Immunological aspects of pulse therapy in systemic lupus erythematosus

Twenty SLE patients were examined against a background of pulse-therapy with methylprednisolone. The analysis showed that there was a decrease in the CIC concentration, antibodies to native DNA and an increase in the level of C3c and C4 components of the complement against a background of pulse-therapy. A more rapid time course of the CIC level was noted shortly after pulse-therapy as compared to changes in other immunological indices. A dynamic study of immunological indices in SLE against a background of pulse-therapy was appropriate for a clinical assessment and a study of the mechanisms responsible for the therapeutic efficacy of this method.     

1例系统性红斑狼疮患儿并发皮肤感染的护理

<正>系统性红斑狼疮(SLE)是一种侵犯多系统和脏器的全身系统性疾病,其临床表现多样,除发热、皮疹表现外,因受累脏器不同而表现不同,常常先后或同时累及泌尿、神经、心血管、血液、呼吸等多个系统,甚至有潜在的致命性。如不积极治疗,儿童SLE预后远比成人严重~([1])。2011年7月我科收治1例系统性红斑狼疮并多处皮肤感染的患儿,经治疗、护理,患儿颜面、躯干、四肢皮疹颜色变深,皮肤感染处结痂,部分脱痂,明显好转出院。现报道如下。     

1例系统性红斑狼疮并舞蹈症患儿的护理

报告了1例系统性红斑狼疮合并舞蹈症患儿的护理。患儿在采用糖皮质激素和免疫抑制剂治疗系统性红斑狼疮过程中,1年来反复出现面部红斑伴发热,首次出现舞蹈症表现及口腔溃疡、真菌感染,全身多部位皮肤癣样改变,患儿极度恐惧、抑郁。入院后给予加强心理支持,积极主动与患儿及家属沟通,改进口腔护理方法,做好皮肤护理,密切观察使用抗精神病药物后的反应。患儿病情好转出院。     

免疫净化联合小剂量环磷酰胺冲击治疗系统性红斑狼疮的临床对照研究

目的了解免疫净化联合小剂量环磷酰胺（CTX）冲击治疗系统性红斑狼疮（SLE）的疗效。方法回顾性分析北京大学人民医院SLE患者61例，其中28例给予免疫净化联合小剂量CTX冲击治疗，33例应用小剂量CTX冲击治疗，疗程1年。CTX每2周1次，每次400mg，连续3～6个月，之后每月1次，1次400mg；联合治疗组在给予CTX前均行免疫净化1～3次，2组患者均给予常规量糖皮质激素，疗程均为1年。监测患者症状，定期检测血常规、24h尿蛋白定量、血肌酐、白蛋白、免疫球蛋白、C3、C4、ANA、抗ENA抗体及抗dsDNA抗体水平。比较治疗前和治疗后1年不同组各项指标的变化。结果小剂量CTX冲击治疗1年后患者SLEDAI评分由治疗前平均13．73分降至平均5．44分；免疫净化联合小剂量CTX冲击治疗1年后患者SLEDAI评分由治疗前平均14．37分降至平均5．17分，2组统计学差异均有显著性。另外，治疗1年后小剂量CTX组和免疫净化联合小剂量CTX冲击治疗组抗ENA抗体分别转阴18．2％和42．9％，抗dsDNA抗体分别转阴39．4％和85．7％，2组统计学差异均有显著性。结论免疫净化联合小剂量CTX冲击治疗SLE的疗效优于小剂量CTX冲击治疗，且起效快，复发率低。     

Therapeutic plasma exchange and cyclosporine in the treatment of systemic lupus erythematosus.

Despite treatment with intensive immunosuppressive drug regimens, often the prognosis of patients suffering from systemic lupus erythematosus (SLE) is poor. Side effects such as infections and malignant tumors often occur. In the present trial, 21 patients (4 male and 17 female, aged 37.9 +/- 12.8 years) suffering from SLE for 9.4 +/- 2.6 years, were treated for 2.3 +/- 1.8 years with drug regimens of corticosteroids, azathioprine and/or cyclophosphamide. Then, over a period of up to 8 years, in addition to conventional therapies, especially in active stages of the disease with extremely high concentrations of anti-DNA-, antinuclear antibodies and circulating immunocomplexes, therapeutic plasma exchange (TPE) sessions were carried out depending on symptomatology. In addition patients received 2.5 +/- 0.6 mg cyclosporine/kg body weight/day. Compared to previous treatment modalities, clinical symptoms improved more quickly and more effectively (p = 0.046). After 5 to 48 (17.5 +/- 13.8) months, cyclosporine was established as a monotherapy for 8 of 21 patients. In the other cases, corticosteroids, azathioprine and cyclophosphamide were reduced by 40 to 100%. No severe side effects were seen. In acute stages of SLE and in forms with persistently high antibody levels, the addition of TPE sessions and cyclosporine as the basic immunosuppressive drug is usually very effective with regard to improving clinical symptomatology.     

系统性红斑狼疮的目标治疗

系统性红斑狼疮（SLE）属于慢性、不可治愈性疾病。治疗高度异质性的SLE需要引入目标治疗的理念,朝着完全缓解的目标进行诱导治疗。根据病人的治疗反应,评估和调整治疗方案,确保疾病朝着缓解或低度活动的目标逐渐改善,直至完全缓解。     

系统性红斑狼疮的个体化治疗

系统性红斑狼疮（systemic lupus erythematosus，SLE）是一种高度异质性的疾病，正确的治疗方案是在根据患者受损的脏器及程度评估SLE病情，并根据患者体质的耐受性，权衡风险与效益之比后，选取个体化的用药方案。     

重症系统性红斑狼疮合并巨噬细胞活化综合征患儿的护理

报道1例重症系统性红斑狼疮(SLE)合并罕见巨噬细胞活化综合征(MAS)患儿的护理.护理上给予严密病情观察,预防感染,加强口腔和皮肤护理、饮食护理与用药护理,重视心理护理,患儿未发生护理并发症,取得良好的救治效果.     

Transfusion-associated GVHD after fludarabine therapy in a patient with systemic lupus erythematosus

BACKGROUND: Fludarabine, a purine antimetabolite with potent immunosuppressive properties, has previously been associated with the development of transfusion-associated GVHD (TA-GVHD) in patients with hematologic malignancies. Its role as a risk factor for TA-GVHD in patients without underlying leukemia or lymphoma is uncertain. STUDY DESIGN AND METHODS: A 42-year-old female with refractory lupus nephritis received three monthly cycles of fludarabine (30 mg/m2/day on Days 1-3) and cyclophosphamide (500 mg/m2 on Day 1). Three months after the last dose of fludarabine, she received 2 units of packed RBCs and 6 units of pooled random platelets, none of which were irradiated. Two weeks later, fever, rash, aminotransferase elevations, hyperbilirubinemia, and pancytopenia developed. RESULTS: Marrow biopsy showed severe aplasia and skin biopsy was consistent with GVHD. Allele-level HLA typing on circulating lymphocytes revealed extra HLA alleles not present in her pretreatment sample, but identical to the HLA haplotypes of an unrelated platelet donor. Treatment with antithymocyte globulin, cyclosporine, and prednisone was followed by preparatory conditioning for a PBPC transplant from an HLA-identical sibling, but the patient died of disseminated candidiasis before transplant. CONCLUSIONS: Fludarabine and other purine analogs are increasingly used in the treatment of disorders other than hematologic malignancy, such as autoimmune disease. The occurence of TA-GVHD after fludarabine therapy in a patient with lupus strongly suggests that this drug is sufficiently immunoablative to be an independent risk factor for TA-GVHD. Irradiation of blood components should be considered in all patients who receive fludarabine therapy.     

吗替麦考酚酯与环孢素A治疗系统性红斑狼疮继发血小板减少的疗效对比

目的比较吗替麦考酚酯与环孢素A治疗系统性红斑狼疮继发血小板减少症的疗效。方法将我科2012年4月～2017年2月收治的48例系统性红斑狼疮患者,随机分为MMF组26例和CsA组22例。MMF组采用激素联合吗替麦考酚酯治疗,CsA组采用激素联合环孢素A治疗。观察两组患者治疗前和治疗6个月后血小板数目变化、SLEDAI评分变化及不良反应的发生情况。结果 MMF组血小板数目改善、尿蛋白下降、SLEDAI指数下降有效率及疗效均优于CsA组,差异有统计学意义(P0.05)。结论吗替麦考酚酯治疗SLE所致血小板减少,疗效显著,副作用更少,值得临床推广应用。     

贝利尤单抗联合环孢素治疗难治性系统性红斑狼疮继发免疫性血小板减少1例并文献复习

目的 探讨贝利尤单抗治疗难治性系统性红斑狼疮(systemic lupus erythematosus, SLE)合并顽固性血液系统损害的疗效。方法 回顾性分析贵州省人民医院收治的1例以顽固性血液系统损害为主要表现的SLE病例的诊治过程。结果 患者为青年女性,SLE病史1年余,因皮下瘀斑伴腹痛10天入院,住院后明确诊断SLE合并难治性血液系统损害,在环孢素等基础治疗上加用贝利尤单抗维持治疗后,患者病情明显改善,且未见不良反应。随访1年余,患者激素用量已减至最低维持量,无复发。结论 贝利尤单抗联合环孢素治疗为难治性SLE继发免疫性血小板减少的治疗提供了新选择,更多的临床应用证据有待长期的大样本病例研究观察、评估。     

三联冲击疗法治疗重症系统性红斑狼疮的临床研究

目的 了解大剂量甲基强的松龙(MP)、环磷酰胺(CTX)、免疫球蛋白(IV-IG)冲击治疗重症系统性红斑狼疮(SSLE)的效果.方法 2000年9月至2004年10月我院住院16例患者应用大剂量免疫球蛋白疗法(IV-IG)+大剂量MP+CTX冲击治疗.结果 16例患者1月内明显缓解者11例,1例因腹泻不能控制死亡.3例5月内明显缓解,1例急进性肺部病变合并急性肾功能衰竭患者在治疗后8月肺部病变缓解,并发感染4例(51%),其中1例原有肺部感染加重,冲击治疗中未出现新发感染.结论 三联冲击疗法不仅能够明显提高重症系统性红斑狼疮患者的缓解率,而且减少感染发生.     

系统性红斑狼疮诊断与治疗

系统性红斑狼疮(SLE)是由自身免疫介导的,以血清中出现多种自身抗体为特征的自身免疫性疾病。 其发病机制复杂,可累及多系统多器官,临床表现多样,病程迁延反复,病因至今尚未完全明确。近年来,随着对SLE 认识的逐步深入,国内外对该疾病的诊断和治疗水平都在不断完善和提高,SLE患者的预后显著改善。本文对SLE 的诊断及治疗进展进行综述,旨在提高对SLE疾病监测、管理水平及改善预后。     

Eosinophilic enteritis with systemic lupus erythematosus

Gastrointestinal manifestations are common in systemic lupus erythematosus (SLE). Eosinophilic enteritis is a rare disorder of uncertain cause that was recently reported for the first time in association with SLE. This report presents a second case of eosinophilic enteritis in a 47-year-old female patient with SLE. The patient presented with recurrent episodes of abdominal pain, nausea, vomiting, and diarrhea. Complete blood counts on occasion showed elevated eosinophil counts. The patient underwent a comprehensive workup over several weeks, culminating in a small bowel biopsy that showed eosinophil infiltration in the muscularis propria, establishing the diagnosis. The patient was treated with a prolonged taper of prednisone with successful resolution of symptoms.