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BACKGROUND: Immune-mediated necrotizing myopathy (IMNM) is an autoimmune myopathy characterized by severe proximal weakness and muscle fiber necrosis, yet its pathogenesis remains unclear. So far, there are few bioinformatics studies on underlying pathogenic genes and infiltrating immune cell profiles of IMNM. Therefore, we aimed to characterize differentially expressed genes (DEGs) and infiltrating cells in IMNM muscle biopsy specimens, which may be useful for elucidating the pathogenesis of IMNM. METHODS: Three datasets (GSE39454, GSE48280 and GSE128470) of gene expression profiling related to IMNM were obtained from the Gene Expression Omnibus database. Data were normalized, and DEG analysis was performed using the limma package. Gene Ontology (GO) and Kyoto Encyclopedia of Genes and Genomes (KEGG) enrichment analyses of DEGs were performed using clusterProfiler. The CIBERSORT algorithm was performed to identify infiltrating cells. Machine learning algorithm and gene set enrichment analysis (GSEA) were performed to find distinctive gene signatures and the underlying signaling pathways of IMNM. RESULTS: DEG analysis identified upregulated and downregulated in IMNM muscle compared to the gene expression levels of other groups. GO and KEGG analysis showed that the pathogenesis of IMNM was notable for the under-representation of pathways that were important in dermatomyositis and inclusion body myositis. Three immune cells (M2 macrophages, resting dendritic cells and resting natural killer cells) with differential infiltration and five key genes (NDUFAF7, POLR2J, CD99, ARF5 and SKAP2) in patients with IMNM were identified through the CIBERSORT and machine learning algorithm. The GSEA results revealed that the key genes were remarkably enriched in diverse immunological and muscle metabolism-related pathways. CONCLUSIONS: We comprehensively explored immunological landscape of IMNM, which is indicative for the research of IMNM pathogenesis.
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Doenças Musculares , Miosite , Humanos , Transcriptoma , Miosite/genética , Miosite/patologia , Doenças Musculares/genética , Perfilação da Expressão Gênica , Aprendizado de Máquina , RNA Polimerase II/genéticaRESUMO
Immune-mediated necrotizing myopathy (IMNM) is a rare and newly recognized autoimmune disease within the spectrum of idiopathic inflammatory myopathies. It is characterized by myositis-specific autoantibodies, elevated serum creatine kinase levels, inflammatory infiltrate, and weakness. IMNM can be classified into three subtypes based on the presence or absence of specific autoantibodies: anti-signal recognition particle myositis, anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase myositis, and seronegative IMNM. In recent years, IMNM has gained increasing attention and emerged as a research hotspot. Recent studies have suggested that the pathogenesis of IMNM is linked to aberrant activation of immune system, including immune responses mediated by antibodies, complement, and immune cells, particularly macrophages, as well as abnormal release of inflammatory factors. Non-immune mechanisms such as autophagy and endoplasmic reticulum stress also participate in this process. Additionally, genetic variations associated with IMNM have been identified, providing new insights into the genetic mechanisms of the disease. Progress has also been made in IMNM treatment research, including the use of immunosuppressants and the development of biologics. Despite the challenges in understanding the etiology and treatment of IMNM, the latest research findings offer important guidance and insights for delving deeper into the disease's pathogenic mechanisms and identifying new therapeutic strategies.
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OBJECTIVE: Myocardial fibrosis occurs in the early subclinical stage of cardiac involvement in idiopathic inflammatory myopathies (IIMs). Soluble suppression of tumorigenicity 2 (sST2) is known to have an immunomodulatory impact during autoimmune disease development. The current study investigated the diagnostic value of sST2 for myocardial fibrosis during early stage of cardiac involvement in IIM. METHODS: A total of 44 IIM patients with normal heart function and 32 age- and gender-matched healthy controls (HCs) were enrolled. Serum sST2 levels were measured by ELISA and cardiac magnetic resonance (CMR) parameters for myocardial fibrosis [native T1, extracellular volume (ECV), late-gadolinium enhancement (LGE)] and oedema (T2 values) were analysed. RESULTS: IIM patients had significantly higher sST2 levels than HCs [67.5 ng/ml (s.d. 30.4)] vs 14.4 (5.5), P < 0.001] and levels correlated positively with diffuse myocardial fibrosis parameters, native T1 (r = 0.531, P = 0.000), ECV (r = 0.371, P = 0.013) and focal myocardial fibrosis index and LGE (r = 0.339, P = 0.024) by Spearman's correlation analysis. sST2 was an independent predictive factor for diffuse and focal myocardial fibrosis after adjustment for age, gender, BMI and ESR. Risk increased ≈15.4% for diffuse [odds ratio (OR) 1.154 (95% CI 1.021, 1.305), P = 0.022] and 3.8% for focal [OR 1.038 (95% CI 1.006, 1.072), P = 0.020] myocardial fibrosis per unit increase of sST2. Cut-off values for diagnosing diffuse and focal myocardial fibrosis were sST2 ≥51.3 ng/ml [area under the curve (AUC) = 0.942, sensitivity = 85.7%, specificity = 98.9%, P < 0.001] and 53.3 ng/ml (AUC = 0.753, sensitivity = 87.5%, specificity = 58.3%, P < 0.01), respectively. CONCLUSION: sST2 showed a marked elevation during the subclinical stage of cardiac involvement in IIM and has potential as a biomarker for predicting diffuse and focal myocardial fibrosis in IIM.
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Cardiomiopatias , Miosite , Humanos , Meios de Contraste , Gadolínio , Cardiomiopatias/diagnóstico por imagem , Cardiomiopatias/etiologia , FibroseRESUMO
BACKGROUND: Rheumatoid arthritis (RA) is a chronic autoimmune disorder characterized by synovial inflammation, causing substantial disability and reducing life quality. While macrophages are widely appreciated as a master regulator in the inflammatory response of RA, the precise mechanisms underlying the regulation of proliferation and inflammation in RA-derived fibroblast-like synoviocytes (RA-FLS) remain elusive. Here, we provide extensive evidence to demonstrate that macrophage contributes to RA microenvironment remodeling by extracellular vesicles (sEVs) and downstream miR-100-5p/ mammalian target of rapamycin (mTOR) axis. RESULTS: We showed that bone marrow derived macrophage (BMDM) derived-sEVs (BMDM-sEVs) from collagen-induced arthritis (CIA) mice (cBMDM-sEVs) exhibited a notable increase in abundance compared with BMDM-sEVs from normal mice (nBMDM-sEVs). cBMDM-sEVs induced significant RA-FLS proliferation and potent inflammatory responses. Mechanistically, decreased levels of miR-100-5p were detected in cBMDM-sEVs compared with nBMDM-sEVs. miR-100-5p overexpression ameliorated RA-FLS proliferation and inflammation by targeting the mTOR pathway. Partial attenuation of the inflammatory effects induced by cBMDM-sEVs on RA-FLS was achieved through the introduction of an overexpression of miR-100-5p. CONCLUSIONS: Our work reveals the critical role of macrophages in exacerbating RA by facilitating the transfer of miR-100-5p-deficient sEVs to RA-FLS, and sheds light on novel disease mechanisms and provides potential therapeutic targets for RA interventions.
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Artrite Reumatoide , Macrófagos , MicroRNAs , Transdução de Sinais , Serina-Treonina Quinases TOR , Animais , Humanos , Masculino , Camundongos , Artrite Experimental/metabolismo , Artrite Experimental/patologia , Artrite Experimental/genética , Artrite Reumatoide/metabolismo , Artrite Reumatoide/genética , Artrite Reumatoide/patologia , Proliferação de Células , Vesículas Extracelulares/metabolismo , Inflamação/metabolismo , Macrófagos/metabolismo , Camundongos Endogâmicos DBA , MicroRNAs/genética , MicroRNAs/metabolismo , Membrana Sinovial/metabolismo , Membrana Sinovial/patologia , Sinoviócitos/metabolismo , Serina-Treonina Quinases TOR/metabolismoRESUMO
OBJECTIVES: N-terminal fragment of titin (N-titin) is a marker of sarcomere damage in striated muscles; however, its value in patients with IIM (idiopathic inflammatory myopathy) is unclear. This study aimed to investigate the diagnostic value of N-titin for skeletal muscle damage in patients with IIM. METHODS: Urine samples from 62 patients with IIM, 59 patients with other CTD diseases, and 29 healthy controls were collected to detect N-titin by ELISA assays. Clinical features and laboratory data were all included in logistic regression analysis to obtain the independent predictive factor for skeletal muscle damage. RESULTS: Urinary N-titin level of the IIM group [168.3 (19.0, 1279.0) pmol/mg cr] was significantly higher than that in CTD controls [2.80 (1.53, 3.60)] and healthy controls [1.83 (1.09, 2.95)] (P < 0.001). IIM patients with skeletal muscle injury had a significantly higher level of urinary N-titin [1001.0, (181.8, 1977.0)] than those without [9.3, (5.8, 23.9)] (P < 0.001). The N-titin level was strongly correlated with CK (r = 0.907, P < 0.001) and muscle disease activity assessment scores by Spearman correlation analysis. After adjusting for the anti-MDA5 antibody and cardiac troponin T, N-titin was shown to independently predict skeletal muscle damage in patients with IIM (odds ratio = 1.035, 95% CI: 1.002, 1.069, P = 0.039). The cut-off value of urinary N-titin to diagnose skeletal muscle damage was 89.9 pmol/mg Cr, with a sensitivity of 87.8% and a specificity of 100% (AUC = 0.971, 95% CI: 0.938, 1.000, P < 0.001). CONCLUSION: Urinary N-titin is a non-invasive and independent predictive factor for determining skeletal muscle damage in patients with IIM.
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Doenças Musculares , Miosite , Humanos , Conectina/urina , Miosite/diagnóstico , Músculo Esquelético , Ensaio de Imunoadsorção EnzimáticaRESUMO
INTRODUCTION: Rheumatoid arthritis (RA) is a chronic autoimmune disorder characterized by hyperplastic synovium, pannus formation, immune cell infiltration, and potential articular cartilage damage. Notably, fibroblast-like synoviocytes (FLS), especially rheumatoid arthritis fibroblast-like synoviocytes (RAFLS), exhibit specific overexpression of glycolytic enzymes, resulting in heightened glycolysis. This elevated glycolysis serves to generate ATP and plays a pivotal role in immune regulation, angiogenesis, and adaptation to hypoxia. Key glycolytic enzymes, such as hexokinase 2 (HK2), phosphofructose-2-kinase/fructose-2,6-bisphosphatase 3 (PFKFB3), and pyruvate kinase M2 (PKM2), significantly contribute to the pathogenic behavior of RAFLS. This increased glycolysis activity is regulated by various signaling pathways. MATERIALS AND METHODS: A comprehensive literature search was conducted to retrieve relevant studies published from January 1, 2010, to the present, focusing on RAFLS glycolysis, RA pathogenesis, glycolytic regulation pathways, and small-molecule drugs targeting glycolysis. CONCLUSION: This review provides a thorough exploration of the pathological and physiological characteristics of three crucial glycolytic enzymes in RA. It delves into their putative regulatory mechanisms, shedding light on their significance in RAFLS. Furthermore, the review offers an up-to-date overview of emerging small-molecule candidate drugs designed to target these glycolytic enzymes and the upstream signaling pathways that regulate them. By enhancing our understanding of the pathogenic mechanisms of RA and highlighting the pivotal role of glycolytic enzymes, this study contributes to the development of innovative anti-rheumatic therapies.
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Artrite Reumatoide , Sinoviócitos , Humanos , Sinoviócitos/metabolismo , Artrite Reumatoide/metabolismo , Membrana Sinovial/patologia , Transdução de Sinais , Fibroblastos/metabolismoRESUMO
Calciphylaxis, also known as calcific uremic arteriopathy, is a rare calcification syndrome that presents as ischemic skin necrosis and severe pain. It has a high mortality rate and is characterised by calcification of the small and medium arteries and micro-thrombosis. Calciphylaxis mainly occurs in patients with end-stage renal disease. In recent years, there have been an increasing number of cases of calciphylaxis associated with connective tissue diseases. Given the absence of clear diagnostic criteria for calciphylaxis thus far, an early diagnosis is crucial for designing an effective multidisciplinary treatment plan. In this article, we review the research progress on calciphylaxis and describe its characteristics in the context of connective tissue diseases.
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Calcinose , Calciofilaxia , Doenças do Tecido Conjuntivo , Falência Renal Crônica , Humanos , Calciofilaxia/complicações , Calciofilaxia/diagnóstico , Calciofilaxia/terapia , Falência Renal Crônica/complicações , Falência Renal Crônica/terapia , Pele , Doenças do Tecido Conjuntivo/complicaçõesRESUMO
OBJECTIVE: Gut dysbiosis contributes to multiple autoimmune diseases, including ankylosing spondylitis, which is commonly treated with tumor necrosis factor (TNF)-α inhibitors (TNFis). Because host TNF-α levels are considered to interact with gut microbiota, we aimed to systematically investigate the microbiota profile of ankylosing spondylitis patients with anti-TNF-α-based treatment and identify potential key bacteria. METHODS: Fecal samples were collected from 11 healthy controls and 24 ankylosing spondylitis patients before/after anti-TNF-α treatment, the microbiota profiles of which were evaluated by 16S ribosomal DNA amplicon sequencing and subsequent bioinformatic analysis. RESULTS: Significantly different microbial compositions were observed in samples from ankylosing spondylitis patients compared with healthy controls, characterized by a lower abundance of short-chain fatty acid (SCFA)-producing bacteria. All patients exhibited a positive response after anti-TNF-α treatment, accompanied by a trend of restoration in the microbiota compositions and functional profile of ankylosing spondylitis patients to healthy controls. In particular, the abundance of SCFA-producing bacteria (e.g. Megamonsa and Lachnoclostridium ) was not only significantly lower in ankylosing spondylitis patients than in healthy controls and restored after anti-TNF-α treatment but also negatively correlated with disease severity (e.g. cor = -0.52, P = 8 × 10 -5 for Megamonsa ). In contrast, Bacilli and Haemophilus may contribute to ankylosing spondylitis onset and severity. CONCLUSIONS: Microbiota dysbiosis in ankylosing spondylitis patients can be restored after anti-TNF-α treatment, possibly by impacting SCFA-producing bacteria.
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Microbioma Gastrointestinal , Espondilite Anquilosante , Bactérias/genética , Disbiose/microbiologia , Microbioma Gastrointestinal/genética , Humanos , Espondilite Anquilosante/tratamento farmacológico , Espondilite Anquilosante/microbiologia , Espondilite Anquilosante/patologia , Inibidores do Fator de Necrose Tumoral , Fator de Necrose Tumoral alfaRESUMO
INTRODUCTION: We performed a cross-sectional study to investigate the clinical usefulness of YKL-40 in patients with dermatomyositis (DM) and conducted a systematic review to summarize the clinical value of YKL-40 in patients with polymyositis (PM)/DM. MATERIALS AND METHODS: A cross-sectional study and a systematic review were performed to study the clinical value of YKL-40 in patients with PM/DM. Serum YKL-40 level was detected using enzyme-linked immunosorbent assay, and its association with clinical and laboratory parameters was analyzed. In the systematic review, electronic databases of OVID Embase, OVID Medline, and web of science were searched to collect studies that reported clinical use of YKL-40 in patients with PM/DM. RESULTS: In the cross-sectional study, serum YKL-40 level was higher in patients with DM than in healthy controls (median [interquartile range]: 84.09 [52.72-176.4] ng/ml versus 27.37 [12.30-53.58] ng/ml, p < 0.0001). Serum levels of YKL-40 were associated with the course of DM (r = -0.469, p < 0.001), CRP (r = 0.303, p = 0.043), CK (r = 0.263, p = 0.037), and global disease activity (r = 0.628, p < 0.001). The area under the ROC curve was 0.835 (95% confidence interval 0.751-0.920). In the systematic review, a total of four studies were included with moderate to high quality. Serum level of YKL-40 has the possibility for diagnosing PM/DM, identifying PM/DM patients with interstitial lung disease (ILD) or rapid progress ILD, and predicting death. CONCLUSION: Serum YKL-40 level is a possible useful biomarker for PM/DM diagnosis and may be used to predict prognosis.
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Proteína 1 Semelhante à Quitinase-3/metabolismo , Dermatomiosite , Doenças Pulmonares Intersticiais , Polimiosite , Estudos Transversais , Humanos , PrognósticoRESUMO
OBJECTIVES: Cardiac dysfunction is commonly noted in patients with idiopathic inflammatory myopathies (IIMs). This study aimed to investigate the characteristics of cardiac dysfunction using cardiac magnetic resonance (CMR) in polymyositis (PM), dermatomyositis (DM) and necrotising myositis (NM). METHODS: Fifty-one patients with IIMs and 20 matched healthy controls (HCs) were assessed using CMR examination. The clinical data, cardiac serum markers and autoimmune antibodies were determined for all patients. Cardiac involvement was identified by myocardial native T1, extracellular volume (ECV), late gadolinium enhancement (LGE) and left ventricular ejection fraction (LVEF). RESULTS: Different subtypes of IIMs showed different patterns of LGE and varying degrees of myocardial damage. The PM subgroup showed higher native T1 (p = 0.010) and ECV (p = 0.000) than the HCs. The prevalence of LGE was comparable between the PM and DM subgroups (40.0% vs. 31.6%, p = 0.741); however, it was higher in the PM subgroup than in the NM subgroup (40% vs. 0.0%, p = 0.014). Patients with positive LGE in the PM subgroup showed a higher proportion of positive LGE (p = 0.018) and lower LVEF (p = 0.024) than those with positive LGE in the DM subgroup. In multivariate analysis, the presence of LGE could be predicted by increased NT-proBNP (p = 0.036, OR = 1.001) and anti-MDA-5 antibody positivity (p = 0.011, OR = 12.4). The risk factors associated with native T1 were NT-proBNP (p = 0.016, ß = 0.353) and body mass index (BMI) (p = 0.024, ß = - 0.331). CONCLUSIONS: Distinct cardiac involvements in different subtypes of IIMs were identified using CMR. Elevated NT-proBNP and a low BMI were the risk factors associated with LGE and elevated native T1. KEY POINTS: ⢠The characteristics of cardiac involvement in different subtypes of IIMs could be identified with cardiac magnetic resonance. ⢠The NT-proBNP levels could reflect focal and diffuse myocardial damage in patients with IIMs.
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Meios de Contraste , Miosite , Gadolínio , Humanos , Imagem Cinética por Ressonância Magnética , Espectroscopia de Ressonância Magnética , Miocárdio , Miosite/diagnóstico por imagem , Valor Preditivo dos Testes , Volume Sistólico , Função Ventricular EsquerdaRESUMO
BACKGROUND: Vibrio scophthalmi is an opportunistic bacterial pathogen, which is widely distributed in the marine environment. Earlier studies have suggested that it is a normal microorganism in the turbot gut. However, recent studies have confirmed that this bacterial strain can cause diseases in many different marine animals. Therefore, it is necessary to investigate its whole genome for better understanding its physiological and pathogenic mechanisms. RESULTS: In the present study, we obtained a pathogenic strain of V. scophthalmi from diseased half-smooth tongue sole (Cynoglossus semilaevis) and sequenced its whole genome. Its genome contained two circular chromosomes and two plasmids with a total size of 3,541,838 bp, which harbored 3185 coding genes. Among these genes, 2648, 2298, and 1915 genes could be found through annotation information in COG, Blast2GO, and KEGG databases, respectively. Moreover, 10 genomic islands were predicted to exist in the chromosome I through IslandViewer online system. Comparison analysis in VFDB and PHI databases showed that this strain had 334 potential virulence-related genes and 518 pathogen-host interaction-related genes. Although it contained genes related to four secretion systems of T1SS, T2SS, T4SS, and T6SS, there was only one complete T2SS secretion system. Based on CARD database blast results, 180 drug resistance genes belonging to 27 antibiotic resistance categories were found in the whole genome of such strain. However, there were many differences between the phenotype and genotype of drug resistance. CONCLUSIONS: Based on the whole genome analysis, the pathogenic V. scophthalmi strain contained many types of genes related to pathogenicity and drug resistance. Moreover, it showed inconsistency between phenotype and genotype on drug resistance. These results suggested that the physiological mechanism seemed to be complex.
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Doenças dos Peixes/microbiologia , Linguados/microbiologia , Vibrioses/veterinária , Vibrio/genética , Animais , Antibacterianos/farmacologia , Farmacorresistência Bacteriana/genética , Doenças dos Peixes/patologia , Genes Bacterianos/genética , Tamanho do Genoma , Genoma Bacteriano/genética , Ilhas Genômicas , Interações Hospedeiro-Patógeno/genética , Testes de Sensibilidade Microbiana , Filogenia , Vibrio/classificação , Vibrio/efeitos dos fármacos , Vibrio/patogenicidade , Vibrioses/microbiologia , Vibrioses/patologia , Fatores de Virulência/genéticaRESUMO
Vibrio rotiferianus is an important marine pathogen of various aquatic organisms and can be found widely distributed in the marine environment. To further characterize this pathogen, the pathogenic properties and genome of V. rotiferianus SSVR1601 isolated from Sebastes schlegelii with skin ulcer were analysed. SSVR1601 was shown to be short rod-shaped cell with a single polar flagellum. Different degrees of pathological changes in fish kidney, intestine, gills and liver were observed after SSVR1601 challenge. The SSVR1601 genome consists of two chromosomes and two plasmids with a total of 5,717,113 bp, 42.04%-44.93% GC content, 5,269 predicted CDSs, 134 tRNAs and 40 rRNAs. The common virulence factors including OMPs, haemolysin, flagellin, DNase, entF, algU, tcpI, acfB and rfaD were found in strain SSVR1601. Furthermore, factors responsible for iron uptake (fur, fepC and ccmC) and types II, IV and VI secretion systems were detected, which are likely responsible for the pathogenicity of SSVR1601. The antimicrobial resistance genes, bacA, tet34 and norM, were detected based on Antibiotic Resistance Genes Database. The phylogenetic analysis revealed SSVR1601 to be most closely related to V. rotiferianus strains CAIM577 and B64D1.
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Doenças dos Peixes/patologia , Peixes , Genoma Bacteriano , Úlcera Cutânea/veterinária , Vibrioses/veterinária , Vibrio/genética , Vibrio/patogenicidade , Animais , Doenças dos Peixes/microbiologia , Filogenia , Análise de Sequência de DNA/veterinária , Úlcera Cutânea/microbiologia , Úlcera Cutânea/patologia , Vibrio/classificação , Vibrioses/microbiologia , Vibrioses/patologiaRESUMO
Comorbidity of autoimmune diseases is a very important issue but easily ignored in the clinical practice. The treatment of comorbidity of autoimmune diseases needs cooperation of multiple disciplines,which is totally different from traditional clinical disciplines division and treatment mode. Based on the clinical features of the disease,we will comprehensively look through genetic,environmental,and immune factors involving in molecular and immunological compatibility pathogenesis,and also generalize common pathological features,such as immune complex deposition and accumulation of lymphocytes. We will also investigate the association and differences between the diseases with comorbidity,and explore the outcome and prognosis of comorbidity of autoimmune diseases. With clarify of the concept of autoimmune comorbidities,we hope bring more and more attention on this aspect,so as to improve the diagnosis,treatments as well as the prognosis of these diseases.
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Doenças Autoimunes/complicações , Comorbidade , Complexo Antígeno-Anticorpo/imunologia , Humanos , Linfócitos/imunologia , PrognósticoRESUMO
OBJECTIVE: To explore the biochemical-immune and pathological characteristics of autoimmune hepatitis (AIH) with Sjögren's syndrome (SS) . METHODS: A total of 76 cases of AIH patients were included from January 2009 to April 2017. Among them,there were 40 cases of AIH with SS and 36 cases without SS. The liver function,immunological index,histological features,length of first diagnosis and treatment costs were compared between the two groups. RESULTS: For AIH+SS group and AIH group,the proportion of women were 97.5% and 77.8%,the proportion of the first diagnosis age less than 60 years were 70% and 47.2%,the median course of disease were 30 months and 9 months,all the difference were statistically significant (P<0.05). The chief complaints in AIH+SS group and AIH group were as follows: cutaneous or scleracterus (52.5% vs. 38.9%),abnormal transaminase (17.5% vs. 44.4%),dryness of mouth and eye (15.0% vs. 2.8%),all the difference were statistically significant (P<0.05). There were no statistically significant difference in hospitalization expenses,and length of stay between the two groups (P>0.05). The median level of total bilirubin (TBIL),direct bilirubin (DBIL) and immunoglobulin (Ig) M of AIH +SS group were higher than those of AIH group,the mean level of albumin (ALB) and complement 3 (C3) of AIH +SS group were lower than those of AIH group,and the positive rate of anti-mitochondrial antibody-M2 (AMA-M2) ,anti-Ro antibody A (SSA),anti-La antibody (SSB) and anti-soluble liver antigen antibody (SLA) of AIH+SS group were higher than those of AIH group (P<0.05). There were no statistically significant difference in histological changes of hepatocytes and bile duct injury rate (P>0.05). CONCLUSION: AIH patients in young and middle-aged women need to be vigilant with SS with main manifestation of skin sclera and high specific autoantibodies positive.
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Hepatite Autoimune/fisiopatologia , Síndrome de Sjogren/fisiopatologia , Anticorpos Antinucleares/sangue , Feminino , Hepatite Autoimune/complicações , Humanos , Fígado/fisiopatologia , Masculino , Pessoa de Meia-Idade , Síndrome de Sjogren/complicaçõesRESUMO
OBJECTIVE: To investigate the correlation of disease activity and thyroid indicators ,immunologic markers of system lupus erythematosus (SLE) in SLE with Hashimoto's thyroiditis (HT). METHODS: The clinical data of 63 cases of SLE with HT were collected. According to Systemic lupus erythematosus disease activity index 2000 (SLEDAI-2000),we classified the patients into four groups,which were remission group (5 cases),low (19 cases),moderate (12 cases) and high (27 cases) disease activity group. Each patient received the measurement of thyroid function indicators and autoantibodies,SLE immunologic indicators,serum complement (C3,C4),C-reactive protein (CRP),erythrocyte sedimentation rate (ESR), and routine blood test. The correlation of thyroid indicators,immunologic markers and disease activity were analyzed. RESULTS: The difference of free triiodothyronine (FT3) level in the four groups was statistically significant (P<0.05),and FT3 was negatively correlated with SLE disease activity (P=0.007) . There was no significant difference in other thyroid indicators and autoantibodies between the different groups (P>0.05). Negative correlation was found between FT3 level and anti-double-stranded DNA (dsDNA),level of anti-La antibody (SSB) and thyroid stimulating hormone (TSH) or thyroid peroxidase antibody (TPOAb). Thyroglobulin autoantibody (TgAb) was negatively related with C4,and positive correlation between FT3 and C3,FT4 and C4,TgAb and IgA. CONCLUSION: The pathogenesis of HT is associated with the disease activity in the patients of SLE with HT.
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Autoanticorpos/sangue , Doença de Hashimoto/complicações , Lúpus Eritematoso Sistêmico/diagnóstico , Anticorpos Antinucleares/sangue , Sedimentação Sanguínea , Proteína C-Reativa/análise , Proteínas do Sistema Complemento/análise , Doença de Hashimoto/sangue , Humanos , Lúpus Eritematoso Sistêmico/sangue , Lúpus Eritematoso Sistêmico/complicações , Glândula Tireoide/fisiopatologia , Tireotropina/sangueRESUMO
OBJECTIVE: To reveal the clinical features of respiratory failure (RF) in dermatomyositis (DM) patients with interstitial lung disease (ILD),and to explore risk factors of RF in these patients. METHODS: The medical data of 122 DM patients with ILD were retrospectively reviewed: 40 developed RF (RF group),82 did not develope RF (Non RF group). Clinical,laboratory and radiological variables were compared between RF patients and Non RF patients. Multivariate Logistic regression was used to analyze risk factors of RF. RESULTS: In RF patients,the female-male ratio was 3â¶1,the median age at DM onset was 49.5 (42.3-58.6) years-old. There were 67.5%,85.0% and 95.0% patients developed RF within 6 months,1 year and 2 years after the onset of DM. The factors significantly associated with RF included DM onset age,clinically amyopathic dermatomyositis (CADM),pneumomediastinum (PNM),aspartate aminotransferase(AST),lactate dehydrogenase (LDH),albumin,neutrophil-lymphocyte ratio,platelet-lymphocyte ratio,anti-Jo-1 antibody presence and ground-glass opacities sign (P<0.05). PMN and anti-Jo-1 antibody failed to be involved in logistic regression model. The regression analysis demonstrated that older DM onset age [odds ratio (OR)=1.791,P=0.025],higher AST level (OR=1.937,P=0.048),CADM diagnosis (OR=3.881,P=0.007) and ground-glass opacities sign (OR=4.187,P=0.014) were independent risk factors of RF in DM patients with ILD. CONCLUSION: RF occurs more often within 2 years of DM onset. The DM patients with older DM onset age,elevated AST level,CADM diagnosis or ground-glass opacities sign took higher risks for RF development.
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Dermatomiosite/complicações , Doenças Pulmonares Intersticiais/complicações , Insuficiência Respiratória/complicações , Adulto , Idade de Início , Aspartato Aminotransferases/sangue , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Fatores de RiscoRESUMO
OBJECTIVE: To investigate the relationship between tumor related markers and the presence of interstitial lung disease (ILD) in dermatomyositis (DM) patients as well as potential serum markers for accompanied ILD. METHODS: Fifty-nine DM patients were included,including 30 patents with ILD. Serum level of anti-transcription intermediary factor1-γ (TIF1-γ) and tumor-associated antigens (TAAs) were detected to analyze the correlation of these markers with ILD. Meanwhile,the diagnostic value of these markers was evaluated by receive operating characteristic (ROC) curve analysis. RESULTS: We found that there were 5 patients bearing malignancies,independent from the presence of ILD (P=0.024). Serum anti-TIF1-γ was positive in 4/5 DM patients with tumor,of which sensitivity and specificity were 36.36% and 97.92%,respectively. In contrast,only one case was found positive anti-TIF1-γ in 30 DM with ILD (P=0.002).Carbohydrate antigen (CA)125,CA153,and cytokeratin-19 fragment (CYFRA21-1) were elevated in DM patients with ILD in compared to those without ILD. The area under curve (AUC) of CYFRA21-1 for the prediction of ILD was 0.745 (P=0.002). The optimal cut-off value was 3.58 ng/mL with a sensitivity of 60.71% and a specificity of 84.00%. The AUC in combination of CA125 and CYFRA21-1 could reach 0.801 (P<0.002) with a sensitivity of 64.29% and a specificity of 82.61%. CONCLUSION: Presence of malignance and positive anti-TIF1γ could be the protective factors for ILD in DM. Elevated serum levels of CA125,CA153,CYFRA21-1 could indicate the accompanying ILD.