Pub Date : 2023-05-08DOI: 10.4081/reumatismo.2023.1568
D G Fernández Ávila, M Rivera Maldonado, L P Charry Anzola
Cutaneous lupus erythematosus (CLE) is classified into three groups - acute, subacute, and chronic - based on clinical and histopathological characteristics. The risk of systemic manifestations varies among these groups. There are few studies on CLE epidemiology. For this reason, this paper aims to describe CLE prevalence and demographics in Colombia between 2015 and 2019. This descriptive, cross-sectional study used the international classification of diseases, tenth revision, for CLE subtypes, utilizing official data from the Colombian Ministry of Health. In people older than 19 years, 26,356 CLE cases were registered, yielding a prevalence of 76 cases per 100,000 population. CLE was more frequent in females, at a 5:1 ratio compared to males. The most common clinical presentation was discoid lupus erythematosus, in 45% of cases. The majority of cases occurred in people between 55 and 59 years old. This is the first study that describes CLE demographics in adults in Colombia. Findings regarding clinical subtypes and female predominance are consistent with those in the medical literature.
{"title":"Epidemiology and demographics of cutaneous lupus erythematosus in Colombia between 2015 and 2019.","authors":"D G Fernández Ávila, M Rivera Maldonado, L P Charry Anzola","doi":"10.4081/reumatismo.2023.1568","DOIUrl":"https://doi.org/10.4081/reumatismo.2023.1568","url":null,"abstract":"<p><p>Cutaneous lupus erythematosus (CLE) is classified into three groups - acute, subacute, and chronic - based on clinical and histopathological characteristics. The risk of systemic manifestations varies among these groups. There are few studies on CLE epidemiology. For this reason, this paper aims to describe CLE prevalence and demographics in Colombia between 2015 and 2019. This descriptive, cross-sectional study used the international classification of diseases, tenth revision, for CLE subtypes, utilizing official data from the Colombian Ministry of Health. In people older than 19 years, 26,356 CLE cases were registered, yielding a prevalence of 76 cases per 100,000 population. CLE was more frequent in females, at a 5:1 ratio compared to males. The most common clinical presentation was discoid lupus erythematosus, in 45% of cases. The majority of cases occurred in people between 55 and 59 years old. This is the first study that describes CLE demographics in adults in Colombia. Findings regarding clinical subtypes and female predominance are consistent with those in the medical literature.</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":"9437885","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}
Pub Date : 2023-05-08DOI: 10.4081/reumatismo.2023.1548
A Camargo-Coronel, H Quiñones-Moya, M R Hernández-Zavala, J R Hernández-Vázquez, M Á Vázquez-Zaragoza
The COVID-19 pandemic represents a global health problem, which has been mitigated by the opportune introduction of vaccination programs. Although we already know the benefit that vaccines provide, these are not exempt from adverse events which can be mild to deadly, such as idiopathic inflammatory myopathies, in which a temporal association has not been defined. It is for this reason that we carried out a systematic review of all reported cases of vaccination against COVID-19 and myositis. To identify previously reported cases of idiopathic inflammatory myopathies associated with vaccination against SARS-CoV-2 we registered this protocol on the website of PROSPERO with identification number CRD42022355551. Of the 63 publications identified in MEDLINE and 117 in Scopus, 21 studies were included, reporting 31 cases of patients with vaccination-associated myositis. Most of these cases were women (61.3%); mean age was 52.3 years (range 19-76 years) and mean time of symptom onset post-vaccination was 6.8 days. More than half of the cases were associated with Comirnaty, 11 cases (35.5%) were classified as dermatomyositis, and 9 (29%) as amyopathic dermatomyositis. In 6 (19.3%) patients another probable trigger was identified. Case reports of inflammatory myopathies associated with vaccination have heterogeneous presentations without any specific characteristics: as a consequence, it is not possible to ensure a temporal association between vaccination and the development of inflammatory myopathies. Large epidemiological studies are required to determine the existence of a causal association.
{"title":"Idiopathic inflammatory myopathies linked to vaccination against SARS-CoV-2: a systematic review.","authors":"A Camargo-Coronel, H Quiñones-Moya, M R Hernández-Zavala, J R Hernández-Vázquez, M Á Vázquez-Zaragoza","doi":"10.4081/reumatismo.2023.1548","DOIUrl":"https://doi.org/10.4081/reumatismo.2023.1548","url":null,"abstract":"<p><p>The COVID-19 pandemic represents a global health problem, which has been mitigated by the opportune introduction of vaccination programs. Although we already know the benefit that vaccines provide, these are not exempt from adverse events which can be mild to deadly, such as idiopathic inflammatory myopathies, in which a temporal association has not been defined. It is for this reason that we carried out a systematic review of all reported cases of vaccination against COVID-19 and myositis. To identify previously reported cases of idiopathic inflammatory myopathies associated with vaccination against SARS-CoV-2 we registered this protocol on the website of PROSPERO with identification number CRD42022355551. Of the 63 publications identified in MEDLINE and 117 in Scopus, 21 studies were included, reporting 31 cases of patients with vaccination-associated myositis. Most of these cases were women (61.3%); mean age was 52.3 years (range 19-76 years) and mean time of symptom onset post-vaccination was 6.8 days. More than half of the cases were associated with Comirnaty, 11 cases (35.5%) were classified as dermatomyositis, and 9 (29%) as amyopathic dermatomyositis. In 6 (19.3%) patients another probable trigger was identified. Case reports of inflammatory myopathies associated with vaccination have heterogeneous presentations without any specific characteristics: as a consequence, it is not possible to ensure a temporal association between vaccination and the development of inflammatory myopathies. Large epidemiological studies are required to determine the existence of a causal association.</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":"9808147","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}
Pub Date : 2023-05-08DOI: 10.4081/reumatismo.2023.1559
G Adami, L Idolazzi, C Benini, E Fracassi, A Carletto, O Viapiana, D Gatti, M Rossini, A Fassio
Psoriatic arthritis (PsA) is an inflammatory disease characterized by peripheral and axial involvement. Biological disease-modifying antirheumatic drugs (bDMARDs) are the mainstream treatment for PsA and bDMARDs retention rate is a proxy for the drug's overall effectiveness. However, it is unclear whether IL-17 inhibitors can have a higher retention rate than tumor necrosis factor (TNF) inhibitors, in particular in axial or peripheral PsA. A real-life observational study was conducted on bDMARD naïve PsA patients initiating TNF inhibitors or secukinumab. Time-to-switch analysis was carried out with Kaplan-Meyer curves (log-rank test) truncated at 3 years (1095 days). Sub-analyses of Kaplan-Meyer curves between patients presenting with prevalent peripheral PsA or prevalent axial PsA were also conducted. Cox regression models were employed to describe predictors of treatment switch/swap. Data on 269 patients with PsA naïve to bDMARD starting either TNF inhibitors (n=220) or secukinumab (n=48) were retrieved. The overall treatment retention at 1 and 2 years was similar for secukinumab and TNF inhibitors (log-rank test p NS). We found a trend towards significance in the Kaplan-Meyer at 3 years in favor of secukinumab (log-rank test p 0.081). Predominant axial disease was significantly associated with a higher chance of drug survival in secukinumab users (adjusted hazard ratio 0.15, 95% confidence interval = 0.04-0.54) but not in TNF inhibitor users. In this real-life, single-center, study on bDMARD naïve PsA patients, axial involvement was associated with longer survival of secukinumab but not of TNF inhibitors. Drug retention of secukinumab and TNF inhibitors were similar in predominantly peripheral PsA.
银屑病关节炎(PsA)是一种以外周和轴向受累为特征的炎症性疾病。生物疾病修饰抗风湿药物(bDMARDs)是治疗PsA的主流药物,bDMARDs保留率是药物总体有效性的一个指标。然而,目前尚不清楚IL-17抑制剂是否比肿瘤坏死因子(TNF)抑制剂具有更高的保留率,特别是在轴向或外周PsA中。一项针对bDMARD naïve PsA患者启动TNF抑制剂或secukinumab的现实观察性研究。切换时间分析采用Kaplan-Meyer曲线(log-rank检验)截断为3年(1095天)。Kaplan-Meyer曲线的亚分析也适用于外周血PsA和轴向PsA的患者。采用Cox回归模型描述治疗切换/交换的预测因子。269例PsA naïve to bDMARD患者的数据被检索,这些患者开始使用TNF抑制剂(n=220)或secukinumab (n=48)。对于secukinumab和TNF抑制剂,1年和2年的总体治疗保留率相似(log-rank test p NS)。我们发现Kaplan-Meyer在3年时对secukinumab有利(log-rank检验p 0.081)。在secukinumab使用者中,显性轴向疾病与较高的药物生存机会显著相关(调整后的风险比为0.15,95%可信区间= 0.04-0.54),但在TNF抑制剂使用者中无显著相关性。在这项针对bDMARD naïve PsA患者的现实单中心研究中,轴向受损伤与secukinumab的更长的生存期相关,而与TNF抑制剂无关。secukinumab和TNF抑制剂的药物保留在主要的外周PsA中相似。
{"title":"Secukinumab retention rate is greater in patients with psoriatic arthritis presenting with axial involvement.","authors":"G Adami, L Idolazzi, C Benini, E Fracassi, A Carletto, O Viapiana, D Gatti, M Rossini, A Fassio","doi":"10.4081/reumatismo.2023.1559","DOIUrl":"https://doi.org/10.4081/reumatismo.2023.1559","url":null,"abstract":"<p><p>Psoriatic arthritis (PsA) is an inflammatory disease characterized by peripheral and axial involvement. Biological disease-modifying antirheumatic drugs (bDMARDs) are the mainstream treatment for PsA and bDMARDs retention rate is a proxy for the drug's overall effectiveness. However, it is unclear whether IL-17 inhibitors can have a higher retention rate than tumor necrosis factor (TNF) inhibitors, in particular in axial or peripheral PsA. A real-life observational study was conducted on bDMARD naïve PsA patients initiating TNF inhibitors or secukinumab. Time-to-switch analysis was carried out with Kaplan-Meyer curves (log-rank test) truncated at 3 years (1095 days). Sub-analyses of Kaplan-Meyer curves between patients presenting with prevalent peripheral PsA or prevalent axial PsA were also conducted. Cox regression models were employed to describe predictors of treatment switch/swap. Data on 269 patients with PsA naïve to bDMARD starting either TNF inhibitors (n=220) or secukinumab (n=48) were retrieved. The overall treatment retention at 1 and 2 years was similar for secukinumab and TNF inhibitors (log-rank test p NS). We found a trend towards significance in the Kaplan-Meyer at 3 years in favor of secukinumab (log-rank test p 0.081). Predominant axial disease was significantly associated with a higher chance of drug survival in secukinumab users (adjusted hazard ratio 0.15, 95% confidence interval = 0.04-0.54) but not in TNF inhibitor users. In this real-life, single-center, study on bDMARD naïve PsA patients, axial involvement was associated with longer survival of secukinumab but not of TNF inhibitors. Drug retention of secukinumab and TNF inhibitors were similar in predominantly peripheral PsA.</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":"9437883","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}
Pub Date : 2023-05-08DOI: 10.4081/reumatismo.2023.1540
D Mohamadzadeh, S Assar, M Pournazari, P Soufivand, M S Soleymani
The aim of this study is to evaluate adherence to treatment and its related risk factors among a sample of rheumatoid arthritis patients (RA) attending the rheumatology outpatient clinic of Kermanshah university of medical sciences. In this cross-sectional study, RA patients were asked to complete the Morisky questionnaire and 19-item compliance questionnaire for rheumatology (CQR). Patients were divided into two groups: adherent and non-adherent to treatment, based on the CQR questionnaire results. Demographic and clinical characteristics (age, sex, marital status, education level, economical condition, occupational status, place of residence, underlying diseases, type, and number of drugs) were compared between the two groups to investigate possible risk associations for poor adherence. 257 patients completed the questionnaires (mean age: 43.22, 80.2% female). 78.6% were married, 54.9% were housekeepers, 37.7% had tertiary education, 61.9% had moderate economic status, and 73.2% were residents of an urban area with a large population. Prednisolone was the most commonly used drug followed by non-steroidal anti-inflammatory drugs, sulfasalazine, hydroxychloroquine, and methotrexate. The mean score of the Morisky questionnaire was 5.528 (standard deviation=1.79). 105 patients (40.9%) were adherent to treatment based on the CQR questionnaire. High education level (college or university) was correlated with non-adherence to treatment [27 (25.71%) vs 70 (46.05%), p=0.004]. We concluded that the prevalence of non-adherence to treatment is 59.1% in rheumatoid arthritis patients in Kermanshah, Iran. Having a higher education level is a risk factor for poor treatment adherence. Other variables could not predict treatment adherence.
{"title":"Adherence to treatment and associated factors in rheumatoid arthritis patients: a cross-sectional study from Iran.","authors":"D Mohamadzadeh, S Assar, M Pournazari, P Soufivand, M S Soleymani","doi":"10.4081/reumatismo.2023.1540","DOIUrl":"https://doi.org/10.4081/reumatismo.2023.1540","url":null,"abstract":"<p><p>The aim of this study is to evaluate adherence to treatment and its related risk factors among a sample of rheumatoid arthritis patients (RA) attending the rheumatology outpatient clinic of Kermanshah university of medical sciences. In this cross-sectional study, RA patients were asked to complete the Morisky questionnaire and 19-item compliance questionnaire for rheumatology (CQR). Patients were divided into two groups: adherent and non-adherent to treatment, based on the CQR questionnaire results. Demographic and clinical characteristics (age, sex, marital status, education level, economical condition, occupational status, place of residence, underlying diseases, type, and number of drugs) were compared between the two groups to investigate possible risk associations for poor adherence. 257 patients completed the questionnaires (mean age: 43.22, 80.2% female). 78.6% were married, 54.9% were housekeepers, 37.7% had tertiary education, 61.9% had moderate economic status, and 73.2% were residents of an urban area with a large population. Prednisolone was the most commonly used drug followed by non-steroidal anti-inflammatory drugs, sulfasalazine, hydroxychloroquine, and methotrexate. The mean score of the Morisky questionnaire was 5.528 (standard deviation=1.79). 105 patients (40.9%) were adherent to treatment based on the CQR questionnaire. High education level (college or university) was correlated with non-adherence to treatment [27 (25.71%) vs 70 (46.05%), p=0.004]. We concluded that the prevalence of non-adherence to treatment is 59.1% in rheumatoid arthritis patients in Kermanshah, Iran. Having a higher education level is a risk factor for poor treatment adherence. Other variables could not predict treatment adherence.</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":"9808144","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}
Pub Date : 2023-03-21DOI: 10.4081/reumatismo.2022.1507
K C Meléndez-Monreal, A J L Brambila-Tapia, V González-Díaz, S Cerpa-Cruz, S R Gutiérrez-Ureña, M D C Yeo-Ayala, R Robles-García, G E Martínez-Bonilla
Rheumatoid arthritis (RA) is a chronic autoimmune disease which has shown positive correlations between negative psychological variables and disease activity in transversal studies and in the follow-up. However, the association of positive psychological variables with disease parameters including disease activity (DAS-28), functional disability (HAQ) and erythrocyte sedimentation rate (ESR) has not been investigated. Patients with RA attending the external consultation of a third level hospital were invited to participate and fill in a questionnaire with personal, disease and psychological variables; body mass index was also obtained as well as ESR. A total of 49 patients were included. The three dependent variables correlated among them, with the highest correlation for DAS-28 and HAQ (r=0.645, p<0.01), followed by somatization and HAQ (r=0.614, p<0.01) or DAS-28 (r=0.537, P<0.01). In addition, HAQ showed negative correlations with environmental mastery (r=- 0.366, p<0.01), personal growth (r=-0.292, p<0.05) and monthly extra money (r=-0.328, p<0.05), and borderline negative correlations with emotion perception (r=-0.279, p=0.053) and self-acceptance (r=-0.250, p=0.08). ESR showed a significant negative correlation with emotion perception (r=-0.475, p<0.01). In conclusion, we observed important correlations of positive psychological variables with disease activity, functional disability and ESR that could be addressed in order to prevent or treat these disease features.
类风湿关节炎(RA)是一种慢性自身免疫性疾病,在横向研究和随访中显示出负性心理变量与疾病活动性呈正相关。然而,积极心理变量与疾病参数的关系,包括疾病活动性(DAS-28),功能残疾(HAQ)和红细胞沉降率(ESR)尚未调查。邀请某三级医院外会诊的RA患者参与并填写包含个人、疾病和心理变量的问卷;获得身体质量指数和ESR。共纳入49例患者。3个因变量之间存在相关性,其中DAS-28与HAQ相关性最高(r=0.645, p
{"title":"Association of positive psychological variables with disease activity, functional disability and erythrocyte sedimentation rate in patients with rheumatoid arthritis: a preliminary study.","authors":"K C Meléndez-Monreal, A J L Brambila-Tapia, V González-Díaz, S Cerpa-Cruz, S R Gutiérrez-Ureña, M D C Yeo-Ayala, R Robles-García, G E Martínez-Bonilla","doi":"10.4081/reumatismo.2022.1507","DOIUrl":"https://doi.org/10.4081/reumatismo.2022.1507","url":null,"abstract":"<p><p>Rheumatoid arthritis (RA) is a chronic autoimmune disease which has shown positive correlations between negative psychological variables and disease activity in transversal studies and in the follow-up. However, the association of positive psychological variables with disease parameters including disease activity (DAS-28), functional disability (HAQ) and erythrocyte sedimentation rate (ESR) has not been investigated. Patients with RA attending the external consultation of a third level hospital were invited to participate and fill in a questionnaire with personal, disease and psychological variables; body mass index was also obtained as well as ESR. A total of 49 patients were included. The three dependent variables correlated among them, with the highest correlation for DAS-28 and HAQ (r=0.645, p<0.01), followed by somatization and HAQ (r=0.614, p<0.01) or DAS-28 (r=0.537, P<0.01). In addition, HAQ showed negative correlations with environmental mastery (r=- 0.366, p<0.01), personal growth (r=-0.292, p<0.05) and monthly extra money (r=-0.328, p<0.05), and borderline negative correlations with emotion perception (r=-0.279, p=0.053) and self-acceptance (r=-0.250, p=0.08). ESR showed a significant negative correlation with emotion perception (r=-0.475, p<0.01). In conclusion, we observed important correlations of positive psychological variables with disease activity, functional disability and ESR that could be addressed in order to prevent or treat these disease features.</p>","PeriodicalId":21222,"journal":{"name":"Reumatismo","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2023-03-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9154410","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}
Pub Date : 2023-03-21DOI: 10.4081/reumatismo.2022.1577
A Mannoni
I met Roy Altman many years ago and had the opportunity to spend a few months working in his laboratory in the basement of the Veterans Hospital in Miami.
多年前,我遇到了罗伊·奥特曼,并有机会在他位于迈阿密退伍军人医院地下室的实验室里工作了几个月。
{"title":"Roy Altman.","authors":"A Mannoni","doi":"10.4081/reumatismo.2022.1577","DOIUrl":"https://doi.org/10.4081/reumatismo.2022.1577","url":null,"abstract":"<p><p>I met Roy Altman many years ago and had the opportunity to spend a few months working in his laboratory in the basement of the Veterans Hospital in Miami.</p>","PeriodicalId":21222,"journal":{"name":"Reumatismo","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2023-03-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9160813","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}
Pub Date : 2023-03-21DOI: 10.4081/reumatismo.2022.1488
P Sukhadiya, P Kumar, D S Meena, A Kumar P H, N Vijayan, P Garg, M K Garg
Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome caused by macrophages and cytotoxic T cells with aberrant activation. The primary (genetic) form, which is caused by mutations that affect lymphocyte cytotoxicity and immune regulation, is most prevalent in children, whereas the secondary (acquired) form is prevalent in adults. Secondary HLH is commonly caused by infections or cancers, but it can also be caused by autoimmune disorders, in which case it is known as macrophage activation syndrome (MAS; or MAS-HLH). A 25-year-old female presented with a high-grade fever that lasted for two weeks. His laboratory results revealed pancytopenia, neutropenia, hypertriglyceridemia, hypofibrinogenemia, and hyperferritinemia. Based on the clinical presentation and laboratory findings, a provisional diagnosis of HLH has been made. A HLH protocol was utilized to treat the patient. During the course of hospitalization, systemic lupus erythematosus (SLE) was identified as the underlying cause. She improved dramatically after receiving an immunosuppressive regimen of etoposide, cyclosporine, and dexamethasone according to HLH protocol-2004 with individualized modifications. The clinician should be aware that HLH may be the initial manifestation of underlying SLE. Early diagnosis and aggressive, individualized treatment are the key to improving outcomes.
{"title":"Unmasking of systemic lupus erythematosus in a patient with hemophagocytic lymphohistiocytosis- macrophage activation syndrome (HLA-MAS) and diffuse alveolar hemorrhage.","authors":"P Sukhadiya, P Kumar, D S Meena, A Kumar P H, N Vijayan, P Garg, M K Garg","doi":"10.4081/reumatismo.2022.1488","DOIUrl":"https://doi.org/10.4081/reumatismo.2022.1488","url":null,"abstract":"<p><p>Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome caused by macrophages and cytotoxic T cells with aberrant activation. The primary (genetic) form, which is caused by mutations that affect lymphocyte cytotoxicity and immune regulation, is most prevalent in children, whereas the secondary (acquired) form is prevalent in adults. Secondary HLH is commonly caused by infections or cancers, but it can also be caused by autoimmune disorders, in which case it is known as macrophage activation syndrome (MAS; or MAS-HLH). A 25-year-old female presented with a high-grade fever that lasted for two weeks. His laboratory results revealed pancytopenia, neutropenia, hypertriglyceridemia, hypofibrinogenemia, and hyperferritinemia. Based on the clinical presentation and laboratory findings, a provisional diagnosis of HLH has been made. A HLH protocol was utilized to treat the patient. During the course of hospitalization, systemic lupus erythematosus (SLE) was identified as the underlying cause. She improved dramatically after receiving an immunosuppressive regimen of etoposide, cyclosporine, and dexamethasone according to HLH protocol-2004 with individualized modifications. The clinician should be aware that HLH may be the initial manifestation of underlying SLE. Early diagnosis and aggressive, individualized treatment are the key to improving outcomes.</p>","PeriodicalId":21222,"journal":{"name":"Reumatismo","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2023-03-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9154411","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}
Pub Date : 2023-03-21DOI: 10.4081/reumatismo.2022.1555
M Cutolo, A Sulli, V Smith, E Gotelli
The SARS-CoV-2 infection causing the Coronavirus disease-19 (COVID-19) is characterized by a broad range of clinical manifestations, implicating microvascular damage with endothelial dysfunction and different organ involvement.
{"title":"Emerging nailfold capillaroscopic patterns in COVID-19: from acute patients to survivors.","authors":"M Cutolo, A Sulli, V Smith, E Gotelli","doi":"10.4081/reumatismo.2022.1555","DOIUrl":"https://doi.org/10.4081/reumatismo.2022.1555","url":null,"abstract":"<p><p>The SARS-CoV-2 infection causing the Coronavirus disease-19 (COVID-19) is characterized by a broad range of clinical manifestations, implicating microvascular damage with endothelial dysfunction and different organ involvement.</p>","PeriodicalId":21222,"journal":{"name":"Reumatismo","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2023-03-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9160814","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}
Pub Date : 2023-03-21DOI: 10.4081/reumatismo.2022.1502
B Cigdem Karacay, T Sahbaz, C Medin Ceylan
This study aims to determine the association between fatigue, kinesiophobia, disease severity, and physical inactivity by comparing fibromyalgia syndrome (FMS) patients with healthy controls. Pain and fatigue are significant barriers to the participation in functional activities. Inactivity is a result of fatigue, but exercise is the foundation of FMS treatment. This case-control study included a total of 203 participants (107 patients with FMS and 96 healthy volunteers). The fibromyalgia impact questionnaire, the fatigue severity scale, the international physical activity questionnaire, and the Tampa scale for kinesiophobia were assessed. The FMS group scored significantly higher on the fatigue severity scale and kinesiophobia than the control group (p<0.001). Significantly lower metabolic task equivalent (MET) scale values were observed in the FMS group compared to the control group (p<0.001). The severity of fatigue and kinesiophobia correlated positively with the FMS impact questionnaire (p=0.001, r=0.621) and negatively with the MET scale (p=0.009, r= -0.287). Patients with FMS experience greater fatigue, kinesiophobia, and inactivity. As the severity of FMS worsens, so do disability, kinesiophobia, and fatigue. This study highlights the importance of breaking the cycle of fatigue and inactivity in the treatment of FMS.
{"title":"The vicious cycle of physical inactivity, fatigue and kinesiophobia in patients with fibromyalgia syndrome.","authors":"B Cigdem Karacay, T Sahbaz, C Medin Ceylan","doi":"10.4081/reumatismo.2022.1502","DOIUrl":"https://doi.org/10.4081/reumatismo.2022.1502","url":null,"abstract":"<p><p>This study aims to determine the association between fatigue, kinesiophobia, disease severity, and physical inactivity by comparing fibromyalgia syndrome (FMS) patients with healthy controls. Pain and fatigue are significant barriers to the participation in functional activities. Inactivity is a result of fatigue, but exercise is the foundation of FMS treatment. This case-control study included a total of 203 participants (107 patients with FMS and 96 healthy volunteers). The fibromyalgia impact questionnaire, the fatigue severity scale, the international physical activity questionnaire, and the Tampa scale for kinesiophobia were assessed. The FMS group scored significantly higher on the fatigue severity scale and kinesiophobia than the control group (p<0.001). Significantly lower metabolic task equivalent (MET) scale values were observed in the FMS group compared to the control group (p<0.001). The severity of fatigue and kinesiophobia correlated positively with the FMS impact questionnaire (p=0.001, r=0.621) and negatively with the MET scale (p=0.009, r= -0.287). Patients with FMS experience greater fatigue, kinesiophobia, and inactivity. As the severity of FMS worsens, so do disability, kinesiophobia, and fatigue. This study highlights the importance of breaking the cycle of fatigue and inactivity in the treatment of FMS.</p>","PeriodicalId":21222,"journal":{"name":"Reumatismo","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2023-03-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9154409","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}
Pub Date : 2023-03-21DOI: 10.4081/reumatismo.2022.1556
A Tincani, G Fontana, C Mackworth-Young
Antiphospholipid Syndrome (APS) is an autoimmune disease which was defined in the early 1980s. The principal features include thromboembolic events and/or pregnancy losses in association with antiphospholipid antibodies (aPL). As an historical note, the full-blown picture of the syndrome resembles the illness suffered by Anne Stuart, Queen of England in the XVIII century, whose repeated miscarriages caused the end of the royal Stuart line and the Hanoverian succession. The identification of aPL started in the early XX century and was linked to the introduction of the serological test for the diagnosis of syphilis. This involves a reaction between an antibody (reagin) and a phospholipid antigen derived from bovine heart (cardiolipin). Later on, it was observed that not all subjects with a positive test had syphilis, and that the so called "false positive reaction" was often reported in patients with systemic lupus erythematosus. Different tests for the identification of aPL were subsequently developed: first lupus anticoagulant (1971) and then immunoassays for anticardiolipin (1983) and anti-beta2 glycoprotein I (1990) antibodies. In the same period the association between the presence of circulating aPL and thrombotic and obstetric events was established, both in patients with autoimmune diseases and in otherwise healthy subjects, leading to the identification of APS as a distinct autoimmune disease. This has allowed better diagnosis and more targeted treatment for many patients.
{"title":"The history of antiphospholipid syndrome.","authors":"A Tincani, G Fontana, C Mackworth-Young","doi":"10.4081/reumatismo.2022.1556","DOIUrl":"https://doi.org/10.4081/reumatismo.2022.1556","url":null,"abstract":"Antiphospholipid Syndrome (APS) is an autoimmune disease which was defined in the early 1980s. The principal features include thromboembolic events and/or pregnancy losses in association with antiphospholipid antibodies (aPL). As an historical note, the full-blown picture of the syndrome resembles the illness suffered by Anne Stuart, Queen of England in the XVIII century, whose repeated miscarriages caused the end of the royal Stuart line and the Hanoverian succession. The identification of aPL started in the early XX century and was linked to the introduction of the serological test for the diagnosis of syphilis. This involves a reaction between an antibody (reagin) and a phospholipid antigen derived from bovine heart (cardiolipin). Later on, it was observed that not all subjects with a positive test had syphilis, and that the so called \"false positive reaction\" was often reported in patients with systemic lupus erythematosus. Different tests for the identification of aPL were subsequently developed: first lupus anticoagulant (1971) and then immunoassays for anticardiolipin (1983) and anti-beta2 glycoprotein I (1990) antibodies. In the same period the association between the presence of circulating aPL and thrombotic and obstetric events was established, both in patients with autoimmune diseases and in otherwise healthy subjects, leading to the identification of APS as a distinct autoimmune disease. This has allowed better diagnosis and more targeted treatment for many patients.","PeriodicalId":21222,"journal":{"name":"Reumatismo","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2023-03-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9160812","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}