成人Still病30例临床分析

目的 探讨成人Still病的临床特点和治疗方法，提高对本病的认识和诊断水平。方法 回顾性分析（30例）经临床和实验室确诊的成人Still病患者的临床表现和实验室检查结果。结果 30例患者中发热100％、皮疹86．67％、关节炎和关节疼痛93．33％、WBC升高100％、ESR增快93．33％。有30例患者测定血清铁蛋白，18例升高（60％），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still病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。     

成人斯蒂尔病104例临床和疗效分析

目的 探讨成人斯蒂尔病(AOSD)的临床特点、实验室检查结果及疗效.方法 回顾性分析104例AOSD患者的临床资料,总结临床特征、药物治疗方法和疗效.结果 临床表现:高热100%,皮疹95%,关节痛和(或)关节炎90%,咽痛78%,淋巴结肿大66%,脾脏肿大57%,浆膜炎30%;实验室检查中,中性粒细胞增多98%,肝功能异常62%,红细胞沉降率增快96%,血清铁蛋白升高99%.淋巴结活检者100%为反应性增生.94%应用糖皮质激素,66%患者联合应用了免疫抑制剂;激素用量≥140 mg/d者平均缓解时间缩短,复发率低.结论 不明原因的高热患者,合并皮疹、关节痛,血白细胞和炎症指标升高,排除感染和肿瘤后应考虑AOSD.糖皮质激素和免疫抑制剂是治疗AOSD的最有效药物.     

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.     

以不明原因发热为首发表现的成人斯蒂尔病17例临床分析

反复发热超过38.3℃,病程持续3周以上,住院1周后仍未明确诊断定义为不明原因发热(FUO).成人斯蒂尔病(AOSD)是以发热为突出表现的原因不明的慢性系统性疾病,缺乏统一的诊断标准,以FUO首发的AOSD更易误诊误治.本研究回顾分析了我院17例以FUO首发的AOSD临床特点,并与81例非AOSD引起的FUO的临床特征进行比较,以提高临床医生对该病的诊治能力.     

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.     

血清铁蛋白检测在成人Still病诊断和治疗中的临床价值

目的探讨血清铁蛋白在成人Still病(AOSD)诊断和治疗中的价值。方法本组入选的24例AOSD住院病人均符合日本Yam aguch i诊断标准。回顾性分析患者的临床特征、生化指标(主要为ALT)、血沉(ESR)、血清铁蛋白和治疗措施,并对血清铁蛋白与ALT、ESR进行了相关性分析。结果24例患者中男7例,女17例,年龄12~51岁,平均年龄27.9岁±10.3岁。主要临床表现为发热,病程平均148.4天±246.4天。血清铁蛋白24.8~74700μg/L(6242.6±14888.0),70.8%的患者较正常值有8倍以上的增高,但血清铁蛋白的增高与ALT和ESR之间没有相关性(P>0.05)。24例患者均对糖皮质激素(GCs)治疗有效,SF水平越高者所需GCs剂量越大。结论检测血清铁蛋白对诊断AOSD具有重要的临床意义,其浓度的检测有助于指导临床治疗,而且其水平增高可能与病情活动度有关。     

成人斯蒂尔病:仍然是个谜

成人斯蒂尔病（adult onset Still’s disease，AOSD）是以发热、皮疹、关节痛，炎为三大临床表现，常有网状内皮系统（RES）累及（如咽峡炎、淋巴结、肝脾肿大），以急性时相反应为突出的实验室特征（白细胞、血清铁蛋白增高）的系统性风湿病。从1897年病理学家George F．Still首先报道．后来以他的名字命名的“全身型”幼年性类风湿关节炎（JRA）．到1971年Eric Bywaters正式定名本病，至今对AOSD仍然知之甚少，AOSD的发病机制还不清楚，诊断目前还是排除性的，主要还是经验性的治疗。     

成人斯蒂尔病11例临床分析

成人斯蒂尔病(AOSD)是一种以发热为主要临床表现、不具特异性实验室指标、确诊依赖于排除感染、肿瘤及其他风湿性疾病的疑难病。现将我院住院确诊的11例成人斯蒂尔病患者的临床资料报道如下。     

血清铁蛋白水平对成人斯蒂尔病诊断的临床价值

目的探讨血清铁蛋白在成人斯蒂尔(Still)病诊断中的意义。方法采用诊断试验的研究方法。研究样本为“不明原因发热”患者,即发热3周以上,住院1周以上未能明确诊断者。根据日本成人Still病的Yamaguchi标准和随访的结果,将患者分为成人Still病组和非成人Still病组。分析血清铁蛋白对成人Still病诊断的临床价值。结果横断面研究资料124例中,38例为成人Still病。在成人Still病中,男女间血清铁蛋白水平差异无统计学意义(P=0.7798,功效=0.80)。成人Still病组血清铁蛋白水平显著高于非成人Still病组(P<0.01)。受试者运算特征(ROC)曲线下方的面积为86.8%(95%可信区间为79.5%~94.2%)提示,血清铁蛋白对成人Still病诊断具有重要意义。根据临床实用意义和ROC曲线,选取血清铁蛋白的3个截断点:750滋g/L,1250滋g/L,2500滋g/L,它们的敏感性分别为81.58%,68.42%和39.47%;特异性分别为82.56%,89.53%和96.51%。血清铁蛋白=1250滋g/L能最大程度兼顾敏感性和特异性。如果以Yamaguchi标准为基础,加上血清铁蛋白>1250滋g/L结合作为诊断成人Still病的条件,其特异性则由92.1%上升至99.2%。结论对“不明原因发热”的病人,检测血清铁蛋白,对诊断成人Still病有重要的临床意义。     

英夫利西单抗治疗难治性成人斯蒂尔病一例

患者女,47岁.因"反复发热1个月伴皮疹、关节痛"入院,患者1个月前出现发热,最高39 ℃,伴有反复皮疹,广泛分布于颜面、躯干、四肢,与发热有关,有四肢关节疼痛,无肿胀,曾于当地医院抗感染治疗效果不佳,行骨髓检查,查抗核抗体(ANA)及抗双链DNA(dsDNA)抗体阴性,请我院会诊后拟诊为"成人斯蒂尔病(AOSD)",予以甲泼尼龙80 mg/d治疗2周,同时予以对症支持治疗,患者体温峰值有下降,仍有反复皮疹以及关节痛,转入我院.     

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.     

成人Still’s病免疫机制的研究进展

成人Still＇s病（AOSD）是一种病因不明的以高热、一过性皮疹、关节炎（痛）和白细胞增高等为主要特征、多系统受累的临床综合征。其病因和发病机制至今仍然不清,多数学者认为其发病与感染、遗传及机体的免疫异常有关,特别是对其免疫机制的研究近年来更趋活跃。本文对细胞因子、免疫细胞、免疫球蛋白及血清铁蛋白与成人Still’s病发病的相关性作了概述,以提高对该病免疫机制的认识。     

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     

Etanercept as a rescue agent in patient with adult onset Still’s disease complicated with congestive heart failure

Adult onset Still’s disease (AOSD) is an uncommon disorder of unknown cause. The clinical symptoms of AOSD are a spiking fever, a typical rash, arthralgia or arthritis, sore throat, lymphadenopathy, and splenomegaly. Pleuropulmonary and cardiac involvement are rare. We report a patient with a two-year history of AOSD with myocarditis refractory to cyclosporine and glucocorticoid. Significant congestive heart failure due to left ventricle dysfunction and hyperferritinemia developed during the hospital course. After therapy with etanercept, the patient’s clinical manifestations recovered and she regained normal left ventricular systolic function.     

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.     

Adult-onset Still’s disease: how to make a diagnosis in an atypical case

Adult-onset Still's disease (AOSD) is a complicated multi-systemic inflammatory disease, the main features of which are high spiking fever, evanescent rash, polyarthralgia, lymphadenopathy, hepatosplenomegaly and leukocytosis. The pathogenesis and etiology of AOSD are still unknown. We report a case of atypical AOSD presenting with only arthralgia, fever, lymphadenopathy, slightly elevated serum C-reactive protein and ferritin levels and a slightly elevated erythrocyte sedimentation rate; these signs and symptoms did not fulfill the proposed diagnostic criteria of both Cush and Yamaguchi. After exclusion of other likely diagnoses, a diagnosis of atypical AOSD was made, and a low dose of corticosteroids was effective in resolving all of the patient's symptoms. More clinical practice and research are needed to determine pathogenesis and etiology of AOSD and to amend the diagnostic criteria to include such atypical cases.     

Adult-onset Still’s disease: a report of 28 cases and review of the literature

Adult-onset Still’s disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology. It is characterized by fever, skin rash, polyarthralgias or polyarthritis, sore throat, hepatosplenomegaly, lymphadenopathy, leukocytosis, liver enzyme elevation, and high serum level of ferritin. Several kinds of skin lesions have been reported in this condition. The aim of this study was to assess the clinical and laboratory aspects of 28 patients with AOSD in central Iran. According to the diagnostic criteria of AOSD, we identified 28 patients between 2002 and 2007. We intended to describe the clinical characteristics, treatment, and outcome of our patients with AOSD. Of 28 patients with AOSD, 21 (75%) were female, 7 (25%) were male. Fever (100%), sore throat (92%), Arthralgia (92%), dermatographism (92%), typical rash (85%) and arthritis (60%) were the most common findings. The mean values of laboratory findings were as follows; C-reactive protein (CRP) level of 14.4 mg/dl, erythrocyte sedimentation rate (ESR) of 91.5 mm/h, leukocyte count of 15744.4/μl. Abnormal levels of aspartate aminotransferase and alanine aminotransferase were observed in 25 (89%) patients. Twenty patients (71%) had high ferritin values (>500 ng/ml). The clinical characteristics were similar to previous series. A febrile polyarthritis was the most frequent presentation form. Dermatographism was frequently encountered phenomenon in our patients with AOSD. Being that dermatographism is a simple inducible skin reaction, along with its sensitivity in active disease, we suggest more controlled studies to validate accuracy and positive predictive value of it in convenient clinical setting in the diagnosis of AOSD and to consider including it in diagnostic criteria.     

Adult-onset Still’s disease: a report of 28 cases and review of the literature

Abstract

Adult-onset Still’s disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology. It is characterized by fever, skin rash, polyarthralgias or polyarthritis, sore throat, hepatosplenomegaly, lymphadenopathy, leukocytosis, liver enzyme elevation, and high serum level of ferritin. Several kinds of skin lesions have been reported in this condition. The aim of this study was to assess the clinical and laboratory aspects of 28 patients with AOSD in central Iran. According to the diagnostic criteria of AOSD, we identified 28 patients between 2002 and 2007. We intended to describe the clinical characteristics, treatment, and outcome of our patients with AOSD. Of 28 patients with AOSD, 21 (75%) were female, 7 (25%) were male. Fever (100%), sore throat (92%), Arthralgia (92%), dermatographism (92%), typical rash (85%) and arthritis (60%) were the most common findings. The mean values of laboratory findings were as follows; C-reactive protein (CRP) level of 14.4 mg/dl, erythrocyte sedimentation rate (ESR) of 91.5 mm/h, leukocyte count of 15744.4/μl. Abnormal levels of aspartate aminotransferase and alanine aminotransferase were observed in 25 (89%) patients. Twenty patients (71%) had high ferritin values (>500 ng/ml). The clinical characteristics were similar to previous series. A febrile polyarthritis was the most frequent presentation form. Dermatographism was frequently encountered phenomenon in our patients with AOSD. Being that dermatographism is a simple inducible skin reaction, along with its sensitivity in active disease, we suggest more controlled studies to validate accuracy and positive predictive value of it in convenient clinical setting in the diagnosis of AOSD and to consider including it in diagnostic criteria.     

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.     

Case Report of a Female Patient with Adult-onset Still's Disease and Review of the Literature

Background: Adult-onset Still's disease (AOSD), which presents many non-specific symptoms, such as rash leukocytosis, spiking fever, and sore throat, is a rare auto inflammatory disease. Other clinical features that are frequently observed include lymphadenopathy, arthralgia, serositis, splenomegaly, and hepatomegaly. Laboratory tests show high levels of C-reactive protein, ferritin, and erythrocyte sedimentation rate reflecting the systemic inflammatory process in AOSD patients. Case presentation: The patient was a middle-aged woman with a high fever (39.8 C), sore throat, rashes on limbs with pruritus, mainly at the joints (elbow, knee, and ankle), muscle aches, dizziness, infirmity, weakness, and poor appetite without arthralgia. The ferritin level was above 1500 (normal value: 14-233) ng/L. Antineutrophil, antinuclear antibodies, and rheumatoid factor were negative. Combining the symptoms such as fever, rash, stress-induced acute inflammation, arthritis, and ferritin levels, the patient was eventually diagnosed with adult Still's disease. She received methylprednisolone 40mg intravenously every 12 hours for one week. On the second week, the dose was reduced to 40mg in the morning and 20mg in the evening, and finally, the dose was reduced to 40mg oral intake in the morning and 8mg in the evening. After half a month of treatment, the patient's high fever and skin rashes subsided, and the other symptoms also gradually relieved. Conclusions: A case of a middle-aged woman diagnosed with adult Still's disease is reported, and the possible pathogenesis and treatment of the disease are discussed. This case highlights the importance of early diagnosis and timely treatment of adult Still's disease to prevent potentially fatal complications.