系统性红斑狼疮并发自身免疫性低血糖1例

1 临床资料 患者,女性,29岁,因意识障碍4 h入院.2年前在外院确诊为系统性红斑狼疮、狼疮性肾炎(Ⅱ型).经激素和细胞毒药物治疗后病情好转,入院前半年左右,停用激素和细胞毒药物.     

外源性胰岛素诱发自身免疫性低血糖3例并文献复习

胰岛素自身免疫综合征（insulin autoimmune syndrome,IAS）于1970年由日本学者Hirata[1]首次报道,故又称Hirata病,在日本,IAS已经成为除胰岛素瘤和胰外肿瘤外,引起自发性低血糖的第3位病因。经典的IAS是指血中非外源性胰岛素诱导的胰岛素自身抗体及高浓度免疫活性胰岛素所致的自发性低血糖症,     

以血小板减少性紫癜为首发表现的系统性红斑狼疮一例

系统性红斑狼疮(SLE)的初发症状常为发热、关节痛、皮疹、日光过敏、血沉增快等。以血小板减少,出血倾向为首发症状者少见。本文报告1例以血小板减少性紫瘢为首发症状的SLE。 患者女性,25岁。1978年(16岁)起反复牙龈出血,鼻(?),月经量多,血小板减小,但无皮疹、     

以血小板减少为首发症状的小儿系统性红斑狼疮2例

<正> 例1 女,7岁。以全身皮肤散在出血点10d入院。体检发现全身皮肤散布瘀点,淋巴结无肿大,心肺无异常,肝脾无肿大,实验室检查Hb110g/L,WBC7.5×10~9/L,NO.60,LO.40,PL28×10~9/L。骨髓象:巨核细胞增多伴成熟障碍。诊断特发性血小板减少性紫癜(ITP),给予肾上腺皮质激素,丹那唑等治疗,住院2周,血小板数升至100×10~9/L出院,1个月后激素渐减量停药。门诊随访,血小板数波动在110～175×10~9/L。10个月后因皮肤出血点,颜面浮肿及关节疼痛再度入院,体检发现轻度贫血貌,面部浮肿,颊部见红斑,颈部淋巴结轻度肿大,心肺无异常,肝肋下3cm,脾未触及,躯干皮肤散在出血点。实验室检查Hb90g/L,WBC3.5×10~9/L,NO.35,LO.60,     

以血栓性血小板减少性紫癜为首发表现的系统性红斑狼疮2例报告

我科近年来收治的2例以(TTP)为首发表现的系统性红斑狼疮(SLE),现报告如下。例1:女,28岁,以发热、头痛1周,于2002年10月5日入院,入院时T38℃,神志清,贫血貌,巩膜轻度黄染,Hb40g/L,WBC4.4×109/L,BPC118×109/L,可见晚幼红细胞,尿胆原( ),尿蛋白( ),总胆红素35μmol/L,直接胆红     

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

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5～6min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、     

以血小板减少为首发表现的系统性红斑狼疮3例报告

系统性红斑狼疮（SLE）是一种多因素参与的异质性自身免疫性疾病,患者突出表现为体内有多种自身抗体,这些自身抗体通过免疫复合物等途径导致几乎全身各系统和各器官均有损害。免疫性血小板减少性紫癜（ITP）,是因血小板免疫性破坏导致外周血小板减少的出血性疾病,以广泛皮肤粘膜及内脏出血、血小板减少、骨骼巨核细胞发育成熟障碍、血小板生成时间缩短及抗血小板自身抗体出现为特征。     

以血栓性血小板减少性紫癜为首发表现的系统性红斑狼疮一例并文献复习

系统性红斑狼疮(systemic lupus erythematosus,SLE)是一种病因未明、累及多脏器多系统的自身免疫性疾病,临床表现复杂多样,以血栓性血小板减少性紫癜(thrombotic thrombocytopenic purpura,TTP)为首发或突出表现时,易误诊为特发性血小板减少性紫癜(idiopathic thrombocytopenic purpura,ITP),两者合并发生时病情凶险,病死率高,临床罕见。我科确诊1例以TTP为首发表现的SLE患者,经积极救治后病情获得缓解,现结合国内外相关文献报道如下。     

以肺炎为首发症状的系统性红斑狼疮复发1例报告并文献复习

目的 提高临床医生对急性狼疮性肺炎的认识.方法 对1例以肺炎为首发症状的系统性红斑狼疮(SLE)复发患者的临床表现、实验室辅助检查以及诊治过程进行回顾性分析,并进行疾病相关文献复习.结果 患者发热,抗核抗体(ANA)(颗粒型)1 ∶100,抗双链-DNA(ds-DNA)阴性,外周血白细胞增高,胸片提示左下肺炎.肺部CT...     

系统性红斑狼疮重叠干燥综合征合并肺部曲霉菌感染1例报告

目的 研究系统性红斑狼疮重叠干燥综合征合并肺部曲霉菌感染的临床表现。方法 回顾分析一例系统性红斑狼疮重叠干燥综合征的患者合并肺部曲霉菌感染的临床资料,并复习相关文献。结果 该患者诊为活动期SLE并干燥综合征,肾上腺皮质激素治疗期间出现咳嗽、咳痰加重,3次痰培养均为曲霉菌。选取伏立康唑治疗后,临床症状消失,痰培养转阴。结论：SLE合并pSS患者出现持续发热、咳嗽、咳痰等症状时应警惕肺部曲霉菌感染的可能,SLE合并肺部曲霉菌感染病死率很高,早期诊断和治疗是改善预后的关键。肺曲霉菌易复发,必须给予足够疗程的治疗。     

系统性红斑狼疮合并胸腺瘤3例并文献复习

目的 探讨系统性红斑狼疮(SLE)与胸腺瘤之间的关系及此类患者的治疗策略.方法 回顾性分析3例系统性红斑狼疮合并胸腺瘤患者的临床资料,并进行文献复习,就两者之间的联系及治疗策略进行讨论.结果 3例患者均为女性,发生胸腺瘤时年龄40～45岁.2例患者首次诊断SLE时即伴发胸腺瘤,1例患者胸腺瘤发生于SLE确诊后2年.2例患者明确行胸腺瘤切除术.2例患者给予激素合并免疫抑制剂治疗.结论 对于中老年SLE患者要警惕胸腺瘤的发生,对胸腺瘤的患者要监测自身免疫病的发生.糖皮质激素及免疫抑制剂联合治疗仍适用于合并胸腺瘤的SLE患者,应根据胸腺瘤的病理分型给予积极的治疗.切除胸腺瘤对于SLE的预后影响不明确,两者之间的机制需要进一步探索.     

老年男性系统性红斑狼疮伴继发性抗磷脂抗体综合征1例报告

1 病例资料 男性,59岁,退休工人,因“发热、咳嗽、咳黄痰3d”于2016年7月4日入院.患者于3d前无诱因出现发热、咳嗽、咳少量黄痰,自测体温最高约39.5℃,发热前无寒战,就诊于当地医院.血常规:白细胞(WBC) 12.10×109/L,中性粒细胞(N)0.932,红细胞(RBC) 2.68×1012/L,血红蛋白(Hb) 85.00 g/L,血小板212×109/L;尿常规:白细胞(卅),蛋白质(++);肝功能:白蛋白(ALB) 28.1 g/L.     

以肺出血为首发表现的儿童系统性红斑狼疮5例报告并文献复习

目的探讨以肺出血为首发症状的儿童系统性红斑狼疮（SLE）的临床特点,提高诊疗水平。方法对5例病人的临床资料进行回顾性分析,对临床特点和治疗结果进行总结。结果5例病人除肺出血表现外,其他SLE症状均不典型;2例病人经过免疫治疗症状改善,3例死亡。结论以肺出血为首发症状的儿童系统性红斑狼疮发病少见,但病情严重,早期诊断和强化治疗有望改善预后。     

系统性红斑狼疮合并获得性血友病A1例

Acquired hemophilia A (AHA) is anunusual disease resulting from autoantibodies (inhibitors) against coagulation factor Ⅷ (FⅧ) and clinically manifests as bleeding, which sometimes can cause potentially limb-threatening or life-threatening situations. AHA is associated with cancers, auto-immune disorders, infections, dermatologic conditions and certain medications, among which it is commonly secondary to multiple rheumatologic conditions,such as rheumatoid arthritis, systemic lupus erythematosus (SLE), pollymyositis, autoimmune hemolytic anemia and undifferentiated connective tissue disease. In autoimmune diseases, it may be the result of autoantibody producing against FⅧ, and some cases of AHA may act as the first manifestation of SLE. AHA should be suspected in patients who have spon-taneously hemorrhagic events with an isolated prolonged activated partial thromboplastin time (APTT), reduced FⅧ activity and a negative lupus anticoagulant (LA). When FⅧ inhibitor is found, it can be diagnosed. The management of AHA focuses on the following goals: (1)controlling and preventing blee-ding,(2)eradication of the inhibitor,(3)treatment of the underlying disease. Here, a case of AHA in a patient with lupus is reported. A 53-year-old man with a 4-year history of SLE developed arthralgia and ecchymotic skin lesions after arthrocentesis of knee joint. Ultrasound confirmed the presence of an intramuscular hematoma. Coagulation tests revealed that FⅧ activity reduced to 1% and a prolonged APTT (92.2 s), FⅧ inhibitors were found to be as high as 60.0 Bethesda Units. Initial treatments with me-thylprednisolone 200 mg/d were started but new hemorrhagic manifestation occurred and hisbiological indexes were not good. Then the patient was treated with intravenous pulse corticosteroids (methylprednisolone 500 mg/d),intravenous cyclophosphamide, and also plasma and prothrombin complex infusion. Sub-sequently, FⅧ activity returned within normal ranges, FⅧ inhibitors decreased and clinical improvement was significantly obtained. The patient’s condition kept stable till now.Hemorrhagic events due to produc-tion of antibodies directed against coagulation factors were rarely observed in SLE and attentions should be paid to the association between SLE and AHA.Bypass treatment was considered as the immediate antihemorrhagic treatment. Corticosteroid combined with immunosuppressor was recommended as the main therapy to eradicate the inhibitors. However we still lack the therapeutic guide-lines and standar-dized treatment in patients of AHA with SLE at present.     

系统性红斑狼疮合并皮肤脂膜炎多发溃疡1例

SUMMARY A 36-year-old woman had an 8-year history of systemic lupus erythematosus (SLE) and was being treated with 10 mg/d of prednisone.She presented with a 6-month history of intermittent fever and m...     

系统性红斑狼疮合并急性冠状动脉综合征4例报道及文献复习

目的 探讨系统性红斑狼疮(SLE)累及患者冠状动脉的流行病学、病理生理学及临床特点。方法 收集分析4例SLE合并急性冠状动脉综合征(ACS)患者的临床和实验室资料并复习相关文献。结果 绝经期前女性SLE患者中ACS发病率明显增高。ACS是病程长的SLE患者的重要合并症。动脉粥样硬化和血栓形成是累及冠状动脉的最主要原因，冠状动脉血管炎及其他与sSLE活动有关的因素则较为少见。结论 SLE患者存在早发ACS，冠状动脉病((CAD)的发生可能与较长的病程及长期应用激素有关。     

Systemic lupus erythematosus and thrombotic thrombocytopenic purpura: report of three cases

Systemic lupus erythematosus (SLE) is a multisystemic disease characterized by an autoimmune reaction.Thrombotic thrombocytopenic purpura (TTP) is a rare but severe syndrome with the manifestations of fever,thrombocytopenia, microangiopathic hemolysis,neurological symptoms, and renal involvement. The     

SLE患者妊娠期并发多脏器功能障碍综合征7例临床分析

目的: 探讨系统性红斑狼疮(systemic lupus erythematosus,SLE)患者妊娠期并发多脏器功能障碍综合征(mutiple organ dysfunctiong syndrome,MODS)的预防与诊治。方法: 对7例SLE妊娠期引起的多脏器功能损害临床资料进行回顾性分析。结果: 2例妊娠前已确诊为SLE,5例入院后确诊SLE。孕产妇抢救成功6例,1例放弃治疗,围生儿死亡6例。结论: SLE患者妊娠并发多脏器功能障碍对孕产妇及围生儿危害极大,处理的关键是尽早确诊并积极治疗原发病;适时终止妊娠,保护相关脏器功能和全身支持疗法至关重要。