Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases最新文献

{"title":"Mucosa-associated Lymphoid Tissue Lymphoma Presenting With Immunoglobulin G4-related Disease-like Features.","authors":"Hirotaka Yamamoto, Taniguchi Yoshinori","doi":"10.1097/RHU.0000000000002286","DOIUrl":"https://doi.org/10.1097/RHU.0000000000002286","url":null,"abstract":"","PeriodicalId":520664,"journal":{"name":"Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases","volume":" ","pages":""},"PeriodicalIF":1.8,"publicationDate":"2025-10-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145246295","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":"Delay in Referral, Diagnosis, and Treatment in Pediatric Patients With Juvenile Systemic Lupus Erythematosus: A Systematic Review.","authors":"Marcela Beatriz Álvarez, Adolfo G Hernandez-Garduno, Ana Victoria Villarreal-Treviño, Virginia Ramírez-Nova, Alfonso Gastelum-Strozzi, Ingris Peláez-Ballestas, Nadina Rubio-Pérez, Fernando García-Rodríguez","doi":"10.1097/RHU.0000000000002284","DOIUrl":"10.1097/RHU.0000000000002284","url":null,"abstract":"<p><strong>Background/objective: </strong>The delay in managing patients with juvenile systemic lupus erythematosus (jSLE) is one of the most important determinants impacting outcomes.</p><p><strong>Methods: </strong>We conducted a systematic review regarding the delay in referral, diagnosis, and treatment of patients with jSLE, and the barriers and facilitators related to these processes. Electronic searches were conducted in Scopus, PubMed, and Web of Science for studies published up to March 4, 2025; additionally, reports were identified through a citation search. The project followed the PRISMA guidelines, and the critical appraisal was based on the Joanna Briggs Institute Checklist (JBI). Meta-analyses, using random effects models, were conducted to estimate the delay.</p><p><strong>Results: </strong>The review included 24 papers from Europe, Asia, the Americas, and Africa. The median JBI quality score was 5 (IQR=4.75 to 6). Only one study presented a definition for the delay to diagnosis of jSLE. The estimated mean time from onset to diagnosis was 3.5 months (95% CI=2.73-4.27, I2 =93.4%, p <0.0001). Only 3 studies reported a referral delay. Two studies indicated that all patients received therapy at diagnosis. There was no difference in the time to diagnosis between countries from the Global North and the Global South. The most common barriers identified for timely diagnosis were male sex, low anti-nuclear antibody titers, low family income, and patients presenting mild clinical manifestations.</p><p><strong>Conclusions: </strong>Currently, no consensus exists on defining the delays in referral, diagnosis, or treatment for jSLE patients. Common barriers are related to both sociodemographic and clinical factors.</p>","PeriodicalId":520664,"journal":{"name":"Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases","volume":" ","pages":""},"PeriodicalIF":1.8,"publicationDate":"2025-10-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145208883","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":"Real-world Use of Canakinumab in Familial Mediterranean Fever and Other Autoinflammatory Disorders: A Medical Records Review Single-center Study From Turkey.","authors":"Rabia Deniz, Oya Altun, Ceren Tansu Yavuz, Sezgi Karabulut Gök, Ferdanur Deniz, Hatice Kübra Yerişenoğlu Demir, Rumeysa Mirza Altintaş, Kübra Uğur, Ayşe Elif Boncukcuoğlu, Bilgin Karaalioğlu, Duygu Sevinç Özgür, Gamze Akkuzu, Fatih Yildirim, Can Erzik, Cemal Bes","doi":"10.1097/RHU.0000000000002285","DOIUrl":"https://doi.org/10.1097/RHU.0000000000002285","url":null,"abstract":"<p><strong>Background/objective: </strong>Canakinumab (CAN), a monoclonal antibody targeting interleukin-1β, has demonstrated efficacy in various autoinflammatory diseases (AIDs), particularly in inadequate response to colchicine in familial Mediterranean fever (FMF). This study aimed to evaluate the indications, efficacy, and safety of CAN based on real-life experience from a tertiary rheumatology clinic.</p><p><strong>Methods: </strong>This single-center study included 54 patients treated with CAN between May 2020 and September 2024. Patients were grouped as MEFV-positive FMF (n=42), MEFV-negative FMF (n=7), non-FMF autoinflammatory diseases (n=2), and adult-onset Still's disease (AOSD; n=3). Demographic and clinical data, treatment indications, response patterns, laboratory parameters, and adverse events were analyzed.</p><p><strong>Results: </strong>CAN was initiated mainly due to adverse effects (40.5%) or inadequate response (42.8%) to anakinra and colchicine. The median duration of CAN therapy was 22 months. Among MEFV-positive FMF patients, 81% achieved a complete response and 19% partial response. CAN significantly reduced attack frequency and duration, and improved inflammatory markers (CRP, ESR, WBC, and neutrophil count). Proteinuria decreased in a statistically significant but clinically modest manner following CAN treatment. Only 1 patient experienced reversible cytopenia. Dose intervals were successfully prolonged in 54.8% of MEFV-positive patients without loss of efficacy.</p><p><strong>Conclusions: </strong>Canakinumab is an effective and well-tolerated IL-1β inhibitor in FMF and other AIDs, particularly in patients who are inadequately responsive or intolerant to colchicine and anakinra. Real-world experience supports its sustained efficacy and the feasibility of dose interval extension in selected cases.</p>","PeriodicalId":520664,"journal":{"name":"Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases","volume":" ","pages":""},"PeriodicalIF":1.8,"publicationDate":"2025-10-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145208948","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":"Therapeutic Choices in Systemic Sclerosis-Associated Interstitial Lung Disease, a Survey of 2 International Research Groups.","authors":"Michael Macklin, Iazsmin Bauer Ventura, Dinesh Khanna","doi":"10.1097/RHU.0000000000002249","DOIUrl":"10.1097/RHU.0000000000002249","url":null,"abstract":"<p><strong>Background: </strong>Options for systemic sclerosis-associated interstitial lung disease (SSc-ILD) have evolved rapidly. Mycophenolate mofetil (MMF) has replaced cyclophosphamide (CYC) in most cases of SSc-ILD, with the recent addition of tocilizumab (TCZ) in SSc-ILD as well. Combination immunosuppressive (CI) therapy with rituximab (RTX) and MMF, along with the antifibrotic nintedanib, have also become options. We aimed to better understand prescribing patterns and examine treatment trends overall to examine guideline penetrance.</p><p><strong>Methods: </strong>A survey polling international SSc experts was conducted from October 2023 through March 2024 by members of the Scleroderma Clinical Trials Consortium and the European Scleroderma Trials and Research Group.</p><p><strong>Results: </strong>MMF was the most common first-line treatment (92%) for SSc-ILD, followed by a split preference for RTX or TCZ for second/third line. Most experts add an antifibrotic (57%) or use CI therapy (24%) with failure of initial therapy. When CI therapy is used, MMF/RTX is used most (71%), followed by MMF/TCZ (38%). Corticosteroids were used for SSc-ILD treatment by 36% of experts, with 12% of these respondents using greater than 20 mg of prednisone equivalent. The survey response rate was 17.4% of total centers and 7.7% of total experts.</p><p><strong>Conclusion: </strong>First-line treatment preferences are in line with current treatment guidelines. CI therapy is not typically used, although the EVER-ILD trial might have influenced prescribing patterns with RTX/MMF CI therapy more typical. Prednisone use was more common than expected. Further studies evaluating combination MMF/TCZ versus MMF/RTX and whether TCZ is effective for SSc-ILD in patients without an inflammatory laboratory profile are necessary to help guide clinical practice. Further adoption of current guidelines may also change prednisone use patterns.</p>","PeriodicalId":520664,"journal":{"name":"Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases","volume":" ","pages":"284-287"},"PeriodicalIF":1.8,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144259830","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":"Ulcerative Holster Sign in Transcriptional Intermediary Factor 1γ-Positive Dermatomyositis.","authors":"Eaman Alhassan","doi":"10.1097/RHU.0000000000002271","DOIUrl":"10.1097/RHU.0000000000002271","url":null,"abstract":"","PeriodicalId":520664,"journal":{"name":"Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases","volume":" ","pages":"e166"},"PeriodicalIF":1.8,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144860071","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":"Clinical Characteristics of Antinuclear Antibody-Negative Systemic Lupus Erythematosus: A Systematic Review.","authors":"Fernando Antonio Glasner da Rocha Araujo, Isabella Vargas de Souza Lima, Mittermayer Barreto Santiago","doi":"10.1097/RHU.0000000000002258","DOIUrl":"10.1097/RHU.0000000000002258","url":null,"abstract":"<p><strong>Abstract: </strong>Although antinuclear antibody (ANA) positivity on HEp-2 cells is a mandatory criterion for the classification of systemic lupus erythematosus (SLE), patients with ANA-negative SLE may still present with severe clinical manifestations. However, this subgroup of patients has been rarely studied, and the most relevant publications are limited to isolated case reports. This systematic review aimed to identify the clinical features and other potential markers (autoantibodies) that may aid in the identification of ANA-negative SLE. The review followed the PRISMA (Preferred Reporting Items for Systematic reviews and Meta-Analyses) guidelines. The PICOS criteria were as follows: population (patients diagnosed with SLE), intervention (ANA panel testing), comparison (ANA-positive vs. ANA-negative patients), outcome (clinical manifestations and other autoantibodies), and study design (prospective and retrospective primary studies, including case series and case reports). One hundred eighty-eight publications were initially identified. After screening, 21 studies (152 patients) were included in the final analysis. Cutaneous was the most common clinical manifestation observed in patients with ANA-negative SLE, followed by photosensitivity and joint complaints. The most commonly detected autoantibodies were anti-SSA/Ro and anti-dsDNA. To our knowledge, this is the first comprehensive systematic review of the clinical and laboratory characteristics of patients with ANA-negative SLE.</p>","PeriodicalId":520664,"journal":{"name":"Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases","volume":" ","pages":"275-278"},"PeriodicalIF":1.8,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144602794","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":"Global Guidelines for Psoriatic Arthritis Management: A Comparative Analysis.","authors":"Shikha Singla, Parjanya Bhatt, Enrique Roberto Soriano","doi":"10.1097/RHU.0000000000002254","DOIUrl":"10.1097/RHU.0000000000002254","url":null,"abstract":"<p><strong>Background: </strong>Psoriatic arthritis (PsA) is a complex immune-mediated heterogeneous inflammatory disease. Treatment decisions are challenging given the multisystem involvement. To further guide management strategies, we conducted a comparative analysis of the latest global guidelines highlighting the contrast in their approach to treat different PsA domains.</p><p><strong>Methods: </strong>Major global guidelines for PsA management were reviewed, including American College of Rheumatology 2018 update, European Alliance of Associations for Rheumatology 2023 update, British Society of Rheumatology 2022, Group for Research and Assessment of Psoriasis and Psoriatic Arthritis 2021, and Pan American League of Associations for Rheumatology 2024.</p><p><strong>Results: </strong>The guidelines unanimously recommend a treat-to-target strategy with a focus on active PsA. Divergence existed in treatment sequencing regarding the use of biologic and targeted disease-modifying antirheumatic drugs (DMARDs). Variations were also noted in the management of enthesitis and dactylitis. Addressing comorbidities and associated conditions is regarded to be a cornerstone for optimizing disease control and preventing flares.</p><p><strong>Conclusion: </strong>This review highlights the different management strategies among the global guidelines. Furthermore, we pointed at promising new therapeutic targets that are likely to be incorporated into future recommendations.</p>","PeriodicalId":520664,"journal":{"name":"Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases","volume":" ","pages":"269-274"},"PeriodicalIF":1.8,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144602795","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":"Heliotrope Rash That Isn't Dermatomyositis.","authors":"Eaman Alhassan, Rohit Aggarwal","doi":"10.1097/RHU.0000000000002273","DOIUrl":"10.1097/RHU.0000000000002273","url":null,"abstract":"","PeriodicalId":520664,"journal":{"name":"Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases","volume":" ","pages":"e168"},"PeriodicalIF":1.8,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144860068","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":"Epidemiological Trends and Future Projections of Osteoarthritis in Mexico: Findings From the Global Burden of Disease Study 2021.","authors":"Pamela Munguía-Realpozo, Claudia Mendoza-Pinto, Ivet Etchegaray-Morales, Eduardo Jiménez-Jiménez, Óscar García-Pérez, Edith Ramírez-Lara, Rolando Espinosa-Morales, Jorge Ayón-Aguilar, Socorro Méndez-Martínez, Álvaro Montiel-Jarquín","doi":"10.1097/RHU.0000000000002234","DOIUrl":"10.1097/RHU.0000000000002234","url":null,"abstract":"<p><strong>Objective: </strong>This study seeks to assess the temporal patterns of osteoarthritis (OA) in Mexico from 1990 to 2021 using the Global Burden of Disease 2021 study and to project future trends over the next 19 years.</p><p><strong>Methods: </strong>Age-standardized rate data for the prevalence (age-standardized prevalence rate [ASPR]), incidence (age-standardized incidence rate [ASIR]), disability-adjusted life year, and years lived with disability of OA in Mexico were extracted. Temporal trends were assessed using the average annual percentage change as a statistical measure. The metrics were forecast to 2040 with a mixed-effects model.</p><p><strong>Results: </strong>The ASPR and the ASIR of OA increased from 6890 (95% uncertainty interval [UI], 6126-7624) and 549 (95% UI, 485-609) in 1990 to 8647 (95% UI, 6919-8647) in 2021 per 100,000 and 617 (95% UI, 545-681), respectively. Similarly, age-standardized disability-adjusted life years increased from 240 (95% UI, 115-485) to 277 (95% UI, 133-559) per 100,000 from 1990 to 2021. Knee OA was the predominant form of OA, followed by hand OA. Joinpoint regression analysis showed significant increases in ASPR and ASIR from 1990 to 2021 (average annual percentage change, 0.4% [95% confidence interval {CI}, 0.3-0.5; p  < 0.001] and 0.4% [95% CI, 0.3-0.4; p  < 0.001], respectively). The forecasted ASPR of OA will be 7843.8 (95% CI, 7811.5-7876.0) per 100,000 in 2022 and 8181.3 (95% CI, 6473.6-9782.9) in 2040.</p><p><strong>Conclusion: </strong>The burden of OA in Mexico increased markedly from 1990 to 2021, with knee OA emerging as the primary contributor amid significant interstate disparities. Demographic shifts and rising life expectancy signal a continued increase in OA burden. These findings call for targeted public health policies and enhanced health care capacity to address emerging challenges across Mexico.</p>","PeriodicalId":520664,"journal":{"name":"Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases","volume":" ","pages":"e142-e149"},"PeriodicalIF":1.8,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144218510","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":"HALP Score for Predicting Renal Relapse in Lupus Nephritis: A Nested Case-Control Study.","authors":"Jeny A Marín-Corte, Alfredo Atl Castillo-Sigales, Hilda Fragoso-Loyo, Erik Cimé-Aké","doi":"10.1097/RHU.0000000000002244","DOIUrl":"10.1097/RHU.0000000000002244","url":null,"abstract":"<p><strong>Objective: </strong>To evaluate the HALP (hemoglobin-albumin-lymphocyte-platelet) score at lupus nephritis (LN) diagnosis in predicting renal relapse (RR).</p><p><strong>Methods: </strong>Nested case-control study, including patients aged ≥18 years with diagnosis of LN between 2010 and 2022. Two patient sets were included: training and validation. Each set had 2 groups of patients: RR and non-RR. Data were obtained from clinical records. The optimal cutoff value of the HALP score at LN diagnosis was established to predict RR. Cox regression analysis was used to associate HALP score at diagnosis with RR.</p><p><strong>Results: </strong>We included 53 LN patients in the training set and 74 LN patients in the validation set. The optimal cutoff value for HALP score at diagnosis was 23.5, with an area under the curve of 0.896, sensitivity of 91.9%, and specificity of 97.3% in the validation set. The median age of patients in this set was 31.0 years, mostly female (93%). In the validation set, LN patients with HALP score at diagnosis ≤23.5 compared with higher HALP score subjects showed a significantly higher baseline SLEDAI-2K (18 [interquartile range {IQR}, 14-20] vs. 14 [IQR, 11-17], p  < 0.001), Systemic Lupus Collaborating Clinics/American College of Rheumatology Damage Index at the end of the follow-up (1 [IQR, 0-4] vs. 0 [IQR, 0-1], p  = 0.002), chronicity index in renal biopsy (2 [IQR, 1-4] vs. 1 [IQR, 1-2], p  = 0.030), and significantly reduced time to RR (4.2 vs. 12.9 years, p  < 0.001). A HALP score at diagnosis ≤23.5 was associated with RR (hazard ratio, 18.2; 95% confidence interval, 5.3-30.1; p  < 0.001).</p><p><strong>Conclusion: </strong>A HALP score ≤23.5 at LN diagnosis was an independent risk factor for RR.</p>","PeriodicalId":520664,"journal":{"name":"Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases","volume":" ","pages":"e158-e165"},"PeriodicalIF":1.8,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144164656","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}