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Immunological Targets in Generalized Myasthenia Gravis Treatment: Where Are We Going Now?

BackgroundGeneralized myasthenia gravis (gMG) is a heterogeneous autoimmune disorder marked by antibody-mediated disruption of neuromuscular transmission. Despite advancements in immunosuppressive therapies and biologics, a subset of patients remains refractory, necessitating more targeted and personalized treatment strategies.ObjectiveThis review aims to synthesize current knowledge of the immunopathological mechanisms across gMG subtypes and to explore emerging therapeutic targets tailored to these diverse disease phenotypes.MethodsA narrative review was conducted, integrating recent finding

"Complimenting the Complement": Mechanistic Insights and Opportunities for Therapeutics in Hepatocellular Carcinoma.

Hepatocellular carcinoma (HCC) is the most common primary malignancy of the liver and a leading cause of death in the US and worldwide. HCC remains a global health problem and is highly aggressive with unfavorable prognosis. Even with surgical interventions and newer medical treatment regimens, patients with HCC have poor survival rates. These limited therapeutic strategies and mechanistic understandings of HCC immunopathogenesis urgently warrant non-palliative treatment measures. Irrespective of the multitude etiologies, the liver microenvironment in HCC is intricately associated with chronic

Совпадение в полном тексте источника
… While not much of the pathobiological role of complement C5/C5a–C5AR1 axis is known in HCC, its ability to critically influence and control signaling processes relevant to HCC is largely evident from several studies. …

B Cells and Antibodies as Targets of Therapeutic Intervention in Neuromyelitis Optica Spectrum Disorders.

The first description of neuromyelitis optica by Eugène Devic and Fernand Gault dates back to the 19th century, but only the discovery of aquaporin-4 autoantibodies in a major subset of affected patients in 2004 led to a fundamentally revised disease concept: Neuromyelits optica spectrum disorders (NMOSD) are now considered autoantibody-mediated autoimmune diseases, bringing the pivotal pathogenetic role of B cells and plasma cells into focus. Not long ago, there was no approved medication for this deleterious disease and off-label therapies were the only treatment options for affected patient

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… Eculizumab epitope on complement c5: Progress towards a better understanding of the mechanism of action. Mol. Immunol. 2016;77:126–131. doi: 10.1016/j.molimm.2016.07.016. 163. Dhillon S. Eculizumab: A review in generalized myasthenia gravis. …

Improving Kidney Disease Care: One Giant Leap for Nephrology.

Nephrology is an ever-evolving field of medicine. The importance of such a discipline is related to the high clinical impact of kidney disease. In fact, abnormalities of kidney function and/or structure are common in the general population, reaching an overall prevalence of about 10%. More importantly, the onset of kidney damage is related to a strikingly high risk of cardiovascular events, mortality, and progression to kidney failure which, in turn, compromises quality and duration of life. Attempts to comprehend the pathogenesis and molecular mechanisms involved in kidney disease occurrence

16th International Congress on Antiphospholipid Antibodies Task Force Report on Antiphospholipid Syndrome Treatment Trends.

Antiphospholipid syndrome (APS), an acquired autoimmune thrombophilia, is characterised by thrombosis and/or pregnancy morbidity in association with persistent antiphospholipid antibodies. The 16th International Congress on Antiphospholipid Antibodies Task Force on APS Treatment Trends reviewed the current status with regard to existing and novel treatment trends for APS, which is the focus of this Task Force report. The report addresses current treatments and developments since the last report, on the use of direct oral anticoagulants in patients with APS, antiplatelet agents, adjunctive ther

Sp140L функционирует как фактор ограничения герпесвируса, подавляющий вирусную транскрипцию и активирующий интерферонально-стимулированные гены.

Герпесвирусы, включая вирус Эпштейн-Барр (ЭБВ), - человеческий онкогенный вирус и важнейший пусковой механизм множественного склероза, - должны обходить механизмы ДНК-разведки, чтобы установить пожизненную скрытую инфекцию. Поэтому герпесвирусы кодируют вирусные белки, чтобы разрушить ключевые факторы, связанные с зондированием ДНК и вирусным ограничением. Первый вирусный белок латентности, выраженный EBNA-LP, крайне важен для преобразования наивных клеток B и установления вирусного генного экспрессии, однако его роль в уклонении от защиты хостов остается неясной. Используя одноклеточную после

Carfilzomib Induced Microangiopathy due to Accumulation With Paxlovid.

Аннотация не предоставлена источником. Откройте карточку для перехода к публикации.

Autoimmune Hemolytic Anemias: Challenges in Diagnosis and Therapy.

BackgroundAutoimmune hemolytic anemia (AIHA) is a rare disease due to increased destruction of erythrocytes by autoantibodies, with or without complement activation.SummaryAIHA is usually classified in warm AIHA (wAIHA) and cold agglutinin disease (CAD), based on isotype and thermal amplitude of the autoantibody. The direct antiglobulin test (DAT) or Coombs test is the cornerstone of AIHA diagnosis, as it is positive with anti-IgG in wAIHA, and with anti-C3d/IgM antisera plus high titer cold agglutinins in CAD. Therapy is quite different, as steroids and rituximab are effective in the former,

Иммунотерапевтический ландшафт амиотропического бокового склероза: библиометрический анализ тенденций в области исследований, приоритетов в области перевода и сетей сотрудничества (см.2006-2025).

Амиотрофный боковой склероз (ALS) по-прежнему является серьезной терапевтической проблемой, при этом иммунная диагностика все чаще признается в качестве одного из важнейших факторов развития заболеваний. Несмотря на обширные механистические исследования, никакой иммунотерапевтический подход не привел к последовательному изменению болезней, в связи с чем возникают вопросы о том, отражает ли этот переводный пробел биологическую сложность или структурную несогласованность в исследовательской экосистеме. Чтобы охарактеризовать интеллектуальную эволюцию исследований иммунотерапевтии ALS, определить

Совпадение в полном тексте источника
… At the molecular level, low-dose IL-2 and autologous Treg-based approaches appear to be among the more promising immunotherapeutic strategies to date, whereas complement C5 inhibition has been de-emphasized for unselected populations based on repeated late-phase failures—though targeted investigation … Efficacy and safety of ravulizumab, a complement C5 inhibitor, in adults with amyotrophic lateral sclerosis: a randomized clinical trial. JAMA Neurol. 2023;80(10):1089–1097. doi: 10.1001/jamaneurol.2023.2851. 32. …

Influenza A H1N1 Triggering Complement-Mediated Hemolytic Uremic Syndrome.

Complement-mediated hemolytic uremic syndrome (CM-HUS) is a condition characterized by hemolytic anemia, thrombocytopenia, and renal impairment, typically associated with a chronic dysregulation of the complement system. Few adult cases of CM-HUS triggered by influenza infection have been described. Here we present a case of a 56-year-old female with multiple myeloma receiving cancer treatment who presented with Influenza A H1N1-associated CM-HUS. She was treated with anti-complement therapy and at her 1-month follow-up had complete resolution of renal dysfunction and hemolytic anemia. This ca

Complication from Desensitization

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Протеолипид Протеин-1 и его периферальная изоформа DM20 у пациентов с аутоиммунными расстройствами CNS.

: Антитела для протеолипидного белка-1 (PLP1-IgG), крупного центрального миелина, также выраженного в периферической нервной системе (PNS) в качестве изоформы DM20, ранее были идентифицированы главным образом у пациентов с множественным склерозом (MS), с неясными клиническими последствиями. Однако большинство исследований основывалось на неконформационных иммуномассах и охватывало лишь небольшое число пациентов с аутоиммунными расстройствами демиелионации (АДД), не относящимися к ПС CNS. Мы стремились изучить конформацию PLP1-IgG во всем спектре ОДЗ.МЕТОДЫ: Мы разработали новый анализ на основ

Complement C3a promotes the formation of osteoclasts by inhibiting Sirt1 to activate the PI3K/PDK1/SGK3 pathway in patients with multiple myeloma.

BACKGROUND: Myeloma bone disease (MBD) is the most common complication of multiple myeloma (MM). Our previous study showed that the complement C3a activates osteoclasts to participate in the pathogenesis of MBD; however, its mechanism of action is diverse and complex. Studies have shown that the Sirtuin (Sirt) family of proteins (i.e., Sirt1-7) are expressed in human bone and cartilage, and participate in bone metabolic balance. METHODS AND RESULTS: We measured the levels of complement C3a, Sirt1, osteoclast-related genes, and bone disease-related biological indicators using enzyme-linked imm

Immunoglobulin A Nephropathy: Molecular Pathogenesis and Targeted Therapy.

Immunoglobulin A nephropathy (IgAN), the most prevalent primary glomerulonephritis globally, is characterized by mesangial IgA deposition and heterogeneous clinical trajectories. Historically, management relied on renin-angiotensin system inhibition and empirical immunosuppression, yet high lifetime kidney failure risk persists despite optimized care. This review synthesizes advances in molecular pathogenesis, highlighting how the traditional multi-hit hypothesis-while foundational for targeted therapy development-fails to capture IgAN's recurrent, self-amplifying nature. We introduce the "spi

Severe autoimmune hemolytic anemia; epidemiology, clinical management, outcomes and knowledge gaps.

Autoimmune hemolytic anemia (AIHA) is an acquired hemolytic disorder, mediated by auto-antibodies, and has a variable clinical course ranging from fully compensated low grade hemolysis to severe life-threatening cases. The rarity, heterogeneity and incomplete understanding of severe AIHA complicate the recognition and management of severe cases. In this review, we describe how severe AIHA can be defined and what is currently known of the severity and outcome of AIHA. There are no validated predictors for severe clinical course, but certain risk factors for poor outcomes (hospitalisation, trans

Global first-in-class drugs approved in 2023-2024: Breakthroughs and insights.

First-in-class (FIC) drugs are considered the main drivers of new drug discovery and have novel targets and mechanisms. The proportion of FIC drugs among new drugs provides fundamental guidance for both academic and pharmaceutical research. From a global perspective, 81 FIC drugs were approved in 2023 and 2024. Among them, small-molecule drugs account for a greater percentage (51.9%), illustrating the discovery of new chemical entities. Macromolecule drugs (48.1%), which mainly consist of antibody analogs, represent a growing trend as new biotechnology techniques have emerged. In terms of FIC

Kidney Disease and Hematopoietic Stem Cell Transplantation.

Hematopoietic stem cell transplantation (HSCT) is a potentially curative therapy for patients with hematologic malignancies and certain solid tumors and nonmalignant hematologic conditions. Both AKI and CKD occur commonly after HSCT and are associated with significant morbidity and mortality. AKI and CKD in this setting may result from direct effects of the transplant or be caused by pretransplant bone marrow conditioning regimens and/or nephrotoxic agents administered in the post-transplant period. In this article, we review the epidemiology, risk factors, etiologies, pathophysiology, diagnos

Исследования " горячих точек " и новые темы для лечения расстройства спектра нейромиелита: Отчёты по библиометрическому анализу.

БИХЕКТ: Эффективное лечение расстройства спектра нейромиелита (NMOSD) может значительно снизить уровень инвалидности и смертности среди пациентов. В научных кругах все большее значение приобретают исследования по лечению НМОУР. Цель этого исследования состоит в том, чтобы отразить эволюцию и интеграцию методов лечения НМОУР в прошлом. 20 Годы, выявление основных участников в этой области, уточнение нынешних < < горячих точек > > для исследований и изучение новых тем, которые могут определять направления будущих исследований. МЕТОД: Изделия в этой области 2004 в адрес 2023 был взят

An Updated Comprehensive Review on Diseases Associated with Nephrotic Syndromes.

There have been exciting advances in our knowledge of primary glomerular diseases and nephrotic syndromes in recent years. Beyond the histological pattern from renal biopsy, more precise phenotyping of the diseases and the use of modern nephrogenetics helps to improve treatment decisions and sometimes also avoid unnecessary exposure to potentially toxic immunosuppression. New biomarkers have led to easier and more accurate diagnoses and more targeted therapeutic decisions. The treatment landscape is becoming wider with a pipeline of promising new therapeutic agents with more sophisticated appr

Флюидные биомаркеры в демиелионирующих синдромах: прошлое, настоящее и перспективы.

По-прежнему продолжается работа над диагностическим алгоритмом демиелионирующих расстройств центральной нервной системы, а также поиск идеальных биомаркеров. Так называемый "идеальный" биомаркер должен обеспечивать точное различие между наиболее распространенными демиелионирующими патологиями ЦНС и подтипами одной и той же патологии (например, переход от релапсирующего многоразового склероза к вторичному прогрессивному фенотипу). Достижения в области технологии способствовали проведению этого исследования, и в нижеследующих разделах мы всесторонне проанализируем большинство из них, охарактериз