托珠单抗治疗重症成人Still病后继发巨噬细胞活化综合征2例报告

<正>成人Still病（adult-onset Still’s disease,AOSD）是一种病因不明的全身性炎症性疾病,临床表现包括高热、皮疹、关节痛和（或）关节炎、咽痛、淋巴结肿大、肝脾肿大等;实验室检查包括血白细胞、红细胞沉降率（ESR）、C反应蛋白（CRP）、白介素（IL）-6及血清铁蛋白升高。治疗上包括非甾体抗炎药物、糖皮质激素、免疫抑制剂及细胞毒性药物等,大多数患者病情都能得到控制,但仍有少部分患者病情危重、难治,传统的治疗常常难以控制病情。有研究证实,AOSD活动期多种炎症细胞因子水平升高,其中IL-6水平在AOSD活动期明显升高,     

成人斯蒂尔病伴严重肝功能异常临床分析7例

目的:总结成人斯蒂尔病(adult onset Still's disease,AOSD)伴严重肝功能异常患者临床特征,提高对该病的认识.方法:回顾性分析北京佑安医院2000年以来诊断为AOSD、同时伴有严重肝功能异常的患者临床资料,总结患者发生严重肝功能异常的可能原因.结果:7例患者病程10 d-5年,全部出现发热(100%);伴随或相继出现的症状包括:皮疹(100%)、咽痛(71.4%)、关节痛(71.4%)、淋巴结肿大(42.8%)、肝大和/或脾大(71.4%),严重肝功能异常(100%),合并亚急性肝衰竭1例,合并噬血综合征1例.AOSD伴严重肝功能异常可能与糖皮质激素剂量或疗程不够、感染和/或药物诱发、未能及时正确诊断、延误治疗有关.结论:加强对AOSD的认识,早期诊断、重视治疗过程的管理有助于减少重症肝损伤的发生.     

成人Still病92例临床分析

<正>成人Still病(adult-onset still disease,AOSD)是一种病因不明的以高热、皮疹、多关节痛、淋巴结肿大、白细胞增多、肝功异常等多系统受累为主要特征的临床综合征。由于本病没有特异性的临床表现和实验室检测指标,因此诊断标准缺乏特异性,临床诊断需要在充分的鉴别诊断基础上做出,易造成误诊和漏诊~([1-2])。同时,本病对于药物的治疗反应变异很大,目前也缺乏统一的包括治疗用药、剂量等指导性     

成人斯蒂尔病并烟雾病一例

成人斯蒂尔病(adult onset Still's disease,AOSD)是一种急性全身性炎性疾病,病因及发病机制尚不清楚,临床特点为高热、一过性多形性皮疹、关节炎或关节痛,并伴有周围血粒细胞增高,肝、脾及淋巴结肿大,浆膜炎等.     

不同指标在成人斯蒂尔病中诊断结果分析

目的 评价临床资料和现行诊断标准在成人斯蒂尔病(AOSD)中的诊断价值.方法 研究对象为2003年1月至2009年6月复旦大学附属中山医院诊断的AOSD患者,同期发热患者为对照.收集临床资料,分析诊断价值.采用美国风湿病学会(ARA)、Yamaguehi、Cush和Calabm标准诊断入选者,确定诊断效率.采用两样本均数的t检验,wricoxon秩和检验进行统计学处理.结果 皮疹、关节疼痛或肿胀、咽痛、肌肉疼痛、淋巴结或肝脾肿大、白细胞升高、中性粒细胞≥80%、铁蛋白≥2000 ng/ml特异度较高(65.87%～98.41%),阳性似然比2.00～5.00.高热(≥39.0℃)、抗核抗体(ANA)和类风湿因子(RF)阴性的敏感度较高(85.25%～93.65%).高热、皮疹、咽痛、白细胞升高、关节痛等指标任意3项组合,阳性似然比>10.00.ARA标准的特异度最高,Yamaguchi标准的敏感度和准确率最高.结论 单一表现诊断AOSD把握度小,不同项目联合可提高诊断效率.目前国际上通用的诊断标准具有较高特异度.     

成人Still病32例临床分析

目的探讨成人Still病的临床特点,提高诊断的正确率。方法回顾分析32例成人Still病的临床表现及化验检查,分析其临床特点。结果高热（体温≥39．0℃）、一过性皮疹、关节痛、白细胞、中性粒细胞增高（白细胞≥12．0×10^9／L,中性粒细胞≥80％）、血清铁蛋白增高,血沉、C-反应蛋白增高,肝脾淋巴结肿大是多见的表现,非甾体类抗炎药加糖皮质激素或／和免疫抑制剂是常用的治疗方法。结论成人Still病临床表现及实验室检查缺乏特异性,诊断时应符合参考诊断标准并除外其他疾病。     

成人still''s病59例临床分析

目的探讨成人斯蒂尔氏病(AOSD)的临床特点、伴随症状和误诊原因,以提高对本病的认识和诊断水平.方法回顾性分析59例经临床和实验室确诊的成人斯蒂尔氏病患者的临床表现和实验室检查结果.结果59例患者中发热100%、皮疹78%、关节炎和关节疼痛74%、WBC升高95%、ESR增快91%.有32例患者测定血清铁蛋白,26例升高(81%),病情好转后均明显下降(P<0.01).结论发热、皮疹和关节/关节疼痛是AOSD的主要临床特点,若有WBC升高则诊断AOSD的敏感性和特异性明显增高,血清铁蛋白可作为疾病活动的指标.     

托珠单抗治疗成人Still病研究进展

正成人Still病(adult-onset Still’s disease,AOSD)是一种病因不明的系统性炎症紊乱性疾病。患者的临床征象与具有系统性幼年特发性关节炎(systemic-onset juvenile idiopathic arthritis,SOJIA)且达不到类风湿关节炎标准的儿童症状相似~([1])。临床表现包括发热、皮疹、关节痛和关节炎、咽喉痛、淋巴结肿大、心包炎和胸腔积液~([2])。实验室检查包括     

成人斯蒂尔病:关注不同的临床表型和治疗

成人斯蒂尔病(adult-onset Still's disease,AOSD)是一种病因及发病机制不明,主要表现为发热、关节痛和(或)关节炎、皮疹、咽痛、肝脾及淋巴结肿大、白细胞总数和中性粒细胞增多,严重者可伴多系统损害的全身综合征,由Bywater[1]在1971年首先报道.     

成人Still病24例临床特征分析

<正>成人Still病(AOSD)是以高热、关节痛、皮疹以及外周血白细胞升高为主要表现的全身性疾病,其病因及发病机制尚未完全明了。AOSD临床表现复杂,无特异性诊断方法,故临床上误诊率较高。以往普遍认为AOSD预后良好,但近年来我院收治的AOSD合并急性肝脏功能衰竭、噬血细胞综合征、急性呼吸窘迫综合征等重症病例亦不在少数。2014—2015年我院收治了24例AOSD患者,其中4例合     

Clinical features and prognosis in adult-onset still's disease: a study of 104 cases

The objective of this study is to evaluate the clinical features and prognosis of adult-onset Still's disease (AOSD). One hundred and four AOSD patients who were analyzed retrospectively were enrolled in this study. Medical charts were systematically reviewed for: demographic data, clinical features, laboratory findings, treatments, and outcomes. The major clinical features were: spiking fever 100%, evanescent maculopapular rash 95%, polyarthralgia 90%, sore throat 78%, lymphadenopathy 66%, hepatosplenomegaly 57%, hydrohymenitis 30%, neutrophilia 98%, liver disfunction 62%, increased erythrocyte sedimentation rate (ESR) 96%, and hyperferritinaemia 99%. Reactive hyperplasia was shown in all patients who underwent lymph node biopsy. Ninety-five percent and 63% of the patients were treated with glucocorticoid and immune suppressant, respectively. Those with prednisone or its equivalent dosage of ≥0.8 mg/kg/d achieved quicker remission and less relapse. Persistent fever, evanescent rash, arthritis, and sore throat were the most prevalent symptoms in patients with AOSD, with laboratory findings of leukocytosis, elevated liver enzymes, elevated ESR and serum ferritin. Glucocorticoid and immune suppressive drugs are effective for AOSD; however, the relapsing rate is relatively high. High levels of white blood cells, serum ferritin and ESR, as well as glucocorticoid dosage were related to relapse.     

Onset of adult-onset Still’s disease following influenza vaccination

We describe that case of a 61-year-old woman who developed high spiking fever, sore throat, polyarthralgia, and salmon pink evanescent rash following influenza vaccination. A diagnosis of adult-onset Still’s disease (AOSD) was made based on clinical and laboratory findings. Methylprednisolone pulse therapy followed by oral prednisolone resulted in a favorable outcome. This is the second published case in which a causal relationship between vaccination and onset of AOSD is suggested. Bystander activation would appear to play an important role in inducing the immune reaction.     

Onset of adult-onset Still’s disease following influenza vaccination

Abstract

We describe that case of a 61-year-old woman who developed high spiking fever, sore throat, polyarthralgia, and salmon pink evanescent rash following influenza vaccination. A diagnosis of adult-onset Still’s disease (AOSD) was made based on clinical and laboratory findings. Methylprednisolone pulse therapy followed by oral prednisolone resulted in a favorable outcome. This is the second published case in which a causal relationship between vaccination and onset of AOSD is suggested. Bystander activation would appear to play an important role in inducing the immune reaction.     

Urticaria as a Presenting Manifestation of Adult-Onset Still’s Disease

Adult-onset Still’s disease (AOSD) is a rare disorder of unknown aetiology, characterised by high spiking fever, an evanescent, erythematous, maculopapular rash, arthralgia or arthritis, lymphadenopathy, hepatosplenomegaly, sore throat and serositis. It is associated with marked leukocytosis, high erythrocyte sedimentation rate, increased level of serum ferritin and negative rheumatoid factor and antinuclear antibody tests. Here we report a patient in whom an urticaria-like rash was an uncommon presenting clinical feature of AOSD. To our knowledge, this association has only been reported once before. Received: 5 October 1999 / Accepted: 13 March 2000     

A case of adult onset Still’s disease with systemic inflammatory response syndrome complicated by fatal status epilepticus

Prolonged spiking fever, an evanescent salmon-colored rash, arthralgia or arthritis, leukocytosis and organ dysfunction are characteristic of adult onset Still's disease (AOSD). A 25-year-old woman with fever lasting over 3 weeks presented to our clinic. The patient had a spiking fever, sore throat, tender lymph nodes, a fine pink-colored skin rash, arthralgia, myalgia with a high ESR, ferritin and elevated hepatic enzymes. NSAID and prednisolone were prescribed for AOSD with SIRS. After 4 days of therapy, with mild confusion, the patient went into status epilepticus lasting several hours and died after cardiovascular collapse. There has been only one case of status epilepticus associated with AOSD in the medical literature. Here we report a case of AOSD with SIRS complicated by fatal status epilepticus.     

Fever of unknown origin: a review of 20 patients with Adult-onset Still's disease

 In this study we aimed to investigate the findings in patients with adult-onset Still's disease (AOSD) admitted with fever of unknown origin (FUO) during the last 18 years in our unit, in order to discover the ratio of such patients to all patients with FUO during the same period, and to determine the clinical features of AOSD in FUO. The number and the aetiologies of the patients with FUO diagnosed between 1984 and 2001, and the clinical features of those with AOSD, were taken from the patient files. The diagnosis of AOSD was reanalysed according to the diagnostic criteria of Cush et al. [11]. The presumed diagnoses before a diagnosis of AOSD was established were also noted. The χ² and Fisher's exact tests were used for statistical analysis. We studied 130 patients with a diagnosis of FUO, 36 (28%) of whom had collagen vascular diseases. Of these 36 patients, 20 (56%, 12 female, 8 male, mean age 34 years, range 16–65) had AOSD. Clinical and laboratory findings were as follows: fever (100%), arthralgia (90%), rash (85%), sore throat (75%), arthritis (65%), myalgia (60%), splenomegaly (40%), hepatomegaly (25%), lymphadenopathy (15%), anaemia (65%), neutrophilic leukocytosis (90%), increased erythrocyte sedimentation rate (100%), elevated transaminase levels (65%), a negative RF (100%), and a negative FANA (80%). Antibiotics had been prescribed in 18 (90%) of cases. The presumed infectious diagnoses were streptococcal tonsillitis/pharyngitis (50%), infective endocarditis (four patients), sepsis (two patients) and acute bacterial meningitis (two patients). The presumed non-infectious diagnoses were acute rheumatic fever (three patients), seronegative rheumatoid arthritis (two patients) and polymyositis (two patients). Sixteen patients were followed for a mean duration of 30 months (range 2–59). A remission was obtained with indomethacin in three cases (19%), and with prednisolone in the remainder. Relapse was detected in three cases (19%). AOSD is one of the most frequent aetiologies of FUO. During the diagnostic course of a patient with FUO, a maculopapular rash and/or arthralgia and/or sore throat should raise the suspicion of AOSD. Because the disease has heterogeneous clinical findings, certain bacterial infections (e.g. streptococcal pharyngitis and sepsis) are generally considered and the prescribing of antibiotics is common. Received: 3 May 2002 / Accepted: 2 October 2002     

Retrospective study of 61 patients with adult-onset Still's disease admitted with fever of unknown origin in China

Adult-onset Still’s disease (AOSD), as a category of connective tissue diseases, has about 5∼9% of fever of unknown origin (FUO) cases. Diagnosis of AOSD was challenging because of its nonspecific characteristics. The present study analyzed clinical manifestations and laboratory findings in a series of patients with AOSD from eastern China. Medical records of 61 patients admitted with FUO and with a discharge diagnosis of AOSD were retrospectively evaluated and analyzed with special focus on clinical manifestations and laboratory findings. Compared with previous reports, most features of our patients had a similar incidence rate. Rash (79%), arthralgia (80%), and sore throat (84%) were the most frequent clinical manifestations in our series. Leukocytosis (80%), elevated ESR (98%) and CRP (100%), negative ANA (90%) and RF (93%), and high ferritin level (94%) were the most sensitive laboratory findings in our patients. AOSD was not a rare reason of FUO in eastern China. Fever, arthralgia, rash, sore throat, leukocytosis, neutrophilia, elevated ESR and CRP, negative ANA and RF, and high ferritin level were the most common clinical features in our series. The lack of highly specific characteristic makes the diagnosis of AOSD difficult compared with other diseases in FUO.     

Comparison of clinical features of childhood and adult onset Still's disease]

Physical findings, laboratory data, treatments and prognosis were investigated in detail using 26 Japanese childhood Still's disease (CHSD) patients and 19 Japanese adult onset Still's disease (AOSD) patients as the subjects. High spiking fever and arthritis were present in all the patients. Seventy and seven percent of CHSD and 53 percent of AOSD had polyarthritis (the number of joints involved being 5 or more during the first 6 months of the disease). A comparison of the groups showed no significant difference in the initial systemic manifestations except for sore throat (CHSD: AOSD; 19%: 68%). Initial laboratory data were the same for these groups except for serum iron levels (CHSD: AOSD; 20.8 +/- 13.7 micrograms/dl: 83.0 +/- 54.2 micrograms/dl). As to joints and physical prognosis, the results were also the same for CHSD and AOSD under the similar treatment. On the basis of these data, we conclude that CHSD and AOSD are of the same disease entity so far as the present clinical features are concerned.