Gabriela Hernández-Molina, Brian Uriel Anaya-Macías, Eduardo Martín-Nares
{"title":"Management of IgG4-Related Disease.","authors":"Gabriela Hernández-Molina, Brian Uriel Anaya-Macías, Eduardo Martín-Nares","doi":"10.1007/s11926-026-01224-0","DOIUrl":"10.1007/s11926-026-01224-0","url":null,"abstract":"<p><strong>Purpose of the review: </strong>IgG4-related disease (IgG4-RD) is a chronic immune-mediated fibroinflammatory condition characterized by tumefactive lesions in multiple organs. Although glucocorticoids remain the cornerstone of therapy, high relapse rates and treatment-related toxicity have prompted the development of steroid-sparing strategies and targeted therapies. This review summarizes current evidence on pharmacological and non-pharmacological management of IgG4-RD and proposes a practical treatment approach based on available data and clinical experience.</p><p><strong>Recent findings: </strong>Glucocorticoids continue to be the first-line therapy for remission induction, achieving high initial response rates; however, relapses are common, particularly after tapering or withdrawal. Conventional synthetic disease-modifying antirheumatic drugs (csDMARDs), such as mycophenolate mofetil, leflunomide, azathioprine, and methotrexate, are frequently used as steroid-sparing agents, although comparative evidence remains limited. B-cell targeted therapies have emerged as key treatment options. Rituximab has demonstrated high efficacy as first-line therapy and in refractory or relapsing disease, and is widely used despite remaining off-label in most regions. More recently, the anti-CD19 monoclonal antibody inebilizumab became the first therapy approved for IgG4-RD following the MITIGATE trial, which showed reduced disease flares and increased rates of glucocorticoid-free remission. Additional emerging therapies include obinutuzumab, obexelimab, CAR-T cell therapy, and cytokine-targeted agents such as dupilumab and tocilizumab, although evidence for most remains limited. Management of IgG4-RD requires an individualized approach based on disease severity, organ involvement, relapse risk, patient's comorbidities and preferences, and access to therapies. B-cell-directed therapies and other targeted agents are emerging as key components of treatment and may enable more effective and steroid-sparing disease control.</p>","PeriodicalId":10761,"journal":{"name":"Current Rheumatology Reports","volume":"28 1","pages":""},"PeriodicalIF":4.9,"publicationDate":"2026-05-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147834696","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}
Kristin Berger, Genna Braverman, Aldis Siltumens, Jessica Gordon, Robert Kaner
{"title":"Care of the Patient with Interstitial Lung Disease Perioperatively.","authors":"Kristin Berger, Genna Braverman, Aldis Siltumens, Jessica Gordon, Robert Kaner","doi":"10.1007/s11926-026-01223-1","DOIUrl":"10.1007/s11926-026-01223-1","url":null,"abstract":"<p><p>PURPOSE OF THIS REVIEW: Patients with interstitial lung disease (ILD) are at elevated risk of postoperative pulmonary complications (PPCs), including acute exacerbation, which carry high mortality. Surgical decision-making in this population requires careful preoperative risk stratification, medical optimization, and tailored intra- and postoperative management. This review summarizes current evidence to guide perioperative care for patients with ILD. RECENT FINDINGS: The ARISCAT (Assess Respiratory Risk in Surgical Patients in Catalonia) risk index is widely used for PPC prediction but likely underestimates risk in ILD. Recent studies highlight the high prevalence of comorbid obstructive sleep apnea in ILD, supporting the need for routine screening. Updated American College of Rheumatology guidelines inform perioperative antirheumatic drug management, while International Society for Heart and Lung Transplantation consensus recommendations guide care of patients with pulmonary hypertension and right heart failure, a common comorbid condition. Emerging evidence suggests that perioperative antifibrotic therapy is safe and may reduce the risk of acute exacerbation, although further investigation is needed. Regional anesthesia can be a feasible alternative to general anesthesia when possible, for further risk mitigation in this patient population. Intraoperative lung-protective ventilation and use of the lowest oxygen concentration that maintains safe oxygen saturation remain critical, while judicious fluid management, postoperative extubation to high-flow nasal cannula or noninvasive ventilation, early mobilization, and multimodal analgesia may further reduce PPC risk. Perioperative management of ILD patients is complex due to heightened vulnerability to PPCs. Current evidence supports adapting general perioperative strategies while incorporating disease-specific considerations. As therapeutic advances extend survival, further prospective studies are needed to establish evidence-based perioperative guidelines for this high-risk group. </p>","PeriodicalId":10761,"journal":{"name":"Current Rheumatology Reports","volume":"28 1","pages":""},"PeriodicalIF":4.9,"publicationDate":"2026-04-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13133189/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147764953","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}
Ted R Mikuls, Lindsay Helget, Jeff Newcomb, Austin Wheeler, James R O'Dell
{"title":"Treat-to-Target Urate-lowering Therapy: Lessons Learned from the STOP Gout Study.","authors":"Ted R Mikuls, Lindsay Helget, Jeff Newcomb, Austin Wheeler, James R O'Dell","doi":"10.1007/s11926-026-01220-4","DOIUrl":"10.1007/s11926-026-01220-4","url":null,"abstract":"<p><p>PURPOSE OF THE REVIEW: The purpose of this review is to provide details of the STOP Gout trial, among the first randomized, blinded studies to compare allopurinol with febuxostat using a treat-to-target strategy. In addition to details of its design and trial results, this review summarizes findings from pre-planned and other analyses, as well as an associated biorepository that has enabled the identification of biomarkers impacted by highly effective urate-lowering therapy (ULT). RECENT FINDINGS: In addition to primary trial findings demonstrating the non-inferiority of allopurinol to febuxostat in flare prevention and the achievement of serum urate goals, data generated from the STOP Gout trial have provided additional insights that have included: 1) the relative efficacy and safety of both allopurinol and febuxostat in participants with stage 3 chronic kidney disease; 2) patient factors associated with the achievement of serum urate goals; 3) the frequency and determinants of mobilization flares during the early phases of treat-to-target treatment; 4) the heightened risk of flare following discontinuation of anti-inflammatory prophylaxis; 5) limited persistence of ULT following transitions back to real-world care; 6) rapid gains in health-related quality of life accompanying highly effective ULT that pre-date reductions in flare risk; and 7) the positive effects of urate-lowering on measures of systemic inflammation and other circulating biomarkers. In addition to informing the comparative efficacy and safety of allopurinol and febuxostat, STOP Gout and the resources generated from this trial have enabled research that has further informed our understanding of gout and its management. </p>","PeriodicalId":10761,"journal":{"name":"Current Rheumatology Reports","volume":"28 1","pages":""},"PeriodicalIF":4.9,"publicationDate":"2026-04-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147764961","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":"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":3.9,"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}
Daniel J Carlson, Laura M Nichols, Larry W Moreland
{"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}