International Journal of Rheumatic Diseases最新文献

{"title":"Clinical Manifestations and Treatment Response of Patients With Syndrome of Undifferentiated Recurrent Fever (SURF)","authors":"Emma Longoni, Riccardo Papa, Francesca Bovis, Bianca Laura Cinicola, Riccardo Castagnoli, Caterina Cancrini, Francesca Conti, Silvia Federici, Anna Bratta, Giuliana Giardino, Lucia Leonardi, Vassilios Lougaris, Maria Sangerardi, Annarosa Soresina, Gian Luigi Marseglia, Michele Miraglia Del Giudice, Marco Gattorno, Fabio Cardinale, the Immunology Task Force of the Italian Society of Pediatric Allergy and Immunology (SIAIP)","doi":"10.1111/1756-185X.70246","DOIUrl":"https://doi.org/10.1111/1756-185X.70246","url":null,"abstract":"<p>Systemic autoinflammatory diseases (AIDs) are a heterogeneous group of inborn errors of immunity characterized by episodes of sterile inflammation without evidence of pathogenic autoantibodies or autoreactive T lymphocytes [<span>1</span>]. The most known manifestation of AIDs is recurrent fever, often associated with inflammatory involvement of joints, brain, eyes, skin, and serous membranes. Peripheral serum inflammatory markers, as C reactive protein and serum amyloid A, always increase during attacks [<span>2</span>]. AIDs are rare (incidence of < 1/2000 individuals) [<span>3</span>], although they are increasingly recognized worldwide. Most AIDs are caused by a single genetic variant, although some may result from somatic mosaicism or low-penetrance variants [<span>4, 5</span>]. Genetic testing is necessary for molecular diagnosis, which is confirmed in patients with typical symptoms and pathogenic variants [<span>1</span>].</p><p>Among patients with clinical signs of AIDs, at least 40% have no molecular diagnosis, and approximately 50% have no pathogenic variants in AID-related genes analyzed with targeted next-generation sequencing panels [<span>6</span>]. Most of these patients meet classification criteria for periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome [<span>7</span>]. PFAPA syndrome is the most common polygenic AID, characterized by recurrent episodes of oral ulcers, bilateral cervical lymphadenopathy, and/or exudative tonsillitis. PFAPA syndrome has a self-limiting clinical course, with remission during late childhood. In cases of frequent and/or severe inflammatory attacks that cause significant discomfort to the patient, tonsillectomy is suggested, with a high rate of efficacy (75%–90%). Diagnostic criteria for PFAPA syndrome have been published elsewhere [<span>8</span>].</p><p>Another group of patients presenting with recurrent fever and negative or inconclusive genetic tests for AIDs has been classified under the term syndrome of undifferentiated recurrent fever (SURF) [<span>9</span>]. SURF patients do not carry variants in monogenic AIDs-associated genes and do not fulfill the diagnostic criteria for PFAPA syndrome or other inherited recurrent fever syndromes. Despite evolving knowledge of the molecular mechanisms underlying AIDs, it remains unclear whether SURF represents a distinct clinical entity with complex, polygenic inheritance or a heterogeneous group of patients with similar manifestations and what is the most effective treatment for these patients. In this study, we performed a systematic review with meta-analysis to highlight the clinical features and the best treatment options in SURF patients.</p><p>SURF is an umbrella clinical entity recently included in AIDs and defined by recurrent fever attacks without any overt infectious disease, absent criteria for PFAPA syndrome, and negative genetic tests for monogenic AIDs. This clinical and laboratory classification of exclusion ","PeriodicalId":14330,"journal":{"name":"International Journal of Rheumatic Diseases","volume":"28 5","pages":""},"PeriodicalIF":2.4,"publicationDate":"2025-05-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/1756-185X.70246","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143914246","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Case Report: Janus Kinase Inhibitors and Tumor Necrosis Factor-α Inhibitors Dual Therapy Is a Potential Treatment Strategy for Difficult-To-Treat Adult-Onset Still's Disease","authors":"Xiuying Lv, Xiaomei Liu, Guixiu Shi, Yan Li","doi":"10.1111/1756-185X.70237","DOIUrl":"https://doi.org/10.1111/1756-185X.70237","url":null,"abstract":"","PeriodicalId":14330,"journal":{"name":"International Journal of Rheumatic Diseases","volume":"28 5","pages":""},"PeriodicalIF":2.4,"publicationDate":"2025-05-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143914255","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":"A Crystalline Mimic of Patellar Tendon Tumor","authors":"Peter Xie, Tristan Rutland, David Massasso","doi":"10.1111/1756-185X.70265","DOIUrl":"https://doi.org/10.1111/1756-185X.70265","url":null,"abstract":"","PeriodicalId":14330,"journal":{"name":"International Journal of Rheumatic Diseases","volume":"28 5","pages":""},"PeriodicalIF":2.4,"publicationDate":"2025-05-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143914517","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":"Comment on “Exploration of Circadian Clock-Related Genes in the Pathogenesis of Psoriatic Arthritis to Identify Potential Therapeutic Targets From Multi-Omics Insight: A Mendelian Randomization Study”","authors":"Ranjana Sah, Rachana Mehta, Shubham Kumar","doi":"10.1111/1756-185X.70270","DOIUrl":"https://doi.org/10.1111/1756-185X.70270","url":null,"abstract":"","PeriodicalId":14330,"journal":{"name":"International Journal of Rheumatic Diseases","volume":"28 5","pages":""},"PeriodicalIF":2.4,"publicationDate":"2025-05-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143914245","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":"Organ Damage and Associated Factors in Chinese Patients With Systemic Lupus Erythematosus","authors":"Jiaying Zhang,&nbsp;Liujun Wang,&nbsp;Dai Gao,&nbsp;Yong Fan,&nbsp;LanLan Ji,&nbsp;Zhuoli Zhang","doi":"10.1111/1756-185X.70268","DOIUrl":"https://doi.org/10.1111/1756-185X.70268","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>To explore organ damages and factors associated with the occurrence of organ damages in Chinese patients with systemic lupus erythematosus (SLE).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>A cross-sectional study was performed in SLE patients with a disease duration of over 6 months at enrollment. The demographics, clinical characteristics, and medication history were collected. The profile of organ damage measured by the SLE damage index (SDI) at enrollment was displayed. The factors associated with the occurrence of organ damage were analyzed by logistic regression analysis.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Three hundred and fifty patients were enrolled in this study. Their mean age was 38.8 ± 13.1 years, and the median disease duration was 7.0 (2.0, 14.0) years. There were 124 (35.5%) patients with organ damage at enrollment. The damage was most frequently located in ocular (12.8%), followed by musculoskeletal (9.4%), gonads (5.7%), neuropsychiatric (4.8%), and lungs (4.6%). The top five prevalent manifestations were cataract (10.5%), avascular osteonecrosis (6.5%), premature gonadal failure (5.7%), pulmonary hypertension (3.4%) and chronic alopecia (3.4%). In logistic analysis, age (OR = 1.06, 95% CI 1.03, 1.08, <i>p</i> &lt; 0.001), disease duration (OR = 1.09, 95% CI 1.05, 1.13, <i>p</i> &lt; 0.001), current/past use of mycophenolate (OR = 2.66, 95% CI 1.49, 4.75, <i>p</i> = 0.001), SLE disease activity index 2000 (SLEDAI-2K) at enrollment (OR = 1.06, 95% CI 1.00, 1.11, <i>p</i> = 0.035) were independent risk factors of organ damage, while current/past use of hydroxychloroquine (OR = 0.20, 95% CI 0.06, 0.66, <i>p</i> = 0.009) was an independent protective factor.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>Organ damage is frequent in Chinese SLE patients. Disease duration, SLEDAI-2K at enrollment, and use of mycophenolate were risk factors, while use of hydroxychloroquine was a protective factor of organ damage. Optimized management is imperative to protect ocular, musculoskeletal, and gonads from damage in clinical practice in the future.</p>\u0000 </section>\u0000 </div>","PeriodicalId":14330,"journal":{"name":"International Journal of Rheumatic Diseases","volume":"28 5","pages":""},"PeriodicalIF":2.4,"publicationDate":"2025-05-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143914253","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":"Comparison of Outcomes Between Japanese Patients With Older-Onset Granulomatosis With Polyangiitis/Microscopic Polyangiitis and Younger-Onset Patients in Daily Clinical Practice: A Two-Center Retrospective Study in Japan","authors":"Shuzo Sato,&nbsp;Makiko Yashiro-Furuya,&nbsp;Yuya Sumichika,&nbsp;Kenji Saito,&nbsp;Shuhei Yoshida,&nbsp;Haruki Matsumoto,&nbsp;Jumpei Temmoku,&nbsp;Yuya Fujita,&nbsp;Naoki Matsuoka,&nbsp;Tomoyuki Asano,&nbsp;Eiji Suzuki,&nbsp;Takashi Kanno,&nbsp;Kiyoshi Migita","doi":"10.1111/1756-185X.70256","DOIUrl":"https://doi.org/10.1111/1756-185X.70256","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Aims</h3>\u0000 \u0000 <p>Although previous studies have reported poor outcomes in older-onset (≥ 75 years old) antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) compared with younger-onset AAV, the distinct cause of poor prognosis remains unclear. This study aimed to investigate the clinical features, therapies, and outcomes of older patients with granulomatosis with polyangiitis (GPA) and patients with microscopic polyangiitis (MPA) compared to younger-onset patients.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>This two-center retrospective cohort study enrolled 70 newly-onset Japanese patients with AAV (GPA and MPA) from the Fukushima Medical University Hospital and Ohta-Nishinouchi Hospital in Fukushima, Japan, between 2004 and 2019. Clinical records were retrospectively reviewed, and clinical features and outcomes (1-year and 3-year survival by the Kaplan–Meier method) were compared between older and younger GPA/MPA groups, respectively.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Clinical features of the older GPA/MPA group were similar to those of the younger GPA/MPA group; however, the older GPA group showed severe inflammation and the older MPA group had an increased frequency of renal involvement and fever. The 1-year survival in the older MPA group was significantly lower than that in the younger MPA group. Immunosuppressive therapy including cyclophosphamide, rituximab, and other immunosuppressive agents was important to sustain the survival of patients with GPA/MPA.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Older patients with GPA/MPA may have specific clinical features; careful observation is needed during the treatment of older patients with MPA. Immunosuppressive therapy may improve the prognosis of patients with AAV.</p>\u0000 </section>\u0000 </div>","PeriodicalId":14330,"journal":{"name":"International Journal of Rheumatic Diseases","volume":"28 5","pages":""},"PeriodicalIF":2.4,"publicationDate":"2025-05-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143914254","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":"Correction to “Poster Abstracts Part B. APLAR 26th Asia-Pacific League of Associations for Rheumatology Congress, 21–25 August 2024”","authors":"","doi":"10.1111/1756-185X.70164","DOIUrl":"https://doi.org/10.1111/1756-185X.70164","url":null,"abstract":"&lt;p&gt;(2024), Poster Abstracts Part B. Int J Rheum Dis, 27(S3): e15346. https://doi.org/10.1111/1756-185X.15346&lt;/p&gt;&lt;p&gt;The following abstract should be added.&lt;/p&gt;&lt;p&gt;&lt;b&gt;Real-world Insights on Tofacitinib in Ankylosing Spondylitis Amongst Indian Rheumatologists: JOINT Survey&lt;/b&gt;.&lt;/p&gt;&lt;p&gt;&lt;span&gt;PD Rath&lt;/span&gt;&lt;sup&gt;&lt;span&gt;1&lt;/span&gt;&lt;/sup&gt;, Ashit Syngle&lt;sup&gt;2&lt;/sup&gt;, Shyamashis Das&lt;sup&gt;3&lt;/sup&gt;, Pravin Patil&lt;sup&gt;4&lt;/sup&gt;, Prasanta Padhan&lt;sup&gt;5&lt;/sup&gt;, Ronak Bhuptani&lt;sup&gt;6&lt;/sup&gt;, Namita Ghag Wadkar&lt;sup&gt;6&lt;/sup&gt; and Sucheta Mehta&lt;sup&gt;6&lt;/sup&gt;.&lt;/p&gt;&lt;p&gt;\u0000 &lt;b&gt;Author Affiliations:&lt;/b&gt;\u0000 &lt;/p&gt;&lt;p&gt;&lt;sup&gt;1&lt;/sup&gt;Director and Head of Rheumatology department, Max Super Speciality Hospital, New Delhi, India.&lt;/p&gt;&lt;p&gt;&lt;sup&gt;2&lt;/sup&gt;Consultant Rheumatologist, Fortis Hospital, Mohali, India.&lt;/p&gt;&lt;p&gt;&lt;sup&gt;3&lt;/sup&gt;Consultant Rheumatologist, Institute of Neurosciences, Kolkata, India.&lt;/p&gt;&lt;p&gt;&lt;sup&gt;4&lt;/sup&gt;Consultant Rheumatologist, Deenanath Mangeshkar Hospital&amp; Research Center, Pune, India.&lt;/p&gt;&lt;p&gt;&lt;sup&gt;5&lt;/sup&gt;Professor, Department of Clinical Immunology and Rheumatology, Kalinga Institute of Medical Sciences, KIIT University, Bhubaneswar, India.&lt;/p&gt;&lt;p&gt;&lt;sup&gt;6&lt;/sup&gt;Medical Affairs, Cipla Ltd., Mumbai, India.&lt;/p&gt;&lt;p&gt;The wider availability of tofacitinib, a Janus kinase inhibitor (JAKi), has created a paradigm shift in the management of rheumatoid arthritis (RA) in resource-limited settings like India. Real-world data on effectiveness and safety of tofacitinib in ankylosing spondylitis (AS) in the Indian scenario is scarce. The present study aimed to evaluate clinical practice and treatment patterns with tofacitinib in RA and AS amongst Indian rheumatologists.&lt;/p&gt;&lt;p&gt;An online questionnaire-based, nationwide survey was conducted amongst rheumatologists. The internally validated questionnaire included a set of 28 questions to gauge the real-world practices with tofacitinib. This sub-analysis focused on the treatment patterns with tofacitinib in AS.&lt;/p&gt;&lt;p&gt;A total of 107 rheumatologists who dealt with~51–100 AS patients/month across academic institutions, corporate hospitals, and private clinics were included. Short courses of non-steroidal anti-inflammatory drugs (NSAIDs) are primarily used for initial treatment and managing acute flares in AS. Rheumatologists preferred to initiate TNFα inhibitors (36.45%) or sulfasalazine (37.38%) in AS patients post inadequate response to NSAIDs. Interestingly, 39% rheumatologists ranked JAKi viz. tofacitinib as the next preferred option (Figure 1) and 51% have used tofacitinib as monotherapy in AS. Notably, 87% rheumatologists preferred tapering tofacitinib dosage once AS patients achieve low disease activity/remission with down-titration usually to 5 mg OD. Extended-release formulation of tofacitinib (11 mg OD) was preferred by 23% rheumatologists with 75% reporting improved patient compliance (Figure 2A and 2B). More than 70% of rheumatologists rated tofacitinib as good to excellent and 27% rated it as moderate for its efficacy and safety in AS (","PeriodicalId":14330,"journal":{"name":"International Journal of Rheumatic Diseases","volume":"28 5","pages":""},"PeriodicalIF":2.4,"publicationDate":"2025-05-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/1756-185X.70164","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143905206","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Correction to “Poster Abstracts Part A. APLAR 26th Asia-Pacific League of Associations for Rheumatology Congress, 21–25 August 2024”","authors":"","doi":"10.1111/1756-185X.70165","DOIUrl":"https://doi.org/10.1111/1756-185X.70165","url":null,"abstract":"<p>(2024), Poster Abstracts Part A. Int J Rheum Dis, 27(S3): e15345. https://doi.org/10.1111/1756-185X.15345</p><p>We apologize for the errors.</p>","PeriodicalId":14330,"journal":{"name":"International Journal of Rheumatic Diseases","volume":"28 5","pages":""},"PeriodicalIF":2.4,"publicationDate":"2025-05-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/1756-185X.70165","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143905219","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Basic Science Free Papers","authors":"","doi":"10.1111/1756-185X.70172","DOIUrl":"https://doi.org/10.1111/1756-185X.70172","url":null,"abstract":"","PeriodicalId":14330,"journal":{"name":"International Journal of Rheumatic Diseases","volume":"28 S1","pages":""},"PeriodicalIF":2.4,"publicationDate":"2025-05-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143901039","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":"Nursing/Allied Health Posters","authors":"","doi":"10.1111/1756-185X.70262","DOIUrl":"https://doi.org/10.1111/1756-185X.70262","url":null,"abstract":"","PeriodicalId":14330,"journal":{"name":"International Journal of Rheumatic Diseases","volume":"28 S1","pages":""},"PeriodicalIF":2.4,"publicationDate":"2025-05-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143900874","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}