{"title":"Anifrolumab for refractory cutaneous lupus lesions in pediatric systemic lupus erythematosus.","authors":"Masaki Shmizu, Shuya Kaneko, Asami Shimbo, Maho Hatano, Futaba Miyaoka, Hitoshi Irabu, Yuko Akutsu, Yuko Hayashi, Keiji Akamine, Masaaki Mori","doi":"10.1177/09612033251330094","DOIUrl":"https://doi.org/10.1177/09612033251330094","url":null,"abstract":"<p><p>Type I interferon (IFN) plays an important role in the pathogenesis of systemic lupus erythematosus (SLE) and cutaneous manifestations. Anifrolumab, a fully humanized monoclonal antibody against type 1 IFN receptor, has shown a significant reduction of the disease activity of SLE and cutaneous manifestations in adults. We reported two pediatric SLE patients with refractory cutaneous lesions that dramatically improved after anifrolumab therapy. This is the first study to show clinical effects of anifrolumab for pediatric SLE patients. These cases suggest the efficacy of anifrolumab for pediatric SLE patients with refractory cutaneous lesions and elevated IFN levels.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"9612033251330094"},"PeriodicalIF":1.9,"publicationDate":"2025-03-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143692692","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}
LupusPub Date : 2025-03-21DOI: 10.1177/09612033251330082
Ömer Uludağ, Akın Işık, Ege Sinan Torun, Burak Alkaç, Yasemin Yalçınkaya, Ahmet Gül, Murat İnanç, Sabahattin Kaymakoğlu, Bahar Artim-Esen
{"title":"Budd Chiari syndrome associated with antiphospholipid syndrome: Clinical characteristics and prognosis of 17 patients from single center.","authors":"Ömer Uludağ, Akın Işık, Ege Sinan Torun, Burak Alkaç, Yasemin Yalçınkaya, Ahmet Gül, Murat İnanç, Sabahattin Kaymakoğlu, Bahar Artim-Esen","doi":"10.1177/09612033251330082","DOIUrl":"https://doi.org/10.1177/09612033251330082","url":null,"abstract":"<p><p>Background and AimIn this retrospective, descriptive study, we aimed to identify clinical and laboratory characteristics, and prognoses of patients with Budd-Chiari syndrome (BCS) secondary to antiphospholipid syndrome (APS) and to compare with non-BCS vascular thrombotic APS patients.MethodsData of 194 patients with thrombotic APS (17 with BCS) ± systemic lupus erythematosus (SLE) in a single center between 1982 and 2023 were evaluated. Antiphospholipid serology consisting of lupus anticoagulant (LA), anticardiolipin (aCL) IgG/IgM, anti-beta2 glycoprotein I (aβ2GPI) IgG/IgM and adjusted global APS score (aGAPSS) were evaluated to determine thrombotic risk. Damage was identified for all patients by applying the damage index for APS (DIAPS). All patients with BCS were screened for hereditary or acquired prothrombotic disorders.ResultsPatients with BCS had higher aGAPSS and recurrent thrombosis (70.6% vs 40.7%) compared to those with non-BCS. BCS was the first thrombotic event in eight patients and three had recurrent thrombosis. The most common presenting manifestation of BCS was abdominal pain which was followed by abdominal distention and fever. The second prothrombotic factor was detected in six patients: three had heterozygous factor V Leiden mutation, three were in pregnancy period. Additionaly, two patients had SLE flare. The DIAPS of the patients in the BCS and non-BCS groups were similar, but those in the BCS group had higher mortality rates.ConclusionsAPS patients with BCS may have a higher risk of recurrent thrombosis and mortality. Acquired or hereditary prothrombotic disorders are not uncommon in this group and should be screened in APS patients with BCS.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"9612033251330082"},"PeriodicalIF":1.9,"publicationDate":"2025-03-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143674216","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}
LupusPub Date : 2025-03-19DOI: 10.1177/09612033251326990
Richard Furie, Jürgen Steffgen, Nora Fagan, Juanita Romero-Diaz, Yingyos Avihingsanon, Dimitrios T Boumpas, Kajohnsak Noppakun, Jing Wu, Ivette Revollo, David R Jayne
{"title":"Two-year treatment experience with BI 655064, an antagonistic anti-CD40 antibody, in patients with active lupus nephritis: An exploratory, phase II maintenance trial.","authors":"Richard Furie, Jürgen Steffgen, Nora Fagan, Juanita Romero-Diaz, Yingyos Avihingsanon, Dimitrios T Boumpas, Kajohnsak Noppakun, Jing Wu, Ivette Revollo, David R Jayne","doi":"10.1177/09612033251326990","DOIUrl":"https://doi.org/10.1177/09612033251326990","url":null,"abstract":"<p><p>ObjectiveTo evaluate the long-term efficacy and safety of different doses of BI 655064 versus placebo added to standard of care during maintenance treatment for lupus nephritis (LN).Methods1293.13 was an exploratory, phase II maintenance trial. Patients were eligible for entry if they had completed 1 year of randomised treatment with BI 655064 (120, 180 or 240 mg) or placebo in the 1293.10 trial, responded to treatment at Year 1 (complete renal response [CRR], partial renal response or urinary protein/creatinine ratio ≤1) and consented to continue treatment. The primary endpoint was the proportion of patients with CRR without renal flares at Year 2. Secondary endpoints included change from baseline in Safety of Estrogens in Lupus Erythematosus National Assessment-Systemic Lupus Erythematosus Disease Activity Index (SELENA-SLEDAI) scores and safety/tolerability.Results69/121 patients (57.0%) from the 1293.10 trial entered 1293.13. The adjusted proportion of patients with CRR decreased in all groups between Year 1 (BI 655064: 53.4%-72.7%; placebo: 71.4%) and Year 2 (BI 655064: 48.2%-59.5%; placebo: 57.5%). At Year 2, mean decreases in total SELENA-SLEDAI scores were greatest with BI 655064 240 mg (-10.6 points), followed by 120 mg (-8.9 points), 180 mg (-7.2 points) and placebo (-5.3 points). SELENA-SLEDAI non-renal scores decreased at Year 1 (BI 655064: -3.0 to -3.4; placebo: -1.8); this pattern remained with BI 655064 during Year 2 (-2.4 to -4.1), whereas placebo returned to near-baseline scores (-0.4). Over 2 years of treatment, almost all patients (97.1%) experienced ≥1 adverse event (AE). Compared with the other groups, higher rates of serious AEs (42.9% vs 23.1%-33.3%)-mainly driven by serious infections (23.8% vs 7.7%-14.3%)-and severe AEs (47.6% vs 13.3%-28.6%) were reported with BI 655064 240 mg.ConclusionsThis exploratory, phase II maintenance trial failed to demonstrate the benefits of BI 655064 on renal outcomes in the treatment of LN. However, some benefits in total and non-renal SELENA-SLEDAI scores were observed.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"9612033251326990"},"PeriodicalIF":1.9,"publicationDate":"2025-03-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143657643","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}
LupusPub Date : 2025-03-17DOI: 10.1177/09612033251325687
Jiali Chen, Jiaying Chen, Zhaorong Huang, Xiaofang Chen, Erwei Sun
{"title":"Summary of best evidence on self-management in adult patients with systemic lupus erythematosus.","authors":"Jiali Chen, Jiaying Chen, Zhaorong Huang, Xiaofang Chen, Erwei Sun","doi":"10.1177/09612033251325687","DOIUrl":"https://doi.org/10.1177/09612033251325687","url":null,"abstract":"<p><p>ObjectiveTo evaluate and summarize the best evidence on self-management in adult patients with systemic lupus erythematosus (SLE) and provide evidence-based recommendations in clinical settings.MethodsLiteratures related to self-management of SLE patients were searched in the guideline websites, professional association websites and relevant databases, including clinical decisions, guidelines, expert consensus, best practice recommendations, evidence summaries, meta-analyses, systematic reviews and randomized controlled trials, etc. The search time was from January 2018 to September 2023.ResultsA total of 19 literatures were included, consisting of 9 guidelines, 4 expert consensus, 4 clinical decisions, and 2 systematic reviews, and 49 pieces of evidence were extracted. After sorting and discussing, the evidence were integrated into six aspects: skin management, diet management, exercise management, vaccination management, complication management, as well as follow-up and lifestyle management.ConclusionThe best evidence of self-management in SLE patients summarized in this study is of high quality, which can provide evidence-based recommendations for clinical medical staff and patients, so as to help self-management of SLE patients in a more scientific and effective way.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"9612033251325687"},"PeriodicalIF":1.9,"publicationDate":"2025-03-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143649587","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}
LupusPub Date : 2025-03-14DOI: 10.1177/09612033251326987
Chris Ching Lam Cheung, Chi Chiu Mok
{"title":"Long COVID in patients with systemic lupus erythematosus: A case-control study.","authors":"Chris Ching Lam Cheung, Chi Chiu Mok","doi":"10.1177/09612033251326987","DOIUrl":"https://doi.org/10.1177/09612033251326987","url":null,"abstract":"<p><p>BackgroundLong COVID was defined by the WHO as continuation or development of new symptoms 3 months after the initial SARS-CoV2 infection, with these symptoms lasting for ≥2 months without alternative explanations.ObjectivesTo compare the incidence of long COVID in patients with SLE with matched controls after a SARS-CoV2 infection.MethodsConsecutive patients (≥18 years) who fulfilled the ACR or SLICC criteria for SLE between July to November 2023 were recruited. Those with SARS-CoV2 infection (positive rapid antigen test [RAT] or PCR) were identified by a self-reported questionnaire, which also captured the duration and severity of a number of pre-defined symptoms of long COVID. An equal number of healthy subjects, matched for gender and age, who had SARS-CoV2 infection in the same period were recruited for the same survey. The incidence and presentation of long COVID was compared between patients and controls, and risk factors for long COVID in SLE patients were studied.ResultsA total of 211 SLE patients (97.6% women, age 44.2 ± 11.9 years) and 211 healthy controls (97.6% women, age 44.2 ± 11.9 years) were studied. In all patients, the most common long COVID symptoms were fatigue (30.1%), worsening of memory (29.1%), difficulty to concentrate (26.3%), cough (23.2%) and insomnia (18.9%). Compared with controls, the incidence of long COVID was significantly higher in SLE patients (54.5% vs 34.6%; OR 2.27 [1.53-3.35]). Symptoms of worsening of memory (OR 2.77 [1.78-4.31]), chest pain (OR 4.18 [1.16-15.0]), palpitation (OR 3.43 [1.58-7.47]) and arthralgia (OR 2.67 [1.29-5.53]) were significantly more common in SLE patients than controls. However, no clinical and serological factors were significantly associated with the occurrence of long COVID in SLE patients except the ever use of hydroxychloroquine (OR 2.03 [1.04-3.96]).ConclusionsLong COVID is more common in SLE patients than matched controls after a SARS-CoV2 infection. The development of long COVID symptoms in SLE is largely unpredictable.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"9612033251326987"},"PeriodicalIF":1.9,"publicationDate":"2025-03-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143625427","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}
LupusPub Date : 2025-03-13DOI: 10.1177/09612033251326986
Bruna Caruso Mazzolani, Fabiana Infante Smaira, Sofia Mendes Sieczkowska, Marina Romero, Heloísa C Santo André, Sandra G Pasoto, Ana Lúcia de Sá Pinto, Fernanda Rodrigues Lima, Fabiana Braga Benatti, Hamilton Roschel, Mary Beth Weber, Bruno Gualano
{"title":"Acceptability and impact of a lifestyle intervention for systemic lupus erythematosus: Qualitative analysis of living well with lupus study.","authors":"Bruna Caruso Mazzolani, Fabiana Infante Smaira, Sofia Mendes Sieczkowska, Marina Romero, Heloísa C Santo André, Sandra G Pasoto, Ana Lúcia de Sá Pinto, Fernanda Rodrigues Lima, Fabiana Braga Benatti, Hamilton Roschel, Mary Beth Weber, Bruno Gualano","doi":"10.1177/09612033251326986","DOIUrl":"https://doi.org/10.1177/09612033251326986","url":null,"abstract":"<p><p>Despite the beneficial effects of a healthier lifestyle, lack of more robust data hampers standardization of recommendations for patients with systemic lupus erythematosus (SLE). Thus, this study explores the experiences, perceptions, and health effects of a newly developed healthy lifestyle intervention in patients with SLE. This was a qualitative, descriptive study using focus group discussions with SLE patients with high cardiovascular risk who participated in a 6-month behavioral lifestyle intervention versus standard of care randomized controlled trial. The intervention group received the Living Well with Lupus (LWWL) program aimed at changing lifestyle behaviors. Focus groups were composed of 4-6 LWWL participants each. Deductive and inductive qualitative content analysis was performed using the MAXQDA data management software. A thematic analysis was conducted wherein thick descriptions of key themes around SLE patients' perceptions related to the intervention and interventions' effects on routine, health-related outcomes and well-being were developed. 19 patients participated in the focus groups (age: 42 ± 7 years; BMI: 28.3 ± 2.4 kg/m<sup>2</sup>; mild disease activity and organ damage index). Overall, (a) participants were mostly positive about the intervention components, although views of home-based exercise and food diaries were both positive and negatives; (b) barriers and facilitators to intervention adherence included socioeconomic, environmental and individual factors; (c) new behaviors and knowledge were achieved, which participants intended to maintain after the study; and (d) the intervention positively impacted daily functioning, physical and mental health, and overall wellbeing. LWWL participants reported success in achieving healthy lifestyle behaviors in addition to improvements in perceived physical and mental health and overall wellbeing during the LWWL program. Results of this qualitative assessment, including an overview of barriers and facilitators to program initiation and adherence, should guide future research on the implementation of lifestyle interventions in SLE care.Clinicaltrials.govNCT04431167.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"9612033251326986"},"PeriodicalIF":1.9,"publicationDate":"2025-03-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143625422","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}
LupusPub Date : 2025-03-11DOI: 10.1177/09612033251325320
Manuel F Ugarte-Gil, Rocío V Gamboa-Cárdenas, Victor Pimentel-Quiroz, Cristina Reategui-Sokolova, Claudia Elera-Fitzcarrald, Francisco Zevallos, Zoila Rodriguez-Bellido, Cesar Pastor-Asurza, Risto Perich-Campos, Graciela S Alarcón
{"title":"A better self-efficacy is predictive of lower fatigue in systemic lupus erythematosus patients: Data from a Almenara Lupus Cohort.","authors":"Manuel F Ugarte-Gil, Rocío V Gamboa-Cárdenas, Victor Pimentel-Quiroz, Cristina Reategui-Sokolova, Claudia Elera-Fitzcarrald, Francisco Zevallos, Zoila Rodriguez-Bellido, Cesar Pastor-Asurza, Risto Perich-Campos, Graciela S Alarcón","doi":"10.1177/09612033251325320","DOIUrl":"https://doi.org/10.1177/09612033251325320","url":null,"abstract":"<p><p><b>Objectives:</b> To determine the possible predictive value of self-efficacy on fatigue in SLE patients. <b>Methods:</b> SLE patients from a single-center prevalent cohort were included. Self-efficacy was ascertained with the five instruments of the Patient-Reported Outcomes Measurement Information System® (PROMIS®) Self-efficacy for Managing Chronic Conditions plus the PROMIS general self-efficacy. For PROMIS instruments, a score of 50 is the average for a clinical population (people with a chronic condition), the higher score, the greater self-efficacy. Fatigue was ascertained with the Functional Assessment of Chronic Illness Therapy - Fatigue (FACIT-F), the higher score, the lower fatigue. Generalized estimating equations were performed, using as outcome the FACIT-F in the subsequent visit, and the self-efficacy instrument in the previous visit; multivariable models were adjusted for possible confounders. All the confounders were measured in the same visit than the self-efficacy instrument. <b>Results:</b> A total of 209 patients and 563 visits were included. At baseline, FACIT-F was 33.0 (10.2), mean general self-efficacy was 47.2 (10.4), self-efficacy for managing emotions was 44.6 (8.0), for managing symptoms was 47.7 (8.2), for managing daily activities was 45.5 (7.5) for managing social interactions was 42.9 (7.9) and for managing medications and treatment was 43.9 (7.0). In the multivariable models a lower fatigue was predicted by self-efficacy for managing symptoms and managing activities. <b>Conclusion:</b> A better self-efficacy is predictive of a subsequent lower fatigue, even after adjustment for possible confounders. Strategies to improve self-efficacy in SLE patients should be encouraged.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"9612033251325320"},"PeriodicalIF":1.9,"publicationDate":"2025-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143605659","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}
LupusPub Date : 2025-03-04DOI: 10.1177/09612033251324482
Hakeem Babatunde Olaosebikan, Abubakar Yerima, Gbenga Joshua Odunlami, Courage Ukponmwan Uhunmwangho, Henry Madu Nwankwo, Musa Bello Kofar Na'isa, Mary Agun-Ebreme, Ibrahim Daiyabu, Ilo Azizat Bamisebi, Etseoghena Igebu, Henry Ekpenyong, Hassana Hindatu Aliyu, Olufemi Oladipo Adelowo
{"title":"Health journey of Nigerian patients with systemic lupus erythematosus.","authors":"Hakeem Babatunde Olaosebikan, Abubakar Yerima, Gbenga Joshua Odunlami, Courage Ukponmwan Uhunmwangho, Henry Madu Nwankwo, Musa Bello Kofar Na'isa, Mary Agun-Ebreme, Ibrahim Daiyabu, Ilo Azizat Bamisebi, Etseoghena Igebu, Henry Ekpenyong, Hassana Hindatu Aliyu, Olufemi Oladipo Adelowo","doi":"10.1177/09612033251324482","DOIUrl":"https://doi.org/10.1177/09612033251324482","url":null,"abstract":"<p><strong>Background: </strong>Although there is an increase in prevalence and incidence of lupus worldwide, the journey to diagnosis is unduly delayed. This delay is associated with worse outcomes. Sub-Saharan Africa has witnessed an increase in lupus diagnosis in recent decades with no specific data on the time to diagnosis of lupus.</p><p><strong>Objectives: </strong>We measured and documented lupus diagnostic delays, patients' experiences, and factors associated with delayed diagnosis and provided recommendations for early diagnosis.</p><p><strong>Methods: </strong>This is a three-month cross-sectional study of 245 patients diagnosed with lupus who are members of a Nigerian lupus support group. Included participants fill out patients' administered questionnaire in a Google doc. The questionnaire captured all aspects of the study objectives. Four diagnostic journey intervals were defined. Delayed diagnosis was defined as a time interval from first symptoms to lupus diagnosis greater than or equal to 6 months.</p><p><strong>Results: </strong>The majority of participants were under 40 years of age (<i>n</i>-187, 76.3%) and predominantly female (<i>n</i>-226, 92.9%). About 53.9% of participants were diagnosed between 6 months and 2 years after their first symptoms, while 42% visited the first doctor within 6 months of symptom onset. Roughly half of the participants were referred to rheumatologists within 6 months of their visit to the last doctor, while 50.2% of the participants were diagnosed within 4 weeks of the rheumatologist's evaluation. Delayed diagnosis and delayed referral were documented in 80% and 66.9% of participants respectively. Low income (OR-7.4), internal organ manifestations (OR-4.5), and multiple doctors' visits (OR-11.6) were independently associated with delayed diagnosis.</p><p><strong>Conclusions: </strong>Diagnostic delay is observed in the majority of our patients. This delay is associated with multiple hospital visits, low income, and internal organ manifestations. There should be concerted efforts in SSA to improve the rheumatology workforce and incorporate non-specialists in clinical service delivery.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"9612033251324482"},"PeriodicalIF":1.9,"publicationDate":"2025-03-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143557244","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}
LupusPub Date : 2025-03-04DOI: 10.1177/09612033251325315
Joaquín Roberto Rodelo-Ceballos, Lina Aguirre, Luis Alonso González
{"title":"Prognostic value of the modified National Institute of health activity and chronicity scoring system in predicting end-stage kidney disease in Latin American lupus nephritis patients.","authors":"Joaquín Roberto Rodelo-Ceballos, Lina Aguirre, Luis Alonso González","doi":"10.1177/09612033251325315","DOIUrl":"https://doi.org/10.1177/09612033251325315","url":null,"abstract":"<p><strong>Objective: </strong>To assess the effectiveness of the modified National Institute of Health (mNIH) activity and chronicity scoring system in predicting the progression to end-stage kidney disease (ESKD) in Latin American lupus nephritis (LN) patients.</p><p><strong>Methods: </strong>We retrospectively analyzed 412 patients with biopsy-proven LN. ESKD was defined as an estimated glomerular filtration rate (eGFR) < 15 mL/min/1.73 m<sup>2</sup> for ≥3 months, dialysis for >3 months, or kidney transplant. Univariable and multivariable Cox proportional hazards regression analyses were performed to evaluate the predictive value of the mNIH activity and chronicity indices for ESKD.</p><p><strong>Results: </strong>84 patients (20.4%) progressed to ESKD at a median of 3.0 months after biopsy. The mNIH activity and chronicity indices were significantly higher in patients who progressed to ESKD compared to those who did not [7 (5-10) versus 4 (1-7), <i>p</i> < .001; and 3 (1-5) versus 0 (0-2), <i>p</i> < .001, respectively]. Multivariable Cox regression analysis revealed that the mNIH activity index (HR 1.17, 95% CI 1.05-1.29), and the mNIH chronicity index (HR 1.21, 95% CI 1.05-1.40) were independently associated with a higher risk of ESKD, adjusting for age, sex, ethnicity, and eGFR. Furthermore, fibrinoid necrosis (HR 4.09, 95% CI 1.54-10.86) and fibrous crescents (HR 2.36; 95% CI 1.06-5.27), components of the activity and chronicity indices, respectively, were also associated with a shorter time to ESKD.</p><p><strong>Conclusions: </strong>In Latin American LN patients, the mNIH activity and chronicity indices are associated with an increased risk of ESKD. Among the components of these indices, shorter time to ESKD was mainly driven by fibrinoid necrosis and fibrous crescents.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"9612033251325315"},"PeriodicalIF":1.9,"publicationDate":"2025-03-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143542427","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":"The performance of the 2019 EULAR/ACR classification criteria in childhood-onset systemic lupus erythematosus.","authors":"Esma Aslan, Sezgin Sahin, Sule Bektas, Nergis Akay, Umit Gul, Elif Kilic Konte, Aybuke Gunalp, Fatih Haslak, Mehmet Yildiz, Oya Koker, Amra Adrovic, Kenan Barut, Ozgur Kasapcopur","doi":"10.1177/09612033251325321","DOIUrl":"https://doi.org/10.1177/09612033251325321","url":null,"abstract":"<p><strong>Objective: </strong>Classification criteria for systemic lupus erythematosus (SLE) are required to ensure consistency in enrolling patients into clinical trials. We aimed to evaluate the performance of the 2019 European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) criteria in patients with childhood-onset SLE (cSLE) by comparing its sensitivity and specificity with the 1997 ACR and the 2012 Systemic Lupus International Collaborating Clinics (SLICC) criteria.</p><p><strong>Methods: </strong>Our study included 111 cSLE patients (82% female) for sensitivity analysis. The diagnostic sensitivity of the three sets of classification criteria were further tested at first year of diagnosis and at last visit, longitudinally.</p><p><strong>Results: </strong>The 2019 EULAR/ACR criteria had the highest sensitivity at diagnosis and the first-year follow-up (93.7% and 96%, respectively). At the last visit, 2019 EULAR/ACR had a sensitivity equal to 2012 SLICC (98% vs 98%) and still continued to exhibit a better sensitivity than 1997 ACR (98% vs 93.9%). Concerning specificity, 1997 ACR criteria demonstrated the highest performance (89.4%), whereas 2019 EULAR/ACR criteria exhibited greater specificity compared to 2012 SLICC (86.5% vs 81.7%, respectively).</p><p><strong>Conclusion: </strong>The 2019 EULAR/ACR criteria classified patients with higher sensitivity at diagnosis and at the first-year follow-up. However, 2019 EULAR/ACR criteria at last visit demonstrated an equal sensitivity to 2012 SLICC. The specificity performance of 2019 EULAR/ACR could not reach to the level of 1997 ACR, but it was more successful than 2012 SLICC. Although the difference was not significant, the new set of criteria seem to be capable of recruiting more children to clinical trials.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"9612033251325321"},"PeriodicalIF":1.9,"publicationDate":"2025-03-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143542429","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}