Objective:To investigate the relationship of Castleman disease (CD) and connective tissue disease (CTD).Methods:Clinical records and laboratory data of 11 patientsdiagnosed with CD and CTD were collected and retrospectively analyzed. All patients were diagnosed at Peking Union Medical College Hospital.Results:① The proportion of CD associated with or mimicking CTD was 5.67% (11/194) in all CD patients during the same period. The average age of these cases at the diagnosis was (51±17) years and the ratio of male to female was 6∶5. ② Lymphadenopathy (10/11), fever (8/11), serosal effusion (6/11), arthralgia (5/11), alopecia (2/11), Raynaud phenomenon (1/11) and photosensitivity (1/11) were the common clinical manifest- ations that could mimic CTD. ③ Elevated ESR (11/11), hypoalbuminemia (11/11), elevated CRP (10/11), elevated IgG (7/11), proteinuria (5/11), hematuria (5/11) and positive ANA(5/11) were commonly found in the patients' laboratory tests. ④ CD was inclined to mimic systemic lupus erythematosus(SLE)(5/11), IgG4-related disease(IgG4-RD)(2/11) and adult onset Still's disease(AOSD)(2/11), as well as 2 cases were associated with Sj?gren's Syndrome(SS)(2/11). ⑤All cases were ultimately diagnosed as multicentric CD, the pathologic subtypes were plasma cell variant (10/11) and mixed(1/11) respectively.Conclusion:CD maybe overlapped with or mimic a variety of clinical manifestations, such as fever, serosal effusion, arthralgia and proteinuria which could mimic CTD. Early biopsy is helpful for the diagnosis and to avoid misdiagnosis and mistreatment.

Objective:To explore the difference between the prevalence of metabolic syndrome (MetS) and its influencing factors between rheumatoid arthritis (RA) patients and osteoarthritis (OA) patients.Methods:2 024 patients with RA were selected as the experimental group inthe First Central Hospital of Tianjin from January 1, 2014 to March 12, 2019. A total of 1 271 patients with OA who were treated at the same time served as the control group.Relevant indicators were measured, and correlation analysis was performed using logistic regression.Results:The prevalence of MetS in the past six years did not differ significantly inpatients with RA ( χ2=5.77, P=0.06) and OA ( χ2=1.11, P=0.57). MetS risk factors were more prevalent inpatients with OA, and the prevalence of MetS in patients with OA was also higher than patients with RA ( χ2=19.07, P<0.01). In logistic regression model, the prevalence of MetS was positively correlated with body weight {RA: 1.14[(95% CI(1.10, 1.18), P<0.01]; OA: 1.11[95% CI(1.07, 1.16), P<0.01]}, blood levels of triglyceride {RA:2.64[95% CI(1.58, 4.40), P<0.01] and OA: 2.91[95% CI(1.32, 6.43), P=0.01]}, and glucose {RA:1.67[95% CI(1.42, 1.97), P<0.01] and OA: 1.76[95% CI(1.31, 2.35), P<0.01]}, and negatively correlated with blood level of high-density lipoprotein cholesterol{RA: 0.09[95% CI(0.02, 0.32), P<0.01] and OA: 0.03[95% CI(0.01, 0.25), P<0.01]} in patients with RA and OA. MetS risk factors such as serum creatinine (Scr) only presented in RA patients 1.04 [ OR: 95% CI(1.01, 1.06), P<0.01]. Conclusion:The prevalence of MetS did not decrease in patients with chronic inflammatory arthritis in the past six years, and OA patients havea greater risk of MetS.

Objective:To investigate the proportion of patients with clinical remission and sustained remission in Chinses patients with rheumatoid arthritis (RA).Methods:We retrospectively reviewed the medical records of RA patients in our center from January 1, 2011 to December 31, 2016. Disease activity scores and remission status at each visit were assessed by the disease activity score in 28 joints (DAS28), the simplified disease activity index (SDAI), the clinical disease activity index (CDAI), and the American Ccollege of Rheumatology/European league Against Rheumatism (ACR/EULAR) Boolean criteria. Patients were considered in sustained remission if they maintained remission during consecutive visits for 6 months. Kaplan-Meier method was applied to plot cumulative possibility of achieving remission and calculate the median time to first clinical remission. Cox multivariate regression analysis was used to analyze the relative factors of sustained remission.Results:A total of 648 patients were included in the present study. During the median follow-up of 24 months, around 70% of patients reached clinical remission at least once (DAS28: 78.7%, SDAI: 70.8%, CDAI: 68.4%, Boolean criteria: 68.7%). Specifically, the cumulative probability of achieving remission at 3-, 6- and 12- month was 10.6%-24.4%, 25.3%-43.5% and 51.8%-65.2% respectively, depending on instruments applied. The median time to first remission was 7.2 (DAS28), 10.1(SDAI), 11.7(CDAI), 11.4(Boolean criteria) months. Regarding the sustained remission, nearly half of patients experienced SR defined by DAS28(52.2%), CDAI(46.6%), SDAI(45.1%), and Boolean definitions (43.7%) respectively. Among those patients in sustained remission, the median time of persistence remission during study period was 16.0 months (DAS28-ESR), 15.4 months (CDAI), 14.9 months (SDAI) and 15.0 months (Boolean criteria). Among patients achieving sustained remission, the percentage was 18.7% and 81.3% for DMARD monotherapy and combination therapy, respectively. Additionally, 22.3% of patients received low-dose glucocorticoids treatment concomitantly and over half of them successfully tapered or discontinued the glucocorticoids during the period of sustained remission.Conclusion:In daily practice, clinical remission is a realistic target in the setting of treat-to-target strategy, and over half of patients achieveclinical remission in the first year of follow-up. Among those patients who achieve clinical remission, around half of them reach sustained remission over the subsequent follow-up period. The median time of persistent remission in patients achieving remission is about 15 months.

Objective:To study the distribution of FC γ receptor ⅢB gene polymorphism in the Han population in the Yellow River Delta, and to explore the correlation between the gene polymorphism of multiple loci and the susceptibility and clinical phenotypes of systemic lupus erythematosus (SLE).Methods:From January 2017 to December 2017, 144 SLE patients in the Affiliated Hospital of Binzhou Medical College of Shandong Province were selected as the experimental group and 150 healthy people as the control group. Whole blood samples, clinical data and laboratory examination test data were collected. Genomic DNA was extracted by single base extension polymerase chain reaction (PCR) technology and single nucleoside was extracted by mass spectrometry. The relationship between the polymorphism of each point of FC γ receptor ⅢB gene and the susceptibility and clinical phenotypes of SLE was analyzed by χ2 test with SPSS 25.0 software. Results:① The frequencies of CT and TT genotype at rs115878669 were 50.7%, 64.0%, 23.6% and 10.0% respectively in the test group and the control group, the difference was statistically significant ( χ2=5.3, P=0.021; χ2=9.8, P=0.002); The frequencies of TT genotype at rs147574249 were 17.4% and 8.0% respectively in the test group and the control group, the difference was statistically significant ( χ2=5.9, P=0.016); The frequencies of GG and GA genotype at rs199705513 were 20.8%, 10.0%, 59.0%, 70.0%, the difference was statistically significant ( χ2=6.7, P=0.010; χ2=3.9, P=0.049); The frequency of CA genotype at rs77717968 was 30.6% and 46.0% in the test group and the control group, respectively, the difference was statistically significant ( χ2=7.4, P=0.007). ② In the experimental group, the frequencies of heterozygous CT genotypes at rs115878669 were 37.2% and 56.4% in the blood system affected group and the unaffected group, respectively, the difference was statistically significant ( χ2=4.5, P=0.035). In the thrombocytopenia group and the normal platelet normal count group, the frequencies of AG genotypes at rs114531649 were 79.2% and 50.0%, respectively, the difference was statistically significant ( χ2=6.8, P=0.009) and GG genotypes were 4.2% and 25.8%. The difference was statistically significant ( χ2=4.3, P=0.039). The frequency of CC genotype at rs147574249 was 4.2%, 25.8% respectively. The difference was statistically significant ( χ2=5.4, P=0.020). The frequency of CT genotype was 79.2%, 56.7% respectively. The difference was statistically significant ( χ2=4.2, P=0.040). The frequency of CT geno-type at rs115878669 was 70.8%, 46.7%, respectively. The difference was statistically significant ( χ2=4.7, P=0.031). The frequency of AA genotype at rs199705513 was 4.2%, and 23.3%, the difference was statistically significant ( χ2=4.6, P=0.033), the frequency of AA genotype at rs77717968 was 4.2%, 26.7%, and the difference was statistically significant ( χ2= 4.5, P=0.033), the frequency of TT genotype was 25.0%, 9.2%, the difference was statistically significant ( χ2=4.8, P=0.028). There was no statistical significance difference in other comparisons ( P>0.05). The GG genotype frequencies of rs114531649 and the CC genotype frequencies of rs147574249 were 31.2% and 15.7%, respectively. The differences were statistically significant ( χ2=4.9, P=0.027). The AA geno- type frequencies of rs199705513 were 29.5% and 13.2%, the difference was statistically significant ( χ2=5.8, P=0.016), the CC genotype frequency of homozygote at rs61803007 was 32.8%, 16.9%, the difference was statistically significant ( χ2=4.9, P=0.026), the TC genotype frequency of heterozygote was 67.2%, 83.1%, the difference was statistically significant ( χ2=4.9, P=0.026), the AA genotype frequency of homozygote at rs77717968 was 31.2%, 16.9%, respectively, the difference was statistically significant ( χ2=4.1, P=0.044), and there was no significant difference in other comparisons ( P>0.05). The frequency of TC genotype in rs146653557 was 39.4% in serositis group and 75.0% in non serositis group, the difference was statistically significant ( χ2=4.3, P=0.037), the other differences were not statistically significant ( P>0.05). The frequency of CG genotype in rs428194 group and rs61803004 group was 53.8% and 22.9% respectively, the difference was statistically significant ( χ2=12.7, P=0.000 4). The frequency of GG genotype in rs428194 group was 46.2% and 77.1%, 46.2% and 78.1% in rs61803004 group respectively, the difference was statistically significant ( χ2=12.7, P=0.000 4; χ2=13.7, P=0.000 2). The frequencies of GT genotype were 46.2% and 21.0%. The differences were statistically significant ( χ2=9.0, P=0.002 7). The frequency of TT genotypes was 7.7% and 1.0%, the difference was statistically significant ( χ2=4.8, P=0.029). The frequencies of CT genotypes at rs61803008 were 46.2% and 23.8% respectively, the difference was statistically significant ( χ2=6.8, P=0.009 2). The frequencies of TT genotypes were 46.2% and 74.3%. The difference was statistically significant ( χ2=10.1, P=0.001 5). Conclusion:There is a significant correlation between Fc gamma receptor ⅢB gene related loci and SLE susceptibility and clinical phenotypes.

Objective:To discuss the clinical features and treatment of 19 patients with granulomatosis with polyangiitis (GPA) complicated with hypertrophic cranial pachymeningitis (HCP).Methods:The clinical features of 19 patients diagnosed with GPA complicated with HCP in Peking Union Medical College Hospital were retrospectively analyzed.Results:Among the 315 patients with GPA, 19 (12 males, 7 females, with) were diagnosed with HCP at Peking Union Medical College Hospital. The median age was 57 (19-64) years. In the neurological manifestations per se, all patients had headache, 16 patients had cerebral involvement, which included 8 cases at the frontal area, 8 cases at the temporal area, 8 cases at the skull base area (4 cases with parasellar involvements including 3 cases with cavernous sinus involvement and 2 cases with orbital involvement), 6 cases of tentorium involvement, 2 cases of cerebral palsy, 1 case of calvarium, 1 case of occipital, and 1 case with combined spinal pachymeningitis, respectively. In systemic manifestations, 10 patients had fever, 8 patients had weight lose, 4 patients had lung involvement, 3 patients had kidney involvement, 16 patients had nasosinusitis, 10 patients had tympanitis, and 16 patients had localized GPA. The laboratory tests showed that 15 patients had positive anti-neutrophil cytoplasmic antibodies (ANCA), including 8 cases with positive proteinase 3 (PR3)-ANCA and 6 cases with positive myeloperoxidase (MPO)-ANCA. Sixteen patients had lumbar puncture examination, 9 cases had elevated cerebrospinal pressure, 10 cases had elevated level of protein in cerebrospinal fluid. Nineteen patients were treated with glucocorticoids (12 patients accepted pulse therapy) and immunosuppressive agents. Twelve patients were treated with intrathecal injections of dexamethasone combined with or without Methotrexate (MTX). All 19 patients were improved.Conclusion:HCP, as a rare but serious manifestation of GPA, is not rare in active cases and should be intensively treated.

Objective:To investigate the long-term outcome and prognostic indicators of diffuse pro-liferative lupus nephritis (DPLN).Methods:The primary endpoint of long-term follow-up and factors pos- sibly influencing the outcome were analyzed retrospectively in DPLN patients admitted to the First Affiliated Hospital of Wenzhou Medical University between Jan 1, 2000 and Dec 31, 2014. Patients were classified into three groups according to the evaluated glomerular filtration rate(eGFR) on the first day of admission: eGFR≥60 ml·min -1·1.73 m -2 (regular illness group); 15 ml·min -1·1.73 m -2≤eGFR<60 ml·min -1·1.73 m -2 (serious illness group); eGFR<15 ml·min -1·1.73 m -2 or dialysis (critical illness group). Clinical, histological, and outcome differences among the three groups were evaluated and compared using one-way analysis of variance (ANOVA) , χ2 test, Kaplan-Meier survival curve and Cox reggression analysis. Results:167 DPLN patients were studied [155 women; mean age (30±10) years; mean follow-up of (61±45) months]. Renal and patient survival of all patients was 86% at 5 years and 79% at 10 years. Kaplan-Meier analysis showed the renal and patient survival rate at 10 years in the regular illness group, serious illness group and critical illness group was 91%, 70% and 8%, respectively ( χ2=121.93, P<0.01, overall); regular illness group vs serious illness group ( χ2=4.05, P<0.05); regular illness group vs critical illness group ( χ2=97.05, P<0.01); serious illness group vs critical illness group ( χ2=52.28, P<0.01). Multivariable Cox regression analysis found that haematoglobin (Hb)<80 g/L [ HR=2.7, 95% CI(1.2, 6.3), P=0.019], eGFR<60 ml·min -1·1.73 m -2 [ HR=4.1, 95% CI(2.0, 8.2), P<0.01] and large crescents ≥30%[ HR=1.8, 95% CI (1.1, 2.9), P=0.021], were risk factors for the long-term outcome. Conclusion:DPLN patients with normal or slightly decreased renal function have a better long-term prognosis. Moderate to severe impairment of renal function, anemia and large crescents are associated with poor outcome.

Objective:To summarize the common sites of osteophytes in patients with hand osteoarthritis (OA), and analyze the correlation between the severity of osteophytes and clinical factors.Methods:One hundred and four patients with hand OA were selected and divided into three groups according to the disease duration: <1 year, 1~5 years, >5 years. The first carpometacarpal joint(CMC1), metacarpophalangeal joint(MCP), proximal interphalangeal joint (PIP) and distal interphalangeal joint (DIP) were detected by high fre- quency ultrasound. The location of osteophytes and osteophyte semi-quantitative grading scores (OSGS) were recorded. The patients age, disease duration, erythrocyte sedimentation rate (ESR), C-reactionprotein (CRP), the visual analogy score (VAS) and Australian/Canadian osteoarthrits hand index (AUSCAN) were collected. The indicators of different groups were compared, the incidence and location of osteophytes was calculated, and the correlation between osteophytes and clinical factors was analyzed. Data were analyzed by Wilcoxon rank sum test, Kruskal-Wallis test, χ2 test, Spearman correlation analysis. Results:① Osteophytes accounted for 33.56%(1 047/3 120) of the 3 120 joints in hands. There were statistically significant differences in OSGS and AUSCAN in different groups ( H=13.485, P<0.01; H=51.491, P<0.01), while no statistically difference in VAS, ESR and CRP ( H=5.808, P=0.055; H=2.878, P=0.237; H=2.319, P=0.314). ② In different joint areas of hands, PIP accounted for the largest proportion (46.54%, 484/1 040), followed by DIP (46.51%, 387/832), CMC1 (30.77%, 64/208), and MCP accounted for the smallest proportion(10.77%, 112/1 040). There were statistically significant differences in the incidence of osteophytes in different joint areas( χ2=384.194, P<0.01).③ In the interphalangeal joint areas of hands, the largest osteophytes composition ratio was MCP3 (46.43%, 52/112), PIP3 (30.58%, 148/484) and DIP2 (31.01%, 120/387), respectively. ④ OSGS were positively associated with age, disease course, VAS and AUSCAN ( r=0.370, P<0.01; r=0.382, P<0.01; r=0.215, P=0.029; r=0.390; P<0.01), there was no correlation between OSGS and ESR or CRP ( r=0.173, P=0.079; r=0.162, P=0.101). Conclusion:PIP are the most common sites of osteo-phytes in hand OA, followed by DIP. High frequency ultrasound can help the diagnosis and evaluate the severity of hand OA.

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.

Objective:To investigate the clinical significance of serum CXCL13 in primary Sj?gren's syndrome (pSS).Methods:Thirty patients with pSS were enrolled as pSS group and 30 healthy individuals were selected as control group. Serum CXC-chemokine ligand 13 (CXCL13) level were detected by enzyme- linked immuno sorbent assay (ELISA), which was analyzed with the related immune parameters (eg. B cell pro-portion, immunoglobulin (Ig)G, IgA, IgM, complement (C)3, C4 and autoantibodies), the index of gland structure and function, and the related disease index of pSS[EULAR Sj?gren's syndrome disease activity index (ESSDAI), EULAR Sjogren's syndrome patient reported inde (ESSPRI)] by Pearson or Spearman analysis method.Results:The level of serum CXCL13 in pSS patients [(139±94) pg/ml]was significantly higher than that in healthy individuals [(36±20) pg/ml] ( t=5.838, P<0.01). The level of serum CXCL13 was positively cor-related with the proportion of B cells ( r=0.364, P=0.048), the level of serum IgG ( r=0.369, P=0.045), focus score ( r=0.592, P=0.001), ESSDAI ( r=0.415, P=0.023) and ESSPRI ( r=0.431, P=0.017). Meanwhile, it was negatively correlated with unstimulated salivary flow rate ( r=-0.381, P=0.038). There were 24 pSS patients with systemic involvement. The serum CXCL13 level of pSS patients with systemic involvement was signifi-cantly higher than that of patients without systemic involvement, and the difference was statistically significant ( Z=-2.256, P=0.024), but there was no significant correlation with the number of systemic involvement ( r=0.344, P=0.062). Conclusion:The level of serum CXCL13 is a useful index in estimating disease activity and gland destruction.

Objective:To investigate the effects of oxidized low density lipoprotein (Ox-LDL) on cell proliferation and mRNA expression of inflammatory factors in fibroblast-like synoviocytes (FLS) from patients with rheumatoid arthritis (RA).Methods:Tissue culture was used to isolate and 4-6 generation cultured RA-FLS cells were used for subsequent experiments. RA-FLS were stimulated for 24 hours with different con-centr-ations of human Ox-LDL, then the MTS cell proliferation and toxicity test kit were used to detect the prolifer-ation of RA-FLS. Real time-polymerase chain reaction (RT-PCR) was used to test the expression of inflamm- atory factors like interleukin (IL)-6, transforming growth factor (TGF)-β, IL-8, tumor necrosis factor (TNF)-α and receptors like CD36 and scavenger receptor binds phosphatidylsed neoxidized lipoprotein (SR-PSOX) inRA-FLS. T test and F test were used in this study. Results:Ox-LDL (10, 25, 50 μg/ml) could obviously promote the proliferation of RA-FLS, and theabsorbance values (490 nm) were (1.04±0.15), (1.05±0.14), and (1.00±0.10), respectively, all higher than the control group (0.81±0.04) and the difference was statistically significant ( F=4.737, P<0.01). In addition, 50 μg/ml and 100 μg/ml Ox-LDL also promoted the expression of IL-6 mRNA ( F=14.709, P<0.01) and inhi-bited the expression of TGF-β mRNA ( F=299.074, P<0.01), but there was no obvious effect on the expression of IL-8 and TNF-α. Ox-LDL stimulation could obviously promote the expression of SR-PSOX receptor on RA-FLS ( F=68.636, P<0.01) and inhibit the expression of CD36( F=18.085, P<0.01). After the transfection of siRNA, SR-PSOX mRNA level was significantly inhibited and the mRNA expression of IL-6 was significantly decreased after Ox-LDL stimulation of RA-FLS ( t=3.875, P<0.01), while TGF-β mRNA expres-sion was not significantly changed( t=-0.193, P>0.05). Conclusion:Ox-LDL may play a role in promoting the activation of RA-FLS proliferation and the expression of IL-6 mRNA by increasing the SR-PSOX receptor of RA-FLS, suggesting that Ox-LDL is involved in the synovial inflammation of RA.