Pembrolizumab and epacadostat induced fatal myocarditis and myositis presenting as a case of ptosis and ophthalmoplegia

ABSTRACT

We report the first case of fatal myocarditis presenting as bilateral ptosis in a patient on combination therapy with pembrolizumab and epacadostat. An 83 year-old man with stage III high-grade urothelial carcinoma presented with acute onset droopy eyelids one month after starting pembrolizumab and epacadostat. Exam showed myogenic ptosis and ophthalmoplegia. He was later found to have acute myocarditis with complete heart block and subsequently passed away. Pembrolizumab in combination with epacadostat can induce a potentially fatal myocarditis. Although immune mediated myocarditis is a rare established side effect, more reported fatalities are needed in the literature to highlight the urgency for standardized cardiac monitoring of even asymptomatic patients to prevent fatal outcomes, as well as a consensus on treatment protocols. Cancer immunotherapy complications are not well known to ophthalmologists. This case is unique in that the presenting sign was ptosis, which prompted the patient to call his ophthalmologist first.

KEYWORDS:

• Chemotherapy induced ptosis
• ophthalmoplegia
• myocarditis

Ethics approval and consent to participate

Retrospective review of this case was done in accordance with the Declaration of Helsinki and the University of California Davis Health Institutional Review Board exempts single case reports from review.

Consent for publication

Verbal consent was obtained from the patient before he passed, and written informed consent for publication of clinical details including images was obtained from the patient’s wife. A copy of the consent form is available for review.

Authors’ contributions

All authors significantly contributed to reviewing the literature and presenting the case. I.T. saw the patient, obtained the clinical history and the photographs of the patient. J.H. wrote the first draft of the manuscript. L.K.L. reviewed all the references and the current literature, and revised the manuscript especially the case presentation, discussion and conclusion section.

Disclosure statement

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the article.