Immune Checkpoint Inhibition-Related Myasthenia-Myositis-Myocarditis Responsive to Complement Blockade

Immune checkpoint inhibitors (ICIs) have revolutionized cancer therapy but come with immune-related adverse events (irAEs) that provide a novel challenge for treating physicians. Neuromuscular irAEs, including myositis, myasthenia gravis (MG), and demyelinating polyradiculoneuropathy, lead to signif...

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Veröffentlicht in:Neurology : neuroimmunology & neuroinflammation 2024-01, Vol.11 (1)
Hauptverfasser: Nelke, Christopher, Pawlitzki, Marc, Kerkhoff, Ruth, Schroeter, Christina B, Aktas, Orhan, Neuen-Jacob, Eva, Polzin, Amin, Meuth, Sven G, Ruck, Tobias
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Sprache:eng
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Zusammenfassung:Immune checkpoint inhibitors (ICIs) have revolutionized cancer therapy but come with immune-related adverse events (irAEs) that provide a novel challenge for treating physicians. Neuromuscular irAEs, including myositis, myasthenia gravis (MG), and demyelinating polyradiculoneuropathy, lead to significant morbidity and mortality. We present a case of severe myasthenia-myositis-myocarditis overlap in a patient receiving ICIs for breast cancer. Clinical findings were recorded. A 47-year-old woman developed tetraparesis, dysphagia, and muscle pain during ICI treatment. MG with a thymoma had been diagnosed earlier. Neuromuscular overlap irAEs with cardiac affection was confirmed, and ICI treatment was discontinued. Given a lack of clinical response to standard therapies, a muscle biopsy was performed demonstrating complement deposition. Eculizumab treatment led to rapid improvement in muscle strength and cardiac function. Neuromuscular irAEs are associated with a high in-hospital mortality, and specific treatment strategies remain an unmet need. Here, early muscle biopsy enabled targeted therapy after standard approaches failed, thereby highlighting the value of identifying a specific treatment target. To improve therapeutic outcomes, the development of patient-tailored strategies for neuromuscular irAEs requires further studies.
ISSN:2332-7812
2332-7812
DOI:10.1212/NXI.0000000000200177