Key result
Thymoma-associated ICI myositis with cardiac dysfunction carries ~42% mortality, potentially reversible with early immunosuppression.
Why the study?
Thymoma patients are particularly vulnerable to severe immune-related adverse events from immune checkpoint inhibitors, which may rapidly progress to cardiogenic shock and death.
Case Report (n=12)
Thymoma-associated immune checkpoint inhibitor myositis with secondary cardiac dysfunction is a high-mortality overlap syndrome that may be reversible with early aggressive immunosuppression and mechanical circulatory support.
Case of rapid ICI toxicity in thymoma warrants heightened vigilance; leaves open optimal monitoring and management strategies.
Immune checkpoint inhibitor (ICI)-associated toxicities are uncommon but may rapidly progress to cardiogenic shock and death. Patients with thymoma are considered particularly vulnerable to severe immune-related adverse events because thymic malignancy is closely associated with impaired central immune tolerance and autoimmune dysregulation. We report a 34-year-old man with stage IV type B2 thymoma who developed initial myalgia and fatigue approximately 15 days after the most recent pembrolizumab-containing treatment cycle and progressed to fulminant ICI-associated myositis with respiratory failure and secondary cardiogenic shock by day 19. He presented with severe immune-mediated myositis, concurrent myocardial injury, left ventricular systolic dysfunction, cardiogenic shock, metabolic acidosis, acute kidney injury, acute liver injury, coagulopathy, and respiratory failure. Coronary angiography excluded obstructive coronary disease, and microbiological and virological investigations did not identify an infectious cause. Endomyocardial biopsy and cardiac magnetic resonance imaging were not feasible because of profound hemodynamic instability, VA-ECMO support, and coagulopathy. The patient was treated with early venoarterial extracorporeal membrane oxygenation (VA-ECMO), high-dose methylprednisolone, intravenous immunoglobulin, continuous renal replacement therapy, mechanical ventilation, and comprehensive organ support. Cardiac function improved from a left ventricular ejection fraction of 25% to 51%, VA-ECMO was discontinued on hospital day 7, and the patient survived to discharge. This case and a focused literature review suggest that thymoma-associated ICI myositis with secondary cardiac dysfunction may represent a high-risk phenotype characterized by early onset, overlap neuromuscular toxicity, severe hemodynamic compromise, and potential reversibility when early immunosuppression and advanced circulatory support are implemented.
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Liu et al. (2026) conducted a case report in Thymoma-associated immune checkpoint inhibitor myositis with cardiac dysfunction (n=12). Immune checkpoint inhibitors (e.g., Pembrolizumab) was evaluated on Mortality. Thymoma-associated immune checkpoint inhibitor myositis with secondary cardiac dysfunction is a high-risk phenotype with a 41.7% mortality rate, potentially reversible with early immunosuppression.
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