Journal of autoimmunity最新文献

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Advances in chimeric antigen receptor T cell therapy for autoimmune and autoinflammatory diseases and their complications.
IF 7.9 1区 医学
Journal of autoimmunity Pub Date : 2024-12-18 DOI: 10.1016/j.jaut.2024.103350
Liuting Zeng, Yan Li, Wang Xiang, Wei Xiao, Zhiyong Long, Lingyun Sun
{"title":"Advances in chimeric antigen receptor T cell therapy for autoimmune and autoinflammatory diseases and their complications.","authors":"Liuting Zeng, Yan Li, Wang Xiang, Wei Xiao, Zhiyong Long, Lingyun Sun","doi":"10.1016/j.jaut.2024.103350","DOIUrl":"https://doi.org/10.1016/j.jaut.2024.103350","url":null,"abstract":"<p><p>Chimeric antigen receptor T (CAR-T) cells are genetically engineered T cells expressing transmembrane chimeric antigen receptors with specific targeting abilities. As an emerging immunotherapy, the use of CAR-T cells has made significant breakthroughs in cancer treatment, particularly for hematological malignancies. The success of CAR-T cell therapy in blood cancers highlights its potential for other conditions in which the clearance of pathological cells is therapeutic, such as liver diseases, infectious diseases, heart failure, and diabetes. Given the limitations of current therapies for autoimmune diseases, researchers have actively explored the potential therapeutic value of CAR-T cells and their derivatives in the field of autoimmune diseases. This review focuses on the research progress and current challenges of CAR-T cells in autoimmune diseases with the aim of providing a theoretical basis for the precise treatment of autoimmune diseases. In the future, CAR-T cells may present new therapeutic modalities and ultimately provide hope for patients with autoimmune diseases.</p>","PeriodicalId":15245,"journal":{"name":"Journal of autoimmunity","volume":"150 ","pages":"103350"},"PeriodicalIF":7.9,"publicationDate":"2024-12-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142864279","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}
引用次数: 0
AIM2 deficiency in CD4+ T cells promotes psoriasis-like inflammation by regulating Th17-Treg axis via AIM2-IKZF2 pathway.
IF 7.9 1区 医学
Journal of autoimmunity Pub Date : 2024-12-16 DOI: 10.1016/j.jaut.2024.103351
Yue Xin, Ming Yang, Zhidan Zhao, Zhenghao He, Yang Mei, Feng Xiong, Fen Tan, Anqun Chen, Christopher Chang, Helong Dai, Haijing Wu, Qianjin Lu
{"title":"AIM2 deficiency in CD4<sup>+</sup> T cells promotes psoriasis-like inflammation by regulating Th17-Treg axis via AIM2-IKZF2 pathway.","authors":"Yue Xin, Ming Yang, Zhidan Zhao, Zhenghao He, Yang Mei, Feng Xiong, Fen Tan, Anqun Chen, Christopher Chang, Helong Dai, Haijing Wu, Qianjin Lu","doi":"10.1016/j.jaut.2024.103351","DOIUrl":"https://doi.org/10.1016/j.jaut.2024.103351","url":null,"abstract":"<p><p>Psoriasis vulgaris remains a common inflammatory skin disease globally. The imbalance between Th17 and Treg cells plays an integral role in the pathogenesis of psoriasis vulgaris, but the underlying mechanisms remain obscure. The role of AIM2 in Treg cell function in psoriasis is unclear. We found that AIM2 expression is upregulated in peripheral blood and skin lesions from patients with psoriasis vulgaris when compared with healthy controls. In a psoriasis-like mouse model, CD4<sup>cre</sup>Aim2<sup>fl/fl</sup> mice showed aggravated psoriatic symptoms, increased Th17 cell and decreased Treg cell numbers in spleens and dLNs compared to Aim2<sup>fl/fl</sup> mice. The loss of AIM2 in naïve CD4<sup>+</sup> T cells promotes Th17 cell differentiation and decreases Treg cell numbers in vitro. Further, IKZF2 was identified as a downstream regulator of AIM2 through RNAseq analysis. AIM2 deficiency in CD4<sup>+</sup> T cells downregulated IKZF2 mRNA expression. Silencing IKZF2 in naïve CD4<sup>+</sup> T cells led to a significant increase in the expression of RORc and diminished FOXP3 expression. In summary, AIM2 may regulate the differentiation of Th17 and Treg cell by affecting IKZF2. Our findings might shed light on the pathogenesis of psoriasis and provide potential therapeutic targets for patients with psoriasis.</p>","PeriodicalId":15245,"journal":{"name":"Journal of autoimmunity","volume":"150 ","pages":"103351"},"PeriodicalIF":7.9,"publicationDate":"2024-12-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142846777","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}
引用次数: 0
External and internal exposome as triggers of biological signalling in systemic sclerosis - A narrative synthesis.
IF 7.9 1区 医学
Journal of autoimmunity Pub Date : 2024-12-05 DOI: 10.1016/j.jaut.2024.103342
Lisa Mf Janssen, Frauke Lemaire, Chiara Longo Sanchez-Calero, François Huaux, Steven Ronsmans, Peter Hm Hoet, Manosij Ghosh
{"title":"External and internal exposome as triggers of biological signalling in systemic sclerosis - A narrative synthesis.","authors":"Lisa Mf Janssen, Frauke Lemaire, Chiara Longo Sanchez-Calero, François Huaux, Steven Ronsmans, Peter Hm Hoet, Manosij Ghosh","doi":"10.1016/j.jaut.2024.103342","DOIUrl":"https://doi.org/10.1016/j.jaut.2024.103342","url":null,"abstract":"<p><p>Systemic sclerosis (SSc) is an autoimmune chronic connective tissue disorder with a complex pathogenesis and a strong gene-environment interaction. Despite the low prevalence of SSc, with around 100-250 cases per million, the morbidity and mortality are high and disproportionately affecting women. In this context, we review the influence of the external and internal exposome on the \"immunome\" in SSc. While several studies have addressed aspects of exposure-induced autoimmunity in general, very few have focused on SSc-specific phenotypes. In epidemiological studies, targeted characterization of the external exposome component in relation to SSc has often been limited to a single exposure. Despite the selective characterization of exposure, such studies play an important role in providing evidence that can be used towards reduction of exposure of modifiable factors, and can lead to proper management and prevention of SSc. Additionally, there is an effort towards integration of external exposome data with health data (health records, medical imaging, diagnostic results, etc.), to significantly improve our understanding of the environmental and occupational causes of SSc. A limited number of studies have identified biological processes related to the vascular homeostasis, fibrotic processes and the immune system. The key findings of the current review show advances in our understanding of the SSc disease phenotype and associated biomarkers in relation to specific pathophysiological features, however most often such studies are not supplemented with external exposome data.</p>","PeriodicalId":15245,"journal":{"name":"Journal of autoimmunity","volume":"150 ","pages":"103342"},"PeriodicalIF":7.9,"publicationDate":"2024-12-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142791803","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}
引用次数: 0
Circulating T cells in sarcoidosis have an aberrantly activated phenotype that correlates with disease outcome. 结节病的循环T细胞具有异常活化表型,与疾病结果相关。
IF 7.9 1区 医学
Journal of autoimmunity Pub Date : 2024-12-01 Epub Date: 2023-10-18 DOI: 10.1016/j.jaut.2023.103120
Jelle R Miedema, Lieke J de Jong, Denise van Uden, Ingrid M Bergen, Mirjam Kool, Caroline E Broos, Vivienne Kahlmann, Marlies S Wijsenbeek, Rudi W Hendriks, Odilia B J Corneth
{"title":"Circulating T cells in sarcoidosis have an aberrantly activated phenotype that correlates with disease outcome.","authors":"Jelle R Miedema, Lieke J de Jong, Denise van Uden, Ingrid M Bergen, Mirjam Kool, Caroline E Broos, Vivienne Kahlmann, Marlies S Wijsenbeek, Rudi W Hendriks, Odilia B J Corneth","doi":"10.1016/j.jaut.2023.103120","DOIUrl":"10.1016/j.jaut.2023.103120","url":null,"abstract":"<p><strong>Rationale: </strong>Disease course in sarcoidosis is highly variable. Bronchoalveolar lavage fluid and mediastinal lymph nodes show accumulation of activated T cells with a T-helper (Th)17.1 signature, which correlates with non-resolving sarcoidosis. We hypothesize that the peripheral blood (PB) T cell phenotype may correlate with outcome.</p><p><strong>Objectives: </strong>To compare frequencies, phenotypes and function of circulating T cell populations in sarcoidosis patients with healthy controls (HCs) and correlate these parameters with outcome.</p><p><strong>Methods: </strong>We used multi-color flow cytometry to quantify activation marker expression on PB T cell subsets in treatment-naïve patients and HCs. The disease course was determined after 2-year follow-up. Cytokine production was measured after T cell stimulation in vitro.</p><p><strong>Measurements and main results: </strong>We observed significant differences between patients and HCs in several T cell populations, including CD8<sup>+</sup> and CD4<sup>+</sup> T cells, Th1/Th17 subsets, CD4<sup>+</sup> T memory stem cells, regulatory T cells (Tregs) and γδ T cells. Decreased frequencies of CD4<sup>+</sup> T cells and increased frequencies of Tregs and CD8<sup>+</sup> γδ T cells correlated with worse outcome. Naïve CD4<sup>+</sup> T cells displayed an activated phenotype with increased CD25 expression in patients with active chronic disease at 2-year follow-up. A distinctive Treg phenotype with increased expression of CD25, CTLA4, CD69, PD-1 and CD95 correlated with chronic sarcoidosis. Upon stimulation, both naïve and memory T cells displayed a different cytokine profile in sarcoidosis compared to HCs.</p><p><strong>Conclusions: </strong>Circulating T cell subpopulations of sarcoidosis patients display phenotypic abnormalities that correlate with disease outcome, supporting a critical role of aberrant T cell activation in sarcoidosis pathogenesis.</p>","PeriodicalId":15245,"journal":{"name":"Journal of autoimmunity","volume":" ","pages":"103120"},"PeriodicalIF":7.9,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49677871","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}
引用次数: 0
Comparison of circulating and excreted metals and of autoimmunity between two Great Plains Tribal communities. 两个大平原部落群落之间循环和排泄金属以及自身免疫的比较。
IF 7.9 1区 医学
Journal of autoimmunity Pub Date : 2024-12-01 Epub Date: 2023-10-07 DOI: 10.1016/j.jaut.2023.103117
Esther Erdei, Elena R O'Donald, Li Luo, Kendra Enright, Marcia O'Leary, Debra MacKenzie, John Doyle, Margaret Eggers, Deborah Keil, Johnnye Lewis, Jeffrey A Henderson, Robert L Rubin
{"title":"Comparison of circulating and excreted metals and of autoimmunity between two Great Plains Tribal communities.","authors":"Esther Erdei, Elena R O'Donald, Li Luo, Kendra Enright, Marcia O'Leary, Debra MacKenzie, John Doyle, Margaret Eggers, Deborah Keil, Johnnye Lewis, Jeffrey A Henderson, Robert L Rubin","doi":"10.1016/j.jaut.2023.103117","DOIUrl":"10.1016/j.jaut.2023.103117","url":null,"abstract":"<p><p>Metals contaminants of the environment from mine waste have been implicated as contributing agents in autoimmune disease. The current study compares metals and autoimmunity in two Tribal communities residing in the Black Hills and the Bighorn Mountains geographical regions that are scattered with extant hard rock mines. With documented drinking water contamination in both communities, in vivo levels of more than half of the measured serum and urine metals differed between the two communities and were substantially different from their national median values. Serum autoantibodies associated with systemic autoimmune disease were rare or at low-level, but antibodies to denatured (single-stranded) DNA and thyroid-specific autoantibodies were commonly elevated, especially in women. A three-tier statistical modeling process was carried out to examine individual metals exposure as predictors of autoantibody levels. For the most part only weak positive associations between individual metals and systemic autoantibodies were found, although univariate quantile regression analysis showed positive statistical associations of serum lead and antimony with anti-chromatin and anti-histone autoantibodies. Using age and gender-adjusted multivariable statistical models, metals did not predict anti-thyroglobulin or -thyroid peroxidase significantly and metals were generally negative predictors of the other autoantibodies. Overall these results suggest that elevated levels of environmental metals and metalloids in these communities may result in suppression of autoantibodies associated with systemic autoimmune disease.</p>","PeriodicalId":15245,"journal":{"name":"Journal of autoimmunity","volume":" ","pages":"103117"},"PeriodicalIF":7.9,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10998922/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41182635","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}
引用次数: 0
Infectious and non-infectious precipitants of sarcoidosis. 肉样瘤病的感染性和非感染性诱因。
IF 7.9 1区 医学
Journal of autoimmunity Pub Date : 2024-12-01 Epub Date: 2024-05-31 DOI: 10.1016/j.jaut.2024.103239
Ozioma S Chioma, ZaDarreyal Wiggins, Samantha Rea, Wonder P Drake
{"title":"Infectious and non-infectious precipitants of sarcoidosis.","authors":"Ozioma S Chioma, ZaDarreyal Wiggins, Samantha Rea, Wonder P Drake","doi":"10.1016/j.jaut.2024.103239","DOIUrl":"10.1016/j.jaut.2024.103239","url":null,"abstract":"<p><p>Sarcoidosis is a chronic inflammatory disease that can affect any organ in the body. Its exact cause remains unknown, but it is believed to result from a combination of genetic and environmental factors. Some potential causes of sarcoidosis include genetics, environmental triggers, immune system dysfunction, the gut microbiome, sex, and race/ethnicity. Genetic mutations are associated with protection against disease progression or an increased susceptibility to more severe disease, while exposure to certain chemicals, bacteria, viruses, or allergens can trigger the formation of immune cell congregations (granulomas) in different organs. Dysfunction of the immune system, including autoimmune reactions, may also contribute. The gut microbiome and factors such as being female or having African American, Scandinavian, Irish, or Puerto Rican heritage are additional contributors to disease outcome. Recent research has suggested that certain drugs, such as anti-Programmed Death-1 (PD-1) and antibiotics such as tuberculosis (TB) drugs, may raise the risk of developing sarcoidosis. Hormone levels, particularly higher levels of estrogen and progesterone in women, have also been linked to an increased likelihood of sarcoidosis. The diagnosis of sarcoidosis involves a comprehensive assessment that includes medical history, physical examination, laboratory tests, and imaging studies. While there is no cure for sarcoidosis, the symptoms can often be effectively managed through various treatment options. Treatment may involve the use of medications, surgical interventions, or lifestyle changes. These disparate factors suggests that sarcoidosis has multiple positive and negative exacerbants on disease severity, some of which can be ameliorated and others which cannot.</p>","PeriodicalId":15245,"journal":{"name":"Journal of autoimmunity","volume":" ","pages":"103239"},"PeriodicalIF":7.9,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11607178/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141183717","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}
引用次数: 0
Lung transplantation in pulmonary sarcoidosis. 肺结节病的肺移植。
IF 7.9 1区 医学
Journal of autoimmunity Pub Date : 2024-12-01 Epub Date: 2023-11-03 DOI: 10.1016/j.jaut.2023.103135
Jin Sun Kim, Rohit Gupta
{"title":"Lung transplantation in pulmonary sarcoidosis.","authors":"Jin Sun Kim, Rohit Gupta","doi":"10.1016/j.jaut.2023.103135","DOIUrl":"10.1016/j.jaut.2023.103135","url":null,"abstract":"<p><p>Sarcoidosis is a systemic inflammatory disease of unknown etiology and variable clinical course. Pulmonary sarcoidosis is the most common presentation and accounts for most morbidity and mortality related to sarcoidosis. While sarcoidosis generally has good outcomes, few patients experience chronic disease. A minority of patients progress to a specific phenotype of sarcoidosis referred to advanced pulmonary sarcoidosis (APS) which includes advanced fibrosis, pulmonary hypertension and respiratory failure, leading to high morbidity and mortality. In patients with advanced disease despite medical therapy, lung transplantation may be the last viable option for improvement in quality of life. Though post-transplant survival is similar to that of other end-stage lung diseases, it is imperative that patients are evaluated and referred early to transplant centers with experience in APS. A multidisciplinary approach and clinical experience are crucial in detecting the optimal timing of referral, initiating comprehensive transplantation evaluation and listing, discussing surgical approach, and managing perioperative and post-transplant care. This review article seeks to address these aspects of lung transplantation in APS.</p>","PeriodicalId":15245,"journal":{"name":"Journal of autoimmunity","volume":" ","pages":"103135"},"PeriodicalIF":7.9,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"71481939","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}
引用次数: 0
Extrapulmonary sarcoidosis. 肺外肉样瘤病
IF 7.9 1区 医学
Journal of autoimmunity Pub Date : 2024-12-01 Epub Date: 2024-10-05 DOI: 10.1016/j.jaut.2024.103323
Paolo Spagnolo, Vasileios Kouranos, Victoria Singh-Curry, Thomas El Jammal, Misha Rosenbach
{"title":"Extrapulmonary sarcoidosis.","authors":"Paolo Spagnolo, Vasileios Kouranos, Victoria Singh-Curry, Thomas El Jammal, Misha Rosenbach","doi":"10.1016/j.jaut.2024.103323","DOIUrl":"10.1016/j.jaut.2024.103323","url":null,"abstract":"<p><p>Sarcoidosis is a chronic disease of unknown origin that develops when a genetically susceptible host is exposed to an antigen, leading to an exuberant immune response characterized by granulomatous inflammation. Although lung involvement is almost universal as well as the leading cause of morbidity and mortality, virtually any organ can be affected. In particular, sarcoidosis of the heart, nervous system, and eyes can be devastating, leading to death, debilitation and blindness, and a multidisciplinary approach involving expert specialists is required for prompt diagnosis and appropriate treatment. Sarcoidosis of the skin can be disfiguring, thus posing a substantial psychologic and social impact on the patients. The diagnosis is often straightforward in the presence of compatible clinical manifestations in patients with biopsy-proven sarcoidosis, but is challenging when extrapulmonary signs/symptoms occur in isolation. Corticosteroids remain the first line therapy, with immunosuppressive or biologic agents being reserved to patients failing or experiencing side effects from steroids or developing refractory disease.</p>","PeriodicalId":15245,"journal":{"name":"Journal of autoimmunity","volume":" ","pages":"103323"},"PeriodicalIF":7.9,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142380945","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}
引用次数: 0
A framework for exclusion of alternative diagnoses in sarcoidosis. 排除肉样瘤病替代诊断的框架。
IF 7.9 1区 医学
Journal of autoimmunity Pub Date : 2024-12-01 Epub Date: 2024-07-31 DOI: 10.1016/j.jaut.2024.103288
Logan J Harper, Carol F Farver, Ruchi Yadav, Daniel A Culver
{"title":"A framework for exclusion of alternative diagnoses in sarcoidosis.","authors":"Logan J Harper, Carol F Farver, Ruchi Yadav, Daniel A Culver","doi":"10.1016/j.jaut.2024.103288","DOIUrl":"10.1016/j.jaut.2024.103288","url":null,"abstract":"<p><p>Sarcoidosis is a multisystem granulomatous syndrome that arises from a persistent immune response to a triggering antigen(s). There is no \"gold standard\" test or algorithm for the diagnosis of sarcoidosis, making the diagnosis one of exclusion. The presentation of the disease varies substantially between individuals, in both the number of organs involved, and the manifestations seen in individual organs. These qualities dictate that health care providers diagnosing sarcoidosis must consider a wide range of possible alternative diagnoses, from across a range of presentations and medical specialties (infectious, inflammatory, cardiac, neurologic). Current guideline-based diagnosis of sarcoidosis recommends fulfillment of three criteria: 1) compatible clinical presentation and/or imaging 2) demonstration of granulomatous inflammation by biopsy (when possible) and, 3) exclusion of alternative causes, but do not provide guidance on standardized strategies for exclusion of alternative diagnoses. In this review, we provide a summary of the most common differential diagnoses for sarcoidosis involvement of lung, eye, skin, central nervous system, heart, liver, and kidney. We then propose a framework for testing to exclude alternative diagnoses based on pretest probability of sarcoidosis, defined as high (typical findings with sarcoidosis involvement confirmed in another organ), moderate (typical findings in a single organ), or low (atypical/findings suggesting of an alternative diagnosis). This work highlights the need for informed and careful exclusion of alternative diagnoses in sarcoidosis.</p>","PeriodicalId":15245,"journal":{"name":"Journal of autoimmunity","volume":" ","pages":"103288"},"PeriodicalIF":7.9,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141859866","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}
引用次数: 0
Pulmonary sarcoidosis: A comprehensive review: Past to present. 肺结节病:一篇综合综述:从过去到现在。
IF 7.9 1区 医学
Journal of autoimmunity Pub Date : 2024-12-01 Epub Date: 2023-10-19 DOI: 10.1016/j.jaut.2023.103107
John A Belperio, Michael C Fishbein, Fereidoun Abtin, Jessica Channick, Shailesh A Balasubramanian, Joseph P Lynch Iii
{"title":"Pulmonary sarcoidosis: A comprehensive review: Past to present.","authors":"John A Belperio, Michael C Fishbein, Fereidoun Abtin, Jessica Channick, Shailesh A Balasubramanian, Joseph P Lynch Iii","doi":"10.1016/j.jaut.2023.103107","DOIUrl":"10.1016/j.jaut.2023.103107","url":null,"abstract":"&lt;p&gt;&lt;p&gt;Sarcoidosis is a sterile non-necrotizing granulomatous disease without known causes that can involve multiple organs with a predilection for the lung and thoracic lymph nodes. Worldwide it is estimated to affect 2-160/100,000 people and has a mortality rate over 5 years of approximately 7%. For sarcoidosis patients, the cause of death is due to sarcoid in 60% of the cases, of which up to 80% are from advanced cardiopulmonary failure (pulmonary hypertension and respiratory microbial infections) in all races except in Japan were greater than 70% of the sarcoidosis deaths are due to cardiac sarcoidosis. Scadding stages for pulmonary sarcoidosis associates with clinical outcomes. Stages I and II have radiographic remission in approximately 30%-80% of cases. Stage III only has a 10%-40% chance of resolution, while stage IV has no change of resolution. Up to 40% of pulmonary sarcoidosis patients progress to stage IV disease with lung parenchyma fibroplasia, bronchiectasis with hilar retraction and fibrocystic disease. These patients are at highest risk for the development of precapillary pulmonary hypertension, which may occur in up to 70% of these patients. Sarcoid patients with pre-capillary pulmonary hypertension can respond to targeted pulmonary arterial hypertension medications. Stage IV fibrocytic sarcoidosis with significant pulmonary physiologic impairment, &gt;20% fibrosis on HRCT or pre-capillary pulmonary hypertension have the highest risk of mortality, which can be &gt;40% at 5-years. First line treatment for patients who are symptomatic (cough and dyspnea) with parenchymal infiltrates and abnormal pulmonary function testing (PFT) is oral glucocorticoids, such as prednisone with a typical starting dose of 20-40 mg daily for 2 weeks to 2 months. Prednisone can be tapered over 6-18 months if symptoms, spirometry, PFTs, and radiographs improve. Prolonged prednisone may be required to stabilize disease. Patients requiring prolonged prednisone ≥10 mg/day or those with adverse effects due to glucocorticoids may be prescribed second and third line treatements. Second and third line treatments include immunosuppressive agents (e.g., methotrexate and azathioprine) and anti-tumor necrosis factor (TNF) medication; respectively. Effective treatments for advanced fibrocystic pulmonary disease are being explored. Despite different treatments, relapse rates range from 13% to 75% depending on the stage of sarcoid, number of organs involved, socioeconomic status, and geography. CONCLUSION: The mortality rate for sarcoidosis over a 5 year follow up is approximately 7%. Unfortunately, 10%-40% of patients with sarcoidosis develop progressive pulmonary disease, and &gt;60% of deaths resulting from sarcoidosis are due to advance cardiopulmonary disease. Oral glucocorticoids are the first line treatment, while methotrexate and azathioprine are considered second and anti-TNF agents are third line treatments that are used solely or as glucocorticoid sparing agents for","PeriodicalId":15245,"journal":{"name":"Journal of autoimmunity","volume":" ","pages":"103107"},"PeriodicalIF":7.9,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49677873","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}
引用次数: 0
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