UIC - Artigos publicados em revistas indexadas na Medline
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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.
- 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.
- Effectiveness of Tocilizumab in the Treatment of Fasciitis with Eosinophilia: Two Case ReportsPublication . Pinheiro, Maria Guiomar; Costa, Ana Rita; Campar, Ana; Mendonça, TeresaFasciitis with eosinophilia (FE) is a rare connective tissue disease. Due to its rarity, large-scale studies are lacking, which makes its treatment challenging. Systemic corticosteroids (SCSs) are the cornerstone of treatment; however, additional immunosuppressive drugs (ISDs) are frequently necessary (usually methotrexate). We report 2 patients, for whom an SCS and methotrexate were not a viable long-term option. In the first case, we were unable to taper the SCS dose without symptom relapse, the patient showed only a partial response to methotrexate and presented side effects. The second case never fully responded to the SCS and methotrexate and demonstrated serious SCS adverse effects. Both patients were started on tocilizumab with extremely favourable results, making this drug a potential therapeutic weapon for these patients. Learning points: The treatment of FE is challenging and mainly based on retrospective reviews, open-label trials and case reports, all of which included a small number of patients.Currently, systemic corticosteroids are the mainstay of treatment; however, other ISDs are frequently necessary.Cases showing a favourable clinical response to tocilizumab have recently been described in patients with corticosteroid-refractory disease, suggesting that this drug may potentially become a therapeutic weapon for these patients.
- From Clinical Presentation to the Outcome: the Natural History of PML in a Portuguese Population of HIV Infected PatientsPublication . Nery, F.; França, M.; Almeida, I.; Vasconcelos, c.Background Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease of the central nervous system, associated with immunosuppression states. As there are only some non-published documents concerning PML in HIV infected patients in Portugal, we pretend to characterize natural history of PML infection in a population of HIV patients. Methods We retrospectively reviewed, from 1992 to 2009, PML cases in a population of 724 HIV infected patients followed in our institution. Clinical, biological, imagery features and outcomes were characterized. Results Twenty-five (3.45%) patients were identified as having PML. The mean time between HIV and PML diagnosis was 20.4 months. PML was the presentation of HIV infection in 40% of the patients, and 92% had CD4 T cell count lower than 200/mm3. Paresis was the most common clinical presentation. No specific characteristics were found in cerebrospinal fluid and JCV DNA was positive in 3 of 7 patients. MRI revealed characteristic findings. Combined antiretroviral therapy was started or changed in 96% of the patients. Neurological condition got worse in 12 patients. From the 14 deaths, 5 were directly attributed to PML progression. Follow-up was lost in 8 patients. Conclusions PML was the presentation of HIV infection in more than 1/3 of patients, frequently associated with advanced immunocompromise. MRI sensitivity to PML is high, and JCV DNA determination in CSF was not revealed to be sensible. PML diagnosis should be taken into account in HIV patients presenting any neurological symptoms, and HIV infection should be suspected when radiological findings suggest PML lesions even in previously healthy individuals.
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