Autoimmunity reviews最新文献

{"title":"Cardiovascular disease in connective tissue disease-associated interstitial lung disease: A systematic review and meta-analysis of observational studies","authors":"Ziyi Hu ,&nbsp;Haolan Wang ,&nbsp;Jinyu Huang ,&nbsp;Guanhui Yang ,&nbsp;Wenxuan Luo ,&nbsp;Jiaxun Zhong ,&nbsp;Xiaoli Zheng ,&nbsp;Xin Wei ,&nbsp;Xiongyan Luo ,&nbsp;Anji Xiong","doi":"10.1016/j.autrev.2024.103614","DOIUrl":"10.1016/j.autrev.2024.103614","url":null,"abstract":"<div><h3>Objectives</h3><div>We performed a systematic review and meta-analysis to assess whether patients with connective tissue disease (CTD)-associated interstitial lung diseases (ILD) have an increased prevalence of cardiovascular (CV) disease and to validate associated risk factors.</div></div><div><h3>Methods</h3><div>The PRISMA guidelines and PICO model were followed. We searched PubMed, Embase, Cochrane Library databases, Scopus, and Directory of Open Access Journals from inception to April 2024.</div></div><div><h3>Results</h3><div>Thirteen studies comprising of 12,520 patients were included. Patients with CTD-ILD had a significantly increased risk of CV disease than patients with CTD (relative risk [RR] = 1.65, 95 % confidence interval [CI]: 1.41, 1.93), which are related to the proportion of men (<em>P</em> = 0.001) and the proportion of smokers (<em>P</em> = 0.045). Subgroup analysis found that patients with CTD-ILD had a higher risk of heart failure (RR = 2.84, 95 % CI: 1.50, 5.39), arrhythmia (RR = 1.55, 95 % CI: 1.22, 1.97) than patients with CTD. Another subgroup analysis showed that RA-ILD and SSc-ILD were associated with an increased risk of CV disease, but not IIM-ILD and MCTD-ILD (RA-ILD: RR = 2.19, 95 % CI: 1.27, 3.80; SSc-ILD: RR = 1.53, 95 % CI: 1.29, 1.82). Besides, patients with CTD-ILD had a higher prevalence of pulmonary arterial hypertension (RR = 2.48, 95 % CI: 1.69, 3.63) than patients with CTD.</div></div><div><h3>Conclusions</h3><div>Patients with CTD-ILD had a 1.65 times increased risk of CV than patients with CTD-non-ILD, with increased prevalence of heart failure and arrhythmia. The risk of CV disease in SSc-ILD and RA-ILD is increased and we should pay more attention to male smokers. In addition, compared with CTD patients, CTD-ILD patients had a higher risk of pulmonary arterial hypertension.</div></div>","PeriodicalId":8664,"journal":{"name":"Autoimmunity reviews","volume":"23 10","pages":"Article 103614"},"PeriodicalIF":9.2,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142118890","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Immunopathogenesis of systemic lupus erythematosus: An update","authors":"Laurent Arnaud ,&nbsp;François Chasset ,&nbsp;Thierry Martin","doi":"10.1016/j.autrev.2024.103648","DOIUrl":"10.1016/j.autrev.2024.103648","url":null,"abstract":"<div><div>Systemic lupus erythematosus (SLE) is a chronic systemic autoimmune disease characterized by dysregulated immune responses leading to widespread inflammation and damage in various organs. Environmental factors such as infections, hormonal influences and exposure to ultraviolet light can trigger the disease in genetically predisposed individuals. Genome-wide association studies have identified over 100 susceptibility loci linked to immune regulation, interferon (IFN) signaling and antigen presentation in SLE. In addition, rare cases of monogenic lupus have been instrumental in understanding critical underlying disease mechanisms. Several immunological abnormalities contribute to the loss of self-tolerance and the perpetuation of autoimmune responses in SLE. In particular, defective clearance of apoptotic cells due to defective phagocytosis and complement activation leads to accumulation of self-antigens. Dysregulated innate immune responses activate the adaptive immune system, amplifying the inflammatory response with an important role for type I IFNs. Abnormalities in B cell development and activation lead to the production of autoreactive antibodies, forming immune complexes that cause tissue damage. Similarly, disturbances in T-cell compartments, altered regulatory T-cell functions and altered cytokine production, particularly IFN-α, contribute to tissue damage. Understanding of the immunopathogenesis of SLE is evolving rapidly, with ongoing research identifying new molecular pathways and potential therapeutic targets. Future classifications of SLE are likely to be based on underlying biological pathways rather than clinical and serological signs alone. This review aims to provide a detailed update on the most recent findings regarding the immunopathogenesis of SLE, focusing on the variability of biological pathways and the implications for future therapeutic strategies, in particular chimeric antigen receptor T (CAR T) cells.</div></div>","PeriodicalId":8664,"journal":{"name":"Autoimmunity reviews","volume":"23 10","pages":"Article 103648"},"PeriodicalIF":9.2,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142340217","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Helminth derivative tuftsin-phopshorylcholine to treat autoimmunity","authors":"Miri Blank ,&nbsp;Yehuda Shoenfeld","doi":"10.1016/j.autrev.2024.103601","DOIUrl":"10.1016/j.autrev.2024.103601","url":null,"abstract":"<div><div>Autoimmune diseases (AIDs) affect 5 to 10% of the population. There are more than ∼100 different autoimmune diseases. The AIDs are one of the top 10 causes of death in women under 65; 2nd highest cause of chronic illness; top cause of morbidity in women in the US. The NIH estimates annual direct healthcare costs for autoimmune diseases about $100 billion, in comparison, with cancers investment of $57 billion, heart and stroke cost of $200 billion.</div><div>The current treatments for autoimmune diseases encompasses: steroids, chemotherapy, immunosuppressants, biological drugs, disease specific drugs (like acethylcholine-estherase for myasthenia gravis). The treatments for autooimmune diseases supress the patient immune network, which leads the patients to be more susceptible to infections. Hence, there is a need to develop immunomodulatory small molecules with minimal side effects to treat autoimmune diseases.</div><div>The helminths developed secreting compounds which modulate the human defense pathways in order to develop tolerance and survive in the host environment.</div><div>We have imitated the immunomodulatory activity of the helminth by using a derivative of the helminth secretory molecule.</div><div>A bi-functional small molecule –tuftsin (T)-phosphorylcholine (PC), coined as TPC, was constructed. This chimeric molecule showed its immunomodulatory activity in 4 murine models of autoimmune diseases, attenuating the clinical score and the inflammatory response by immunomodutating the host immune system. <em>Ex-vivo</em> in human peripheral blood mononuclear cells (PBMCs) and biopsies originated from arteries of patients with giant cell arteritis. This paper decipher the mode of action of TPC immunomodulatory activity. Our data propose the potential for this small molecule to be a novel therapy for patients with autoimmune diseases.</div></div>","PeriodicalId":8664,"journal":{"name":"Autoimmunity reviews","volume":"23 10","pages":"Article 103601"},"PeriodicalIF":9.2,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142003502","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Corrigendum to “Practical guidance for the early recognition and follow-up of patients with connective tissue disease-related interstitial lung disease” [Autoimmunity Reviews - Volume 23, Issue 6, June 2024, 103582]","authors":"Julien Guiot ,&nbsp;Jelle Miedema ,&nbsp;Ana Cordeiro ,&nbsp;Jeska K. De Vries-Bouwstra ,&nbsp;Theodoros Dimitroulas ,&nbsp;Klaus Søndergaard ,&nbsp;Argyrios Tzouvelekis ,&nbsp;Vanessa Smith","doi":"10.1016/j.autrev.2024.103641","DOIUrl":"10.1016/j.autrev.2024.103641","url":null,"abstract":"","PeriodicalId":8664,"journal":{"name":"Autoimmunity reviews","volume":"23 10","pages":"Article 103641"},"PeriodicalIF":9.2,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142380001","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Navigating the landscape of SLE treatment: An expert viewpoint on the rationality and limitations of early biologic intervention","authors":"Mariele Gatto ,&nbsp;Margherita Zen ,&nbsp;Claudio Cruciani ,&nbsp;Luca Iaccarino ,&nbsp;Andrea Doria","doi":"10.1016/j.autrev.2024.103612","DOIUrl":"10.1016/j.autrev.2024.103612","url":null,"abstract":"<div><div>The approval of biologics, namely belimumab and anifrolumab, is being a game-changer in the approach to systemic lupus erythematosus (SLE). Currently we are indeed facing a revolution in the treatment paradigm of SLE, encompassing early combination of biologics with standard treatment in severe manifestations. In this regard, a lively discussion is taking place regarding the better positioning of biologics in the treatment of not necessarily severe, yet refractory and/or disfiguring manifestations which expose patients to worsened quality of life, reduced workability and enhanced risk of organ damage especially related to the misuse of glucocorticoids in the long run. Growing evidence supports the early use of targeted treatments in those patients, including the use of biologics before traditional immunosuppression, to achieve control of disease activity while minimizing treatment-related damage, privileging the timely use of therapeutics selectively impacting on key disease mechanisms in spite of a widespread immunosuppression. Patient profiling on a clinical and endotypical basis is helping in identifying better candidates to targeted drugs. More inflammatory organ involvement including persistent arthritis and infiltrating skin lesions seem likely to respond to anifrolumab, while B-mediated manifestations, a lively serology and a relapsing-remitting SLE course hint at a suitable role for belimumab. This seems at least partially connected to the inner effect of either drug, dampening inflammation through down-regulation of interferon signalling in the case of anifrolumab, while plastically modulating the B cell pool composition and function when coming to belimumab. Nevertheless, the mechanisms of both drugs are immunologically entangled at some extent, thereby requiring careful management especially in patients with longer disease history burdened with mixed manifestations. In this viewpoint we go over pros and cons of anticipatory biologic use in SLE, exploring features linked with better efficacy of either drug and the pathogenic and practical rationale for their positioning before traditional immunosuppression in moderate refractory SLE to be optimally managed in the 21st Century.</div></div>","PeriodicalId":8664,"journal":{"name":"Autoimmunity reviews","volume":"23 10","pages":"Article 103612"},"PeriodicalIF":9.2,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142103891","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Blood-dominant disease in late-and-early-onset lupus: A systematic review and meta-analysis","authors":"Sarah Abi Doumeth ,&nbsp;Jeries Kort ,&nbsp;Omer Nuri Pamuk","doi":"10.1016/j.autrev.2024.103652","DOIUrl":"10.1016/j.autrev.2024.103652","url":null,"abstract":"<div><h3>Objectives</h3><div>Numerous studies have explored hematological manifestations in early-onset systemic lupus erythematosus (erSLE) (age ≤ 50) and late-onset SLE (ltSLE) patients (age &gt; 50), yielding diverse results. This study employs a meta-analysis to examine differences in hematologic manifestations between ltSLE and erSLE.</div></div><div><h3>Methods</h3><div>Studies investigating the frequency of hematological manifestations in ltSLE patients were included. The frequencies of autoimmune hemolytic anemia (AIHA), thrombocytopenia (TP), lymphopenia, leukopenia, lymphadenopathy, and thrombosis were compared between erSLE and ltSLE groups. Two authors independently reviewed and assessed data consistency among abstracts, tables, and text to mitigate bias. Forest plots were utilized to compare odds ratios (95 % CI) of hematological manifestations by age groups, and study heterogeneity was evaluated using I².</div></div><div><h3>Results</h3><div>The analysis included 39 eligible studies with 19,103 SLE patients (16,314 erSLE, 2789 ltSLE). Among these studies, 28 reported AIHA which was found to be more frequent in erSLE (OR = 1.29, 95 %CI = 1.11–1.39, <em>p</em> = 0.0008). Twenty studies provided data on lymphopenia which was found to be more frequent in erSLE (OR = 1.184, 95 %CI = 1.063–1.318, <em>p</em> = 0.0021). 32 studies included data on leukopenia and the frequency was higher in erSLE (OR: 1.338, 95 %CI: 1.22–1.47, <em>p</em> &lt; 0.0001). Lymphadenopathy was more prevalent in erSLE (OR = 2.32, 95 % CI = 1.61–3.34, p &lt; 0.0001). No significant difference was observed in thrombosis and TP frequency between the two groups.</div></div><div><h3>Conclusion</h3><div>Attributing hematological findings to SLE in late-onset patients presents challenges due to comorbidities and polypharmacy. Overall, the frequencies of AIHA, lymphopenia, leukopenia, and lymphadenopathy were more common in erSLE patients compared to ltSLE in this study.</div></div>","PeriodicalId":8664,"journal":{"name":"Autoimmunity reviews","volume":"23 11","pages":"Article 103652"},"PeriodicalIF":9.2,"publicationDate":"2024-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142364192","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Association between Omega-3 fatty acids and autoimmune disease: Evidence from the umbrella review and Mendelian randomization analysis","authors":"Kimsor Hong ,&nbsp;Marady Hun ,&nbsp;Feifeng Wu ,&nbsp;Jueyi Mao ,&nbsp;Yang Wang ,&nbsp;Junquan Zhu ,&nbsp;Xin Zhou ,&nbsp;Haotian Xie ,&nbsp;Jidong Tian ,&nbsp;Chuan Wen","doi":"10.1016/j.autrev.2024.103651","DOIUrl":"10.1016/j.autrev.2024.103651","url":null,"abstract":"<div><h3>Background</h3><div>Autoimmune diseases are a group of disorders characterized by abnormal immune responses that mistakenly target and attack healthy cells, tissues, and organs, resulting in inflammation and tissue damage. Omega-3 fatty acids possess anti-inflammatory activities and may decrease abnormal immune activity. However, the role of omega-3 fatty acids in various autoimmune diseases is still unclear. This umbrella review and Mendelian randomization (MR) study aims to summarize the highest available evidence on omega-3 fatty acids and autoimmune disease.</div></div><div><h3>Methods</h3><div>We conducted an umbrella review by searching electronic databases to identify systematic reviews and meta-analyses. The selection criteria included systematic reviews with or without meta-analysis, which evaluated omega-3 fatty acids as the exposure and autoimmune disease as the outcome variable. Two authors independently assessed the overlapping and quality of the reviews using the AMSTAR-2 tool. We also performed MR studies to investigate the potential causal effect of fatty acids on the risk of various autoimmune diseases, utilizing data from the meta-analysis of the UKB-TOPMed and FinnGen cohorts.</div></div><div><h3>Result</h3><div>The umbrella review identified 21 studies (8 systematic reviews and 13 meta-analyses) on 9 autoimmune diseases and 30 diseases in the MR study. AMSTAR 2 categorized the quality of evidence in six studies as critically low, six studies as low, eight studies as moderate, and one as high-quality evidence. The consistent result between the review and the MR study demonstrated the benefit of omega-3 fatty acids on rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE). Additionally, in our summary review, omega-3 fatty acids can improve disease activity and inflammation biomarkers; however, MR studies provided no consistent evidence for the causal effects of omega-3 fatty acids on psoriasis, multiple sclerosis (MS), type 1 diabetes (T1D), IgA nephropathy (IgAN), juvenile idiopathic arthritis (JIA), Crohn's disease (CD), and ulcerative colitis (UC).</div></div><div><h3>Conclusion</h3><div>The current study presented solid evidence highlighting the advantageous impact of omega-3 fatty acids on SLE and RA. This was achieved through the reduction of disease risk, the decrease of disease activity, and the mitigation of inflammatory biomarkers. To stratify another autoimmune illness, it is necessary to carry out rigorous evaluations to surpass the existing findings and enhance understanding in this domain.</div></div>","PeriodicalId":8664,"journal":{"name":"Autoimmunity reviews","volume":"23 11","pages":"Article 103651"},"PeriodicalIF":9.2,"publicationDate":"2024-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142364191","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Tyrosine kinase 2 inhibitors in autoimmune diseases","authors":"Chethana Ramakrishna ,&nbsp;Alice Mason ,&nbsp;Christopher J. Edwards","doi":"10.1016/j.autrev.2024.103649","DOIUrl":"10.1016/j.autrev.2024.103649","url":null,"abstract":"<div><div>Tyk2 is a member of the JAK kinase family. It is an important mediator in pro-inflammatory signalling, implicated in both innate and adaptive immune system. Activation of Tyk2 is believed to be integral to cellular processes that contribute to the development and progression of autoimmune disorders. Selective targeting of Tyk2 may reduce the number of adverse events as compared to non-selective JAK inhibitors. Therefore, in recent years there has been a growing body of research examining the inhibition of Tyk2 as a therapeutic intervention in autoimmune disease. Deucravacitinib has been approved for the treatment of moderate to severe skin psoriasis. This drug and other novel Tyk2 inhibitors are now being explored as therapies for multiple autoimmune diseases, including psoriatic arthritis, SLE, Sjogren's, dermatomyositis, inflammatory bowel disease, uveitis, hidradenitis suppurativa and others. Tyk2 inhibitors offer a potentially exciting new treatment option across a wide range of autoimmune diseases. We discuss Tyk2 inhibition, the current evidence for its usage to date, ongoing trials and what the future might hold.</div></div>","PeriodicalId":8664,"journal":{"name":"Autoimmunity reviews","volume":"23 11","pages":"Article 103649"},"PeriodicalIF":9.2,"publicationDate":"2024-09-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142340219","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cutting edge confusion about cut-off settings in autoimmune diagnostics","authors":"Jan Damoiseaux ,&nbsp;Hetty Bontkes ,&nbsp;Leontine Mulder","doi":"10.1016/j.autrev.2024.103650","DOIUrl":"10.1016/j.autrev.2024.103650","url":null,"abstract":"<div><div>In autoimmune diagnostics results are interpreted in relation to a single or multiple cut-off value(s) in order to decide if the test is negative, weak positive, positive, or even strong positive. The way a cut-off is established appears to be very heterogeneous and this hampers harmonization of test results obtained in assays, either established in-house or obtained from different companies. In this context it is surprising that in diagnostic and classification criteria for distinct autoimmune diseases referral is made to cut-off values with the intention of harmonization. In this review article distinct ways to establish cut-off values will be described and discussed in relation to some disease criteria in order to increase the awareness of the confusion matrix of cut-off values and, as a consequence, the implications for test result interpretation.</div></div>","PeriodicalId":8664,"journal":{"name":"Autoimmunity reviews","volume":"23 11","pages":"Article 103650"},"PeriodicalIF":9.2,"publicationDate":"2024-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142340216","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Indian Rheumatology Association guidelines for the management of ANCA associated vasculitis","authors":"Aadhaar Dhooria ,&nbsp;G.S.R.S.N.K. Naidu ,&nbsp;Durga Prasanna Misra ,&nbsp;Benzeeta Pinto ,&nbsp;M.B. Adarsh ,&nbsp;Saket Jha ,&nbsp;Rajiv Ranjan Kumar ,&nbsp;Arghya Chattopadhyay ,&nbsp;Vikas Sharma ,&nbsp;Debashish Mishra ,&nbsp;Nupoor Acharya ,&nbsp;Sakshi Mittal ,&nbsp;Siddharth Jain ,&nbsp;Joydeep Samanta ,&nbsp;Chengappa Kavadichanda ,&nbsp;Sahajal Dhooria ,&nbsp;Raja Ramachandran ,&nbsp;Ramesh Jois ,&nbsp;Banwari Sharma ,&nbsp;Canchi Balakrishnan ,&nbsp;Aman Sharma","doi":"10.1016/j.autrev.2024.103647","DOIUrl":"10.1016/j.autrev.2024.103647","url":null,"abstract":"<div><h3>Background</h3><div>The ACR in 2021 and the EULAR in 2022 published recommendations for management of ANCA-associated vasculitis. Given the differences in the demographic, clinical profiles, and the socio-economic realities between various countries, there is a need for development of guidelines for the management of AAV for less economically developed regions of the world.</div></div><div><h3>Methods</h3><div>These guidelines were made following the GRADE methodology. After the systematic literature review, recommendations were formulated and opinion was sought from the 18-member expert panel consisting of 17 clinicians and one patient representative.</div></div><div><h3>Results</h3><div>Twenty recommendations were formulated. We recommend ANCA testing by ELISA over IIF. For remission induction in active GPA or MPA, we recommend use of intravenous cyclophosphamide or rituximab in combination with glucocorticoids. We conditionally recommend the use of reduced dose glucocorticoids over standard dose glucocorticoids for remission induction in active GPA or MPA. For remission maintenance in patients with GPA or MPA, we recommend the use of rituximab over azathioprine for at least 48 months from diagnosis. We conditionally recommend the use of plasma exchange in patients with severe renal vasculitis. For remission induction in EGPA, we recommend use of cyclophosphamide or rituximab in severe disease and mepolizumab or azathioprine or methotrexate or mycophenolate mofetil in non-severe disease.</div></div><div><h3>Conclusions</h3><div>These are the first ever Indian recommendations for the management of AAV. Despite our effort to formulate these recommendations based on high quality evidence, some recommendations were still based on low quality evidence but with high rate of agreement among expert panel members.</div></div>","PeriodicalId":8664,"journal":{"name":"Autoimmunity reviews","volume":"23 11","pages":"Article 103647"},"PeriodicalIF":9.2,"publicationDate":"2024-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142340218","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}