Current Rheumatology Reports最新文献

{"title":"Myositis Mimics: Recognizing Red Flags and Diagnostic Pitfalls.","authors":"Bhaskar Roy, Latika Gupta, Teerin Liewluck","doi":"10.1007/s11926-026-01219-x","DOIUrl":"10.1007/s11926-026-01219-x","url":null,"abstract":"<p><strong>Purpose of review: </strong>This review aims to provide clinicians with a practical framework for distinguishing idiopathic inflammatory myopathies (IIMs) from their numerous non–immune-mediated mimics, including hereditary, toxic, metabolic, and endocrine myopathies, by integrating clinical, serologic, imaging, electrophysiologic, and histopathologic clues.</p><p><strong>Recent findings: </strong>IIMs represent a heterogeneous group of immune-mediated muscle diseases. Despite major advances in antibody discovery, imaging, and classification criteria, accurate diagnosis remains challenging because many non-immune-mediated myopathies can mimic IIMs. Such resemblance may lead to misdiagnosis, delay in genetic evaluation, prolonged exposure to offending agents in toxic myopathy, and unnecessary treatment with immunosuppressive therapy that carries significant adverse effects. The temporal course of weakness, pattern of muscle involvement, presence of extramuscular manifestations, and ancillary testing offer important diagnostic clues, but none are pathognomonic when interpreted in isolation. We introduce the mnemonic “MYOSITIS” to summarize key diagnostic red flags that should raise concern for mimicking disorders: Myopathic motor unit potentials without fibrillation potentials, Young age of symptom onset or positive family history, Onset atypical for IIMs, Seronegative or weakly positive myositis specific autoantibodies, Iatrogenic causes, Treatment refractoriness, Irregular weakness patterns, and Systemic features.</p><p><strong>Summary: </strong>Recognizing these pitfalls and adopting an integrated diagnostic approach that combines clinical pattern recognition with selective use of serologic, imaging, and genetic testing can help clinicians differentiate true IIMs from their mimics and ensure timely, accurate diagnosis and optimal patient management.</p>","PeriodicalId":10761,"journal":{"name":"Current Rheumatology Reports","volume":"28 1","pages":""},"PeriodicalIF":4.9,"publicationDate":"2026-04-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147688711","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":"Management of Severe Behçet's Disease.","authors":"Kerem Abacar, Haner Direskeneli, Fatma Alibaz-Oner","doi":"10.1007/s11926-026-01217-z","DOIUrl":"10.1007/s11926-026-01217-z","url":null,"abstract":"<p><p>PURPOSE OF REVIEW: Behçet’s disease (BD) is a multisystemic inflammatory disorder in which treatment decisions are largely driven by the pattern and severity of organ involvement. This review provides a practical, organ-based overview of current therapeutic strategies for severe and refractory BD, with an emphasis on treatment selection and timing in routine clinical practice. RECENT FINDINGS: Recent evidence supports an upfront, intensive treatment approach for life-threatening or damage-prone organ involvement, particularly neurological, vascular, and ocular disease, where delays in inflammation control are closely associated with irreversible damage. Anti–tumour necrosis factor agents form the core of therapy in these settings, supported by the strongest disease-specific data, while other biologic and targeted therapies are increasingly used in refractory cases. In contrast, mucocutaneous and articular manifestations predominantly affect quality of life and are commonly managed with stepwise strategies. Colchicine remains a widely used first-line treatment in routine practice and provides effective control of mucocutaneous and joint manifestations in a substantial proportion of patients, with escalation to additional systemic or targeted therapies guided by persistence, refractoriness, and patient burden. Importantly, treatment responses vary across organ systems, and benefit in one domain cannot be assumed to translate to others, reinforcing the need for organ-specific treatment planning. Optimal management of BD depends on matching treatment intensity to organ-specific risk, prioritising early aggressive therapy for major organ involvement while adopting proportionate, stepwise approaches for non–life-threatening disease. Improved outcomes are likely to be achieved through timely recognition of patients at risk of progression and early use of appropriately targeted therapies to prevent irreversible organ damage. </p>","PeriodicalId":10761,"journal":{"name":"Current Rheumatology Reports","volume":"28 1","pages":""},"PeriodicalIF":4.9,"publicationDate":"2026-03-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13038468/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147580713","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":"Behçet's Syndrome.","authors":"Yusuf Yazici, Gulen Hatemi","doi":"10.1007/s11926-026-01218-y","DOIUrl":"10.1007/s11926-026-01218-y","url":null,"abstract":"<p><p>PURPOSE OF THIS REVIEW: Behçet's syndrome (BS) frequently comes up in the differential diagnosis of many conditions, including but not limited to rheumatological, dermatological, ophthalmological, neurological and gastrointestinal diseases. We present here a summary of what is currently known about this condition and recent published literature that may help in the diagnosis and management of BS patients. RECENT FINDINGS: A better understanding of the pathogenesis of BS may help potentially better treatment options to be developed. New epidemiological studies have shown that BS may not be as rare in certain areas as previously thought. New treatment options and clinical trials are providing both patients and doctors treating BS with more options and potentially better outcomes. New imaging modalities are helping the diagnosis and monitoring of BS patients. Better understanding of pathogenesis and availability of treatment options are helping improve outcomes in BS patients. </p>","PeriodicalId":10761,"journal":{"name":"Current Rheumatology Reports","volume":"28 1","pages":""},"PeriodicalIF":4.9,"publicationDate":"2026-03-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147510218","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":"Is Gout an Autoinflammatory Disease?","authors":"Naomi Schlesinger, Dan Kaufmann","doi":"10.1007/s11926-026-01216-0","DOIUrl":"10.1007/s11926-026-01216-0","url":null,"abstract":"<p><p>PURPOSE OF REVIEW: This review recognizes gout as an autoinflammatory disease rather than a solely metabolic condition. It provides an overview of autoinflammatory diseases, the NOD-, LRR, and pyrin domain-containing protein 3 (NLRP3) inflammasome, and how soluble and crystalline forms of uric acid (UA) act as inflammatory triggers, prompting NLRP3 inflammasome formation and activation. It also highlights the genetics associated with the autoinflammatory features of gout and discusses NLRP3 inflammasome-targeted treatments. RECENT FINDINGS: Autoinflammatory diseases result from hyperactivity of the innate immune system, and the NLRP3 inflammasome complex, an innate immune sentinel and nonspecific sensor of cellular perturbation, is the initiator and key participant in gouty inflammation. Both the soluble and crystalline forms of UA act as inflammatory triggers, leading to the formation and activation of the NLRP3 inflammasome further promoting caspase 1-dependent release of the pro-inflammatory cytokines interleukin (IL)-1β and IL-18, as well as to gasdermin D-mediated pyroptotic cell death. Intracellular soluble UA also induces endothelial nitric oxide synthase dysfunction, oxidative stress, and inflammation. These mechanisms explain UA’s important role in promoting inflammatory pathways in patients. Gout is now recognized as a disease rooted in innate immune dysregulation, with uric acid-driven activation of the NLRP3 inflammasome. Integrating the autoinflammatory perspective highlights the opportunities for developing targeted treatments. Emerging therapies focused on NLRP3 inhibition coupled with urate-lowering strategies may offer a better approach for managing gout flares. </p>","PeriodicalId":10761,"journal":{"name":"Current Rheumatology Reports","volume":"28 1","pages":""},"PeriodicalIF":4.9,"publicationDate":"2026-03-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147493386","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":"Management of Takayasu Arteritis - A 2026 Update.","authors":"Tanaz A Kermani, Alessandro Tomelleri, Daniele Scaramuzzi, Swapnil Jagtap, Durga Prasanna Misra","doi":"10.1007/s11926-026-01214-2","DOIUrl":"10.1007/s11926-026-01214-2","url":null,"abstract":"<p><strong>Purpose of this review: </strong>Takayasu arteritis (TAK) is a chronic, large-vessel vasculitis disproportionately affecting young women. The consequences of vascular inflammation include stenotic and occlusive disease with resultant end organ ischemia and dysfunction impacting quality of life. In this review, we highlight the recent advances in the assessment of disease activity and damage and treatment of TAK.</p><p><strong>Recent findings: </strong>Distinguishing disease activity from vascular damage in patients with TAK is challenging. The PET Vascular Activity Score (PETVAS) and Vasculitis Activity using MR and PET Vasculitis Activity using MR and PET (VAMP) hold promise. Composite scores such as the TAK Integrated Disease Activity Index (TAIDAI) combine information from prevalent clinical features and FDG-PET. For damage assessment, the validation of the Large Vessel Vasculitis Index of Damage (LVVID) in patients with TAK and reports of serum biomarkers of fibrosis are important advances. The usual practice for treating patients with TAK is to combine glucocorticoids with DMARDs. Recent studies have shown the effectiveness of methotrexate or mycophenolate mofetil (alone or in combination), secukinumab, tofacitinib, and baricitinib while confirming the role of tumor necrosis factor alpha inhibitors or tocilizumab in the management of TAK. Vascular interventions are reserved for vascular damage or ischemia of major organs threatening their function or affecting the quality of life. Advances in the assessment of disease activity and damage using composite tools hold promise to improve the management of patients with TAK. More high-quality trials are required to better inform the evidence base for treating patients with TAK.</p>","PeriodicalId":10761,"journal":{"name":"Current Rheumatology Reports","volume":"28 1","pages":""},"PeriodicalIF":4.9,"publicationDate":"2026-03-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147490831","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":"Correction: Emerging Therapeutics in Rheumatoid Arthritis.","authors":"Daniel J Carlson, Laura M Nichols, Larry W Moreland","doi":"10.1007/s11926-026-01215-1","DOIUrl":"10.1007/s11926-026-01215-1","url":null,"abstract":"","PeriodicalId":10761,"journal":{"name":"Current Rheumatology Reports","volume":"28 1","pages":""},"PeriodicalIF":4.9,"publicationDate":"2026-03-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147472970","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":"Mechanistic Insights from Human Studies in Axial Spondyloarthritis: A T Cell Story.","authors":"Joy Um, Michael A Paley","doi":"10.1007/s11926-026-01213-3","DOIUrl":"10.1007/s11926-026-01213-3","url":null,"abstract":"<p><p>PURPOSE OF REVIEW: Ankylosing spondylitis (AS) represents the archetype of the spondyloarthritis family defined by its strong association with HLA-B*27. While genetic susceptibility has long pointed toward adaptive immunity, the precise cellular pathways linking MHC class I alleles to axial inflammation have remained an enigma. In this article, we review the fundamental molecular and clinical evidence positioning CD8  and CD4  T cells as primary pathogenic drivers of disease in the HLA-B*27 +  patients. RECENT FINDINGS: High-throughput T cell receptor (TCR) sequencing and single-cell RNA sequencing have identified “public” TRAV21/TRBV9 TCRs, that are expanded in the inflamed joints and eyes of patients. These clones initiate disease via molecular mimicry, recognizing both microbial and self-peptides presented by HLA-B*27. The clinical success of seniprutug, a monoclonal antibody selectively targeting TRBV9 +  T cells, provided the first proof-of-concept that these specific clonotypes drive clinical disease. Furthermore, high-resolution profiling has identified CD4 Th17 cells as the dominant producers of IL-17 within the synovial niche, providing a cellular basis for the efficacy of IL-17A and IL-17F blockade. AS is a disease characterized by convergent cross-reactive T cell responses. The identification of pathogenic TCR signatures and their candidate cognate antigens has moved diagnostics and management toward precision medicine. </p>","PeriodicalId":10761,"journal":{"name":"Current Rheumatology Reports","volume":"28 1","pages":""},"PeriodicalIF":4.9,"publicationDate":"2026-03-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12963204/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147354152","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":"NOD2-Related Multisystem Inflammatory Disorders and Recent Advances.","authors":"Di Wu, Tomoko Matsuda, Danielle Xie, Min Shen, Atika Dhar, John M Davis, Bo Shen, Naotomo Kambe, Warren Strober, Qingping Yao","doi":"10.1007/s11926-026-01212-4","DOIUrl":"10.1007/s11926-026-01212-4","url":null,"abstract":"<p><strong>Purpose of review: </strong>Nucleotide-binding oligomerization domain-containing protein 2 (NOD2) is an intracellular innate immune sensor. Its functions have been extensively studied. Variants in the NOD2 gene are associated with several human diseases. This report provides a comprehensive review of these diseases and biomedical and immunological roles of NOD2.</p><p><strong>Recent findings: </strong>Blau syndrome is an autosomal dominant disease primarily occurring in children and is caused by highly penetrant NOD2 variants. Approximately 40% of Caucasian patients with Crohn's disease (CD) are associated with three main NOD2 variants of low penetrance. Yao syndrome (YAOS), a recently reported novel disease, possesses characteristic clinical pattern or constellation of recurrent fever, dermatitis, arthralgia, distal leg swelling, gastrointestinal, sicca-like symptoms, and eyelid swelling among others. This disease is associated with specific NOD2 variants including the CD-associated three main variants. Our recent large case-control study of population genetics and haplotype analyses confirms the association between certain NOD2 variants and YAOS and the coinheritance in cis of the commonly encountered variants. Molecular testing is required for the diagnosis of YAOS. The prevalence of the disease is estimated to approach that of CD. There are identified effective drugs to manage this condition. Functional studies of NOD2 have revealed its contribution to both innate and adaptive immune responses, and there are interplays between these cellular components and cytokines. NOD2 has been extensively studied for innate immune response. Specific NOD2 variants are associated with different diseases, highlighting the fact that the same genotype can contribute to different phenotypes. Further studies are warranted to focus on adaptive immunity and bridge the gap between innate and adaptive immune responses in individual diseases.</p>","PeriodicalId":10761,"journal":{"name":"Current Rheumatology Reports","volume":"28 1","pages":"5"},"PeriodicalIF":4.9,"publicationDate":"2026-02-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146164509","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":"Thrombo-inflammation and Rethinking the Role of Aspirin in Kawasaki Disease.","authors":"Begüm Kocatürk, Beyda Berberoğulları, Emil Aliyev, Erdal Sağ, Seza Özen, Moshe Arditi","doi":"10.1007/s11926-026-01211-5","DOIUrl":"10.1007/s11926-026-01211-5","url":null,"abstract":"<p><p>PURPOSE OF REVIEW: Kawasaki Disease (KD) is a medium vessel vasculitis of young children that affects coronary arteries (CAs). The aim of therapy is to control inflammation and prevent coronary artery damage and alleviate thrombosis. This review aims to summarize the thrombosis-related complications and complementary therapy approaches to combat them. It also focuses on the ambiguity regarding the optimal aspirin dose in standard KD therapy. RECENT FINDINGS: KD patients with large or giant coronary artery aneurysms (CAAs) in particular face the risk of developing thrombosis. The standard therapy regimen during the acute phase consists of intravenous immunoglobulin (IVIG) plus aspirin. Although the dosing for IVIG is constant, the aspirin dose continues to be debated. The standard treatment with aspirin and IVIG might be adequate for individuals with small aneurysms whereas, patients suffering from large aneurysms and in a hypercoagulable state might need additional and extended therapy consisting of several antiplatelet reagents and anticoagulants. In case of thrombosis formation, thrombolytic therapy is also recommended. Cardiac and thrombotic problems are severe outcomes of KD. Patients need to be screened on a regular basis with echocardiography. The ideal aspirin dose during acute KD treatment is still controversial and while several recent studies pinpoint the superiority of low doses, there is no universal consensus. The variation in treatment response might be due to the different ethnic composition of study groups and inter-individual differences. Overall, close monitoring of patient response and finding the optimal treatment strategy is crucial. </p>","PeriodicalId":10761,"journal":{"name":"Current Rheumatology Reports","volume":"28 1","pages":"4"},"PeriodicalIF":4.9,"publicationDate":"2026-02-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12881031/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146131496","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":"Still's Disease and Autoinflammation: Positioning an Inflammatory Syndrome on the Autoinflammation-Autoimmunity Spectrum.","authors":"Daniel Pietsch, Sinisa Savic","doi":"10.1007/s11926-026-01210-6","DOIUrl":"10.1007/s11926-026-01210-6","url":null,"abstract":"<p><strong>Purpose of this review: </strong>Still's disease exemplifies systemic inflammatory disorders existing on a continuum between autoinflammation and autoimmunity. This review examines Still's disease through this spectrum lens, integrating recent advances in pathogenesis, clinical heterogeneity, and therapeutic approaches.</p><p><strong>Recent findings: </strong>Emerging mechanistic insights reveal complex innate-adaptive immune interactions. Type I interferon signalling and neutrophil extracellular trap formation drive inflammation, while hyperferritinemia actively perpetuates disease through Msr1-mediated signaling. mTORC1 has emerged as a central integration hub converging multiple cytokine signals. Adaptive mechanisms increasingly contribute to complications: both macrophage activation syndrome and lung disease demonstrate IFNγ-dominant pathology with T cell hyperactivation. Clinical phenotyping identifies distinct patient clusters-from hyperferritinemic monocyclic to catastrophic multiorgan phenotypes-reflecting varying innate-adaptive contributions. Current classification criteria permit considerable diagnostic latitude and may inadvertently group mechanistically distinct conditions under a single diagnostic label. IL-1 and IL-6 receptor blockade remain therapeutic cornerstones, with evidence supporting early intervention during a window of opportunity. Novel approaches including IL-18 binding protein, JAK inhibitors, and IFNγ blockade show promise in refractory disease. Still's disease predominantly reflects autoinflammatory pathology driven by innate immune dysregulation, yet adaptive mechanisms contribute meaningfully to disease heterogeneity and complications. Recognition as a spectrum disorder-with variable innate-adaptive contributions across patients and disease phases-supports unification of pediatric and adult forms, guides mechanistically targeted therapies, and emphasizes the need for biomarker-driven patient stratification to enable personalized treatment approaches.</p>","PeriodicalId":10761,"journal":{"name":"Current Rheumatology Reports","volume":"28 1","pages":"3"},"PeriodicalIF":4.9,"publicationDate":"2026-02-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12864328/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146104363","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}