Emanuel Costa, Ana Margarida Correia, Carla Ferreira-Campinho, Joana Sousa-Neves
{"title":"Everyday complaints - a rare form of arthropathy.","authors":"Emanuel Costa, Ana Margarida Correia, Carla Ferreira-Campinho, Joana Sousa-Neves","doi":"","DOIUrl":"","url":null,"abstract":"","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139088835","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}
Elif Durak Ediboğlu, Dilek Solmaz, Gökhan Kabadayı, Sercan Gücenmez, Haluk Cinakli, Eda Otman Akat, Mustafa Özmen, Servet Akar
{"title":"Non-steroidal anti-inflammatory drug use is determined by disease activity in axSpA and decreased by biologicals: a longitudinal analysis.","authors":"Elif Durak Ediboğlu, Dilek Solmaz, Gökhan Kabadayı, Sercan Gücenmez, Haluk Cinakli, Eda Otman Akat, Mustafa Özmen, Servet Akar","doi":"","DOIUrl":"","url":null,"abstract":"<p><strong>Objective: </strong>To evaluate non-steroidal anti-inflammatory drug (NSAID) use and Assessment in Spondyloarthritis International Society (ASAS)-NSAID scores in patients with axial spondyloarhritis (axSpA) in a longitudinal study.</p><p><strong>Methods: </strong>In total, 429 patients with axSpA (59% male; 63.6% with AS) were included in this study. Data about disease activity, C-reactive protein (CRP) levels, and NSAID use and dosage were collected at 0, 12, 24, and 52 weeks retrospectively. The relationship with NSAID use /ASAS-NSAID scores and other factors were tested using generalized estimating equations (GEE).</p><p><strong>Results: </strong>At baseline (0 weeks), 92.8% of patients in biologic disease-modifying anti-rheumatic drugs (bDMARDs) group and 82.1% of patients in conventional treatment group were treated with NSAIDs. At baseline, the proportion (p=0.03) and the median (IQR) ASAS-NSAID scores were higher in bDMARDs group [100 (50) vs 50 (83.4); p<0.001]. During follow-up, NSAID use and ASAS-NSAID scores decreased significantly in patients treated with bDMARDs (p<0.001) and the reduction remained stable throughout the follow-up However, neither NSAID use (p=0.06) nor ASAS-NSAID scores changed in conventional treatment group (p=0.15). In bDMARD-treated patients, ASDAS-CRP and BASFI scores were independent determinants for NSAID use, and BASDAI and PGA were determinants for NSAID dosage. There was no independent significant predictor for ASAS-NSAID scores; PGA was the only significant predictor for NSAID use in the conventional treatment group.</p><p><strong>Conclusion: </strong>Concurrent biologic treatment was associated with low NSAID intake in patients with axSpA, and NSAID use was determined mainly by disease activity and partly by function during bDMARD treatment.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139088840","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}
Ana Martins, Sofia Pimenta, Frederico Rajão Martins, Beatriz Samões, Rafaela Nicolau, Eva Mariz, Lúcia Costa
{"title":"Pulmonary sarcoidosis and immune-mediated necrotizing myopathy: an uncommon coincidence.","authors":"Ana Martins, Sofia Pimenta, Frederico Rajão Martins, Beatriz Samões, Rafaela Nicolau, Eva Mariz, Lúcia Costa","doi":"","DOIUrl":"","url":null,"abstract":"<p><strong>Introduction: </strong>Immune-mediated necrotizing myopathy (IMNM) is characterized by acute or subacute, severe proximal muscle weakness and myofiber necrosis with minimal inflammatory cell infiltrate observed on muscle biopsy. On the other hand, sarcoidosis is characterised by the presence of non-caseating granulomas that can develop in several organs.</p><p><strong>Case report: </strong>We present the unique case of a 49-year-old woman, with no previous medical history, who had a rare concomitant occurrence of IMNM and pulmonary sarcoidosis. This condition was successfully treated with a combination of corticosteroids and rituximab along with rehabilitation program.</p><p><strong>Discussion: </strong>This association has been reported in only two previous case reports. This highlights the importance of further research on the connection between sarcoidosis and other forms of inflammatory myopathies.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139088841","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}
Carlos Eduardo Garcez Teixeira, Daniel Alvarenga Fernandes, Fabiano Reis, Zoraida Sachetto
{"title":"Mediastinal mass in a patient with granulomatosis with polyangiitis.","authors":"Carlos Eduardo Garcez Teixeira, Daniel Alvarenga Fernandes, Fabiano Reis, Zoraida Sachetto","doi":"","DOIUrl":"","url":null,"abstract":"","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139088838","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}
Maria Emilia Romero Noboa, Rafaella Litvin, Faria Sami, Saman Tanveer
{"title":"Nationwide analysis of adult hospitalizations with hematologic malignancies and systemic sclerosis.","authors":"Maria Emilia Romero Noboa, Rafaella Litvin, Faria Sami, Saman Tanveer","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Introduction Systemic sclerosis (SSc) is a connective tissue disease with multi-system involvement and it has an increased risk of developing hematologic malignancies. This study aims to report the association between hematologic malignancies with SSc and to characterize in-hospital demographics and outcomes in patients with hematologic malignancies with and without SSc. Methods We performed a retrospective review of pooled data from the National Inpatient Sample (NIS) database from 2016 to 2020. Crude prevalence of hematologic malignancies among hospitalized patients with and without SSc was calculated. Logistic regression was used for statistical significance of differences in prevalence while adjusting for confounders. Demographic characteristics and outcomes of patients with hematologic malignancies with and without SSc was compared. Statistical analysis was done using chi-square and multivariate logistic regression. Results Among all adult hospitalizations, the prevalence of hematologic malignancy was 1.87% compared to 2.66% among patients with SSc (adjusted odds ratio (aOR) 1.52, p <0.01). Relative to the non-SSc group, the SSc group had higher odds of in-patient mortality (OR 1.43; 95% confidence interval (CI) 1.11 - 1.87; p<0.01). The prevalence of lymphoma was 0.71% compared to 1.04% among patients with SSc (aOR 1.6, p < 0.01). Relative to the non-SSc group, the lymphoma-SSc group had similar odds of in-patient mortality (OR 0.93; 95% CI 0.55 - 1.59; p=0.80). The prevalence of leukemia was 0.79% compared to 1.28% among patients with SSc (aOR 1.74, p < 0.01). The leukemia-SSc group had higher odds of in-patient mortality (OR 1.78; 95% CI 1.29 - 2.46; p<0.01). For myeloma, there was no difference in the prevalence in adults with and without SSc (0.4 vs. 0.38%, aOR 0.96, p=0.64) and there was no difference of in-hospital mortality. Conclusions There is a positive significant association between hematologic malignancies including lymphoma and leukemia, and SSc. This association was not seen between myeloma and SSc. There is increased in-hospital mortality of patients with leukemia and SSc.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139088839","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}
Gizem Ayan, Sofia Ramiro, Fernando M Santos, Anneke Spoorenberg, Suzanne Arends, Levent Kilic
{"title":"Translation and cross-cultural adaptation of the modified Short QUestionnaire to Assess Health-enhancing physical activity (mSQUASH) into Turkish.","authors":"Gizem Ayan, Sofia Ramiro, Fernando M Santos, Anneke Spoorenberg, Suzanne Arends, Levent Kilic","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Aims The aim was to translate and cross-culturally adapt the modified Short Questionnaire to Assess Health-enhancing physical activity (mSQUASH) into Turkish Methods The mSQUASH was translated into Turkish and backward-translation into Dutch was performed afterwards using the Beaton method. After the Turkish version was reviewed and revised by an expert committee that included translators, two patients and the research team a pre-final version was produced. The-pre final version then entered a field-test with cognitive debriefing in 10 patients with axSpA. The final result was the Turkish mSQUASH version. Results The translation process went without difficulties. Small discrepancies were either resolved during the synthesis or expert consensus meetings. Mean (SD) time to complete the mSQUASH was 6.1 (2.4) minutes in field-test procedure. The cognitive debriefing showed that the items of the Turkish mSQUASH were clear, relevant, easy to understand and easy to complete. None of the patients reported that an important aspect of physical activity was missing from the questionnaire items. Patients raised the concern that not all sport examples were culturally suitable; tennis was replaced by volleyball and basketball after the cognitive debriefing, to make it more appropriate to the Turkish culture. Conclusion The final Turkish version of the mSQUASH showed acceptable linguistic and field validity for use in both clinical practice and research. However, further assessment of the psychometric properties (validity and reliability) of the Turkish version of the mSQUASH is needed before it can be implemented.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139088844","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}
Ana Marta Pinto, Iris Ascenção, Mariana Rodrigues, Iva Brito
{"title":"Biotechnological therapeutic in Juvenile Idiopathic Arthritis: Pathophysiological implications and targeted therapies.","authors":"Ana Marta Pinto, Iris Ascenção, Mariana Rodrigues, Iva Brito","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Objective In this retrospective cohort study, we aim to investigate the most used biological disease modifying anti-rheumatic drugs (bDMARDs) in Juvenile Idiopathic Arthritis (JIA) patients in a pediatric rheumatologic unit from a Portuguese tertiary hospital, along with their effectiveness and safety. We also intended to link their effectiveness and the pathophysiology of the disease. Methods The medical records of JIA patients exposed to bDMARDs, between January 2018 and January 2023, in a pediatric rheumatologic unit from a Portuguese tertiary hospital were reviewed. Therapy effectiveness was accessed based on achievement of inactive disease according to Wallace Criteria. Effectiveness of different bDMARDs in the several JIA subtypes was linked to the disease´s pathophysiology. Adverse effects were also reviewed. Results Thirty-four patients were included in the study. Overall, nineteen patients (67,9%) had inactive disease at last evaluation. Six patients with missing data on inactive disease status were excluded from this analysis. Number of affected joints, ESR and CRP were significantly lower at 3, 6, 12 and 24 months after bDMARD therapy. All systemic JIA patients (n=10) were initially treated with Anakinra. Six (60%) achieved inactive disease. Two (20%) switched to Tocilizumab due to ineffectiveness in the control of articular features. Patients who switched to tocilizumab achieved inactive disease until the end of the follow-up. All patients with the other subtypes of JIA (n=24) were treated with TNF inhibitors. Inactive disease was achieved in 55,6%. Adverse effects occurred in eight patients (23,5%). Conclusions The results of the present study demonstrate the effectiveness of bDMARs in the study population. bDMARDs reduced the number of affected joints, CRP and ESR after three months of treatment, and this effectiveness was sustained over the two years of follow-up. For systemic JIA, preferred drug was Anakinra, an interleukin 1 inhibitor, and its effectiveness was consistent with previous studies. In the other JIA subtypes, TNF inhibitors were the most used bDMARDs, and showed an effectiveness consistent with previous studies. The most used bDMARDs for each JIA subtype are in line with pathophysiological differences. Our results demonstrated the safety of these drugs.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139088832","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}
Mariana Emília Santos, Maria João Gonçalves, Manuela Costa, Ana Ramalhal Jorge, Joana F Vasconcelos, Sância Ramos, Jaime C Branco, Alexandre Sepriano
{"title":"Life-threatening hypereosinophilic syndrome in a patient with rheumatoid arthritis: a case report.","authors":"Mariana Emília Santos, Maria João Gonçalves, Manuela Costa, Ana Ramalhal Jorge, Joana F Vasconcelos, Sância Ramos, Jaime C Branco, Alexandre Sepriano","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Hypereosinophilia is unusual in rheumatoid arthritis (RA), but can occur in severe long-lasting disease, especially in patients with extra-articular manifestations and high titers of rheumatoid factor (RF). The association of RA and hypereosinophilic syndrome (HES) remains yet poorly known. We present a case of a 46 years old woman with long-standing untreated RA, that presented to emergency department with severe symptoms of constrictive pericarditis with cardiac tamponade and bilateral pleural effusion, that progressed to cardiac arrest, associated to symmetrical polyarthritis and pruritic erythematous skin papules. She was submitted to urgent pericardial drainage and partial pericardiotomy. Laboratory analyses revealed hypereosinophilia, and elevated inflammatory parameters and immunoglobulin E. The histological study of the pericardium showed results consistent with inflammatory fibrinous pericarditis. Taking into account the presence of some characteristics that are usually present in cases of reactive HES instead of idiopathic HES, and after an intensive diagnostic study, that could rule out other potential causes of secondary HES, the diagnosis of HES associated with RA was made. She started glucocorticoids during hospitalization and methotrexate 15mg per week at the first outpatient rheumatology visit. After 12 weeks of treatment, we considered that she was in clinical and analytical remission, consistently maintaining that after a complete tapering of glucocorticoids. This case illustrates that clinicians should be aware that HES (including severe life-threatening cases) can occur in patients with RA, especially in cases of long-lasting disease with high titters of RF and without treatment, even in the absence of extra-articular features.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139088837","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}
Bruna Kellen Wanderley Porto Uchôa, Amanda Calfa Nogueira, Larissa da Silva Pinto, Thiago Sotero Fragoso
{"title":"Risk factors for work disability in Brazilian patients with systemic lupus erythematosus.","authors":"Bruna Kellen Wanderley Porto Uchôa, Amanda Calfa Nogueira, Larissa da Silva Pinto, Thiago Sotero Fragoso","doi":"","DOIUrl":"","url":null,"abstract":"<p><strong>Background: </strong>Systemic Lupus Erythematosus (SLE) predominantly affects young females who are in their most productive years of life. SLE can cause organ damage and affects daily functioning and quality of life, causing work disability (WD).</p><p><strong>Methods: </strong>We developed a longitudinal study with 110 SLE patients, whose data were collected through individual standardized interview and review of medical records. We aimed to determine the prevalence of WD and its possible associated risk factors (sociodemographic, lifestyle habits, quality of life, clinical characteristic, cumulative organ damage and disease activity). To identify variables associated with work disability, two different multivariate regression models using a stepwise backward method were performed.</p><p><strong>Results: </strong>The percentage of WD due to SLE was 76.3%. An association was found between WD and lack of physical exercise (p=0.017) and high physical work demand (p=0.037). Clinical characteristics were not significant predictors of work dysfunction.</p><p><strong>Conclusion: </strong>76.3% of our sample developed WD after SLE diagnosis. Participants who did not practice physical exercise and those who had a high-demand physical work were, respectively, 3.78 and 4.80 times more likely to have WD. Although we were not able to analyze the influence of COVID-19 in WD development, COVID-19 pandemic could have exacerbated the inequalities among people with chronic health conditions, especially in a low-income population, which could have influenced our results. Additional researches to evaluate risk factors for WD in low-income SLE patients and on strategies for reducing its impact are needed.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139088842","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}
Hugo Parente, Maria Pontes Ferreira, Catarina Dantas Soares, Emanuel Costa, Francisca Guimarães, Soraia Azevedo, Daniela Santos-Faria, José Tavares-Costa, Filipa Teixeira, Carmo Afonso, Daniela Peixoto
{"title":"Barriers and alternatives to pediatric rheumatology referrals: a survey of family doctors and pediatricians in Portugal.","authors":"Hugo Parente, Maria Pontes Ferreira, Catarina Dantas Soares, Emanuel Costa, Francisca Guimarães, Soraia Azevedo, Daniela Santos-Faria, José Tavares-Costa, Filipa Teixeira, Carmo Afonso, Daniela Peixoto","doi":"","DOIUrl":"","url":null,"abstract":"<p><strong>Background: </strong>Access to pediatric rheumatology (PR) is not well described in Portugal. The main goal of this study was to ascertain barriers to PR referrals and subsequent alternative referral patterns among family doctors and pediatricians.</p><p><strong>Methods: </strong>A web-based survey was e-mailed to family doctors and pediatricians practicing in Portugal, in order to investigate access to PR care issues. Descriptive and comparative analysis was performed.</p><p><strong>Results: </strong>Two hundred and ninety-two responses were obtained, 24.7% from pediatricians and 75.3% from family doctors. Only 12% claimed to have had specific education on PR. Nearly 70% worked less than one hour away from a PR center. Twenty eight percent had referred a patient to PR at least once, and 9.3% experienced a situation in which they considered referring to PR but ultimately did not. Many referred to other specialties, primarily pediatrics, adult rheumatology, and pediatric orthopedics. Pediatricians encountered more diversified rheumatic diseases. Fifty five percent had no opinion on PR centers' support, while 24% found it sufficient. Having specific training on PR, being a pediatrician and a specialist were associated with greater referrals to PR. The most rated measure for PR referrals' improvement was promoting education. Regional access to PR's discrepancies were documented.</p><p><strong>Conclusion: </strong>Mainly lack of education on PR, but also uneven national coverage and greater distances to some PR centers were the main barriers to PR referrals, in Portugal. Pediatricians seem to have better education, greater experience and more referrals to PR. The current alternatives for referral are pediatrics, adult rheumatology and pediatric orthopedics. Educational consolidation was the biggest and most rewarding inconsistency to battle against.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41111132","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}