicate MM and SLE complex in 2 cases. Larger, well-developed studies focusing on clinical presentation, diagnostic strategy, treatment, and outcomes are needed to better understand the association between SLE and MM. Healthcare workers should be aware of the increased risk of malignancy in SLE and customize screening accordingly.
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  • 文章类型: Case Reports
    背景:指坏疽是系统性红斑狼疮(SLE)的一种罕见但严重的并发症。它通常发生在疾病持续时间较长的中年患者中。案例:在此,我们报告了一个56岁男子的案例(没有历史暗示雷诺现象,糖尿病,吸烟,创伤,感染,或化学暴露),出现SLE和数字坏疽的人是最初的迹象。他有一个月的关节痛史,脱发,光敏性,口腔溃疡,Malar皮疹,呼吸困难,数字疼痛体格检查显示四肢和背部疼痛和弥漫性红斑皮肤病变,还有手指上的发紫.我们注意到淋巴细胞减少症(600个细胞/mm3),和实验室测试中C反应蛋白升高(15.1mg/l)。抗核抗体(ANA)的免疫学测试呈阳性,标题1:400。肺计算机断层扫描显示肺纤维化,肺功能检查显示限制性肺病。保留了SLE肺部受累的诊断。在寻找有利于患者皮肤病变血管起源的元素时,免疫学评估为阴性。用200mg/天的羟氯喹开始治疗。对于皮肤和肺部受累,静脉注射(IV)脉冲治疗与甲基强的松龙(1,000mg/d,连续3天,每月)和环磷酰胺(1g/月)联用.还规定了钙阻断剂。然而,病变没有改善。患者在14天的间隔内两次输注利妥昔单抗(1克),大多数血管炎病变明显改善,和呼吸困难的部分改善。结论:指坏疽是晚发性SLE的罕见并发症,尤其是作为主要表现。
    Background: Digital gangrene is a rare but serious complication of systemic lupus erythematosus (SLE). It occurs usually in middle-aged patients with longer disease duration. Case: Herein we report the case of a 56-year-old man (with no history suggestive of Raynaud\'s phenomenon, diabetes mellitus, smoking, trauma, infection, or chemical exposure), who presented with SLE and digital gangrene was among the first signs. He presented with a one-month history of joint pain, hair loss, photosensitivity, mouth ulcers, malar rash, dyspnea, and digital pain. Physical examination revealed painful and diffuse erythematous skin lesions in the extremities and back, as well as cyanosis in the fingers. We noted lymphocytopenia (600 cells/mm 3), and an elevated C-reactive protein (15.1 mg/l) on laboratory tests. Immunological tests were positive for antinuclear antibodies (ANA) with Title 1:400. Pulmonary computed tomography revealed pulmonary fibrosis, and pulmonary function tests revealed the restrictive pulmonary disease. Diagnosis of SLE with lung involvement was retained. The immunological assessment in search of elements in favor of a vascular origin of the patient\'s skin lesions was negative. Treatment was initiated with 200 mg/day hydroxychloroquine. For dermal and pulmonary involvement, intravenous (IV) pulse therapy was used with methylprednisolone (1,000 mg/d for three consecutive days monthly) and cyclophosphamide (1 g/month). Calcium blocking agents were also prescribed. However, the lesions did not improve. The patient was given two infusions of rituximab (1 g) at a 14-day interval with a marked improvement ofthe majority of vasculitis lesions, and a partial improvement of dyspnea. Conclusions: Digital gangrene is a rare complication of late-onset SLE, especially as a primary manifestation.
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