Modern Rheumatology Journal最新文献

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Efficacy and safety of levilimab in the treatment of rheumatoid arthritis in real-life clinical practice: first results of the HELIOS observational study 左利单抗在实际临床实践中治疗类风湿性关节炎的疗效和安全性:HELIOS 观察性研究的初步结果
Modern Rheumatology Journal Pub Date : 2024-06-14 DOI: 10.14412/1996-7012-2024-3-32-43
A. Lila, I. Z. Gaydukova, O. Anoshenkova, I. G. Bannikova, I. B. Vinogradova, M. L. Goldman, S. Davidian, L. Evstigneeva, O. Epifanova, E. Zemerova, A. Zagrebneva, L. V. Ivanova, A. K. Karibova, I. Menshikova, O. Mironenko, M. P. Mikhailova, N. E. Nikulenkova, I. Patrikeeva, T. Plaksina, G. R. Savvina, R. Samigullina, L. E. Sarantseva, J. V. Usacheva, O. Ukhanova, G. F. Fatkhullina, A. Chudinov, I. Shafieva, S. Yakupova
{"title":"Efficacy and safety of levilimab in the treatment of rheumatoid arthritis in real-life clinical practice: first results of the HELIOS observational study","authors":"A. Lila, I. Z. Gaydukova, O. Anoshenkova, I. G. Bannikova, I. B. Vinogradova, M. L. Goldman, S. Davidian, L. Evstigneeva, O. Epifanova, E. Zemerova, A. Zagrebneva, L. V. Ivanova, A. K. Karibova, I. Menshikova, O. Mironenko, M. P. Mikhailova, N. E. Nikulenkova, I. Patrikeeva, T. Plaksina, G. R. Savvina, R. Samigullina, L. E. Sarantseva, J. V. Usacheva, O. Ukhanova, G. F. Fatkhullina, A. Chudinov, I. Shafieva, S. Yakupova","doi":"10.14412/1996-7012-2024-3-32-43","DOIUrl":"https://doi.org/10.14412/1996-7012-2024-3-32-43","url":null,"abstract":"   The efficacy and safety of levilimab (LVL) in patients with active rheumatoid arthritis (RA) has been confirmed in controlled clinical trials. This article presents the results of a preliminary analysis of a non-interventional observational study of LVL in RA patients.   Objective: to evaluate the efficacy and safety of LVL in the treatment of patients with RA in real-world clinical practice.   Materials and methods. The HELIOS study is a retrospective-prospective, multicenter, non-interventional study of retention rate of LVL therapy and the safety of LVL in patients with RA in real-world clinical practice. Patients received medical care, including LVL, according to routine clinical practice for the treatment of RA and Russian instructions for medical use of the drug. This article presents the results of an analysis of the efficacy and safety of LVL after 12 and 24 weeks of treatment. Efficacy was assessed using the DAS28-CRP/ESR, SDAI, CDAI and patient assessment of pain, fatigue and morning stiffness according to VAS (0–100 mm).   Results and discussion. 524 patients from 42 medical centers in the Russian Federation were enrolled in the study from June 2022 to November 2023. The majority of patients were female (83.2 %) and the mean age of patients was 53 years. A statistically significant decrease in DAS28-CRP/ESR, SDAI, CDAI, patient assessment of pain, fatigue and morning stiffness (VAS) was observed after 12 and 24 weeks of treatment, regardless of previous treatment with biologics or Jak inhibitors (JAKi). LVL was well tolerated by patients, the most frequently reported adverse events were infections, changes in peripheral blood and laboratory abnormalities characteristic of treatment with IL-6R inhibitors.   Conclusion. In real-world clinical practice, LVL has been shown to be highly effective and well tolerated in patients with RA when prescribed as the first biologic disease-modifying antirheaumatic drus (bDMARD) and after switching from other bDMARDs or JAKi.","PeriodicalId":18651,"journal":{"name":"Modern Rheumatology Journal","volume":"54 24","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-06-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141339287","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
Management of patients with infective endocarditis: current state-of-art. Part I: clinical picture, diagnosis, differential diagnosis 感染性心内膜炎患者的治疗:最新进展。第一部分:临床表现、诊断、鉴别诊断
Modern Rheumatology Journal Pub Date : 2024-06-13 DOI: 10.14412/1996-7012-2024-3-7-13
B. Belov, G. Tarasova, N. V. Muravyova
{"title":"Management of patients with infective endocarditis: current state-of-art. Part I: clinical picture, diagnosis, differential diagnosis","authors":"B. Belov, G. Tarasova, N. V. Muravyova","doi":"10.14412/1996-7012-2024-3-7-13","DOIUrl":"https://doi.org/10.14412/1996-7012-2024-3-7-13","url":null,"abstract":"   The problem of infective endocarditis (IE) remains relevant due to the high mortality rate and the development of severe complications. IE is a polyetiological disease, the occurrence and development of which can be caused by an extremely extensive list of pathogens, which is replenished almost annually. The pronounced clinical polymorphism of IE determines the importance of its early diagnosis, including the use of new medical technologies. Timely informing physicians about the modern principles of monitoring of patients with IE is of great practical importance. Part I of the article describes the clinical features, basic principles of diagnosis and differential diagnosis of IE, taking into account the recommendations of the European Society of Cardiology 2023.","PeriodicalId":18651,"journal":{"name":"Modern Rheumatology Journal","volume":"14 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-06-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141347518","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
The role of repeat kidney biopsy in patients with lupus nephritis (case report) 狼疮性肾炎患者重复肾活检的作用(病例报告)
Modern Rheumatology Journal Pub Date : 2024-04-22 DOI: 10.14412/1996-7012-2024-2-88-94
N. Y. Nikishin, E. Aseeva, A. Mesnyankina, S. Soloviev, E. S. Stolyarevich, V. Zelenov, A. Lila
{"title":"The role of repeat kidney biopsy in patients with lupus nephritis (case report)","authors":"N. Y. Nikishin, E. Aseeva, A. Mesnyankina, S. Soloviev, E. S. Stolyarevich, V. Zelenov, A. Lila","doi":"10.14412/1996-7012-2024-2-88-94","DOIUrl":"https://doi.org/10.14412/1996-7012-2024-2-88-94","url":null,"abstract":"Lupus nephritis (LN) occurs in 35–60% of patients with systemic lupus erythematosus (SLE), often in the early stages of the disease. LN is one of the most severe manifestations of SLE and, if not treated promptly and effectively, can lead to rapid and severe loss of kidney function. Despite modern pharmacotherapy, 5–20% of patients develop end-stage renal failure within 10 years of diagnosis of LN. The main principle of LN therapy is to prevent deterioration of renal function, but a consensus on outcome assessment criteria and clinically relevant short- and long-term goals for LN therapy has not yet been reached. There is increasing evidence of the importance of repeat kidney biopsies to assess the outcomes of the initial phase of therapy and to determine the long-term prognosis of renal failure. It is believed that the information obtained from repeat biopsies can help to make optimal treatment decisions and thus increase the likelihood of achieving a complete renal response in the short term and a more favorable renal prognosis in the long term.We describe a clinical case of a young patient with highly active SLE and morphologically confirmed class IV-S LN. Repeat biopsy and clinical and laboratory examination, had confirmed a clinical and laboratory remission of the disease on the background of the therapy. This made it possible to reduce the dose of methylpednisolone and cytostatic drugs (mycophenolate mofetil) and to discontinue therapy with biologic diseasemodifying antirheumatic drug (rituximab).","PeriodicalId":18651,"journal":{"name":"Modern Rheumatology Journal","volume":"79 23","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140675295","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
Systemic lupus erythematosus with juvenile onset: current status of the problem (literature review) 幼年发病的系统性红斑狼疮:问题的现状(文献综述)
Modern Rheumatology Journal Pub Date : 2024-04-22 DOI: 10.14412/1996-7012-2024-2-95-102
M. I. Kaleda, I. Nikishina
{"title":"Systemic lupus erythematosus with juvenile onset: current status of the problem (literature review)","authors":"M. I. Kaleda, I. Nikishina","doi":"10.14412/1996-7012-2024-2-95-102","DOIUrl":"https://doi.org/10.14412/1996-7012-2024-2-95-102","url":null,"abstract":"Patients with juvenile-onset systemic lupus erythematosus (jSLE) account for up to 25% of all SLE patients. The main difference between jSLE and SLE in adults is the greater role of genetic factors in the pathogenesis, higher activity, earlier development of complications and the need for more aggressive immunosuppressive therapy, which allows us to consider the onset of the disease in childhood as a special phenotype of SLE. The relevance of the study of jSLE arises from the variability of clinical manifestations and the unpredictability of the course, the difficulty of early diagnosis, the rapid development of organ damage and the unfavorable life prognosis.The article presents the most important modern data on the diagnosis, classification, features of the clinical picture and treatment approaches of jSLE from a practical point of view.","PeriodicalId":18651,"journal":{"name":"Modern Rheumatology Journal","volume":"58 4","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140675844","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
Alfacalcidol in clinical practice: focus on the efficacy and safety of the Russian generic drug 阿法骨化醇在临床实践中的应用:关注俄罗斯仿制药的疗效和安全性
Modern Rheumatology Journal Pub Date : 2024-04-22 DOI: 10.14412/1996-7012-2024-2-62-69
N. Toroptsova, M. Kozyreva, O. Dobrovolskaya
{"title":"Alfacalcidol in clinical practice: focus on the efficacy and safety of the Russian generic drug","authors":"N. Toroptsova, M. Kozyreva, O. Dobrovolskaya","doi":"10.14412/1996-7012-2024-2-62-69","DOIUrl":"https://doi.org/10.14412/1996-7012-2024-2-62-69","url":null,"abstract":"The most important factors that determine the risk of fractures are not only the presence of osteoporosis (OP), but also the risk of falls. In addition to sufficient physical activity, medications are needed that have a positive effect on the strength and functional performance of the skeletal muscles. Vitamin D supplements play a key role in this therapeutic approach.Objective: to investigate the tolerability, safety and efficacy of the generic drug Alfacalcidol Canon on the functional status of patients with reduced bone mineral density in clinical practice.Material and methods. The study involved 30 women with OP or osteopenia who were at high risk of falls (mean age 68.0±7.2 years) and who were administered Alfacalcidol Canon at a dose of 1 mcg/day for 3 months. The follow-up period included 4 visits to the clinic, during which laboratory tests were performed and falls and fractures, adverse reactions and patients' adherence to treatment were registered. At the first and last visits, quality of life was assessed using the EQ-5D and the functional status of the skeletal muscles was examined. At the 4th visit, the tolerability of the medication was assessed.Results and discussion. After 3 months of therapy, an increase in muscle strength of the dominant arm was observed by an average of 15.4% (p=0.00002). The time for the \"Chair Stand Test\" and the \"Time up and go\" test decreased by 17.4% (p=0.000012) and 13.6% (p=0.00004) respectively. The gait speed test increased on average by 13.9% (p=0.000013), it increased in 26 (86.7%) women. The SPPB test score increased on average by 12.5% (p=0.000001), and achievement of the best indicators was found in 23 (76.7%) women. There were no cases of hypercalcemia, hyperphosphatemia and hypermagnesemia, but 1 case of hypercalciuria was detected. Falls occurred in 2 women (6.7%) who had fallen in the previous year, one of whom suffered a fracture of humeral neck. Adverse reactions were observed in 3 (10 %) women, one of whom stopped taking the drug. 90% of the patients tolerated the treatment excellently. The average compliance rate was 94.3%.Conclusion. Alfacalcidol Canon is a safe and effective drug that can be used to improve the physical performance of skeletal muscles in patients with a high risk of falls.","PeriodicalId":18651,"journal":{"name":"Modern Rheumatology Journal","volume":"8 5","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140673451","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
The first clinical experience with selective DNA plasmasorption using the NucleoCapture Device in the treatment of systemic lupus erythematosus 使用 NucleoCapture 设备进行选择性 DNA 等离子吸附治疗系统性红斑狼疮的首次临床经验
Modern Rheumatology Journal Pub Date : 2024-04-22 DOI: 10.14412/1996-7012-2024-2-75-80
E. Aseeva, N. S. Pokrovsky, S. Soloviev, E. V. Nikolaeva, N. Nikishina, E. T. Abdullin, T. Reshetnyak, E. Zotkin, A. Lila
{"title":"The first clinical experience with selective DNA plasmasorption using the NucleoCapture Device in the treatment of systemic lupus erythematosus","authors":"E. Aseeva, N. S. Pokrovsky, S. Soloviev, E. V. Nikolaeva, N. Nikishina, E. T. Abdullin, T. Reshetnyak, E. Zotkin, A. Lila","doi":"10.14412/1996-7012-2024-2-75-80","DOIUrl":"https://doi.org/10.14412/1996-7012-2024-2-75-80","url":null,"abstract":"Systemic lupus erythematosus (SLE) is a systemic autoimmune disease of unknown etiology characterized by the overproduction of autoantibodies against various components of the nucleus of the patient's own cells with the development of immunoinflammatory tissue damage. In recent years, more and more data have accumulated on the involvement of neutrophils in the development of the clinical symptoms of SLE, and DNA-containing structures and neutrophil extracellular traps (NETs) playing an important role in this process. Effective neutralization of NETs in SLE can be achieved by removing circulating proteins and molecules associated with NETs from the bloodstream by selective plasma sorption of DNA using the NucleoCapture Device.This article describes the case of a patient who underwent three plasma sorption sessions aiming to suppress the activity of SLE. During the therapy, significant positive dynamics were achieved: the SLEDAI-2K index decreased from 32 to 12 points, the number of leukocytes in the blood normalized, renal function improved, and the immunological activity of the disease decreased.","PeriodicalId":18651,"journal":{"name":"Modern Rheumatology Journal","volume":"51 16","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140672673","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
New molecular aspects of the pathogenesis of osteoporosis – perspectives for early diagnosis and treatment 骨质疏松症发病机制的新分子方面--早期诊断和治疗的前景
Modern Rheumatology Journal Pub Date : 2024-04-22 DOI: 10.14412/19967012-2024-2-103-110
A. Tyurin, K. E. Akhiyarova, B. Yalaev, T. S. Zagidullin, R. Khusainova
{"title":"New molecular aspects of the pathogenesis of osteoporosis – perspectives for early diagnosis and treatment","authors":"A. Tyurin, K. E. Akhiyarova, B. Yalaev, T. S. Zagidullin, R. Khusainova","doi":"10.14412/19967012-2024-2-103-110","DOIUrl":"https://doi.org/10.14412/19967012-2024-2-103-110","url":null,"abstract":"Osteoporosis (OP) is a common disease leading to low-trauma fractures and is a serious medical and social problem. Often a fracture is the first clinical manifestation of OP that has been asymptomatic for a long time, necessitating the development of methods for early detection and risk assessment of this disease. OP is a multifactorial disease with a strong hereditary component. However, as the data from the study of genetic factors show, only 15% of the heritability of this trait can be explained. In this context, the focus of research is shifting to the area of epigenetic regulation, which controls gene activity without altering the primary structure of the DNA. One of the most promising mechanisms of epigenetic control is methylation, which affects DNA as well as RNA and histones. The characteristics of these mechanisms and the possibilities of their use for the diagnosis and treatment of OP are presented in this review.","PeriodicalId":18651,"journal":{"name":"Modern Rheumatology Journal","volume":"72 12","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140675338","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
Libman–Sachs endocarditis: regression after immunosuppressive therapy in a patient with systemic lupus erythematosus 利伯曼-萨克斯心内膜炎:一名系统性红斑狼疮患者接受免疫抑制治疗后病情缓解
Modern Rheumatology Journal Pub Date : 2024-04-22 DOI: 10.14412/1996-7012-2024-2-70-74
V. A. Pugach, N. Bulanov, T. P. Shevtsova, K. V. Kurginyan, P. I. Novikov, S. V. Moiseev
{"title":"Libman–Sachs endocarditis: regression after immunosuppressive therapy in a patient with systemic lupus erythematosus","authors":"V. A. Pugach, N. Bulanov, T. P. Shevtsova, K. V. Kurginyan, P. I. Novikov, S. V. Moiseev","doi":"10.14412/1996-7012-2024-2-70-74","DOIUrl":"https://doi.org/10.14412/1996-7012-2024-2-70-74","url":null,"abstract":"Libman–Sachs endocarditis (nonbacterial thrombotic endocarditis, NBTE) may be one of the cardiac manifestations of systemic lupus erythematosus (SLE). It is characterized by the presence of sterile platelet thrombi on previously normal heart valves. The diagnosis of NBTE is difficult as it is often asymptomatic until complications such as systemic emboli or valvular dysfunction occur. Patients with Libman–Sachs endocarditis and SLE are treated with immunosuppressive and anticoagulant therapy and, if it is ineffective, surgical treatment.A clinical case of SLE and Libman–Sachs endocarditis is presented. The clinical manifestations, diagnostic methods and treatment of NBTE in SLE are discussed. The difficulties in differential diagnosis and selection of optimal treatment tactics faced by physicians are highlighted.","PeriodicalId":18651,"journal":{"name":"Modern Rheumatology Journal","volume":"89 18","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140677035","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
On the problem of differential diagnosis in the detection of antineutrophil cytoplasmic antibodies 抗中性粒细胞胞浆抗体检测中的鉴别诊断问题
Modern Rheumatology Journal Pub Date : 2024-04-19 DOI: 10.14412/1996-7012-2024-2-7-15
O. N. Egorova, G. M. Tarasova, B. Issaeva, A. Datsina, D. S. Dilmanova, S. M. Issaeva, M. G. Nogaeva, A. G. Issaeva, A. S. Amanzholova, M. Saparbayeva, A. Atasheva, A. B. Kanapina
{"title":"On the problem of differential diagnosis in the detection of antineutrophil cytoplasmic antibodies","authors":"O. N. Egorova, G. M. Tarasova, B. Issaeva, A. Datsina, D. S. Dilmanova, S. M. Issaeva, M. G. Nogaeva, A. G. Issaeva, A. S. Amanzholova, M. Saparbayeva, A. Atasheva, A. B. Kanapina","doi":"10.14412/1996-7012-2024-2-7-15","DOIUrl":"https://doi.org/10.14412/1996-7012-2024-2-7-15","url":null,"abstract":"Vasculitides associated with antineutrophil cytoplasmic antibodies (ANCA) are a group of systemic autoimmune diseases characterized by necrotizing lesions of the walls of predominantly small vessels and the presence of ANCA against proteinase 3 or myeloperoxidase. However, an increase in ANCA levels can also be observed in other diseases, including autoimmune, malignant and infectious diseases, which complicates the interpretation of clinical and laboratory data and requires a differential diagnosis.","PeriodicalId":18651,"journal":{"name":"Modern Rheumatology Journal","volume":" 9","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-04-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140684177","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
CINCA/NOMID is a rare autoinflammatory syndrome in rheumatological practice. Experience of diagnosis, management and therapy with interleukin-1 inhibitors CINCA/NOMID是风湿病学实践中一种罕见的自身炎症综合征。白细胞介素-1抑制剂的诊断、管理和治疗经验
Modern Rheumatology Journal Pub Date : 2024-04-19 DOI: 10.14412/1996-7012-2024-2-25-32
S. Salugina, E. S. Fedoro, A. Torgashina
{"title":"CINCA/NOMID is a rare autoinflammatory syndrome in rheumatological practice. Experience of diagnosis, management and therapy with interleukin-1 inhibitors","authors":"S. Salugina, E. S. Fedoro, A. Torgashina","doi":"10.14412/1996-7012-2024-2-25-32","DOIUrl":"https://doi.org/10.14412/1996-7012-2024-2-25-32","url":null,"abstract":"Objective: to present the experience of diagnosis, management and therapy with interleukin-1 inhibitors (iIL1) in patients with Chronic Infantile Onset Neurologic Cutaneous Articular/Neonatal Onset Multisystem Inflammatory Disease (CINCA/NOMID) according to the Russian Federal Rheumatological Center data.Material and methods. From 2007 to 2023, eight patients were included in the study (7 men) aged 10 months to 33 years, including 3 with a disease duration of more than 10 years (13, 17 and 33 years). Genetic testing was performed in all patients and mutations in the NLRP3 gene were identified in 6 cases.Results and discussion. The age of onset of the disease ranged from 0 to 6 months. The delay in diagnosis and prescription of therapy ranged from 10 months to 33 years. All patients had the classic manifestations of CINCA/NOMID, including fever, rash, central nervous system (CNS) involvement, elevated ESR and CRP levels, 6 patients had articular manifestations, 7 had ocular manifestations and 6 had sensorineural hearing loss. Amyloidosis was detected in 1 case. All patients were prescribed iIL1. Anakinra was used in 6 patients (in 5 as the first line, in 1 as the second line therapy) with a positive response; subsequently 2 of these patients were switched to canakinumab once every 4 weeks (1 patient deteriorated and was readministered anakinra). Five patients received canakinumab (3 as first-line therapy, 2 as second-line therapy), 1 patient was switched to anakinra due to insufficient CNS response. The response to iIL1 therapy was positive in all patients, but incomplete in some of them due to the severity of the manifestations and the presence of irreversible organ damage.Conclusion. Patients with CINCA/NOMID have a severe disease and a poor prognosis. In this context, early administration of iIL1 is necessary. In the case of CNS involvement, the use of anakinra is preferable, as it is characterized by better penetration of the blood-brain barrier and is therefore more effective. Later it is possible to switch the patient to canakinumab, however, to achieve a complete response, it is sometimes necessary to increase the dose of the drug and reduce the interval between doses.","PeriodicalId":18651,"journal":{"name":"Modern Rheumatology Journal","volume":" 40","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-04-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140683884","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
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