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A middle-aged male patient initially appeared scattered erythema with pruritus all over the body without obvious cause. According to the skin manifestations of the patient, combined with pathological diagnosis, direct immunofluorescence examination, and different serum autoantibody spectrum, the diagnosis and differential diagnosis of the patient was made by clinicians. The diagnosis of dermatitis herpetiformis was confirmed by the use of autoantibodies in the absence of any apparent history of pasta discomfort. With targeted treatment, the patient′s symptoms and laboratory indicators improved significantly.
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As the research on autoimmune diseases has been more developed and supplemented, the related markers started to play an important role in clinical prediction, disease diagnosis, concomitant diagnosis, treatment evaluation, and prognosis determination. Biomarkers such as neurological autoimmune disease indicators, special proteins, lymphocyte subpopulation, reproduction related autoantibodies are in urgent need of translation from laboratory studies to clinical precise diagnosis and treatment.
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The Idiopathic inflammatory myopathy (IIM), which collectively referred to as myositis, has been considered as a series of heterogeneous diseases characterized by the proximal symmetrical muscle weakness and involvement of multiple organs (such as skin, joints, lungs, gastrointestinal tract, and heart). IIM mainly includes the polymyositis, dermatomyositis, inclusion body myositis and other clinical subtypes. Although the pathogenesis of IIM is not clear, but myositis autoantibodies have been considered to play a certain role in the development and diagnosis of the disease. Myositis autoantibodies can be divided into myositis-specific autoantibodies or myositis-associated autoantibodies. This review provides a comprehensive summary of the progress in the related field of myositis autoantibodies in recent years, hoping to further demonstrate the clinical value of myositis autoantibodies and promote the clinical application of myositis autoantibodies.
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As specific serological markers for autoimmune diseases, autoantibodies are significantly related to diseases or their clinical phenotypes with exclusiveness. Therefore, autoantibodies can not only favor clinical comprehension of different pathophysiological processes of autoimmune diseases, but also contribute to the early screening or diagnosis, severity evaluation, prognosis prediction and the decision-making process for treatment. An increasing number of international clinical practice guidelines have included autoantibodies for disease classification indicators, which further promoted the laboratory utility of them. However, there still exists many problems in the quality management of the determination and clinical application of autoantibodies in China. Thus, attentions should be paid to the standardized detection of autoantibodies and the promotion of new clinical applications.
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Systemic lupus erythematosus (SLE), commonly seen in clinical cases, is a systemic inflammatory disease of connective tissue, which involves multiple systems and organs. It is mainly characterized by a wide range of clinical complications, such as lupus nephritis, neuropsychiatric lupus, and SLE-related cardiovascular events, however, the pathogenesis of SLE has not yet been elucidated. Early diagnosis is of great significance in estimating the severity of SLE, disease activity, predicting relevant progression, supervising therapeutic effects, as well as, improving prognosis. This review will mainly focus on the research advances in clinical application of auto-antibodies and biomarkers previously and recently discovered in patients who has been suffered from SLE.
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Objective:To establish autoverification rules for coagulation tests in multicenter cooperative units, in order to reduce workload for manual review of suspected results and shorten turnaround time (TAT) of test reports, while ensure the accuracy of results.Methods:A total of 14 394 blood samples were collected from fourteen hospitals during December 2019 to March 2020. These samples included: Rules Establishment Group 11 230 cases, including 1 182 cases for Delta check rules; Rules Validation Group 3 164 cases, including 487cases for Delta check; Clinical Application Trial Group 77 269 cases. Samples were analyzed for coagulation tests using Sysmex CS series automatic coagulation analyzers, and the clinical information, instrument parameters, test results, clinical diagnosis, medication history of anticoagulant and other relative results such as HCT, TG, TBIL, DBIL were summarized; on the basis of historical data, the 2.5 and 97.5 percentile of all data arranged from low to high were initially accumulated; on the basis of clinical suggestions, critical values and specific drug use as well as relative guidelines, autoverification rules and limits were established.The rules were then input into middleware, in which Stage I/Stage II validation was done. Positive coincidence, negative coincidence, false negative, false positive, autoverification pass rate, passing accuracy (coincidence of autoverification and manual verification) were calculated. Autoverification rules underwent trial application in coagulation results reports.Results:(1) The autoverification algorisms involve 33 rules regarding PT/INR, APTT, FBG, D-dimer, FDP,Delta check, reaction curve and sample abnormalities; (2)Autoverification Establishment Group showed autoverification pass rate was 68.42% (7 684/11 230), the false negative rate was 0%(0/11230), coincidence of autoverification and manual verification was 98.51%(11 063/11 230), in which positive coincidence and negative coincidence were respectively 30.09% (3 379/11 230) and 68.42%(7 684/11 230); Autoverification Validation Group showed autoverification pass rate was 60.37%(1 910/3 164), the false negative rate was 0%(0/11 230), coincidence of autoverification and manual verification was 97.79%(3 094/3 164), in which positive coincidence and negative coincidence were respectively 37.42%(1 184/3 164) and 60.37%(1 910/3 164); (3) Trialed implementation of these autoverification rules on 77 269 coagulation samples showed that the average TAT shortened by 8.5 min-83.1 min.Conclusions:This study established 33 autoverification rules in coagulation tests. Validation showedthese rules could ensure test quality while shortening TAT and lighten manual workload.
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Objective@#To analyze the changes of peripheral blood leukocyte in patients with Behçet uveitis (BU) at different stages.@*Methods@#Case control study was performed.Twenty active stage BU patients and 21 quiet stage BU patients were enrolled from July to November 2015 in Peking Union Medical College Hospital.Ten active stage BU patients treated with glucocorticoid and/or immunosuppressive agents were served as improvement stage BU patients.Meanwhile, 82 healthy controls were collected from the physical examination center.Peripheral blood was obtained and then analyzed by using Hematoflow method.The percentages of leukocytes in peripheral blood of different stage BU patients were compared.This study was approved by the Ethics Committee of Peking Union Medical College Hospital (ZS-1341) and all participants signed informed consent.@*Results@#The percentage of mature neutrophils, eosinophils, basophils, B lymphocytes, non-cytotoxic T and NK lymphocytes, granular T and NK lymphocytes, T blasts, B blasts and immature granulocytes were all significantly different among active stage BU group, quiet stage BU group and healthy control group (F=42.324, 10.220, 8.660, 11.254, 29.795, 31.305, 23.742, 27.738 and 34.638, all at P<0.001). The percentage of mature neutrophils in active stage BU group and quiet stage BU group were (73.10±10.21)% and (62.40±12.09)%, which were significantly higher than (54.95±6.07)% in healthy control group.The percentage of mature neutrophils in active stage BU group was significantly higher than that in quiet stage BU group (P<0.05). The percentages of non-cytotoxic T, NK lymphocytes, granular T and NK lymphocytes in active stage BU group and quiet stage BU group were significantly lower than that in healthy control group, the percentage of non-cytotoxic T, NK lymphocytes and granular T, NK lymphocytes in active stage BU group were significantly lower than those in quiet stage BU group (all at P<0.05). The percentage of immature granulocytes after treatment was significantly higher than that before treatment in improvement stage BU group (t=-2.469, P=0.036).@*Conclusions@#Increase of peripheral blood mature neutrophil was observed in BU patients, which may help to monitor the inflammatory activity of BU.
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Rheumatoid arthritis (RA) is a systemic chronic inflammatory disease of unclear pathogenesis that is manifested by a progressive and destructive poly-arthritis. The early diagnosis of RA is critical to determine the severity of the disease, which may have an impact on improving the prognosis and life quality of patients with RA. A variety of autoantibodies and protein biomarkers have been reported in the serum of RA patients. Herein will be reviewed updates in the field of serum autoantibodies and biomarkers of RA, as well as their application in diagnosis, prognosis, and potential future directions for study.
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Rheumatoid arthritis (RA) is a systemic chronic inflammatory disease of unclear pathogenesis that is manifested by a progressive and destructive poly-arthritis. The early diagnosis of RA is critical to determine the severity of the disease, which may have an impact on improving the prognosis and life quality of patients with RA. A variety of autoantibodies and protein biomarkers have been reported in the serum of RA patients. Herein will be reviewed updates in the field of serum autoantibodies and biomarkers of RA, as well as their application in diagnosis, prognosis, and potential future directions for study.
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Objective To explore the influence of prozone effect on anti-nuclear antibodies (ANA) testing by indirect immunofluorescence assay (IIFA).Methods The samples with high titer of ANA (≥1∶1 000) were selected from 880fresh serum samples, and were subsequently diluted in 1∶100, 1∶1 000and 1∶10 000ratio.Prozone effect was defined as fluorescence intensity from 1∶1 000dilution was stronger than that from1∶100dilution.The samples with prozone effect were determined manually or by Sprinter XL and EUROPattern.The samples with prozone effect were further characterized by combinations of fluorescence patterns, fluorescence intensities and autoantibody specificities.Results A total of 880samples were tested.Importantly, 34samples displayed prozone effect (3.86％in total and 29.57％in samples with ANA≥1∶1 000).Interestingly, prozone effect was identified by manual detection as well as by Sprinter XL with similar fluorescence patterns and fluorescence intensities.Notably, EUROPattern can only select the central area for identification.Among all samples with prozone effect, 74.42％samples exhibited fluorescence intensities of≥1∶10 000.Speckled pattern was the most prevalent fluorescence patterns in samples with prozone effect (46.51％).In addition, anti-RNP antibodies (62.79％) were the most popular autoantibodies in samples with prozone effect, followed by anti-dsDNA antibodies (51.16％) and anti-SSA antibodies (51.16％).Conclusion Prozone effect was present in ANA testing, especially in samples with high titers, resulting in underestimating the titers.The study highlighted that special attention should be paid to the prozone effect in clinical practice.
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Objective To investigate the expression of programmed death receptor-1 (PD-1) in CD8+ T cells and FoxP3+CD4+ cells in patients with primary biliary cholangitis (PBC).Methods The peripheral blood and clinical data of 69 PBC patients in Peking Union Medical College Hospital and 58 health controls (HC) were collected.They were divided into initial treatment group and follow-up group according to whether they were treated or not.Patients in the treatment group were further divided into the refractory group and stable group according to treatment response.Flow cytometry was used to detect the expression of PD-1 in CDS+T cells and FoxP3+CD4+ cells.T-test and Person correlation analysis were used for data analysis.Results The PD-1 expression in peripheral blood mononuclear cells (PBMCs) of 69 PBC patients (12±9)％ was lower than that of HC (20±12)％ (t=-3.687,P＜0.01).The percentage of PD-1+ in FoxP3+ CD4+T cells was significantly increased in PBC (5.6±3.7)％ than HC (7.4±2.4)％ (t=2.048,P＜0.01).The proportion of CD8+T cells,PD-1 expression in CD8+T cells and the proportion of FoxP3+CD4+ cells weren't correlated with clinical parameters (P＞0.05).There was a negative correlation between the expression of PD-1 cells in FoxP3+CD4+ cells and GLOBE score (r=-0.307,P＜0.05).Conclusion The expression of PD-1 in peripheral CD8+T lymphocytes of PBC patients is lower than that of HC,and decreases more significantly in the refractory group.The expression of PD-1 on FoxP3+CD4+T cells is higher than that in HC,and is negatively correlated with the prognostic GLOBE score.It suggests that PD-1/PD-L1 pathway participates in the immune mechanism of PBC.
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Objective To investigate the health related quality of life score [primary biliary cholangitis (PBC)-40] in patients with PBC,and the relationship between PBC-40 and clinical presentations.Methods The PBC-40 score and clinical presentations in PBC patients (n=65) were adapted in this study.Patients were divided into the untreated group and the treated group,and the treated group was further divided into ursodesoxycholic acid (UDCA) response group and UDCA non-response group.PBC-40 scores of different groups were analyzed by t-test and the relationship between PBC-40 and clinical presentations were analyzed with Pearson's test.Results Dimensions of PBC-40 scores of this group of patients were as follows:symptoms were (15.8±4.1) points,itch was (4.9±2.8) points,atigue was (23.8±8.9) points,cognitive dysfunction score was (11.4±4.7) points,social activity score was (17.0±7.5) points,and the emotion score was (6.5±3.1) points.The untreated group had higher emotion scores than the treated group (t=2.024,P=0.045).Compared with the UDCA response group,UDCA non-response group had higher scores in cognitive,social and emotion dimension (t =2.126,2.309,2.062,respectively,P=0.039,0.025,0.045,respectively).Itch score was significantly correlated with total bilirubin (TBil),direct bilirubin (DBil),alkaline phosphatase (ALP) and total bile acid (TBA) (r=0.349,0.345,0.324,0.427,respectively,P＜0.01),while the social scores were correlated with TBil,DBil,aspartate aminotransferase (AST) and TBA (r=0.361,0.383,0.316,0.331,P＜0.01) and emotion scores were associated with ALT,TBil,GGT,ALP,AST and TBA (r=0.332,0.430,0.265,0.326,0.297,0.353,P＜0.05).ConclusionPBC-40 can be used as a health-related quality of life assessment for PBC patients inChinese population.Itch,social and emotion dimensions are correlated with clinical activity indicators.Hyperbilirubin,ALP and TBA can predict low health quality of life in PBC patients.Conclusion PBC-40 can be used as a health-related quality of life assessment for PBC patients in Chinese population.Itch,social and emotion dimensions are correlated with clinical activity indicators.Hyperbilirubin,ALP and TBA can predict low health related quality of life in PBC patients.
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Autoimmune blistering skin diseases are a group of organ-specific autoimmune disorders that are characterized by autoantibodies against desmosome and hemidesmosome which are structural proteins of the epidermis or the dermal-epidermal junction and clinically by blisters and erosions on skin and/or mucous membranes.According to the skin level at which the blister occurs and the structural proteins that the autoantibodies target,autoimmune blistering diseases can be categorized into intraepithelial blister group and subepidermal blister group.The treatment options and prognosis are different among the various diseases.Since clinical criteria and histopathological characteristics are not sufficient for an accurate diagnosis of autoimmune blistering skin diseases,direct immunofluorescence microscopy,indirect immunofluorescence microscopy,ELISA,immunoblotting and immunoprecipitation are needed for exact diagnosis.The detection of serum autoantibodies have been shown to correlate with disease activity and thus may be helpful in deciding treatment options for the patients.
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According to the consensus criteria of antiphospholipid syndrome (APS),the diagnosis of APS requires the persistent presence of antiphospholipid antibodies (aPLs),indicating the critical role of aPLs in the diagnosis of APS.During the last decade,great efforts have been made to improve the laboratory detection and standardization of aPLs testing.Unfortunately,the heterogeneous nature of aPLs,lacking of standardization in aPLs test,and significant inter-laboratory variation have hampered the clinical application of aPLs test.In this commentary,the clinical application and standardization of aPLs test are focused on,and how to establish the standardization system in aPLs test in order to improve the performance of aPLs test in clinical practice are discussed.
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Objectives To explore the clinical significance of autoantibodiesin individuals who accept a routine physical examination.Methods From April to June 2012, the serum of 932 individualsincluding 649 males and 283 females, from department of routine physical examination center of Peking Union Medical College Hospitalwerecollected , it uesd IIF for ANA, line immunoassay ( LIA) for specific ANAs antibodies and ELISA for the other antibodies , includinganti-CCP antibodies , AMA-M2, ACL antibodies and anti-anti-β2GPⅠantibodies.Chi-square test was used for data measurement of positive rate of autoantibodies in men and women;Fisher′s exact test was used when the data not meet the conditions of Chi-square test.Individualswith high-risk of autoimmune disease according to the results ofautoantibodies ( Titersof autoantibodies≥2-fold cut-off and accompanied with other autoimmune diseases related laboratory abnormalities) were recalled to visit doctor.Results Of the 932 cases, the overall positive rate of ANA was 11.27%.The positive rate of ANA was19.79%inwomen, which was significantly higher than thatin men (7.09%)(χ2 =32.6, P<0.01); the overall positive rate of ANAswas 8.69%, and the positive rate of ANA was13.43%inwomen, whichwas significantly higher than that in men ( 6.63%) (χ2 =11.49, P <0.01);the overall positive rates of AMA-M2, anti-CCP antibodies , anti ACL antibodies and anti β2GPⅠantibodies were 3.22%, 0.54%, 2.90%and 0.21% respectively , which were 2.83%, 0.71%, 3.18%and 0.71 % in women , and 3.39%, 0.46%, 2.77% and 0.00% in men respectively , there was no statistically significant of positive rate between female and male 58 patients accounting for 6.22% in high-risk of autoimmune disease were recalled , of which 15 cases, accounting for 1.61% were diagnosed or highly suspected of autoimmune diseases (AID) of the 15 patients, 11 patients accounting for 1.18% were diagnosed AID, including 6 CTD, 3 pSS, 1 RA and 1 pSS/PBC;4 patients were highly suspected as AID , including 3 suspected CTD and 1 suspected pSS.The titers concentration of the positive antibodies in patients with confirmed or suspected AID ≥ 3 times cut-off.Conclusions The positive rate of autoantibodies in individuals of physical examination is high , but there is clinical significance when the titers concentration of positive autoantibodies ≥ 3 times of the cut-off.Positive-autoantibodies patients with high-risk of autoimmune disease need professional clinician to provide follow-up, consulting and health education for early discovery, timely diagnosis, and proper treatment of AID.
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IgG4-related diseases are a class of chronic,progressive,systemic inflammatory disorders that are characterized by lymphoplas-macytic inflammation,as well as elevated IgG4 levels in serum and tissue,and may involve multiple organs or tissues.When involving the pancreas,liver,and biliary tracts,they are called IgG4-related hepatobiliary diseases.IgG4-related autoimmune pancreatitis,IgG4-re-lated sclerosing cholangitis,and IgG4-associated autoimmune hepatitis are reviewed in terms of their clinical manifestations and laboratory findings.Accurate identification of the laboratory results for IgG4-related hepatobiliary diseases and correct diagnosis of these diseases help to avoid unnecessary surgery and wrong treatment.
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Objective To investigate the prevalence and clinical significance of different subtypes (IgG,IgM and IgA) of anticardiolipin antibodies (aCL) and anti-β2-glycoprotein Ⅰ antibodies (aβ 2GP1),as well as lupus anticoagulant (LA) in systemic lupus erythematosus (SLE).Methods IgG/IgM/IgA,IgG,IgM,IgA aCL and anti-β2GP1 were tested by enzyme-linked immunosorbent assay (ELISA) in 100 patients with SLE (42 patients were diagnosed as secondary antiphospholipid syndrome),44 healthy controls and 32 patients with other connective tissue diseases excluding SLE and antiphospholipid syndrome (APS).Meanwhile,LA was tested by modified Dilute Russell's viper venom time (dRVVT).The correlation between antiphospholipid antibodies and clinical manifestation was analyzed by Spearman correlation analysis.The postiverate of antiphospholipid antibodies in SLE patients,health controls and patients with other connective tissue diseases were compared by chi square test.The concentrations of antiphospholipid antibodies in different groups were compared using independent sample Kruskal Wallis test.The diagnostic efficacy of antiphospholipid antibodies in SLE patients was analyzed by crosstable using clinical diagnosis of APS as gold standard.P ＜ 0.05 was considered statistically significant.Results The prevalence of IgG aCL (x2 =15.031,P ＜ 0.001),IgA/G/M (x2 =11.678,P =0.003) and IgA (x2 =6.17,P =0.036) antiβ2GP1 were significantly higher in patients with SLE than in the other two groups.IgA/G/M (r =0.207,P=0.039),IgG (r=0.230,P=0.021) and IgA (r=0.217,P=0.030) aCL,IgA/G/M (r=0.218,P=0.029) and IgA (r =0.255,P =0.01) anti-β2GP1,as well as LA (r =0.233,P =0.02) were associated with thrombotic events.IgA/G/M anti-β2GP1 (r =0.22,P =0.029) and LA (r =0.254,P =0.011) were associated with pathological pregnancy.23.1％ (6/26) aCL positive SLE patients were IgM and/or IgA aCL positive.53.6％ (15/28) anti-β2GP1 positive SLE patients were IgM and/or IgA antiβ2GP1 positive.In SLE patients,the specificity and sensitivity of IgA/G/M aCL for APS were 98.3％ and 26.2％,respectively.The specificity and sensitivity of IgA/G/M anti-β2GP1 were 84.5％ and 40.5％,respectively.The specificity of at least two isotypes positive for APS (both aCL and anti-β2GP1 were 98.3％),was higher than IgG aCL (94.8％) or anti-β2GP1 (93.1％).The sensitivity of at least one isotype of aCL (47.6％) or anti-β2GP1 (42.9％) positive for APS were higher than IgG aCL (40.5％)and anti-β2GP1 (21.4％).Conclusions IgG and IgM aCL together would be better than IgA/G/M aCL for APS screening.IgA/G/M anti-β2GP1 would be better for APS screen due to higher sensitivity and strong association with thromboembolic events and pathologic pregnance.IgA aCL or anti-β2GP1 was associated with thromboembolic events.IgA aCL or anti-β2GP1 would be useful for APS diagnosis in IgG and IgM aCL or anti-β2GPl negative patients.
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Antiphospholipid antibodies (APLs) are important for the diagnosis of antiphospholipid syndrome (APS),especially for predicting the risk of thrombosis and pathological pregnancy.However,the heterogeneity of antiphospholipid antibodies,lacking of standardization and significant interlaboratory variation binder the clinical application of APLs and better understanding of APS diagnosis and treatment.Therefore,it is urgent to establish a standardize system for antiphospholipid antibodies test and to improve the performance of the test and perform well-designed clinical evaluation.
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Objective To investigate the growth and exocrine function of BM-MSC derived from PBC patients.Methods To compare the growth patterns and cytokines secretions between PBC patients and healthy controls by student's t test.Results ① There was no difference in growth profile and speed between PBC patients and healthy controls.② The level of TGF-β1 was much lower in the supernatant of BM-MS from OBC patients than health controls [(2.6±1.9)vs (8.2±6.7)ng/ml,t=-3.641,P=0.001].There were no other differences between two groups' BM-MSC.③ The super natant concentration of interlukin-10 of the third BM-MSC subculture from healthy controls was lower than that of the primary subculture [(18.5±5.0) vs (12.4±3.1) pg/ml,t=2.368,P=0.045],and that of hepatic growth factor from the second subcuhure was higher than the primary subculture [(0.21±0.07) vs (0.35±0.08) ng/ml,t=-2.874,P=0.021].There were seldom discrepancies in other cytokines between different generations of BM-MSC.Conclusion BM-MSC from PBC patients may have almost the similar characters in growth pattern and cytokines secretion as,except the TGF-β1,which was much lower than those from healthy controls.The second subculture of BM-MSC might be more suitable for the treatment to patients with PBC.
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The detection of autoimmune liver disease (AILD) relevant autoantibodies is of important value in the diagnosis and treatment of AILD and especially in autoimmune hepatitis and primary biliary cirrhosis.With the increasing of patients clinically diagnosed AILD,the detection of AILD relevant autoantibodies is gradually clinically concerned and appreciated.As the detection of AILD relevant autoantibodies affected by various factors,there are still many problems in the detection and clinical applications of AILD relevant autoantibodies.We should promote the universal clinical application of AILD relevant autoantibodies,emphasis on the quality management and improve the quality of detection constantly,attend to the standard detection and rational application of AILD relevant autoantibodies.