Rheumatology最新文献

{"title":"A secukinumab dose-escalation study in patients with ankylosing spondylitis not achieving inactive disease after 16 weeks of treatment.","authors":"Atul Deodhar, Alan J Kivitz, Marina Magrey, Jessica A Walsh, Philip J Mease, Maria Greenwald, Farid Kianifard, Chelsea Elam, Gopi M Bommidi, Adam Winseck, Lianne S Gensler","doi":"10.1093/rheumatology/keae432","DOIUrl":"10.1093/rheumatology/keae432","url":null,"abstract":"<p><strong>Objective: </strong>To investigate the clinical response at week 52 in patients with AS who received secukinumab 300 vs 150 mg after inadequate response to 150 mg at week 16.</p><p><strong>Methods: </strong>ASLeap (NCT03350815) was a randomized, double-blind, parallel-group, multicentre, phase 4 trial. After 16 weeks of open-label secukinumab 150 mg (Treatment Period 1), patients who did not achieve inactive disease [AS Disease Activity Score (ASDAS) <1.3] at both week 12 and 16 were considered to have an inadequate response and were randomized 1:1 to receive secukinumab 300 or 150 mg every 4 weeks until week 52 (Treatment Period 2). The primary efficacy variable was achievement of ASDAS <1.3 at week 52 using week 16 as baseline. Safety was evaluated by the incidence of treatment-emergent adverse events (TEAEs) through week 52.</p><p><strong>Results: </strong>Of 322 patients treated with secukinumab in Treatment Period 1, 207 (64.3%) had inadequate response. Similar proportions of patients with inadequate response randomized to secukinumab 300 mg (n = 101) and 150 mg (n = 105) in Treatment Period 2 completed the study (83.8% and 84.3%, respectively). At week 52, 8.8% and 6.7% of patients receiving secukinumab 300 and 150 mg, respectively, achieved ASDAS <1.3. The incidence of TEAEs was similar in both groups through week 52. No new safety signals were observed.</p><p><strong>Conclusion: </strong>Patients with AS who did not achieve ASDAS <1.3 after receiving secukinumab 150 mg for 16 weeks experienced similar clinical response and safety through week 52 regardless of dose escalation.</p><p><strong>Trial registration: </strong>ClinicalTrials.gov, http://clinicaltrials.gov, NCT03350815.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":"1864-1872"},"PeriodicalIF":4.7,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11962956/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141917407","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":"Incidence of chronic recurrent multifocal osteomyelitis in children and adolescents in the UK and Republic of Ireland.","authors":"Daphne Theresa Chia, Andoni Paul Toms, Anish Sanghrajka, Athimalaipet V Ramanan, Orla G Killeen, Cristina Ilea, Kamran Mahmood, Sandrine Compeyrot-Lacassagne, Kathryn Bailey, Neil Martin, Kate Armon, Chenqu Suo","doi":"10.1093/rheumatology/keae447","DOIUrl":"10.1093/rheumatology/keae447","url":null,"abstract":"<p><strong>Objectives: </strong>Chronic recurrent multifocal osteomyelitis (CRMO), also known as chronic non-bacterial osteomyelitis (CNO), is a rare autoinflammatory condition affecting the bones in children and teenagers. The actual incidence of CRMO remains uncertain. The objective of this study was to identify the incidence of CRMO in children and young people under the age of 16 years in the United Kingdom (UK) and Republic of Ireland (ROI). We also aimed to delineate the demographics, clinical presentation, investigations, initial management and healthcare needs for children and adolescents with CRMO.</p><p><strong>Methods: </strong>We conducted monthly surveys among all paediatric consultants and paediatric orthopaedic surgeons to identify patients newly diagnosed with CRMO between October 2020 and November 2022. A standardized questionnaire was sent to reporting clinicians to collect further information.</p><p><strong>Results: </strong>Over the surveillance period, 288 patients were reported, among which, 165 confirmed and 20 probable cases were included in the analysis. The highest incidences were among 8-10 year-olds. A two-to-one female-to-male difference in incidence was observed, and male patients were more likely to present with multifocal disease. A negative correlation was observed between reporting clavicular and leg pain. Investigation-wise, 80.0% of patients were reported to have undergone whole-body MRI and 51.1% had bone biopsies. The most common initial treatments were NSAIDs (93.9%) and bisphosphonates (44.8%).</p><p><strong>Conclusion: </strong>This study estimates an average annual CRMO incidence of 0.65 cases per 100 000 children and adolescents in the UK and ROI. These findings establish a crucial baseline for ongoing research and improvement in the care of individuals with CRMO.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":"2162-2170"},"PeriodicalIF":4.7,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11962910/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142018497","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":"Trimethoprim sulfamethoxazole prophylaxis and serious infections in granulomatosis with polyangiitis treated with rituximab.","authors":"Arielle Mendel, Hassan Behlouli, Évelyne Vinet, Jeffrey R Curtis, Sasha Bernatsky","doi":"10.1093/rheumatology/keae368","DOIUrl":"10.1093/rheumatology/keae368","url":null,"abstract":"<p><strong>Objective: </strong>To assess the association of trimethoprim sulfamethoxazole (TMP-SMX) prophylaxis with serious infections in rituximab-treated patients with granulomatosis with polyangiitis (GPA).</p><p><strong>Methods: </strong>This retrospective cohort study included adults with GPA (2011-2020) within the United States Merative™ Marketscan® Research Databases with ≥6 months' enrolment prior to first (index) rituximab treatment. We defined TMP-SMX prophylaxis as a ≥28-day prescription dispensed after or overlapping the index date. Serious infection was a hospital primary diagnosis for infection (excluding viral or mycobacterial codes). Secondary outcomes were outpatient infection, Pneumocystis jirovecii pneumonia (PJP) and adverse events potentially attributable to TMP-SMX. Cox proportional hazards regression assessed the association of time-varying TMP-SMX with outcomes of interest, adjusting for potential confounders. Individuals were followed until the outcome of interest, end of database enrolment or 31 Decamber 2020.</p><p><strong>Results: </strong>Among 919 rituximab-treated individuals (53% female), mean (s.d.) age was 52.1 (16) years and 281 (31%) were dispensed TMP-SMX within 30 days of index date. Over a median of 496 (interquartile range 138-979) days, 130 serious infections occurred among 104 individuals (incidence 6.1 [95% CI: 5.0, 7.4] per 100 person-years). Time-varying TMP-SMX was negatively associated with serious infection (adjusted hazard ratio [aHR] 0.5; 95% CI: 0.3, 0.9). The aHR for outpatient infections was 0.8 (95% CI: 0.6, 1.1). The estimate for PJP was imprecise (13 events, unadjusted HR 0.2; 95% CI: 0.03-1.8). TMP-SMX was potentially associated with adverse events (aHR 1.3; 95% CI: 0.9, 1.9).</p><p><strong>Conclusions: </strong>TMP-SMX prophylaxis was associated with reduced serious infections in rituximab-treated GPA, but may increase adverse events, warranting further study of optimal prophylaxis strategies.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":"2041-2049"},"PeriodicalIF":4.7,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11962977/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141724331","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":"Performance of clinical and histological prognostic scores for kidney survival in ANCA-associated vasculitis.","authors":"Marlon J Sandino-Bermúdez, Adriana Hernández-Andrade, Andrea Hinojosa-Azaola, Eduardo Martín-Nares, Juan M Mejía-Vilet","doi":"10.1093/rheumatology/keae336","DOIUrl":"10.1093/rheumatology/keae336","url":null,"abstract":"<p><strong>Objectives: </strong>Integrating clinical and histological parameters into prognostic scores may enhance the prediction of progression to kidney failure in anti-neutrophil cytoplasm antibodies-associated vasculitis (AAV). This study aimed to evaluate the prognostic performance of histological classifications and scoring systems for kidney survival in AAV.</p><p><strong>Methods: </strong>This retrospective cohort study included 101 AAV patients with kidney involvement diagnosed by biopsy and followed for ≥12 months. The main outcome was the time to kidney failure. The prognostic performance of each histological and prognostic score was evaluated using Harrell's C statistic and Akaike's Information Criteria.</p><p><strong>Results: </strong>Among the 101 patients, 37 progressed to kidney failure over a median follow-up of 75 months (IQR 39-123). The Harrell's C statistic was 0.702 (0.620-0.784), 0.606 (0.473-0.738), 0.801 (0.736-0.867), 0.782 (0.706-0.858) and 0.817 (0.749-0.885) for the EUVAS/Berden classification, Mayo Clinic Chronicity Score, Percentage of ANCA Crescentic Score (PACS), ANCA renal risk score (ARRS), and the improved ANCA kidney risk score (AKRiS), respectively. The AKRiS best discriminated the risk of kidney failure progression among subgroups. The AKRiS performance decreased with longer follow-up intervals. Adding the peak estimated glomerular filtration rate attained post-therapy improved the AKRiS performance at all follow-up intervals. Kidney relapses precipitated kidney failure in 71% of cases that progressed after the first year of follow-up.</p><p><strong>Conclusion: </strong>The novel AKRiS enhances the prediction of kidney failure in AAV with kidney involvement. As the prognostic yield of AKRiS decreases over time, a second calculation of AKRiS, including post-therapy kidney function, may improve its long-term performance.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":"1981-1988"},"PeriodicalIF":4.7,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141321541","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":"Multimorbidity in psoriasis as a risk factor for psoriatic arthritis: a population-based study.","authors":"Paras Karmacharya, Rikesh Chakradhar, Cassondra A Hulshizer, Tina M Gunderson, Alexis Ogdie, John M Davis Iii, Kerry Wright, Megha M Tollefson, Alí Duarte-García, Delamo Bekele, Hilal Maradit-Kremers, Cynthia S Crowson","doi":"10.1093/rheumatology/keae040","DOIUrl":"10.1093/rheumatology/keae040","url":null,"abstract":"<p><strong>Objectives: </strong>To examine multimorbidity in psoriasis and its association with the development of psoriatic arthritis (PsA).</p><p><strong>Methods: </strong>A retrospective cohort study was performed using the Rochester Epidemiology Project. Population-based incidence (2000-09) and prevalence (1 January 2010) cohorts of psoriasis were identified by manual chart review. A cohort of individuals without psoriasis (comparators) were identified (1:1 matched on age, sex and county). Morbidities were defined using two or more Clinical Classification Software codes ≥30 days apart within prior 5 years. PsA was defined using ClASsification of Psoriatic ARthritis (CASPAR) criteria. χ2 and rank-sum tests were used to compare morbidities, and age-, sex- and race-adjusted Cox models to examine the association of baseline morbidities in psoriasis with development of PsA.</p><p><strong>Results: </strong>Among 817 incident psoriasis patients, the mean age was 45.2 years with 52.0% females, and 82.0% moderate/severe psoriasis. No multimorbidity differences were found between incident psoriasis patients and comparators. However, in the 1088 prevalent psoriasis patients, multimorbidity was significantly more common compared with 1086 comparators (odds ratio 1.35 and 1.48 for two or more and five or more morbidities, respectively). Over a median 13.3-year follow-up, 23 patients (cumulative incidence: 2.9% by 15 years) developed PsA. Multimorbidity (two or more morbidities) was associated with a 3-fold higher risk of developing PsA.</p><p><strong>Conclusion: </strong>Multimorbidity was more common in the prevalent but not incident cohort of psoriasis compared with the general population, suggesting that patients with psoriasis may experience accelerated development of multimorbidity. Moreover, multimorbidity at psoriasis onset significantly increased the risk of developing PsA, highlighting the importance of monitoring multimorbid psoriasis patients for the development of PsA.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":"2199-2203"},"PeriodicalIF":4.7,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11962958/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139642862","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":"TNF-alpha blockade in primary chronic non-bacterial osteomyelitis of the mandible.","authors":"Jeanne de La Rochefoucauld, Raphaël Lhote, Mourad-Azzedine Benassarou, Thomas Schouman, Chloé Bertolus, Zahir Amoura, Miguel Hié","doi":"10.1093/rheumatology/keae380","DOIUrl":"10.1093/rheumatology/keae380","url":null,"abstract":"<p><strong>Objectives: </strong>Primary chronic non-bacterial osteomyelitis of the mandible (CNOM) is a rare auto-inflammatory disease of unknown aetiology that bears pathophysiological resemblance to both SAPHO syndrome in adults and chronic recurrent multifocal osteomyelitis (CRMO) in children. Both SAPHO and CRMO respond to TNF-α blockade. Previously reported treatment regimens in CNOM including NSAIDs, corticosteroids, antibiotics, anti-resorptive therapy and surgery all bear disappointing results. TNF-α blockade is suggested as a treatment option by some experts but this is not backed by any clinical data. We sought to retrospectively and exhaustively report our experience of anti-TNF-α therapy in refractory CNOM.</p><p><strong>Methods: </strong>Fifteen patients with refractory CNOM and high disease burden were referred to our centre. TNF-α blockade was attempted in 10 cases, given its efficacy in neighbouring diseases, its good tolerance profile and failure of previous treatment strategies. We herein retrospectively report detailed outcomes for all patients having received anti-TNF-α therapy for this indication in our centre.</p><p><strong>Results: </strong>TNF-α-targeting therapy resulted in a rapid and sustained remission in a majority of patients with CNOM, without serious adverse events. Treatment was tapered and stopped without relapse in some patients despite a refractory course of several years. Male sex seems to be associated with a poorer outcome.</p><p><strong>Conclusion: </strong>Our results suggest that blocking TNF-α is efficient and safe in CNOM.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":"1770-1774"},"PeriodicalIF":4.7,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141749051","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":"De novo manifestations during adalimumab treatment in Behçet's syndrome.","authors":"Sinem Nihal Esatoglu, Ozge Sonmez, Didar Ucar, Elif Kaymaz, Yesim Ozguler, Serdal Ugurlu, Emire Seyahi, Melike Melikoglu, Izzet Fresko, Vedat Hamuryudan, Ugur Uygunoglu, Zekayi Kutlubay, Ali Ibrahim Hatemi, Aykut Ferhat Celik, Gulen Hatemi","doi":"10.1093/rheumatology/keae416","DOIUrl":"10.1093/rheumatology/keae416","url":null,"abstract":"<p><strong>Objectives: </strong>Treatment response may be variable across organ manifestations of Behçet's syndrome (BS). We aimed to determine the frequency of de novo manifestations during adalimumab treatment.</p><p><strong>Methods: </strong>We conducted a chart review of all BS patients who received adalimumab in our centre between 2008 and 2023. Demographic data, reasons for initiating adalimumab, concurrent medications, previous treatments, and outcomes were recorded. We defined de novo manifestations as new BS manifestations that occurred for the first time during treatment with adalimumab. For patients with vascular involvement, a new vascular event at another vessel was also considered as a de novo manifestation.</p><p><strong>Results: </strong>Among the 335 patients, a de novo manifestation developed in 14 (4%) patients. De novo manifestations were vascular involvement in five patients, arthritis in three, anterior uveitis in two, nervous system involvement in two, gastrointestinal involvement in one, and epididymitis in one patient. The primary reasons for adalimumab treatment were vascular involvement in five patients, uveitis in four, arthritis in three, mucocutaneous involvement in one, and epididymitis in one patient. Upon the development of de novo manifestation, adalimumab was switched to another biologic in four patients, dose was intensified in three, colchicine, conventional immunosuppressives and/or glucocorticoids were added in five, and topical eye drops were added in two patients, leading to remission of de novo manifestations in all patients.</p><p><strong>Conclusion: </strong>De novo manifestations were infrequent (4%) among BS patients treated with adalimumab. Of these, 57% were major organ involvement, mainly vascular involvement. None of the patients developed posterior uveitis.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":"2034-2040"},"PeriodicalIF":4.7,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141902783","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":"Tendon involvement and its association with pain and hand function in patients with osteoarthritis of the hand.","authors":"Irina Gessl, Anna Vinatzer, Gabriela Supp, Michael Zauner, Martina Durechova, Lisa Lechner, Valentin Ritschl, Josef Smolen, Tanja Stamm, Daniel Aletaha, Peter Mandl","doi":"10.1093/rheumatology/keae395","DOIUrl":"10.1093/rheumatology/keae395","url":null,"abstract":"<p><strong>Objective: </strong>To characterize the frequency and influence of tenosynovitis and tendon damage on pain and hand function using clinical examination and US in hand OA.</p><p><strong>Methods: </strong>We included 86 patients with hand OA and 23 age- and sex-matched control subjects. Extensor and flexor tendons of both hands were assessed by clinical examination and US for tenosynovitis and tendon damage. Conventional radiographs were acquired. Hand function was evaluated by the function subtest of the M-SACRAH (modified Score for the Assessment and Quantification of Chronic Rheumatoid Affections of the Hands) questionnaire and the Moberg pick-up test. K-means cluster analyses was calculated to assess clusters based on radiographic features and sonographic tendon scores.</p><p><strong>Results: </strong>US identified the involvement of one or more tendon in 60/86 (69.8%) hand OA patients compared with 2/23 (8.7%) subjects (P < 0.01) in the control group. In the hand OA group, US detected tendon damage more often in flexor tendons compared with extensor tendons (2.1% vs 0.9%, P = 0.03), while tenosynovitis was observed more often in extensor tendons compared with flexor tendons (8% vs 0.6%, P < 0001). The sensitivity and specificity of clinical examination to detect tendon involvement was 81.4% and 34.6%, respectively, on the patient level and 14.5% and 83.8% on the tendon level. The cluster analyses revealed one cluster with more radiographic features of hand OA and more tendon damage while more tenosynovitis was found in cluster 2. M-SACRAH function did not correlate with tendon involvement on US.</p><p><strong>Conclusion: </strong>This study revealed a high frequency of tendon involvement in hand OA. Tendon involvement on US did not impact hand function or self-reported pain.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":"1775-1782"},"PeriodicalIF":4.7,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11962883/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141902784","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":"Short- and long-term outcomes of patients with pure membranous lupus nephritis compared with patients with proliferative disease.","authors":"Fadi Kharouf, Qixuan Li, Laura P Whittall Garcia, Arenn Jauhal, Dafna D Gladman, Zahi Touma","doi":"10.1093/rheumatology/keae436","DOIUrl":"10.1093/rheumatology/keae436","url":null,"abstract":"<p><strong>Objectives: </strong>Membranous LN (MLN) is thought to have a more benign course than proliferative LN (PLN). We aimed to determine the differences in short- and long-term outcomes between patients with MLN and PLN.</p><p><strong>Methods: </strong>We included patients with first biopsy-proven MLN and PLN. Short-term outcomes included complete proteinuria recovery (CPR), complete renal response (CRR) and primary efficacy renal response (PERR). Long-term outcomes included a sustained ≥40% reduction in baseline estimated glomerular filtration rate, end-stage kidney disease (ESKD), cardiovascular (CV) events, ≥2 increase in SLICC/ACR Damage Index and death. Univariable and multivariable Cox proportional hazard models were used to examine the effect of baseline characteristics on long-term outcomes.</p><p><strong>Results: </strong>Of 215 patients, 51 had pure MLN and 164 had PLN. We found no significant differences between the two groups in achieving CPR, CRR and PERR at 1 and 2 years. Median time to outcomes was slightly, but insignificantly, longer in the MLN group. For long-term outcomes, PLN was associated with worse renal and non-renal outcomes, but this was not statistically significant. In the multivariable Cox proportional hazard models, ESKD was associated with the following baseline variables: younger age [hazard ratio (HR) 0.92, 95% confidence interval (CI) 0.87-0.97], higher creatinine (HR 1.01, 95% CI 1.01-1.02), low complement (HR 4.0, 95% CI 1.04-11.10) and higher chronicity index (HR 1.28, 95% CI 1.08-1.51).</p><p><strong>Conclusion: </strong>The resolution of proteinuria in LN is slow. MLN is not a benign disease and may be associated with deterioration of renal function, ESKD, damage, CV events and death.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":"1912-1922"},"PeriodicalIF":4.7,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141917410","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":"Distinct phenotype and prognosis of immune-mediated necrotizing myopathy based on clinical-serological-pathological classification.","authors":"Hongxia Yang, Lining Zhang, Xiaolan Tian, Wenli Li, Qingyan Liu, Qinglin Peng, Wei Jiang, Guochun Wang, Xin Lu","doi":"10.1093/rheumatology/keae361","DOIUrl":"10.1093/rheumatology/keae361","url":null,"abstract":"<p><strong>Objective: </strong>The aim of the study was to investigate the characteristics and prognosis of patients with immune-mediated necrotizing myopathy (IMNM) based on clinical, serological and pathological classification.</p><p><strong>Methods: </strong>A total of 138 patients with IMNM who met the 2018 European Neuromuscular Center criteria for IMNM including 62 anti-SRP, 32 anti-HMGCR-positive and 44 myositis-specific antibody-negative were involved in the study. All patients were followed up and evaluated remission and relapse. Clustering analysis based on clinical, serological and pathological parameters was used to define subgroups.</p><p><strong>Results: </strong>Clustering analysis classified IMNM into three clusters. Cluster 1 patients (n = 35) had the highest creatine kinase (CK) levels, the shortest disease course, severe muscle weakness and more inflammation infiltration in muscle biopsy. Cluster 2 patients (n = 79) had the lowest CK level and moderate inflammation infiltrate. Cluster 3 patients (n = 24) had the youngest age of onset, the longest disease course and the least frequency of inflammatory infiltration. Patients in cluster 3 had the longest time-to-remission [median survival time: 61 (18.3, 103.7) vs 20.5 (16.2, 24.9) and 27 (19.6, 34.3) months] and shorter relapse-free time than those in cluster 1 and 2 [median remission time 95% CI 34 (19.9, 48.0) vs 73 (49.0, 68.7) and 73 (48.4, 97.6) months]. Patients with age of onset >55 years, more regeneration of muscle fibres, more CD4+ T infiltration and membrane attack complex deposition had more favourable outcomes regarding time to achieving remission.</p><p><strong>Conclusions: </strong>Stratification combining clinical, serological and pathological features could distinguish phenotypes and prognosis of IMNM. The pathological characteristics may impact the long-term prognosis of patients with IMNM.</p>","PeriodicalId":21255,"journal":{"name":"Rheumatology","volume":" ","pages":"2252-2264"},"PeriodicalIF":4.7,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141727718","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}