Rheumatology最新文献

{"title":"Associations between 6-minute walk distance and physiologic measures and clinical outcomes in myositis-associated interstitial lung disease.","authors":"Sangmee Sharon Bae, Daniela Markovic, Didem Saygin, Daniel Sullivan, Koichi Yamaguchi, Siamak Moghadam-Kia, Chester V Oddis, Fereidoun Abtin, Grace Hyun J Kim, Galina Marder, Swamy Venuturupalli, Paul F Dellaripa, Sonye Danoff, Tracy Doyle, Gary Hunninghake, Joyce S Lee, Jeremy Falk, Cheilonda Johnson, Jonathan Goldin, Donald Tashkin, Christina Charles-Schoeman, Rohit Aggarwal","doi":"10.1093/rheumatology/keae477","DOIUrl":"https://doi.org/10.1093/rheumatology/keae477","url":null,"abstract":"<p><strong>Objective: </strong>The 6-min walk test (6MWT) is a simple test widely used to assess sub-maximal exercise capacity in chronic respiratory diseases. We explored the relationship of 6-min walk distance (6MWD) with measurements of physiological, clinical, radiographic measures in patients with myositis-associated interstitial lung disease (MA-ILD).</p><p><strong>Method: </strong>We analyzed data from the Abatacept in Myositis Associated Interstitial lung disease (Attack My-ILD) study, a 48-week multicentre randomized trial of patients with anti-synthetase antibodies and active MA-ILD. 6MWD, forced vital capacity (FVC), diffusing capacity (DLCO), high resolution CT, and various physician/patient reported outcome measures were obtained during the trial. Spearman's correlations and repeated-measures analysis with linear mixed-effects models were used to estimate the associations between 6MWD and various physiologic, clinical and radiographic parameters both cross-sectionally and longitudinally.</p><p><strong>Results: </strong>Twenty participants with a median age of 57, 55% male and 85% white were analyzed. Baseline 6MWD did not associate with baseline PFTs. Repeated-measures analysis showed 6MWD over time associated with FVC over time, but not with DLCO. 6MWD over time also correlated with UCSD dyspnea score, Borg scores, as well as global disease activity and muscle strength over time. Emotional role functioning, vitality, general health and physical functioning scores by short form 36 also correlated with 6MWD over time.</p><p><strong>Conclusions: </strong>: Exploratory work in a small cohort of MA-ILD demonstrated 6MWD over time associated with parallel changes in FVC and patient reported outcomes of dyspnea, but not with DLCO. Larger studies are needed to validate the reliability, responsiveness and utility of the 6MWT in MA-ILD.</p><p><strong>Clinical trial registration: </strong>ClinicalTrials.gov, NCT03215927.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":null,"pages":null},"PeriodicalIF":4.7,"publicationDate":"2024-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142120514","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"An objective diagnosis of gout and calcium pyrophosphate deposition disease with machine learning of Raman spectra acquired in a point-of-care setting.","authors":"Tom Niessink, Tim L Jansen, Frank A W Coumans, Tim J M Welting, Matthijs Janssen, Cees Otto","doi":"10.1093/rheumatology/keae472","DOIUrl":"https://doi.org/10.1093/rheumatology/keae472","url":null,"abstract":"<p><strong>Objective: </strong>Raman spectroscopy is proposed as a next-generation method for the identification of monosodium urate (MSU) and calcium pyrophosphate (CPP) crystals in synovial fluid. As the interpretation of Raman spectra requires specific expertise, the method is not directly applicable for clinicians. We developed an approach to demonstrate that the identification process can be automated with the use of machine learning techniques. The developed system is tested in a point-of-care-setting at our outpatient rheumatology department.</p><p><strong>Methods: </strong>We collected synovial fluid samples from 446 patients with various rheumatic diseases from three centra. We analyzed all samples with our Raman spectroscope and used 246 samples for training and 200 samples for validation. Trained observers classified every Raman spectrum as MSU, CPP or else. We designed two one-against-all classifiers, one for MSU and one for CPP. These classifiers consisted of a principal component analysis model followed by a support vector machine.</p><p><strong>Results: </strong>The accuracy for classification of CPP using the 2023 ACR/EULAR CPPD classification criteria was 96.0% (95% CI 92.3-98.3), while the accuracy for classification of MSU with using the 2015 ACR/EULAR gout classification criteria was 92.5% (95% CI 87.9-95.7). Overall, the accuracy for classification of pathological crystals was 88.0% (95% CI 82.7-92.2). The model was able to discriminate between pathologic crystals, artifacts, and other particles such as microplastics.</p><p><strong>Conclusion: </strong>We here demonstrate that potentially complex Raman spectra from clinical patient samples can be successfully classified by a machine learning approach, resulting in an objective diagnosis independent of the opinion of the medical examiner.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":null,"pages":null},"PeriodicalIF":4.7,"publicationDate":"2024-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142120512","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Long-term use of interleukin-1 inhibitors reduce flare activity in patients with fibrodysplasia ossificans progressiva.","authors":"Ruby Haviv, Leonid Zeitlin, Veronica Moshe, Amit Ziv, Noa Rabinowicz, Fabrizio De Benedetti, Giusi Prencipe, Valentina Matteo, Carmen Laura De Cunto, Edward C Hsiao, Yosef Uziel","doi":"10.1093/rheumatology/keae255","DOIUrl":"10.1093/rheumatology/keae255","url":null,"abstract":"<p><strong>Objectives: </strong>Fibrodysplasia ossificans progressiva (FOP) is one of the most catastrophic forms of genetic heterotopic ossification (HO). FOP is characterized by severe, progressive inflammatory flare-ups, that often lead to HO. The flare-ups are associated with increased inflammatory cytokine production, suggesting auto-inflammatory features driven by IL-1β. This study describes the short- and long-term responses of FOP patients to anti-IL-1 therapy.</p><p><strong>Methods: </strong>Previously, we reported that a patient with FOP treated with anti-IL-1 agents showed dramatically lower rates of flare-ups, improved flare-up symptoms, decreased use of glucocorticoids and apparently decreased size of residual lesions. Plasma analyses also showed marked elevation in IL-1β levels during a FOP flare, further supporting a role of IL-1β in the pathogenesis of FOP flares. Here, we report results from long-term therapy with IL-1 inhibitors in that patient and describe 3 additional patients, from two medical centres.</p><p><strong>Results: </strong>All 4 patients showed persistent improvement in flare activity during treatment with IL-1 inhibitors, with minimal formation of new HO sites. Two patients who stopped therapy experienced a resurgence of flare activity that was re-suppressed upon re-initiation. These patients had IL-1β levels comparable to those in IL-1β-driven diseases. Child Health Assessment Questionnaires confirmed extensive subjective improvements in the pain and general health visual analogue scales.</p><p><strong>Conclusion: </strong>This case series demonstrates significant benefits from IL-1 inhibitors for reducing flare activity and improving the general health of patients with FOP. These data provide strong support for additional studies to better understand the function of IL-1 inhibition, primarily in reducing the formation of new HO.</p><p><strong>Funding: </strong>RH received support from the International FOP Association ACT grant; ECH received support from NIH/NIAMS R01AR073015 and the UCSF Robert Kroc Chair in Connective Tissue and Rheumatic Diseases III.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":null,"pages":null},"PeriodicalIF":4.7,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11371373/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140909188","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":"Individuals with ACPA-negative clinically suspect arthralgia experience more symptom burden: is seronegative disease truly less severe?","authors":"Michaël Doumen, René Westhovens, Patrick Verschueren","doi":"10.1093/rheumatology/keae106","DOIUrl":"10.1093/rheumatology/keae106","url":null,"abstract":"","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":null,"pages":null},"PeriodicalIF":4.7,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139716338","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"CD19.CAR T cells versus obinutuzumab-who will win the race for use in deep B cell depletion therapy in systemic autoimmunity?","authors":"Peter Kvacskay, Wolfgang Merkt","doi":"10.1093/rheumatology/keae144","DOIUrl":"10.1093/rheumatology/keae144","url":null,"abstract":"","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":null,"pages":null},"PeriodicalIF":4.7,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140068675","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Assessing the sensitivity and specificity of myositis-specific and associated autoantibodies: a sub-study from the MyoCite cohort.","authors":"Aravinthan Loganathan, Latika Gupta, Alex Rudge, Hui Lu, Elizabeth Bowler, Fionnuala McMorrow, R Naveen, Anamika K Anuja, Vikas Agarwal, Neil McHugh, Sarah Tansley","doi":"10.1093/rheumatology/keae167","DOIUrl":"10.1093/rheumatology/keae167","url":null,"abstract":"<p><strong>Objectives: </strong>Myositis-specific and associated autoantibodies are important biomarkers in routine clinical use. We assessed local testing performance for myositis autoantibodies by comparing line immunoassay (LIA) to protein radio-immunoprecipitation and identifying clinical characteristics associated with each myositis autoantibody in the MyoCite cohort.</p><p><strong>Methods: </strong>Serum samples from patients within the MyoCite cohort, a well-characterized retro-prospective dataset of adult and juvenile idiopathic inflammatory myopathy (IIM) patients in Lucknow, India (2017-2020), underwent LIA at Sanjay Gandhi Postgraduate Institute of Medical Science (SGPGIMS), Lucknow. Immunoprecipitation of 147 IIM patients' serum samples (125 adult-onset, 22 juvenile-onset) was conducted at the University of Bath, with researchers blind to LIA results. LIA performance was assessed against immunoprecipitation as the reference standard, measuring sensitivity, specificity and inter-rater agreement. Univariate and multivariate logistic regression determined clinical associations for specific myositis-specific autoantibodies.</p><p><strong>Results: </strong>Immunoprecipitation identified myositis autoantibodies in 56.5% (n = 83) of patient samples, with anti-Jo1 (n = 16; 10.9%) as the most common, followed by anti-MDA5 (n = 14, 9.5%). While LIA showed good agreement for anti-Jo1, anti-PL7 and anti-PL12 (Cohen's κ 0.79, 0.83 and 1, respectively), poor agreement was observed in other subgroups, notably anti-TIF1γ (Cohen's κ 0.21). Strongly positive samples, especially in myositis-specific autoantibodies, correlated more with immunoprecipitation results. Overall, 59 (40.1%) samples exhibited non-congruence on LIA and immunoprecipitation, and κ values for LIAs for anti-TIF1γ, anti-Ku, anti-PmScl, anti-Mi2 and anti-SAE ranged between 0.21 and 0.60.</p><p><strong>Conclusion: </strong>While LIA reliably detected anti-Jo1, anti-PL7, anti-PL12, anti-MDA5 and anti-NXP-2, it also displayed false positives and negatives. Its effectiveness in detecting other autoantibodies, such as anti-TIF1γ, was poor.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":null,"pages":null},"PeriodicalIF":4.7,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140120454","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Greater DMARD use, improved clinical but not patient-reported outcomes in juvenile idiopathic arthritis: what are we missing?","authors":"Stephanie J W Shoop-Worrall, Hanna Lythgoe","doi":"10.1093/rheumatology/kead702","DOIUrl":"10.1093/rheumatology/kead702","url":null,"abstract":"","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":null,"pages":null},"PeriodicalIF":4.7,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11381682/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139098562","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":"Extra-osseous manifestations in chronic recurrent multifocal osteomyelitis: a retrospective study.","authors":"Marie Robert, Anna Giolito, Heloise Reumaux, Linda Rossi-Semerano, Claire Guillemin, Louis Biarrotte, Damia Leguevaques, Alexandre Belot, Agnès Duquesne, Cécile Frachette, Audrey Laurent, Marine Desjonquères, Jean-Paul Larbre, Caroline Galeotti, Isabelle Koné-Paut, Perrine Dusser","doi":"10.1093/rheumatology/kead473","DOIUrl":"10.1093/rheumatology/kead473","url":null,"abstract":"<p><strong>Objectives: </strong>Extra-osseous (EO) manifestations are poorly characterized in chronic recurrent multifocal osteomyelitis (CRMO). This study aimed to further define the frequency, characteristics and treatment of EO events in CRMO and whether different phenotypes can be distinguished and benefit from special management.</p><p><strong>Methods: </strong>This multicentre retrospective study included CRMO patients followed in several paediatric rheumatology departments in France between 2015 and 2022. EO manifestations were defined as skin lesions, gastrointestinal manifestations, arthritis, enthesitis, sacroiliitis, uveitis, vasculitis and fever. At the last visit, the physician defined CRMO as active in the presence of clinical manifestations including both osseous and EO symptoms.</p><p><strong>Results: </strong>We included 133 patients; 87 (65.4%) were girls and the median age at first symptoms was 9.0 years (interquartile range 7.0-10.0). EO manifestations were described in 90 (67.7%) patients, with a predominance of skin lesions [n = 51/90 (56.7%)], followed by sacroiliitis [n = 38/90 (42.2%)], enthesitis [n = 21/90 (23.3%)], arthritis [n = 14/90 (15.6%)] and gastrointestinal manifestations [n = 6/90 (6.7%)]. The use of non-steroidal anti-inflammatory drugs and bisphosphonates did not differ by the presence or not of EO manifestations. Biologics were taken more frequently by patients with than without EO manifestations (P < 0.001); TNF inhibitors were used in 33 (36.7%) EO-positive patients. Under this treatment, 18 (54.5%) patients achieved complete remission of osseous and EO manifestations. At the last visit, more EO+ than EO- patients were on treatment (P = 0.009), with active disease in 58 (64.4%) patients.</p><p><strong>Conclusion: </strong>The analysis of EO manifestations in CRMO delineates two groups of patients in terms of severity and treatments used. Our study opens up new pathophysiological leads that may underlie the wide range of CRMO phenotypes.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":null,"pages":null},"PeriodicalIF":4.7,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10277603","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Trismus as a new feature of VEXAS syndrome.","authors":"Alexis Archambeaud, Claire Le Dreau, Adrien Bigot, Olivier Kosmider, Assia Taleb, Lara Boucher, Marie Temple, Jean-Philippe Cottier, François Maillot, Alexandra Audemard-Verger","doi":"10.1093/rheumatology/keae135","DOIUrl":"10.1093/rheumatology/keae135","url":null,"abstract":"","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":null,"pages":null},"PeriodicalIF":4.7,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140050225","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Sarcoid myopathy: an insidious diagnosis that can mimic inclusion body myositis.","authors":"Kristen Mathias, Maximilian F Konig, Thomas Lloyd, Jemima Albayda","doi":"10.1093/rheumatology/keae155","DOIUrl":"10.1093/rheumatology/keae155","url":null,"abstract":"","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":null,"pages":null},"PeriodicalIF":4.7,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140102366","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}