Advances in Rheumatology最新文献

{"title":"Rare diseases: What rheumatologists need to know?","authors":"Renan Rodrigues Neves Ribeiro do Nascimento, Daniela Gerent Petry Piotto, Eutilia Andrade Medeiros Freire, Fabricio de Souza Neves, Flavio Roberto Sztajnbok, Blanca Elena Rios Gomes Bica, Frederico Augusto Gurgel Pinheiro, Katia Tomie Kozu, Ivanio Alves Pereira, Valderilio Feijo Azevedo, Rafael Alves Cordeiro, Henrique Ayres Mayrink Giardini, Marco Túlio Muniz Franco, Margarida de Fátima Fernandes Carvalho, Nilton Salles Rosa-Neto, Sandro Félix Perazzio","doi":"10.1186/s42358-024-00407-6","DOIUrl":"https://doi.org/10.1186/s42358-024-00407-6","url":null,"abstract":"<p><p>Although the terms \"rare diseases\" (RD) and \"orphan diseases\" (OD) are often used interchangeably, specific nuances in definitions should be noted to avoid misconception. RD are characterized by a low prevalence within the population, whereas OD are those inadequately recognized or even neglected by the medical community and drug companies. Despite their rarity, as our ability on discovering novel clinical phenotypes and improving diagnostic tools expand, RD will continue posing a real challenge for rheumatologists. Over the last decade, there has been a growing interest on elucidating mechanisms of rare autoimmune and autoinflammatory rheumatic diseases, allowing a better understanding of the role played by immune dysregulation on granulomatous, histiocytic, and hypereosinophilic disorders, just to name a few. This initiative enabled the rise of innovative targeted therapies for rheumatic RD. In this review, we explore the state-of-the art of rare RD and the critical role played by rheumatologists in healthcare. We also describe the challenges rheumatologists may face in the coming decades.</p>","PeriodicalId":48634,"journal":{"name":"Advances in Rheumatology","volume":"64 1","pages":"74"},"PeriodicalIF":2.0,"publicationDate":"2024-09-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142356510","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Ultrasound nail assessment in patients with psoriasic arthritis: is there an association of findings with clinical scores?","authors":"Andrieli Caroline Mehl, Leonardo Michaelis Schmidt, Valderílio Feijó Azevedo","doi":"10.1186/s42358-024-00398-4","DOIUrl":"https://doi.org/10.1186/s42358-024-00398-4","url":null,"abstract":"&lt;p&gt;&lt;strong&gt;Background: &lt;/strong&gt;Psoriatic arthritis can involve several domains. Due to its multifaceted nature and its frequent comorbidities such as depression, obesity, osteoarthritis and fibromyalgia, it is difficult to monitor these patients because the clinical scores involve subjective data. High-resolution ultrasound probes allowed the evaluation of more superficial structures, such as the nails and their synovio-entheseal framework, in close relationship with the enthesis of the distal extensor digitorum tendon. Nail ultrasound studies vary in terms of the parameters and fingers studied and in their findings.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Objectives: &lt;/strong&gt;To describe the most significant sonographic nail changes and the most affected fingers in psoriatic arthritis and to verify the association of nail ultrasound findings with clinical scores (nail psoriasis severity index (NAPSI), ankylosing spondylitis disease activity score with C-reactive protein (ASDAS-CRP), minimal disease activity (MDA), disease activity index for psoriatic arthritis (DAPSA)).&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Methods: &lt;/strong&gt;This was a cross-sectional study with 52 patients with psoriatic arthritis at the Hospital de Clínicas do Paraná and 50 controls. A total of 1016 nails were analyzed (517 from patients with psoriatic arthritis and 499 from controls). Ultrasonography of the nails of the 10 fingers was performed to assess the trilaminar appearance, measure the distance from the nail bed, identify synovitis of the distal interphalangeal joints and the presence of a power Doppler signal from the nail matrix/nail bed. The captured images were independently evaluated by a rheumatologist with expertise in musculoskeletal ultrasound. Data analysis was performed using IBM SPSS Statistics v.28.0.0 software, and the association of nail plate changes, nail bed distance and power Doppler signal with the NAPSI, DAPSA, MDA and ASDAS-PCR were calculated. Spearman correlation coefficients were estimated to analyze the correlations between pairs of quantitative variables. Student's t test and the Mann‒Whitney U test were used to compare quantitative variables, and Fisher's exact test was used to compare categorical variables between patients and controls. The nonparametric Mann‒Whitney U and Kruskal‒Wallis tests were used to compare groups according to the MDA or DAPSA classification.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Results: &lt;/strong&gt;The Doppler signal of the nail matrix and nail bed was more frequently identified in patients (44.2%) than in controls (6%), and the difference in the mean power Doppler signal between the two groups was significant (p &lt; 0.001). Changes in the nail plate were more common in the right thumb (44.2%), left thumb (36.5%) and second finger on the right hand (32.7%). The number of fingers with nail plate changes, enthesitis, paratendinitis, grayscale synovitis and DIP involvement in the distal interphalangeal joints was higher among patients with psoriatic arthritis (p &lt; 0.001). There were found some ","PeriodicalId":48634,"journal":{"name":"Advances in Rheumatology","volume":"64 1","pages":"75"},"PeriodicalIF":2.0,"publicationDate":"2024-09-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142356411","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Gout comorbidities: results from the Korean National Health and Nutrition Examination Survey.","authors":"Hyemin Jeong, Young-Soo Chang, Chan Hong Jeon","doi":"10.1186/s42358-024-00413-8","DOIUrl":"https://doi.org/10.1186/s42358-024-00413-8","url":null,"abstract":"<p><strong>Objectives: </strong>Gout is associated with several comorbidities. This study aimed to evaluate the prevalence of comorbidities in the Korean adult population with gout and investigated the association of gout with these comorbidities.</p><p><strong>Methods: </strong>Data from 15,935 (weighted n = 39,049,167) participants aged 19 years and older in the Korean National Health and Nutrition Examination Survey from 2019 to 2021 were used for analysis. Weighted prevalence and odds ratios (OR) of comorbidities in individuals with gout were compared to a non-gout population.</p><p><strong>Results: </strong>The weighted prevalence of gout was 2.1% (weighted n = 808,778). Among individuals with gout, 66.5% had metabolic syndrome, 54.9% had hypertension, 41.2% had hypercholesterolemia, 19.1% had diabetes, 13.5% had chronic kidney disease (CKD), 4.1% had myocardial infarction or angina, 3.8% had stroke, and 2.8% had rheumatoid arthritis (RA). After adjusting for socioeconomic and lifestyle characteristics, gout was independently associated with the increased prevalence of metabolic syndrome (male OR = 2.0, 95% confidence interval (CI): 1.5-2.8; female OR = 3.7, 95% CI: 1.5-9.2), hypercholesterolemia (male OR = 1.9, 95% CI: 1.4-2.5; female OR = 3.1, 95% CI: 1.3-7.5), CKD (male OR = 4.5, 95% CI: 2.7-7.3; female OR = 11.5, 95% CI: 4.1-32.1), and RA (male OR = 2.8, 95% CI: 1.1-7.1; female OR = 3.1, 95% CI: 1.1-8.7) compared to the non-gout population.</p><p><strong>Conclusions: </strong>Gout was associated with several comorbidities, including RA, in both males and females. These results suggest that the prevention and treatment of comorbidities at the individual level, carried out by clinicians, and knowledge of these comorbidities would help guide health policies for the Korean population.</p>","PeriodicalId":48634,"journal":{"name":"Advances in Rheumatology","volume":"64 1","pages":"76"},"PeriodicalIF":2.0,"publicationDate":"2024-09-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142356509","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Brazilian Society of Rheumatology and Brazilian Society of Clinical Pathology/Laboratory Medicine recommendation for serum uric acid test reports on patients undergoing treatment for gout.","authors":"Geraldo da Rocha Castelar Pinheiro, Marco Antônio Araújo da Rocha Loures, Luís Eduardo Coelho Andrade, Fabiano de Almeida Brito, Leonardo de Souza Vasconcellos","doi":"10.1186/s42358-024-00415-6","DOIUrl":"https://doi.org/10.1186/s42358-024-00415-6","url":null,"abstract":"","PeriodicalId":48634,"journal":{"name":"Advances in Rheumatology","volume":"64 1","pages":"73"},"PeriodicalIF":2.0,"publicationDate":"2024-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142356508","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Development of a questionnaire to assess the patient perspective regarding challenges in psoriatic arthritis treatment—a mixed-methods study","authors":"André Lucas Ribeiro, Júlia Andressa Tessari, Charles Lubianca Kohem, Penélope Esther Palominos, Rafael Mendonça da Silva Chakr","doi":"10.1186/s42358-024-00414-7","DOIUrl":"https://doi.org/10.1186/s42358-024-00414-7","url":null,"abstract":"Limited data exist on psoriatic arthritis (PsA) treatment in lower-income regions, particularly from the patient perspective. This study explores the challenges faced by socioeconomically vulnerable PsA patients and the reasons for non-adherence to treatment guidelines. The main objective of the study is to develop a questionnaire to identify the primary challenges in PsA treatment adherence and to analyze its feasibility while simultaneously understanding the target population’s unique characteristics. We included PsA patients meeting the Classification Criteria for PsA (CASPAR), excluding those with other overlapping inflammatory diseases. The study, supported by two patient-research partners, began with focus groups to identify treatment challenges, leading to the creation of a 26-item questionnaire. Its reliability was verified using the test-retest method, targeting a percent agreement ≥ 0.8. Then, PsA patients at a rheumatology clinic completed the final survey. The study involved 69 PsA patients. The final questionnaire contained 26-questions across five-domains, with a 92.2% agreement rate and an average completion time of 8.3 minutes. Diagnostic delays exceeded a year for 59% of patients and more than two years for 33%. Daily life disruptions affected 43.2% of patients, with 35.3% taking sick leave or retiring. Around 25% waited over 8 weeks for drug approval, and 17.6% required legal intervention to access medication. Drug dispensation issues impacted about 60% of patients. Furthermore, 66.7% lived far from their rheumatologist, with 49% traveling over an hour for appointments. Approximately 30% were unaware of the risks of methotrexatein relation to alcohol consumption and pregnancy. The questionnaire was feasible and reliable, with its results underscoring patient-centric challenges in PsA management, particularly concerning diagnostic delays and medication access, as well as daily life disruptions and misinformation. These findings emphasize the urgency for healthcare reforms aimed at improving diagnosis efficiency, patient education, and streamlined medication access, emphasizing the need for tailored initiatives to improve the healthcare experience for PsA patients.","PeriodicalId":48634,"journal":{"name":"Advances in Rheumatology","volume":"164 1","pages":""},"PeriodicalIF":2.3,"publicationDate":"2024-09-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142263056","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Uncovering the knowledge about systemic amyloidosis relevant to the rheumatologists","authors":"Ivanio Alves Pereira, Nilton Salles Rosa Neto, Renan Rodrigues Neves Ribeiro do Nascimento, Eutilia Andrade Medeiros Freire, Fabricio de Souza Neves, Blanca Elena Rios Gomes Bica, Frederico Augusto Gurgel Pinheiro, Sandro Félix Perazzio, Rafael Alves Cordeiro, Henrique Ayres Mayrink Giardini, Valderilio Feijo Azevedo, Flavio Roberto Sztajnbok","doi":"10.1186/s42358-024-00399-3","DOIUrl":"https://doi.org/10.1186/s42358-024-00399-3","url":null,"abstract":"Amyloidosis is a localized or systemic disease caused by deposition of proteins in the extracellular space of various organs and tissues. As part of the disease, proteins that were originally soluble misfold and acquire a fibrillar conformation that renders them insoluble and resistant to proteolysis. Systemic amyloidosis is a rare, often underdiagnosed condition. In recent years, the incidence of newly diagnosed cases of amyloidosis has been increasing in association with the aging of the population and greater access to diagnostic tests. From a clinical perspective, systemic amyloidosis is frequently associated with involvement of the kidneys (causing nephrotic syndrome), heart (cardiac failure and arrhythmia), and peripheral nervous system (sensorimotor polyneuropathy and autonomic dysfunction). This condition is important to the rheumatologist for several reasons, such as its systemic involvement that mimics autoimmune rheumatic diseases, its musculoskeletal manifestations, which when recognized can allow the diagnosis of amyloidosis, and also because reactive or secondary AA amyloidosis is a complication of rheumatic inflammatory diseases. The treatment of amyloidosis depends on the type of amyloid protein involved. Early recognition of this rare disease is fundamental for improved clinical outcomes.","PeriodicalId":48634,"journal":{"name":"Advances in Rheumatology","volume":"95 1","pages":""},"PeriodicalIF":2.3,"publicationDate":"2024-09-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142263057","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Anti-protein phosphatase magnesium-dependent 1A-IgM levels in patients with active ankylosing spondylitis: a potential biomarker","authors":"Yeo-Jin Lee, Eun-Ju Lee, Soo Min Ahn, Seokchan Hong, Ji Seon Oh, Chang-Keun Lee, Bin Yoo, Yong-Gil Kim","doi":"10.1186/s42358-024-00412-9","DOIUrl":"https://doi.org/10.1186/s42358-024-00412-9","url":null,"abstract":"Ankylosing spondylitis (AS) has been known to have auto-inflammatory nature; hence, the efficacy of autoantibodies is low. However, studies on autoantibodies are ongoing, with some studies showing associations. Previous studies showed that anti-protein phosphatase magnesium-dependent 1A (PPM1A) IgG was increased in patients with AS and associated with radiographic progression. However, the diagnostic usefulness was limited due to relatively low sensitivity and specificity. This pilot study evaluated the diagnostic utility of anti-PPM1A-IgM and anti-PPM1A-IgG in patients with active AS. Serum samples were obtained from the registry cohort of a single tertiary center in Korea. Serum levels of anti-PPM1A-IgG/IgM were measured by direct ELISA. Receiver operating characteristic (ROC) analysis was used to predict the diagnostic sensitivity and specificity of serum anti-PPM1A-IgG/IgM. Samples were collected from 28 patients with active AS, 16 healthy controls (HCs), and 28 patients with rheumatoid arthritis (RA). Although total serum IgM was lower in the RA and AS groups than in the HC group, anti-PPM1A-IgM was significantly lower in the AS group than in the other groups. In evaluating the diagnostic utility of anti-PPM1A-IgG/IgM for AS patients compared with HCs, the area under the curve (AUC) of anti-PPM1A-IgM was 0.998 (sensitivity 96.4%, specificity 100.0%). When ROC analysis of anti-PPM1A-IgM for AS patients compared with RA patients was conducted, sensitivity was 78.6% and specificity was 71.4%, with an AUC of 0.839. Decreased anti-PPM1A-IgM levels in AS patients suggests a potential role for anti-PPM1A-IgM in the diagnosis of active AS.","PeriodicalId":48634,"journal":{"name":"Advances in Rheumatology","volume":"393 1","pages":""},"PeriodicalIF":2.3,"publicationDate":"2024-09-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142185939","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Tuberculin skin test repetition after TNF-α inhibitors in patients with chronic inflammatory arthritis: a long-term retrospective cohort in endemic area","authors":"Vanessa de Oliveira Magalhães, Karina Rossi Bonfiglioli, Carina More Frade Gomes, Eloisa Bonfá, Ana Cristina de Medeiros-Ribeiro, Carla Gonçalves S. Saad, Marcelo de Medeiros Pinheiro","doi":"10.1186/s42358-024-00406-7","DOIUrl":"https://doi.org/10.1186/s42358-024-00406-7","url":null,"abstract":"To evaluate the tuberculin skin test (TST) conversion in chronic inflammatory arthropathies (CIA) patients on TNFα inhibitors (TNFi) and without previous latent tuberculosis infection (LTBI) treatment. Patients with rheumatoid arthritis (RA), ankylosing spondylitis (AS) and psoriatic arthritis (PsA) with negative LTBI were retrospectively evaluated for TST conversion and active tuberculosis (TB) after six months of exposition to TNFi. Two groups were compared: patients who repeated TST (TST-repetition) during the follow-up and patients who did not (non-TST-repetition). A total of 355 CIA patients on TNFi were screened and 138 (38.9%) did not fulfill the inclusion criteria. Of the remaining 217 CIA patients, 81 (37.3%) repeated TST during TNFi treatment. TST conversion rate was observed in 18 (22.2%) patients without significant differences among CIA (p = 0.578). The number of TB cases was low (n = 10; 4.6%) and was similar in TST-repetition and non-TST-repetition groups [2 (2.5%) vs. 8 (5.9%), p = 0.328]. Of note, 30% of active TB occurred early (6–12 months of TNFi exposure) and the median (full range) time to incident TB was 1.3 (0.6–10.6) years, whereas the median (full range) time to TST repetition was later [3.3 (0.5–13.4) years]. The incidence of active TB was lower among RA patients than AS patients [342 (95% CI 41 − 1446) vs. 1.454 (95% CI 594-2993)/100,000 patient-years, p = 0.049]. These results indicate that TST repetition is associated with a high conversion rate, suggesting the need for recommended treatment. The delayed repetition of TST and low number of active TB cases hampered the evaluation of this strategy effectiveness to prevent active infection. Larger studies with systematic repetition patterns are necessary. In addition, the study highlights the need for a greater surveillance for TB in AS patients. • TST repetition is associated with a high conversion rate for positive LTBI after long-term TNFi. • Active tuberculosis diagnosis occurs early (1.3 years) during TNFi therapy. • AS patients require greater surveillance for incident TB.","PeriodicalId":48634,"journal":{"name":"Advances in Rheumatology","volume":"30 1","pages":""},"PeriodicalIF":2.3,"publicationDate":"2024-09-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142263058","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Sacroiliac and spine imaging in spondyloarthritis: Does phenotype or sex matter?","authors":"Gabriel Caetano Pereira, Natalia Pereira Machado, André Francisco Gomes, Rodrigo Luppino Assad, Fabio Henrique Carneiro, Valderílio Feijó Azevedo","doi":"10.1186/s42358-024-00411-w","DOIUrl":"https://doi.org/10.1186/s42358-024-00411-w","url":null,"abstract":"Spondyloarthritis (SpA) encompasses a spectrum of immune-mediated inflammatory conditions primarily affecting the axial skeleton, including sacroiliitis and spondylitis, each with distinct features. This study aimed to investigate imaging disparities, focusing on sacroiliac magnetic resonance and spine radiography, across phenotypes and between males and females in axial SpA. A cross-sectional study was conducted to assess clinical data, laboratory findings, magnetic resonance imaging (MRI) scores of sacroiliac joints using the Spondyloarthritis Research Consortium of Canada (SPARCC) and Sacroiliac Joint Structural Score (SSS), and cervical and lumbar spine radiographs utilizing the Modified Stoke Ankylosing Spondylitis Spine Score (mSASSS). The study aimed to compare these parameters between two groups: axial spondyloarthritis (axSpA, radiographic and non-radiographic) and axial psoriatic arthritis (axPsA), as well as between males and females. Ninety-four patients were included, with 62 patients in the axSpA group and 32 patients in the axPsA group. There were no differences in disease activity, mobility, radiographic damage in the spine (Modified Stoke Ankylosing Spondylitis Spine Score– mSASSS), or sacroiliac magnetic resonance imaging (MRI) scores (Spondyloarthritis Research Consortium of Canada Magnetic Resonance Imaging Index - SPARCC and Sacroiliac Joint Structural Score - SSS) between the two phenotypes. Regarding sex, in imaging exams, men had higher mSASSS (p = 0.008), SSS (p = 0.001), and fat metaplasia (MG) score based on SSS (p = 0.001), while women had significantly higher SPARCC scores (p = 0.039). In the male group, the presence of HLA-B27 allele had an impact on more structural lesions on MRI (SSS), p = 0.013. In this study, imaging of sacroiliac joints and spine in patients with axial SpA did not show differences in phenotypes but did reveal differences based on sex, which may have an impact on future diagnostic recommendations. Further studies are needed to confirm these findings.","PeriodicalId":48634,"journal":{"name":"Advances in Rheumatology","volume":"37 1","pages":""},"PeriodicalIF":2.3,"publicationDate":"2024-09-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142185940","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Progranulin mediates the onset of pristane induced systemic lupus erythematosus","authors":"Michun He, Aubryanna Hettinghouse, Yufei Bi, Yuehong Chen, Chuanju Liu","doi":"10.1186/s42358-024-00405-8","DOIUrl":"https://doi.org/10.1186/s42358-024-00405-8","url":null,"abstract":"Progranulin (PGRN) is a growth factor-like molecule with diverse roles in homeostatic and pathogenic processes including the control of immune and inflammatory responses. Pathogenic inflammation is a hallmark of systemic lupus erythematosus (SLE) and elevated serum levels of PGRN has been evaluated as a biomarker of disease activity in SLE. However, the role of PGRN in SLE has not been fully investigated. This study is aimed to determine the potential involvements of PGRN in SLE. Wild type (WT) and PGRN knockout (PGRN-/-) C57BL/6 mice received intraperitoneal injection of pristane for induction of a murine model of SLE. Sera were collected every biweekly and levels of anti-dsDNA antibody, IgG, and inflammatory factors were measured. Mice were sacrificed 5 months later and the renal lesions, as well as the proportions of T cell subtypes in the spleen were analyzed. Following exposure to pristane, PGRN-/- mice generated significantly lower levels of anti-dsDNA antibody and IgG relative to WT mice. PGRN-/- mouse kidneys had less IgG and collagen deposition compared with WT mice after pristane injection. The results indicate that PGRN participates in inflammatory response and renal damage in pristane induced SLE models, suggesting that PGRN mediates the onset of SLE.","PeriodicalId":48634,"journal":{"name":"Advances in Rheumatology","volume":"35 1","pages":""},"PeriodicalIF":2.3,"publicationDate":"2024-09-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142185941","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}