LupusPub Date : 2025-05-28DOI: 10.1177/09612033251344059
Farhanah Paruk, Dzifa Dey, Anisa Mosam, Oluwaytoyin Christina Amira, Mohammed Tikly
{"title":"Physician perceptions of challenges and barriers to optimal care of systemic lupus erythematosus in Africa.","authors":"Farhanah Paruk, Dzifa Dey, Anisa Mosam, Oluwaytoyin Christina Amira, Mohammed Tikly","doi":"10.1177/09612033251344059","DOIUrl":"https://doi.org/10.1177/09612033251344059","url":null,"abstract":"<p><p>IntroductionPrevious studies indicate that the prognosis of systemic lupus erythematosus (SLE) is poor in Africa. We surveyed African physicians for their perceptions of factors that impact negatively on optimal SLE care and interventions to improve care in Africa.MethodsA cross-sectional, online survey of African dermatologists, rheumatologists, nephrologists, and internists was conducted.ResultsResponses from 226 respondents mostly from West Africa and East Africa, majority practicing in university and state-funded hospitals showed that the commonest reasons for late diagnosis of SLE in Africa were lack of awareness of the disease amongst primary care doctors (92.4%), financial constraints (80.3%) and lack of access to health care (62.5%). Consulting traditional healers, the belief of bewitchment, lack of availability of diagnostic tests and concomitant chronic infections, (tuberculosis and HIV) were also perceived to have resulted in diagnostic delays, especially amongst East and West African respondents. The overwhelming majority (>90%) of respondents felt that increased health care and financial resources was the top priority to improving SLE care in Africa. Continuing medical education for generalists, training of specialists and patient education and awareness programs were considered less important interventions to improve SLE care.ConclusionThe study reveals that the primary reasons for late diagnosis of SLE in Africa are lack of awareness among primary healthcare doctors and financial constraints. Thus, the need for greater financial resources, especially for appropriate medications, medical education and improving patient understanding of the disease through support groups, to improve SLE care and outcomes in Africa.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"9612033251344059"},"PeriodicalIF":1.9,"publicationDate":"2025-05-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144174339","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":"Macrophage activation syndrome associated with neonatal lupus erythematosus.","authors":"Yuko Hayashi, Maho Hatano, Shuya Kaneko, Asami Shimbo, Hitoshi Irabu, Masaaki Mori, Masaki Shimizu","doi":"10.1177/09612033251346372","DOIUrl":"https://doi.org/10.1177/09612033251346372","url":null,"abstract":"<p><p>Neonatal lupus erythematosus (NLE) is a multi-organ systemic inflammatory disease caused by passively transmitted maternal autoantibodies including anti-SSA/Ro antibody. NLE is clinically characterized by skin symptoms, atrioventricular block, liver dysfunction, and cytopenia. In addition, NLE can cause fever and is a rare cause for fever of unknown origin in the neonatal period. Furthermore, although rare, macrophage activation syndrome (MAS) can complicate NLE. However, the pathogenesis of MAS associated with NLE is still unknown. We report a case of NLE-associated MAS along with the results of serum cytokine profile analysis. A feature of the serum cytokine profile at the onset of disease in our patient was significant increase of serum IL-6 and sTNF-RII levels. Furthermore, in MAS phase, serum sTNF-RII level was massively increased and serum IFN-α level was persistently elevated. MAS complicating NLE might have similar \"pathophysiology to MAS secondary to systemic lupus erythematosus.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"9612033251346372"},"PeriodicalIF":1.9,"publicationDate":"2025-05-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144128115","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":"Prevalence, clinical features, and possible associated factors of mesenteric vasculitis occurrence in patients with systemic lupus erythematosus: An age-, year-at SLE diagnosis, and disease duration-controlled study.","authors":"Wanitcha Gumtorntip, Piyanut Thanunchai, Amonlaya Amantakul, Suwalee Pojchamarnwiputh, Nuntana Kasitanon, Worawit Louthrenoo","doi":"10.1177/09612033251344154","DOIUrl":"https://doi.org/10.1177/09612033251344154","url":null,"abstract":"<p><p>ObjectivesStudies on associated or risk factors of lupus mesenteric vasculitis (LMV) in patients with systemic lupus erythematosus (SLE) are limited. This study aimed to determine the prevalence, clinical features, treatment, and outcomes of LMV in Thai SLE patients, and to identify potential factors that predispose to its occurrence.MethodsSLE patients with LMV were identified in a lupus cohort. Controls were matched to cases by age-, year-at SLE diagnosis, and disease duration (cases:controls = 1:2).ResultsOf 1538 patients in the cohort, 34 (2.2%) had 42 LMV episodes, but 39 episodes from 31 patients had adequate data for analysis. Nausea, vomiting and diarrhea were noted in 61.5%-69.2%. All of the patients had abdominal pain (diffuse in 64.1%), and 12.8%-15.4% had signs of peritoneal irritation. Small bowel and colon were common sites involved. All episodes responded well to corticosteroids and immunosuppressive drugs. When compared to the controls, at LMV onset, LMV patients had more active disease in renal, mucocutaneous and hematologic systems. They had a higher degree of proteinuria, and SLE disease activity (mSLEDAI-2K score). At 3 months prior to LMV onset, the LMV group was more anemic and had higher proteinuria. The LMV patients received less prednisolone and fewer immunosuppressive drugs at LMV onset, and fewer immunosuppressive drugs at 3 months prior to it. They received a higher daily dose of prednisolone in both periods.ConclusionLMV is a rare manifestation in SLE. Active disease, particularly in the renal and hematologic systems, was common prior to LMV onset.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"9612033251344154"},"PeriodicalIF":1.9,"publicationDate":"2025-05-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144128116","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":"Clinical characteristics and efficacy of intrathecal therapy in pediatric lupus encephalopathy.","authors":"Ruoyu Li, Lizhi Chen, Liping Rong, Mengjie Jiang, Yuxin Pei, Ying Mo, Xiaoyun Jiang, Yuanyuan Xu","doi":"10.1177/09612033251344192","DOIUrl":"https://doi.org/10.1177/09612033251344192","url":null,"abstract":"<p><p>BackgroundTo summarize the clinical characteristics of pediatric lupus encephalopathy and to investigate the therapeutic efficacy of intrathecal methotrexate and dexamethasone in the treatment of pediatric lupus encephalopathy.MethodsA retrospective study was conducted on 83 children diagnosed with Neuropsychiatric systemic lupus erythematosus (NPSLE) at the Department of Pediatric Nephrology and Rheumatology of the First Affiliated Hospital of Sun Yat-sen University from January 2002 to December 2023. The intrathecal injection and non-injection groups were divided based on whether they received intrathecal injections of methotrexate and dexamethasone. Clinical symptoms, laboratory tests, renal biopsy pathology, disease activity, and treatments were compared between the two groups, and the efficacy of intrathecal injection therapy for NPSLE was also assessed.ResultsOf the 83 children with NPSLE, 14 were male and 69 were female. NPSLE was the initial manifestation in 12 (14.46%) patients, while 71 (85.54%) developed it after systemic lupus erythematosus onset. The most frequently observed symptoms were headache and seizures. Imaging (CT/MRI) in 81 children showed abnormalities in 64 (79.01%), with cerebral atrophy being most common. The results of electroencephalography in 21 patients demonstrated abnormalities in 14 cases, and 7 of the 29 patients exhibited abnormal cerebrospinal fluid findings. A total of 68.67% of NPSLE patients were classified as chronic kidney disease (CKD) stage 1, while 31.33% were CKD stage 2 or higher. Renal biopsy in 60 children commonly indicated class IV or IV+V. The SLEDAI score at initial consultation was 20.93 ± 6.41. Among the 83 patients, 10 (12.05%) received intrathecal injections with an average of 5.2 per patient. Before treatment, the injection group had higher SLEDAI scores (<i>p</i> < 0.05). After treatment, the resolution times for NPSLE-related symptoms and imaging were shorter in the injection group, although the difference did not reach statistical significance. Notably, the injection group had a lower SLEDAI score and a more pronounced reduction (<i>p</i> < 0.05).ConclusionChildren with NPSLE in our center demonstrated more severe disease and higher disease activity index. Methotrexate and dexamethasone intrathecal therapy provided faster symptomatic relief and reduced disease activity in children with NPSLE.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"9612033251344192"},"PeriodicalIF":1.9,"publicationDate":"2025-05-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144120217","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 : 2025-05-21DOI: 10.1177/09612033251345020
James Orozco, Jessica Nguyen, Sara Mm Yasrebi, Marnie Blalock, Sara L Grisales, Craig L Jensen, Marietta M DeGuzman
{"title":"Severe gastrointestinal manifestations in childhood-onset lupus: A single-center cohort and review of the literature.","authors":"James Orozco, Jessica Nguyen, Sara Mm Yasrebi, Marnie Blalock, Sara L Grisales, Craig L Jensen, Marietta M DeGuzman","doi":"10.1177/09612033251345020","DOIUrl":"https://doi.org/10.1177/09612033251345020","url":null,"abstract":"<p><p>ObjectiveSystemic lupus erythematosus (SLE) is a complex autoimmune condition with diverse manifestations. Childhood-onset SLE (cSLE) presents more severely than adult-onset disease with a higher incidence of severe neurologic or renal manifestations and lower estimated survival rates. There is limited literature on the range of severe gastrointestinal manifestations at presentation in cSLE, and this lack of information results in underappreciation of these potentially life-threatening complications and little guidance regarding management of these patients.MethodsWe reviewed cases of patients with cSLE and severe GI manifestations at presentation diagnosed at our institution and additionally provide a comprehensive review of existing cases in the literature to discuss presenting symptoms, other clinical features, longitudinal course, treatment, and long-term outcomes.ResultsWe identified six cases of cSLE with primary, severe GI symptoms at time of diagnosis at our institution and an additional 25 patients who presented similarly in the literature. Severe GI manifestations included protein-losing enteropathy, thoracoabdominal aortitis, severe pancreatitis, lupus enteritis or mesenteric vasculitis, and intestinal pseudo-obstruction. Delays in diagnosis affected several patients, and 26% of patients required surgical intervention. Many patients required intensive immunomodulatory treatment in addition to prolonged bowel rest and parenteral nutrition. Long-term outcomes of GI manifestations varied overall, however, most patients at our institution achieved clinical remission.ConclusionsUnderstanding these rare cases of severe GI manifestations in cSLE can aid clinicians in prompt diagnosis and collaborative management, improving clinical outcomes for this vulnerable population.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"9612033251345020"},"PeriodicalIF":1.9,"publicationDate":"2025-05-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144120221","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 : 2025-05-21DOI: 10.1177/09612033251344195
Hiba I Khogali, Aaesha M Alhebsi, Meera A Altamimi, Ghada Sm Al-Bluwi, Virgie Guy Pedo, Ali M Al Dhanhani
{"title":"Infection vulnerability in pregnant women with and without systemic lupus erythematosus.","authors":"Hiba I Khogali, Aaesha M Alhebsi, Meera A Altamimi, Ghada Sm Al-Bluwi, Virgie Guy Pedo, Ali M Al Dhanhani","doi":"10.1177/09612033251344195","DOIUrl":"https://doi.org/10.1177/09612033251344195","url":null,"abstract":"<p><p>ObjectiveSystemic lupus erythematosus (SLE) is a heterogeneous autoimmune disease that predominantly affects females. SLE poses risks to the mother and fetus during pregnancy. Information on infection rates during pregnancy in patients with SLE is scarce. Therefore, this study assessed infection risk during pregnancy in women with and without SLE, and identified the factors influencing susceptibility to infections.MethodsWe conducted a retrospective cohort analysis using medical records from Tawam Hospital, Abu Dhabi, United Arab Emirates. Emirati women diagnosed with SLE who experienced one or more pregnancies between January 2010 and August 2023 were included in the study. Patients with SLE were compared with an age-matched control group of women without SLE. The control group was systematically selected from the hospital records of the same period. Demographic details, clinical data, and comprehensive infection histories during and before pregnancy were examined. Univariate and multivariate regression analyses were used to identify the factors contributing to the risk of infection in pregnant women with SLE.ResultsData from 87 pregnancies in 41 women with SLE were analyzed. The mean age at conception was higher in women with SLE compared to the control group. The rates of infections (54.7% vs 29.8%), adverse fetal outcomes (48.3% vs 24.2%), and maternal outcomes (71.3% vs 43%) were significantly higher in the SLE group than in the control group. Respiratory infections were the most prevalent infection type. No variable was found to increase the risk of infection; however, hydroxychloroquine use during pregnancy was significantly associated with a reduced risk of infection.ConclusionsThe risks of infection and adverse outcomes are significantly higher in pregnant women with SLE than in the non-SLE population. Hydroxychloroquine use during pregnancy was crucial in reducing infection risk. These findings underscore the need for targeted interventions and a multidisciplinary care approach to improve pregnancy outcomes in women with SLE.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"9612033251344195"},"PeriodicalIF":1.9,"publicationDate":"2025-05-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144110800","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 : 2025-05-21DOI: 10.1177/09612033251345193
Lin-Lin Li, Qiu-Rui Li, Lu Li, Hui-Xia Cao, Feng-Min Shao
{"title":"Commonalities between thyroid disease and systemic lupus erythematosus (SLE).","authors":"Lin-Lin Li, Qiu-Rui Li, Lu Li, Hui-Xia Cao, Feng-Min Shao","doi":"10.1177/09612033251345193","DOIUrl":"https://doi.org/10.1177/09612033251345193","url":null,"abstract":"<p><p>Systemic lupus erythematosus (SLE) is characterized by multiple organ involvements. One notable manifestation of SLE is thyroid disease, which has been documented in various studies with significant variability in prevalence and presentation. Observational studies have indicated that thyroid dysfunction, particularly autoimmune thyroid disease (AITD) characterized by hypothyroidism, is commonly associated with SLE. Despite these observation, the exact relationship between SLE and thyroid disease still remain poorly understood. Several factors may contribute to the pathogenesis of SLE companied with AITD, including genetic susceptibility, environmental influences, pharmacological agents, infections and immune system dysregulation. In light of these considerations, we conducted a review of the literature to observe the associations and potential mechanisms linking thyroid disease to SLE. This review aims to shed light on the complexities of these interrelated conditions and provide insights into their co-occurrence.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"9612033251345193"},"PeriodicalIF":1.9,"publicationDate":"2025-05-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144120220","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 : 2025-05-20DOI: 10.1177/09612033251344045
Paulo Rogério Julio, Carla Helena Capello, Jaqueline Cristina de Amorim, Samuel de Oliveira Andrade, Laura Silveira-Moriyama, Ana Carolina Coan, Simone Appenzeller
{"title":"Chorea associated with systemic lupus erythematosus- a systematic review.","authors":"Paulo Rogério Julio, Carla Helena Capello, Jaqueline Cristina de Amorim, Samuel de Oliveira Andrade, Laura Silveira-Moriyama, Ana Carolina Coan, Simone Appenzeller","doi":"10.1177/09612033251344045","DOIUrl":"https://doi.org/10.1177/09612033251344045","url":null,"abstract":"<p><p>ObjectiveChorea is one of the 19 neuropsychiatric manifestations observed in systemic lupus erythematosus (SLE). This article reviews the literature to determine the prevalence and clinical and laboratory features associated with chorea in SLE.MethodsWe performed a literature review following the PRISMA guidelines and using the following MESH terms in the search and analysis: \"Chorea\", \"movement disorders\", \"systemic lupus erythematosus\", \"SLE\", \"neuropsychiatric manifestations\", \"neuropsychiatric lupus (NPSLE)\", with full articles in English, Portuguese, and Spanish, between September 1999 and January 2024, in PubMed and Scielo.Results13,922 patients from 60 cohort studies and 41 patients from 18 case/series reports were included. Chorea had a pooled prevalence of 1.1% in SLE patients. We found no association between chorea and clinical or immunological features reported in cohort studies. In case series and case reports, chorea occurred more frequently before or at SLE diagnosis and more frequently in childhood-onset SLE. We propose an investigation and treatment scheme based on the literature review.ConclusionChorea is a rare manifestation in SLE and factors associated with its occurrence have not been addressed in cohort studies.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"9612033251344045"},"PeriodicalIF":1.9,"publicationDate":"2025-05-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144102190","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 : 2025-05-20DOI: 10.1177/09612033251344983
Weiwei Huang, Jianhua Dong, Song Luo, Jun Zhang, Lingyan Zhang, Yan Ma, Bin Wang, Jingxin Wang, Weiqiang Dou, Li Qi, Weixin Hu, Longjiang Zhang
{"title":"Cardiac magnetic resonance feature tracking evaluates left atrial strain in patients with systemic lupus erythematosus and mitral regurgitation.","authors":"Weiwei Huang, Jianhua Dong, Song Luo, Jun Zhang, Lingyan Zhang, Yan Ma, Bin Wang, Jingxin Wang, Weiqiang Dou, Li Qi, Weixin Hu, Longjiang Zhang","doi":"10.1177/09612033251344983","DOIUrl":"https://doi.org/10.1177/09612033251344983","url":null,"abstract":"<p><p>PurposeTo explore left atrial (LA) function in patients with systemic lupus erythematosus (SLE) and mitral regurgitation assessed by cardiac magnetic resonance imaging feature tracking (MRI-FT).MethodIn this study, 64 SLE patients (35.0 ± 12.3 years, 49 females) and 50 age- and gender-matched healthy controls (32.1 ± 11.2 years, 32 females) were analyzed. Patients with SLE were further classified into two subgroups according to mitral regurgitation defined by echocardiography: mitral regurgitation subgroup (<i>n</i> = 32) and non-mitral regurgitation subgroup (<i>n</i> = 32). All subjects underwent cardiac magnetic resonance (CMR) examination and SLE patients underwent extra laboratory testing. LA volume and strain parameters were compared among the three groups, and a correlation analysis was further performed between CMR parameters and clinical variables.ResultsPatients in both mitral regurgitation and non-mitral regurgitation subgroups showed higher LAVmin (29.15 ± 7.5, 26.22 ± 8.23 vs 21.68 ± 7.67, <i>p</i> = .003, .026) and LAVimin (17.44,14.9 vs 12.62, <i>p</i> = .002,.046) than healthy control subjects. Abnormal LA reservoir, conduit and bump strain were also observed in SLE patients (all <i>p</i> < .05), with more severe reservoir (<i>p</i> = .006, .031) and bump strain (<i>p</i> = .01, .033) abnormality found in mitral regurgitation subgroup. In the SLE group, LA reservoir, bump and conduit strain were significantly positively correlated with LVEF (B = 0.467, <i>p</i> < .001 vs B = 0.019, <i>p</i> = .01 vs B = 0.168, <i>p</i> = .001 vs B = 0.036, <i>p</i> < .001 vs B = 0.020, <i>p</i> = .024). Conduit strain (εe, SRe) was positively correlated with LAEF (B = 0.229, <i>p</i> = .032 vs B = 0.027, <i>p</i> = .008).ConclusionMRI-FT based LA parameters may be considered a helpful tool to evaluate LA function in SLE patients, and LA strain may indicate severity of the cardiac dysfunction in SLE patients.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"9612033251344983"},"PeriodicalIF":1.9,"publicationDate":"2025-05-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144102163","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 : 2025-05-20DOI: 10.1177/09612033251344180
Santiago Dans-Caballero, Irene Cecchi, Massimo Radin, Savino Sciascia
{"title":"JAK inhibitors for the management of rheumatic diseases when antiphospholipid syndrome co-exists: case-based safety considerations.","authors":"Santiago Dans-Caballero, Irene Cecchi, Massimo Radin, Savino Sciascia","doi":"10.1177/09612033251344180","DOIUrl":"https://doi.org/10.1177/09612033251344180","url":null,"abstract":"<p><p>JAK inhibitors (JAKi) are small molecules that interact with JAK proteins, modulating the JAK-STAT signaling pathway, which plays a significant, though not yet fully understood, role in immune regulation. Due to the breadth of their mechanism of action, JAKi have shown promising results in the treatment of various immune-mediated diseases across different fields such as rheumatology or dermatology, and may represent a valuable therapeutic option for patients with multiple coexisting immune-mediated conditions. However, recent years have seen growing concerns regarding their use due to an observed increase in cardiovascular and thromboembolic events compared to anti-TNF drugs, which may complicate administration in patients with additional associated risk factors.Given the possible increase in thromboembolic events among patients treated with JAKi, various regulatory agencies advise against their use in patients with additional risk factors, such as advanced age or a prior thromboembolic event. This poses challenges in conditions like antiphospholipid syndrome (APS), an acquired hypercoagulability disorder mediated by antibodies, where thromboembolic events are a hallmark feature. To date, no publications have formally evaluated the safety of JAKi in APS patients in real-world clinical practice.Through a case-based approach, we aim to highlight the safety of these drugs for patients with APS and coexisting immune-mediated diseases, while emphasizing the importance of a careful assessment of additional risk factors and shared decision-making with the patient.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"9612033251344180"},"PeriodicalIF":1.9,"publicationDate":"2025-05-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144110839","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}