June 2022
Volume 63, Issue 7
Open Access
ARVO Annual Meeting Abstract  |   June 2022
Fallacy of Disease: Equivocation
Author Affiliations & Notes
  • May Ameri
    The University of Texas Health Science Center at Houston John P and Katherine G McGovern Medical School, Houston, Texas, United States
  • David Thomas Szynkarski
    The University of Texas Medical Branch at Galveston, Galveston, Texas, United States
  • Ray Cortez
    The University of Texas Medical Branch at Galveston, Galveston, Texas, United States
  • Daniel S Gombos
    The University of Texas MD Anderson Cancer Center, Houston, Texas, United States
  • Sapna P Patel
    The University of Texas MD Anderson Cancer Center, Houston, Texas, United States
  • Nagham S Al-Zubidi
    The University of Texas MD Anderson Cancer Center, Houston, Texas, United States
  • Footnotes
    Commercial Relationships   May Ameri None; David Szynkarski None; Ray Cortez None; Daniel Gombos None; Sapna Patel None; Nagham Al-Zubidi None
  • Footnotes
    Support  none
Investigative Ophthalmology & Visual Science June 2022, Vol.63, 3583 – A0012. doi:
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    • Get Citation

      May Ameri, David Thomas Szynkarski, Ray Cortez, Daniel S Gombos, Sapna P Patel, Nagham S Al-Zubidi; Fallacy of Disease: Equivocation. Invest. Ophthalmol. Vis. Sci. 2022;63(7):3583 – A0012.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract

Purpose : It is extremely difficult to clinically differentiate between ocular immune-related adverse events (IrAEs) of immune checkpoint inhibitors (ICIs) and paraneoplastic-associated retinopathy. Although rare, early detection of IrAE retinopathies is essential, given the degree of tissue damage is often disproportionate to the symptoms. The purpose of this study is to exaCancmine and discuss the diagnostic challenge of distinguishing between melanoma-associated retinopathy and immune checkpoint inhibitor (ICI) related retinopathy - which poses a unique diagnostic challenge.

Methods : Dilated fundus exam (DFE), electroretinogram (ERG), MRI, PET scan, cytokine panel, and a literature search were conducted.

Results : A 55-year-old male with a history of metastatic malignant melanoma of left forearm Stage IV (rpT2a, pN0, cM1c) on ipilimumab and nivolumab presented with symptoms of nyctalopia, diminished color contrast and light/dark contrast, abnormal visual phenomena, and constricted visual field OU. The exam showed 20/20 vision OU, bilateral color vision deficiency (9/14 OD and 10/14 OS), and no RAPD OU. The DFE showed no optic nerve swelling, pallor, or retina abnormalities. ERG did not show the classic “electronegative response” which we would expect to see with MAR. Instead, we see a normal b-wave and absent a-wave which denotes pure rod dysfunction. Thus, the patient’s symptoms are likely attributed to an ICI-related adverse event such as bilateral retinal vasculitis.

Conclusions : Although severe and insidious in nature, ocular IrAEs are a lesser-known effect of ICIs in the medical community; moreover, even in specialized oncology clinics, it is still extremely difficult to diagnose due to the ambiguity of the disease. Further, immediate appropriate treatment through discontinuation of ICI and starting a trial of steroids, IVIG, and PLEX is necessary for ocular IrAEs to prevent permanent vision loss. Especially, given that these retinopathies often require further specialized testing such as ERGs or mfERG to differentiate between them and cancer-related retinopathies, recognition by oncologists and early referral to an ophthalmologist is essential.

This abstract was presented at the 2022 ARVO Annual Meeting, held in Denver, CO, May 1-4, 2022, and virtually.

 

ERG of pt with ocular IrAE vs standard ERG

ERG of pt with ocular IrAE vs standard ERG

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