Sabine Arve-Butler, Tobias Schmidt, Anki Mossberg, Elisabet Berthold, Birgitta Gullstrand, Anders A Bengtsson, Fredrik Kahn, Robin Kahn
{"title":"Synovial fluid neutrophils in oligoarticular juvenile idiopathic arthritis have an altered phenotype and impaired effector functions.","authors":"Sabine Arve-Butler, Tobias Schmidt, Anki Mossberg, Elisabet Berthold, Birgitta Gullstrand, Anders A Bengtsson, Fredrik Kahn, Robin Kahn","doi":"10.1186/s13075-021-02483-1","DOIUrl":"https://doi.org/10.1186/s13075-021-02483-1","url":null,"abstract":"<p><strong>Background: </strong>Neutrophils are the most prevalent immune cells in the synovial fluid in inflamed joints of children with oligoarticular juvenile idiopathic arthritis (JIA). Despite this, little is known about neutrophil function at the site of inflammation in JIA and how local neutrophils contribute to disease pathogenesis. This study aimed to characterize the phenotype and function of synovial fluid neutrophils in oligoarticular JIA.</p><p><strong>Methods: </strong>Neutrophils obtained from paired blood and synovial fluid from patients with active oligoarticular JIA were investigated phenotypically (n = 17) and functionally (phagocytosis and oxidative burst, n = 13) by flow cytometry. In a subset of patients (n = 6), blood samples were also obtained during inactive disease at a follow-up visit. The presence of CD206-expressing neutrophils was investigated in synovial biopsies from four patients by immunofluorescence.</p><p><strong>Results: </strong>Neutrophils in synovial fluid had an activated phenotype, characterized by increased CD66b and CD11b levels, and most neutrophils had a CD16<sup>hi</sup> CD62L<sup>low</sup>aged phenotype. A large proportion of the synovial fluid neutrophils expressed CD206, a mannose receptor not commonly expressed by neutrophils but by monocytes, macrophages, and dendritic cells. CD206-expressing neutrophils were also found in synovial tissue biopsies. The synovial fluid neutrophil phenotype was not dependent on transmigration alone. Functionally, synovial fluid neutrophils had reduced phagocytic capacity and a trend towards impaired oxidative burst compared to blood neutrophils. In addition, the effector functions of the synovial fluid neutrophils correlated negatively with the proportion of CD206<sup>+</sup> neutrophils.</p><p><strong>Conclusions: </strong>Neutrophils in the inflamed joint in oligoarticular JIA were altered, both regarding phenotype and function. Neutrophils in the synovial fluid were activated, had an aged phenotype, had gained monocyte-like features, and had impaired phagocytic capacity. The impairment in phagocytosis and oxidative burst was associated with the phenotype shift. We speculate that these neutrophil alterations might play a role in the sustained joint inflammation seen in JIA.</p>","PeriodicalId":51225,"journal":{"name":"Arthritis Research & Therapy","volume":"23 1","pages":"109"},"PeriodicalIF":4.9,"publicationDate":"2021-04-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s13075-021-02483-1","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"25576183","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Keno K Bressem, Janis L Vahldiek, Lisa Adams, Stefan Markus Niehues, Hildrun Haibel, Valeria Rios Rodriguez, Murat Torgutalp, Mikhail Protopopov, Fabian Proft, Judith Rademacher, Joachim Sieper, Martin Rudwaleit, Bernd Hamm, Marcus R Makowski, Kay-Geert Hermann, Denis Poddubnyy
{"title":"Deep learning for detection of radiographic sacroiliitis: achieving expert-level performance.","authors":"Keno K Bressem, Janis L Vahldiek, Lisa Adams, Stefan Markus Niehues, Hildrun Haibel, Valeria Rios Rodriguez, Murat Torgutalp, Mikhail Protopopov, Fabian Proft, Judith Rademacher, Joachim Sieper, Martin Rudwaleit, Bernd Hamm, Marcus R Makowski, Kay-Geert Hermann, Denis Poddubnyy","doi":"10.1186/s13075-021-02484-0","DOIUrl":"10.1186/s13075-021-02484-0","url":null,"abstract":"<p><strong>Background: </strong>Radiographs of the sacroiliac joints are commonly used for the diagnosis and classification of axial spondyloarthritis. The aim of this study was to develop and validate an artificial neural network for the detection of definite radiographic sacroiliitis as a manifestation of axial spondyloarthritis (axSpA).</p><p><strong>Methods: </strong>Conventional radiographs of the sacroiliac joints obtained in two independent studies of patients with axSpA were used. The first cohort comprised 1553 radiographs and was split into training (n = 1324) and validation (n = 229) sets. The second cohort comprised 458 radiographs and was used as an independent test dataset. All radiographs were assessed in a central reading session, and the final decision on the presence or absence of definite radiographic sacroiliitis was used as a reference. The performance of the neural network was evaluated by calculating areas under the receiver operating characteristic curves (AUCs) as well as sensitivity and specificity. Cohen's kappa and the absolute agreement were used to assess the agreement between the neural network and the human readers.</p><p><strong>Results: </strong>The neural network achieved an excellent performance in the detection of definite radiographic sacroiliitis with an AUC of 0.97 and 0.94 for the validation and test datasets, respectively. Sensitivity and specificity for the cut-off weighting both measurements equally were 88% and 95% for the validation and 92% and 81% for the test set. The Cohen's kappa between the neural network and the reference judgements were 0.79 and 0.72 for the validation and test sets with an absolute agreement of 90% and 88%, respectively.</p><p><strong>Conclusion: </strong>Deep artificial neural networks enable the accurate detection of definite radiographic sacroiliitis relevant for the diagnosis and classification of axSpA.</p>","PeriodicalId":51225,"journal":{"name":"Arthritis Research & Therapy","volume":"23 1","pages":"106"},"PeriodicalIF":4.9,"publicationDate":"2021-04-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8028815/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"25572263","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Yoshinari Matsumoto, Nitin Shivappa, Yuko Sugioka, Masahiro Tada, Tadashi Okano, Kenji Mamoto, Kentaro Inui, Daiki Habu, James R Hebert, Tatsuya Koike
{"title":"Change in dietary inflammatory index score is associated with control of long-term rheumatoid arthritis disease activity in a Japanese cohort: the TOMORROW study.","authors":"Yoshinari Matsumoto, Nitin Shivappa, Yuko Sugioka, Masahiro Tada, Tadashi Okano, Kenji Mamoto, Kentaro Inui, Daiki Habu, James R Hebert, Tatsuya Koike","doi":"10.1186/s13075-021-02478-y","DOIUrl":"https://doi.org/10.1186/s13075-021-02478-y","url":null,"abstract":"<p><strong>Background: </strong>The dietary inflammatory index (DII®), a quantitative measure of the inflammatory potential of daily food and nutrient intake, and associations between a variety of health outcomes have been reported. However, the association between DII score and disease activity of rheumatoid arthritis (RA) is unclear. Therefore, this study was designed to test whether higher DII score contributes to disease activity and as a corollary, whether reducing DII score helps to achieve or maintain low disease activity or remission in patients with RA.</p><p><strong>Methods: </strong>We performed a cross-sectional and longitudinal analysis using 6 years of data (from 2011 to 2017) in TOMORROW, a cohort study consisting of 208 RA patients and 205 gender- and age-matched controls started in 2010. Disease activity of RA patients was assessed annually using DAS28-ESR (disease activity score 28 joints and the erythrocyte sedimentation rate) as a composite measure based on arthritic symptoms in 28 joints plus global health assessment and ESR. Dietary data were collected in 2011 and 2017 using the brief-type self-administered diet history questionnaire (BDHQ). Energy-adjusted DII (E-DII™) score was calculated using 26 nutrients derived from the BDHQ. Data were analyzed with two-group comparisons, correlation analysis, and multivariable logistic regression analysis.</p><p><strong>Results: </strong>One hundred and seventy-seven RA patients and 183 controls, for whom clinical and dietary survey data were available, were analyzed. RA patients had significantly higher E-DII (pro-inflammatory) score compared to controls both in 2011 and 2017 (p < 0.05). In RA patients, E-DII score was not a factor associated with significant change in disease activity. However, anti-inflammatory change in E-DII score was associated maintaining low disease activity (DAS28-ESR ≤ 3.2) or less for 6 years (OR 3.46, 95% CI 0.33-8.98, p = 0.011).</p><p><strong>Conclusions: </strong>The diets of RA patients had a higher inflammatory potential than controls. Although E-DII score was not a factor associated with significant disease activity change, anti-inflammatory change in E-DII score appeared to be associated with maintaining low disease activity in patients with RA.</p><p><strong>Trial registration: </strong>UMIN Clinical Trials Registry, UMIN000003876 . Registered 7 Aug 2010-retrospectively registered.</p>","PeriodicalId":51225,"journal":{"name":"Arthritis Research & Therapy","volume":"23 1","pages":"105"},"PeriodicalIF":4.9,"publicationDate":"2021-04-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s13075-021-02478-y","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"25573772","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Mukundan Attur, Xin Duan, Lei Cai, Tianzhen Han, Weili Zhang, Eric D Tycksen, Jonathan Samuels, Robert H Brophy, Steven B Abramson, Muhammad Farooq Rai
{"title":"Periostin loss-of-function protects mice from post-traumatic and age-related osteoarthritis.","authors":"Mukundan Attur, Xin Duan, Lei Cai, Tianzhen Han, Weili Zhang, Eric D Tycksen, Jonathan Samuels, Robert H Brophy, Steven B Abramson, Muhammad Farooq Rai","doi":"10.1186/s13075-021-02477-z","DOIUrl":"https://doi.org/10.1186/s13075-021-02477-z","url":null,"abstract":"<p><strong>Background: </strong>Elevated levels of periostin (Postn) in the cartilage and bone are associated with osteoarthritis (OA). However, it remains unknown whether Postn loss-of-function can delay or prevent the development of OA. In this study, we sought to better understand the role of Postn in OA development and assessed the functional impact of Postn deficiency on post-traumatic and age-related OA in mice.</p><p><strong>Methods: </strong>The effects of Postn deficiency were studied in two murine experimental OA models using Postn<sup>-/-</sup> (n = 32) and littermate wild-type (wt) mice (n = 36). Post-traumatic OA was induced by destabilization of the medial meniscus (DMM) in 10-week-old mice (n = 20); age-related OA was analyzed in 24-month-old mice (n = 13). Cartilage degeneration was assessed histologically using the OARSI scoring system, and synovitis was evaluated by measuring the synovial lining cell layer and the cells density in the synovial stroma. Bone changes were measured by μCT analysis. Serum levels of Postn were determined by ELISA. Expression of Postn and collagenase-3 (MMP-13) was measured by immunostaining. RNA-seq was performed on chondrocytes isolated from 21-day old Postn<sup>-/-</sup> (n = 3) and wt mice (n = 3) to discover genes and pathways altered by Postn knockout.</p><p><strong>Results: </strong>Postn<sup>-/-</sup> mice exhibited significantly reduced cartilage degeneration and OARSI score relative to wt mice in post-traumatic OA after 8 weeks (maximum: 2.37 ± 0.74 vs. 4.00 ± 1.20, P = 0.011; summed: 9.31 ± 2.52 vs. 21.44 ± 6.01, P = 0.0002) and spontaneous OA (maximum: 1.93 ± 0.45 vs. 3.58 ± 1.16, P = 0.014; summed: 6.14 ± 1.57 vs. 11.50 ± 3.02, P = 0.003). Synovitis was significantly lower in Postn<sup>-/-</sup> mice than wt only in the DMM model (1.88 ± 1.01 vs. 3.17 ± 0.63; P = 0.039). Postn<sup>-/-</sup> mice also showed lower trabecular bone parameters such as BV/TV, vBMD, Tb.Th, and Tb.N and high Tb. Sp in both models. Postn<sup>-/-</sup> mice had negligible levels of serum Postn compared with wt. Immunofluorescent studies of cartilage indicated that Postn<sup>-/-</sup> mice expressed lower MMP-13 levels than wt mice. RNA-seq revealed that cell-cell-adhesion and cell-differentiation processes were enriched in Postn<sup>-/-</sup> mice, while those related to cell-cycle and DNA-repair were enriched in wt mice.</p><p><strong>Conclusions: </strong>Postn deficiency protects against DMM-induced post-traumatic and age-related spontaneous OA. RNA-seq findings warrant further investigations to better understand the mechanistic role of Postn and its potential as a therapeutic target in OA.</p>","PeriodicalId":51225,"journal":{"name":"Arthritis Research & Therapy","volume":"23 1","pages":"104"},"PeriodicalIF":4.9,"publicationDate":"2021-04-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s13075-021-02477-z","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"25573774","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Shingo Ishihara, Alia M Obeidat, David L Wokosin, Dongjun Ren, Richard J Miller, Anne-Marie Malfait, Rachel E Miller
{"title":"The role of intra-articular neuronal CCR2 receptors in knee joint pain associated with experimental osteoarthritis in mice.","authors":"Shingo Ishihara, Alia M Obeidat, David L Wokosin, Dongjun Ren, Richard J Miller, Anne-Marie Malfait, Rachel E Miller","doi":"10.1186/s13075-021-02486-y","DOIUrl":"https://doi.org/10.1186/s13075-021-02486-y","url":null,"abstract":"<p><strong>Background: </strong>C-C chemokine receptor 2 (CCR2) signaling plays a key role in pain associated with experimental murine osteoarthritis (OA) after destabilization of the medial meniscus (DMM). Here, we aimed to assess if CCR2 expressed by intra-articular sensory neurons contributes to knee hyperalgesia in the early stages of the model.</p><p><strong>Methods: </strong>DMM surgery was performed in the right knee of 10-week-old male wild-type (WT), Ccr2 null, or Ccr2<sup>RFP</sup> C57BL/6 mice. Knee hyperalgesia was measured using a Pressure Application Measurement device. CCR2 receptor antagonist (CCR2RA) was injected systemically (i.p.) or intra-articularly (i.a.) at different times after DMM to test its ability to reverse knee hyperalgesia. In vivo Ca<sup>2+</sup> imaging of the dorsal root ganglion (DRG) was performed to assess sensory neuron responses to CCL2 injected into the knee joint cavity. CCL2 protein in the knee was measured by ELISA. Ccr2<sup>RFP</sup> mice and immunohistochemical staining for the pan-neuronal marker, protein gene product 9.5 (PGP9.5), or the sensory neuron marker, calcitonin gene-related peptide (CGRP), were used to visualize the location of CCR2 on intra-articular afferents.</p><p><strong>Results: </strong>WT, but not Ccr2 null, mice displayed knee hyperalgesia 2-16 weeks after DMM. CCR2RA administered i.p. alleviated established hyperalgesia in WT mice 4 and 8 weeks after surgery. Intra-articular injection of CCL2 excited sensory neurons in the L4-DRG, as determined by in vivo calcium imaging; responses to CCL2 increased in mice 20 weeks after DMM. CCL2, but not vehicle, injected i.a. rapidly caused transient knee hyperalgesia in naïve WT, but not Ccr2 null, mice. Intra-articular CCR2RA injection also alleviated established hyperalgesia in WT mice 4 and 7 weeks after surgery. CCL2 protein was elevated in the knees of both WT and Ccr2 null mice 4 weeks after surgery. Co-expression of CCR2 and PGP9.5 as well as CCR2 and CGRP was observed in the lateral synovium of naïve mice; co-expression was also observed in the medial compartment of knees 8 weeks after DMM.</p><p><strong>Conclusions: </strong>The findings suggest that CCL2-CCR2 signaling locally in the joint contributes to knee hyperalgesia in experimental OA, and it is in part mediated through direct stimulation of CCR2 expressed by intra-articular sensory afferents.</p>","PeriodicalId":51225,"journal":{"name":"Arthritis Research & Therapy","volume":"23 1","pages":"103"},"PeriodicalIF":4.9,"publicationDate":"2021-04-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s13075-021-02486-y","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"25568233","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Clinical characteristics and outcome of IgG4-related disease with hypocomplementemia: a prospective cohort study.","authors":"Linyi Peng, Hui Lu, Jiaxin Zhou, Panpan Zhang, Jieqiong Li, Zheng Liu, Di Wu, Shangzhu Zhang, Yunjiao Yang, Wei Bai, Li Wang, Yunyun Fei, Wen Zhang, Yan Zhao, Xiaofeng Zeng, Fengchun Zhang","doi":"10.1186/s13075-021-02481-3","DOIUrl":"10.1186/s13075-021-02481-3","url":null,"abstract":"<p><strong>Background: </strong>Immunoglobulin G4-related disease (IgG4-RD) is a newly recognized systemic, immune-mediated, and fibro-inflammatory disease. Hypocomplementemia was found in part of IgG4-RD patients especially in the setting of active disease.</p><p><strong>Objectives: </strong>This study aimed to clarify the clinical features, treatment efficacy, and outcome in IgG4-RD patients with hypocomplementemia.</p><p><strong>Methods: </strong>312 IgG4-RD patients were recruited in our prospective cohort conducted in Peking Union Medical College Hospital. Patients were divided into hypocomplementemia group and normal complement group according to serum C3 and C4 levels measured at baseline before treatment. Low serum C3 levels (< 0.73 g/L) and/or C4 levels (< 0.10 g/L) were defined as hypocomplementemia. Demographic data, clinical characteristics, laboratory parameters, treatment, and outcome of two groups were analyzed and compared.</p><p><strong>Results: </strong>Hypocomplementemia was identified in 65 (20.8%) cases of untreated IgG4-RD patients at baseline. The average age of hypocomplementemia group was 55.85 ± 10.89 years, with male predominance (72.3%). Compared with normal complement group, patients with hypocomplementemia were likely to have more involved organs, higher IgG4-RD responder index (IgG4-RD RI), and higher laboratory parameters such as counts of eosinophils, inflammatory markers, immunoglobulin G (IgG), IgG1, IgG3, IgG4, and IgE. In addition, lymph nodes, lacrimal gland, submandibular gland, parotid gland, paranasal sinus, bile ducts, and prostate gland were more commonly affected (p < 0.05). Serum C3 and C4 showed a significant positively correlation with each other. Both C3 and C4 were negatively correlated with the number of involved organs, IgG, IgG3, IgG4, and IgG4-RD RI, as well as positively correlated with IgA and hypersensitive C reactive protein (hsCRP). 64 (98.5%) patients responded quickly to initial therapy at a 3-month follow-up. Fifteen (23.1%) patients relapsed during follow-up with mean recurrence time of 14.2 ± 13.8 months. Compared with normal complement group, there was no significant difference of relapse rate in two groups (P = 0.401).</p><p><strong>Conclusions: </strong>Clinical characteristics of IgG4-related disease with hypocomplementemia differ from normal complement group. Serum C3 and C4 at baseline before treatment could be biological markers for disease activity. IgG4-RD with hypocomplementemia responded well to treatment and had no significant difference of relapse rate in IgG4-RD with normal complement.</p>","PeriodicalId":51225,"journal":{"name":"Arthritis Research & Therapy","volume":"23 1","pages":"102"},"PeriodicalIF":4.9,"publicationDate":"2021-04-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8025345/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"25568237","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Bon San Koo, Kyu Yong Park, Hyun Jung Lee, Hyun Jung Kim, Hyeong Sik Ahn, Shin-Young Yim, Jae-Bum Jun
{"title":"Effect of combined pulmonary fibrosis and emphysema on patients with connective tissue diseases and systemic sclerosis: a systematic review and meta-analysis.","authors":"Bon San Koo, Kyu Yong Park, Hyun Jung Lee, Hyun Jung Kim, Hyeong Sik Ahn, Shin-Young Yim, Jae-Bum Jun","doi":"10.1186/s13075-021-02494-y","DOIUrl":"https://doi.org/10.1186/s13075-021-02494-y","url":null,"abstract":"<p><strong>Background: </strong>This study aimed to analyze the literature systematically to determine the clinical characteristics and prognosis of patients with connective tissue disease (CTD) with combined pulmonary fibrosis and emphysema (CPFE) compared to those of patients with CTD-interstitial lung disease (CTD-ILD) without emphysema.</p><p><strong>Methods: </strong>We searched MEDLINE, EMBASE, Cochrane Library, and KoreaMed for relevant articles published before July 2019. Studies meeting all the following criteria were included: (1) original research studies evaluating the effect of CPFE on CTD, (2) studies that compared patients with CTD-CPFE to those with CTD-ILD without emphysema, and (3) studies providing data on physical capacity, pulmonary function, or death in patients with CTD. Clinical characteristics of patients with CTD-CPFE were compared with those of patients with CTD-ILD without emphysema, and the influence of CPFE on physical capacity, pulmonary function, and death was analyzed.</p><p><strong>Results: </strong>Six studies between 2013 and 2019 were included. Two hundred ninety-nine (29.5%) and 715 (70.5%) patients had CTD-CPFE and CTD-ILD without emphysema, respectively. Regarding the type of CTD, 711 (68.3%) patients had systemic sclerosis, 263 (25.3%) rheumatoid arthritis, and 67 (6.4%) other CTDs. Patients with CTD-CPFE had a higher frequency of pulmonary hypertension and pulmonary fibrosis > 20% of the total lung volume, higher ratio of the forced vital capacity to the diffusion capacity of the lung for carbon monoxide (DLCO), lower arterial oxygen pressure at rest, and lower DLCO compared to those in patients with CTD-ILD without emphysema. In addition, more deaths occurred among those with CTD-CPFE (odds ratio, 2.95; 95% confidence interval, 1.75-4.96).</p><p><strong>Conclusion: </strong>CTD-CPFE is associated with worse physical and pulmonary function and more deaths compared to those in CTD-ILD without emphysema. These findings indicate the need for increased awareness and close monitoring of patients with CTD-CPFE.</p>","PeriodicalId":51225,"journal":{"name":"Arthritis Research & Therapy","volume":"23 1","pages":"100"},"PeriodicalIF":4.9,"publicationDate":"2021-04-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s13075-021-02494-y","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"25576088","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Emily Mirizio, Tracy Tabib, Xinjun Wang, Wei Chen, Christopher Liu, Robert Lafyatis, Heidi Jacobe, Kathryn S Torok
{"title":"Correction to: Single-cell transcriptome conservation in a comparative analysis of fresh and cryopreserved human skin tissue: pilot in localized scleroderma.","authors":"Emily Mirizio, Tracy Tabib, Xinjun Wang, Wei Chen, Christopher Liu, Robert Lafyatis, Heidi Jacobe, Kathryn S Torok","doi":"10.1186/s13075-021-02490-2","DOIUrl":"https://doi.org/10.1186/s13075-021-02490-2","url":null,"abstract":"","PeriodicalId":51225,"journal":{"name":"Arthritis Research & Therapy","volume":"23 1","pages":"101"},"PeriodicalIF":4.9,"publicationDate":"2021-04-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s13075-021-02490-2","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"25565290","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Clinical features and relapse risks of IgG4-related ophthalmic disease: a single-center experience in China.","authors":"Zhen Zhao, Dapeng Mou, Ziqiao Wang, Qiaozhu Zeng, Zhenfan Wang, Jimeng Xue, Limin Ren, Yanying Liu, Yin Su","doi":"10.1186/s13075-021-02489-9","DOIUrl":"https://doi.org/10.1186/s13075-021-02489-9","url":null,"abstract":"<p><strong>Background: </strong>IgG4-related ophthalmic disease (IgG4-ROD) is one of the phenotypes of IgG4-related disease (IgG4-RD), and its lesions are mainly located in the ocular. Currently, there are few studies on IgG4-ROD and no study has compared the phenotypic differences between IgG4-ROD and non IgG4-ROD (nIgG4-ROD). Thus, it is difficult to establish the optimal treatment strategy for IgG4-ROD. The aim of this study was to identify the disparities between the two groups and to clarify the risk factors for IgG4-ROD relapse.</p><p><strong>Methods: </strong>434 IgG4-RD patients met comprehensive diagnostic criteria and diagnosed at Peking University People's Hospital between January 2009 and January 2020 were recruited in this study. Patients were divided into IgG4-ROD and nIgG4-ROD group according to the ophthalmic involvement. Demographic, clinical, and laboratory data of two groups were collected and compared. Cox regression analysis was used to identify the independent risk factors for IgG4-ROD relapse.</p><p><strong>Results: </strong>255 IgG4-ROD patients were identified in this study. IgG4-ROD group had almost equal sex ratio, younger age of disease onset and diagnosis comparing with nIgG4-ROD patients. As compared to nIgG4-ROD group, higher percentage of IgG4-ROD patients met the 2019 American College of Rheumatology/European League Against Rheumatism classification criteria (AECC) for IgG4-RD; moreover, IgG4-ROD patients had higher AECC scores and IgG4-RD responder index (RI). Allergic diseases and multiorgan involvement were more common in IgG4-ROD group. IgG4-ROD was frequently associated with salivary gland, paranasal sinus, lung, and lymph node involvement, while retroperitoneal fibrosis and biliary system lesions were more common in nIgG4-ROD. IgG4-ROD patients had higher serum IgG4 levels, IgG4/IgG ratio, IgE levels, and lower CRP levels. The initial glucocorticoid plus immunosuppressant was a protective factor for IgG4-ROD relapse. IgG4-ROD patients treated with initial glucocorticoid plus immunosuppressant had longer relapse-free survival time than patients treated with initial glucocorticoid monotherapy.</p><p><strong>Conclusions: </strong>IgG4-ROD patients had distinctive clinical features compared with nIgG4-ROD patients. The initial glucocorticoid plus immunosuppressant was a protective factor for IgG4-ROD relapse, which could prolong the relapse-free survival time of IgG4-ROD patients. These findings may have important implications for understanding and management of IgG4-ROD.</p>","PeriodicalId":51225,"journal":{"name":"Arthritis Research & Therapy","volume":"23 1","pages":"98"},"PeriodicalIF":4.9,"publicationDate":"2021-03-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s13075-021-02489-9","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"25537509","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Total metabolic lesion volume of lymph nodes measured by <sup>18</sup>F-FDG PET/CT: a new predictor of macrophage activation syndrome in adult-onset Still's disease.","authors":"Liyan Wan, Yuting Gao, Jieyu Gu, Huihui Chi, Zhihong Wang, Qiongyi Hu, Jinchao Jia, Tingting Liu, Biao Li, Jialin Teng, Honglei Liu, Xiaobing Cheng, Junna Ye, Yutong Su, Chengde Yang, Hui Shi, Min Zhang","doi":"10.1186/s13075-021-02482-2","DOIUrl":"https://doi.org/10.1186/s13075-021-02482-2","url":null,"abstract":"<p><strong>Background: </strong>To investigate the potential utility of quantitative parameters obtained by <sup>18</sup>F-fluorodeoxyglucose positron emission tomography/computed tomography (<sup>18</sup>F-FDG PET/CT) in the assessment of disease severity and the occurrence of macrophage activation syndrome (MAS) in adult-onset Still's disease (AOSD).</p><p><strong>Methods: </strong>Fifty-seven patients with AOSD who underwent pre-treatment <sup>18</sup>F-FDG PET/CT were recruited in this study and compared with 60 age- and sex-matched healthy controls. Clinical features and laboratory data were recorded. The systemic score was assessed to determine the disease severity. The maximal standardized uptake value (SUV<sub>max</sub>), metabolic lesion volume (MLV), and total lesion glycolysis (TLG) were used to evaluate the involved organs and tissues that abnormally accumulated <sup>18</sup>F-FDG. Multivariate analysis was performed to identify the PET/CT-derived risk factors contributing to the AOSD-related MAS, and their diagnostic efficiency was evaluated.</p><p><strong>Results: </strong>High <sup>18</sup>F-FDG accumulation was observed in the bone marrow (SUV<sub>max</sub> median, 5.10), spleen (SUV<sub>max</sub> median, 3.70), and lymph nodes (LNs, SUV<sub>max</sub> median, 5.55). The SUV<sub>max</sub> of the bone marrow (rho = 0.376, p = 0.004), SUV<sub>max</sub> of the spleen (rho = 0.450, p < 0.001), TLG<sub>total</sub> of LNs (rho = 0.386, p = 0.017), and MLV<sub>total</sub> of LNs (rho = 0.391, p = 0.015) were correlated with the systemic score. The SUV<sub>max</sub> of the spleen (p = 0.017), TLG<sub>total</sub> of LNs (p = 0.045), and MLV<sub>total</sub> of LNs (p = 0.012) were higher in patients with MAS than in those without MAS. A MLV<sub>total</sub> of LNs > 62.2 (OR 27.375, p = 0.042) was an independent predictive factor for MAS with a sensitivity of 80.0% and a specificity of 93.9%.</p><p><strong>Conclusions: </strong>The glucose metabolic level of the spleen could be an effective and easy-to-use imaging indicator of disease severity, and MLV<sub>total</sub> of LNs > 62.2 was a strong predictor of MAS occurrence in patients with AOSD.</p>","PeriodicalId":51225,"journal":{"name":"Arthritis Research & Therapy","volume":"23 1","pages":"97"},"PeriodicalIF":4.9,"publicationDate":"2021-03-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s13075-021-02482-2","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"25531256","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}