European journal of rheumatology最新文献

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Minimal Change Disease and Primary Sjogren Syndrome Concurrence: Case-Based review. 微小变化疾病和原发性干燥综合征并发:基于病例的回顾。
IF 1.9
European journal of rheumatology Pub Date : 2022-10-01 DOI: 10.5152/eurjrheum.2022.20246
Nazife Şule Yaşar Bilge, Sultan Özkurt, Mustafa Fuat Açıkalın, Timuçin Kaşifoğlu
{"title":"Minimal Change Disease and Primary Sjogren Syndrome Concurrence: Case-Based review.","authors":"Nazife Şule Yaşar Bilge,&nbsp;Sultan Özkurt,&nbsp;Mustafa Fuat Açıkalın,&nbsp;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}
引用次数: 1
Multidisciplinary models for pregnancy care in patients with rheumatic diseases: Clinical experiences and experts opinion. 风湿病患者妊娠护理的多学科模式:临床经验和专家意见。
IF 1.9
European journal of rheumatology Pub Date : 2022-10-01 DOI: 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
{"title":"Multidisciplinary models for pregnancy care in patients with rheumatic diseases: Clinical experiences and experts opinion.","authors":"Andrea Pluma,&nbsp;Laia Alsina,&nbsp;Ofelia Baniandres,&nbsp;Rafael Caliz,&nbsp;Manel Casellas,&nbsp;Dolors Grados,&nbsp;Juan Antonio Martınez Lopez,&nbsp;Erika Padron Perez,&nbsp;Ana Polo,&nbsp;Jose Maria Pego Reigosa,&nbsp;Elisa Trujillo,&nbsp;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}
引用次数: 3
Ruxolitinib for Refractory Macrophage Activation Syndrome Complicating Adult-Onset Still's Disease. 鲁索利替尼治疗难治性巨噬细胞激活综合征并发成人发病斯蒂尔氏病。
IF 1.9
European journal of rheumatology Pub Date : 2022-10-01 DOI: 10.5152/eurjrheum.2022.21064
Ofer Levy, Arie Apel, Hossam Alhdor, Avraham Mizrachi, Nancy Agmon-Levin, Maya Koren-Michowitz, Mirit Amit-Vazina
{"title":"Ruxolitinib for Refractory Macrophage Activation Syndrome Complicating Adult-Onset Still's Disease.","authors":"Ofer Levy,&nbsp;Arie Apel,&nbsp;Hossam Alhdor,&nbsp;Avraham Mizrachi,&nbsp;Nancy Agmon-Levin,&nbsp;Maya Koren-Michowitz,&nbsp;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}
引用次数: 2
Takayasu arteritis early diagnosis by noninvasive imaging. 高须动脉炎的无创影像学早期诊断。
IF 1.9
European journal of rheumatology Pub Date : 2022-10-01 DOI: 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,&nbsp;Pedro Del Valle Rodriguez-Flores,&nbsp;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}
引用次数: 1
Concurrent use of tumor necrosis factor inhibitor and tyrosine kinase inhibitor in ankylosing spondylitis and myeloid neoplasm. 肿瘤坏死因子抑制剂和酪氨酸激酶抑制剂在强直性脊柱炎和髓系肿瘤中的同时应用。
IF 1.9
European journal of rheumatology Pub Date : 2022-10-01 DOI: 10.5152/eurjrheum.2022.21097
Akash Gupta, Yuliya Afinogenova, Nikolai A Podoltsev, Abhijeet Danve
{"title":"Concurrent use of tumor necrosis factor inhibitor and tyrosine kinase inhibitor in ankylosing spondylitis and myeloid neoplasm.","authors":"Akash Gupta,&nbsp;Yuliya Afinogenova,&nbsp;Nikolai A Podoltsev,&nbsp;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}
引用次数: 0
An Unusual Case of Extrapulmonary Tuberculosis in Rheumatology Consultation. 风湿病会诊一例罕见的肺外结核。
IF 1.9
European journal of rheumatology Pub Date : 2022-10-01 DOI: 10.5152/eurjrheum.2022.21207
Kayra Somay, İlker Eren, Mahir Kapmaz, Benan Çağlayan, Afak Durur Karakaya, Nilüfer Alpay Kanıtez
{"title":"An Unusual Case of Extrapulmonary Tuberculosis in Rheumatology Consultation.","authors":"Kayra Somay,&nbsp;İlker Eren,&nbsp;Mahir Kapmaz,&nbsp;Benan Çağlayan,&nbsp;Afak Durur Karakaya,&nbsp;Nilüfer Alpay Kanıtez","doi":"10.5152/eurjrheum.2022.21207","DOIUrl":"https://doi.org/10.5152/eurjrheum.2022.21207","url":null,"abstract":"","PeriodicalId":12066,"journal":{"name":"European journal of rheumatology","volume":"9 4","pages":"227-228"},"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/62/a5/ejr-9-4-227.PMC10089129.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9641561","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}
引用次数: 0
What are the main factors affecting the outcome of tocilizumab therapy in COVID-19-induced cytokine release syndrome? 在COVID-19诱导的细胞因子释放综合征中,影响tocilizumab治疗结果的主要因素是什么?
IF 1.9
European journal of rheumatology Pub Date : 2022-07-01 DOI: 10.5152/eurjrheum.2022.21010
Cansu Akleylek, Seray Gizem Gür, İbrahim Halil Sever, Safiye Koçulu Demir, Esin Çevik, Egemen Eken, Zafer Gökkaya, Yonca Çağatay, Neslihan Yılmaz
{"title":"What are the main factors affecting the outcome of tocilizumab therapy in COVID-19-induced cytokine release syndrome?","authors":"Cansu Akleylek,&nbsp;Seray Gizem Gür,&nbsp;İbrahim Halil Sever,&nbsp;Safiye Koçulu Demir,&nbsp;Esin Çevik,&nbsp;Egemen Eken,&nbsp;Zafer Gökkaya,&nbsp;Yonca Çağatay,&nbsp;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}
引用次数: 2
Identifying physician-perceived barriers to a pragmatic treatment trial in rheumatoid arthritis. 确定医生感知的障碍,以实际治疗类风湿关节炎试验。
IF 1.9
European journal of rheumatology Pub Date : 2022-07-01 DOI: 10.5152/eujrheum.2022.21038
Haiyan Qu, Shamly Austin, Jasvinder A Singh
{"title":"Identifying physician-perceived barriers to a pragmatic treatment trial in rheumatoid arthritis.","authors":"Haiyan Qu,&nbsp;Shamly Austin,&nbsp;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}
引用次数: 0
Physical activity and psychosomatic status in patients with Behçet's disease during coronavirus disease pandem. 冠状病毒大流行期间behaperet病患者的身体活动和心身状况
IF 1.9
European journal of rheumatology Pub Date : 2022-07-01 DOI: 10.5152/eurjrheum.2021.20166
Songül Bağlan Yentür, Furkan Bilek, Süleyman Serdar Koca
{"title":"Physical activity and psychosomatic status in patients with Behçet's disease during coronavirus disease pandem.","authors":"Songül Bağlan Yentür,&nbsp;Furkan Bilek,&nbsp;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}
引用次数: 0
Updates in ANCA-associated vasculitis. anca相关血管炎的最新进展。
IF 1.9
European journal of rheumatology Pub Date : 2022-07-01 DOI: 10.5152/eujrheum.2022.20248
Carolyn Ross, Jean-Paul Makhzoum, Christian Pagnoux
{"title":"Updates in ANCA-associated vasculitis.","authors":"Carolyn Ross,&nbsp;Jean-Paul Makhzoum,&nbsp;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}
引用次数: 5
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