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BACKGROUND@#Antiphospholipid syndrome (APS) is an autoimmune prothrombotic condition with significant morbidity. The objective of this study was to identify additional clinical and epidemiological risks of arterial thrombosis, venous thrombosis, and pregnancy morbidities in a large cohort of persistent antiphospholipid antibodies (aPLs)-positive carriers.@*METHODS@#This was a cross-sectional cohort study of 453 consecutive patients with a documented positive aPL who attended Peking University People's Hospital. Among 453 patients screened, 297 patients had persistent positive aPL. We compared asymptomatic aPL carriers with thrombotic and obstetric APS patients. And the univariate analysis and multivariable logistic regression were used to evaluate the association between different risk factors and APS clinical manifestations. The levels of circulating markers of neutrophil extracellular traps (NETs) (cell-free DNA and citrullinated histone H3 [Cit-H3]) were assessed and compared among aPL-positive carriers with or without autoimmune disease and APS patients.@*RESULTS@#Additional risk factors associated with arterial thrombosis among aPL-positive carriers included: smoking (odds ratio [OR] = 6.137, 95% confidence interval [CI] = 2.408-15.637, P = 0.0001), hypertension (OR = 2.368, 95% CI = 1.249-4.491, P = 0.008), and the presence of underlying autoimmune disease (OR = 4.401, 95% CI = 2.387-8.113, P < 0.001). Additional risks associated with venous thrombosis among aPL carriers included: smoking (OR = 4.594, 95% CI = 1.681-12.553, P = 0.029) and the presence of underlying autoimmune disease (OR = 6.330, 95% CI = 3.355-11.940, P < 0.001). The presence of underlying autoimmune disease (OR = 3.301, 95% CI = 1.407-7.744, P = 0.006) is the additional risk, which demonstrated a significant association with APS pregnancy morbidity. Higher circulating levels of cell-free DNA and Cit-H3 were observed among APS patients and aPL patients with autoimmune diseases compared with those aPL carriers without underlying autoimmune diseases. Furthermore, control neutrophils that are conditioned with APS patients'sera have more pronounced NET release compared with those treated with aPL carriers'sera without underlying autoimmune diseases.@*CONCLUSIONS@#We identified several potential additional risk factors for APS clinical manifestations among a large cohort of Chinese aPL carriers. Our data may help physicians to risk stratify aPL-positive Asian patients.
Subject(s)
Female , Humans , Pregnancy , Antibodies, Antiphospholipid , Antiphospholipid Syndrome/complications , Autoimmune Diseases , Cell-Free Nucleic Acids , Cohort Studies , Cross-Sectional Studies , Morbidity , Risk Factors , Thrombosis/etiologyABSTRACT
Objective:To analyze the association between thrombocytopenia and clinical manifestations in patients with antiphospholipid syndrome (APS), and the significance of adding thrombocytopenia to the classification criteria of APS.Methods:One hundred and sixty one APS patients admitted to Peking University People's Hospital from January 2003 to August 2017 were retrospectively analyzed. Clinical and laboratory data were compared between patients with and without thrombocytopenia. Chi-square test, Fisher's exact test and t test were used for statistical analysis. Results:Among 161 APS patients, 48 patients (29.8%) were primary APS and 113 patients (70.2%) were secondary APS. Ninety-one patients (56.5%) experienced thrombocytopenia, while 62 patients (38.5%) had thrombocytopenia as the initial symptom. Among primary APS patients, compared with patients without thrombocytopenia, the prevalence of thrombosis was significantly lower in patients with thrombocytopenia (40.0% vs 69.6%, χ2=4.218, P=0.040), especially venous thrombosis (20.0% vs 47.8%, χ2=4.174, P=0.041). Among secondary APS patients, compared with patients without thrombocyt-openia, the prevalence of thrombosis, arterial thrombosis (21.2% vs 42.6%, χ2=5.944, P=0.015) and venous thrombosis (22.7% vs 51.1%, χ2=9.753, P=0.002) was significantly lower in patients with thrombocytopenia (43.9% vs 80.9%, χ2=15.496, P<0.01). As for laboratory findings, primary APS patients with thrombocytopenia showed a higher proportion of positive aCL findings (80.0% vs 52.2%, χ2=4.174, P=0.041), while secondary APS patients with thrombocytopenia experienced more leukopenia (25.8% vs 10.6%, χ2=4.002, P=0.045), lower complement C3 levels (78.7% vs 44.4%, χ2=13.205, P<0.01) and complement C4 levels (74.6% vs 46.7%, χ2=8.485, P=0.004). The proportion of patients fulfilling 1988, 1989, 1999 and 2006 APS classification criteria in our cohort was 88.2%, 87.0%, 64.0% and 70.8%, respectively. Inclusion of thrombocytopenia into 2006 criteria improved the proportion to 96.3%. Conclusion:The prevalence of thrombosis in APS patients with thrombocytopenia is significantly low. Thrombocytopenia is an important clinical manifestation of APS which can improve the diagnostic accuracy of APS.
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Objective:To investigate the level and the influencing factors of blood uric acid in monks and nuns in Wutai Mountain area, and to explore the relationship between blood uric acid level and BMI and blood lipids levels.Methods:Physical examinations and laboratory tests were performed on monks and nuns in Wutai Mountain area. There were 207 males and 261 females. Physical examination includes height, weight, blood pressure, blood uric acid, blood lipid, blood glucose and other indicators. The blood uric acid level was measured using the uricase method. Chi-square test for trend, and t test were utilized for statistical analysis. Results:The average blood uric acid level of the monk and the nun was (372±6) μmol/L and (290±4) μmol/L, respectively. Obviously, the average blood uric acid level of the monk was significantly higher than it in the nun ( t=11.636, P<0.01). The total incidence rate of hyperuricemia, which was diagnosed when the blood uric acid level was higher than 420 μmol/L in males and the blood uric acid level was higher than 360 μmol/L in females. In particular, the incidence rate of hyperuricemia was much higher in the monk (24.3%, 50/207) than in the nuns (13.4%, 35/261) ( χ2=8.966, P<0.01) . Analysis by age, the prevalence of hyperuricemia in men was 20.3%(42/207) before the age of 50, which was higher than that after the age of 50 (3.9%, 8/207) ( χ2=26.3, P< 0.01); The prevalence of hyperuricemia in women before the age of 50 was 2.7%(7/261), which was lower than that after the age of 50 (10.7%, 28/261) ( χ2=13.51, P<0.01). The uric acid level of men and women between 50-60 years old, showed the opposite trend. The level of uric acid in men decreased and increased in women. In addition, the prevalence of triglyceride abnormalities and overweight was more significantly in monks and nuns with high uric acid than those with the normal uric acid level [71.8%(61/85) vs 45.2%(173/383), χ2=19.68, P<0.01; 54.1%(46/85) vs 19.8%(76/383), χ2=42.4, P<0.01]; while no significant difference of total cholesterol and blood glucose levels was observed between these two groups. Conclusion:There are differences in blood uric acid levels among Wutai Mountain area monks and nuns of different ages and genders. The level of blood uric acid in male is significantly higher than that in female. Lipid metabolism and over weight are closely related to elevated level of the uric acid, which might be the risk factors of uric acid abnormality in Wutai Mountain population.
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Objective:To investigate the role of conjunctival impression cytology in the diagnosis of Sj?gren's syndrome (SS) and the immunological factors influencing conjunctival lesions.Methods:A total of 57 patients complaining about dry eye were collected, including 38 patients with primary Sj?gren's syndrome (pSS) and 19 patients with non-SS. Conjunctival impression cytology tests were performed for all patients, and they were scored by the Nelson method. Thirty-one patients with SS underwent serological tests such as autoantibodies, immunoglobulins, and complement. The correlation between the relevant data was compared using the t test and the rank sum test. Results:The Nelson grade ≥2 is the positive cut-off value for the diagnosis of SS. The sensitivity was 68.4%, and the specificity was 89.5%, and the area under the receiver operating characteristic curve (ROC) was 0.767. In patients with SS, there was statistical correlation between the results of conjunctival impression cytology and antinuclear antibody (ANA) ( χ2=4.664, P=0.031), anti-SSA antibody ( χ2=8.58, P<0.01), anti-SSB antibody ( χ2=6.13, P=0.013), anti-SSA-52 antibody ( χ2=6.48, P=0.011), immunoglobulin (Ig)G ( t=-4.344, P<0.01) and rheumatoid factor (RF) ( U=25.0, P<0.01). Conclusion:Con-junctival impression cytology has certain value in the diagnosis of SS and can be used to evaluate conjunctival lesions in SS. Serum ANA, anti-SSA antibody, anti-SSB antibody, anti-SSA-52 antibody, IgG, and RF levels are significantly associated with the degree of conjunctival lesions, and can be considered as an indirect evidence of conjunctival involvement in SS.
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Objective To investigate the causes of misdiagnosis related to rheumatism diseasecases.Methods A search was performed in database WanFang to identify the misdiagnosed clinical cases reports which were published in Chinese Journal and a retrospective analysis was conducted.All data were analyzed by chi-square test and Fisher's exact test.Results We screened 705 citations and identified 215 articles on the rheumatic diseases,finally,187 reports and 195 cases in total with definite diagnosis were included,accounting for 25.5％ of the total number of misdiagnosed cases.Primary vasculitis (72 cases),rheumatoid arthritis (25 cases),spondyloarthropathy (17 cases),polymyositis (14 cases),systemic lupus erythematosus (13 cases) and Sj(o)gren's syndrome (SS) (12 cases) were amongst the top misdiagnosed rheumatic diseases.There was no difference between 1998-2006 and 2007-2015 in the overall misdiagnosis cases.Single disease comparison,polymyositis,spondyloarthropathy and IgG4 related disease were increased (P=0.002;P=0.034;P=0.060;respectively),while polymyositis was delayed (P=0.002).Rheumatism misdiagnosed cases reported mainly came from tertiary hospitals and the department of rheumatology.Conclusion Misdiagnosed rheumatism are common in clinic.Strengthen the physicians' continuous education,the validity of clinical thinking mode and rational use of diagnostic criteria are important to make correct diagnosis.
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Objective@#To evaluate the effect of epigallocatechin gallate (EGCG) on T helper cell 1 (Th1) and Th2 in psoriasis patients.@*Methods@#A total of 33 patients with plaque-type psoriasis vulgaris were enrolled, and peripheral blood mononuclear cells (PBMC) were isolated and cultured. The appropriate concentration of EGCG was determined by methyl thiazol tetrazolium (MTT) assay. PBMC at exponential growth phase were divided into 2 groups to be treated with EGCG (EGCG group) or not (control group) for 24 hours. Flow cytometry was performed to determine proportions of Th1 and Th2 cells, enzyme-linked immunosorbent assay (ELISA) to detect levels of Th1 (interleukin[IL]-2, interferon[IFN]-γ) and Th2 cytokines (IL-4, IL-10) in the cell culture supernatant, and real-time quantitative RCR (qRT-PCR) to determine the mRNA expression of T-bet (a Th1 transcription factor) and GATA3 (a Th2 transcription factor) . Statistical analysis was carried out by using t test.@*Results@#According to the MTT assay results, EGCG at a non-toxic concentration of 60 μmol/L was chosen for subsequent experiments. Compared with the control group, the EGCG group showed significantly decreased number of Th1 cells (t = 3.43, P = 0.026) , increased number of Th2 cells (t = 6.68, P = 0.026) , and decreased Th1/Th2 ratio (P < 0.05) . The levels of IL-2 and IFN-γ in the culture supernatant of PBMC were both significantly lower in the EGCG group (824.45 ± 101.21 ng/L, 1 623.62 ± 185.56 ng/L respectively) than in the control group (1 568.32 ± 196.45 ng/L, 3 287.63 ± 235.54 ng/L respectively) , while the levels of IL-4 and IL-10 were significantly higher in the EGCG group (389.48 ± 46.63 ng/L, 285.95 ± 53.28 ng/L respectively) than in the control group (225.38 ± 26.92 ng/L, 165.46 ± 32.25 ng/L respectively) . Compared with the control group, the EGCG group showed significantly decreased T-bet mRNA expression (t = 11.99, P < 0.001) , but increased GATA3 mRNA expression (t = 18.62, P < 0.001) .@*Conclusion@#EGCG can reduce the number of Th1 cells, inhibit the production of Th1 cytokines and transcription factors, and increase the number of Th2 cells and the production of Th2 cytokines and transcription factors, followed by the modulation of Th1/Th2 immune imbalance.
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Objective To evaluate the effect of epigallocatechin gallate (EGCG) on T helper cell 1 (Th1) and Th2 in psoriasis patients.Methods A total of 33 patients with plaque-type psoriasis vulgaris were enrolled,and peripheral blood mononuclear cells (PBMC) were isolated and cultured.The appropriate concentration of EGCG was determined by methyl thiazol tetrazolium (MTT) assay.PBMC at exponential growth phase were divided into 2 groups to be treated with EGCG (EGCG group) or not (control group) for 24 hours.Flow cytometry was performed to determine proportions of Th 1 and Th2 cells,enzyme-linked immunosorbent assay (ELISA) to detect levels of Th1 (interleukin [IL]-2,interferon [IFN]-γ) and Th2 cytokines (IL-4,IL-10) in the cell culture supernatant,and real-time quantitative RCR (qRT-PCR) to determine the mRNA expression of T-bet (a Th1 transcription factor) and GATA3 (a Th2 transcription factor).Statistical analysis was carried out by using t test.Results According to the MTT assay results,EGCG at a non-toxic concentration of 60 μmol/L was chosen for subsequent experiments.Compared with the control group,the EGCG group showed significantly decreased number of Th1 cells (t =3.43,P =0.026),increased number of Th2 cells (t =6.68,P =0.026),and decreased Th1/Th2 ratio (P ＜ 0.05).The levels of IL-2 and IFN-γin the culture supernatant of PBMC were both significantly lower in the EGCG group (824.45 ± 101.21 ng/L,1 623.62 ± 185.56 ng/L respectively) than in the control group (1 568.32 ±196.45 ng/L,3 287.63 ± 235.54 ng/L respectively),while the levels of IL-4 and IL-10 were significantly higher in the EGCG group (389.48 ± 46.63 ng/L,285.95 ± 53.28 ng/L respectively) than in the control group (225.38 ± 26.92 ng/L,165.46 ± 32.25 ng/L respectively).Compared with the control group,the EGCG group showed significantly decreased T-bet mRNA expression (t =11.99,P ＜ 0.001),but increased GATA3 mRNA expression (t =18.62,P ＜ 0.001).Conclusion EGCG can reduce the number of Th1 cells,inhibit the production of Th 1 cytokines and transcription factors,and increase the number of Th2 cells and the production of Th2 cytokines and transcription factors,followed by the modulation of Th 1/Th2 immune imbalance.
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Objective To investigate the clinical characteristics,immunological features,treatment and follow-ups of Sj(o)gren's syndrome-associated monoclonal gammopathy (SS-MG).Methods A retro-spective,case-control study was conducted for 18 cases diagnosed with SS-MG and 36 age-and sex-matched non-MG-SS patients from Janurary 2010 to Janurary 2017 in Peking University People's Hospital.The clinical and laboratory features,treatment and follow-ups were recorded and compared.Comparisons between groups were made using t test for normally distributed numerical data,Mann-Whitney U test for non-normally distributed numerical data,and Pearson Chi-square,continuity correction or Fisher's exact tests for categorical data.Results SS patients,when complicated with MG,had significantly increased level of TP [(78± 11) g/L,(71±10) g/L,t=-2.382,P=0.021] and erythrocyte sedimentation rate (ESR) [52.5(45.3) mm/1 h,33.0(42.5) mm/1 h,Z=-2.179,P=0.029],higher prevalence of urine NAG positivity [75％(9/12),28％(7/25),x2=7.298,P=0.007],hypoglobulinemia [33％(6/18),3(1/36),x2=7.407,P=0.006] and thrombotic events [17％(3/18),0％(0/36),P=0.033],and less previous exposure to glucocorticoid [22％(4/18),64％(23/36),x2=8.333,P=0.004],compared to the control group.Primary SS patients complicated with MG had significantly higher ESSDAI [26.0(25.0),12.0 (9.0),Z=-2.724,P=0.006] and Clin EULAR Sj(o)gren's syndrome disease activity index (ESSDAI) [24.0(25.0),10.5 (10.0),Z=-2.523,P=0.011].Among the 18 patients,2 were diagnosed with multiple myeloma,1 was diagnosed with non-Hodgkin lymphoma,and the left were diagnosed as MG of undetermined significance (MGUS).Ten patients were followed up,among whom 2 patients with MGUS experienced increased levels of M protein,newly developed genetic abnormalities,and renal involvement.Conclusion SS patients may be complicated with MG.MGUS is the most common form.However,malignant hematologic disorders are revealed as well.In SS patients with high serum TP and ESR,hypoglobulinemia,tubulointerstitial kidney involvement and unexplained thrombotic events,especially in those with high disease activity,so MG should be an alert for further work-ups,monitoring and treatment.
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Objective To investigate the diagnostic and prognostic value of immunoglobulin (Ig)G isotype rheumatoid factors (IgG-RF) in rheumatoid arthritis (RA).Methods Five hundred patients with RA were enrolled randomly.IgG-RF antibody was detected by enzyme-linked immunosorbent assay (ELISA).The correlations between serum IgG-RF antibody and clinical features,disease activities,laboratory of RA patients were evaluated.The comparison of continuous variables was performed by using the Student t-test or Mann-Whitney U test in accordance with normality testing.Chi-square test was performed for categorical variables.A value of P less than 0.05 was considered statistically significant.Results ① IgG-RF was positive in 41.0％ (205/500) of RA patients.In patients with anti-citrullinated protein/peptide autoanti-bodies (ACPA) negative,RF negative or the seronegative patients (both ACPA and RF were negative),the positive rate of IgG-RF was 22.4％(24/107),13.2％(17/129) and 9.1％(5/55),respectively.② Compared with patients with negative IgG-RF,patients with positive IgG-RF had higher rates of joint deformity [(58.5％(120/205) vs 39.3％(116/295),x2=17.918] and bone erosion [(75.6％(118/156) vs 60.3％(140/232),x2=9.796] (P＜0.01,respectively).③ The patients with positive IgG-RF had higher rates of elevated ESR(86.3％ vs 67.8％,x2=22.426),IgG(29.9％ vs 20.0％,x2=6.310),compared to patients with negative IgG-RF (P＜0.05,respectively),and levels of ESR [(59±35) mm/1 h vs (47±32) mm/1 h,t=3.989] and CRP [(390±450) mg/L vs (290±340) mg/L,t=3.004] was higher in IgG-RF positive group than the negative (P＜0.01,respectivelys).④ Compared with the IgG-RF negative patients,the positive group had higher smoking rates (22.9％ vs 12.5％,x2=9.227),higher current smoking rates (16.6％ vs 7.1％,x2=11.119) and higher smoking index [(107±238) vs (49±161),t=3.199](P＜0.05,respectively).Conclusion IgG-RF had its clinical values in RA diagnosis.IgG-RF is significantly associated with joint deformity,bone erosions and smoking.
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Objective To evaluate the clinical value of Chinese Multi-Dimensional Health Assessment Questionnaire (MDHAQ-C) in patients with rheumatoid arthritis (RA). Methods From December 2015 to October 2016,four hundred and twelve RA patients were recruited in this study and completed the MDHAQ-C independently and routine assessment of patient index data 3 (RAPID3) was calculated. Then correlations were assessed by Spearman correlation coefficient of RAPID3 with indices of clinical relevance and disease activity to evaluate its clinical values. Results MDHAQ-C is moderately correlated with ESR (r=0.399), CRP (r=0.381), SJC (r=0.499), TJC (r=0.558) (P<0.01), but highly correlation with VAS of pain (r=0.836), VAS of global status (r=0.915) and overall assessment of physician (r=0.784)(P<0.01), and highly correlated with a Spearman's coefficient of 0.784 for DAS 28-ESR, 0.734 for CDAI and 0.682 for SDAI (P<0.001). Conclusion MDHAQ is a reliable, valid instrument for disease activity assessment and a feasible index for clinical settings in Chinese RA patients.
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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 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 investigate the common initial clinical presentations of primary Sj(o)gren's syndrome (pSS) with pulmonary complications,and to explore the differences between patients with extraglandular manifestations at disease onset (EGM) and those with glandular manifestations at disease onset (GM).Methods A total of 1 341 hospitalized SS patients from 2003 to 2012 were retrospectively reviewed.Of them,102 hospitalized patients with pSS'associated lung disease were analyzed and included.Case control study was performed to explore the differences between the EGM group and the GM group.Results Fifty-one percent of patients were presented with EGM at onset,with significantly shorter disease duration [36 (12,156) m vs 102 (48,159) m,x2=-2.41,P=0.016].Although the mean diagnose time was similiar,only 4％ of the EGM group could be confirmed the pSS diagnose at onset,which was significantly less frequently than that of the GM group (34％,22=15.29,P＜0.01).Case control study revealed that hyperglobulinemia,elevated RF titers and anti-SSA and/or anti-SSB test positive were less predominant in the EGM group [IgG 16(12,21) g/L vs 21 (15,28) g/L,x2=-2.15,P=0.032;22 (20,171) U/ml vs 104 (20,238) U/ml,x2=-l.98,P=0.048;33％ vs 72％,x2=15.78,P＜0.01].The predicted value of TLC and FVC were lower [(87±23)％ vs (97±20)％,x2=-1.96,P=0.050;(8±28)％ vs (100±27)％,x2=-1.70,P=0.089] and HRCT score was higher in EMG group [12(88,15) vs 8(5,13),x2=-1.82,P=0.070].Conclusion EMG at onset is the common initial manifestation of pSS'associated lung involvement.Pulmonary complication is more progressively and severe than those with MG at onset.Anti'SSA positive,elevated RF titer and hyperglobulinemia are not predominant for patients with EMG at onset.
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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 detect the anti-citrullinated alpha-enolase peptide 1 (CEP-1) antibody in rheumatoid arthritis (RA). Methods One hundred and twenty-nine patients with RA were enrolled randomly. Thirty-one patients with primary Sj?gren's syndrome (pSS), 32 patients with systemic lupus erythematosus (SLE), 32 patients with osteoarthritis (OA), and 106 healthy controls (HC) were include into this study. Anti-CEP-1 antibody was detected by enzyme-linked immunosorbent assay (ELISA). The correlations between serum anti-CEP-1 antibody and clinical features, disease activities,laboratory tests or Sharp scores of RA patients were evaluated. Mann-Whitney U test and χ2 test were used for statistical analysis. Results ①Anti-CEP-1 antibodies were positive in 64.3%(83/129) of RA patients, 22.6%(7/32) of pSS patients, 12.5%(4/32) of SLE patients, none of OA patients (0/32) or healthy controls. The positivity of anti-CEP-1 antibody was significantly higher than those in pSS ( χ2=17.7), SLE ( χ2=25.7), OA ( χ2=42.5), and healthy controls ( χ2=102.6) (P<0.01, respectively). The specificity of anti-CEP-1 antibody in RA was 94.5%. ②In patients without anti-citrullinated protein/peptide autoantibodies (ACPA), rheumatoid factor (RF) or the patients without ACPA and RF, the positive rate of anti-CEP-1 antibody was 30.3%(10/33), 41.9%(18/43) and 22.7%(5/22), respectively. ③Compared with patients without anti-CEP-1 antibodies, patients with anti-CEP-1 anti-bodies had higher rates of joint deformity, bone erosion and high disease activities (P<0.05, respectively). ④ Higher rate of interstitial lung disease (ILD) was found in RA patients with anti-CEP-1 antibody (19.3% vs 4.3%, χ2=5.494, P<0.05). ⑤The patients with anti-CEP-1 anti-body had higher rates of elevated erythrocyte sedimentation rate (ESR) ( χ2=6.543) and decreased serum albumin ( χ2=6.59), compared to patients without anti-CEP-1 antibody (P<0.05, respectively). Conclusion Anti-CEP-1 antibody has high sensitivity and specificity for RA diagnosis. Combination of anti-CEP-1 antibody with other RA antibodies might improve the early diagnosis of RA. Anti-CEP-1 antibody is significantly associated with joint damage, disease activity and pulmonary interstitial fibrosis.
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Objective To detect the anti-citrullinated alpha-enolase peptide 1 (CEP-1) antibody in rheumatoid arthritis (RA). Methods One hundred and twenty-nine patients with RA were enrolled randomly. Thirty-one patients with primary Sj?gren's syndrome (pSS), 32 patients with systemic lupus erythematosus (SLE), 32 patients with osteoarthritis (OA), and 106 healthy controls (HC) were include into this study. Anti-CEP-1 antibody was detected by enzyme-linked immunosorbent assay (ELISA). The correlations between serum anti-CEP-1 antibody and clinical features, disease activities,laboratory tests or Sharp scores of RA patients were evaluated. Mann-Whitney U test and χ2 test were used for statistical analysis. Results ①Anti-CEP-1 antibodies were positive in 64.3%(83/129) of RA patients, 22.6%(7/32) of pSS patients, 12.5%(4/32) of SLE patients, none of OA patients (0/32) or healthy controls. The positivity of anti-CEP-1 antibody was significantly higher than those in pSS ( χ2=17.7), SLE ( χ2=25.7), OA ( χ2=42.5), and healthy controls ( χ2=102.6) (P<0.01, respectively). The specificity of anti-CEP-1 antibody in RA was 94.5%. ②In patients without anti-citrullinated protein/peptide autoantibodies (ACPA), rheumatoid factor (RF) or the patients without ACPA and RF, the positive rate of anti-CEP-1 antibody was 30.3%(10/33), 41.9%(18/43) and 22.7%(5/22), respectively. ③Compared with patients without anti-CEP-1 antibodies, patients with anti-CEP-1 anti-bodies had higher rates of joint deformity, bone erosion and high disease activities (P<0.05, respectively). ④ Higher rate of interstitial lung disease (ILD) was found in RA patients with anti-CEP-1 antibody (19.3% vs 4.3%, χ2=5.494, P<0.05). ⑤The patients with anti-CEP-1 anti-body had higher rates of elevated erythrocyte sedimentation rate (ESR) ( χ2=6.543) and decreased serum albumin ( χ2=6.59), compared to patients without anti-CEP-1 antibody (P<0.05, respectively). Conclusion Anti-CEP-1 antibody has high sensitivity and specificity for RA diagnosis. Combination of anti-CEP-1 antibody with other RA antibodies might improve the early diagnosis of RA. Anti-CEP-1 antibody is significantly associated with joint damage, disease activity and pulmonary interstitial fibrosis.
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Objective To evaluate effects of downregulation of glucose?6?phosphate dehydrogenase(G6PD) expression on proliferation and cell cycle distribution of cutaneous squamous cell carcinoma(CSCC)cells. Methods Western blot analysis was performed to measure the protein expression of G6PD in normally cultured human HaCaT keratinocytes, SCL?1 and A431 CSCC cells. When A431 cells grew to 85%-90%confluence, a small interfering RNA (siRNA)targeting G6PD(G6PD?siRNA group)and a negative control siRNA(siRNA control group)were transfected into them separately, and untransfected A431 cells served as the untransfected group. CCK?8 assay was performed to evaluate proliferative activity of the A431 cells on days 0, 1, 2, 3 and 4 after transfection, Western blot analysis to measure G6PD, cyclin D1 and CDK4 protein expressions in A431 cells, and flow cytometry to analyze cell cycle distribution in A431 cells after 48 hours of additional culture. Results The protein expression of G6PD was significantly higher in normally cultured SCL?1 cells(0.308 ± 0.023)and A431 cells(0.643 ± 0.046)than in HaCaT cells(0.100 ± 0.019, both P 0.05). Compared with the untransfected group and siRNA control group, the G6PD?siRNA group showed significantly higher proportions of A431 cells in G0/G1 phase(both P < 0.001), but significantly lower proportions of A431 cells in S phase(both P<0.001). Conclusion G6PD may play important roles in the regulation of proliferation and cell cycle distribution of CSCC cells.
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Objective:To investigate the serum level of C-C chemokine ligand 19 (CCL19)and its clinical significance in rheumatoid arthritis.Methods:The serum CCL19 levels in both rheumatoid ar-thritis (RA)patients and health controls were detected by ELISA.The proportion of peripheral blood B cells and memory B cell subsets were also detected in some patients.Then the clinical and laboratory data of the patients were collected.The CCL19 levels in patients with different clinical features were analyzed. And the correlation between the clinical data,laboratory parameters,B cell subsets proportion and serum CCL19 levels were also analyzed.Independent samples t test,paired t test,Pearson and Spearman corre-lation were used for statistical analysis.Results:The levels of CCL19 was higher in the RA patients than the health controls (P 0.05).The levels of CCL19 were higher in the serum positive (RF and anti-CCP antibody)patients,but there were no differences between low and high disease activity RA,as well as early and non-early RA. There was no correlation between the serum CCL19 levels and the proportion of B cells as well as memory B subsets.All the proportion of peripheral blood CD27 + memory B cell subsets in RA was lower than the healthy controls,including CD27 +IgD +,CD27 +IgD - and CD27 + B cells.Conclusion:The increased serum CCL19 levels in RA patients are associated with the activity of B cells,so CCL19 might predict whether the RA type is a B cell mediated RA,and specify the treatment directions for the rheumatologist.
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Objective To study the disease spectrum,clinical and lab characteristic of cryoglobulinaemia.Methods The clinical and laboratory data of 58 patients with positive cryoglobulin admitted in Peking University People's Hospital from April 2010 to May 2014 were retrospectively analyzed.Results Among 58 patients,34 were diagnosed as autoimmune disease,8 as infectious disease,4 as hematological disease and 12 as primary cryoglobulinemia.Renal involvement was the most frequent clinical presentation among all cryoglobulin positive patients.Patients with autoimmune disease presented all clinical manifestations related to cryoglobulinaemia.Renal involvement (7/8) was prominent in patients with HBV/HCV infection,while other clinical presentations were rare.Among 4 patients with hematological disease,purpura was presented in 3 cases,renal involvement in 2,arthralgia in 2,fatigue,thrombosis or hyperviscosity was presented in 1 case,respectively;however,none of these patients had elevated rheumatoid factor (RF) level.Renal lesions were the most common reason for patients with primary cryoglobulinaemia to consult doctors,and 5 of them had positive antinuclear antibodies (ANA).Conclusions There is a broad spectrum of disease in cryoglobulinaemia.Multi-system involvement was most common in patients with autoimmune disease.For patients with HBV/HCV infection,extra-hepatic presentations were rare except renal involvement.Hyperviscosity syndrome tended to occur in patients with hematological disease.Since patients with primary cryoglobulinaemia had a relatively high rate of positive antinuclear antibodies,we should keep vigilance at the occurrence of autoimmune disease.
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Objective To reduce the misdiagnosis rate of spondyloarthritis (SPA) by reviewing the rare cases misdiagnosed as SpA.Methods Cases misdiaguosed as SpA were collected from our hospital from January 2004 to April 2014.Reported cases among Chinese journals from January 1998 to October,2014 were also collected.According to the Assessment of Spondylo Arthritis international Society (ASAS) axial SpA criteria (2009) and peripheral SpA criteria (2011),the diagnostic accordance rate was studied.Results There were 112 cases within the objective scope,out of which,27 cases (24.1％) were infectious diseases,47 cases (42.0％) were heredity and metabolic diseases,25 cases (22.3％) were hematonosis or tumor,13 cases (11.6％) were osteoarthropathies.Also,only 10 cases (8.9％) out of 112 had the symptoms of inflammatory back pain (IBP),23 cases (20.5％) exhibited fever.Human leukocyte antigen (HLA)-B27 was positive in 20.4％ (21/103) of the cases.Eleven cases out of those 29 cases performed X-ray in the sacroiliac joint and showed blurred articular surface,narrowing of joint space or bone destruction.Four cases were diagnosed based on magnetic resonance imaging (MRI).18/91 (19.8％) cases met the criteria of ASAS axial SpA criteria (2009),2/6(33.3％) cases were in accordance to the ASAS peripheral SpA criteria (2011).Conclusion For patients with atypical back pain,if accompanied with fever,other diseases such as tumor,infection,heredity and metabolic diseases should be considered.The diagnosis should not only based on HLA-B27 for SpA diagnosis.Due to the ambiguity of X-ray in sacroiliac joint,CT or MRI may be recommended to assist the diagnosis.Careful clinical history taken is also with great significance.