الملخص
Objective To study the clinical char acteristics and outcome of two lymphoma patients mimicking Beh?et's disease. Methods Lymphoma was diagnosed in two patients mimicking Beh?et's disease referred to our Department in 2015. A search on published similar cases in Chinese database and the Pubmed was also performed and then analyzed. Results Eight patients were indentified in this pooled analysis, six of which were non-Hodgkin lymphoma (NHL). All of the eight cases presented with cutaneous lesion, seven cases with fever, seven cases with oral ulceration and six cases with orogenital ulceration, respectively. Ocular involvement was present in four of the eight cases, two were with a positive pathergy test. Among feverish patients, six were moderate or high fever, four were high fever, one was low-grade fever. Neutropenia was found in four patients, and lymphocytoponia in four of five patients with detailed data. All patients fulfilled the 2014 International Criteria for Beh?et's Disease (ICBD) with an average score of (5.8 ±1.5), ranging from 4 to 8. Survival period ranged from one month to 36 months, with an average of 8 months. Conclusion For patients diagnosed as Beh?et's disease are finally diagnosed as lymphoma. For patients with Beh?et's disease present-ation but also present with mediate to high fever, atypical deepseated ulcer, neutropenia or lymphocytoponia, malignancy especially lymphoma should be investigated.
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Objective To analyze the clinical features of systemic lupus erythematosus (SLE) com-plicated by noncirrhotic portal hypertention (NCPH),and improve the recognition of NCPH.Methods Clinical data from SLE complicated by NCPH in our hospital were retrospectively analyzed and summarized,while the related literatures were reviewed.Results Four patients diagnosed as SLE complicated by NCPH were all women.NCPH presented with the clinical features of portal hypertension with normal or slightly elevated transaminase.Anticardiolipin (ACL) antibodies were positive in 2 patients.Two patients underwent liver needle biopsy,showing nodular regenerative hyperplasia,of which,one with liver portal fibrosis.The treatment strategy was managing the primary disorder and controling of portal hypertention in four patients.Twenty-two cases of SLE complicated by NCPH were reviewed and analyzed,including 18 cases from related literatures and our 4 cases.Among the 22 cases,the mean time between the diagnosis of SLE and NCPH was eight years,of which one patient with NCPH before SLE,one diagnosed at the same time and the rest with NCPH after SLE.19％ (4/21) of patients presented with Raynaud's phenomenon and 18％ (4/22) complicated by pulmonary hypertension.In serological tests,patients presented with positive ACL anti-bodies [33％(7/21)] and anti-dsDNA [48％(10/21)],as well as increased IgG and γ-Globulin [38％(8/21)].Liver needle biopsy showed nodular regenerative hyperplasia or liver portal fibrosis with the prevalence of 80％ (16/20) and 25％ (5/20),respectively.Conclusion SLE complicated by NCPH is very rare clinically and is easily being misdiagnosed without obvious symptoms and signs in the early stage.Positive ACL antibodies and Raynaud's phenomenon maybe be closely related to SLE complicated by NCPH.
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Objective To evaluate the clinical and radiographic characteristics and function of erosive hand osteoarthritis (EOA) patients. Methods Data were obtained from 19 patients with EOA, including their social conditions, clinical conditions, radiographic scores and hand function evaluation. The number of hand osteoarthritis (HOA) patients was 312. The control group consisted of non-EOA patients with hand osteoarthritis with a ratio of 4:1 to EOA patients. A non-parameter test analysis was performed. All data were analyzed by SPSS 23.0 statistical analysis, t test, χ2 test, Fisher exact probility and Spearman's correlations analysis were used for statistical analysis. Results Totally data of 19 patients were collected. Eighteen were female. Onset age was (56±8). Average duration was 56 (12~120) months. FIHOA scores of all the EOA patients were at least 5. All the erosions of 39 joints were characteristically central and erosive changes in 7 joints (18%) showed up as gull-wing. Among 39 erosive joints, including 12 (31%) E and 27 (69%) R, 34 (87%) distal interphalangeal joints were involved. Data analysis found out that EOA patients had longer disease duration (Z=2.610, P=0.009), more severe K-L level (44 ±11 vs 26 ±7, t=7.134, P<0.01), higher AUSCAN total score (28±6 vs 21±7, t=3.781, P<0.01) and higher AUSCAN function score (18±6 vs 12±6, t=4.042, P<0.01). The differences of ESR and CRP were not significant between EOA and non-EOA patients. Conclusion Erosions seen in EOA patients are centrally located gull-wing in the DIP joints. EOA patients have longer duration, more severe radiographic damage and worse joint function.
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Objective To investigate the current national situation of autoantibody test in order to improve the quality of autoantibodies test.Methods Hospitals or departments in the whole country participated voluntarily or on invitation.Fifteen samples in total were distributed double-blindly,and autoantibodies including anti-nuclear antibody (ANA),anti-double-stranded DNA (anti-dsDNA) antibody,anti-extractable nuclear antigens (anti-ENA),anti-citrulline antibody (anti-CCP),anti-mitochondria antibody (AMA) and anti-smooth muscle antibody (ASMA) were tested in 6 samples.The samples were used for AMA and ASMA tests.Results A total of 148 hospitals or departments participated and multiple testing methods were adopted.The accurate rate of ANA(97.3％),AMA (96.1％),ASMA (92.1％) and anti-CCP (97.4％) were higher than that of anti-dsDNA (81.9％) and anti-ENA (77.2％).Anti-RNP and anti-Scl70 in anti-ENAs had the lower accurate rate,90.9％ and 80.3％ respectively.Taking data of the past 10 years together,the accuracy of antiSSA,anti-SSB,anti-Sm had been stable since 2009,while that of anti-RNP and anti-Scl70 decreased slightly.For methodology,indirect immunofluorescence was mainly adopted in the testing of ANA,anti-dsDNA,AMA and ASMA,immunoblotting was mainly adopted in anti-ENA detection and enzyme linked immunosorbent serologic assay was used for anti-CCP test.Conclusion No major variation of primary testing method is found in recent 10 years.Although diverged greatly among different methods,the accuracy of antibody detection has improved year by year.
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Objective To analyze the causes of death and prognostic indicators of rheumatoid arthritis (RA).Methods A retrospective investigation was performed on 91 RA patients who were admitted into our hospital from 1990 to 2011.The clinical features and laboratory data were studied to assess the causes of death and the relationship between causes of death and disease activity and treatment.T test and x2 test were used for statistical analysis.Prognostic indicators of mortality were studied by Cox propor-tional hazards models.The prognostic indicators of RA were also analyzed.Results The three most common causes of death in patients with RA were infection (57％),cardiac-cerebral vascular diseases (13％) and cancer (12％).Severe disease activity and presence of extra-articular manifestations were more common among the patients who died.Seventy-five percent patients presented with systemic symptoms including fever,fatigue and weight loss,followed by pulmonary fibrosis (32％) and pleural effusion (27％).Cox regression analysis revealed that the presence of extra-articular features was independent risk determinant for mortality while DMARDs treatment was the independent protect determinant.Conclusion Infection,cardiac-cerebral vascular diseases and cancer are the main causes of death in RA.The presence of extra-articular features is the independent risk factor of RA.
الملخص
Objective To consecutively investigate the quality of auto-antibody testing of the whole country and to improve quality.Methods A nation-wide investigation was carried out and hospitals or departments participating were notified by letter or telephone communication.Autoantibodies tested for quality control survey included anti-nuclear antibody (anti-ANA),anti-double-stranded DNA (anti-dsDNA)antibody,anti-extractable nuclear antigens (anti-ENA) antibody,anti-mitochondria antibody (AMA),anti-smooth muscle antibody (ASMA) and anti-citrulline antibody (anti-CCP).There were 15 samples in total for testing,including 3 control samples for each test.Same sample was used for both AMA and ASMA test.Sample distribution and data analysis were carried out double-blindly.A total of 114 hospitals or departments participated in the survey.Multiple testing methods were adopted including indirect immumo-fluo-rescence (IIF),immuno-blot (IB),dot-blot (DB),double immuno-diffusion (DID),enzyme linked immuno-sorbent assay (ELISA),chemiluminescent assay,dot-immunogold filtration assay.Results The accurate rates for this survey were 98％,96.6％,89.5％,98.1％,92.1％,96.4％ respectively for ANA,anti-dsDNA,anti-ENA,AMA,ASMA and anti-CCP.Anti-ENAs were further divided into anti-RNP,anti-Sm,anti-SSA,anti-SSB and anti-Scl-70 subgroups,and the accurate rates were 88.4％,96.8％,100％,100％ and 95.8％,respectively.Titers of ANA varied greatly among different labs,so did quantitative analysis of anti-CCP,AMA and antidsDNA by ELISA.However,the accuracy of ANA types determined by IIF was greatly improved.Detection rate of AMA and AMSAwas still low.Conclusion Among detected antibodies,ANA,anti-dsDNA and antiCCP are the most prominently improved.Accurate rate of anti-ENA antibody is slightly increased.
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Objective To investigate the clinical and immunological features,therapeutic response as well as prognosis of adult onset Still's disease (AOSD).Methods AOSD was diagnosed in 137 patients referred to our department.Clinical and immunological data were retrospectively analyzed.Therapeutic response and prognosis were systemically reviewed during the follow-up period.Intergroup incidence divergence was analyzed by chi-square test.Cox regression analysis was adopted to determine factors related with relapse.Results Fever,rash and arthritis were the cardinal clinical features of AOSD patients.Elevated inflammatory indices including ferritin (128 suhjects,97.1％ ) along with neutrophilia and liver dysfunction were the main laboratory findings.Ninety-eight patients were followed up and 75％ (73 subjects) had achieved complete remission after 4 weeks treatment.Forty-one patients (42％) who had achieved remission relapsed during follow-up period.Combination of glucocorticoid steroid and disease modifying antirheumatic drugs (DMARDs) were more effective than glucocorticoid steroid only in inducing remission and preventing relapse.More patients received glucocorticoid combined with methotrcxate and hydroxychloroquine achieved remission (8 of 8 patients) than patients who were treated with glucocorticoid and methotrexate (25 of 28 patients,89％ ) and those treated with glucocorticoid and hydroxychloroquine (14 of 16 patients,88％ ).Patients with glucocorticoid were more likely to suffer disease recurrence than those who took glucocorticoid combined with DMARDs (61％ vs 29％,P=0.004).Cox regression analysis suggested that methotrexate had protec-tive effect against recurrence [RR=0.418,95％CI (0.192-0.909),P=0.028].5％ of patients were diagnosed to other diseases during the follow up period.Conclusion Initial treatment with combined glucocorticoid and DMARDs is beneficial to induce remission and prevent reoccurrence.Methotrcxate has a protective effect against recurrence.
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Objective To explore smoking -related health knowledge and attitudes and their association with smoking behaviors among high school students in Shanghai. Methods With stratified random sampling, 9239 students aged 11~20 years old from 57 high schools (19 from each of primary, secondary and professional schools) distributing in whole city were investigated with questionnaire which self -completed anonymously by the students. The contents of the questionnaire included smoking behavior, relation between smoking and health, and attitude to tobacco control policies. Results Of those 9 239 students, there were 19.5% (male 28.2%, female 10.2%) of them who ever smoked; the total mean score of their knowledge and attitude to smoking and health-related issues was 54.42?7.274 (the maximum was 65), and the ratio of total mean score and the maximum score was 0.8065, the highest ratio (0.9767) was found in awareness that smoking was harmful and the lowest (0.3825) in awareness of tobacco control rules and regulations. The scores of each smoking and health -related knowledge and attitude were decreased with increase of cigarette number smoked by students. Compared with other high schools, the lowest scores of each indexes and the highest rate of smoking were found in vocational schools. Conclusion It was found that the smoking behavior was related with their knowledge and attitude that smoking was harmful among high school students in Shanghai. And the tobacco control and intervention should be mainly developed in students of vocational schools.