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Immune Checkpoint Inhibitor-Induced Myocarditis, M ...
Immune Checkpoint Inhibitor-Induced Myocarditis, Myositis, and Myasthenia Gravis Overlap Syndrome
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This case report describes a 76-year-old woman with metastatic melanoma who developed a life-threatening immune checkpoint inhibitor (ICI) toxicity syndrome after her first infusion of nivolumab and relatlimab. About three weeks later, she presented with 2 weeks of progressive myalgias, weakness, diplopia, and dyspnea, along with severe hypoxemia and exam findings of bilateral ptosis and abducens nerve palsy.<br /><br />Laboratory studies showed marked muscle and cardiac injury, including CK 8,494 U/L and high-sensitivity troponin peaking at 1,995 ng/L, plus elevated liver enzymes and LDH. Her echocardiogram was normal with preserved ejection fraction, and CT chest/head were unrevealing. EMG showed myopathic changes without evidence of neuromuscular transmission disorder. Autoimmune testing, including acetylcholine receptor and MuSK antibodies, was negative.<br /><br />She was diagnosed with the rare “triple M” overlap syndrome: ICI-induced myocarditis, myositis, and myasthenia gravis. Her course deteriorated quickly with worsening respiratory failure, requiring ICU transfer, intubation based on low negative inspiratory force and vital capacity, and later tracheostomy. She ultimately improved enough for tracheostomy decannulation but had a prolonged, complicated hospitalization including MRSA ventilator-associated pneumonia, perforated diverticulitis requiring surgery, upper GI bleeding, and another brief intubation after aspiration.<br /><br />The report emphasizes that ICI-related myocarditis has high mortality and may occur together with myositis and myasthenia gravis. Early recognition is critical, especially because respiratory failure is a feared complication. Recommended management includes prompt high-dose IV corticosteroids, early IVIG or plasmapheresis when MG is suspected, and additional immunosuppressive agents in refractory or severe cases. The authors stress the need for multidisciplinary care and close monitoring of respiratory function.
Asset Subtitle
Daria Seriak
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Author List
Daria Seriak, Matthew Basciotta
Category
Clinical Vignettes
Concept
Immune Checkpoint Inhibitor
Concept
Myocarditis
Concept
Myositis
Concept
Myasthenia Gravis
Concept
Corticosteroids
Distinguished
Non-Finalist
Presenter Organization
Mass General Brigham-Salem Hospital
Presenting Author
Daria Seriak
Track
Adult
Keywords
metastatic melanoma
nivolumab
relatlimab
immune checkpoint inhibitor toxicity
myocarditis
myositis
myasthenia gravis
triple M overlap syndrome
respiratory failure
high-dose corticosteroids
Immune Checkpoint Inhibitor
Myocarditis
Myositis
Myasthenia Gravis
Corticosteroids
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