Myasthenia Gravis
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  • ||||||||||  Review, Journal:  New-onset Myasthenia Gravis after SARS-CoV-2 infection: case report and literature review. (Pubmed Central) -  Nov 16, 2022   
    This is a report of new-onset Myasthenia Gravis correlated to COVID-19 in which thymic resection was described and the histologic analysis of the thymus was performed showing thymic hyperplasia despite negative thoracic Magnetic Resonance Imaging. SARS-CoV-2 infection releases inflammatory cytokines that could dysregulate the immune system and lead to Myasthenia Gravis in susceptible subjects.
  • ||||||||||  Review, Journal:  Granulomatous myopathy: Sarcoidosis and beyond. (Pubmed Central) -  Nov 16, 2022   
    In cases of unexplained granulomatous myopathy, natural killer/T-cell lymphoma should be considered and careful histopathological examination for atypical cells and appropriate immunostaining is crucial. Identifying the etiology of granulomatous myopathy in each patient can guide appropriate treatment.
  • ||||||||||  Journal:  Developing and validating a nomogram to predict myasthenia gravis exacerbation in patients with postoperative thymoma recurrence. (Pubmed Central) -  Nov 16, 2022   
    The training cohort were 75.7% (95% CI: 66.1-83.4%), 64.7% (95% CI: 38.6-84.7%), 92.9% (95% CI: 84.5-97.1%) and 30.6% (95% CI: 16.9-48.3%) respectively, while the corresponding validation cohort were 84.1% (95% CI: 69.3-92.8%), 66.7% (95% CI: 35.4-88.7%), 90.2% (95% CI: 75.9-96.8%) and 53.3% (95% CI: 27.4-77.7%) respectively. We identified the risk factors for MG exacerbation in patients with postoperative recurrence of thymoma and drew a nomogram, which can be used to calculate the probability of MG exacerbation and guide clinicians to choose post-operative treatment.
  • ||||||||||  Journal:  Diplopia: characteristics and etiologic distribution in a referral-based university hospital. (Pubmed Central) -  Nov 16, 2022   
    We identified the risk factors for MG exacerbation in patients with postoperative recurrence of thymoma and drew a nomogram, which can be used to calculate the probability of MG exacerbation and guide clinicians to choose post-operative treatment. Given the differences in the etiologic distribution of diplopia according to the patients' age and the specialties involved in the management, the results of previous reports on the characteristics and etiology of diplopia, primarily performed in a single specialty department, should be interpreted with a possible selection bias.
  • ||||||||||  Review, Journal:  New Targeted Agents in Myasthenia Gravis and Future Therapeutic Strategies. (Pubmed Central) -  Nov 16, 2022   
    Positive clinical trial results have been reported, and other studies are ongoing. Finally, we briefly discuss how the introduction of these novel targeted immunological therapies in a changing management paradigm would affect not only clinical outcomes, disease burden, safety, and tolerability, but also health spending in a condition that is increasingly managed based on a patient-centred model.
  • ||||||||||  Journal, Adverse events, Checkpoint inhibition:  Neurological adverse events of immune checkpoint blockade: from pathophysiology to treatment. (Pubmed Central) -  Nov 15, 2022   
    Recent advances in the knowledge of pathophysiological mechanisms (behind these toxicities) should be taken into account for the management of these patients. Drugs targeting T-cell mediated inflammation should be preferred in patients who are refractory to steroids, whereas therapies targeting humoral mechanisms should be considered in specific cases associated with autoantibodies such as immune-related myasthenic syndrome.
  • ||||||||||  Review, Journal:  Neuromuscular Weakness in Intensive Care. (Pubmed Central) -  Nov 13, 2022   
    Care benefits from a multidisciplinary approach including a neuromuscular specialist, rehabilitation services, and a specialty pharmacist in addition to the neurocritical care team. In general, survivors achieve a good functional recovery relative to their preintensive care unit baseline.
  • ||||||||||  Rituxan (rituximab) / Biogen, Zenyaku Kogyo, Roche
    Trade Ya: An Interesting Twist on Eating Disorder Diagnosis Swapped for Myasthenia Gravis (Anaheim Convention Center, Hall A, Board # 155) -  Nov 10, 2022 - Abstract #AAPNCE2022AAP_NCE_2350;    
    Since the diagnosis, she has received multiple rounds of IVIG and plasma exchange, and most recently was initiated on weekly rituximab infusions given difficulty in treating Anti-MuSk positive MG with just pyridostigmine [1]... It is now thought that her ARFID symptoms were manifestations of MG rather than an eating disorder.
  • ||||||||||  Humira (adalimumab) / Eisai, AbbVie
    All Eyes on the Gastrointestinal Tract: Orbital Pseudotumor Leading to Diagnosis of Crohn's Disease (Anaheim Convention Center, Hall A, Board # 185) -  Nov 10, 2022 - Abstract #AAPNCE2022AAP_NCE_2278;    
    This case exemplifies the importance of remembering the rare association between orbital pseudotumor and Crohn’s disease in children. This can help in early diagnosis and management of Crohn’s disease, which will eventually help improve the patient’s symptom profile and also prevent EIMs.
  • ||||||||||  Journal:  Smoking and younger age at onset in anti-acetylcholine receptor antibody-positive myasthenia gravis. (Pubmed Central) -  Nov 9, 2022   
    Among patients with smoking exposure within 10 years prior to or at the onset of MG, women were significantly younger at the onset of MG compared with men. Our results suggest that smoking is an independent risk factor for the earlier development of anti-acetylcholine receptor antibody-positive MG and further support the putative link between smoking and MG.
  • ||||||||||  Journal:  Distal upper limb involvement in myasthenia-myositis association. (Pubmed Central) -  Nov 8, 2022   
    We provide the histopathological evidence of a prominent inflammatory process responsible of distal upper limb weakness in MG-IM association. Muscle biopsy does not reveal any typical histopathological feature of other nosologically definite inflammatory myopathy, leading MG-IM association to come close to the group of overlap-myositis (OM) with the myopathological features of non-specific myositis (NSM).
  • ||||||||||  Keytruda (pembrolizumab) / Merck (MSD)
    Journal:  Pembrolizumab-induced Myasthenia Gravis Relapse After Immunosuppressive Therapy. (Pubmed Central) -  Nov 8, 2022   
    irAE-MG is a rare disease that has not yet been fully characterized, and our case shows that MG symptoms may relapse. Therefore, regular follow-up is necessary, even after the symptoms improve with immunosuppressive therapy.
  • ||||||||||  Descartes-08 / Cartesian Therap
    Phase classification, Enrollment change, Trial primary completion date, CAR T-Cell Therapy:  Descartes-08 CAR-T Cells in Generalized Myasthenia Gravis (MG) (clinicaltrials.gov) -  Nov 8, 2022   
    P2,  N=30, Recruiting, 
    Significant racial differences exist in both treatment utilization and inpatient outcomes for patients hospitalized for MG. Phase classification: P1/2 --> P2 | N=18 --> 30 | Trial primary completion date: Dec 2022 --> Dec 2023
  • ||||||||||  nipocalimab (M281) / J&J
    Mode of Nipocalimab Action and Pharmacology in Cells and Preclinical Models (ENMCC - Hall D) -  Nov 4, 2022 - Abstract #ASH2022ASH_5430;    
    P2/3
    Nipocalimab is under clinical investigation for multiple IgG-mediated autoantibody disorders, including wAIHA. The close mechanistic relationship between nipocalimab concentration, FcRn occupancy and inhibition of IgG recycling resulting in rapid, deep and sustained IgG lowering in vivo is consistent between preclinical and clinical studies.
  • ||||||||||  Plasma Exchange for Immune-Related Adverse Events Due to Immune Checkpoint Inhibitor Therapy: Implications for Clinical Care and Clinical Trial Design (ENMCC - Hall D) -  Nov 4, 2022 - Abstract #ASH2022ASH_2032;    
    ICI constructs were IgG1 anti-PD-L1 (envafolimab, durvalumab) and IgG4 anti-PD-1 (nivolumab, cemiplimab)...Grade 4 bleeding occurred in the patient receiving enoxaparin and DAPT and led to discontinuation of TPE after 2 sessions. Our experience supports the following when considering TPE for irAEs: 1) exclusion of patients with high bleeding risk, or use of 100% FFP replacement and every other day TPE; 2) meticulous non-biomarker irAE assessment during and after TPE; 3) RCT design includes irAE relapse timing and rates and long term follow-up to assess cumulative immunosuppression and tumor outcome; 4) serial drug levels and correlative studies (e.g. cytokines, autoantibodies) to better understand TPE mechanisms, persistence of irAEs, and long term ICI anti-tumor benefit.
  • ||||||||||  Journal:  SOX-1 antibodies in a patient with Crohn's disease: a case report. (Pubmed Central) -  Nov 4, 2022   
    As of now, testing for anti-neuronal antibodies appeared unnecessary within the diagnostic assessment of gastroenterological disorders, which may lead to overlooking incident neurologic autoimmune diseases. Further exploration of such research hypothesis in clinical grounds appears intriguing.
  • ||||||||||  Journal:  Like a Game of Chess, Every Move Matters: The Role of Antibodies in the Myasthenia Gravis Treatment Landscape. (Pubmed Central) -  Nov 3, 2022   
    New treatments have the potential of being both effective and well tolerated, addressing the trade-off of choosing either efficacy or tolerability when selecting treatments. Promising investigational treatments that may become available in the future may allow more patients than ever before to achieve an asymptomatic state, with the ultimate goal being to turn off abnormal antibody production.