Vasiliki-Kalliopi Bournia, George E Fragoulis, Panagiota Mitrou, Anastasios Tsolakidis, Konstantinos Mathioudakis, Dimitrios Vassilopoulos, Maria Tektonidou, Dimitrios Paraskevis, Petros P Sfikakis
{"title":"Increased prevalence of inflammatory arthritis, systemic lupus erythematosus and systemic sclerosis, during 2020-2023 versus 2016-2019 in a Nation-Wide Cohort Study.","authors":"Vasiliki-Kalliopi Bournia, George E Fragoulis, Panagiota Mitrou, Anastasios Tsolakidis, Konstantinos Mathioudakis, Dimitrios Vassilopoulos, Maria Tektonidou, Dimitrios Paraskevis, Petros P Sfikakis","doi":"10.1007/s00296-024-05733-y","DOIUrl":"10.1007/s00296-024-05733-y","url":null,"abstract":"<p><p>Although several studies have explored the geoepidemiology of autoimmune rheumatic diseases (ARD), trends of their frequency overtime are under-investigated. Herein, in a nation-wide study, we examine trends in the prevalence of various ARD over-time, taking also into account the Covid-19 pandemic. In this retrospective study in the entire Greek adult population (approximately 10.000.000 people), we searched the electronic prescription database of the e-Government Centre for Social Security Services using prespecified ICD-10 codes to capture all adult patients with Psoriatic Arthritis (PsA), Rheumatoid Arthritis (RA), Ankylosing Spondylitis (AS), Systemic Lupus Erythematosus (SLE), Systemic Sclerosis (SSc) and Polymyalgia Rheumatica or Giant Cell Arteritis (PMR/GCA). Two sequential 4-year periods, namely 2016-2019 and 2020-2023 were compared. Prevalence of RA, PsA, AxSpA, SLE and SSc increased significantly during 2020-2023 compared to 2016-2019. This applies to both genders and to all age groups for RA, PsA and AxSpA, to female patients in SLE and SSc and to patients 18-39 years in SLE and ≥ 60 years in SSc. Overall, there was 47% increase in prevalence for AxSpA (0.100% in 2016-19 vs 0.147% in 2020-23), 36.5% for PsA (0.148% vs 0.202%), 20.6% for RA (0.467% vs 0.563%), 19% for SLE (0.137% vs 0.163%) and 13% for SSc (0.023% vs 0.026%). A 16.3% decrease was evident in GCA/PMR, limited to those ≥ 40 years old. In a nation-wide study we confirm that ARD prevalence increases over-time, whereas a contribution of Covid-19 pandemic to our results during 2020-2023, cannot be excluded. Additional human, medical and financial resources will be needed to cover the increased needs of ARD patients.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":" ","pages":"2837-2846"},"PeriodicalIF":3.2,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142392925","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Uncovering the alterations in extrinsic foot muscle mechanical properties and foot posture in fibromyalgia: a case-control study.","authors":"Ceren Ornek, Ilke Coskun Benlidayi, Aylin Sariyildiz","doi":"10.1007/s00296-024-05743-w","DOIUrl":"10.1007/s00296-024-05743-w","url":null,"abstract":"<p><strong>Objectives: </strong>The aim of the study was to evaluate foot posture and the mechanical properties of extrinsic foot muscles in fibromyalgia.</p><p><strong>Methods: </strong>Patients with fibromyalgia (n = 86) and age- and gender-matched controls (n = 41) were included in the study. Foot Posture Index (FPI), Beighton and Brighton criteria were used to evaluate static foot posture, joint hypermobility, and benign joint hypermobility syndrome (BJHS), respectively. Tonus, elasticity, and stiffness of the extrinsic foot muscles including gastrocnemius medialis, tibialis anterior, and peroneus longus were measured by the MyotonPRO<sup>®</sup> device. Foot function, quality of life, and physical activity level were assessed by the Foot Function Index (FFI), Short Form-36 (SF-36), and the International Physical Activity Questionnaire-Short Form (IPAQ-SF), respectively.</p><p><strong>Results: </strong>The frequency of abnormal foot posture in the fibromyalgia and control groups was 68.6% versus 39%, respectively; and neutral foot posture was approximately twice as prevalent in the control group compared to the fibromyalgia group (p = 0.006). Pronated foot posture was the most common abnormality in fibromyalgia (61.6%) and observed in 85.5% of the patients with joint hypermobility and in 87.1% of those meeting BJHS criteria. The elasticity of peroneus longus at rest and the elasticity of tibialis anterior in the standing position were significantly different between the fibromyalgia group and the control group [1.08 (0.22) vs. 1.02 (0.25), p = 0.037 and 0.92 (0.29) vs. 0.87 (0.24), p = 0.011, respectively]. Regarding the fibromyalgia group, no difference was detected among foot posture groups in terms of myotonometric data.</p><p><strong>Conclusions: </strong>Pronation foot posture is common in fibromyalgia with a much higher frequency in fibromyalgia patients with hypermobility. In daily clinical practice for fibromyalgia, particular attention should be given to foot alignment.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":" ","pages":"2997-3008"},"PeriodicalIF":3.2,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142584257","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Gernot Kriegshäuser, Hasmik Hayrapetyan, Christian Oberkanins, Tamara Sarkisian
{"title":"Plasminogen activator inhibitor-1 genotype 4G/5G associates with skin involvement in Armenian familial Mediterranean fever patients.","authors":"Gernot Kriegshäuser, Hasmik Hayrapetyan, Christian Oberkanins, Tamara Sarkisian","doi":"10.1007/s00296-024-05653-x","DOIUrl":"10.1007/s00296-024-05653-x","url":null,"abstract":"<p><p>There is little and conflicting data on the role of the plasminogen activator inhibitor-1 (PAI-1, SERPINE1) 4G/5G polymorphism in familial Mediterranean fever (FMF). Therefore this study aimed at evaluating the impact of this polymorphism on the disease course in a cohort of 303 Armenian FMF patients. Genotyping for 12 Mediterranean fever (MEFV) gene mutations and the PAI-1 4G/5G (rs1799762) polymorphism were performed by PCR/reverse-hybridization (StripAssay) and real-time PCR, respectively. PAI-1 genotypes 4G/4G, 4G/5G, and 5G/5G could be identified in 4 (5.88%), 30 (18.63%) and 9 (12.16%) patients with erysipelas-like erythema (ELE), while this was the case for 64 (94.12%), 131 (81.37%), and 65 (87.84%) patients without ELE, respectively (P < 0.033). We have identified a significant relationship between the PAI-1 4G/5G genotype and the occurence of ELE in a relatively large cohort of Armenian FMF patients. Because of conflicting results concerning the impact of this polymorphism on the clinical course of FMF in different populations, further studies are desirable to substantiate the findings reported here.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":" ","pages":"2555-2559"},"PeriodicalIF":3.2,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141555404","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Oya Koker, Sezgin Sahin, Mehmet Yildiz, Amra Adrovic, Ozgur Kasapcopur
{"title":"The emerging paradigm in pediatric rheumatology: harnessing the power of artificial intelligence.","authors":"Oya Koker, Sezgin Sahin, Mehmet Yildiz, Amra Adrovic, Ozgur Kasapcopur","doi":"10.1007/s00296-024-05661-x","DOIUrl":"10.1007/s00296-024-05661-x","url":null,"abstract":"<p><p>Artificial intelligence algorithms, with roots extending into the past but experiencing a resurgence and evolution in recent years due to their superiority over traditional methods and contributions to human capabilities, have begun to make their presence felt in the field of pediatric rheumatology. In the ever-evolving realm of pediatric rheumatology, there have been incremental advancements supported by artificial intelligence in understanding and stratifying diseases, developing biomarkers, refining visual analyses, and facilitating individualized treatment approaches. However, like in many other domains, these strides have yet to gain clinical applicability and validation, and ethical issues remain unresolved. Furthermore, mastering different and novel terminologies appears challenging for clinicians. This review aims to provide a comprehensive overview of the current literature, categorizing algorithms and their applications, thus offering a fresh perspective on the nascent relationship between pediatric rheumatology and artificial intelligence, highlighting both its advancements and constraints.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":" ","pages":"2315-2325"},"PeriodicalIF":3.2,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11424736/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141620889","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Donya Nemati, Daniel Quintero, Thomas M Best, Navin Kaushal
{"title":"Investigating the association between knee osteoarthritis symptoms with pain catastrophizing domains between Hispanics and non-Hispanic Whites.","authors":"Donya Nemati, Daniel Quintero, Thomas M Best, Navin Kaushal","doi":"10.1007/s00296-023-05396-1","DOIUrl":"10.1007/s00296-023-05396-1","url":null,"abstract":"<p><p>Knee osteoarthritis (KOA) is a chronic disease accompanied by debilitating symptoms including pain, stiffness, and limited physical functionality, which have been shown to be associated with pain catastrophizing. Previous studies have revealed racial discrepancies in pain catastrophizing, notably between Hispanics and non-Hispanics while pointing to potential health disparities. Using a conceptual model, this study aimed to investigate racial differences in associations between KOA symptoms with specific pain catastrophizing domains (rumination, magnification, and helplessness). Patients with KOA (n = 253; 147 Hispanics, 106 non-Hispanic Whites) completed a survey that included measures of knee symptoms, pain catastrophizing, and demographic variables. Structural equation modeling revealed that among Hispanics, each pain catastrophizing domain (rumination, magnification, and helplessness) was associated with at least two symptomatic experiences, including pain severity and difficulty in physical function. Specifically, pain severity was associated with (a) rumination: β = 0.48, p < 0.001, (b) magnification: β = 0.31, p = 0.003; and (c) helplessness: β = 0.39, p < 0.001). Additionally, a lower score in physical function was associated with higher magnification (β = 0.26, p = 0.01), and helplessness (β = 0.25, p = 0.01). Among non-Hispanic White patients, pain severity was further associated with two domains of pain catastrophizing, including rumination (β = 0.39, p < 0.001) and helplessness (β = 0.35, p = 0.01). In addition, association pathways for demographic variables revealed that older Hispanics experienced greater challenges with higher pain severity (β = 0.26, p = 0.01) and greater difficulty with physical function (β = 0.31, p < 0.001) while Hispanics females experienced higher pain (β = 0.19, p = 0.03). These findings highlight the importance of designing tailored interventions that consider key demographic factors such as age, and gender, to improve physical function that might alleviate pain catastrophizing among Hispanics with KOA.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":" ","pages":"2539-2546"},"PeriodicalIF":3.2,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10018101","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Sandra Garrote-Corral, Diana Botello Corzo, Jesús Loarce-Martos, Carlos de la Puente Bujidos, Loreto Carmona
{"title":"Efficacy and safety of intravenous immunoglobulin therapy in systemic sclerosis: a systematic review.","authors":"Sandra Garrote-Corral, Diana Botello Corzo, Jesús Loarce-Martos, Carlos de la Puente Bujidos, Loreto Carmona","doi":"10.1007/s00296-024-05613-5","DOIUrl":"10.1007/s00296-024-05613-5","url":null,"abstract":"<p><strong>Background and objective: </strong>Systemic sclerosis (SSc) is a highly heterogeneous disease whose treatment is based mainly on immunosuppressants, antifibrotics, and vasodilators. Intravenous immunoglobulin (IVIG) have proved effective in other autoimmune diseases. The objective of this study is to evaluate the efficacy and safety of IVIG in SSc.</p><p><strong>Methods: </strong>The systematic review was conducted according to the PRISMA Statement. Medline, Embase and Cochrane Library databases were searched until March 2024. We assessed the quality of included studies using the Cochrane Risk of Bias 2.0 tool (RoB 2) for randomised clinical trials and the Cochrane Risk in non-randomized studies (ROBINS-I) tool for observational studies.</p><p><strong>Results: </strong>From 1242 studies identified, 15 studies were included, of which 14 were observational studies. In total, 361 patients with SSc were included, and 295 received treatment with IVIG. Most of the studies used a dose of 2 g/kg IVIG. Ten studies, including the clinical trial, showed high risk of bias, and five had a critical risk. Skin involvement was assessed using modified Rodnan skin score, in 11 studies and the authors reported cutaneous efficacy in 9 of them. The 6 studies that assessed muscle involvement reported an improvement. Six studies reported data on gastrointestinal efficacy. Other domains such as lung and joint involvement and steroid-sparing effect were evaluated. The most frequent adverse events were mild, including headache, abdominal pain, fever, and skin rash.</p><p><strong>Conclusion: </strong>Treatment with IVIG in SSc patients could be helpful and safe in patients with cutaneous, muscular, or digestive manifestations.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":" ","pages":"2357-2370"},"PeriodicalIF":3.2,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140923116","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Kerem Yiğit Abacar, Şeyma Çolakoğlu-Özkaya, Erhan Bıyıklı, Onur Buğdaycı, Meltem Kurşun, Ayberk Denizli, Beril Koçak, Aysun Aksoy, Can Erzik, Pınar Ay, Murat Bezer, Mehmet Tuncay Duruöz, Haner Direskeneli, Pamir Atagündüz
{"title":"Pelvic parameters as prognostic factors of radiographic progression in classical Ankylosing Spondylitis: A prospective follow-up data.","authors":"Kerem Yiğit Abacar, Şeyma Çolakoğlu-Özkaya, Erhan Bıyıklı, Onur Buğdaycı, Meltem Kurşun, Ayberk Denizli, Beril Koçak, Aysun Aksoy, Can Erzik, Pınar Ay, Murat Bezer, Mehmet Tuncay Duruöz, Haner Direskeneli, Pamir Atagündüz","doi":"10.1007/s00296-024-05646-w","DOIUrl":"10.1007/s00296-024-05646-w","url":null,"abstract":"<p><p>Radiographic progression in Ankylosing spondylitis (AS) is driven by mechanical strain. A well-balanced spine provides a favorable weight distribution across the entheses. Pelvic parameters are useful in assessing the shape of the spine. The present study aimed to prospectively investigate the predictive value of pelvic parameters for radiographic progression in AS. This non-interventional, observational, and prospective study enrolled AS patients fulfilling the modified New York criteria (mNY) currently under follow-up in the MARS (MARmara Spondyloarthritis) outpatient clinics. The primary objective was to investigate the relationship between the baseline pelvic parameters and radiographic progression in the spine. Two trained radiologists (EB, OB) independently assessed the modified Stoke Ankylosing Spondylitis Spine Score (mSASSS). An orthopedic surgeon (AHA) and a radiologist (EB) derived the pelvic parameters. Patients with no bridging or bamboo spine were included in the final analysis. Risk assessment for radiographic progression, defined as a two-unit increase in mSASSS or developing a new syndesmophyte every two years, was done using uni- and multivariate logistic regression analyses. Radiographs of 69 AS patients were analyzed. The median (IQR 25-75) prospective follow-up was 47.7 (34.6-52.8) months. Only 33.3% (23/69) had radiographic progression. The pelvic tilt (PT) was lower in patients with radiographic progression (p = 0.037) and each degree of decrease in PT provided a 9% increase in risk for radiographic progression. Male patients were 7.5 times more likely to progress. Pelvic parameters provide a prognostic insight into the radiographic progression in AS. Our observations may aid in selecting patient-specific interventions in addition to anti-inflammatory treatments.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":" ","pages":"2421-2430"},"PeriodicalIF":3.2,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141564190","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Kristian Vogt, Christian Bijan Fink, Teresa Maria Schreibing, Stefan Krämer, Sebastian Reinartz, Thomas Rauen
{"title":"Distinct pulmonary patterns in ANCA-associated vasculitides: insights from a retrospective single center cohort study.","authors":"Kristian Vogt, Christian Bijan Fink, Teresa Maria Schreibing, Stefan Krämer, Sebastian Reinartz, Thomas Rauen","doi":"10.1007/s00296-024-05664-8","DOIUrl":"10.1007/s00296-024-05664-8","url":null,"abstract":"<p><p>ANCA-associated vasculitides (AAV) comprise granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis. All forms may involve different organ systems, yet kidney and lung involvement are common and fatal in many cases. Here, we aimed to determine the predictive value of pulmonary disease manifestation and individual CT findings in AAV patients. Available CT scans and clinical information on mortality, renal outcomes, occurrence of relapses and damage scores were analysed retrospectively from a tertiary rheumatology center in Germany. We included a total of 94 AAV patients (49 with GPA, 41 with MPA). Forty-four patients had lung involvement with available CT scans, 70.5% of which with GPA and 72.7% with renal involvement. Nodule formation and cavities were more frequent among GPA patients, whereas ground-glass opacities (GGO), ILD and pleural effusion were observed predominantly in MPA patients. Over a median follow-up of 37 months, GPA patients had a slightly higher overall mortality, whereas end-stage kidney failure rates were significantly increased in MPA patients. Relapse frequencies were comparable between both entities. The presence of GGO and pleural effusion were associated with higher relapse rates, whereas nodules were negatively correlated with relapses. Notably, RTX-treated patients had less infections as compared to individuals under different therapies. Our data demonstrate the outstanding importance of characteristic CT patterns in AAV diagnosis assessment. Especially certain CT patterns including GGO and pleura effusion may help to identify patients who are at higher risk for relapsing disease.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":" ","pages":"2435-2443"},"PeriodicalIF":3.2,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141971755","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
María Jesús Garcia-Villanueva, Sandra Garrote-Corral, Jose María Pego-Reigosa, Norman Jiménez Otero, Esther Uriarte Isazelaia, Alejandro Olivé Marqué, Clara Sangüesa Gómez, Mercedes Freire González, Elena Aurrecoechea Aguinaga, Enrique Raya Álvarez, Eva Tomero Muriel, Carlos Montilla Morales, María Galindo Izquierdo, Jaime Calvo-Alén, Raúl Menor-Almagro, Belén Serrano Benavente, Julia Martinez-Barrio, Jose Angel Hernández-Beriain, Mónica Ibañez Barceló, Gema Bonilla Hernan, Jose Rosas, Eva Salgado Pérez, Antonio Fernández-Nebro, Iñigo Rua-Figueroa
{"title":"Diffuse alveolar hemorrhage in patients with systemic lupus erythematosus: data from the Spanish society of rheumathology Lupus Register (RELESSER).","authors":"María Jesús Garcia-Villanueva, Sandra Garrote-Corral, Jose María Pego-Reigosa, Norman Jiménez Otero, Esther Uriarte Isazelaia, Alejandro Olivé Marqué, Clara Sangüesa Gómez, Mercedes Freire González, Elena Aurrecoechea Aguinaga, Enrique Raya Álvarez, Eva Tomero Muriel, Carlos Montilla Morales, María Galindo Izquierdo, Jaime Calvo-Alén, Raúl Menor-Almagro, Belén Serrano Benavente, Julia Martinez-Barrio, Jose Angel Hernández-Beriain, Mónica Ibañez Barceló, Gema Bonilla Hernan, Jose Rosas, Eva Salgado Pérez, Antonio Fernández-Nebro, Iñigo Rua-Figueroa","doi":"10.1007/s00296-024-05684-4","DOIUrl":"10.1007/s00296-024-05684-4","url":null,"abstract":"<p><strong>Introduction: </strong>Diffuse alveolar hemorrhage (DAH) is a rare complication with high mortality in patients with systemic lupus erythematosus (SLE). Early diagnosis and treatment are essential to improve patient prognosis. To determine the characteristics of patients with DAH and their mortality in a Spanish cohort of patients with SLE.</p><p><strong>Methods: </strong>Patients from the RELESSER (Spanish Society of Rheumatology Lupus Register) who had had at least one confirmed episode of DAH were included. Epidemiological, clinical, and laboratory characteristics were analyzed.</p><p><strong>Results: </strong>4024 patients were included in the RELESSER register, 37 (0.9%), had at least one recorded episode of DAH. Only further data for 14 patients could be analyzed. In total, 92.9% were women, and for 4 (28.6%) DAH coincided with the debut of SLE. More than 80% of patients had renal involvement and thrombocytopenia. The most frequent manifestations were dyspnea (85.7%) and hypoxemia (100%), with the classic triad of hemoptysis, anemia and pulmonary infiltrates, appearing in 6 (46.2%) patients. The most frequently used treatments were glucocorticoids (85.7%) and cyclophosphamide (69.2%); plasmapheresis was utilized in 5 patients (35.7%) and 8, (57.1%) received intravenous immunoglobulins; 12 (85.7%) patients required admission to the ICU and 5 (35.7%) died. Tobacco use, history of lupus nephritis (LN), concomitant infection, and treatment with cyclophosphamide were more frequent in patients who died.</p><p><strong>Conclusions: </strong>DAH is rare in patients with SLE; in up to one-third of patients, it may appear at the onset of the disease. Some factors, such as smoking, a history of LN, treatment with cyclophosphamide, or concomitant infection, are more prevalent in patients with an unfavorable outcome.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":" ","pages":"2445-2455"},"PeriodicalIF":3.2,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142047121","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}