What medications should be avoided in myasthenia gravis patients with risk level for acute flares?

Comment by InpharmD Researcher

Several classes of medications have been implicated in causing de novo myasthenia gravis (MG), MG exacerbation, or MG-like symptoms (see Tables 1 and 2). However, for many of these, the probability of such effects is not explicitly defined, and sometimes ranges from probable to possible, necessitating monitoring if used.

A literature search was conducted in PubMed, Google Scholar, and tertiary drug‐information sources using combinations of the terms “myasthenia gravis,” “drug-induced,” “medication avoidance,” “exacerbation,” and “neuromuscular transmission,” with emphasis on review articles, consensus guidance, and clinically relevant safety data. Relevant publications were screened for medications associated with de novo MG, MG exacerbation, or pharmacologic worsening of neuromuscular transmission, with key evidence identified from a contemporary narrative review and supporting tertiary references.

Background

Drugs that cause de novo myasthenia gravis (MG) do so primarily by disrupting immune homeostasis and promoting autoimmunity against components of the neuromuscular junction. Immune checkpoint inhibitors (ICIs), including PD-1 inhibitors (pembrolizumab, nivolumab), PD-L1 inhibitors (avelumab, atezolizumab), and CTLA-4 inhibitors (ipilimumab), are the most clearly implicated agents. These drugs enhance T-cell activation by blocking inhibitory pathways that normally maintain self-tolerance, increasing the effector-to-regulatory T-cell ratio, stimulating B-cell activation, and promoting autoantibody production and proinflammatory cytokines such as IL-17. ICI-associated MG may occur de novo or as an exacerbation of pre-existing MG, with symptom onset typically within weeks of treatment initiation. It is frequently severe, often associated with respiratory failure, and may overlap with myositis and myocarditis, contributing to high morbidity and mortality. [1]

Alemtuzumab, an anti-CD52 monoclonal antibody, has also been associated with secondary autoimmunity following lymphocyte depletion and repopulation. Although most commonly linked to thyroid disease, rare cases of acetylcholine receptor (AChR) antibody–positive MG have been reported after treatment, suggesting that immune reconstitution may precipitate autoimmune neuromuscular disease. D-penicillamine is a well-established cause of drug-induced MG, occurring in approximately 1–7% of treated patients. The condition is typically mild and often ocular, with AChR antibodies present in the majority of cases. Symptom onset usually occurs months after initiation, and most patients experience remission within months of drug discontinuation. The proposed mechanism involves modification of major histocompatibility complex (MHC) molecules or related immune targets, promoting loss of tolerance and autoantibody formation. [1]

Tyrosine kinase inhibitors (TKIs) have been associated with MG through two possible mechanisms: immune dysregulation leading to autoantibody production and, in certain cases (e.g., tandutinib), direct interference with neuromuscular transmission via inhibition of muscle-specific kinase (MuSK) signaling. Reported cases include AChR antibody–positive MG developing during treatment with agents such as lorlatinib, nilotinib, imatinib, and BRAF/MEK inhibitors, although causality is not always definitively established. Interferon-α therapy has also been linked to de novo MG and exacerbation of pre-existing disease, likely through cytokine-mediated immune activation and alterations in lymphocyte profiles. The type I interferon pathway is broadly implicated in autoimmune pathogenesis, and interferon treatment may promote antibody-mediated autoimmunity at the neuromuscular junction. [1]

Finally, statins have been reported to induce de novo MG or exacerbate established disease. Proposed mechanisms include immune modulation with shifts in T-cell polarization toward a humoral immune response, mitochondrial toxicity affecting neuromuscular junction function, or superimposed myopathy. Although most patients tolerate statins without difficulty, rare cases of AChR antibody–positive MG have been documented, sometimes recurring upon rechallenge. Overall, these agents induce MG by promoting autoimmune responses rather than directly impairing neuromuscular transmission, distinguishing them mechanistically from drugs that cause MG-like symptoms through pharmacologic interference at the neuromuscular junction. [1]

References: [1] Sheikh S, Alvi U, Soliven B, Rezania K. Drugs That Induce or Cause Deterioration of Myasthenia Gravis: An Update. J Clin Med. 2021;10(7):1537. Published 2021 Apr 6. doi:10.3390/jcm10071537
Literature Review

A search of the published medical literature revealed 2 studies investigating the researchable question:

What medications should be avoided in myasthenia gravis patients with risk level for acute flares?

Level of evidence

C - Multiple studies with limitations or conflicting results  Read more→



Please see Tables 1-2 for your response.


Drugs reported to cause de novo MG or MG exacerbation through altering the immune response
Drug Mechanism ADR Probability Comments
Immune Checkpoint inhibitors T cell activation
Increased ratio of T effector to T regulatory cells, B cell activation, autoantibody production, cytokines such as IL-17
Definite Avoid after emergence of life-threatening MG
If to be used in MG patients, pre-treat with steroids, IVIG, or plasmapheresis
D-Penicillamine Modification of MHC or other molecules on the surface of antigen-presenting cells Definite Discontinue and avoid if MG occurs
Tyrosine kinase inhibitors * Unspecified immune dysregulation
Inhibition of neuromuscular transmission (with tandutinib)
Doubtful (probable with tandutinib) Not contraindicated, association rarely reported
Interferons Immune dysregulation through changes in cytokines, natural killer cells, alteration of lymphocyte profiles Possible Not contraindicated, association rarely reported
Statins * Shift in T cell polarization
Superimposed myopathy
Mitochondrial toxicity
Probable Discontinue and avoid in rare cases of emergence or exacerbation of MG

*also may affect neuromuscular transmission

ADR: adverse drug reaction

References:
[1] [1] Sheikh S, Alvi U, Soliven B, Rezania K. Drugs That Induce or Cause Deterioration of Myasthenia Gravis: An Update. J Clin Med. 2021;10(7):1537. Published 2021 Apr 6. doi:10.3390/jcm10071537
Drugs associated with MG-like symptoms, unmasking and exacerbation of MG, through their adverse effect on neuromuscular transmission
Drug Mechanism ADR Probability Comments
Macrolides Impair neuromuscular transmission, possibly at presynaptic level Definite Avoid in MG patients if there is another alternative, otherwise closely monitor
Fluoroquinolones Impair neuromuscular transmission, pre and postsynaptic levels Probable Avoid in MG patients if there is another alternative, otherwise closely monitor
Aminoglycosides Impair neuromuscular transmission, pre and postsynaptic levels Definite Avoid in MG patients if there is another alternative, otherwise closely monitor
Penicillins Unclear, impaired neuromuscular transmission in an animal model Probable Can be used in MG patients as MG exacerbation is rare
β-adrenergic blockers Unclear effect on neuromuscular transmission Possible Can be used in stable MG patients, monitor closely, especially early after starting.
L type Calcium channel blockers Unclear effect on neuromuscular transmission Possible Can be used in stable MG patients, monitor closely, especially early after starting.
Class Ia antiarrhythmics Impair neuromuscular transmission, pre and postsynaptic levels Definite Avoid in MG patients if there is another alternative, otherwise closely monitor
Magnesium Presynaptic (blocks release of ACh) and postsynaptic Definite Caution and close monitoring are advised in magnesium replacement (specially parenteral) in MG patients
Neuromuscular blockers and inhalation anesthetics Postsynaptic neuromuscular block Definite Nondepolarizing NMBs and inhalation anesthetics better be avoided; if used, observe close postop monitoring, consider using acetylcholinesterase inhibitor and sugammadex
Antipsychotics Impair neuromuscular transmission at presynaptic and postsynaptic levels Possible Can be used in MG patients as MG exacerbation is rarely reported
Lithium * Presynaptic: reduction in ACh synthesis and release; postsynaptic: reduction in number of AChRs Probable Can be used in MG patients as MG exacerbation is rarely reported
Corticosteroids Unknown; possible direct effect on neuromuscular transmission at high doses Definite Avoid starting high doses, if high doses are to be started, consider pretreatment with IVIG or plasmapheresis
Botulinum toxin Presynaptic reduction in ACh release Definite Avoid if possible, may be offered with caution and slow dose titration to patients with mild/stable MG who also have blepharospasm or cervical dystonia
*reported to cause de novo MG
References:
[1] [1] Sheikh S, Alvi U, Soliven B, Rezania K. Drugs That Induce or Cause Deterioration of Myasthenia Gravis: An Update. J Clin Med. 2021;10(7):1537. Published 2021 Apr 6. doi:10.3390/jcm10071537