ReumatismoPub Date : 2023-07-17DOI: 10.4081/reumatismo.2023.1542
N Ughi, D P Bernasconi, C Gagliardi, F Del Gaudio, A Dicuonzo, A Maloberti, C Giannattasio, C Rossetti, M G Valsecchi, O M Epis
{"title":"Trends in severe outcomes in SARS-CoV-2-positive hospitalized patients with rheumatic diseases: a monocentric observational and case-control study in northern Italy.","authors":"N Ughi, D P Bernasconi, C Gagliardi, F Del Gaudio, A Dicuonzo, A Maloberti, C Giannattasio, C Rossetti, M G Valsecchi, O M Epis","doi":"10.4081/reumatismo.2023.1542","DOIUrl":"https://doi.org/10.4081/reumatismo.2023.1542","url":null,"abstract":"<p><p>Rheumatic disease patients are at greater risk of infection due to their disease, comorbidities, and immunosuppressive therapy. COVID-19 outcomes in this patient setting appeared to be similar to those of the general population. However, data on this topic were mainly related to small studies on a limited number of patients. Consequently, to date, this field remains poorly explored, particularly in the pre-vaccine era. This monocentric study aimed to describe the intrahospital mortality in rheumatic patients with SARS-CoV-2 consecutively hospitalized from 21 February to 31 December 2020, before anti-SARS-CoV-2 vaccine administration spread, compared with non-rheumatic patients. Of 2491 included patients, 65 [3%, median (interquartile range) age 75 (64.76-82.239 years, 65% women] were suffering from rheumatic diseases. A total of 20 deaths were reported [case fatality rate 31%, 95% confidence interval (CI): 19-42] compared with 433 deaths (19%, 95% CI: 17-20) in patients without rheumatic diseases (p=0.024). However, the rheumatic disease was not associated with a significant increase in univariate mortality hazards (hazard ratio 1.374, 95% CI: 0.876-2.154), and after adjustment (hazard ratio 1.199, 95% CI: 0.759-1.894) by age, sex and Charlson comorbidity index. The incidence of intensive care unit admission, death, and discharge in the case-control study was comparable between rheumatic and non-rheumatic patients. The presence of rheumatic diseases in SARS-CoV-2-hospitalized patients did not represent an independent risk factor for severe disease or mortality.</p>","PeriodicalId":21222,"journal":{"name":"Reumatismo","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2023-07-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10189562","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
ReumatismoPub Date : 2023-07-17DOI: 10.4081/reumatismo.2023.1597
L Fioravante da Silveira, A Zago, D Zanchet, D Chemello
{"title":"Macrophage activation syndrome in a patient with systemic lupus erythematosus undergoing cyclophosphamide treatment: a case report.","authors":"L Fioravante da Silveira, A Zago, D Zanchet, D Chemello","doi":"10.4081/reumatismo.2023.1597","DOIUrl":"https://doi.org/10.4081/reumatismo.2023.1597","url":null,"abstract":"<p><p>Macrophage activation syndrome (MAS) is a disorder related to hemophagocytic lymphohistiocytosis and is a life-threatening complication of rheumatic diseases. The diagnosis is challenging because MAS symptoms are quite similar to those of many active autoimmune diseases or severe sepsis. We describe the case of a female patient with systemic lupus erythematosus that presented with symptoms suggesting acute decompensation of autoimmune disease and sepsis. She was later diagnosed with MAS. Despite an aggressive immunosuppressive treatment, she developed a fatal outcome.</p>","PeriodicalId":21222,"journal":{"name":"Reumatismo","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2023-07-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9836307","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
ReumatismoPub Date : 2023-07-17DOI: 10.4081/reumatismo.2023.1564
K Kaynar, B Güvercin, A Şengör, S Mungan
{"title":"A case of Sjögren syndrome and anti-neutrophil cytoplasmic antibody-associated vasculitis.","authors":"K Kaynar, B Güvercin, A Şengör, S Mungan","doi":"10.4081/reumatismo.2023.1564","DOIUrl":"https://doi.org/10.4081/reumatismo.2023.1564","url":null,"abstract":"<p><p>Sjögren's syndrome (SS) is a rare disease with the highest reported prevalence of 0.01-0.09%. Anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) is another rare auto-immune disease (prevalence of 0.0009-0.01%). The co-occurrence of these two separate clinical entities in one patient might rarely be encountered as an overlap syndrome. Here, we present the case of a 60-year-old female patient who had complaints of headache, nausea, weakness, gritty sensation in her eyes, and dry mouth [unstimulated saliva production of 0.033 mL/minute (normal; >0.1 mL/minute)] with a blood pressure of 190/110 mmHg, hypertensive retinopathy, proteinuric kidney disease, positivity of myeloperoxidase anti-neutrophil cytoplasmic antibodies, anti-Ro-52, anti-Ro, and anti-La antibodies. Pauci-immune crescentic proliferative glomerulonephritis was found in a kidney biopsy and successfully treated with cyclophosphamide and methylprednisolone. The co-occurrence of these diseases was first reported in 1992 by Böttinger et al. Since then, nearly 37 cases of SS and AAV have been reported. By reporting this case of primary SS and AAV, we emphasize the importance of auto-antibody tests in searching for the etiology of patients with proteinuria.</p>","PeriodicalId":21222,"journal":{"name":"Reumatismo","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2023-07-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10213066","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
ReumatismoPub Date : 2023-07-17DOI: 10.4081/reumatismo.2023.1544
M Ferrito, G Cincinelli, M Manara, R Di Taranto, E G Favalli, R Caporali
{"title":"Retention rate of tumor necrosis factor inhibitors, anti-interleukin 17, and anti-interleukin 12/23 drugs in a single-center cohort of psoriatic arthritis patients.","authors":"M Ferrito, G Cincinelli, M Manara, R Di Taranto, E G Favalli, R Caporali","doi":"10.4081/reumatismo.2023.1544","DOIUrl":"https://doi.org/10.4081/reumatismo.2023.1544","url":null,"abstract":"<p><p>The objective of this study was to evaluate biological disease-modifying anti-rheumatic drugs (bDMARDs) survival in several therapy courses of patients affected by psoriatic arthritis (PsA) and to compare tumor necrosis factor inhibitors (TNFi) and non-TNFi retention rates. A total of 241 bDMARD therapy courses (155 TNFi drugs, 65 anti-interleukin (IL)-17 drugs, and 21 anti-IL12/23) were analyzed. Bivariate analyses were performed to assess the presence of demographic and clinical features, as well as comorbidities, associated with bDMARD discontinuation in TNFi and non-TNFi groups. In the bivariate analyses of TNFi and non-TNFi groups, we found a lower age at the start of TNFi therapy in the former group [46 years, interquartile range (IQR) 45-54 vs 50.5 years, IQR 42-61; p=0.004] as well as a lower proportion of patients with skin psoriasis (65.8% vs 88.4%; p<0.001). Survival analysis showed no significant differences between TNFi and non-TNFi groups. Cox regression found fibromyalgia as a predictor of drug failure [hazard ratio (HR) 3.40, confidence interval (CI) 1.92-6.03; p<0.001] and first-line bDMARDs as a protective factor (HR 0.46, CI 0.25-0.88; p=0.019). Lastly, among TNFi courses, fibromyalgia was associated with drug suspension (HR 6.52, CI 3.16-13.46; p<0.001), while only a trend of significance for skin psoriasis as a risk factor for drug failure was shown (HR 2.38, CI 1.00-5.66, p=0.05). This study provides information about clinical and demographic factors associated with retention rates of bDMARDs from a real-life, single-center cohort of PsA patients.</p>","PeriodicalId":21222,"journal":{"name":"Reumatismo","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2023-07-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9853517","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
ReumatismoPub Date : 2023-07-17DOI: 10.4081/reumatismo.2023.1509
R Nicolau, T Martins Rocha, L Costa
{"title":"Pathergy-like reaction induced by laser hair removal in a patient with Behçet disease.","authors":"R Nicolau, T Martins Rocha, L Costa","doi":"10.4081/reumatismo.2023.1509","DOIUrl":"https://doi.org/10.4081/reumatismo.2023.1509","url":null,"abstract":"<p><p>Behçet disease (BD) is a rare systemic vasculitis of unknown etiology, primarily characterized by recurrent oral aphthous ulcers, genital ulcers, uveitis, and skin lesions. Pathergy test positivity is a nonspecific inflammatory response of the skin to trauma and supports the diagnosis. Recently, new inducers of pathergy reactions have been identified, for example, the placement of dental braces and laser hair removal. Our clinical case highlights the importance of thinking about this potential pathergy inducer in BD patients, to improve their quality of life and avoid complications.</p>","PeriodicalId":21222,"journal":{"name":"Reumatismo","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2023-07-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10213070","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
ReumatismoPub Date : 2023-07-17DOI: 10.4081/reumatismo.2023.1541
I Acosta-Colman, Z Morel, A Ayala Lugo, V Jolly, I De Guillén, P Langjahr, M Vazquez, M T Martínez de Filártiga, M E Acosta
{"title":"Clinical features and genetic biomarkers associated with different phenotypes of systemic lupus erythematosus in Paraguayan patients.","authors":"I Acosta-Colman, Z Morel, A Ayala Lugo, V Jolly, I De Guillén, P Langjahr, M Vazquez, M T Martínez de Filártiga, M E Acosta","doi":"10.4081/reumatismo.2023.1541","DOIUrl":"https://doi.org/10.4081/reumatismo.2023.1541","url":null,"abstract":"<p><p>Systemic lupus erythematosus (SLE) is a complex autoimmune disease characterized by a heterogeneous clinical picture that makes the diagnosis and follow-up of these patients difficult. This study aimed to identify correlations between clinical, immunological, and genetic biomarkers and clinical manifestations in SLE. A retrospective study of data from medical records and immunological and genetic studies of SLE patients in Paraguay was carried out. A descriptive analysis was performed based on the type of variable. Human leukocyte antigen (HLA) allele frequencies (DPA1, DPB1, DQA1, DQB1, and DRB1) were calculated, and univariate logistic regression analyses were performed between each of the explanatory variables and the presence or absence of each phenotype. Odds ratios, 95% confidence intervals, and p values were recorded. Associations with p<0.05 were considered statistically significant. 104 SLE patients were included: 86% were female, with a mean age of 32.80±10.36 years. An association was identified between anti-double stranded DNA (anti-dsDNA) and the presence of the renal phenotype and between anti-dsDNA and the absence of the joint and hematological phenotypes. Immunoglobulin M isotype rheumatoid factor was associated with the absence of a renal phenotype. HLA-DQB1*02:02 and HLA-DRB1*07:01 were associated with the cutaneous phenotype. An association was identified between age at disease onset over 30 years and the presence of the joint phenotype. No other associations were identified. Potential clinical, immunological, and genetic biomarkers of phenotypes have been identified in SLE Paraguayan patients.</p>","PeriodicalId":21222,"journal":{"name":"Reumatismo","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2023-07-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10213068","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
ReumatismoPub Date : 2023-05-08DOI: 10.4081/reumatismo.2023.1561
A Martins, S Ganhão, F Oliveira Pinheiro, C Granjo Morais, M Rodrigues, M Leuzinger-Dias, L Figueira, I Brito
{"title":"Uveitis in patients with oligoarticular juvenile idiopathic arthritis and juvenile spondyloarthritis/enthesitis related arthritis: is there any difference?","authors":"A Martins, S Ganhão, F Oliveira Pinheiro, C Granjo Morais, M Rodrigues, M Leuzinger-Dias, L Figueira, I Brito","doi":"10.4081/reumatismo.2023.1561","DOIUrl":"https://doi.org/10.4081/reumatismo.2023.1561","url":null,"abstract":"<p><p>Juvenile idiopathic arthritis (JIA) is the most common systemic disease causing uveitis in childhood and adolescence.</p>","PeriodicalId":21222,"journal":{"name":"Reumatismo","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2023-05-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9431295","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
ReumatismoPub Date : 2023-05-08DOI: 10.4081/reumatismo.2023.1571
F H C De Souza, D B De Araújo, L S Hoff, B G Baldi, M S M S Faria, L F Da Rocha Junior, L R S Da Silva, G L Behrens Pinto, M C Bezerra, R Miossi, R A Cordeiro, S K Shinjo
{"title":"Diagnosis and treatment of interstitial lung disease related to systemic autoimmune myopathies: a narrative review.","authors":"F H C De Souza, D B De Araújo, L S Hoff, B G Baldi, M S M S Faria, L F Da Rocha Junior, L R S Da Silva, G L Behrens Pinto, M C Bezerra, R Miossi, R A Cordeiro, S K Shinjo","doi":"10.4081/reumatismo.2023.1571","DOIUrl":"https://doi.org/10.4081/reumatismo.2023.1571","url":null,"abstract":"<p><p>Systemic autoimmune myopathies (SAMs) are rare diseases that lead to muscle inflammation and may be associated with a variety of systemic manifestations. Although there is great heterogeneity in the spectrum of extra-muscular involvement in SAMs, interstitial lung disease (ILD) is the most frequent lung manifestation. SAM-related ILD (SAM-ILD) presents significant variations according to geographic location and temporal trends and is associated with increased morbidity and mortality. Several myositis autoantibodies have been discovered over the last decades, including antibodies targeting aminoacyl-tRNA synthetase enzymes, which are associated with a variable risk of developing ILD and a myriad of other clinical features. In this review, the most relevant topics regarding clinical manifestations, risk factors, diagnostic tests, autoantibodies, treatment, and prognosis of SAM-ILD are highlighted. We searched PubMed for relevant articles published in English, Portuguese, or Spanish from January 2002 to September 2022. The most common SAM-ILD patterns are nonspecific interstitial pneumonia and organizing pneumonia. The combination of clinical, functional, laboratory, and tomographic features is usually sufficient for diagnostic confirmation, without the need for additional invasive methods. Glucocorticoids remain the first-line treatment for SAM-ILD, although other traditional immunosuppressants, such as azathioprine, mycophenolate, and cyclophosphamide have demonstrated some efficacy and, therefore, have an important role as steroid-sparing agents.</p>","PeriodicalId":21222,"journal":{"name":"Reumatismo","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2023-05-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9431294","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
ReumatismoPub Date : 2023-05-08DOI: 10.4081/reumatismo.2023.1551
F I Gorial, N I Awadh, A D Al-Obaidi, M N Al-Obaidi, H T Hashim, H J Hasan
{"title":"Post-streptococcal scleredema an unusual rare mimicker of scleroderma: a case report.","authors":"F I Gorial, N I Awadh, A D Al-Obaidi, M N Al-Obaidi, H T Hashim, H J Hasan","doi":"10.4081/reumatismo.2023.1551","DOIUrl":"https://doi.org/10.4081/reumatismo.2023.1551","url":null,"abstract":"<p><p>cleredema of Buschke is a rare pathological disorder of connective tissue, which is characterized by a woody, diffuse induration of the skin, most often in the upper extremities. We report an extremely rare complication of post-streptococcal infection in a six-year-old male complaining of gradually progressing, painless skin thickening and tightness which was preceded by a one-month history of fever, cough, and tonsillitis. By reporting this case, we hope to contribute to the creation of a database for future research aimed at better understanding the incidence, pathophysiology, and management of this extremely rare complication.</p>","PeriodicalId":21222,"journal":{"name":"Reumatismo","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2023-05-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9808143","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
ReumatismoPub Date : 2023-05-08DOI: 10.4081/reumatismo.2023.1591
The Editors
{"title":"Prof. Innocenzo Caruso.","authors":"The Editors","doi":"10.4081/reumatismo.2023.1591","DOIUrl":"https://doi.org/10.4081/reumatismo.2023.1591","url":null,"abstract":"<p><p>On July 29, 2022, Prof. Innocenzo Caruso, one of the first and best-known Italian rheumatologists, died at the age of 91 years.</p>","PeriodicalId":21222,"journal":{"name":"Reumatismo","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2023-05-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9437882","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}