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Background: Extrahepatic manifestations in patients with primary biliary cholangitis (PBC) are frequently observed recently. We aimed in this study to explore the clinicopathological characteristics and prognosis of glomerulonephritis in patients with PBC. Methods: Consecutive PBC patients admitted to Peking Union Medical College Hospital from January 2002 to May 2019 were retrospectively enrolled. PBC patients with other autoimmune diseases which may have nephritis were excluded. Structured interview, systemic rheumatologic examination, and laboratory tests were conducted for each patient. Literature about patients with PBC and glomerulonephritis was reviewed and summarized. Results: Among the 330 PBC patients enrolled, glomerulonephritis were identified in 10 patients (3.0%). Eight (80.0%) were females and 2 (20.0%) were males. The mean age was 58.4 ± 9.5 years old. Membranous nephropathy (MN) was revealed in 4 patients, IgA nephropathy (IgA N) in 2 patients, minimal change disease (MCD) in 2 patients, mesangial proliferative glomerulonephritis in 1 patient, and renal amyloidosis in 1 patient. Compared to the literature reviewed, 10 cases of MN, 1 case of MCD, 1 case of IgA N, and 1 case of acute poststreptococcal glomerulonephritis (APSGN) were observed. Conclusions: Glomerulonephritis may not be a well-recognized feature of PBC and is not a rare complication and deserved to be routinely screened in clinical practice. As MN is the most common form of glomerulonephritis in PBC patients and PBC can be asymptomatic at an early stage, patients presented with MN should be screened for PBC, so to avoid cirrhosis. Key Points • Patients with primary biliary cholangitis (PBC) can be complicated with glomerulonephritis, and membranous nephropathy (MN) is the most common form. [ABSTRACT FROM AUTHOR]

Primary Sjögren's syndrome (pSS) is a prevalent autoimmune disorder wherein CD4+ T cells play a pivotal role in its pathogenesis. However, the underlying mechanisms driving the hyperactivity of CD4+ T cells in pSS remain poorly understood. This study aimed to investigate the potential role of immunometabolic alterations in driving the hyperactivity of CD4+ T cells in pSS. We employed Seahorse XF assay to evaluate the metabolic phenotype of CD4+ T cells, conducted flow cytometry to assess the effector function and differentiation of CD4+ T cells and measured the level of intracellular reactive oxygen species (ROS). Additionally, transcriptome sequencing, PCR, and Western blotting were utilized to examine the expression of glycolytic genes. Our investigation revealed that activated CD4+ T cells from pSS patients exhibited elevated aerobic glycolysis, rather than oxidative phosphorylation, resulting in excessive production of IFN-γ and IL-17A. Inhibition of glycolysis by 2-Deoxy-D-glucose reduced the expression of IFN-γ and IL-17A in activated CD4+ T cells and mitigated the differentiation of Th1 and Th17 cells. Furthermore, the expression of glycolytic genes, including CD3E, CD28, PIK3CA, AKT1, mTOR, MYC, LDHA, PFKL, PFKFB3, and PFKFB4, was upregulated in activated CD4+ T cells from pSS patients. Specifically, the expression and activity of LDHA were enhanced, contributing to an increased level of intracellular ROS. Targeting LDHA with FX-11 or inhibiting ROS with N-acetyl-cysteine had a similar effect on reversing the dysfunction of activated CD4+ T cells from pSS patients. Our study unveils heightened aerobic glycolysis in activated CD4+ T cells from pSS patients, and inhibition of glycolysis or its metabolite normalizes the dysfunction of activated CD4+ T cells. These findings suggest that aerobic glycolysis may be a promising therapeutic target for the treatment of pSS. [ABSTRACT FROM AUTHOR]

Inflammatory arthritis (IA) is a common rheumatic adverse event following immune checkpoint inhibitors treatment. The clinical disparities between IA and rheumatoid arthritis (RA) imply disease heterogeneity and distinct mechanisms, which remain elusive. Here, we profile CD45+ cells from the peripheral blood or synovial fluid (SF) of patients with PD-1-induced IA (PD-1-IA) or RA using single-cell RNA sequencing. We report the predominant expansion of IL1Bhi myeloid cells with enhanced NLRP3 inflammasome activity, in both the SF and peripheral blood of PD-1-IA, but not RA. IL1Bhi macrophages in the SF of PD-1-IA shared similar inflammatory signatures and might originate from peripheral IL1Bhi monocytes. Exhausted CD8+ T cells (Texs) significantly accumulated in the SF of patients with PD-1-IA. IL1Bhi myeloid cells communicated with CD8+ Texs possibly via the CCR1-CCL5/CCL3 and CXCL10-CXCR3 axes. Collectively, these results demonstrate different cellular and molecular pathways in PD-1-IA and RA and highlight IL1Bhi macrophages as a possible therapeutic target in PD-1-IA.Patients undergoing treatment with PD-1 blockade can experience inflammatory arthritis as an adverse event. Here, the authors use single-cell RNA sequencing to identify IL1Bhi macrophages in PD-1-inhibitor-induced arthritis, but not rheumatoid arthritis. [ABSTRACT FROM AUTHOR]

Objective: Combination therapy of glucocorticoids (GCs) plus leflunomide (LEF) and GCs plus mycophenolate mofetil (MMF) was reported to have good efficacy and safety in the management of IgG4-RD. However, studies comparing the efficacy and safety of these two combination therapies were unavailable. Herein, this study aimed to compare the efficacy and safety of GCs plus LEF and GCs plus MMF in treating IgG4-RD. Methods: This study included 130 newly diagnosed IgG4-RD patients who received the therapy of GCs plus LEF (group I) and GCs plus MMF (group II). Clinical data at baseline and after treatment, treatment response, relapse rate, and adverse effects were recorded and analyzed. Results: Patients in both groups responded well to the treatment in the 1st-month follow-up, and 100% of patients achieved treatment response. However, at the 6th and 12th-month follow-up, the total response rate of group II was higher than that in group I (75.6 vs. 53.7%, p = 0.038 and 85.4% vs. 61.0%, p = 0.013, respectively). In addition, the duration of disease remission in group II was longer than that in group I (9 (6–9) vs. 6 (6–6) months, p = 0.014). Moreover, more patients in group I had adverse effects compared with group II (36.6 vs. 7.3%, p < 0.01); and the most common adverse events of LEF were rash (12.2%) and elevation of liver enzymes (9.8%). Conclusion: The combination therapy of GCs plus low-dose MMF had better efficacy and safety in the management of IgG4-RD compared with the therapy of GCs plus LEF. Key points • This is the first study comparing the efficacy and safety of GCs plus LEF/MMF against IgG4-RD. • GCs plus MMF had better efficacy and safety in the management of IgG4-RD. • This study provides physicians with decision-making advice in choosing a treatment strategy for IgG4-RD. [ABSTRACT FROM AUTHOR]

Objectives: This study aims to clarify the relationship between the changes of pancreatic size after glucocorticoid (GC) therapy and relapse in IgG4-related autoimmune pancreatitis (AIP). Methods: We prospectively enrolled 205 newly diagnosed IgG4-related AIP patients. 145 patients were followed up for more than 3 years. These patients were divided into three groups according to the changes of pancreatic size after treatment of 6 months: pancreatic swelling, normal size, and pancreatic atrophy. Baseline clinical and laboratory parameters were compared among three groups. Kaplan–Meier survival analysis was performed in the 134 patients based on GC therapy. Besides, Cox regression analysis and logistic regression analysis were performed to identify risk factors associated with relapse and the potential variables affecting changes of pancreatic size after treatment. Results: Age at diagnosis, white blood cell count, and serum IgG1 level at baseline were significantly different among the three groups. After treatment of 6 months, the pancreas of most patients (n = 81, 55.9%) could return to normal size, while persistent pancreatic swelling was found in 24.1% patients (n = 35), and atrophy was observed in 20.0% of the patients (n = 29). Kaplan–Meier survival analysis presented patients with pancreatic swelling after 6 months of GC therapy were more likely to relapse in the follow-up of 3 years. Persistent pancreatic swelling after treatment and salivary gland involvement at baseline were independent risk variables associated with relapse in IgG4-related AIP patients, while GC-based therapy was a protective factor of relapse. Logistic regression analysis revealed that older age at diagnosis was associated with pancreatic atrophy and higher baseline serum IgG1 level was associated with pancreatic swelling after treatment of 6 months. Conclusions: Patients with persistent pancreatic swelling after GC-based therapy of 6 months were more likely to relapse in the follow-up of 3 years. Older age at diagnosis and higher baseline serum IgG1 level were potential variables associated with pancreatic atrophy or swelling after treatment of 6 months. Key Points • Patients with persistent pancreatic swelling after glucocorticoid-based therapy were more likely to relapse in IgG4-related autoimmune pancreatitis. • Older age at diagnosis was associated with pancreatic atrophy after glucocorticoid-based therapy. • Higher baseline serum IgG1 level was associated pancreatic swelling after glucocorticoid-based therapy. [ABSTRACT FROM AUTHOR]

The presence or absence of anti-citrullinated peptide antibodies (ACPA) and associated disparities in patients with rheumatoid arthritis (RA) implies disease heterogeneity with unknown diverse immunopathological mechanisms. Here we profile CD45+ hematopoietic cells from peripheral blood or synovial tissues from both ACPA+ and ACPA- RA patients by single-cell RNA sequencing and identify subsets of immune cells that contribute to the pathogenesis of RA subtypes. We find several synovial immune cell abnormalities, including up-regulation of CCL13, CCL18 and MMP3 in myeloid cell subsets of ACPA- RA compared with ACPA+ RA. Also evident is a lack of HLA-DRB5 expression and lower expression of cytotoxic and exhaustion related genes in the synovial tissues of patients with ACPA- RA. Furthermore, the HLA-DR15 haplotype (DRB1/DRB5) conveys an increased risk of developing active disease in ACPA+ RA in a large cohort of patients with treatment-naive RA. Immunohistochemical staining shows increased infiltration of CCL13 and CCL18-expressing immune cells in synovial tissues of ACPA- RA. Collectively, our data provide evidence of the differential involvement of cellular and molecular pathways involved in the pathogenesis of seropositive and seronegative RA subtypes and reveal the importance of precision therapy based on ACPA status. Patients with rheumatoid arthritis are commonly stratified by ACPA serology, with positivity being associated with more severe disease and joint destruction. Here the authors present a single cell RNA sequencing resource comparing peripheral blood and synovial tissue cells from patients with ACPA+ versus ACPA- rheumatoid arthritis. [ABSTRACT FROM AUTHOR]

Objectives: To analyze the clinical and laboratory features of primary biliary cholangitis (PBC) patients complicated with cancer, and explore the potential factors associated with cancer. Methods: We consecutively enrolled PBC patients from January 2002 to February 2016 in Peking Union Medical College Hospital and performed a structured interview, systemic rheumatologic evaluation, and laboratory tests. The risk factors associated with cancer were analyzed with univariate and multivariable logistic regression and proportional hazard model. Results: Among the 580 PBC patients enrolled, 51 cancers were identified in 51 patients (8.8%), including 45 (88.2%) solid tumors and 6 (11.8%) hematologic malignancies. Patients with cancer were older (62.1 ± 9.6 vs. 55.4 ± 11.6 years, p < 0.01) than patients without cancer. Additionally, positive anti-centromere antibody (ACA) was more frequently observed in patients without cancer (25.9% vs 4.3%, p = 0.019) compared with patients with cancer diagnosed after establishing PBC. The median follow-up after the diagnosis of PBC was 4 years (IQR 2.0–6.6). Furthermore, multivariable logistic regression confirmed that older age was associated with cancer in PBC patients (odds ratio (OR) = 1.045, 95% confidence interval (CI): 1.006–1.085), and positive ACA was a protective factor (OR = 0.116, 95% CI: 0.015–0.876). Additionally, proportional hazard model analysis revealed that age was a risk factor (hazard ratio = 1.045, 95% CI: 1.012–1.080), and positive ACA was a protective factor (hazard ratio = 0.232, 95% CI: 0.055–0.977) for cancer. Conclusions: Both solid tumor and hematologic malignancy were prevalent in PBC patients. Older age was associated with cancer, and positive ACA was a protective factor of cancer in PBC patients. Key Points • Patients with PBC could present with both solid tumors and hematologic malignancies. • Multivariable logistic regression and proportional hazard model analysis revealed that age was a risk factor as we know, and positive ACA was a protective factor. [ABSTRACT FROM AUTHOR]

Mouth and genital ulcer with inflamed cartilage syndrome (MAGIC syndrome) is a rare autoinflammatory disorder with unknown etiology. Except for the common clinical manifestations mimicking Behçet's disease and relapsing polychondritis, some other clinical entities are occasionally observed. In this report, we present a case in which a patient developed anaplastic large cell lymphoma 1 year after the diagnosis of MAGIC syndrome. Additionally, we review the clinical manifestations, management, and prognosis of MAGIC syndrome. [ABSTRACT FROM AUTHOR]

Systemic lupus erythematosus (SLE) is a highly heterogeneous disease affecting multiple organs and is characterized by an aberrant immune response. Although the mortality of SLE has decreased significantly since the application of glucocorticoids, severe or refractory SLE can potentially cause irreversible organ damage and contribute to the disease morbidity and mortality. Early recognition of severe SLE or life-threatening conditions is of great challenge to clinicians since the onset symptoms can be rapid and aggressive, involving the visceral organs of the neuropsychiatric, gastrointestinal, hematologic, renal, pulmonary, and cardiovascular systems, etc. Additionally, SLE patients with specific comorbidities and detrimental complications could lead to a clinical dilemma and contribute to poor prognosis. Prompt and adequate treatment for severe refractory SLE is crucial for a better prognosis. However, as evidence from well-designed randomized controlled trials is limited, this review aims to provide real-world evidence based on cohort studies from Peking Union Medical College Hospital, the national tertiary referral center in China, together with the literature, on clinical characteristics, risks and prognostic factors, and treatment strategies for severe and/or refractory SLE. [ABSTRACT FROM AUTHOR]

This prospective cohort study aims to investigate the incidence, related factors and prognosis of IgG4-related disease (IgG4-RD) with malignancies in the Chinese cohort. We prospectively analyzed the IgG4-RD patients recruited in Peking Union Medical College Hospital from January 2011 to August 2018 and identified patients diagnosed with IgG4-RD complicating malignancies. Data regarding demographics, clinical features, treatment and prognosis of IgG4-RD patients complicating malignancies were collected and compared to those of age- and sex-matched controls. Among the 587 Chinese patients with IgG4-RD, 17 malignancies were identified. Ten of them developed malignancy after the diagnosis of IgG4-RD, given a standard incidence ratio (SIR) of 2.78 (95%CI 1.33–5.12). Multivariate logistic analysis indicated that autoimmune pancreatitis (OR = 6.230, 95%CI 1.559–24.907, p = 0.010) was positively associated with malignancy, whereas eosinophilia (OR = 0.094, 95%CI 0.010–0.883, p = 0.039) was negatively related with malignancies. During a median follow-up period of 61.4 ± 26.4 months, all patients with IgG4-RD and malignancies survived. We conclude that an increased incidence of malignancy was found in Chinese IgG4-RD cohort. Autoimmune pancreatitis is a potential risk factor, whereas eosinophilia is a possible protective factor for complicating malignancies. [ABSTRACT FROM AUTHOR]

IgG4-related disease (IgG4-RD) and Castleman’s disease (CD) share similar clinical manifestations. When the histopathology coincides with the diagnosis of both IgG4-RD and CD, it is hard to depart the two disease entities utterly; here we call it IgG4-CD provisionally. In this study, we aim to review the clinical features of IgG4-CD. This study is based on a retrospective analysis of a prospectively acquired database. IgG4-CD was defined histopathologically in patients who fulfilled the diagnosis of both IgG4-RD and CD. Forty-five definite IgG4-RD and 16 multicentric CD (MCD) patients were recruited as controls. Clinical features including organ involvement, serum IgG4, IgG, IgE, ESR, CRP, and IL-6 levels were collected and analyzed. Fifteen patients (2.8%) out of 534 patients with IgG4-RD in China’s largest prospective IgG4-RD and Mimicry cohort fulfilled the definition of IgG4-CD. There were 14 males and 1 female, whose mean age was 47 ± 18 years old, and the median disease duration before diagnosis was 12 (1-132) months. Eight patients have allergic disease history. IgG4-CD patients had more lymph node involvement (100 vs 57.8%, P < 0.01), while IgG4-RD patients had more submandibular (33.3 vs 77.1%, P < 0.01) and parotid gland (13.3 vs 40.9%, P < 0.05) affected. IgG4-CD patients had significantly higher levels of ESR, CRP, IgG, IgG1, IgG3, IgG4, and IgE than IgG4-RD patients. Compared with MCD patients, IgG4-CD patients showed higher incidence of salivary gland and paranasal sinus involvement, higher hemoglobin, eosinophil count, serum IgG4 level and IgG4/IgG ratio, and lower CRP, IL-6 levels, and IgG1/IgG, IgG2/IgG ratio. All patients with IgG4-CD exhibited relatively favorable outcomes. Both IgG4-RD and CD can involve multiple organs. There are a small group of patients who had clinical and pathological characteristics of both CD and IgG4-RD showed better clinical outcome. In the long-term prognosis of these patients, the relationship of CD and IgG4-RD is waiting to be further elucidated. [ABSTRACT FROM AUTHOR]

Monocytes are an important component in the innate immune system. However, studies to date have failed to conclude whether their levels are altered in patients with systemic lupus erythematosus (SLE). We applied the cytodiff counting method and comprehensively measured the circulating levels of distinct white blood cell (WBC) subsets, including CD16+, CD16−, and total monocytes, in 61 SLE patients as well as in 203 age-matched healthy controls (HCs). The absolute number of CD16− monocytes, total monocytes, immature granulocytes, mature neutrophils, total neutrophils, and T cell blasts was significantly higher, that of non-cytotoxic T lymphocytes, cytotoxic T + NK lymphocytes, T + NK lymphocytes, total lymphocytes, basophils, and eosinophils significantly lower (all p < 0.05), but that of CD16+ monocytes, B lymphocytes, B cell blasts, non-B and non-T cell blasts, and total blasts was not statistically different in SLE patients, as compared to HC. Specifically, among all subsets examined, the percentage of CD16− monocytes and total monocytes was the only one that could discriminate active SLE from quiescent SLE ( p = 0.033 and 0.026, respectively). SLE patients with lupus nephritis were also associated with higher levels of circulating CD16− monocytes and total monocytes, in comparison with that of controls (both p < 0.0001). This study suggests the significance of distinct WBC subsets, particularly the differential regulations of monocyte subsets, in the pathogenesis and development of SLE. [ABSTRACT FROM AUTHOR]

The aims of our study are to observe the pregnancy outcome of anti-Sjögren's syndrome-related antigen A (SSA)/Ro-positive women and to predict the risk factors for adverse pregnancy outcome and neonatal lupus erythematosus (NLE). Clinical data of 126 anti-SSA/Ro-positive patients with 140 pregnancies were evaluated retrospectively, and the newborns were followed up as a cohort in 3 months. χ test or logistic regression was used to predict the risk factors of lupus flares during pregnancy, fetal loss, and NLE. Twenty-six out of 93 pregnancies with systemic lupus erythematosus (SLE) experienced flares during pregnancy. Active disease prior to conception was the independent risk factor for flares [ P = 0.002, odds ratio (OR) = 10.41 (95 % confidence interval (CI) = 2.34∼46.26)]. Continuous use of steroids and hydroxychloroquine (HCQ) might help decrease the risk ( P = 0.041 and 0.015, respectively). Eleven out of 140 pregnancies ended with fetal loss, and 9 out of 113 live births were diagnosed with NLE. The presence of anti-phospholipid syndrome (APS) was associated with fetal loss ( P = 0.018, OR = 6.41 (95 % CI = 1.57-26.14)). The presence of anti-Sjögren's syndrome-related antigen B (SSB)/La antibodies tended to increase the risk of giving birth to an infant with NLE ( P = 0.140); on the other hand, duration of disease, history of renal involvement, and active SLE during pregnancy did not contribute to the incidence of NLE ( P = 0.649, 0.685, and 1.000, respectively). Active disease without regular follow-up before conception significantly increased the risk of lupus flares during pregnancy. The continuous use of low-dose steroids and hydroxychloroquine might help maintain lower SLE activity. Concurrent APS instead of high titer of anti-SSA/Ro might raise the risk of fetal loss in anti-SSA/Ro-positive patients. [ABSTRACT FROM AUTHOR]

The aim of this study is to investigate the correlation between clinical parameter, Th17, and degree of lymphocytes infiltrating in labial salivary gland in primary Sjögren's syndrome (pSS). Minor labial gland biopsies from a cohort of 103 patients fulfilling the American-European consensus classification criteria for pSS were examined and grouped into G1 to G3 according to infiltration degree of lymphocytes in labial salivary glands and formation of germinal center. IL-17 level and Th17 proportion of peripheral blood samples in 54 patients with pSS were analyzed simultaneously. Among the 103 labial salivary glands samples, there were 10.5 % of G1, 68.4 % of G2, and 21.1 % of G3, respectively. Some clinical parameters were markedly associated with the degree of inflammatory cells infiltration in labial glands, including white blood cell counts, lymphocytes, liver enzymes, and pulmonary function diffusion rates. The average optical density of IL-17 correlated with the severity of lymphocytic infiltration in the labial glands, while the proportion of Th17 cells in the peripheral blood mononuclear cells showed no significant relationship to the degree of lymphocytic infiltration in the labial glands from the SS patients. IL-17A mRNA levels in peripheral blood mononuclear cells from pSS patients before immunosuppressant treatment were significantly higher than that in patients after immunosuppressant treatment. The degree of inflammatory cells infiltration in labial glands was related to some clinical manifestations in pSS. IL-17 may play a role in the pathogenesis of lymphocytic infiltration in the labial glands. Immunosuppressive treatment might affect Th17 cells. [ABSTRACT FROM AUTHOR]

Different causes of mortality have been described over different decades followed by description of pathogens identified from infective episodes that led to death. A retrospective review was performed in 3,831 hospitalized systemic lupus erythematosus (SLE) patients in Peking Union Medical College Hospital from January 1986 to April 2012. The primary causes of death were identified, and the constituent ratio of specific death causes during different periods was compared. Among 3,831 hospitalized SLE patients, 268 patients died, accounting for 7.0 %. No significant difference of death rate was found between men and women, P = 0.404. The three most frequent death causes according to decade were as follows: for 1986-1995, renal involvement, lupus encephalopathy, and infections; for 1996-2005, infections, lupus encephalopathy, and renal involvement; and for 2006-2012, infections, lupus encephalopathy, and pulmonary hypertension. Certain types of deaths, primarily related to lupus activity, have decreased over time, whereas infections, often attributed to the use of corticosteroid and immunosuppressant medications, have increased gradually and changed to the most frequent death causes of SLE. Early mortality (<3 years) occurred more commonly in lupus encephalopathy, while late death (>3 years) happened more frequently in renal involvement, pulmonary artery hypertension, cardiovascular events, and cancer. In SLE death cases mainly dying from infection, mixed infections were more frequent than single pathogen infection (60.5 vs. 39.5 %), including common bacteria, fungal infection, and cytomegalovirus. Aspergillus fumigatus and Pneumocystis carinii were the two most commonly infected pathogens, and Cytomegalovirus was a frequent pathogen of mixed infection. Aggressive therapy has effectively reduced the mortality related to disease activity but also was associated with life-threatening infections. Mixed and fungal infection should be considered when SLE patients have severe infection. [ABSTRACT FROM AUTHOR]

Behçet's disease (BD) is a multi-systemic inflammatory disorder which can affect all types and sizes of blood vessels. This study aims to evaluate the prevalence and characteristics of vascular involvement in BD. Among 796 patients diagnosed with BD, 102 patients (81 male, 21 female) with vascular involvement were included, whose detailed clinical characteristics were recorded. The diagnosis of vascular lesions was made on clinical signs, by Doppler ultrasonography, and/or angiography using computed tomographic or magnetic resonance techniques. Vascular involvement occurred in 12.8 % of BD patients. Male to female ratio was 3.86:1. Mean age at onset of vascular involvement was 29.5 ± 11.3 years. Vascular lesion was the initial sign of BD in 28 patients, accounting for 27.5 %. Of 102 BD patients with vascular involvement, 72 had venous lesions (70.6 %) and 56 had arterial lesions (54.9 %), among which 26 (25.5 %) patients had both venous and arterial involvements. Female BD patients were more often involved with arterial lesions, whereas male BD patients developed venous lesions more often than females, P = 0.000. The most common type of vascular involvement was deep venous thrombosis in lower extremities ( n = 49), other affected venous sites including inferior vena cava, superior vena cava, and cerebral venous. The prominent type of arterial lesions was dilatation ( n = 25, including 24 cases of aneurysms); other types included eight cases of occlusion and 23 cases of stenosis. The main locations of arterial lesions were the aorta ( n = 19), lower extremity arteries ( n = 15), pulmonary arteries ( n = 13), coronary arteries ( n = 5), and subclavian arteries ( n = 5). Compared with those without vascular lesions, ocular involvement, genital ulcers, and arthritis were significantly less frequent among patients with vasculo-BD (23.5 vs 35.2 %, P = 0.024; 54.9 vs 76.5 %, P = 0.000; 19.6 vs 30.5 %, P = 0.026), whereas a higher frequency of cardiac involvement was found in vasculo-BD patients (20.6 vs 3.6 %, P = 0.000). Vascular involvement is a complication in BD patients. This study illustrated that venous lesions are more frequently involved than arterial lesions. Vascular lesions correlated with a high frequency of cardiac involvement and a low incidence of ocular lesions, genital ulcers, and arthritis. [ABSTRACT FROM AUTHOR]

The aim of this study was to elucidate the expression profile and the potential role of long non-coding RNA (LncRNA) in the peripheral blood mononuclear cells of primary Sjögren's syndrome (pSS) patients. RNA-seq technology was used to detect the differentially expressed LncRNAs and mRNAs between five age-and sex-matched paired pSS patients and healthy control PBMCs. The selected LncRNAs were detected in the validation study by RT-qPCR in 16 paired pSS patients and healthy controls. The GO, KEGG, co-localization, and co-expression analysis were performed to enrich the potential gene functions and pathways. In this study, 44 out of 1772 LncRNAs and 1034 out of 15,424 mRNAs were expressed differentially in the PBMCs of pSS patients. LINC00426, TPTEP1-202, CYTOR, NRIR, and BISPR were validated as aberrantly expressed, and these LncRNAs strongly correlated with disease activity of pSS. GO and KEGG pathway analysis revealed the significant enrichment of biological processes, cellular components, and molecular function of the up and down-regulated mRNAs, which were mainly concentrated in the immune response and immune system processes. Co-localization and co-expression analysis also revealed that differentially expressed LncRNAs in the PBMCs of pSS were strongly correlated to the mRNA functioning associated with immune response and cell metastasis. Numerous LncRNAs and mRNAs were found differentially expressed in the PBMCs of pSS patients, especially NRIR and BISPR; they interacted with the co-localized and co-expressed mRNAs, which might participate in the pathogenesis of pSS through the NF-κB, JAK-STAT, and other signaling pathways that regulate cell metastasis. [ABSTRACT FROM AUTHOR]

It has been reported that patients with IgG4-related disease (IgG4-RD) showed an elevated incidence of eosinophilia. We aim to explore the clinical patterns of IgG4-RD patients with and without eosinophilia. Four hundred and twenty-five IgG4-RD patients referred to Peking Union Medical College Hospital were enrolled. Blood eosinophil count higher than 0.5 × 109/L was defined as eosinophilia. Clinical features of all the participants were collected and analyzed statistically. Eighty-seven patients (20%) with eosinophilia were found. As compared to those with a normal range of blood eosinophil count, male predominance, longer disease duration, increased prevalence of dacryoadenitis, sialadenitis, lymphadenopathy, and skin rash, higher IgG4-RD responder index, more organ involvement and higher levels of serum IgG4 (17.0 g/L vs 6.5 g/L, P < 0.001) was found in patients with eosinophilia. There was no significant difference in the incidence of allergic disease between the two groups. Peripheral eosinophil counts were positively correlated with disease duration, the number of involved organs, IgG4-RD responder index, and serum IgG4. Higher recurrence rate during follow-up period was found in patients with eosinophilia [28.6% (20/70) vs 17.1% (42/245), P = 0.034]. IgG4-RD patients with eosinophilia exhibited different clinical patterns from patients without. Eosinophilia appeared independent of allergies in IgG4-RD. [ABSTRACT FROM AUTHOR]