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Immune-related myocarditis

aka immune myocarditis, checkpoint myocarditis, myocarditis, immune-mediated myocarditis, troponin, troponin monitoring, myositis, myasthenia, myasthenia gravis, immune-related myositis

Rare but frequently fatal inflammation of the heart muscle triggered by checkpoint inhibitors, usually in the first two months. It often comes with muscle and nerve-muscle inflammation, and needs urgent high-dose steroids.

Occurs in about 1% of patients (more with combination CTLA-4 plus PD-1 and with some other combinations) with mortality of 25-50%; presents with chest pain, breathlessness, arrhythmia or heart block, often alongside myositis and myasthenia (the 'triad'). Diagnosis rests on troponin, ECG, echocardiography, cardiac MRI and biopsy; treatment is immediate methylprednisolone with abatacept, mycophenolate or ruxolitinib for steroid-refractory cases, and the drug is permanently stopped. Baseline and early troponin monitoring is debated. Distinct from anthracycline and trastuzumab cardiomyopathy, which damage the heart without inflammation.

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