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- 2023 ACR/EULAR antiphospholipid syndrome classification criteriaPublication . Barbhaiya, Medha; Zuily, Stephane; Naden, Ray; Hendry, Alison; Manneville, Florian; Amigo, Mary-Carmen; Amoura, Zahir; Andrade, Danieli; Andreoli, Laura; Artim-Esen, Bahar; Atsumi, Tatsuya; Avcin, Tadej; Belmont, Michael H; Bertolaccini, Maria Laura; Branch, D Ware; Carvalheiras, Graziela; Casini, Alessandro; Cervera, Ricard; Cohen, Hannah; Costedoat-Chalumeau, Nathalie; Crowther, Mark; de Jesús, Guilherme; Delluc, Aurelien; Desai, Sheetal; Sancho, Maria De; Devreese, Katrien M; Diz-Kucukkaya, Reyhan; Duarte-García, Ali; Frances, Camille; Garcia, David; Gris, Jean-Christophe; Jordan, Natasha; Leaf, Rebecca K; Kello, Nina; Knight, Jason S; Laskin, Carl; Lee, Alfred I; Legault, Kimberly; Levine, Steve R; Levy, Roger A; Limper, Maarten; Lockshin, Michael D; Mayer-Pickel, Karoline; Musial, Jack; Meroni, Pier Luigi; Orsolini, Giovanni; Ortel, Thomas L; Pengo, Vittorio; Petri, Michelle; Pons-Estel, Guillermo; Gomez-Puerta, Jose A; Raimboug, Quentin; Roubey, Robert; Sanna, Giovanni; Seshan, Surya V; Sciascia, Savino; Tektonidou, Maria G; Tincani, Angela; Wahl, Denis; Willis, Rohan; Yelnik, Cécile; Zuily, Catherine; Guillemin, Francis; Costenbader, Karen; Erkan, DorukObjective: To develop new antiphospholipid syndrome (APS) classification criteria with high specificity for use in observational studies and trials, jointly supported by the American College of Rheumatology (ACR) and EULAR. Methods: This international multidisciplinary initiative included four phases: (1) Phase I, criteria generation by surveys and literature review; (2) Phase II, criteria reduction by modified Delphi and nominal group technique exercises; (3) Phase III, criteria definition, further reduction with the guidance of real-world patient scenarios, and weighting via consensus-based multicriteria decision analysis, and threshold identification; and (4) Phase IV, validation using independent adjudicators' consensus as the gold standard. Results: The 2023 ACR/EULAR APS classification criteria include an entry criterion of at least one positive antiphospholipid antibody (aPL) test within 3 years of identification of an aPL-associated clinical criterion, followed by additive weighted criteria (score range 1-7 points each) clustered into six clinical domains (macrovascular venous thromboembolism, macrovascular arterial thrombosis, microvascular, obstetric, cardiac valve, and hematologic) and two laboratory domains (lupus anticoagulant functional coagulation assays, and solid-phase enzyme-linked immunosorbent assays for IgG/IgM anticardiolipin and/or IgG/IgM anti-β2-glycoprotein I antibodies). Patients accumulating at least three points each from the clinical and laboratory domains are classified as having APS. In the validation cohort, the new APS criteria vs the 2006 revised Sapporo classification criteria had a specificity of 99% vs 86%, and a sensitivity of 84% vs 99%. Conclusion: These new ACR/EULAR APS classification criteria were developed using rigorous methodology with multidisciplinary international input. Hierarchically clustered, weighted, and risk-stratified criteria reflect the current thinking about APS, providing high specificity and a strong foundation for future APS research.
- Anti-Ro52 Antibodies and Interstitial Lung Disease in ConnectiveTissue Diseases Excluding SclerodermaPublication . Ferreira, J.; Almeida, I.; Marinho, A.; Cerveira, C.; Vasconcelos, C.The presence of anti-Ro52 antibodies has been reported in a wide variety of autoimmune diseases, particularly in myositis, scleroderma, and autoimmune liver diseases. Clinical significance of anti-Ro52 antibodies remains controversial, and studies are lacking for clarifying the association of anti-Ro52 with interstitial lung disease (ILD) in connective tissue diseases (CTD). Objectives. To determine if anti-Ro52 antibodies are associated with ILD in CTD other than scleroderma. Methods. Singlecenter, retrospective study based on immunoblotting panel analysis and patients clinical records. Results. In our connective tissue disease cohort, 162 patients had immunoblotting panels with anti-Ro52 reactivity analysis, 41 (25,3%) had inclusion criteria. Among the 41 selected sera, 85.4% (n = 35) had anti-Ro52 reactivity. The prevalence of ILD in the positive anti-Ro52 antibodies was 71.4% (n = 25), and 16.7% (n = 1) in the negative anti-Ro52 group (P = 0.018). Overall sensitivity (96.2%), specificity (83.3%), positive (71.4%) and negative (83.3%) predictive values of anti-Ro52 antibodies to determine ILD in CTD is detailed in this study. Conclusion. Ro52 autoantibodies are associated with ILD in CTD excluding scleroderma. We suggest that the presence of anti-Ro52 reactivity in CTD should increase the clinician curiosity for the search of ILD.
- Are Anti-Ro52 Antibodies Associated with Pulmonary Involvement in Scleroderma?Publication . Ferreira, J.; Almeida, I.; Marinho, A.; Cerveira, C.; Vasconcelos, C.Abstract Introduction: The presence of anti-Ro52 antibodies has been reported in a wide variety of autoimmune diseases, particularly in myositis, scleroderma and autoimmune liver diseases. Clinical significance of anti-Ro52 antibodies remains controversial. Studies are lacking in clarifying the association of anti-Ro52 with pulmonary involvement in scleroderma. Objectives: To determine if anti-Ro52 antibodies are associated with pulmonary involvement (interstitial, indirect pulmonary hypertension, or both) in scleroderma. Methods: Single center, retrospective study based on immunoblotting panel analysis and patients clinical records. Pulmonary manifestations were sub-grouped in: 1) interstitial (alveolitis and/or fibrosis), 2) pulmonary artery systolic pressure (PASP) ≥40 mmHg plus interstitial pulmonary disease, and 3) isolated PASP≥40 mmHg (purely vascular). Results: Our scleroderma cohort included 200 patients, of which 137 had immunoblotting panels with anti-Ro52 reactivity analysis. The search was conducted between January 2010 and July 2011. The frequency of pulmonary manifestations in patients with positive anti-Ro52 antibodies was 67.7% (n=31), and 60% (n=24) in the negative anti-Ro52 group, showing no significant differences between groups (p=0.621). Still no significant differences were found when pulmonary manifestations were evaluated according to the subgroups (p=0.525). Sensitivity, specificity, positive and negative predictive values of anti-Ro52 reactivity for determining pulmonary involvement in scleroderma were low. Conclusion: No association was found between positive anti-Ro52 antibodies and pulmonary involvement in scleroderma.
- Autologous stem cell transplantation in a patient with severe systemic sclerosisPublication . Vaz, C.; Almeida, I.; Guedes, M.; Rosário, C.; Branca, R.; Campilho, F.; Roncon, S.; Vasconcelos, C.; Campos, A.Systemic Sclerosis (SSc) is a chronic disease of the connective tissue, whose pathogenesis involves abnormalities of the immunological system. It has a variable course and there is a subgroup of patients with rapidly progressive disease or unresponsive to conventional treatment. Thesepatients can benefit from intensive immunosuppression and autologous hematopoietic stem cell transplant. Clinical case: 19-year-old (y.o.) woman diagnosed with SSc at the age of 13 y.o. with cutaneous, vascular and articular involvement with initial response to me thotrexate. Three years later the disease progressed with severe digestive involvement (dysphagia, delayed gastric emptying and weight loss) needing gastrostomy for nutritional support. She was treated with cyclophosphamide without improvement. In May 2012 she had an autologous transplant with myeloablative regimen (BEAM): carmustine 300 mg/m2 x1day; etoposide 120 mg/kg x4days; cytarabine 120 mg/kg 12/12:h x4days; melphalan 140 mg/m2 x1day. A year and a half after transplantation she is asymptomatic, without any signs or symptoms of the disease, feeds by mouth and the gastric emptying study is normal. Currently she is free of medication.
- AUTOMATED COLLECTION OF QUALITY-OF-LIFE DATA: TOUCH-SCREEN COMPUTER SYSTEMS IN PATIENTS WITH IMMUNE DISEASESPublication . Ribeiro, C.; Silveira, A.; Silva, I.; Ribeiro, C.; Vasconcelos, C.AUTOMATED COLLECTION OF QUALITY-OF-LIFE DATA: TOUCH-SCREEN COMPUTER SYSTEMS IN PATIENTS WITH IMMUNE DISEASES Claúdia Ribeiro1,2,3, Augusta Silveira4,3, Isabel Silva4, Catarina Ribeiro4, Carlos Vasconcelos2,3 1UCP, 2HSA/CHP, 3ICBAS/UP, 4UFP e 5HSMF. Universidade Católica Portuguesa (UCP), Porto. Hospital de Santo António, Centro Hospitalar do Porto (HSA/CHP), Porto. Instituto Ciências Biomédicas Abel Salazar, Universidade do Porto (ICBAS/UP), Porto. Universidade Fernando Pessoa (UFP), Porto. Hospital Santa Maria da Feira (HSMF), Porto. Background The increase of technological knowledge and methodology has allowed the practice of redirecting epidemiological research, particularly in the field of chronic disease. Although capable of controlling the accumulation of events and constraints imposed by the treatment can lead to a drastic change in quality of life (QoL) of subjects progressed to varying degrees of disability and death. In this perspective the Quality of Life Related to Health (HRQOL) has taken a leading role and its evaluation is indispensable in Medicine. Aim The aims of this study were (1) analyze the importance of HRQOL assessment as a tool for health promotion and a way of measuring the effectiveness of interventions in daily practice in patients with coexisting chronic immune system (PCSI), (2) evaluation of alternative methods for the automated collection of data on HRQOL and development of an electronic interface in sample of 320 patients, (3) creating a database to ascertain the epidemiological profile of PCSI, and identification of socio-economic, demographic and clinical data of these individuals, (4) using the QoL indicator as a predictor in decision treatment and use the preferences of patients. Methods A total of 473 patients with chronic diseases of the immune system, which were applied Graffar Index, SF-36v2, a demographic questionnaire and identification of clinical variables. Results The results of this investigation suggest that the demographic, socio-economic and clinics are associated with significant differences in QoL cumulative and chronic complications associated with different pathologies. The results verified the existence of significant correlations between the different diagnoses, duration of disease and therapy. In general, patients who have chronic diseases of the immune system such as rheumatoid arthritis, lupus, scleroderma, Bechet's disease, Sjögren's syndrome or infection with human immunodeficiency virus 1 or 2 showed a worse QoL than the general population. The derivations of preferences from the SF-36v2 exhibit strong correlations with the preferences measured with the SF-6D. Conclusions This suggests that both the application of the SF-36v2 as the SF-6D can be important sources of preferences to implement measures in economic evaluation in healthcare. HRQL can and should be integrated into immune clinical practice. The translation of graphical results given to the clinician at the beginning of the consultation, favors the rapid analysis of global values of the patient's HRQL. This assessment can be an excellent diagnostic tool to be used routinely in clinical practice or assisting in disease management and therapeutic decision making. Apresentador: Cláudia Ribeiro, Médica Dentista. Doutoranda, Faculdade de Medicina da Universidade de Santiago de Compostela.
- AVALIAÇÂO DE UMA NOVA TÉCNICA DE QUIMIOLUMINESCÊNCIA PARA DETERMINAÇÃO DE ANTICORPOS ANTI-DSDNAPublication . Carneiro, P.; Figueiras, O.; Lima, S.; Neves, E.; Cerveira, C.Introdução A determinação dos anticorpos anti-dsDNA é um teste de grande importância para o diagnóstico e monitorização de doentes com Lúpus Eritematoso Sistémico (LES), fazendo parte dos critérios de classificação de LES do ACR. (American College of Rheumathology). Existem actualmente vários métodos laboratoriais disponíveis, que respondem de forma desigual na determinação destes anticorpos nos doentes, em diferentes fases de evolução da patologia. Objectivo Avaliar o desempenho do novo método automatizado de determinação dos anticorpos anti-dsDNA por técnica de quimioluminescência (CLIA), Zenit RA dsDNA (Menarini), comparando-o com os métodos de imunofluorescência indirecta (IFI) e fluoroimunoensaio (FEIA), utilizados na rotina assistencial no Serviço de Imunologia do CHP. Material e Métodos A população estudada incluiu 151 amostras seriadas de doentes com LES, 33 doentes com doença infecciosa, 28 doentes com outras patologias com envolvimento autoimune e 38 indivíduos saudáveis. Realizou-se a determinação dos anticorpos anti-dsDNA por técnica CLIA no equipamento ZENIT RA (Menarini), por técnica FEIA no equipamento ImmunoCAP 250 (Phadia) e por IFI em lâminas de Crithidia luciliae (BioRad) processadas no aparelho PhD (BioRad). Resultados Todos os testes apresentaram uma baixa sensibilidade nos doentes com LES (33,1% a 44,4%), traduzindo o facto de um grande número de doentes se encontrar em tratamento e com fraca actividade da doença. O teste CLIA apresentou uma especificidade semelhante à da IFI (93,9% vs. 95,6%), superior à observada no FEIA (85,9%). Conclusões O teste dsDNA ZENIT RA revelou uma sensibilidade inferior ao FEIA mas uma melhor especificidade e valor preditivo positivo, semelhantes aos observados na técnica de IFI. Sendo um teste totalmente automatizado e sem a subjectividade da IFI, será agora importante a sua avaliação numa população com critérios de actividade bem definidos.
- Behçet's Disease and Pregnancy: A Retrospective Case-control StudyPublication . Barros, Tânia; Braga, António; Marinho, António; Braga, JorgeBackground: Behçet's disease (BD) is a rare chronic multisystemic vasculitis of unknown etiology. It is usually diagnosed between the 2nd and 4th decades of life, so its association with pregnancy is not unusual. This study aims to characterize the evolution of pregnancy in a group of pregnant women with BD and the impact of this pathology in embryo-fetal morbidity. Methods: A retrospective case-control study included 49 pregnancies in women suffering from BD, followed in our institution. Pregnancy outcomes were compared with a control group of healthy pregnant women. Two controls per case were randomly selected. Statistical analysis used SPSS 25.0, and a p-value of 0.05 was considered statistically significant. Results: Forty-nine pregnancies were included in 27 patients with BD. BD exacerbation occurred in 32.6% of the pregnancies. There were no significant statistical differences between the two groups regarding the rate of preterm delivery, gestational diabetes, and preeclampsia (p>0.05). In the BD group, we found a higher rate of miscarriage (24.5%) and fetal growth restriction (FGR, 13.3%, p<0.05). In the study group, 13 (32.5%) of the pregnant patients did not need treatment. The cesarean rate was significantly higher in the BD group (43.2% vs 20.4% in the control group, p<0.05), and there were no significant differences in median gestational age at the time of delivery (p>0.05). The birth weight of newborns did not differ significantly between the groups. There was no association of BD with maternal morbidity and neonatal complications. Conclusion: In this study, the majority of pregnant with BD did not present clinical exacerbation of their pathology. However, BD may have an adverse influence on pregnancy outcomes. FGR and miscarriage rates were significantly higher in the study group.
- Development and preliminary validation of the Behçet’s syndrome Overall Damage Index (BODI)Publication . Piga, Matteo; Floris, Alberto; Espinosa, Gerard; Serpa Pinto, Luísa; Kougkas, Nikolaos; Lo Monaco, Andrea; Lopalco, Giuseppe; Orlando, Ida; Pirani, Vittorio; Santos, Ernestina; Bertsias, George; Cantarini, Luca; Cauli, Alberto; Cervera, Ricard; Correia, João; Govoni, Marcello; Iannone, Florenzo; Neri, Piergiorgio; Martins da Silva, Ana; Vasconcelos, Carlos; Muntoni, Monica; Mathieu, AlessandroObjective: To develop and validate the evidence-based and consensus-based Behçet's Syndrome Overall Damage Index (BODI). Methods: Starting from 120 literature-retrieved preliminary items, the BODI underwent multiple Delphi rounds with an international multidisciplinary panel consisting of rheumatologists, internists, ophthalmologists, neurologists, and patient delegates until consensus was reached on the final content. The BODI was validated in a cross-sectional multicentre cohort of 228 patients with Behçet's syndrome (BS) through the study of (a) correlation between BODI and Vasculitis Damage Index (VDI) and (b) correlation between BODI and disease activity measures (ie, Behçet's Disease Current Activity Form (BDCAF), Physician Global Assessment (PGA), Patient Global Assessment (PtGA)), c) content and face validity and (d) feasibility. Results: The final BODI consists of 4 overarching principles and 46 unweighted-items grouped into 9 organ domains. It showed good to excellent reliability, with a mean Cohen's k of 0.84 (95% CI 0.78 to 0.90) and a mean intra-class correlation coefficient of 0.88 (95% CI 0.80 to 0.95). Overall, 128 (56.1%) patients had a BODI score ≥1, with a median score of 1.0 (range 0-14). The BODI significantly correlated with the VDI (r=0.693, p<0.001), demonstrating to effectively measure damage (construct validity), but had greater sensitivity in identifying major organ damage and did not correlate with disease activity measures (ie, BDCAF: p=0.807, PGA: p=0.820, PtGA: p=0.794) discriminating damage from the major confounding factor. The instrument was deemed credible (face validity), complete (content validity) and feasible by an independent group of clinicians. Conclusions: Pending further validation, the BODI may be used to assess organ damage in patients with BS in the context of observational and controlled trials.
- Development and use of touch-screen computer-assisted self interviewing in Portuguese patients with chronic immune diseases: Evaluation of an electronic version of SF-36v2Publication . Ribeiro, C.; Moreira, L.; Silveira, A.; Silva, I.; Gestal, J.; Vasconcelos, C.Abstract Aim:The major purpose of this study was to evaluate alternative automated methods of collecting data on health related quality of life (HR-QoL). In order to achieve this, we developed a study with the following objectives: (1) to evaluated the feasibility of electronic version in patients with different chronic pathologies of the immune system using Short Form 36version2 (SF-36v2), (2) to evaluate the construct validity of SF-36v2 using the electronic data capture, and (3) to compare electronic version questionnaires with paper questionnaires in terms of patients ´ acceptance, data quality, and reliability. Methods:Out-patients with chronic immune diseases (HIV infection, lupus, scleroderma, rheumatoid arthritis, Behçet and Sjögren), were randomly selected to completed electronic and paper SF- 36v2 (n=50) before consultation in Clinical Immunology Unit, in Hospital Santo António-Centro Hospitalar do Porto (CI-HGSA). Results: There were very high correlations in SF- 36v2 responses (p< .001) between the paper and electronic forms. Internal reliability coefficients (Cronbach’s a) showed good internal consistency for all reported responses in either, computer and paper. There were no missing data in electronic version or paper. About 84% of the patients prefer to use the computer version in future. Conclusion: The electronic HR-QoL assessment is technically possible and it can provide reliable and valid clinically significant information which can either be used in routine care appointments.
- Discordance between patient and physician global assessment of disease activity in Behçet's syndrome: a multicenter study cohortPublication . Floris, Alberto; Espinosa, Gerard; Serpa Pinto, Luísa; Kougkas, Nikolaos; Lo Monaco, Andrea; Lopalco, Giuseppe; Orlando, Ida; Bertsias, George; Cantarini, Luca; Cervera, Ricard; Araújo Correia, João; Govoni, Marcello; Iannone, Florenzo; Mathieu, Alessandro; Neri, Piergiorgio; Martins da Silva, Ana; Vasconcelos, Carlos; Muntoni, Monica; Cauli, Alberto; Piga, MatteoBackground: To compare the patients' and physician's global assessment of disease activity in Behçet's syndrome (BS) and investigate the frequency, magnitude, and determinants of potential discordance. Methods: A total of 226 adult BS patients with a median (IQR) age of 46.9 (35.6-55.2) years were enrolled across Italy, Greece, Portugal, and Spain. Demographic, clinical, and therapeutic variables, as well as the patient reported outcomes, were collected at the recruitment visit. The physical (PCS) and mental (MCS) component summary scores of the Short Form Questionnaire 36 (SF-36) and the Behçet's syndrome Overall Damage Index (BODI) were calculated. Disease activity was assessed by the patients' (PtGA) and physician's global assessment (PGA) in a 10-cm visual analog scale, as well as the Behçet Disease Current Activity Form (BDCAF). Discordance (∆) was calculated by subtracting the PGA from the PtGA and defined as positive (PtGA>PGA) and negative (PtGA 80%) of disagreements were due to patients rating higher their disease activity. Higher values of BDCAF were associated to increased rate of positive discordance. When BDCAF = 0, the median (IQR) values of PtGA and PGA were 0.2 (0-2) and 0 (0-1), respectively. PCS (adjusted odds ratio (adjOR) 0.96 per unit, 95% CI 0.93-0.98, p = 0.006) and MCS (adjOR 0.96 per unit, 95% CI 0.93-0.99, p = 0.003) were independently associated with positive discordance using both cutoffs. Active ocular involvement emerged as a potential determinant of negative discordance (adjOR 5.88, 95% CI 1.48-23.30, p = 0.012). Conclusions: PtGA and PGA should be considered as complementary measures in BS, as patients and physicians may be influenced by different factors when assessing active disease manifestations. Particularly, PtGA may be a useful tool in the assessment of BS disease activity, as it carries a low risk to misclassify an inactive disease, and may allow to capture aspects of the patient's health that negatively affect his well-being and the treatment.