Adult onset still's disease: Clinical features and course

Summary Fiftieen patients with adult onset Still's disease are described, all diagnosed according to recognized criteria. Mean delay in reaching a firm diagnosis was 16 months. Besides the typical clinical picture, there was a high frequency of pruriginous rash, one instance of overlapping polymyositis and recurrent systemic manifeistations in most cases. Chronic polyarticular involvement predominated, with radiological progression particularly in wrist, proximal interphalangeal and hip joints. However, functional prognosis at the end of mean 4.8-year course was satisfactory, as also the response to treatment maionly with steroid drugs and, on occasion, with remiting agent to alleviate arthritis.     

Adult onset Still's disease in North India

Summary Six patients suffering from adult onset of Still's disease were seen over a 10-year period. Contrary to expectations, complications of deformative arthritis (two patients), amyloidosis (one patient), uveitis (one patient) and chronic granulomatous liver disease (one patient) were observed during follow-up. One patient succumbed due to iatrogenic causes; autopsy showed that the pathological changes, like the clinical features, were non-specific. Four patients responded to aspirin and indomethacin; two required corticosteroids.     

Polyserositis with adult Still's disease onset during pregnancy

Summary We report about a 25-year-old female patient, who met all the criteria of adult-onset Still's disease except for the typical nonpruric rash. The disease exacerbated during pregnancy. Polyserositis was the predominant clinical manifestation. The histological examination of the bone marrow biopsy revealed changes similar to those seen in a myelodysplastic syndrome. The clinical condition improved after therapy with high-dose steroids and cyclophosphamid.     

Adult Still's disease and mesangial glomerulonephritis. Report of two cases

Summary Two patients with adult Still's disease and abnormal urinalysis underwent kidney biopsy 2 to 21 years after onset of the disease. Unexpectedly, mesangial glomerulonephritis was discovered. Even if a fortuitous association could not be excluded, the real prevalence of glomerular involvement in ASD should be determined by further studies in view of a potential immune complex pathogenesis of this condition.     

Elderly onset of adult Still's disease: Report of a case

Summary Adult Still's disease is a variant of juvenile rheumatoid arthritis occurring most frequently in women 16–35 years of age, but rarely in elderly people. We describe a 75-year-old woman who was considered as having adult Still's disease.     

Leucopenia in adult Still's disease during treatment with azathioprine and sulphasalazine

Summary The experience is reported concerning treatment of adult Still's disease with a combination of azathioprine and sulphasalazine. In three patients a favourable effect on the symptoms was noted. In one of these patients a year-long prednisone treatment was withdrawn and replaced by sulphasalazine. In all these cases, however, a decrease was seen in leucocyte counts to subnormal levels after the addition of sulphasalazine to a basis therapy with azathioprine 150–200 mg/d (2.1–3.3 mg/kg). The effect was only transitory in two patients, while it lasted several months in the third. In a fourth patient agranulocytosis developed after four days following the combined treatment with azathioprine 150 mg/d (2.3 mg/kg) and 250–1000 mg/d (4–15 mg/kg) of sulphasalazine, lasting four days before recovery. A later resumption of the combination therapy with these same drugs, but in a lower dosage, did not induce any acute changes in leucocyte counts. The skin rash provoked by sulphasalazine in two of the patients did not recur after desensitization. It is concluded, that this combination therapy is valuable in adult Still's disease, but that caution should be exerted during the initiation of sulphasalazine therapy. This should be monitored by daily measurements of leucocyte counts in the first weeks and preferably started by the use of a desensitising kit.     

Adult Onset Still's Disease: A Cause of Pyrexia of Unknown Origin

Abstract: Adult onset Still's disease: a cause of pyrexia of unknown origin. S. Hall, I. Epstein and G. O. Littlejohn. Aust. N.Z. J. Med., 1982, 12 , pp. 522–524.
A case of adult onset Still's disease is reported. A middle aged woman presented with prolonged high fever, rash, polyarthritis, leukocytosis and abnormal liver function tests. The clinical features and multisystem nature of this condition are emphasised.     

Successful methotrexate therapy for adult Still's disease with Marked thrombocytopenia

A 34-year-old Japanese woman developed spiking fever, splenomegaly, arthritis, neutrophilia, hyperferritinaemia (22517 ng/ml), elevated C-reactive protein (9.1 mg/ml) and severe thrombocytopenia (1.7×10⁴/l). The patient had depressed antithrombin III activity and abnormally high concentrations of both fibrin degradation products and thrombin-antithrombin complexes. This condition was resistant to high-dose prednisolone therapy (120 mg/day) and non-steroidal anti-inflammatory drugs. We initiated oral methotrexate therapy (7.5 mg/week, orally) with a favourable outcome. The patient's spiking fever subsided on the first day of methotrexate administration. Elevated levels of ferritin and C-reactive protein in the sera rapidly normalised. Methotrexate rapidly improved the disease state which suggested that methotrexate act via modulation of cytokine production or secretion.     

Biochemical analysis of ferritin subunits in sera from adult Still's disease patients

To determine the origin of the increased serum ferritin that occurs in adult Still's disease (ASD), we analyzed subunits of the serum ferritin as follows. Gel filtration with Sepharose CL-6B demonstrated that the molecular weight of serum ferritin was about 490 kDa. Western blot analysis revealed only L-subunits (molecular weight 19 kDa) in patients with serum ferritin levels higher than 1.000 ng/ml. Patients with serum ferritin levels lower than 1,000 ng/ml, however, showed G-subunits (molecular weight 23 kDa) in addition to the L-subunits. When concanavalin A (Con-A) Sepharose 4B was used in an absorption test, the percentage absorption was extremely low in the patients with serum ferritin levels higher than 1,000 ng/ml. Isoferritin patterns of the patients determined by chromatofocusing revealed traces of acidic ferritin. The findings suggested that glycosylated ferritin does not account for the major portion of the increased serum ferritin.     

Adult-onset Still's disease associated to toxoplasma gondii infection

Summary Adult-onset Still's disease is characterized by high spiking fever, evanescent maculopapular rash and arthritis. Several recent reports have associated its development with a variety of infectious agents. We describe the case of a 25-year old woman presenting high fever, maculopapular rash and seronegative polyarthritis associated with lymphoadenopathy, splenomegaly and neutrophil leucocytosis together with acute acquired toxoplasmosis. Other causes of systemic illness were excluded by appropriate laboratory, radiological and histological investigations. Clinical, radiological and laboratory findings as well as possible etiopathogenetic correlations among both pathological conditions are discussed. Toxoplasma gondii should be considered as a further possible triggering agent associated with the development of adult-onset Still's disease.     

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.     

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。