Multimodal imaging diagnosis and analysis of prognostic factors in patients with adult-onset Coats disease

AIM:To describe the multimodal imaging features,treatment,and outcomes of patients diagnosed with adultonset Coats disease.METHODS:This retrospective study included patients first diagnosed with Coats disease at≥18 years of age between September 2017 and September 2021.Some patients received anti-vascular endothelial growth factor(VEGF)therapy(conbercept,0.5 mg)as the initial treatment,which was combined with laser photocoagulation as needed.All the patients underwent best corrected visual acuity(BCVA)and intraocular pressure examinations,fundus color photography,spontaneous fluorescence tests,fundus fluorescein angiography,optical coherence tomography(OCT),OCT angiography,and other examinations.BCVA alterations and multimodal image findings in the affected eyes following treatment were compared and the prognostic factors were analyzed.RESULTS:The study included 15 patients who were aged 24-72(57.33±12.61)y at presentation.Systemic hypertension was the most common associated systemic condition,occurring in 13(86.7%)patients.Baseline BCVA ranged from 2.0 to 5.0(4.0±1.1),which showed improvement following treatment(4.2±1.0).Multimodal imaging revealed retinal telangiectasis in 13 patients(86.7%),patchy hemorrhage in 5 patients(33.3%),and stage 2B disease(Shield’s staging criteria)in 11 patients(73.3%).OCT revealed that the baseline central macular thickness(CMT)ranged from 129 to 964μm(473.0±230.1μm),with 13 patients(86.7%)exhibiting a baseline CMT exceeding 250μm.Furthermore,8 patients(53.3%)presented with an epiretinal membrane at baseline or during follow-up.Hyper-reflective scars were observed on OCT in five patients(33.3%)with poor visual prognosis.Vision deteriorated in one patient who did not receive treatment.Final vision was stable in three patients who received laser treatment,whereas improvement was observed in one of two patients who received anti-VEGF therapy alone.In addition,8 of 9 patients(88.9%)who received laser treatment and conbercept exhibited stable or improved BCVA.CONCLUSION:Multimodal imaging can help diagnose adult-onset Coats disease.Anti-VEGF treatment combined with laser therapy can be an option for improving or maintaining BCVA and resolving macular edema.The final visual outcome depends on macular involvement and the disease stage.

Managing adult-onset Still's disease in pregnancy:A case report

BACKGROUND Adult-onset Still’s disease(AOSD)is a rare systemic inflammatory disorder characterized by fever,arthritis,skin rash,and systemic symptoms.The etiology of AOSD is unknown;however,it is thought to be related to immune dysregulation.Although a rare disease,AOSD can significantly impact reproductive health,particularly during pregnancy.This case study assesses the implications of pregnancy in a patient with AOSD,as well as the potential for heredity of the disease.Neonatal hemophagocytic lympho-histiocytosis(HLH)is a rare and lifethreatening disorder characterized by hyperinflammation and uncontrolled activation of immune cells,leading to multiple organ dysfunction.This case report aimed to introduce neonatal HLH from a mother with AOSD.CASE SUMMARY This case study presents a 29-year-old female with AOSD who became pregnant and gave birth to a premature infant who was diagnosed with neonatal HLH.AOSD can significantly impact pregnancy and childbirth,as it may become more severe during pregnancy,with an increased risk of fetal loss and preterm birth.The management of AOSD during pregnancy involves the use of nonsteroidal anti-inflammatory drugs and glucocorticoids,as well as immunosuppressive agents in severe cases.However,the use of immunosuppressive agents during pregnancy may be associated with potential risks to the fetus.The hereditary implications of AOSD are unclear;however,available evidence suggests that genetic factors may play a role in the disease development.CONCLUSION AOSD can have significant implications for pregnancy and childbirth,including an increased risk of fetal loss and preterm birth.Neonatal HLH,a complication of AOSD in pregnancy,requires prompt diagnosis and management.Women with AOSD who are considering pregnancy should discuss their options with their healthcare provider and develop a management plan that addresses the potential risks to both mother and fetus.

Adult-Onset Still's Disease Misdiagnosed as Acute Fibrinous and Organizing Pneumonia: A Case Report and Literature Review

Adult-onset Stil's disease(AOSD)is a rare condition that lies between autoinflammatory syndrome and autoimmune disease.The main clinical manifestations include fever,chills,rash,joint swelling and pain,peripheral blood leukocytosis,splenomegaly,etc.It is a systemic disease affecting between 1 and 34 people per million.The average age of onset is 35 years old,with a slightly higher prevalence rate in women.Since AOSD lacks early specific symptoms and signs,non-specialist doctors have limited understanding of the disease,and patients are prone to clinical misdiagnosis,mistreatment,and delayed disease progression.This paper reports a patient whose AOSD was misdiagnosed as acute fibrinous and organizing pneumonia.

Hepatitis A virus-associated acute acalculous cholecystitis in an adult-onset Still’s disease patient:A case report and review of the literature

BACKGROUND Acute acalculous cholecystitis(AAC)is inflammation of the gallbladder without evidence of calculi.Although rarely reported,its etiologies include hepatitis virus infection(e.g.,hepatitis A virus,HAV)and adult-onset Still’s disease(AOSD).There are no reports of HAV-associated AAC in an AOSD patient.CASE SUMMARY Here we report a rare case of HAV infection-associated AAC in a 39-year-old woman who had a history of AOSD.The patient presented with an acute abdomen and hypotension.Elevated hepatobiliary enzymes and a thickened and distended gallbladder without gallstones on ultrasonography suggested AAC,but there were no signs of anemia nor thrombocytopenia.Serological screening revealed anti-HAV IgM antibodies.Steroid treatment did not alleviate her symptoms,and she was referred for laparoscopic cholecystectomy.The resected gallbladder was hydropic without perforation,and her clinical signs gradually improved after surgery.CONCLUSION AAC can be caused by HAV in AOSD patients.It is crucial to search for the underlying etiology for AAC,especially uncommon viral causes.

Prevalence of Myocilin Gene Mutation in Adult-Onset Primary Open Angle Glaucoma and Non-Glaucoma Subjects Who Are Indigenes of Rivers State, Nigeria

Background: Glaucoma is the leading cause of irreversible blindness incapacitating over 80 million people worldwide. Several pathogenetic mechanisms have been postulated to explain the optic nerve damage that occur in POAG among which genetic predisposition is prominent. Gene-Linkage-based studies have identified genes associated with POAG: Myocilin, Optineurin, WDR36, Tank-Binding Kinase (TBK1) and APbb-2. Objective: To investigate the prevalence of myocilin gene mutation in adult-onset POAG patients and non-glaucoma subjects who are indigenes of Rivers State. Methodology: In this comparative cross-sectional study, 393 POAG patients attending the Glaucoma Clinic of UPTH were compared with 393 age and sex-matched phenotypically normal participants. Clinical assessment combined with findings from clinical records was used. Venous blood was obtained for genomic analyses. Extracted DNA was sequenced with specific primers for myocilin and polymerase chain reaction. Zymo-Bead Genomic DNA kit protocol was used to detect allelic differences. Results: Total of 786 participants participated in the study. The mean age was 59.8 ± 11.8 years. The prevalence of myocilin gene mutation (MYOC) in the study population was 5.3%, in the POAG group was 8.4%, and 2.3% in the non-glaucoma group. This observed difference was statistically significant (p = 0.001). Location of the mutant myocilin gene was in GLC1A 171638779, 171638703, 171638610 and 171638608. Conclusion: Mutations in myocilin gene are associated with adult-onset POAG in Rivers State. Its relevance as a biomarker for diagnosis of adult-onset POAG needs further investigations.

Adult-onset Still's disease evolving with multiple organ failure and death:A case report and review of the literature

BACKGROUND Adult-onset Still’s disease(AOSD)is a rare systemic inflammatory disease,which is characterized by daily fever and arthritis,with an evanescent rash and neutrophilic leukocytosis.To date,there has been no definite laboratory or imaging test available for diagnosing AOSD;the diagnosis is one of exclusion,which can be very challenging.In particular,AOSD patients may experience different complications affecting their clinical picture,management,and prognosis.The treatment of AOSD remains largely empirical and involves therapeutic agents.CASE SUMMARY We report the case of a 36-year-old woman who presented with fever,red rash,arthralgia,and sore throat.Her serum ferritin level and white blood cell count were markedly elevated,and the first diagnosis 22 years prior was"juvenile rheumatoid arthritis of systemic type".The patient was treated with prednisone,sulfasalazine,methotrexate,and leflunomide.After remission of her symptoms,the patient stopped taking the medications,and the disease recurred.Ultimately,the patient was diagnosed with adult-onset Still's disease.Relapse occurred several times due to self-medication withdrawal,and an interleukin-6 antagonist(tocilizumab/Actemra)was administered to control the disease.Recently,she was hospitalized because an incision did not heal,and the patient suddenly developed high fever and diarrhea during hospitalization.The patient's disease progressed violently and quickly developed into macrophage activation syndrome,disseminated intravascular coagulation,shock,and multiple organ failure.The patient had sudden cardiac arrest,and she died despite emergency rescue efforts.CONCLUSION AOSD patients need regular follow-up in the long-term treatment process,and must press formulary standard medication,and do not voluntarily withdraw or reduce the dose.Otherwise it may cause disease back-and-forth or serious lifethreatening complications.Meanwhile,strict management of trauma,infections,tumors,and other diseases may contribute to improved outcomes in patients with complications.

Adult-onset Still's disease: A case report

Rationale: Fever of unknown origin (FUO) is a frequently observed phenomenon in clinical practice. Definite diagnosis of FUO is a great challenge in clinical practice since potential causes for FUO involve more than 200 diseases. Adult-onset Still's disease is a defined clinical entity and a known rare cause of FUO. Patient's concern: A 19-year girl was referred to the clinic with the concern of intermittent fevers and shivering for almost a year despite multiple investigations and consultations. She had undergone intensive serologic, radiologic, laboratory investigations to exclude infectious diseases, connective tissue diseases, and malignancy, and all the investigation showed no conclusive diagnosis. Diagnosis: Adult-onset Stills disease. Intervention: Steroids and supportive treatment. Outcomes:The symptoms were relieved within three days, and the patient became asymptomatic. Lessons: Physicians need to be familiar with the diagnostic criteria of adult-onset Still's disease, or it shall remain a diagnostic dilemma. Besides, all shivers are not infections.

Successful treatment of adult-onset still disease caused by pulmonary infection-associated hemophagocytic lymphohistiocytosis: A case report

BACKGROUND Adult-onset still disease(AOSD) and hemophagocytic syndrome(HPS) are two inflammatory diseases with very similar clinical manifestations. HPS is one of the most serious complications of AOSD and its risk of death is very high. It is difficult to identify HPS early in patients with AOSD, but early identification and proper treatment directly affects the prognosis.CASE SUMMARY A 39-year-old male showed a high spiking fever and myalgia. Laboratory data revealed elevated white blood cell, serum ferritin, and neutrophil percentage.However, his fever failed to relieve after a clear diagnosis of AOSD caused by pulmonary infection and treatment by antibiotics and corticosteroids;further laboratory data showed elevated serum ferritin, C-reactive protein, erythrocyte sedimentation rate and triglyceride, as well as liver abnormalities. Bone marrow smear showed hemophagocytosis. Secondary HPS was definitely diagnosed. The high fever disappeared and the laboratory findings returned to normal values after treatment by high-dose intravenous methylprednisolone and methotrexate.CONCLUSION For AOSD patients with high suspicion of HPS, active examination needs to be considered for early diagnosis, and timely using of adequate amount of corticosteroids is the key to reducing risk of HPS death.

Identification of novel biomarkers for adult-onset-immunodeficiency(AOID)syndrome using serum proteomics

Objective:To identify the candidate protein biomarkers of adult-onset-immunodeficiency(AOID) syndrome using serum proteomics. Methods:Screening and verification phases were performed in the study. A total of 97 serum samples were classified into three groups:AOID patients with opportunistic infections(active AOID),AOID patients without opportunistic infections(inactive AOID),and healthy control. In the screening phase,pooled sera collected from patients and healthy control in each group were separated by 2D-gel electrophoresis,analyzed for differentially expressed proteins and identified for biomarkers using LC/MS. In the verification phase,the protein candidates were selected for confirmation by western blotting. Results:The analysis revealed 35 differentially expressed proteins. Three proteins including haptoglobin,gelsolin,and transthyretin,were selected for verification. The results showed that the levels of haptoglobin in both active and inactive AOID groups were significantly higher than that in the control group,while the levels of gelsolin in the active AOID group were significantly lower than that in the inactive AOID group. The level of transthyretin in the active AOID group was also significantly lower than that in the control group. Conclusions:The comparison of serum proteins between the three groups revealed three candidates which are related to chronic inflammatory diseases. Haptoglobin and transthyretin biomarkers could be applied in clinical assessment for monitor of disease outcome,including for the study of AOID pathogenesis.

Atypical adult-onset Still’s disease with an initial and sole manifestation of liver injury: A case report and review of literature

BACKGROUND Adult-onset Still's disease(AOSD)typically presents with a high spiking fever,polyarthritis,transient maculopapular rash,neutrophilic leukocytosis,and hepatosplenomegaly.It has a wide spectrum of clinical symptoms ranging from mild to severe,with extensive involvement of almost every organ.Although liver involvement in the form of increased hepatic enzymes and bilirubin is common,no AOSD case with liver involvement as the initial manifestation of AOSD has been reported.CASE SUMMARY A 35-year-old woman presented to the hepatology department with progressively worsening jaundice for one week.Liver chemistry tests revealed a significantly increased liver enzymes and bilirubin level.Given that the clinical examination was unremarkable,liver biopsy was considered because the patient had a history of AOSD 6 years ago.Liver histopathology revealed that most hepatic lobules were still recognizable.Fusional necrosis was observed around most central veins.A few bridging necrotic zones were also found.Infiltration of multiple plasma cells were observed in the necrotic zone,and the reticular scaffold was still expanded.Additionally,no obvious fibrosis was observed in the portal area.Mild mixed inflammatory cell infiltration was noted in the interstitium of the portal area.Further examination was unremarkable except for a remarkably high level of ferritin.Collectively,a presumptive diagnosis of liver injury secondary to AOSD was made.The hepatic involvement responded well to glucocorticoid treatment.CONCLUSION This case highlights that hepatic involvement as an initial and sole manifestation could be a pattern of relapsed AOSD.The diagnosis of AOSD should be considered in the case of nonresolving liver injury after the exclusion of common etiologies for liver diseases.A liver biopsy can be useful for the differential diagnosis of liver injury associated with AOSD.

Increased human neutrophil lipocalin and its clinical relevance in adult-onset Still’s disease

Background:Human neutrophil lipocalin(HNL)has been used extensively to differentiate acute bacterial infection from febrile diseases as a biomarker to reflect the activation of the neutrophil.The serum HNL levels in the adult-onset Still’s disease(AOSD)patients with and without infection,as well as the healthy controls(HCs),were analyzed statistically in this study to evaluate the value of HNL for the diagnosis of AOSD.Methods:A total of 129 AOSD patients were enrolled,from whom blood samples were drawn and the AOSD diagnosis was confirmed through the review of the medical records,where the systemic score,demographic characteristics,clinical manifestations,and laboratory parameters were also collected for the patients;in addition,a total of 40 HCs were recruited among the blood donors from the healthcare center with the relevant information collected.The HNL test was done for the blood samples with the enzyme-linked immunosorbent assay and the analyses were done for the correlations of HNL with clinical manifestations and diagnostic effectiveness.Results:The serum HNL increased significantly in the patients with only AOSD as compared with that in the HCs(139.76±8.99 ng/mL vs.55.92±6.12 ng/mL;P<0.001).The serum HNL level was correlated with the white blood cell(WBC)count(r=0.335,P<0.001),neutrophil count(r=0.334,P<0.001),erythrocyte sedimentation rate(r=0.241,P=0.022),C-reactive protein(r=0.442,P<0.0001),and systemic score(r=0.343,P<0.0001)in the AOSD patients significantly.Patients with fever,leukocytosis≥15,000/mm^(3),and myalgia in the HNL-positive group were observed relatively more than those in the HNL-negative group(P=0.009,P=0.023,and P=0.007,respectively).HNL was a more sensitive indicator than ferritin and C-reactive protein(CRP)to differentiate the AOSD patients with bacterial infection from AOSD-only patients,and the Youden index was 0.6 for HNL and 0.29 for CRP.Conclusion:Serum HNL can be used as a biomarker for the diagnosis of the AOSD,and HNL is also observed to be associated with the disease activity.

王振亮运用四逆汤治疗成人斯蒂尔病经验

成人斯蒂尔病是一种多因素参与的自身炎症性疾病,以发热、皮疹、关节疼痛和多器官受累为特点,目前尚无根治办法。西医治疗以非甾体类抗炎药、糖皮质激素、免疫抑制剂及生物制剂治疗为主。王振亮教授认为阳虚寒凝血瘀是成人斯蒂尔病核心病机,临床表现以阴阳不和、寒凝血瘀为主,也常夹湿、郁热,病位在半表半里,病性寒热错杂,以温阳益气立法,以四逆汤为核心方,同时重视顾护脾胃以及治血药的运用,谨守病机随证配伍,灵活运用。

LMNB1相关常染色体显性遗传成人型脑白质营养不良家系分析

目的总结一个LMNB1相关常染色体显性遗传成人型脑白质营养不良(adult-onset autosomal dominant leukodystrophy,ADLD)家系的临床、影像学特点,并进行致病性变异分析。方法选取2023年11月首都医科大学宣武医院确诊为ADLD患者1例,收集先证者及家系其他患者的临床和影像学资料,对先证者进行全外显子组测序分析及LMNB1基因的多重连接探针扩增(multiplex ligation-dependent probe amplification,MLPA)检测,并进行致病性分析和表型匹配分析。结果本例患者(先证者)男,44岁,因“大小便障碍3年,双下肢乏力2年余”就诊,基因检测示LMNB1基因1-11号外显子重复变异,评定为致病性变异,表型与ADLD匹配,先证者(Ⅲ:9)和Ⅲ:6以自主神经功能障碍起病,系ADLD典型表现,Ⅱ:11首发症状为头部震颤,与其他患者不同。结论ADLD患者的LMNB1基因1-11号外显子重复变异,符合常染色体显性遗传方式。成年患者以自主神经功能障碍起病,随后累及小脑、锥体束,MRI示广泛对称的脑白质病变、脊髓萎缩时应考虑ADLD,建议行LMNB1基因检测。

CSF1R基因突变致ALSP发病研究进展

成人发病的白质脑病合并轴索球样变和色素性胶质细胞(adult-onset leukoencephalopathy with axonal spheroids and pigmented glia,ALSP)是临床罕见的常染色体显性遗传病,其具体的发病机制目前还未明确。集落刺激因子1受体(colony-stimulating factor 1 receptor,CSF1R)是一种细胞表面跨膜酪氨酸激酶受体,与其相关的编码基因突变已被证实是ALSP的潜在致病因素。然而,目前关于CSF1R基因突变致使ALSP发病的具体机制尚不清楚。本文回顾CSF1R基因在ALSP发病过程中的突变位点及致病机制研究,发现CSF1R突变可以通过显性负性效应、功能丧失、单倍体剂量不足及功能获得等机制导致小胶质细胞功能异常,进而引起ALSP的发病。对ALSP病因的深入认识有助于更好地探索潜在的治疗方法。

Pathogenesis,disease course,and prognosis of adult-onset Still’s disease:an update and review

Objective:Adult-onset Still's disease(AOSD)is a rare but clinically well-known polygenic systemic autoinflammatory disease.In this review,we aim to present frontiers in the pathogenesis,clinical features,diagnosis,biomarkers,disease course,prognosis,and treatment in AOSD.Data sources:We retrieved information from the PubMed database up to July 2019,using various search terms and relevant words,including AOSD and Still's disease.Study selection:We included data from peer-reviewed journals.Both basic and clinical studies were selected.Results:Pathogenesis of AOSD involves genetic background,infectious triggers,and immunopathogenesis,mainly the activation of macrophages and neutrophils followed by a cytokine storm.Diagnosis and prognosis evaluation of AOSD is still challenging;therefore,there is an urgent need to identify better biomarkers.Biologic agents,including interleukin(IL)-ip,IL-6,and tumor necrosis factor-a antagonists in the treatment of AOSD,have good prospect.Conclusion:This review highlights the advances in pathogenesis,potential biomarkers,disease course,and treatment in AOSD.

线粒体丙氨酰t-RNA合成酶2基因突变相关的脑白质病1例及文献回顾

线粒体丙氨酰t-RNA合成酶2(alanyl-transfer RNA synthetase 2,AARS2)基因突变相关的脑白质病(AARS2 leukoencephalopathy,AARS2-L)是一种罕见的迟发性脑白质营养不良,文献报道仅32例。本文报道1例44岁女性患者进行性认知障碍下降、精神行为异常5年,同时合并卵巢功能衰退;头部磁共振成像显示双侧大脑半球广泛的白质脱髓鞘;基因全外显子测序发现AARS2基因复合杂合突变(c.179 C>A和c.2752 C>T),结合临床特征该患者被诊断为AARS2-L。同时,对目前已报道的AARS2-L病例的临床特征和基因表达特点等进行了回顾与分析。

Adult-onset Still’s disease successfully treated with Chinese herbal medicine: A case report with 15-month follow-up

Adult-onset Still’s disease(AOSD) is a rare but clinically well-known, polygenic, and systemic autoinflammatory disease, which is characterized by spiking fever, evanescent skin rash, arthralgia, and sore throat.The application of non-steroidal anti-inflammatory drugs and glucocorticoids, which are first-line therapies of AOSD, is limited due to their side effects such as liver injury or disorder of blood glucose.Therefore, patients who suffer from systemic diseases in China prefer to seek help from Chinese herbal medicine(CHM), which is an important part of complementary and alternative medicine. In this case,we report a 28-year-old male badminton coach presenting with a 15-day history of fever and skin rash,accompanied by sore throat, fatigue, myalgia and chills. Additionally, hepatosplenomegaly, multiple lymphadenopathies, aminotransferase abnormality, and elevated inflammatory factor levels were observed during hospitalization. Infectious diseases, solid tumors, hematological diseases, and common autoimmune diseases were excluded. Not benefitting from antibiotic therapy, the patient was finally diagnosed with AOSD, after a careful examination, then showed rapid remission after a six-week treatment with CHM granules based on Xiaochaihu Decoction and Yinqiao Powder. After stopping the treatment, there was no relapse within a 15-month follow-up period. To the best of our knowledge, this is the first well-documented case of this successful treatment. The present case report suggests that CHM is a reliable choice for complementary and alternative therapy for AOSD, but confirming the utility of CHM for AOSD requires further support from prospective studies.

健脾清热除湿汤治疗成人发病型特应性皮炎经验

成人发病型特应性皮炎指患者发病年龄大于等于18岁的特应性皮炎,其临床表现与婴儿期、儿童期特应性皮炎有所不同,适应的诊断标准也不同,近年来逐渐受到重视。宋业强教授结合多年临床经验,提出脾虚湿热是成人发病型特应性皮炎的关键病机,并自拟健脾清热除湿汤治疗该证型患者,取得了很好的临床效果。文章以验案为例,介绍成人发病型特应性皮炎的相关内容。

成人斯蒂尔病与其他不明原因发热疾病的鉴别诊断指标

目的探讨成人斯蒂尔病(AOSD)与其他不明原因发热(FUO)疾病的鉴别诊断指标。方法收集2010年1月-2021年5月在陆军军医大学第一附属医院住院的177例AOSD患者及163例待鉴别FUO患者的临床资料及实验室指标,随机分为训练组及验证组。通过单因素分析提取有统计学意义的变量进行受试者工作特征(ROC)曲线分析并获取变量的最佳截断值,进一步通过多因素logistic回归分析筛选出具有鉴别诊断意义的指标,并构建列线图模型;采用ROC曲线、校准曲线及决策曲线分析列线图的准确性及稳定性。结果单因素分析结果显示,4项临床特征(关节痛、皮疹、咽痛、肌痛)及14项实验室参数[白细胞计数(WBC)、单核细胞百分比、中性粒细胞百分比、淋巴细胞百分比、血小板计数、C反应蛋白、白细胞介素-6(IL-6)、铁蛋白、球蛋白、免疫球蛋白A、免疫球蛋白G(IgG)、肌酸激酶、肌酐、补体C3]差异均有统计学意义(P<0.05)。多因素分析结果显示,关节痛、WBC≥9.995×109/L、IL-6≥98.13ng/L、铁蛋白≥507.37ng/ml、球蛋白≤36.58g/L、IgG≤13.59g/L、补体C3≥1.27g/L均与AOSD相关(P<0.05)。训练组及验证组的曲线下面积(AUC)分别为0.917[95%可信区间(95%CI)0.883~0.951]、0.869(95%CI0.802~0.936);校准曲线表现出良好的一致性;决策曲线分析表明,训练组及验证组分别在5%~85%、10%~85%广大风险范围内显示出较大的正向收益率。结论该研究建立起一个相对准确的AOSD鉴别诊断模型,关节痛、WBC、IL-6、铁蛋白、球蛋白、IgG及补体C3多指标联合应用有助于鉴别AOSD与其他FUO病因。

成人斯蒂尔病发病机制及相关临床特征的研究进展

成人斯蒂尔病是一种少见的、病因尚不明确的全身性自身炎症性疾病,其具有临床症状表现复杂而不典型、缺乏统一有效的相关检查以辅助诊断、临床治疗效果差异性大等特点,在临床上通常难以明确诊断并给予规范化治疗。目前关于成人斯蒂尔病发病机制的研究方向较多,本文结合相关文献总结以上研究成果,供后续相关科研工作参考。