Pub Date : 2022-10-01DOI: 10.5152/eurjrheum.2022.20246
Nazife Şule Yaşar Bilge, Sultan Özkurt, Mustafa Fuat Açıkalın, Timuçin Kaşifoğlu
Primary Sjogren's syndrome is a chronic autoimmune disease with glandular and extraglandular features. Renal involvement is less frequent when compared with other systemic manifestations. Glomerulonephritis is a relatively rare manifestation of primary Sjogren's syndrome. Among all types of glomerular manifestations, minimal change disease is rarely identified, and there are only a few cases in the literature. Herein, we present a 53-year-old male patient who was diagnosed with primary Sjogren's syndrome and minimal change disease while searching for the etiopathogenesis of nephrotic syndrome. The patient had edema, dyspnea, hypertension, and 12 g/day proteinuria at admission. Serum albumin level was 1.82 g/dL, and renal function tests were within normal ranges. Renal biopsy findings were consistent with minimal change disease. At the same time, he was diagnosed with primary Sjogren's syndrome based on dry eyes demonstrated with Schirmer's test, positive antinuclear antibody, anti-SS-A, and anti-SS-B antibodies. Hydroxychloroquine with methylprednisolone 1 mg/kg (64 mg/day) was started, and methylprednisolone was slowly tapered. His proteinuria regressed to 79.2 mg/day, creatinine level was 0.83 mg/dL, and serum albumin level increased to 3.88 g/dL on the second week of the glucocorticoid treatment. In this case-based review, we present our case with 5 other reports of minimal change disease associated with primary Sjogren's syndrome. Our aim was to increase the awareness of this rare concurrence both among rheumatologists and nephrologists in light of the literature review.
{"title":"Minimal Change Disease and Primary Sjogren Syndrome Concurrence: Case-Based review.","authors":"Nazife Şule Yaşar Bilge, Sultan Özkurt, Mustafa Fuat Açıkalın, Timuçin Kaşifoğlu","doi":"10.5152/eurjrheum.2022.20246","DOIUrl":"https://doi.org/10.5152/eurjrheum.2022.20246","url":null,"abstract":"<p><p>Primary Sjogren's syndrome is a chronic autoimmune disease with glandular and extraglandular features. Renal involvement is less frequent when compared with other systemic manifestations. Glomerulonephritis is a relatively rare manifestation of primary Sjogren's syndrome. Among all types of glomerular manifestations, minimal change disease is rarely identified, and there are only a few cases in the literature. Herein, we present a 53-year-old male patient who was diagnosed with primary Sjogren's syndrome and minimal change disease while searching for the etiopathogenesis of nephrotic syndrome. The patient had edema, dyspnea, hypertension, and 12 g/day proteinuria at admission. Serum albumin level was 1.82 g/dL, and renal function tests were within normal ranges. Renal biopsy findings were consistent with minimal change disease. At the same time, he was diagnosed with primary Sjogren's syndrome based on dry eyes demonstrated with Schirmer's test, positive antinuclear antibody, anti-SS-A, and anti-SS-B antibodies. Hydroxychloroquine with methylprednisolone 1 mg/kg (64 mg/day) was started, and methylprednisolone was slowly tapered. His proteinuria regressed to 79.2 mg/day, creatinine level was 0.83 mg/dL, and serum albumin level increased to 3.88 g/dL on the second week of the glucocorticoid treatment. In this case-based review, we present our case with 5 other reports of minimal change disease associated with primary Sjogren's syndrome. Our aim was to increase the awareness of this rare concurrence both among rheumatologists and nephrologists in light of the literature review.</p>","PeriodicalId":12066,"journal":{"name":"European journal of rheumatology","volume":"9 4","pages":"221-224"},"PeriodicalIF":1.9,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/5a/b8/ejr-9-4-221.PMC10089134.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9286588","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2022-10-01DOI: 10.5152/eujrheum.2022.20012
Andrea Pluma, Laia Alsina, Ofelia Baniandres, Rafael Caliz, Manel Casellas, Dolors Grados, Juan Antonio Martınez Lopez, Erika Padron Perez, Ana Polo, Jose Maria Pego Reigosa, Elisa Trujillo, Paloma Vela
Objectives: To describe different models of multidisciplinary pregnancy care for patients with inflammatory and autoimmune rheumatic diseases, and the steps to follow concerning their implementation.
Methods: A qualitative study was conducted including: (1) a comprehensive literature search in PUBMED focused on multidisciplinary care models; (2) structured interviews with seven rheumatologists from multidisciplinary pregnancy clinics for patients with inflammatory and autoimmune rheumatic diseases. Data were collected related to the hospitals, medical departments, populations cared for, and multidisciplinary care models (type, material, and human resources, professional requirements, objectives, referral criteria, agendas, protocols, responsibilities, decision-making, research and educational activities, multidisciplinary clinical sessions, initiation/start, planning, advantages/disadvantages, and barriers/facilitators for implementation); (3) a nominal meeting group in which the results of searches and interviews were analyzed and the recommendations for the implementation of the multidisciplinary care models defined.
Results: We analyzed seven models of multidisciplinary care in pregnancy, implemented 3-10 years ago, which can all be summarized by two different subtypes: parallel (patients are assessed the same day in the involved medical services) and preferential (patients are assessed on different days in the involved medical services) circuits. The implementation of a specific model results rather from an adaptation to the hospital's and professionals' circumstances. Correct planning and good harmony among professionals are key points to implementing a model.
Conclusion: Different multidisciplinary care models have been implemented for patients with inflammatory and autoimmune rheumatic diseases during pregnancy. They pretend to improve care, system efficiency, and collaboration among specialists and should be carefully implemented.
{"title":"Multidisciplinary models for pregnancy care in patients with rheumatic diseases: Clinical experiences and experts opinion.","authors":"Andrea Pluma, Laia Alsina, Ofelia Baniandres, Rafael Caliz, Manel Casellas, Dolors Grados, Juan Antonio Martınez Lopez, Erika Padron Perez, Ana Polo, Jose Maria Pego Reigosa, Elisa Trujillo, Paloma Vela","doi":"10.5152/eujrheum.2022.20012","DOIUrl":"https://doi.org/10.5152/eujrheum.2022.20012","url":null,"abstract":"<p><strong>Objectives: </strong>To describe different models of multidisciplinary pregnancy care for patients with inflammatory and autoimmune rheumatic diseases, and the steps to follow concerning their implementation.</p><p><strong>Methods: </strong>A qualitative study was conducted including: (1) a comprehensive literature search in PUBMED focused on multidisciplinary care models; (2) structured interviews with seven rheumatologists from multidisciplinary pregnancy clinics for patients with inflammatory and autoimmune rheumatic diseases. Data were collected related to the hospitals, medical departments, populations cared for, and multidisciplinary care models (type, material, and human resources, professional requirements, objectives, referral criteria, agendas, protocols, responsibilities, decision-making, research and educational activities, multidisciplinary clinical sessions, initiation/start, planning, advantages/disadvantages, and barriers/facilitators for implementation); (3) a nominal meeting group in which the results of searches and interviews were analyzed and the recommendations for the implementation of the multidisciplinary care models defined.</p><p><strong>Results: </strong>We analyzed seven models of multidisciplinary care in pregnancy, implemented 3-10 years ago, which can all be summarized by two different subtypes: parallel (patients are assessed the same day in the involved medical services) and preferential (patients are assessed on different days in the involved medical services) circuits. The implementation of a specific model results rather from an adaptation to the hospital's and professionals' circumstances. Correct planning and good harmony among professionals are key points to implementing a model.</p><p><strong>Conclusion: </strong>Different multidisciplinary care models have been implemented for patients with inflammatory and autoimmune rheumatic diseases during pregnancy. They pretend to improve care, system efficiency, and collaboration among specialists and should be carefully implemented.</p>","PeriodicalId":12066,"journal":{"name":"European journal of rheumatology","volume":"9 4","pages":"191-196"},"PeriodicalIF":1.9,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9101323","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}
Macrophage activation syndrome is the most frequent life-threatening complication of adult-onset Still's disease. This is a nearly fatal case of a young patient, which has been refractory to corticoste- roids, anakinra, tocilizumab, cyclosporine A, and etoposide, but eventually responded miraculously to salvage therapy with ruxolitinib. We review recent pertinent data related to the therapeutic value of ruxolitinib for macrophage activation syndrome triggered by adult-onset Still's disease.
{"title":"Ruxolitinib for Refractory Macrophage Activation Syndrome Complicating Adult-Onset Still's Disease.","authors":"Ofer Levy, Arie Apel, Hossam Alhdor, Avraham Mizrachi, Nancy Agmon-Levin, Maya Koren-Michowitz, Mirit Amit-Vazina","doi":"10.5152/eurjrheum.2022.21064","DOIUrl":"https://doi.org/10.5152/eurjrheum.2022.21064","url":null,"abstract":"<p><p>Macrophage activation syndrome is the most frequent life-threatening complication of adult-onset Still's disease. This is a nearly fatal case of a young patient, which has been refractory to corticoste- roids, anakinra, tocilizumab, cyclosporine A, and etoposide, but eventually responded miraculously to salvage therapy with ruxolitinib. We review recent pertinent data related to the therapeutic value of ruxolitinib for macrophage activation syndrome triggered by adult-onset Still's disease.</p>","PeriodicalId":12066,"journal":{"name":"European journal of rheumatology","volume":"9 4","pages":"217-220"},"PeriodicalIF":1.9,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/2b/52/ejr-9-4-217.PMC10089131.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9285011","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2022-10-01DOI: 10.5152/eurjrheum.2022.21119
Joaquin J Barjau-Vallet, Pedro Del Valle Rodriguez-Flores, Yeison Arias Morales
{"title":"Takayasu arteritis early diagnosis by noninvasive imaging.","authors":"Joaquin J Barjau-Vallet, Pedro Del Valle Rodriguez-Flores, Yeison Arias Morales","doi":"10.5152/eurjrheum.2022.21119","DOIUrl":"https://doi.org/10.5152/eurjrheum.2022.21119","url":null,"abstract":"","PeriodicalId":12066,"journal":{"name":"European journal of rheumatology","volume":"9 4","pages":"225-226"},"PeriodicalIF":1.9,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10591688","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 : 2022-10-01DOI: 10.5152/eurjrheum.2022.21097
Akash Gupta, Yuliya Afinogenova, Nikolai A Podoltsev, Abhijeet Danve
Biologic disease-modifying agents (bDMARDs) are highly effective in controlling the symptoms of autoimmune rheumatic diseases. The decision on whether to continue bDMARDs following a cancer diagnosis can be challenging for patients and physicians. Here, we describe a case of a middle-aged male with ankylosing spondylitis who was controlled on infliximab (IFX) and found to have a myeloid neoplasm with Platelet-Derived Growth Factor Receptor Beta rearrangement. The patient was started on a tyrosine kinase inhibitor imatinib. Given its significant positive effect on patient's quality of life, IFX was continued with a favorable outcome. This case highlights the importance of shared decisionmaking in balancing risks and benefits of immunosuppressants in appropriate cases of hematologic malignancy.
{"title":"Concurrent use of tumor necrosis factor inhibitor and tyrosine kinase inhibitor in ankylosing spondylitis and myeloid neoplasm.","authors":"Akash Gupta, Yuliya Afinogenova, Nikolai A Podoltsev, Abhijeet Danve","doi":"10.5152/eurjrheum.2022.21097","DOIUrl":"https://doi.org/10.5152/eurjrheum.2022.21097","url":null,"abstract":"<p><p>Biologic disease-modifying agents (bDMARDs) are highly effective in controlling the symptoms of autoimmune rheumatic diseases. The decision on whether to continue bDMARDs following a cancer diagnosis can be challenging for patients and physicians. Here, we describe a case of a middle-aged male with ankylosing spondylitis who was controlled on infliximab (IFX) and found to have a myeloid neoplasm with Platelet-Derived Growth Factor Receptor Beta rearrangement. The patient was started on a tyrosine kinase inhibitor imatinib. Given its significant positive effect on patient's quality of life, IFX was continued with a favorable outcome. This case highlights the importance of shared decisionmaking in balancing risks and benefits of immunosuppressants in appropriate cases of hematologic malignancy.</p>","PeriodicalId":12066,"journal":{"name":"European journal of rheumatology","volume":"9 4","pages":"215-216"},"PeriodicalIF":1.9,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9101321","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}
Objective: Recommendations for the treatment of cytokine release syndrome/macrophage activation syndrome (MAS) associated with coronavirus disease-2019 (COVID-19) are still of poor quality. IL-6 is an important therapeutic target as a main mediator of cytokine storm. The aim of our study was to evaluate the tocilizumab (TCZ) efficacy and factors affecting the therapy outcome.
Methods: This retrospective study included 27 patients treated with TCZ for COVID-19-MAS. All patients in this study were treated with TCZ (intravenously, at a dose of 8 mg kg1 ) in addition to standard therapy. Clinical improvement (survival and decreased oxygen demand) on the 10-14th days and secondary infection rate were assessed.
Results: In our 27 treated patients, 14 (51.8%) received TCZ in the intensive care unit (ICU) and seven (25.9%) were need to invasive mechanical ventilation (IMV). Fifteen (55.6%) of these patients revealed a good clinical response (four patients discharge from the ICU and 11 patients who followed-up in nonICU beds showed a decrease in oxygen demand). TCZ was significantly less effective in patients having high Murray lung injury score, low PO2/FiO2 ratio, IMV, and ICU admission (P < .05). Severity of hypoxemia was found as a single independent risk factor in the multivariable analysis (P < .05). Secondary bacterial infections rate was significantly higher in intubated patients (P < .01) or treated in the ICU (P ¼ .01).
Conclusion: TCZ was showed limited efficacy for COVID-19-related MAS. The most important predictive indicator for therapy outcome was found as the severity of hypoxemia. In addition, IMV and/or ICU was associated with the poor outcome and high side effect. So, controlled trials are still needed to confirm the indications and timing of TCZ therapy.
{"title":"What are the main factors affecting the outcome of tocilizumab therapy in COVID-19-induced cytokine release syndrome?","authors":"Cansu Akleylek, Seray Gizem Gür, İbrahim Halil Sever, Safiye Koçulu Demir, Esin Çevik, Egemen Eken, Zafer Gökkaya, Yonca Çağatay, Neslihan Yılmaz","doi":"10.5152/eurjrheum.2022.21010","DOIUrl":"https://doi.org/10.5152/eurjrheum.2022.21010","url":null,"abstract":"<p><strong>Objective: </strong>Recommendations for the treatment of cytokine release syndrome/macrophage activation syndrome (MAS) associated with coronavirus disease-2019 (COVID-19) are still of poor quality. IL-6 is an important therapeutic target as a main mediator of cytokine storm. The aim of our study was to evaluate the tocilizumab (TCZ) efficacy and factors affecting the therapy outcome.</p><p><strong>Methods: </strong>This retrospective study included 27 patients treated with TCZ for COVID-19-MAS. All patients in this study were treated with TCZ (intravenously, at a dose of 8 mg kg1 ) in addition to standard therapy. Clinical improvement (survival and decreased oxygen demand) on the 10-14th days and secondary infection rate were assessed.</p><p><strong>Results: </strong>In our 27 treated patients, 14 (51.8%) received TCZ in the intensive care unit (ICU) and seven (25.9%) were need to invasive mechanical ventilation (IMV). Fifteen (55.6%) of these patients revealed a good clinical response (four patients discharge from the ICU and 11 patients who followed-up in nonICU beds showed a decrease in oxygen demand). TCZ was significantly less effective in patients having high Murray lung injury score, low PO2/FiO2 ratio, IMV, and ICU admission (P < .05). Severity of hypoxemia was found as a single independent risk factor in the multivariable analysis (P < .05). Secondary bacterial infections rate was significantly higher in intubated patients (P < .01) or treated in the ICU (P ¼ .01).</p><p><strong>Conclusion: </strong>TCZ was showed limited efficacy for COVID-19-related MAS. The most important predictive indicator for therapy outcome was found as the severity of hypoxemia. In addition, IMV and/or ICU was associated with the poor outcome and high side effect. So, controlled trials are still needed to confirm the indications and timing of TCZ therapy.</p>","PeriodicalId":12066,"journal":{"name":"European journal of rheumatology","volume":"9 3","pages":"126-131"},"PeriodicalIF":1.9,"publicationDate":"2022-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39623314","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 : 2022-07-01DOI: 10.5152/eujrheum.2022.21038
Haiyan Qu, Shamly Austin, Jasvinder A Singh
Objective: The aim of this qualitative research was to identify physician-perceived patient and clinic barriers to patient recruitment in a rheumatoid arthritis (RA) pragmatic trial of anti-tumor necrosis factor (TNF) biologic versus non-TNF biologic/Janus-Kinase inhibitor initiation after an inadequate response to methotrexate.
Methods: Semistructured telephone interviews were conducted with 26 rheumatologists in March 2019. An exploratory thematic analysis approach was used to analyze the interview data.
Results: Physician perceived patient barriers to the implementation of an RA pragmatic trial. This theme covers three subthemes: (1) patients' personal barriers, (2) patients' treatment-related factors, and (3) trial-related factors (eg, patient recruitment, side effects, mode of use, etc). Physicians perceived clinic barriers interfered with the pragmatic trial enrollment from the clinic or the healthcare system perspective. This theme covered four subthemes: (1) clinic-related factors, (2) patient-related factors, (3) research personnel, and (4) facilitators (positive factors of the clinic).
Conclusion: Our results from the inductive thematic analysis will help researchers understand the key patient and clinic/system factors/barriers that may influence pragmatic RA trial implementation. The themes suggest there are factors that can be modified (eg, coordinator effort needed, effective patient recruitment during clinic visits, provider engagement) and challenges to overcome (patient insurance status, busy clinic flow, and space issues including limited number of patient rooms). In summary, these themes provide a basis for our and other research teams to develop clinic-centered and patientcentered strategies to implement a pragmatic RA trial.
{"title":"Identifying physician-perceived barriers to a pragmatic treatment trial in rheumatoid arthritis.","authors":"Haiyan Qu, Shamly Austin, Jasvinder A Singh","doi":"10.5152/eujrheum.2022.21038","DOIUrl":"https://doi.org/10.5152/eujrheum.2022.21038","url":null,"abstract":"<p><strong>Objective: </strong>The aim of this qualitative research was to identify physician-perceived patient and clinic barriers to patient recruitment in a rheumatoid arthritis (RA) pragmatic trial of anti-tumor necrosis factor (TNF) biologic versus non-TNF biologic/Janus-Kinase inhibitor initiation after an inadequate response to methotrexate.</p><p><strong>Methods: </strong>Semistructured telephone interviews were conducted with 26 rheumatologists in March 2019. An exploratory thematic analysis approach was used to analyze the interview data.</p><p><strong>Results: </strong>Physician perceived patient barriers to the implementation of an RA pragmatic trial. This theme covers three subthemes: (1) patients' personal barriers, (2) patients' treatment-related factors, and (3) trial-related factors (eg, patient recruitment, side effects, mode of use, etc). Physicians perceived clinic barriers interfered with the pragmatic trial enrollment from the clinic or the healthcare system perspective. This theme covered four subthemes: (1) clinic-related factors, (2) patient-related factors, (3) research personnel, and (4) facilitators (positive factors of the clinic).</p><p><strong>Conclusion: </strong>Our results from the inductive thematic analysis will help researchers understand the key patient and clinic/system factors/barriers that may influence pragmatic RA trial implementation. The themes suggest there are factors that can be modified (eg, coordinator effort needed, effective patient recruitment during clinic visits, provider engagement) and challenges to overcome (patient insurance status, busy clinic flow, and space issues including limited number of patient rooms). In summary, these themes provide a basis for our and other research teams to develop clinic-centered and patientcentered strategies to implement a pragmatic RA trial.</p>","PeriodicalId":12066,"journal":{"name":"European journal of rheumatology","volume":"9 3","pages":"132-138"},"PeriodicalIF":1.9,"publicationDate":"2022-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39792798","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 : 2022-07-01DOI: 10.5152/eurjrheum.2021.20166
Songül Bağlan Yentür, Furkan Bilek, Süleyman Serdar Koca
Objective: The aim of this study is to examine the changes in physical activity level, fatigue, depression, and sleep quality in patients with Behçet's disease during the COVID-19 pandemic.
Methods: The study was designed as an online questionnaire applied to individuals who are being followed up with the diagnosis of Behçet's disease in the rheumatology department. Data were collected using multiple scales including International Physical Activity Questionnaire (IPAQ), Fatigue Severity Scale (FSS), Beck Depression Inventory (BDI), Pittsburg Sleep Quality Index (PSQI), and Visual Analogue Scale (VAS) to evaluate physical activity level, fatigue, depression, sleep quality, and pain, respectively.
Results: Sixteen patients diagnosed with Behçet's disease were included in the study. No statistically significant difference was observed between the IPAQ, FSS, BDI, PSQI, and VAS assessment scores before COVID-19 and during COVID-19 period (P > .05 for all).
Conclusion: Thinking of the negative effects of aggressive clinical symptoms, Behçet's disease patients should be supported in physical activity and psychosocial status.
{"title":"Physical activity and psychosomatic status in patients with Behçet's disease during coronavirus disease pandem.","authors":"Songül Bağlan Yentür, Furkan Bilek, Süleyman Serdar Koca","doi":"10.5152/eurjrheum.2021.20166","DOIUrl":"https://doi.org/10.5152/eurjrheum.2021.20166","url":null,"abstract":"<p><strong>Objective: </strong>The aim of this study is to examine the changes in physical activity level, fatigue, depression, and sleep quality in patients with Behçet's disease during the COVID-19 pandemic.</p><p><strong>Methods: </strong>The study was designed as an online questionnaire applied to individuals who are being followed up with the diagnosis of Behçet's disease in the rheumatology department. Data were collected using multiple scales including International Physical Activity Questionnaire (IPAQ), Fatigue Severity Scale (FSS), Beck Depression Inventory (BDI), Pittsburg Sleep Quality Index (PSQI), and Visual Analogue Scale (VAS) to evaluate physical activity level, fatigue, depression, sleep quality, and pain, respectively.</p><p><strong>Results: </strong>Sixteen patients diagnosed with Behçet's disease were included in the study. No statistically significant difference was observed between the IPAQ, FSS, BDI, PSQI, and VAS assessment scores before COVID-19 and during COVID-19 period (P > .05 for all).</p><p><strong>Conclusion: </strong>Thinking of the negative effects of aggressive clinical symptoms, Behçet's disease patients should be supported in physical activity and psychosocial status.</p>","PeriodicalId":12066,"journal":{"name":"European journal of rheumatology","volume":"9 3","pages":"144-147"},"PeriodicalIF":1.9,"publicationDate":"2022-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39623311","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 : 2022-07-01DOI: 10.5152/eujrheum.2022.20248
Carolyn Ross, Jean-Paul Makhzoum, Christian Pagnoux
Antineutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAV) are small-vessel vasculitides that include granulomatosis with polyangiitis (formerly Wegener's granulomatosis), microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (Churg - Strauss syndrome). Renal-limited AAV can be considered a fourth entity. Despite their rarity and still unknown cause(s), research into AAV has been very active over the past decades and has allowed for the development of new therapeutic regimens. The pathogenesis is a complex process of immune dysregulations with genetic and environmental influences. Recent genome-wide association studies have identified multiple genetic predisposing variants, especially at the major histocompatibility complex region. The pathogenic role of antimyeloperoxidase ANCA (MPO-ANCA) is well supported by several animal models, but that of antiproteinase 3 ANCA (PR3-ANCA) is not as strongly demonstrated. B cells likely play a major role in the pathogenesis because they produce ANCAs, as do neutrophil abnormalities, imbalances in T-cell subtypes, and/or cytokine - chemokine networks. The role of the alternative complement pathway was established more recently, and studies of the antagonist of human C5a receptor (avacopan) in AAV have just been completed, with promising results. The current standard management of severe AAV still consists of remission induction therapy with glucocorticoids combined with rituximab or, less often now, cyclophosphamide. Several studies showed that reduced-dose regimens of glucocorticoids are noninferior to the previously used heavier regimens, for therefore less cumulative exposure to glucocorticoids. Avacopan use may even lead to new steroid-free therapeutic approaches, at least for some selected patients. Several trials and studies have now shown the superiority of rituximab over azathioprine or methotrexate as maintenance therapy. However, the optimal dosing regimen and duration for maintenance remain to be better defined, at the individual patient level. Many changes have occurred in the standard of care for AAV over the past decades, and more are expected soon, including with use of avacopan, but also, likely, a few other agents under investigation or development.
抗中性粒细胞细胞质抗体(ANCA)相关血管炎(AAV)是小血管血管炎,包括肉芽肿病合并多血管炎(原Wegener肉芽肿病)、显微镜下的多血管炎和嗜酸性肉芽肿病合并多血管炎(Churg - Strauss综合征)。肾受限型AAV可被视为第四个实体。尽管AAV罕见且病因不明,但在过去的几十年里,对AAV的研究一直非常活跃,并为新的治疗方案的开发提供了条件。其发病机制是一个复杂的免疫失调过程,受遗传和环境的影响。最近的全基因组关联研究已经确定了多种遗传易感变异,特别是在主要组织相容性复合体区域。抗髓过氧化物酶ANCA (MPO-ANCA)的致病作用已得到多种动物模型的支持,但抗蛋白酶3 ANCA (PR3-ANCA)的致病作用尚未得到充分证实。B细胞可能在发病机制中起主要作用,因为它们产生anca,中性粒细胞异常、t细胞亚型失衡和/或细胞因子-趋化因子网络也会产生anca。替代补体途径的作用是最近才确立的,而人类C5a受体拮抗剂(avacopan)在AAV中的研究刚刚完成,结果很有希望。目前严重AAV的标准治疗仍然包括糖皮质激素联合利妥昔单抗或环磷酰胺(现在较少使用)的缓解诱导治疗。几项研究表明,减少糖皮质激素剂量的方案并不逊于以前使用的更大剂量方案,因此减少了糖皮质激素的累积暴露。Avacopan的使用甚至可能导致新的无类固醇治疗方法,至少对于一些选定的患者。一些试验和研究表明,作为维持治疗,利妥昔单抗优于硫唑嘌呤或甲氨蝶呤。然而,在个体患者水平上,最佳给药方案和维持时间仍有待更好地确定。在过去的几十年里,AAV的治疗标准发生了许多变化,预计很快会有更多的变化,包括使用avacopan,但也可能有其他一些正在研究或开发的药物。
{"title":"Updates in ANCA-associated vasculitis.","authors":"Carolyn Ross, Jean-Paul Makhzoum, Christian Pagnoux","doi":"10.5152/eujrheum.2022.20248","DOIUrl":"https://doi.org/10.5152/eujrheum.2022.20248","url":null,"abstract":"<p><p>Antineutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAV) are small-vessel vasculitides that include granulomatosis with polyangiitis (formerly Wegener's granulomatosis), microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (Churg - Strauss syndrome). Renal-limited AAV can be considered a fourth entity. Despite their rarity and still unknown cause(s), research into AAV has been very active over the past decades and has allowed for the development of new therapeutic regimens. The pathogenesis is a complex process of immune dysregulations with genetic and environmental influences. Recent genome-wide association studies have identified multiple genetic predisposing variants, especially at the major histocompatibility complex region. The pathogenic role of antimyeloperoxidase ANCA (MPO-ANCA) is well supported by several animal models, but that of antiproteinase 3 ANCA (PR3-ANCA) is not as strongly demonstrated. B cells likely play a major role in the pathogenesis because they produce ANCAs, as do neutrophil abnormalities, imbalances in T-cell subtypes, and/or cytokine - chemokine networks. The role of the alternative complement pathway was established more recently, and studies of the antagonist of human C5a receptor (avacopan) in AAV have just been completed, with promising results. The current standard management of severe AAV still consists of remission induction therapy with glucocorticoids combined with rituximab or, less often now, cyclophosphamide. Several studies showed that reduced-dose regimens of glucocorticoids are noninferior to the previously used heavier regimens, for therefore less cumulative exposure to glucocorticoids. Avacopan use may even lead to new steroid-free therapeutic approaches, at least for some selected patients. Several trials and studies have now shown the superiority of rituximab over azathioprine or methotrexate as maintenance therapy. However, the optimal dosing regimen and duration for maintenance remain to be better defined, at the individual patient level. Many changes have occurred in the standard of care for AAV over the past decades, and more are expected soon, including with use of avacopan, but also, likely, a few other agents under investigation or development.</p>","PeriodicalId":12066,"journal":{"name":"European journal of rheumatology","volume":"9 3","pages":"153-166"},"PeriodicalIF":1.9,"publicationDate":"2022-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39792801","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}