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Toxicity to immune checkpoint inhibitors presenting as pulmonary arterial vasculopathy and rapidly progressing right ventricular dysfunction

Prakash Sanzgiri, Smruti Koppikar, Krishna BA, Pallavi Patil, Charan Reddy


Introduction: Immune Checkpoint Inhibitors (ICIs) are antitumor drugs associated with a number of serious immune-related adverse events (IRAEs). ICIs enhance anti-tumor immunity, thereby  energized  patient's immune system to fight cancer. IRAEs may affect functions of various organs, including heart, and may lead to morbidity and, to some  extent  mortality. Left ventricle (LV) myocarditis with dysfunction is a known side effect of this class of drugs. However, right ventricle (RV) myocarditis and pulmonary vasculitis are an unknown entity and has not been previously reported.  Here, we present the first case of  IRAEs causing selective RV involvement with dysfunctions, attributed to immune checkpoint inhibitors described till date in medical literature.

Presentation of Case: A 58-year male presented  with history of low-grade fever and  weight loss. On palpation, he had diffuse cervical lymphadenopathy. Histopathology evaluation of  lymph node revealed  metastatic lesions of Renal Cell Carcinoma (RCC).

Conclusion: Fatal cardiovascular adverse events can occur as a side effect of ICI. The combination of RV myocarditis with progressive pulmonary hypertension is fatal. Treatment with high dose corticosteroids and immunomodulators may help in patient survival. Physicians treating patients with ICIs should be aware of their lethal cardiotoxic side effects  to reduce adverse cardiac outcomes. Because the number of patients exposed to this new immune therapy is expected to increase remarkably in the near future, our study encourages further work to define guidelines for cardiovascular monitoring and management.


Immune checkpoint inhibitors; myocarditis; pulmonary vasculitis; renal cell carcinoma

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