What is the plan of care for a patient with Myasthenia Gravis (MG) in a hospital setting?

From the Guidelines

A comprehensive hospital plan of care for Myasthenia Gravis (MG) should prioritize symptomatic treatment, management of acute exacerbations, and prevention of respiratory compromise, as outlined in the most recent guidelines 1. The plan should include initial treatment with acetylcholinesterase inhibitors such as pyridostigmine, starting at 30 mg PO three times a day and gradually increasing to a maximum of 120 mg PO four times a day as tolerated and based on symptoms 1. For immunosuppression, corticosteroids such as prednisone should be administered, with a dose of 0.5 mg/kg orally daily for grade 2 symptoms and 1-1.5 mg/kg orally daily for more severe symptoms, with tapering based on symptom improvement 1. In cases of acute exacerbations or myasthenic crisis (grade 3-4), permanent discontinuation of immune checkpoint inhibitors, admission to the hospital or ICU, and initiation of IVIG or plasmapheresis should be considered, along with frequent pulmonary function assessment and daily neurologic review 1. It is also essential to avoid medications that can exacerbate MG, including certain antibiotics, beta-blockers, and magnesium, and to consider thymectomy for patients with thymoma or generalized MG 1. Key aspects of the plan include:

• Symptomatic treatment with pyridostigmine and corticosteroids
• Management of acute exacerbations with IVIG or plasmapheresis
• Prevention of respiratory compromise through close monitoring and early intervention
• Avoidance of exacerbating medications
• Consideration of thymectomy for eligible patients By following this comprehensive plan, patients with MG can receive optimal care and management, reducing the risk of morbidity, mortality, and improving quality of life.

From the FDA Drug Label

Although failure of patients to show clinical improvement may reflect underdosage, it can also be indicative of overdosage. As is true of all cholinergic drugs, overdosage of pyridostigmine bromide may result in cholinergic crisis, a state characterized by increasing muscle weakness which, through involvement of the muscles of respiration, may lead to death Myasthenic crisis due to an increase in the severity of the disease is also accompanied by extreme muscle weakness, and thus may be difficult to distinguish from cholinergic crisis on a symptomatic basis For detailed information on the management of patients with myasthenia gravis, the physician is referred to one of the excellent reviews such as those by Osserman and Genkins2, Grob3 or Schwab4,5.

The plan of care for Myasthenia Gravis in a hospital setting is not directly addressed in the provided drug label.

• The label provides information on the differential diagnosis of myasthenic crisis and cholinergic crisis.
• It also mentions the importance of clinical judgment in managing patients with myasthenia gravis.
• However, for detailed information on the management of patients with myasthenia gravis, the physician is referred to external reviews 2.

From the Research

Myasthenia Gravis Plan of Care in a Hospital Setting

• The plan of care for Myasthenia Gravis (MG) in a hospital setting should focus on prompt symptom control and the induction of remission or minimal manifestations 3.
• Symptom control can be achieved with acetylcholinesterase inhibitors such as pyridostigmine, which is commonly employed and may be sufficient in mild disease 3.
• In cases of myasthenic crisis, treatment should target the inciting event and airway support, with consideration given to noninvasive positive-pressure ventilation for ventilatory support 4.
• Corticosteroids are the mainstay of immunosuppressive treatment in patients with more than mild MG to induce remission, while immunosuppressive therapies such as azathioprine can be prescribed in addition to or instead of corticosteroids 3.

Management of Myasthenic Crisis

• Myasthenic crisis is a clinical diagnosis defined by respiratory failure caused by exacerbation of MG, and can be precipitated by any stressor, with infection being the most common 4.
• Treatment of myasthenic crisis should focus on ruling out infection and respiratory support, with strong consideration given to beginning with noninvasive positive-pressure ventilation for ventilatory support 4.
• All acetylcholinesterase inhibitors should be avoided in crisis, including edrophonium testing and corticosteroids initially, while depolarizing paralytics and acetylcholinesterase inhibitors should be avoided in patients with myasthenic crisis in the emergency department 4.

Treatment Options

• Pyridostigmine is the most commonly used drug in the symptomatic treatment of MG, with a median effectiveness of 60 and net benefit of 65, but can cause side effects such as flatulence, urinary urgency, and muscle cramps 5.
• Azathioprine has been shown to be effective in the long-term treatment of MG, with marked clinical improvement and reduction in the dose of pyridostigmine and prednisolone 6.
• Novel therapies are being developed and trialed, including ones that inhibit complement-induced immunological pathways or interfere with antibody-recycling pathways 3.