Firdapse (Amifampridine) Approved for Rare Autoimmune Disorder

THURSDAY, Nov. 29, 2018 -- Firdapse (amifampridine) tablets have been approved by the U.S. Food and Drug Administration for adults with Lambert-Eaton myasthenic syndrome (LEMS). It's the first agency-sanctioned treatment for the autoimmune disease...
Source: Drugs.com - Daily MedNews - Category: General Medicine Source Type: news

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Authors: Yilmaz V, Ulusoy C, Hajtovic S, Turkoglu R, Kurtuncu M, Tzartos J, Lazaridis K, Tuzun E Abstract Antigen-specific immune responses are crucially involved in both multiple sclerosis (MS) and myasthenia gravis (MG). Teriflunomide is an immunomodulatory agent approved for treatment of MS through inhibition of lymphocyte proliferation. MG associated with muscle-specific tyrosine kinase (MuSK) antibodies often manifests with a severe disease course, prompting development of effective treatment methods. To evaluate whether teriflunomide treatment may ameliorate MuSK-autoimmunity, experimental autoimmune MG (EAMG...
Source: Immunological Investigations - Category: Allergy & Immunology Tags: Immunol Invest Source Type: research
Myasthenia Gravis (MG) is a chronic autoimmune disease (AID) with a prevalence of 1-2 per 10.000 [1]. The clinical hallmark is fluctuating weakness of striated muscles with antibodies directly affecting the neuromuscular junction. In approximately 85% of patients, the initial presenting symptoms are asymmetric ptosis and/or diplopia. Approximately 80% of initially ocular MG patients will develop generalized MG within two years of disease onset [2].
Source: Neuromuscular Disorders - Category: Neurology Authors: Tags: Systematic review article Source Type: research
CONCLUSIONS AND CLINICAL IMPLICATIONS: Our study proves that the use of plasmapheresis conducted by filtration in the treatment of autoimmune neurological diseases is safe and well tolerated. PMID: 32557528 [PubMed - as supplied by publisher]
Source: Neurologia i Neurochirurgia Polska - Category: Neurology Authors: Tags: Neurol Neurochir Pol Source Type: research
The thymus, a primary lymphoid organ, provides a complex environment essential for the generation of the T-cell repertoire. Thymic alterations occur during life either in the context of thymic involution upon aging or the pathophysiological context of Myasthenia Gravis (MG). These changes involve complicated regulatory networks, in which microRNAs (miRNAs) are key players. Here, we analyzed the role of miRNAs in thymocyte maturation and differentiation sustained by thymic epithelial cells. We compared data from the literature regarding the role of mouse thymic miRNAs and original data obtained from a human thymic miRnome s...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
Complement activation as a driver of pathology in myasthenia gravis (MG) has been appreciated for decades. The terminal complement component [membrane attack complex (MAC)] is found at the neuromuscular junctions of patients with MG. Animals with experimental autoimmune MG are dependent predominantly on an active complement system to develop weakness. Mice deficient in intrinsic complement regulatory proteins demonstrate a significant increase in the destruction of the neuromuscular junction. As subtypes of MG have been better defined, it has been appreciated that acetylcholine receptor antibody-positive disease is driven ...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
Authors: Hanley PJ, Vollmer V, Bähler M Abstract Class IX myosins are simultaneously motor and signaling molecules. In addition to myosin class-specific functions of the tail region, they feature unique motor properties. Within their motor region they contain a long insertion with a calmodulin- and a F-actin-binding site. The rate-limiting step in the ATPase cycle is ATP hydrolysis rather than, typical for other myosins, the release of either product. This means that class IX myosins spend a large fraction of their cycle time in the ATP-bound state, which is typically a low F-actin affinity state. Nevertheless...
Source: Advances in Experimental Medicine and Biology - Category: Research Tags: Adv Exp Med Biol Source Type: research
Four retrospective studies from the United States, Europe, and Asia address outcomes in juvenile myasthenia gravis. Common features include earlier onset with ocular myasthenia gravis (MG) and generally good outcomes overall. Patients who were seronegative, had equivocal acetylcholine receptor (AChR) antibodies, or had only clustered AChR antibodies had better outcomes. An article highlighting the utility of median nerve slow-repetitive stimulation is reviewed and another showing the high sensitivity of repetitive nerve stimulation in myasthenic crisis is covered. Two articles address the thymus and thymectomy in non-AChR ...
Source: Journal of Clinical Neuromuscular Disease - Category: Neurology Tags: Review Article Source Type: research
Myasthenia gravis (MG) is a prototypical autoantibody mediated disease. The autoantibodies in MG target structures within the neuromuscular junction (NMJ), thus affecting neuromuscular transmission. The major disease subtypes of autoimmune MG are defined by their antigenic target. The most common target of pathogenic autoantibodies in MG is the nicotinic acetylcholine receptor (AChR), followed by muscle-specific kinase (MuSK) and lipoprotein receptor-related protein 4 (LRP4). MG patients present with similar symptoms independent of the underlying subtype of disease, while the immunopathology is remarkably distinct. Here we...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
AbstractThe idea that B  cells participate in immune regulation was initially postulated from observations in animals in the 1970s. It is now established that certain B‐cell populations, known as regulatory B cells, regulate immune reactions in various animal models of autoimmunity, chiefly through the production of in terleukin‐10. Subsequent to these findings in animals, several B‐cell subsets have been identified in human blood that are capable of producing interleukin‐10 when stimulatedex vivo. Although we still do not have direct evidence showing that these interleukin ‐10‐producing B cells ...
Source: Clinical and Experimental Neuroimmunology - Category: Neurology Authors: Tags: REVIEW ARTICLE Source Type: research
Myasthenia gravis (MG) is an autoimmune disease mediated by autoantibodies predominantly against the acetylcholine receptor (AChR). Specific T cell subsets are required for long-term antibody responses, and cytokines secreted mainly from CD4+ T cells regulate B cell antibody production. The aim of this study was to assess the differences in the cytokine expressions of CD4+ T cells in MG patients with AChR antibodies (AChR-MG) and the effect of immunosuppressive (IS) therapy on cytokine activity and to test these findings also in MG patients without detectable antibodies (SN-MG). Clinically diagnosed AChR-MG and SN-MG patie...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
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