Emma Rubenstein, C. Maldini, A. Vaglio, F. Bello, J. Bremer, F. Moosig, P. Bottero, Alberto Pesci, R. Sinico, J. Grosskreutz, Claudia Feder, D. Saadoun, Giorgio Trivioli, F. Maritati, B. Rewerska, W. Szczeklik, P. Fraticelli, Giuseppe Guida, G. Gregorini, G. Moroncini, B. Hellmich, J. Zwerina, Matthieu Resche-Rigon, G. Emmi, T. Neumann, A. Mahr
{"title":"Cluster Analysis To Explore Clinical Subphenotypes Of Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss).","authors":"Emma Rubenstein, C. Maldini, A. Vaglio, F. Bello, J. Bremer, F. Moosig, P. Bottero, Alberto Pesci, R. Sinico, J. Grosskreutz, Claudia Feder, D. Saadoun, Giorgio Trivioli, F. Maritati, B. Rewerska, W. Szczeklik, P. Fraticelli, Giuseppe Guida, G. Gregorini, G. Moroncini, B. Hellmich, J. Zwerina, Matthieu Resche-Rigon, G. Emmi, T. Neumann, A. Mahr","doi":"10.3899/jrheum.2022-0325","DOIUrl":"https://doi.org/10.3899/jrheum.2022-0325","url":null,"abstract":"OBJECTIVE\u0000Previous studies suggested that distinct phenotypes of eosinophilic granulomatosis with polyangiitis (EGPA) could be determined by presence or absence of antineutrophil cytoplasmic antibodies (ANCA), reflecting predominant vasculitic or eosinophilic processes, respectively. This study explored whether ANCA-based clusters or other clusters can be identified in EGPA.\u0000\u0000\u0000METHODS\u0000This study used standardized data of 15 European centers for patients with EGPA fulfilling widely accepted classification criteria. We used multiple correspondence analysis, hierarchical cluster analysis, and a decision tree model. The main model included 10 clinical variables (musculoskeletal, mucocutaneous, ophthalmological, ENT, cardiovascular, pulmonary, gastrointestinal, renal, central or peripheral neurological involvement); a second model also included ANCA results.\u0000\u0000\u0000RESULTS\u0000The analyses included 489 patients diagnosed in 1984-2015. ANCA were detected in 37.2% of patients, mostly P-ANCA (85.4%) and/or anti-myeloperoxidase (87.0%). Compared with ANCA-negative patients, those with ANCA had more renal (P<0.001) and peripheral neurological involvement (P=0.04), fewer cardiovascular signs (P<0.001) and fewer biopsies with eosinophilic tissue infiltrates (P=0.001). The cluster analyses generated four (model without ANCA) and five clusters (model with ANCA). Both models identified three identical clusters of 34, 39 and 40 patients according to the presence or absence of ENT, CNS and ophthalmological involvement. Peripheral neurological and cardiovascular involvement were not predictive characteristics.\u0000\u0000\u0000CONCLUSION\u0000Although reinforcing the known association of ANCA status with clinical manifestations, cluster analysis does not support a complete separation of EGPA in ANCA-positive and -negative subsets. Collectively, these data indicate that EGPA should be regarded as a phenotypic spectrum rather than a dichotomous disease.","PeriodicalId":35278,"journal":{"name":"The Journal of rheumatology. Supplement","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"76588458","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Eveline Y. Wu, M. Oliver, Joshua Scheck, S. Lapidus, U. Akca, S. Yasin, S. Stern, A. Insalaco, M. Pardeo, Gabriele Simonini, E. Marrani, Xing Wang, Bin Huang, L. Kovalick, Natalie Rosenwasser, Gabriel Casselman, Adriel Liau, Yurong Shao, Claire Yang, D. M. Mosa, Lori B. Tucker, H. Girschick, R. Laxer, J. Akikusa, C. Hedrich, K. Onel, F. Dedeoğlu, M. Twilt, P. Ferguson, Seza Ozen, Yongdong Zhao
{"title":"Feasibility of Conducting Comparative Effectiveness Research and Validation of a Clinical Disease Activity Score for Chronic Nonbacterial Osteomyelitis","authors":"Eveline Y. Wu, M. Oliver, Joshua Scheck, S. Lapidus, U. Akca, S. Yasin, S. Stern, A. Insalaco, M. Pardeo, Gabriele Simonini, E. Marrani, Xing Wang, Bin Huang, L. Kovalick, Natalie Rosenwasser, Gabriel Casselman, Adriel Liau, Yurong Shao, Claire Yang, D. M. Mosa, Lori B. Tucker, H. Girschick, R. Laxer, J. Akikusa, C. Hedrich, K. Onel, F. Dedeoğlu, M. Twilt, P. Ferguson, Seza Ozen, Yongdong Zhao","doi":"10.1101/2022.10.03.22280351","DOIUrl":"https://doi.org/10.1101/2022.10.03.22280351","url":null,"abstract":"Objective Prospective comparative effectiveness research (CER) in chronic nonbacterial osteomyelitis (CNO) is lacking. Our objectives were to (1) determine the use and safety of each consensus treatment plan (CTP) regimen for CNO, (2) assess the feasibility of using the Chronic Nonbacterial Osteomyelitis International Registry (CHOIR) data for CER, and (3) develop and validate a CNO clinical disease activity score (CDAS) using CHOIR. Methods Consenting children or young adults with CNO were enrolled into CHOIR. Demographic, clinical, and imaging data were prospectively collected. The CNO CDAS was developed through a Delphi survey and nominal group technique. External validation surveys were administered to CHOIR participants. Results One hundred forty (78.2%) CHOIR participants enrolled between August 2018 and September 2020 received at least 1 CTP regimen. Baseline characteristics from different CTP groups were well matched. Patient pain, patient global assessment, and clinical CNO lesion count were key variables included in the CNO CDAS. The CDAS showed a strong correlation with patient/parent report of difficulty using a limb, back, or jaw and patient/parent report of disease severity, but a weak correlation with patient/parent report of fatigue, sadness, and worry. The change in CDAS was significant in patients reporting disease worsening or improvement (P < 0.001). The CDAS significantly decreased after initiating second-line treatments from median 12.0 (IQR 8.0-15.5) to 5.0 (IQR 3.0-12.0; P = 0.002). Although second-line treatments were well tolerated, psoriasis was the most common adverse event. Conclusion The CNO CDAS was developed and validated for disease monitoring and assessment of treatment effectiveness. CHOIR provided a comprehensive framework for future CER.","PeriodicalId":35278,"journal":{"name":"The Journal of rheumatology. Supplement","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-10-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"78323006","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Stephanie J W Shoop-Worrall, Louisa Moull, Janet E McDonagh, Kimme L Hyrich
{"title":"The Role of Age in Delays to Rheumatological Care in Juvenile Idiopathic Arthritis.","authors":"Stephanie J W Shoop-Worrall, Louisa Moull, Janet E McDonagh, Kimme L Hyrich","doi":"10.3899/jrheum.211316","DOIUrl":"10.3899/jrheum.211316","url":null,"abstract":"<p><strong>Objective: </strong>To investigate the relationship between age and symptom duration at initial presentation to pediatric rheumatology for juvenile idiopathic arthritis (JIA).</p><p><strong>Methods: </strong>In children and young people (CYP) enrolled in the Childhood Arthritis Prospective Study prior to March 2018, an association between age at presentation (< 5, 5-11, and > 11 yrs) and symptom duration was tested by multivariable linear regression.</p><p><strong>Results: </strong>In 1577 CYP, 5- to 11-year-olds took 3.2 months longer and > 11-year-olds 6.9 months longer to reach pediatric rheumatology than < 5-year-olds.</p><p><strong>Conclusion: </strong>Adolescents take longer to reach pediatric rheumatology, potentially affecting their longer-term outcomes given the window of opportunity for JIA treatment.</p>","PeriodicalId":35278,"journal":{"name":"The Journal of rheumatology. Supplement","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"74695125","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ecem Sevim, Salma Siddique, Madhavi Latha S Chalasani, Susan Chyou, William D Shipman, Orla O'Shea, Joanna Harp, Oral Alpan, Stéphane Zuily, Theresa T Lu, Doruk Erkan
{"title":"Mammalian Target of Rapamycin Pathway Assessment in Antiphospholipid Antibody-Positive Patients with Livedo.","authors":"Ecem Sevim, Salma Siddique, Madhavi Latha S Chalasani, Susan Chyou, William D Shipman, Orla O'Shea, Joanna Harp, Oral Alpan, Stéphane Zuily, Theresa T Lu, Doruk Erkan","doi":"10.3899/jrheum.220049","DOIUrl":"10.3899/jrheum.220049","url":null,"abstract":"<p><strong>Objective: </strong>In antiphospholipid antibody (aPL) nephropathy, activation of the mammalian target of rapamycin (mTOR) contributes to endothelial cell proliferation, a key finding of aPL microvascular disease. Here, we examined mTOR activation in the skin of aPL-positive patients with livedo.</p><p><strong>Methods: </strong>Three patient groups with livedo were studied: (1) persistently aPL-positive with systemic lupus erythematosus (SLE); (2) persistently aPL-positive without SLE; and (3) aPL-negative SLE (control). After collecting aPL-related medical history, two 5-mm skin biopsies of livedo were performed on each patient: (1) peripheral (erythematous-violaceous lesion); and (2) central (nonviolaceous area). We stained specimens for phosphorylated protein kinase B (p-AKT) and phosphorylated S6 ribosomal protein (p-S6RP) as mTOR activity markers, CD31 to identify endothelial cells, and Ki-67 to show cellular proliferation. We counted cells in the epidermis and compared mTOR-positive cell counts between peripheral and central samples, and between patient groups, using Freidman test and Wilcoxon signed-rank test.</p><p><strong>Results: </strong>Ten patients with livedo reticularis were enrolled: 4 aPL-positive without SLE (antiphospholipid syndrome [APS] classification met, n = 3), 4 aPL-positive SLE (APS classification met, n = 3), and 2 aPL-negative SLE (control). In all aPL-positive patients, epidermal p-AKT and p-S6RP staining were significantly increased in both peripheral and central skin samples when compared to aPL-negative SLE controls; both were more pronounced in the lower basal layers of epidermis.</p><p><strong>Conclusion: </strong>Our study demonstrates increased mTOR activity in livedoid lesions of aPL-positive patients with or without SLE compared to aPL-negative patients with SLE, with more prominent activity in the lower basal layers of the epidermis. These findings may serve as a basis for further investigating the mTOR pathway in aPL-positive patients.</p>","PeriodicalId":35278,"journal":{"name":"The Journal of rheumatology. Supplement","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"78796513","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ludovic Trefond, Agathe Sauret, Julien Stievenart, Louis Olagne, Benedicte Guelon, Perrine Smets, Olivier Aumaître, Marc André
{"title":"Dr. Trefond et al reply: Giant Cell Arteritis After SARS-CoV-2 Vaccination-Coincidence or Trigger ?","authors":"Ludovic Trefond, Agathe Sauret, Julien Stievenart, Louis Olagne, Benedicte Guelon, Perrine Smets, Olivier Aumaître, Marc André","doi":"10.3899/jrheum.211158","DOIUrl":"10.3899/jrheum.211158","url":null,"abstract":"","PeriodicalId":35278,"journal":{"name":"The Journal of rheumatology. Supplement","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"87958339","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Febuxostat Use and Safety in Patients With Hyperuricemia","authors":"T. Kawada","doi":"10.3899/jrheum.220147","DOIUrl":"https://doi.org/10.3899/jrheum.220147","url":null,"abstract":"I read the paper by Deng et al,1 in which the authors conducted a metaanalysis to evaluate whether febuxostat use increased the risk of developing cardiovascular (CV) events, cardiac death, and all-cause mortality. The adjusted relative risk (RR) of febuxostat use for all-cause mortality was 0.87 (95% CI 0.57-1.32).","PeriodicalId":35278,"journal":{"name":"The Journal of rheumatology. Supplement","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-06-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"78087288","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Dr. Deng et al reply","authors":"Hao Deng, X. Yang, H. Jin","doi":"10.3899/jrheum.220179","DOIUrl":"https://doi.org/10.3899/jrheum.220179","url":null,"abstract":"We sincerely appreciate Dr. Kawada's comments in response to our metaanalysis.1 We agree with most of these opinions and would like to clarify our views here.","PeriodicalId":35278,"journal":{"name":"The Journal of rheumatology. Supplement","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-06-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"88534387","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"The Patient Experience of Drug Side Effects in Rheumatoid Arthritis: Intriguing Data From an Exploratory Online Survey","authors":"John M. Davis","doi":"10.3899/jrheum.220412","DOIUrl":"https://doi.org/10.3899/jrheum.220412","url":null,"abstract":"Understanding adverse events (AEs) of disease-modifying antirheumatic drugs (DMARDs) for treatment of rheumatoid arthritis (RA) is critical to both patients and clinicians. AEs-\"side effects\" from the patient perspective-contribute significantly to patients' disease experience by interfering with activities of daily living and quality of life (QOL).1.","PeriodicalId":35278,"journal":{"name":"The Journal of rheumatology. Supplement","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-06-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"88370109","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
J. Hwang, Sanghoon Lee, J. Shin, Ki Woong Kim, H. Gong
{"title":"The Association of Index-to-Ring Finger Ratio With Trapeziometacarpal Joint Osteoarthritis in an Elderly Korean Population","authors":"J. Hwang, Sanghoon Lee, J. Shin, Ki Woong Kim, H. Gong","doi":"10.3899/jrheum.211355","DOIUrl":"https://doi.org/10.3899/jrheum.211355","url":null,"abstract":"Objective Index-to-ring finger ratio (IRFR) has been reported to be associated with joint osteoarthritis (OA). We aimed to evaluate the association between IRFR and trapeziometacarpal joint (TMCJ) OA in an elderly Korean population. Methods A population-based sample included 604 participants with a mean age of 74.8 years. IRFR was radiographically measured by the ratio of the length of the right second to fourth phalangeal bones (“phalangeal IRFR”) and metacarpal bones (“metacarpal IRFR”), and was visually classified as either type 1 (index finger longer than or equal to ring finger) or type 2 (index finger shorter than ring finger). Odds ratios (ORs) for the presence of OA (Kellgren-Lawrence [KL] grade > 1) and for severe OA (KL grade > 2) were analyzed using logistic regression. Results The phalangeal IRFR averaged 91.3%, the metacarpal IRFR 116.7%, and 304 out of 604 participants (50.3%) had type 2 IRFR. We found TMCJ OA in 112 participants (18.5%), and severe TMCJ OA in 33 participants (5.5%). Presence of TMCJ OA was significantly associated with age (OR 1.04; 95% CI 1.01-1.06) and metacarpal IRFR (OR 0.94; 95% CI 0.88-0.99), and severe TMCJ OA with age (OR 1.08; 95% CI 1.03-1.12) and type 2 IRFR (OR 3.07; 95% CI 1.13-8.33). Conclusion Radiographic IRFR, specifically metacarpal IRFR, was associated with the presence of TMCJ OA, and visual IRFR with severe TMCJ OA in both elderly Korean men and women. The results of this study suggest that IRFR might serve as an easily measurable biomarker to identify patients vulnerable to TMCJ OA.","PeriodicalId":35278,"journal":{"name":"The Journal of rheumatology. Supplement","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-06-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"90201892","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
B. Glintborg, D. Jensen, L. Terslev, O. Hendricks, M. Østergaard, S. Rasmussen, M. P. Jensen, T. Adelsten, A. Colic, K. Danebod, M. Kildemand, A. Loft, H. Munk, J. K. Pedersen, R. Østgård, C. M. Sørensen, N. Krogh, J. Agerbo, C. Ziegler, M. Hetland
{"title":"Long-term Behavioral Changes During the COVID-19 Pandemic and Impact of Vaccination in Patients With Inflammatory Rheumatic Diseases","authors":"B. Glintborg, D. Jensen, L. Terslev, O. Hendricks, M. Østergaard, S. Rasmussen, M. P. Jensen, T. Adelsten, A. Colic, K. Danebod, M. Kildemand, A. Loft, H. Munk, J. K. Pedersen, R. Østgård, C. M. Sørensen, N. Krogh, J. Agerbo, C. Ziegler, M. Hetland","doi":"10.3899/jrheum.211280","DOIUrl":"https://doi.org/10.3899/jrheum.211280","url":null,"abstract":"Objective To explore anxiety and self-isolation in patients with inflammatory rheumatic disease (IRD)15 months into the coronavirus disease 2019 (COVID-19) pandemic, including attitudes toward and effects of SARS-CoV-2 vaccination. Methods A nationwide online survey was conducted at 3 timepoints: May 2020, November 2020, and May 2021. Patients with IRD followed in the Danish Rheumatology Quality Registry (DANBIO) were asked about the effects of the pandemic, including SARS-CoV-2 infection and their behavior, anxiety, and concerns. The May 2021 survey included attitudes toward SARS-CoV-2 and influenza vaccination. Characteristics associated with self-isolation in May 2021 were explored with adjusted logistic regression analyses that included patient characteristics and SARS-CoV-2 vaccination status. Results Respondents to surveys 1, 2, and 3 included 12,789; 14,755; and 13,921 patients, respectively; 64% had rheumatoid arthritis and 63% were female. Anxiety and concerns were highest in May 2020 and decreased to stable levels in November 2020 and May 2021; 86%, 50%, and 52% of respondents reported self-isolation, respectively. In May 2021, 4% of respondents self-reported previous SARS-CoV-2 infection. The SARS-CoV-2 vaccine acceptance rate was 86%, and the proportion of patients vaccinated against influenza had increased from 50% in winter 2019-2020 to 64% in winter 2020-2021. The proportion of patients with anxiety appeared similar among those vaccinated and unvaccinated against SARS-CoV-2. In multivariable analyses, being unvaccinated, female gender, receiving biologic drugs, and poor quality of life were independently associated with self-isolation. Conclusion Levels of anxiety and self-isolation decreased after the initial lockdown period in patients with IRD. Half of the patients reported self-isolation in May 2021, a phase that included widespread reopening of society and large-scale vaccination. The lack of prepandemic data prevented a full understanding of the long-term effects of the pandemic on anxiety and self-isolation in patients with IRD.","PeriodicalId":35278,"journal":{"name":"The Journal of rheumatology. Supplement","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-06-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"81540457","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}