Objective:To investigate the clinical features and prognosis of male with anti-melanoma differentiation-associated gene 5 (MDA5) autoantibody.Methods:The clinical data of 246 patients with DM and anti-MDA5 autoantibodies hospitalized by Jiangsu Myositis Cooperation Group from 2017 to 2020 were collected and retrospectively analyzed. Chi-square test was performed to compared between counting data groups; Quantitative data were expressed by M ( Q1, Q3), and rank sum test was used for comparison between groups; Single factor survival analysis was performed by Kaplan-Meier method and Log rank test; Cox regression analysis were used for multivariate survival analysis. Results:①The male group had a higher proportion of rash at the sun exposure area [67.1%(47/70) vs 52.8%(93/176), χ2=4.18, P=0.041] and V-sign [50.0%(35/70) vs 30.7%(54/176), χ2=8.09, P=0.004] than the female group. The male group had higher levels of creatine kinase [112(18, 981)U/L vs 57 (13.6, 1 433)U/L, Z=-3.50, P<0.001] and ferritin [1 500 (166, 32 716)ng/ml vs 569 (18, 14 839)ng/ml, Z=-5.85, P<0.001] than the female group. The proportion of ILD [40.0%(28/70) vs 59.7%(105/176), χ2=7.82, P=0.020] patients and the red blood cell sedimentation rate[31.0(4.0, 101.5)mm/1 h vs 43.4(5.0, 126.5)mm/1 h, Z=-2.22, P=0.026] in the male group was lower than that of the female group, but the proportion of rapidly progressive interstitial lung disease (PR-ILD) [47.1%(33/70) vs 31.3%(55/176), χ2=5.51, P=0.019] was higher than that of the female group. ②In male patients with positive anti-MDA5 antibodies,the death group had a shorter course of disease[1.0(1.0, 3.0) month vs 2.5(0.5,84) month, Z=-3.07, P=0.002], the incidence of arthritis [16.7%(4/24) vs 42.2%(19/45), χ2=4.60, P=0.032] were low than those in survival group,while aspartate aminotransferase (AST)[64(22.1, 565)U/L vs 51(14,601)U/L, Z=-2.42, P=0.016], lactate dehydrogenase (LDH) [485(24,1 464)U/L vs 352(170, 1 213)U/L, Z=-3.38, P=0.001], C-reactive protein (CRP) [11.6(2.9, 61.7) mg/L vs 4.95(0.6, 86.4) mg/L, Z=-1.96, P=0.050], and ferritin levels [2 000(681, 7 676) vs 1 125 (166, 32 716)ng/ml, Z=-3.18, P=0.001] were higher than those in the survival group, and RP-ILD [95.8%(23/24) vs 22.2%(10/45), χ2=33.99, P<0.001] occurred at a significantly higher rate. ③Cox regression analysis indicated that the course of disease LDH level, and RP-ILD were related factors for the prognosis of male anti-MDA5 antibodies [ HR (95% CI)=0.203(0.077, 0.534), P=0.001; HR (95% CI)=1.002(1.001, 1.004), P=0.003; HR (95% CI)=95.674 (10.872, 841.904), P<0.001]. Conclusion:The clinical manifestations of male anti-MDA5 antibody-positive patients are different from those of female. The incidence of ILD is low, but the proportion of PR-ILD is high. The course of disease, serum LDH level, and RP-ILD are prognostic factors of male anti-MDA5 antibody-positive patients.

Objective:To investigate the clinical characteristics and treatment of Beh?et′s disease complicated with cardiac valve involvement.Methods:We searched the wanfang medical database and Medline database to reviewed the domestic and foreign literature reports on cardiac Beh?et′s disease and analyzed their clinical features and therapeutic strategies. Chi-squared test was used for data analysis.Results:It was shown that Beh?et′s disease with cardiac valve involvement mainly affect men. The male to female ratio was 3.86∶1 in China and 2.50∶1 in foreign patients( χ2=1.32, P=0.251). The preoperative diagnosis rate was not high(60.3% in China, 57.1% abroad) ( χ2=0.13, P=0.716). Aortic valve and perivalvular lesions were the most common involved sites, of which aortic regurgitation was the most frequenty occurred, followed by mitral valve lesions. Glucocorticoids was still the main means treatment for medical(93/235 in China, 28/420 abroad), cyclophosphamide was more widely used in China(28/235), azathioprine was more widely used in foreign countries (12/42). Aortic replacement (AVR) was the mainly surgical approach, followed by artificial aortic valve replacement and left ventricular outflow tract plasty (Bentall).The incidence of postoperative perivalvular leakage or valve prolapse was higher with AVR than with Bentall(AVR 76.3%/Bentall 21.8% at home, χ2=32.60, P<0.001, AVR 71.4%/Bentall 0 abroad, χ2=13.84, P<0.001). Conclusions:Cardiac valve involvement is a severe complication of Beh?et′s disease. Heart involvement are more common, and the preoperative diagnosis rate is lower in China. The incidence of perivalve leakage (PVL) or valve prolapse (PD) after operation is higher with AVR than with Bentall surgery.The Bentall operation could improve prognosis and the postoperative complications abroad are lower than domestic.

Objective:To explore the effect of leptin on B cells in patients with systemic lupus erythematosus (SLE).Methods:Peripheral blood mononuclear cells (PBMCs) were isolated from SLE patients, and then CD19 + B cells were purified with magnetic bead sorting method. PBMCs or purified B cells were cultured with recombinant leptin at 0, 100, 250 ng/ml for 3 or 5 days. The frequencies of plasma cells, follicular helper T (Tfh) cells and peripheral helper T (Tph) cells, as well as activation markers (CD80, CD86) and leptin receptor and the proliferation of B cells were determined with flow cytometry. The concentrations of antibodies and cytokines were examined with enzyme-linked immunosorbnent assay (ELISA). Data were analyzed with t test and analysis of variance (ANOVA). Results:Increased levels of leptin were positively correlated with systematic lupus erythematosus disease activity index (SLEDAI) and the frequency of plasma cells in SLE patients. Leptin receptor could be detected on SLE B cells, and recombinant leptin elevated the levels of its receptor on CD19 + B cells [(7.8±1.3)% vs (6.1±0.9)%, t=3.36, P=0.006]. Leptin enhanced the expression of CD80 [(21±4)% vs (19±4)%, t=2.84, P=0.004] and CD86 [(22±4)% vs (19±4)%, t=4.92, P=0.004] on SLE B cells in vitro. It also promoted B cells to differentiate into plasma cells [(7.6±1.5)% vs (5.2±1.3)%, t=6.42, P=0.025]. There was no statistical significant difference of the effect of leptin on B cell proliferation. Leptin also increased the levels of antibodies [IgG: (62±3) ng/ml vs (45±4) ng/ml, t=7.75, P<0.001; IgM: (112±24) ng/ml vs (56±18) ng/ml, t=5.38, P<0.001] and inflammatory cytokines [IL-6: (24±5) pg/ml vs (20±5) pg/ml, t=4.09, P=0.002; TNF-α: (19.1±3.8) pg/ml vs (14.1±2.9) pg/ml, t=3.38, P=0.006; IL-10: (24±5) pg/ml vs (20±5) pg/ml, t=4.09, P=0.002] secreted by B cells. In addition, leptin significantly upregulated the frequencies of Tfh cells[(2.82±0.49)% vs (1.28±0.20)%, t=4.56, P=0.001] and Tph cells [(4.5±0.5)% vs (3.4±0.4)%, t=3.88, P=0.003]. Conclusion:Leptin could directly promote the activation, differentiation and secretory capacity of B cells by binding to its receptor, and also modulate B cell responses indirectly via enhancement of Tfh and Tph cells, which may be involved in the pathogenesis of SLE.

Objective:To improve the diagnosis of anti-synthetase antibody syndrome(ASS) by analyzing the clinical features of 6 patients.Methods:Six cases of ASS with complete data were included in this study as they were diagnosed or other CTD during the period of hospitalization in Wuxi People's Hospital from January 2016 to February 2020. Their clinical and laboratory characteristics, and follow-up information were analyzed. Features and changes in the course of disease were analyzed.Results:Four out of 6 patients were females, with age of disease onset as 34-72 years, and an interval of 4-59 months from the first diagnosis to the diagnosis of ASS. The first diagnosis was Sjogren's syndrome (SS) in 2 cases, rheumatoid arthritis (RA) in 1 case, mixed connective tissue disease (MCTD) in 1 case, systemic sclerosis (SSc) in 1 case, and undifferentiated connective tissue disease (UCTD) in 1 case. At the first diagnosis, 5 cases had dry cough and/or dyspnea, followed by fever (4 cases), arthritis and Raynaud's phenomenon (3 cases). Anti-nuclear antibody(ANA), which was more common in cytoplasmic type, and anti-SSA/52 000 antibody were mostly positive(5 cases). The presence of non-specific interstitial pneumonia (NSIP) pattern (6 cases) in high resolution CT(HRCT) was found at the initial diagnosis. During follow-up, patients developed repeated liver function or muscle enzyme abnor-malities (3 cases), mechanic's hand (MH) (3 cases), and lung interstitial disease progression (4 cases). The myositis antibodies were found to be positive.Conclusion:ASS can occur in the course of or at the same time as other CTDs. ASS should be considered in patients with interstitiallung disease (ILD) (especially NSIP pattern) in HRCT, and/or positive of cytoplasmic type ANA and/or anti-SSA/52 000 antibodies At the same time, if repeated liver function or muscle enzyme abnormalities, new onset of MH, and lung interstitial disease progresses during treatment, consideration may be given to the possibility of complicated ASS. Myositis-specific antibodies are helpful in the diagnosis of ASS.

Objective:To investigate the clinical, treatment responses and outcomes of SLE patients with lupus podocytopathy (LP).Methods:Seven hospitalized cases in Wuxi people's hospital were diagnosed with LP based on the renal biopsy study during January 2011 to May 2019. Their clinical, immunological and pathological features, treatment responses and prognosis were analyzed.Results:Six cases were women. The mean onset age was (33±12), and the mean duration of systemic lupus erythematosus (SLE) was (69±64) months. Among these patients, 3 cases were initially diagnosed of SLE, and 6 with kidney damage caused by SLE. Six cases presented as nephrotic syndrome (NS) in which 2 cases were complicated with acute kidney injury(AKI). The extrarenal manifestations were not parallel to the renal manifestations as the positive rate of autoantibody and the incidence of hypocomplementemia were both low. After treatments with glucocorticoids and immunosuppressants, renal disease of 6 cases was improved. During follow-up, 2 cases developed renal disease recurrence.Conclusion:LP tends to occur in women in reproductive age, and NS or AKI is the main clinical manifestations. Renal manifestations are not parallel to extra-renal manifestations. Glucocorticoids and immunosuppressive therapy is sensitive, and some patients may relapse after treatment.

The current document is based on a consensus reached by a panel of experts from the Chinese Society of Allergy and the Chinese Society of Otorhinolaryngology-Head and Neck Surgery, Rhinology Group. Chronic rhinosinusitis (CRS) affects approximately 8% of Chinese adults. The inflammatory and remodeling mechanisms of CRS in the Chinese population differ from those observed in the populations of European descent. Recently, precision medicine has been used to treat inflammation by targeting key biomarkers that are involved in the process. However, there are no CRS guidelines or a consensus available from China that can be shared with the international academia. The guidelines presented in this paper cover the epidemiology, economic burden, genetics and epigenetics, mechanisms, phenotypes and endotypes, diagnosis and differential diagnosis, management, and the current status of CRS in China. These guidelines—with a focus on China—will improve the abilities of clinical and medical staff during the treatment of CRS. Additionally, they will help international agencies in improving the verification of CRS endotypes, mapping of eosinophilic shifts, the identification of suitable biomarkers for endotyping, and predicting responses to therapies. In conclusion, these guidelines will help select therapies, such as pharmacotherapy, surgical approaches and innovative biotherapeutics, which are tailored to each of the individual CRS endotypes.
Adult ; Asian Continental Ancestry Group ; Biomarkers ; China ; Consensus ; Diagnosis ; Diagnosis, Differential ; Drug Therapy ; Eosinophils ; Epidemiology ; Epigenomics ; Genetics ; Humans ; Hypersensitivity ; Inflammation ; International Agencies ; Medical Staff ; Neck ; Phenotype ; Precision Medicine

Objective To explore the clinical characteristics and prognostic indicators that classify patients with systemic lupus erythematosus (SLE) at risk of in-hospital mortality.Methods Medical records of 1611 SLE patients admitted between 1999-2009 were collected from 26 centers across Jiangsu province,and patients were divided into two groups based on the outcomes.The suspected risk factors of poor outcomes were selected and then analyzed by chi-square test,independent-samples t test,Wilcoxon rank sum test and Logistic regression.Results Among the 1 611 enrolled patients,91 patients were in the death group (5.6％) and 1 520 patients in the control group (94.4％).The duration of disease [28(4,60) m vs 12(2,47) m,Z=-3.290,P＜0.05),the rate of male/female (13.2％ vs 7.1％,x2=4.606,P＜0.05),as well as the occurrence rateof seizure (8.8％ vs 1.7％,x2=17.550,P＜0.05),psychosis (41.8％ vs 23.8％,x2=14.809,P＜0.05),lupus headache (19.8％ vs 6.0％,x22=-25.898,P＜0.05),alopecia (47.3％ vs 30.3％,x2=11.541,P＜0.05),pericarditis (35.2％ vs 22.0％,x2=8.408,P＜0.05),myocarditis (4.4％ vs 1.0％,x2=5.885,P＜0.05),fever (55.0％ vs 28.5％,x2=28.632,P＜0.05),decreased hemoglobin levels (60.9％ vs 44.8％,x2=8.603,P＜0.05),urinary casts (24.2％ vs 12.2％,x2=10.884,P＜0.05),hematuria (51.7％ vs 37.8％,x2=6.988,P＜0.05),decreased estimate glomerular filtration rate (eGFR)levels (27.6％ vs 11.0％,x2=18.12,P＜0.05),and elevated glutamic-oxaloacetic transaminase (AST) levels (30.2％ vs 17.9％,x2=8.176,P＜0.05) were higher in the death group.The frequency of arthritis (34.3％ vs 18.7％,x2=9.459,P＜0.05),proteinuria (31.6％ vs 14.3％,x2=12.169,P＜0.05),elevated erythrocyte sedimentation rate (ESR) levels (80.4％ vs 71.8％,x2=4.192,P＜0.05),decreased complement levels (44.2％ vs 17.6％,x2=24.881,P＜0.05) and anti-dsDNA antibodies positivity rate (39.7％ vs 23.1％,x2=9.963,P＜0.05) were higher in the control group.Logistic regression analysis showed seizure [OR =4.035,95％ CI(1.338,12.164),P＜0.05],lupus headache [OR=3.026,95％CI (1.406,6.511),P＜0.05],decreased hemoglobin (Hb) levels [OR =2.116,95％ CI(1.139,3.934),P＜0.05],decreased eGFR levels [OR =2.159,95％ CI(1.0 11,4.610),P＜0.05] and fever [OR=2.567,95％CI (1.422,4.634),P＜0.05] were positively correlated with in-hospital mortality,with elevated ESR levels [OR=0.418,95％CI (0.218,0.802),P＜0.05] and decreased complement levels [OR=0.328,95％CI (0.120,0.894),P＜0.05] negatively correlated (P＜0.05).Conclusion The results sugest that seizure,lupus headache,decreased Hb levels,decreased eGFR levels and fever are the most important predictors of in-hospital mortality.Clinicians should pay more attention to these symptoms in admission.

Objective To investigate the clinical significance of PET/CT in the diagnosis of giant cell arteritis .Methods The clinical manifestations ,laboratory detection and PET/CT results in 4 patients with giant cell arteritis treated in this hospital were retrospectively analyzed .And the relevant literatures were reviewed .Results The age of 4 cases in this hospital and 29 cases in lit-eratures all were over 50 years old ;clinical manifestations were mainly the non-specific symptoms such as fever ,weight loss and my-algia;the laboratory detection results mainly manifested by the increase of ESR and CRP ;PET-CT indicated that the continuous dif-fuse metabolism of the wall in aorta and its primary branching was elevated ;the maximal standardized uptake value(SUVmax) of 4 cases in this hospital was 2 .5-9 .6 .Conclusion PET/CT has a certain value for diagnosing giant cell arteritis ,especially the pa-tients with early stage or atypical clinical manifestations .PET/CT has certain significance for determining the lesion range and jud-ging the curative effect .

Objective To retrospectively analyze the clinical features of elderly-onset systemic lupus erythematosus (SLE).Methods A total of 916 patients were enrolled in this retrospective study,and their clinical data were collected by the same methods in the past ten years (1999 2009) in fifteen hospitals in Jiangsu Province.Patients were divided into two groups based on the age of onset disease:control group and elderly group.The relationships between clinical features,immunology index and disease activity were analyzed in different age group.Results Among 916 SLE patients,24 patients were selected in the elderly onset SLE group,and 892 patients were considered as the control group.The ratio of male/female,mortality rate,the number of complications were higher in elderly onset SLE group than those in the control group (all P ＜ 0.05),and discoid rash,thrombocytopenia,elevated C-reactive protein (CRP) level,abnormality of serum albumin were found more common in elderly onset SLE group than in control group (all P＜0.05).The incidences of Malar rash and photosensitivity,antinuclear antibodies (ANA) positivity rate,anti-Sm antibodies positivity rate were lower in elderly onset SLE group than in control group (all P＜0.05).The time of final diagnosis,mean time of onset to death,positive family history,oral ulcers,arthritis,serositis,nervous involvement,musculoskeletal disorder,renal involvement,elevated serum creatinine (Scr) level,leucopenia,hemolytic anemia,elevated proteinurine,Erythrocyte Sedimentation Rate (ESR) levels,anti-dsDNA antibodies positivity rate,decreased complement C3 level,SLEDAl score had no significant differences between the two groups.Conclusions There were many differences in the clinical features between the elderly onset SLE patients and the controls,and the mortality rate is higher in the elderly onset SLE patients,which should be pay more attention to in clinical medcine.

ObjectiveTo investigate the association of complement C3 with clinical and serological features of patients with systemic lupus erythematosus.MethodsData was collected by the same methods in the past ten years in fifteen hospitals in Jiangsu Province and then data weres summarized for retrospective analysis.Clinical and laboratory data were selected and then analyzed by Chi-square test,Wilcoxon rank sum test and Logistic regression.ResultsOne thousand four hundred and five patients were investigated.One thousand and forty two had low serum complement C3 level.In this case control study,hospitalization age,disease course,admission times,pleurisy,gastrointestinal involvement,general lymphadenopathy/hepatosplenomegaly,white blood cell count, haemoglobin level,platelet count, serum C-reactive protein level,serum albumin level,serum creatinine level,Urine protein quantification,anti-nuclear antibodies (ANA),anti-dsDNAantibodies, anti-SmantibodiesandSLEDAIscore were possible factors associatedwith complement C3 reduction(P＜0.05).Logistic regression analysis showed that CRP (OR=0.396,0.254-0.617,P=0.000),ANA (OR=2.907,1.267-6.670,P=0.012),urine protein level(OR=1.702,1.043-2.779,P=0.033) and SLEDAI score (OR-0.930, 0.886-0.975,P-0.003) were correlated with complement C3 reduction.Conclusion Complement C3 level is valuable for lupus flare assessment.The complement C3 reduction is a risk factor for renal impairment.