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A clinical perspective on muscle specific kinase antibody positive myasthenia gravis - PubMed

A clinical perspective on muscle specific kinase antibody positive myasthenia gravis - PubMed

Source : https://pubmed.ncbi.nlm.nih.gov/39703505/

The discovery of autoantibodies directed against muscle-specific kinase (MuSK) in "seronegative" myasthenia gravis (MG) patients marked a milestone in MG research. In healthy muscle, MuSK regulates a phosphorylation pathway, which...

MuSK-MG is an autoimmune disorder characterized by IgG4 antibodies targeting muscle-specific kinase, impairing acetylcholine receptor clustering. Rituximab is effective in treatment, improving clinical outcomes compared to corticosteroids and immunosuppressants.

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Inebilizumab treatment in a patient with co-occurring AQP4-IgG positive neuromyelitis optica spectrum disorder and myasthenia gravis: a case report and literature review - PubMed

Inebilizumab treatment in a patient with co-occurring AQP4-IgG positive neuromyelitis optica spectrum disorder and myasthenia gravis: a case report and literature review - PubMed

Source : https://pubmed.ncbi.nlm.nih.gov/39872531/

Though MG typically precedes NMOSD and thymectomy is frequently performed, it is not a prerequisite for NMOSD development but may represent a potential risk factor. MG generally follows a benign...

This study explores the clinical features and treatment strategies for concurrent neuromyelitis optica spectrum disorder (NMOSD) and myasthenia gravis (MG), highlighting the efficacy of inebilizumab in disease control and relapse prevention.

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VYVGART Hytrulo: the first and only IgG Fc-antibody fragment for the treatment of adults with anti-AChR antibody positive gMG is available for subcutaneous administration1,2

VYVGART Hytrulo: another way to deliver individualized dosing to your patients

  • Four once-weekly injections per treatment cycle
  • Flexibility in meeting patients' needs with HCP administration in office
  • Fast delivery for your patients—typically ~30-90–second injection*
  • Fixed dose (1,008 mg efgartigimod alfa and 11,200 units hyaluronidase)

EXPLORE INDIVIDUALIZED DOSING

The overall safety profile of VYVGART Hytrulo, except for a higher rate of injection site reactions, was consistent with the proven safety profile of VYVGART® (efgartigimod alfa-fcab)1,3

In the ADAPT clinical trial, the most common adverse reactions for VYVGART-treated patients were respiratory tract infection, headache, and urinary tract infection. Additionally, a higher frequency of patients who received VYVGART compared to placebo were observed to have below normal levels for white blood cell counts (12% vs 5%), lymphocyte counts (28% vs 19%), and neutrophil counts (13% vs 6%) that were mild to moderate in severity.

In ADAPT-SC, injection site reactions occurred in 38% of patients receiving VYVGART Hytrulo. These were injection site rash, erythema, pruritus, bruising, pain, and urticaria.

SEE FULL SAFETY PROFILES

Could VYVGART Hytrulo be right for your adult patients who prefer subcutaneous administration?

EXPLORE VYVGART Hytrulo

*Refers to actual injection time of VYVGART Hytrulo. Allow for appropriate storage, preparation, and setup time before use.

AChR=acetylcholine receptor; Fc=fragment, crystallized; IgG=immunoglobulin G; gMG=generalized myasthenia gravis; HCP=healthcare provider.

Would your adult patients with anti-AChR antibody positive gMG be interested in a subcutaneous treatment option?

How would you determine which patients could be right for a subcutaneous treatment option?

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IMPORTANT SAFETY INFORMATION AND INDICATION

CONTRAINDICATIONS

VYVGART HYTRULO is contraindicated in patients with serious hypersensitivity to efgartigimod alfa products, to hyaluronidase, or to any of the excipients of VYVGART HYTRULO. Reactions have included anaphylaxis and hypotension leading to syncope.

WARNINGS AND PRECAUTIONS

Infection

VYVGART HYTRULO may increase the risk of infection. The most common infections observed in Study 1 were urinary tract infection (10% of efgartigimod alfa-fcab-treated patients vs 5% of placebo-treated patients) and respiratory tract infections (33% of efgartigimod alfa-fcab-treated patients vs 29% of placebo-treated patients). Patients on efgartigimod alfa-fcab vs placebo had below normal levels for white blood cell counts (12% vs 5%, respectively), lymphocyte counts (28% vs 19%, respectively), and neutrophil counts (13% vs 6%, respectively). The majority of infections and hematologic abnormalities were mild to moderate in severity. Delay VYVGART HYTRULO administration in patients with an active infection until the infection has resolved; monitor for clinical signs and symptoms of infections. If serious infection occurs, administer appropriate treatment and consider withholding VYVGART HYTRULO until the infection has resolved.

Immunization

Immunization with vaccines during VYVGART HYTRULO treatment has not been studied; the safety with live or live-attenuated vaccines and the response to immunization with any vaccine are unknown. Because VYVGART HYTRULO causes a reduction in immunoglobulin G (IgG) levels, vaccination with live-attenuated or live vaccines is not recommended during VYVGART HYTRULO treatment. Evaluate the need to administer age-appropriate vaccines according to immunization guidelines before initiation of a new treatment cycle with VYVGART HYTRULO.

Hypersensitivity Reactions

In clinical trials, hypersensitivity reactions, including rash, angioedema, and dyspnea were observed in patients treated with VYVGART HYTRULO or intravenous efgartigimod alfa-fcab. Urticaria was also observed in patients treated with VYVGART HYTRULO. Hypersensitivity reactions were mild or moderate, occurred within 1 hour to 3 weeks of administration, and did not lead to treatment discontinuation. Anaphylaxis and hypotension leading to syncope have been reported in postmarketing experience with intravenous efgartigimod alfa-fcab. Anaphylaxis and hypotension occurred during or within an hour of administration and led to infusion discontinuation and in some cases to permanent treatment discontinuation. Healthcare professionals should monitor for clinical signs and symptoms of hypersensitivity reactions for at least 30 minutes after administration. If a hypersensitivity reaction occurs, the healthcare professional should institute appropriate measures if needed or the patient should seek medical attention.

Infusion-Related Reactions

Infusion-related reactions have been reported with intravenous efgartigimod alfa-fcab in postmarketing experience. The most frequent symptoms and signs were hypertension, chills, shivering, and thoracic, abdominal, and back pain. Infusion-related reactions occurred during or within an hour of administration and led to infusion discontinuation. If a severe infusion-related reaction occurs, initiate appropriate therapy. Consider the risks and benefits of readministering VYVGART HYTRULO following a severe infusion-related reaction. If a mild to moderate infusion-related reaction occurs, patients may be rechallenged with close clinical observation, slower infusion rates, and pre-medications.

ADVERSE REACTIONS

In Study 1, the most common (≥10%) adverse reactions in efgartigimod alfa-fcab-treated patients were respiratory tract infection, headache, and urinary tract infection. In Study 2, the most common (≥10%) adverse reactions in VYVGART HYTRULO-treated patients were injection site reactions and headache. Injection site reactions occurred in 38% of VYVGART HYTRULO-treated patients, including injection site rash, erythema, pruritus, bruising, pain, and urticaria. In Study 2 and its open-label extension, all injection site reactions were mild to moderate in severity and did not lead to treatment discontinuation. The majority occurred within 24 hours after administration and resolved spontaneously. Most injection site reactions occurred during the first treatment cycle, and the incidence decreased with each subsequent cycle.

USE IN SPECIFIC POPULATIONS

Pregnancy

As VYVGART HYTRULO is expected to reduce maternal IgG antibody levels, reduction in passive protection to the newborn is anticipated. Risks and benefits should be considered prior to administering live or live-attenuated vaccines to infants exposed to VYVGART HYTRULO in utero.

Lactation

There is no information regarding the presence of efgartigimod alfa or hyaluronidase, from administration of VYVGART HYTRULO, in human milk, the effects on the breastfed infant, or the effects on milk production. Maternal IgG is known to be present in human milk. The developmental and health benefits of breastfeeding should be considered along with the mother’s clinical need for VYVGART HYTRULO and any potential adverse effects on the breastfed infant from VYVGART HYTRULO or from the underlying maternal condition.

INDICATION

VYVGART® HYTRULO (efgartigimod alfa and hyaluronidase-qvfc) is indicated for the treatment of generalized myasthenia gravis in adult patients who are anti-acetylcholine receptor (AChR) antibody positive.

Please see the full Prescribing Information.

You may report side effects to the US Food and Drug Administration by visiting http://www.fda.gov/medwatch or calling 1-800-FDA-1088. You may also report side effects to argenx US, Inc, at 1-833-argx411 (1-833-274-9411).

References: 1. VYVGART Hytrulo. Prescribing information. argenx US Inc; 2024. 2. Wolfe GI et al. J Neurol Sci. 2021;430:118074. doi:10.1016/j.jns.2021.118074 3. VYVGART. Prescribing information. argenx US Inc; 2024.

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For US audiences only.

VYVGART is a registered trademark of argenx.

VYVGART Hytrulo is a trademark of argenx.

© 2024 argenx US-VYV-23-00218v2 03/2024. All Rights Reserved.

 

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Physiological and pathological roles of the thymus and value of thymectomy in myasthenia gravis: a narrative review - PubMed

Physiological and pathological roles of the thymus and value of thymectomy in myasthenia gravis: a narrative review - PubMed

Source : https://pubmed.ncbi.nlm.nih.gov/38881805/

Overall, in light of the known pathogenesis and association of the thymus with MG, thymectomy remains an extremely effective approach for long-term management and improved clinical outcomes.

This review explores thymectomy's role in managing Myasthenia Gravis (MG), highlighting its effectiveness in improving clinical outcomes, addressing thymic pathologies, and examining factors influencing patient outcomes and complications.

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Transient Neonatal Myasthenia Gravis as a Common Complication of a Rare Disease: A Systematic Review - PubMed

Transient Neonatal Myasthenia Gravis as a Common Complication of a Rare Disease: A Systematic Review - PubMed

Source : https://pubmed.ncbi.nlm.nih.gov/38398450/

Myasthenia gravis (MG) is a rare autoimmune disease. Transient neonatal myasthenia gravis (TNMG) is caused by pathogenic maternal autoantibodies that cross the placenta and disrupt signaling at the neuromuscular junction....

Transient neonatal myasthenia gravis (TNMG) is a rare, IgG-mediated autoimmune disease affecting neonates of mothers with myasthenia gravis, often self-limiting, but can cause severe symptoms or permanent complications.