Myasthenia Gravis
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  • ||||||||||  remifentanil / Generic mfg.
    Total thyroidectomy in a patient with Myasthenia gravis – case report (Virtual Platform - Poster section) -  Jun 17, 2022 - Abstract #Euroanaesthesia2022Euroanaesthesia_709;    
    The patient was induced with remifentanil and propofol and an endotracheal tube was placed...This elective procedure was performed during a stable phase of the disease, scheduled as early as possible in the day to avoid excess fatigability, and with appropriate preoperative assessment for bulbar/respiratory symptoms. Anaesthetic medications used considered this disease, with NBMAs not being used, so increased monitoring was used to avoid movement, with no complications and reaching a good outcome.2,3
  • ||||||||||  Keytruda (pembrolizumab) / Merck (MSD)
    Myastenia gravis secondary to pembrolizumab as a directed treatment of colorectal cancer. About a case (Virtual Platform - Poster section) -  Jun 17, 2022 - Abstract #Euroanaesthesia2022Euroanaesthesia_610;    
    She required a tracheostomy at 10 days, and could be discharged to the ward after 36 days of stay in the ICU. The development of drugs targeting specific tumours represents a new approach in antineoplastic therapies.PBZ selectively inhibits PD-1, a programmed death receptor that decreases the activity of T cells through the overexpressed PD-L1 and PD-L2 ligands in certain tumours, thus enhancing the immune and antitumor response of T cells.In MG, autoantibodies destroy acetylcholine receptors, producing muscle weakness, which may affect the respiratory muscles and require intubation, as was the case in our patient.Although MG is considered a rare adverse reaction in clinical trials published to date, the increasingly widespread use of this drug in cancer patients is causing an increase in its incidence. It is important to consider it in all patients with symptoms compatible with MG undergoing treatment with PBZ.
  • ||||||||||  Journal:  Giant cell myositis associated with metastatic thymoma and granulomatous hypercalcaemia. (Pubmed Central) -  Jun 14, 2022   
    Her course was complicated by several opportunistic infections in the setting of her immunosuppression. Despite the high morbidity associated with GCM, she demonstrated clinical improvement after initiating immunosuppressive therapy and continues to be managed in the outpatient setting.
  • ||||||||||  Journal:  Outcomes after Thymectomy in Patients with Thymomatous Myasthenia Gravis. (Pubmed Central) -  Jun 14, 2022   
    Conclusion  The present study shows the beneficial effect of thymectomy in patients with T-MG. Postoperative clinical outcomes seem to be better when the preoperative severity of myasthenic symptoms is mild (MGFA class II).
  • ||||||||||  Review, Journal:  Paraneoplastic syndromes in ophthalmology. (Pubmed Central) -  Jun 11, 2022   
    The presence of autoantibodies is helpful in clinical practice but negativity does not exclude this diagnosis. Although evolution and prognosis is linked to primary disease, in some cases specific treatment, usually immunosuppressive therapy, can help improving patients quality of life.
  • ||||||||||  Keytruda (pembrolizumab) / Merck (MSD)
    Review, Journal:  Pembrolizumab-induced Myasthenia Gravis: Literature Review of Ocular Manifestations and a Refractory Case. (Pubmed Central) -  Jun 10, 2022   
    Patients with immune-related myasthenia gravis experience increased mortality and morbidity but if steroid-responsive, may benefit from the reintroduction of anti-programmed cell death protein 1 therapy for end-stage malignancy with close monitoring. A high index of clinical suspicion for immune-related adverse effects are critical in an era of rising immunotherapy use.
  • ||||||||||  Retrospective data, Journal, Checkpoint inhibition:  Immune checkpoint inhibition in patients with inactive pre-existing neuromuscular autoimmune diseases. (Pubmed Central) -  Jun 9, 2022   
    In this case-series, exacerbations of neurological autoimmune disease were less common and less severe than expected. Further research is needed to determine which individuals are at greatest risk of neurological autoimmune disease complication while receiving immune checkpoint inhibitor therapy.
  • ||||||||||  Journal:  Association of Smoking and Generalized Manifestations of Myasthenia Gravis. (Pubmed Central) -  Jun 8, 2022   
    The smoking history before or at onset of MG was not associated with the age of onset. Conclusion Smoking exposure after the onset is associated with generalized manifestations of MG in our cohort of patients with a broad clinical spectrum of presentations.
  • ||||||||||  Clinical, Retrospective data, Review, Journal:  A systematic review and meta-analysis of HLA class II associations in patients with IgG4 autoimmunity. (Pubmed Central) -  Jun 8, 2022   
    Genotype (G) and allele (A) frequencies of HLA-DQB1*05 (G: OR 3.8; 95% CI 2.44-5.9; p < 0.00001; A: OR 2.54; 95% CI 1.82-3.55; p < 0.00001) and HLA-DRB1*14 (G: OR 4.31; 95% CI 2.82-6.59; p < 0.00001; A: OR 4.78; 95% CI 3.52-6.49; p < 0.00001) and the HLA-DRB1*14-DQB1*05 haplotype (OR 6.3; 95% CI 3.28-12.09; p < 0.00001/OR 4.98; 95% CI 3.8-6.53; p < 0.00001) were increased while HLA-DRB1*13 (G: OR 0.48; 95% CI 0.34-0.68; p < 0.0001; A: OR 0.46; 95% CI 0.34-0.62; p < 0.00001) was decreased in IgG4-AID patients. In conclusion, the HLA-DQB1*05, HLA-DRB1*14 alleles and the HLA-DQB1*05-DRB1*14 haplotype could be genetic risk factors that predispose for the production of pathogenic IgG4 autoantibodies and the HLA-DRB1*13 allele may protect from IgG4 autoimmunity.
  • ||||||||||  Journal:  An industry update: what's new in the field of therapeutic delivery? (Pubmed Central) -  Jun 4, 2022   
    Lastly, this month brought good news on several diseases with poor prognosis and no or limited treatment options. This included Novartis's acquisition of Gyroscope and its gene therapy for age-related macular degeneration, Merck's purchase of Chord Therapeutics and the rights of develop cladribine for generalized myasthenia gravis and neuromyelitis optica spectrum disorders, positive phase III interim results for UCB's, Bimzelx, in ankylosing spondylitis and AstraZeneca and Ionis co-development and commercialization deal on eplontersen for transthyretin amyloidosis.
  • ||||||||||  Vyvgart (efgartigimod alfa-fcab) / argenx, Broteio
    Review, Journal:  A Review of Disease Mechanisms and Current and Emerging Treatment Options for Generalized Myasthenia Gravis. (Pubmed Central) -  Jun 4, 2022   
    However, with the novel neonatal Fc receptor antagonist efgartigimod, levels of all immunoglobulin G subfractions are reduced, addressing an important aspect of the underlying pathophysiology of gMG. Through this program, clinicians will consider novel mechanisms in gMG therapy, learn to counsel patients on the changing landscape of gMG therapies, and find ways to incorporate the latest efficacy and safety data into practice.
  • ||||||||||  Hizentra (human immune globulin subcutaneous 20%) / CSL Behring, NN1213 / Novo Nordisk
    Trial completion date, Trial primary completion date:  A Pilot Trial To Assess The Feasibility And Efficacy Of SCIG In Patients With MG Exacerbation (SCIG-MG) (clinicaltrials.gov) -  May 31, 2022   
    P3,  N=30, Recruiting, 
    This can complicate the detection or exclusion of abnormal NMT. Trial completion date: Dec 2018 --> Apr 2024 | Trial primary completion date: Oct 2018 --> Apr 2024
  • ||||||||||  Review, Journal:  Skeletal Muscle and COVID-19: The Potential Involvement of Bioactive Sphingolipids. (Pubmed Central) -  May 30, 2022   
    This review summarizes the consequences of SARS-CoV-2 infection on SkM and the potential involvement of SLs in the tissue responses to virus infection. In particular, we highlight the role of sphingosine 1-phosphate signaling in order to aid the prediction of novel targets for preventing and/or treating acute and long-term musculoskeletal manifestations of virus infection in COVID-19.
  • ||||||||||  Review, Journal:  The humanistic burden of myasthenia gravis: A systematic literature review. (Pubmed Central) -  May 26, 2022   
    However, all tests should be interpreted with caution by general ophthalmologists. While the literature demonstrates that symptoms associated with MG get better with disease improvement and remission, additional options in efficacious therapy that adequately address the disease-related symptoms and also improve HRQoL may contribute to beneficial outcomes in a greater number of patients with MG.
  • ||||||||||  Review, Journal:  Multiple Sclerosis and Autoimmunity: A Veiled Relationship. (Pubmed Central) -  May 25, 2022   
    This article has compiled various studies elaborating MS and other autoimmune diseases (ADs) co-occurrence. Several conditions that fall into this category, including type 1 diabetes (T1D), rheumatoid arthritis (RA), Guillain-Barre syndrome (GBS), myasthenia gravis (MG), and many others, are found in MS patients and their relatives, suggesting one or more common etiologic mechanisms, including genetic, environmental, and immunological factors, supporting the concept of a possible influence of poly-autoimmunity on MS and the rest of ADs, as well as providing a significant feature for early detection of the disease and also a potential treatment option by clinical neurologists.
  • ||||||||||  Vyvgart (efgartigimod alfa-fcab) / argenx, Broteio
    Journal:  What is in the Neuromuscular Junction Literature? (Pubmed Central) -  May 25, 2022   
    This update covers a number of treatment topics starting with Fc receptor inhibitors and the Federal Drug Administration approval of efgartigimod...The topics of myasthenia gravis and pregnancy, and another look at thymectomy in MG are provided. Finally, a couple of case reports on Lambert-Eaton myasthenic syndrome concentrate on the ice pack test and an autoantibody association with paraneoplastic cerebellar degeneration and Lambert-Eaton myasthenic syndrome in the same patient.