Lupus最新文献

{"title":"Investigating the association between antiphospholipid syndrome and ovarian reserve: A systematic review and meta-analysis of the literature.","authors":"Nooshin Hemmati, Maryam Sahebari, Mona Larki, Vahid Ghavami, Elham Manouchehri","doi":"10.1177/09612033251332051","DOIUrl":"https://doi.org/10.1177/09612033251332051","url":null,"abstract":"<p><p>BackgroundAutoimmune diseases can reduce ovarian reserves. Women in reproductive ages are susceptible to an autoimmune disorder known as antiphospholipid syndrome (APS). The aim of this study is to investigate the association between APS and ovarian reserve (OR).MethodPubMed, Scopus, Web-of-Science, Science Direct, and the Google scholar search engine were searched (22 June 2024) for studies that investigated the effect of APS on OR. Literature screening, data extraction, and assessment of the risk of bias of the included studies were conducted by two reviewers independently. Mean differences were computed using a random effects model. Heterogeneity was assessed by I²%.ResultsFour cross-sectional studies were included in this meta-analysis. None of the studies had a high risk of bias. There was no significant association identified between primary (MD = -0.27, 95% CI, -1.42 to 0.87, <i>p</i> = 0.639) and secondary APS (SMD = -0.38, 95% CI, -2.46 to 1.69, <i>p</i> = 0.717) with antimullerian hormone amounts. The antral follicle count (AFC) was investigated in two studies revealed lower levels of AFC in women with primary APS. Regarding the levels of gonadotropins and estradiol in the participants' serum, the results are contradictory.ConclusionsThe results of this meta-analysis identified there is no relationship between primary and secondary APS with the reduction of ovarian reserves in women with APS. This issue should be considered in the reproductive health of women with APS, who can have children at the right time by consulting a rheumatologist and reproductive health specialist.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"9612033251332051"},"PeriodicalIF":1.9,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143753340","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":"Disease exacerbation and COVID-19 following mRNA COVID-19 vaccination in adolescents with Systemic Lupus Erythematosus.","authors":"Sutheera Thepveera, Sirirat Charuvanij, Maynart Sukharomana, Yanarin Thunsiribuddhichai, Kraisoon Lomjansook, Thanaporn Chaiyapak, Anirut Pattaragarn, Achra Sumboonnanoda, Nuntawan Piyaphanee","doi":"10.1177/09612033251331244","DOIUrl":"https://doi.org/10.1177/09612033251331244","url":null,"abstract":"<p><p>ObjectivesTo evaluate disease flares and associated factors, as well as the Coronavirus disease 2019 (COVID-19) among adolescents with systemic lupus erythematosus (SLE) after receiving COVID-19 vaccination. Additionally, it sought to determine any difference in year-on-year flare rates before and after vaccination.MethodsWe conducted a 12-month prospective study in adolescent SLE (adoSLE) patients aged 12-18 years who had no prior history of COVID-19 and received a 2-dose BNT162b2 mRNA COVID-19 vaccine. A booster dose was administered 4-6 months later, depending on vaccine availability and patient acceptance. Clinical characteristics, the safety of estrogens in lupus erythematosus national assessment-SLE disease activity index (SELENA-SLEDAI) flare index, and renal flare were evaluated and contrasted against pre-vaccination data. COVID-19 during follow-up were noted.ResultsSixty-nine vaccinated adoSLE patients, with the mean age of 15.8 ± 1.6 years and female predominant (92.8%), were included. Forty-six (66.7%) patients received a booster dose at 4-6 months after primary series. Compared between pre- and post- COVID-19 vaccination, year-on-year flare rates remained consistent [20 (29.0%) versus 24 (34.8%), <i>p</i> = .371]. Non-use of hydroxychloroquine (adjusted odds ratio [aOR] 18.83, 95% CI: 1.97, 179.60, <i>p</i> = .011) and a SELENA-SLEDAI score ≥8 within 12 months prior to vaccination (aOR 5.33, 95% CI: 1.38, 20.55, <i>p</i> = .015) were independent factors of disease flares. An increment in post-vaccine renal flare rate was observed [6 (8.7%) versus 14 (20.3%), <i>p</i> = .046]. Among 14 adoSLE patients with renal flare, 13 (92.9%) patients had previous lupus nephritis, and new-onset proteinuria or increased proteinuria (71.4%) was the most common finding. Thirty-four (49.3%) patients contracted COVID-19 within a year post-vaccination, all presenting with mild to moderate symptoms; among the 46 patients who received a booter, 15 (32.6%) experienced COVID-19.ConclusionsCOVID-19 vaccination is effective and safe in preventing severe COVID-19 among adoSLE patients, without increasing annual SLE flare rates. However, close monitoring for renal flares is recommended, particularly for patients with a history of LN. Although vaccinated adoSLE patients contracted COVID-19, their outcomes were favorable.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"9612033251331244"},"PeriodicalIF":1.9,"publicationDate":"2025-03-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143743616","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":"Reversible lymphoproliferative disorder in systemic lupus erythematosus treated with mycophenolate mofetil: A case report and literature review.","authors":"Hiroto Tomoda, Muneo Nakaya, Seiichiro Nakabeppu, Hiroaki Miyoshi, Koichi Ohshima, Yuji Miyoshi","doi":"10.1177/09612033251330101","DOIUrl":"https://doi.org/10.1177/09612033251330101","url":null,"abstract":"<p><p>BackgroundMycophenolate mofetil (MMF) is widely used to treat systemic lupus erythematosus (SLE), particularly in cases with severe and organ-threatening complications such as lupus nephritis. However, multiple reports have documented lymphoproliferative disorder (LPD) in patients with SLE undergoing MMF therapy, predominantly developing in the central nervous system and requiring aggressive treatment, including chemotherapy, radiation, and surgery.Case ReportWe herein report the case of a 58-year-old female patient with SLE who developed cervical, hepatic hilar, and para-aortic lymphadenopathy 8 years after initiating MMF treatment. Histopathological examination of the left cervical lymph node revealed features consistent with polymorphic LPD. MMF was discontinued, and after 2 months of surveillance, the enlarged lymph nodes regressed without need for additional treatment.ConclusionTo the best of our knowledge, this is the first reported case of MMF-associated LPD occurring outside the central nervous system in a patient with SLE, which resolved spontaneously upon MMF withdrawal. Clinicians should remain vigilant regarding the possibility of MMF-associated LPD when administering MMF to patients with SLE.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"9612033251330101"},"PeriodicalIF":1.9,"publicationDate":"2025-03-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143729521","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":"Skin and beyond: Demographics, clinical features and systemic involvement of cutaneous lupus erythematosus in Pakistani population.","authors":"Misbah Zakir Abowath, Erum Ashraf, Umair Zakir Abowath","doi":"10.1177/09612033251330115","DOIUrl":"https://doi.org/10.1177/09612033251330115","url":null,"abstract":"<p><p>BackgroundLupus erythematosus (LE) is an autoimmune disease with skin being the most important organ involved and presents as LE specific and LE nonspecific lesions.ObjectiveTo determine the clinical demographics, systemic involvement and disease impact on daily lives of patients of cutaneous lupus erythematosus (CLE).MethodologyA cross sectional study of a total of 135 patients who presented with cutaneous symptoms were enrolled. Informed consent was taken. Detailed History, examination, clinical features, laboratory and radiograph investigations were conducted to assess the systemic involvement of internal organs.ResultsOf the 135 patients, 73.3% patients were female and 26.7% were male. Mean age was 28.1 years and duration of CLE was 8.4 months. 56.3% of patients belonged to the rural area. Isolated Chronic CLE (CCLE) was observed in 35.5%, CCLE with overlap Acute CLE (ACLE) in 30.3% and Isolated ACLE in 33.3%. Only two patients had Sub acute CLE (SCLE). In LE specific lesions, limited and generalized discoid rash was noted in 33.3% and 17.7% respectively. 46.6% patients had Malar rash. Oral and nasal ulcers were observed in 51.1 %. Non scarring alopecia was seen in 53.3% of patients. Scarring alopecia was observed in 35.5%. 34% patients had Raynaud's phenomenon, 23.7% had Periungual telangiectasia, and 14.8% had Vasculitis. In systemic involvement, joint involvement was observed in 49 patients (36.3%), renal in 48 patients (35.6%), haematological in 42 patients (31.1%), serositis, 23 patients (17%) and neurological in 14 patients (10.4%). No difference in sexes was observed. ANA positivity was seen in 61 patients, (45.1%), most of whom were females. (56.5%) Anti DSDNA was positive in 42 patients (31.1%) of which 40 were women. Mean DLQI was 11.01 ± 8.2. 45 patients had no or slight effect on their lives. 22.9%, 27.4% and 16.3% had moderate, large and very large effects of CLE on their lifestyle.ConclusionThe clinical characteristics of cutaneous lupus is similar to other studies, but the renal involvement is higher with cutaneous involvement in this study, while Serositis is less prevalent. Localized discoid lupus did not show extra cutaneous involvement, but showed significant impact on daily life.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"9612033251330115"},"PeriodicalIF":1.9,"publicationDate":"2025-03-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143729914","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":"Significant association of functional variants in the promoter sequence of <i>IL18</i> with disease susceptibility and systemic lupus erythematosus clinical parameters.","authors":"Yousef Mohammadi, Mozhdeh Saghaei, Mostafa Saghi, Seyed Amirhosein Mazhari, Behrang Alani, Naeim Ehtesham, Taiebe Kenarangi, Mohsen Soosanabadi","doi":"10.1177/09612033251331256","DOIUrl":"https://doi.org/10.1177/09612033251331256","url":null,"abstract":"<p><p>ObjectiveSystemic lupus erythematosus (SLE) is a chronic autoimmune disease with a complex etiology. Interleukin-18 (IL-18) possesses pro-inflammatory properties and plays a central role in the development of SLE. In this study, we assessed the association between two functional variants that affect the expression of <i>IL-18</i>, namely -607C > A (rs1946518) and -137G > C (rs187238), and the risk of SLE development.MethodsAs a case-control study, 251 peripheral blood samples were collected from 121 SLE patients and 130 healthy participants. Genotyping of these polymorphisms was performed using the high-resolution melting (HRM) method, which employs real-time polymerase chain reaction.ResultsOur findings revealed a significant association between the AA genotype and A allele in rs1946518, showing a decreased risk of SLE (AA vs CC; OR: 0.386; 95% CI [0.174-0.828], A vs C; OR: 0.548; 95% CI [0.369-0.809]). Analogously, the CC genotype and C allele in rs187238 exhibited a similar trend (CC vs GG; OR: 0.240; 95% CI [0.055-0.803], C vs G; OR: 0.604; 95% CI [0.390-0.928]), indicating a reduced risk of SLE Moreover, SLE subjects with the protective allele in rs1946518 (AA + AC) demonstrated significantly lower levels of CRP, and Anti-dsDNA, suggesting lower disease activity. These patients also had a later age of onset, and a lower incidence of renal involvement and creatinine levels, indicating milder disease severity (<i>p</i> < <i>.05</i>).ConclusionThe study indicates a significant relationship between the rs1946518 and rs187238 variants in <i>IL-18</i> and a reduced risk of SLE. Furthermore, rs1946518 was found to be associated with certain clinical features related to disease activity and severity.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"9612033251331256"},"PeriodicalIF":1.9,"publicationDate":"2025-03-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143743618","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":"Progenitor cells and circulating endothelial cells are associated with disease activity and damage in systemic lupus erythematosus patients.","authors":"Gonzalo Silveira, Sabrina Ranero, Adriana Carlomagno, Andreina Brugnini, Natalia Trias, Daniela Lens, Martín Rebella, Álvaro Danza, Sofía Grille","doi":"10.1177/09612033251330124","DOIUrl":"https://doi.org/10.1177/09612033251330124","url":null,"abstract":"<p><p>BackgroundDespite advancements in treatment, patients with Systemic Lupus Erythematosus (SLE) frequently experience disease flares, which contribute to organ damage and increase the risk of premature death. Assessing disease activity is essential for optimizing treatment and preventing further organ damage. This study aimed to investigate the relationship between levels of progenitor and circulating endothelial cells and SLE disease activity, as well as accumulated organ damage.MethodsWe conducted a case-control study measuring levels of CD34+CD45low/- progenitor cells, CD34+CD45low/-CD133+ progenitor cells, Endothelial Progenitor Cells (EPC), and Circulating Endothelial Cells (CEC) in peripheral blood using flow cytometry.ResultsThe study included 32 SLE patients and 28 matched controls. SLE patients exhibited significantly lower levels of CD34+CD45low/- progenitor cells (<i>p</i> = .001), CD34+CD45low/-CD133+ progenitor cells (<i>p</i> = .016), EPC (<i>p</i> = .018), and CEC (<i>p</i> < .001) compared to controls. Additionally, the cell subpopulations correlated with SLE activity biomarkers, with CD34+CD45low/- progenitor cells showing a moderate negative correlation with C3 and C4 levels. Notably, patients with an SDI score ≥1 had significantly higher levels of CD34+CD45low/- progenitor cells, CD34+CD45low/- CD133+ progenitor cells, EPC, and CEC compared to those without organ damage (<i>p</i> = .0073, <i>p</i> = .018, <i>p</i> = .018, and <i>p</i> = .020, respectively).ConclusionOur findings reveal that CD34+CD45low/- progenitor cells, CD34+CD45low/-CD133+ progenitor cells, EPC, and CEC are significantly reduced in SLE patients and are associated with disease activity and organ damage. These results suggest that CD34+CD45low/- progenitor cells, in particular, could serve as potential biomarkers for monitoring disease activity and organ damage in SLE patients. Prospective studies are warranted to confirm these findings.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"9612033251330124"},"PeriodicalIF":1.9,"publicationDate":"2025-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143700792","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":"Barriers and motivations to exercise participation among women with severe systemic lupus erythematosus who have recently undergone glucocorticoid pulse therapy: A qualitative analysis.","authors":"Juliana M Barboza, Gabriel P Esteves, Willian Jd Ribeiro, Vithor R Fickert, Andre S Franco, Luciana Parente Costa Seguro, Hamilton Roschel, Bruno Gualano, Eimear Dolan","doi":"10.1177/09612033251330116","DOIUrl":"https://doi.org/10.1177/09612033251330116","url":null,"abstract":"<p><p>BackgroundPhysical activity is important in the management of systemic lupus erythematosus, yet available evidence indicates that inactivity and poor physical fitness are commonplace. Individuals with SLE, and particularly those whose condition severity warrants aggressive pharmacological treatment such as glucocorticoid pulse therapy, may face substantial barriers to exercise participation. A better understanding of these may be useful to develop targeted and effective exercise recommendations and strategies.PurposeTo explore motivations and barriers for exercise participation in a group of women with SLE who have recently undergone glucocorticoid pulse therapy.MethodsThis is a cross-sectional, exploratory study, whereby participants underwent individually administered semi-structured interviews related to personal motivations and barriers to exercise training. Self-reported well-being and quality of life were assessed using the Systemic Lupus Erythematosus Quality of Life (SLEQOL) and SF-36 questionnaires.ResultsTwenty-three women, with a high median level of disease activity (SLEDAI 8; IQR: 4-12) participated in the study. All participants reported wide-ranging health benefits as a motivation to exercise, but the majority did not exercise. They cited numerous health-related, personal and social barriers to exercise participation. SF-36 results indicated that the group experienced difficulties in participating in work and other regular daily activities.ConclusionAlthough the group recognized the health-related benefits of exercise, this knowledge was insufficient to encourage exercise participation. Exercise professionals must remain cognizant of the wide-ranging health-related, personal and social barriers that may exist for this group, and to consider these when formulating exercise recommendations.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"9612033251330116"},"PeriodicalIF":1.9,"publicationDate":"2025-03-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143692694","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":"Association between extracellular vesicles (EVs) and thrombosis in antiphospholipid syndrome.","authors":"Bruna Cardoso Jacintho-Robison, Jose Diogo Oliveira, Lucas Matheus Bispo Césped, Cristiane Maria de Souza, Bárbara Gomes Barion, Camila de Oliveira Vaz, Bruna De Moraes Mazetto, Fernanda Andrade Orsi","doi":"10.1177/09612033251330099","DOIUrl":"https://doi.org/10.1177/09612033251330099","url":null,"abstract":"<p><p>BackgroundAntiphospholipid syndrome (APS) is characterized by thrombosis or pregnancy complications associated with the presence of antiphospholipid antibodies (aPLs). Although the exact mechanisms are unclear, aPLs can increase the expression of tissue factor on platelets, leukocytes, and endothelial cells, leading to hypercoagulability. Extracellular vesicles (EVs) can also be released during this process and play a key role in immune regulation and thrombosis related to APS.AimsTo evaluate the association between circulating levels of EVs and thrombosis related to APS, as well as inflammatory markers.MethodsCase-control study including patients with thrombotic APS (t-APS) and healthy controls (HC). EVs expressing the following antigens were quantified by flow cytometry: CD41 (platelet integrin alpha IIb), CD162 (P-selectin glycoprotein ligand 1), CD31 (platelet and endothelial cell adhesion molecule 1), CD142 (tissue factor), and CD62 (P-selectin). EV levels were compared between groups and correlated with APS clinical and inflammatory parameters.ResultsA total of 69 t-APS patients and 46 HC were included. CD162+EV, CD31+EV, and CD41+EV levels were higher in t-APS patients compared to controls. CD41+EV levels were associated with venous thrombosis (<i>p</i> = .04) and multiple thrombosis (<i>p</i> = .07). Levels of CD162+EV, CD31+EV, CD142+EV and CD62P + EV were positively correlated with levels of interleukin-1 beta (IL-1β).ConclusionEVs expressing antigens related to platelet and endothelial cell activation and adhesion, as well as platelet-leukocyte interaction, were associated with thrombosis related to APS. The correlation between EV levels and IL-1β levels further underscore the association between EV release and thromboinflammatory responses in APS. Our results demonstrate the involvement of EVs in the interaction between inflammation and thrombosis in APS.</p>","PeriodicalId":18044,"journal":{"name":"Lupus","volume":" ","pages":"9612033251330099"},"PeriodicalIF":1.9,"publicationDate":"2025-03-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143692693","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":"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}

{"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}