Pub Date : 2024-07-01DOI: 10.1016/j.autrev.2024.103544
Gut-tropic T cells primarily originate from gut-associated lymphoid tissue (GALT), and gut-tropic integrins mediate the trafficking of the T cells to the gastrointestinal tract, where their interplay with local hormones dictates the residence of the immune cells in both normal and compromised gastrointestinal tissues. Targeting gut-tropic integrins is an effective therapy for inflammatory bowel disease (IBD). Gut-tropic T cells are further capable of entering the peripheral circulatory system and relocating to multiple organs. There is mounting evidence indicating a correlation between gut-tropic T cells and extra-intestinal autoimmune disorders. This review aims to systematically discuss the origin, migration, and residence of gut-tropic T cells and their association with extra-intestinal autoimmune-related diseases. These discoveries are expected to offer new understandings into the development of a range of autoimmune disorders, as well as innovative approaches for preventing and treating the diseases.
肠道趋向性 T 细胞主要来源于肠道相关淋巴组织(GALT),肠道趋向性整合素介导 T 细胞向胃肠道的迁移,它们与当地激素的相互作用决定了免疫细胞在正常和受损胃肠组织中的驻留。以肠道趋向整合素为靶点是治疗炎症性肠病(IBD)的有效疗法。肠道趋向性 T 细胞还能进入外周循环系统并转移到多个器官。越来越多的证据表明,肠道T细胞与肠道外自身免疫性疾病之间存在关联。本综述旨在系统地讨论肠道T细胞的起源、迁移和驻留及其与肠道外自身免疫相关疾病的联系。这些发现有望为一系列自身免疫性疾病的发展提供新的认识,并为预防和治疗这些疾病提供创新方法。
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Pub Date : 2024-07-01DOI: 10.1016/j.autrev.2024.103592
{"title":"Are the new 2023 ACR/EULAR classification criteria suitable for advancing the knowledge of obstetric antiphospholipid syndrome?","authors":"","doi":"10.1016/j.autrev.2024.103592","DOIUrl":"10.1016/j.autrev.2024.103592","url":null,"abstract":"","PeriodicalId":8664,"journal":{"name":"Autoimmunity reviews","volume":null,"pages":null},"PeriodicalIF":9.2,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141911565","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}
Pub Date : 2024-07-01DOI: 10.1016/j.autrev.2024.103589
Introduction
An altered immune tolerance disturbed by immune checkpoint inhibitors (ICIs) may contribute to new-onset polymyalgia rheumatica (PMR) and giant cell arteritis (GCA). This systematic literature review (SLR) examines the characteristics of PMR and GCA-like syndromes following anticancer treatment with ICIs, summarizing their demographic, clinical and treatment-related features to provide insights whether they differ from the idiopathic forms.
Methods
The SLR was conducted in Medline and EMBASE databases from inception to July 2024, and in the EULAR/ACR abstract database (2021−2023). ICI-induced PMR and GCA syndromes were compared to the primary forms of the diseases using data from studies that included both groups as comparators. For manuscripts lacking direct comparisons, we summarized the main findings and discussed the differences using systematic reviews or large observational studies on the primary forms.
Results
From 1237 screened abstracts, 46 met the inclusion criteria, involving 358 patients (314 with ICI-PMR and 44 with ICI-GCA). ICI-PMR had an estimated pooled prevalence of 0.1% [95% CI: 0.07%, 0.14%] among ICI recipients and 15.9% [95% CI: 12.6%, 19.9%] among patients experiencing rheumatic immune-related adverse events.
Patients with ICI-PMR had a male-to-female ratio of 1.7:1 and a mean age of 71 ± 4 years. Most cases were associated with PD1/PDL1 blockers (87%). Clinical features included inflammatory pain in the girdles (100%), though pelvic girdle involvement was under-reported in some cases (3/28 studies). Peripheral arthritis was present in 35% of patients. Laboratory tests showed normal or slightly elevated inflammatory markers in 26% of cases. Glucocorticoids (GCs) led to symptom improvement in 84% of cases although 20% required immunosuppressive treatment and 14% experienced relapses.
ICI-GCA had a prevalence of 0.06% among ICI recipients, with equal gender distribution and a mean age of 71 ± 5 years. Most patients received anti-PD1/PDL1 blockers (57%). Clinical manifestations included cephalic symptoms (75%), permanent visual loss (23%) and symptoms related to large-vessel involvement (54%). High-dose GCs were effective, with 96% achieving remission, though 17% experienced relapses.
Conclusions
ICI-induced PMR and GCA may have distinct clinical profiles compared to idiopathic forms, with potentially milder symptoms and better treatment responses. Further studies are needed to confirm these findings and better understand the long-term outcomes and pathophysiology of these conditions.
{"title":"Polymyalgia rheumatica and giant cell arteritis induced by immune checkpoint inhibitors: A systematic literature review highlighting differences from the idiopathic forms","authors":"","doi":"10.1016/j.autrev.2024.103589","DOIUrl":"10.1016/j.autrev.2024.103589","url":null,"abstract":"<div><h3>Introduction</h3><p>An altered immune tolerance disturbed by immune checkpoint inhibitors (ICIs) may contribute to new-onset polymyalgia rheumatica (PMR) and giant cell arteritis (GCA). This systematic literature review (SLR) examines the characteristics of PMR and GCA-like syndromes following anticancer treatment with ICIs, summarizing their demographic, clinical and treatment-related features to provide insights whether they differ from the idiopathic forms.</p></div><div><h3>Methods</h3><p>The SLR was conducted in Medline and EMBASE databases from inception to July 2024, and in the EULAR/ACR abstract database (2021−2023). ICI-induced PMR and GCA syndromes were compared to the primary forms of the diseases using data from studies that included both groups as comparators. For manuscripts lacking direct comparisons, we summarized the main findings and discussed the differences using systematic reviews or large observational studies on the primary forms.</p></div><div><h3>Results</h3><p>From 1237 screened abstracts, 46 met the inclusion criteria, involving 358 patients (314 with ICI-PMR and 44 with ICI-GCA). ICI-PMR had an estimated pooled prevalence of 0.1% [95% CI: 0.07%, 0.14%] among ICI recipients and 15.9% [95% CI: 12.6%, 19.9%] among patients experiencing rheumatic immune-related adverse events.</p><p>Patients with ICI-PMR had a male-to-female ratio of 1.7:1 and a mean age of 71 ± 4 years. Most cases were associated with PD1/PDL1 blockers (87%). Clinical features included inflammatory pain in the girdles (100%), though pelvic girdle involvement was under-reported in some cases (3/28 studies). Peripheral arthritis was present in 35% of patients. Laboratory tests showed normal or slightly elevated inflammatory markers in 26% of cases. Glucocorticoids (GCs) led to symptom improvement in 84% of cases although 20% required immunosuppressive treatment and 14% experienced relapses.</p><p>ICI-GCA had a prevalence of 0.06% among ICI recipients, with equal gender distribution and a mean age of 71 ± 5 years. Most patients received anti-PD1/PDL1 blockers (57%). Clinical manifestations included cephalic symptoms (75%), permanent visual loss (23%) and symptoms related to large-vessel involvement (54%). High-dose GCs were effective, with 96% achieving remission, though 17% experienced relapses.</p></div><div><h3>Conclusions</h3><p>ICI-induced PMR and GCA may have distinct clinical profiles compared to idiopathic forms, with potentially milder symptoms and better treatment responses. Further studies are needed to confirm these findings and better understand the long-term outcomes and pathophysiology of these conditions.</p></div>","PeriodicalId":8664,"journal":{"name":"Autoimmunity reviews","volume":null,"pages":null},"PeriodicalIF":9.2,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141905776","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}
Pub Date : 2024-07-01DOI: 10.1016/j.autrev.2024.103591
Autoimmune hepatitis (AIH) is a severe hepatopathy characterized by hypergammaglobulinemia, presence of serum autoantibodies and histological appearance of interface hepatitis. Liver damage in AIH is initiated by the presentation of a liver autoantigen to uncommitted Th0 lymphocytes, followed by a cascade of effector immune responses culminating with the production of inflammatory cytokines, activation of cytotoxic cells and subsequent hepatocyte injury. B cells actively participate in AIH liver damage by presenting autoantigens to uncommitted T lymphocytes. B cells also undergo maturation into plasma cells that are responsible for production of immunoglobulin G and autoantibodies, which mediate antibody dependent cell cytotoxicity. Perpetuation of effector immunity with consequent progression of liver damage is permitted by impairment in regulatory T cells (Tregs), a lymphocyte subset central to the maintenance of immune homeostasis. Treg impairment in AIH is multifactorial, deriving from numerical decrease, reduced suppressive function, poor response to IL-2 and less stable phenotype. In this review, we discuss the role of B and T lymphocytes in the pathogenesis of AIH. Immunotherapeutic strategies that could limit inflammation and halt disease progression while reconstituting tolerance to liver autoantigens are also reviewed and discussed.
自身免疫性肝炎(AIH)是一种严重的肝病,以高丙种球蛋白血症、血清自身抗体和界面性肝炎组织学表现为特征。AIH 中的肝损伤是由肝脏自身抗原呈现给未就位的 Th0 淋巴细胞引起的,随后是一连串的效应免疫反应,最终产生炎症细胞因子、激活细胞毒性细胞并导致肝细胞损伤。B 细胞通过向未就位的 T 淋巴细胞展示自身抗原,积极参与 AIH 肝损伤。B 细胞还会成熟为浆细胞,负责产生免疫球蛋白 G 和自身抗体,从而介导抗体依赖性细胞毒性。调节性 T 细胞(Tregs)是维持免疫平衡的核心淋巴细胞亚群,其功能受损可导致效应免疫持续存在,进而加重肝损伤。AIH中Treg的损伤是多因素的,包括数量减少、抑制功能降低、对IL-2反应差和表型不稳定。在这篇综述中,我们将讨论 B 淋巴细胞和 T 淋巴细胞在 AIH 发病机制中的作用。我们还回顾并讨论了可限制炎症和阻止疾病进展,同时重建对肝脏自身抗原耐受性的免疫治疗策略。
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Pub Date : 2024-07-01DOI: 10.1016/j.autrev.2024.103600
The lungs are a principal factor in the increased morbidity and mortality observed in patients with Connective Tissue Disease (CTD), frequently presenting as CTD-associated Interstitial Lung Disease (ILD). Currently, there is a lack of comprehensive descriptions of the pulmonary cells implicated in the development of CTD-ILD. This review leverages the Human Lung Cell Atlas (HLCA) and spatial multi-omics atlases to discuss the advancements in research on the pathogenesis of CTD-ILD from a pulmonary cell perspective. This facilitates a more precise localization of disease sites and a more systematic consideration of disease progression, supporting further mechanistic studies and targeted therapies.
{"title":"Research Progress in pathogenesis of connective tissue disease-associated interstitial lung disease from the perspective of pulmonary cells","authors":"","doi":"10.1016/j.autrev.2024.103600","DOIUrl":"10.1016/j.autrev.2024.103600","url":null,"abstract":"<div><p>The lungs are a principal factor in the increased morbidity and mortality observed in patients with Connective Tissue Disease (CTD), frequently presenting as CTD-associated Interstitial Lung Disease (ILD). Currently, there is a lack of comprehensive descriptions of the pulmonary cells implicated in the development of CTD-ILD. This review leverages the Human Lung Cell Atlas (HLCA) and spatial multi-omics atlases to discuss the advancements in research on the pathogenesis of CTD-ILD from a pulmonary cell perspective. This facilitates a more precise localization of disease sites and a more systematic consideration of disease progression, supporting further mechanistic studies and targeted therapies.</p></div>","PeriodicalId":8664,"journal":{"name":"Autoimmunity reviews","volume":null,"pages":null},"PeriodicalIF":9.2,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141995205","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}
Pub Date : 2024-07-01DOI: 10.1016/j.autrev.2024.103603
A growing body of evidence supports an association between systemic autoimmune disease and exposure to amphibole asbestos, a form of asbestos typically with straight, stiff, needle-like fibers that are easily inhaled. While the bulk of this evidence comes from the population exposed occupationally and environmentally to Libby Amphibole (LA) due to the mining of contaminated vermiculite in Montana, studies from Italy and Australia are broadening the evidence to other sites of amphibole exposures. What these investigations have done, that most historical studies have not, is to evaluate amphibole asbestos separately from chrysotile, the most common commercial asbestos in the United States. Here we review the current and historical evidence summarizing amphibole asbestos exposure as a risk factor for autoimmune disease. In both mice and humans, amphibole asbestos, but not chrysotile, drives production of both antinuclear autoantibodies (ANA) associated with lupus-like pathologies and pathogenic autoantibodies against mesothelial cells that appear to contribute to a severe and progressive pleural fibrosis. A growing public health concern has emerged with revelations that a) unregulated asbestos minerals can be just as pathogenic as commercial (regulated) asbestos, and b) bedrock and soil occurrences of asbestos are far more widespread than previously thought. While occupational exposures may be decreasing, environmental exposures are on the rise for many reasons, including those due to the creation of windborne asbestos-containing dusts from urban development and climate change, making this topic an urgent challenge for public and heath provider education, health screening and environmental regulations.
{"title":"Amphibole asbestos as an environmental trigger for systemic autoimmune diseases","authors":"","doi":"10.1016/j.autrev.2024.103603","DOIUrl":"10.1016/j.autrev.2024.103603","url":null,"abstract":"<div><p>A growing body of evidence supports an association between systemic autoimmune disease and exposure to amphibole asbestos, a form of asbestos typically with straight, stiff, needle-like fibers that are easily inhaled. While the bulk of this evidence comes from the population exposed occupationally and environmentally to Libby Amphibole (LA) due to the mining of contaminated vermiculite in Montana, studies from Italy and Australia are broadening the evidence to other sites of amphibole exposures. What these investigations have done, that most historical studies have not, is to evaluate amphibole asbestos separately from chrysotile, the most common commercial asbestos in the United States. Here we review the current and historical evidence summarizing amphibole asbestos exposure as a risk factor for autoimmune disease. In both mice and humans, amphibole asbestos, but not chrysotile, drives production of both antinuclear autoantibodies (ANA) associated with lupus-like pathologies and pathogenic autoantibodies against mesothelial cells that appear to contribute to a severe and progressive pleural fibrosis. A growing public health concern has emerged with revelations that a) unregulated asbestos minerals can be just as pathogenic as commercial (regulated) asbestos, and b) bedrock and soil occurrences of asbestos are far more widespread than previously thought. While occupational exposures may be decreasing, environmental exposures are on the rise for many reasons, including those due to the creation of windborne asbestos-containing dusts from urban development and climate change, making this topic an urgent challenge for public and heath provider education, health screening and environmental regulations.</p></div>","PeriodicalId":8664,"journal":{"name":"Autoimmunity reviews","volume":null,"pages":null},"PeriodicalIF":9.2,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141999388","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}
Pub Date : 2024-07-01DOI: 10.1016/j.autrev.2024.103602
Skeletal muscle involvement is common in patients with small- and medium-sized vasculitis, particularly polyarteritis nodosa (PAN) and antineutrophil cytoplasmic antibody-associated vasculitis (AAV). Despite being not included in the standard classification criteria for PAN and AAV, skeletal muscle involvement is an important clinical indicator, particularly when vasculitic myopathy is the only pathological evidence in the absence of other organ involvement. Herein, we comprehensively reviewed and compared the clinical features of 71 and 135 patients with PAN and AAV, respectively, with skeletal muscle involvement at the time of disease onset. Most patients with PAN and AAV exhibited skeletal muscle involvement, often characterized by myalgia and occasional muscular weakness, predominantly in the lower extremities. Myalgia and weakness were observed more frequently in the distal lower extremities in patients with PAN than in those with AAV. In contrast, skeletal muscle involvement tended to exhibit a more dispersed distribution across all four extremities in those with AAV. Muscle magnetic resonance imaging T2-weighted and short-tau inversion recovery sequences can effectively identify hyperintense areas attributed to hypervascularity of affected muscle tissues and serve as a sensitive and useful modality for visually determining the suitable biopsy site. >90% of patients with PAN and AAV demonstrated perivascular inflammation in their affected muscle tissues, whereas fibrinoid necrosis of the vessel walls was reported in two-thirds of patients. Serum creatine kinase (CK) levels were within the normal range in approximately 80% of patients presenting with skeletal muscle involvement in PAN and AAV. Furthermore, muscle fiber damage was milder in patients with skeletal muscle involvement in PAN and AAV than those with idiopathic inflammatory myositis. Meanwhile, serum CK levels were elevated in 65–85% of patients with PAN and AAV who had myofiber necrosis and degeneration in the affected muscles. Most patients with PAN and AAV showed improvement in skeletal muscle involvement following glucocorticoids (GCs) administration; however, relapse was observed in some patients during the tapering of GCs. In summary, skeletal muscle involvement is a potential indicator for establishing PAN and AAV diagnoses during the early phases of the disease.
骨骼肌受累常见于中小型血管炎患者,尤其是结节性多动脉炎(PAN)和抗中性粒细胞胞浆抗体相关性血管炎(AAV)。尽管骨骼肌受累未被纳入 PAN 和 AAV 的标准分类标准,但骨骼肌受累是一项重要的临床指标,尤其是在没有其他器官受累的情况下,血管性肌病是唯一的病理证据。在此,我们分别对 71 例和 135 例 PAN 和 AAV 患者发病时骨骼肌受累的临床特征进行了全面回顾和比较。大多数 PAN 和 AAV 患者表现为骨骼肌受累,通常以肌痛和偶尔的肌无力为特征,主要表现在下肢。与 AAV 患者相比,PAN 患者下肢远端的肌痛和无力更为常见。与此相反,AAV 患者的骨骼肌受累往往更分散地分布在四肢。肌肉磁共振成像 T2 加权和短陶反转恢复序列可有效识别受累肌肉组织血管过多导致的高密度区,是一种敏感而有用的方法,可用于直观地确定合适的活检部位。>90%以上的 PAN 和 AAV 患者受累肌肉组织的血管周围出现炎症,三分之二的患者血管壁出现纤维素性坏死。约80%的PAN和AAV骨骼肌受累患者的血清肌酸激酶(CK)水平在正常范围内。此外,与特发性炎症性肌炎相比,PAN 和 AAV 骨骼肌受累患者的肌纤维损伤程度较轻。同时,65%-85%的 PAN 和 AAV 患者血清 CK 水平升高,这些患者受累肌肉的肌纤维坏死和变性。大多数 PAN 和 AAV 患者在服用糖皮质激素(GCs)后,骨骼肌受累的情况有所改善;但也有一些患者在减量服用 GCs 期间病情复发。总之,骨骼肌受累是在疾病早期阶段确定 PAN 和 AAV 诊断的一个潜在指标。
{"title":"Early skeletal muscle manifestations in polyarteritis nodosa and ANCA-associated vasculitis","authors":"","doi":"10.1016/j.autrev.2024.103602","DOIUrl":"10.1016/j.autrev.2024.103602","url":null,"abstract":"<div><p>Skeletal muscle involvement is common in patients with small- and medium-sized vasculitis, particularly polyarteritis nodosa (PAN) and antineutrophil cytoplasmic antibody-associated vasculitis (AAV). Despite being not included in the standard classification criteria for PAN and AAV, skeletal muscle involvement is an important clinical indicator, particularly when vasculitic myopathy is the only pathological evidence in the absence of other organ involvement. Herein, we comprehensively reviewed and compared the clinical features of 71 and 135 patients with PAN and AAV, respectively, with skeletal muscle involvement at the time of disease onset. Most patients with PAN and AAV exhibited skeletal muscle involvement, often characterized by myalgia and occasional muscular weakness, predominantly in the lower extremities. Myalgia and weakness were observed more frequently in the distal lower extremities in patients with PAN than in those with AAV. In contrast, skeletal muscle involvement tended to exhibit a more dispersed distribution across all four extremities in those with AAV. Muscle magnetic resonance imaging T2-weighted and short-tau inversion recovery sequences can effectively identify hyperintense areas attributed to hypervascularity of affected muscle tissues and serve as a sensitive and useful modality for visually determining the suitable biopsy site. >90% of patients with PAN and AAV demonstrated perivascular inflammation in their affected muscle tissues, whereas fibrinoid necrosis of the vessel walls was reported in two-thirds of patients. Serum creatine kinase (CK) levels were within the normal range in approximately 80% of patients presenting with skeletal muscle involvement in PAN and AAV. Furthermore, muscle fiber damage was milder in patients with skeletal muscle involvement in PAN and AAV than those with idiopathic inflammatory myositis. Meanwhile, serum CK levels were elevated in 65–85% of patients with PAN and AAV who had myofiber necrosis and degeneration in the affected muscles. Most patients with PAN and AAV showed improvement in skeletal muscle involvement following glucocorticoids (GCs) administration; however, relapse was observed in some patients during the tapering of GCs. In summary, skeletal muscle involvement is a potential indicator for establishing PAN and AAV diagnoses during the early phases of the disease.</p></div>","PeriodicalId":8664,"journal":{"name":"Autoimmunity reviews","volume":null,"pages":null},"PeriodicalIF":9.2,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141995204","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}
Pub Date : 2024-07-01DOI: 10.1016/j.autrev.2024.103541
The role of infections in the pathogenesis of autoimmune diseases has long been recognized and reported. In addition to infectious agents, the internal composition of the “friendly” living bacteria, (microbiome) and its correlation to immune balance and dysregulation have drawn the attention of researchers for decades. Nevertheless, only recently, scientific papers regarding the potential role of transferring microbiome from healthy donor subjects to patients with autoimmune diseases has been proposed. Fecal microbiota transplantation or FMT, carries the logic of transferring microorganisms responsible for immune balance from healthy donors to individuals with immune dysregulation or more accurately for our paper, autoimmune diseases. Viewing the microbiome as a pathogenetic player allows us to consider FMT as a pathogenetic-based treatment. Promising results alongside improved outcomes have been demonstrated in patients with different autoimmune diseases following FMT. Therefore, in our current extensive review, we aimed to highlight the implication of FMT in various autoimmune diseases, such as inflammatory bowel disease, autoimmune thyroid and liver diseases, systemic lupus erythematosus, and type 1 diabetes mellitus, among others. Presenting all the aspects of FMT in more than 12 autoimmune diseases in one paper, to the best of our knowledge, is the first time presented in medical literature. Viewing FMT as such could contribute to better understanding and newer application of the model in the therapy of autoimmune diseases, indeed.
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Pub Date : 2024-07-01DOI: 10.1016/j.autrev.2024.103588
Cystoid macular edema (CME) is considered a rare adverse effect of rituximab use, with only a limited number of cases published in the literature. Although its etiopathogenesis is still unknown, its mechanism seems to be related to a transient elevation of cytokines after rituximab infusion resulting in an increased permeability of retinal vessels.
We report the first case of rituximab-induced CME in a patient with systemic lupus erythematosus (SLE), where rituximab was used to treat hematological complications. A month after the 2nd infusion, the patient developed blurred vision and decreased visual acuity in the right eye. An optic coherence tomography (OCT) was performed, being diagnosed with CME. Rituximab was then discontinued, exhibiting a complete resolution of the condition within 4 weeks. The aim of our work is to report the first case in a patient with SLE and also carry out a brief review of the subject comparing it to all previously published cases.
{"title":"Rituximab-induced cystoid macular edema in a patient with systemic lupus erythematosus: A case report and literature review","authors":"","doi":"10.1016/j.autrev.2024.103588","DOIUrl":"10.1016/j.autrev.2024.103588","url":null,"abstract":"<div><p>Cystoid macular edema (CME) is considered a rare adverse effect of rituximab use, with only a limited number of cases published in the literature. Although its etiopathogenesis is still unknown, its mechanism seems to be related to a transient elevation of cytokines after rituximab infusion resulting in an increased permeability of retinal vessels.</p><p>We report the first case of rituximab-induced CME in a patient with systemic lupus erythematosus (SLE), where rituximab was used to treat hematological complications. A month after the 2nd infusion, the patient developed blurred vision and decreased visual acuity in the right eye. An optic coherence tomography (OCT) was performed, being diagnosed with CME. Rituximab was then discontinued, exhibiting a complete resolution of the condition within 4 weeks. The aim of our work is to report the first case in a patient with SLE and also carry out a brief review of the subject comparing it to all previously published cases.</p></div>","PeriodicalId":8664,"journal":{"name":"Autoimmunity reviews","volume":null,"pages":null},"PeriodicalIF":9.2,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141916002","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}
Pub Date : 2024-07-01DOI: 10.1016/j.autrev.2024.103520
Background
Undifferentiated autoinflammatory diseases are characterized by recurrent or persistent fever, usually combined with other inflammatory manifestations, and negative or inconclusive genetic studies for monogenic autoinflammatory disorders.
Aims
To define and characterize disease phenotypes in adult patients diagnosed in an adult reference center with undifferentiated autoinflammatory diseases, and to analyze the efficacy of the drugs used in order to provide practical diagnostic and therapeutic recommendations.
Methods
Retrospective study (2015–2022) of patients with undifferentiated autoinflammatory diseases among all patients visited in our reference center. Demographic, clinical, laboratory features and detailed therapeutic information was collected.
Results
Of the 334 patients with a suspected autoinflammatory disease, 134 (40%) patients (61% women) were initially diagnosed with undifferentiated autoinflammatory diseases. Mean age at disease onset and at diagnosis was 28.7 and 37.7 years, respectively. In 90 (67.2%) patients, symptoms started during adulthood. Forty-four (32.8%) patients met diagnostic/classification criteria for adult periodic fever with aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome. In the remaining patients, four additional phenotypes were differentiated according to the predominant manifestations: a) Predominantly fever phenotype (n = 18; 13.4%); b) Predominantly abdominal/pleuritic pain phenotype (n = 9; 6.7%); c) Predominantly pericarditis phenotype (n = 18; 13.4%), and d) Complex syndrome phenotype (n = 45; 33.6%). Prednisone (mainly on demand), colchicine and anakinra were the drugs commonly used. Overall, complete responses were achieved with prednisone in 41.3%, colchicine in 40.2%, and anakinra in 58.3% of patients in whom they were used. By phenotypes, prednisone on demand was more effective in adult PFAPA syndrome and colchicine in patients with the abdominal/pleuritic pain pattern and PFAPA syndrome. Patients with complex syndrome achieved complete responses with prednisone (21.9%), colchicine (25.7%) and anakinra (44.4%), and were the group more often requiring additional immunosuppressive drugs.
Conclusions
The analysis of the largest single-center series of adult patients with undifferentiated autoinflammatory diseases identified and characterized different disease phenotypes and their therapeutic approaches. This study is expected to contribute to increase the awareness of physicians for an early identification of these conditions, and to provide the best known therapeutic options.
{"title":"Disease phenotypes in adult patients with suspected undifferentiated autoinflammatory diseases and PFAPA syndrome: Clinical and therapeutic implications","authors":"","doi":"10.1016/j.autrev.2024.103520","DOIUrl":"10.1016/j.autrev.2024.103520","url":null,"abstract":"<div><h3>Background</h3><p>Undifferentiated autoinflammatory diseases are characterized by recurrent or persistent fever, usually combined with other inflammatory manifestations, and negative or inconclusive genetic studies for monogenic autoinflammatory disorders.</p></div><div><h3>Aims</h3><p>To define and characterize disease phenotypes in adult patients diagnosed in an adult reference center with undifferentiated autoinflammatory diseases, and to analyze the efficacy of the drugs used in order to provide practical diagnostic and therapeutic recommendations.</p></div><div><h3>Methods</h3><p>Retrospective study (2015–2022) of patients with undifferentiated autoinflammatory diseases among all patients visited in our reference center. Demographic, clinical, laboratory features and detailed therapeutic information was collected.</p></div><div><h3>Results</h3><p>Of the 334 patients with a suspected autoinflammatory disease, 134 (40%) patients (61% women) were initially diagnosed with undifferentiated autoinflammatory diseases. Mean age at disease onset and at diagnosis was 28.7 and 37.7 years, respectively. In 90 (67.2%) patients, symptoms started during adulthood. Forty-four (32.8%) patients met diagnostic/classification criteria for adult periodic fever with aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome. In the remaining patients, four additional phenotypes were differentiated according to the predominant manifestations: a) Predominantly fever phenotype (<em>n</em> = 18; 13.4%); b) Predominantly abdominal/pleuritic pain phenotype (<em>n</em> = 9; 6.7%); c) Predominantly pericarditis phenotype (n = 18; 13.4%), and d) Complex syndrome phenotype (<em>n</em> = 45; 33.6%). Prednisone (mainly on demand), colchicine and anakinra were the drugs commonly used. Overall, complete responses were achieved with prednisone in 41.3%, colchicine in 40.2%, and anakinra in 58.3% of patients in whom they were used. By phenotypes, prednisone on demand was more effective in adult PFAPA syndrome and colchicine in patients with the abdominal/pleuritic pain pattern and PFAPA syndrome. Patients with complex syndrome achieved complete responses with prednisone (21.9%), colchicine (25.7%) and anakinra (44.4%), and were the group more often requiring additional immunosuppressive drugs.</p></div><div><h3>Conclusions</h3><p>The analysis of the largest single-center series of adult patients with undifferentiated autoinflammatory diseases identified and characterized different disease phenotypes and their therapeutic approaches. This study is expected to contribute to increase the awareness of physicians for an early identification of these conditions, and to provide the best known therapeutic options.</p></div>","PeriodicalId":8664,"journal":{"name":"Autoimmunity reviews","volume":null,"pages":null},"PeriodicalIF":9.2,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S1568997224000077/pdfft?md5=fc590fae74c4fd13c9b544cd7993f7f9&pid=1-s2.0-S1568997224000077-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140334585","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}