What is the management of a myasthenia gravis (Myasthenia Gravis, MG) exacerbation?

From the Guidelines

Management of a myasthenia gravis flare requires immediate intervention, with inpatient admission considered for all grades due to the potential for rapid deterioration and respiratory compromise. The approach to management is guided by the severity of the flare, with grading based on symptoms and impact on activities of daily living (ADLs) [ 1 ].

Grading and Management

• For G2 symptoms (some symptoms interfering with ADLs, MGFA severity class I and II), holding immune checkpoint inhibitors (ICPi) and possibly resuming them if symptoms resolve, along with neurology consultation, is recommended [ 1 ].
• Pyridostigmine is initiated at 30 mg PO three times a day and can be increased up to 120 mg PO four times a day as tolerated and based on symptoms [ 1 ].
• Corticosteroids, such as prednisone 0.5 mg/kg orally daily, are administered and weaned based on symptom improvement [ 1 ].

Severe Flares

• For G3-4 symptoms (limiting self-care, weakness limiting walking, ANY dysphagia, facial weakness, respiratory muscle weakness, or rapidly progressive symptoms), permanent discontinuation of ICPi, admission for possible ICU-level monitoring, and continuation of steroids with a taper starting 3-4 weeks after initiation are recommended [ 1 ].
• Initiation of IVIG 2 G/kg IV over 5 days or plasmapheresis for 3-5 days, and consideration of adding rituximab if refractory to IVIG or plasmapheresis, are also part of the management [ 1 ].
• Frequent pulmonary function assessment and daily neurologic review are crucial in these cases [ 1 ].

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 The treatment of the two conditions obviously differs radically. Whereas the presence of myasthenic crisis suggests the need for more intensive anticholinesterase therapy, the diagnosis of cholinergic crisis, according to Osserman and Genkins1, calls for the prompt withdrawal of all drugs of this type.

The management of a myasthenia gravis flare (myasthenic crisis) may involve:

• More intensive anticholinesterase therapy Key points to consider:
• Differentiation between myasthenic crisis and cholinergic crisis is crucial, as the treatment approaches differ significantly.
• For detailed information on the management of patients with myasthenia gravis, the physician is referred to reviews such as those by Osserman and Genkins2

From the Research

Management of Myasthenia Gravis Flare

The management of a myasthenia gravis (MG) flare is crucial to control symptoms and prevent further complications. The following are some key points to consider:

• Symptomatic treatment: Acetylcholinesterase inhibitors such as pyridostigmine are commonly used to control symptoms 3, 4, 5.
• Immunosuppressive therapy: Corticosteroids are the mainstay of immunosuppressive treatment in patients with more than mild MG to induce remission 6, 4, 7.
• Thymectomy: Thymectomy is indicated for thymoma and is an option for acetylcholine receptor antibody-positive patients with generalized weakness developing under the age of 45 years 6, 4.
• Plasma exchange or intravenous immunoglobulin: These treatments may provide short-term benefit in patients with severe weakness or MG crisis 6, 7.
• Individualized treatment: Treatment needs to be individualized based on disease severity, antibody status, comorbidities, and other factors 4, 5.

Treatment Options

Some other treatment options for MG include:

• Azathioprine: An immunosuppressive therapy that can be used in addition to or instead of corticosteroids 6, 4.
• Mycophenolate mofetil: An option for patients who are intolerant of azathioprine 6.
• Rituximab: A treatment option for refractory MG 4.
• Active physical training: Encouraged to improve quality of life and functional ability 7.