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A Phenotype of Primary Open-angle Glaucoma With Systemic Vasospasm

Alizadeh, Reza, MD*; Vickers, Laura, MD*; Hirunpatravong, Pradtana, MD*; Romero, Pablo, MD*; Lin, Mark C., MD*; Sharifipour, Farideh, MD; Caprioli, Joseph, MD*

doi: 10.1097/IJG.0000000000001083
Original Studies
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Purpose: Primary open-angle glaucoma (POAG) patients constitute a heterogenous group. Identification of phenotypic subtypes among these patients may provide a deeper understanding of the disease and aid associations with genotypes. We describe a phenotype of POAG patients associated with a constellation of systemic disorders; patients with this phenotype seem to be vulnerable to optic nerve damage at low intraocular pressures.

Materials and Methods: In this retrospective study, we evaluated the medical records of active Jules Stein Eye Institute glaucoma patients from January 1996 to 2017 and included subjects with POAG, acquired pits of the optic nerve (APON), and at least one of the following: systolic blood pressure persistently ≤100 mm Hg, history of migraine headaches or migraine variant, and the Raynaud syndrome.

Results: Of 87 patients (125 eyes) with APON, 37 patients (55 eyes) met the study criteria. In total, 34 patients were female (92%). The median age at the time of diagnosis was 55 years. Nineteen patients (73%) had low systolic blood pressures, same number had Raynaud syndrome, and 25 (68%) had a history of migraine.

Conclusions: We describe a POAG subtype with APON and systemic vascular instability, predominantly female in their sixth decade of life who demonstrate progressive glaucomatous visual field damage at low intraocular pressure. We suggest that this clinical picture represents an important phenotype of POAG, and that identification and further study of it will help guide diagnosis and development of individualized treatments.

*Glaucoma Division, Jules Stein Eye Institute, University of California Los Angeles (UCLA), Los Angeles, CA

Department of Ophthalmology, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran

Supported in part by unrestricted research grants from Research to Prevent Blindness (New York, NY), the Simms/Mann Family Foundation Los Angeles, CA and the Simms-Mann Foundation and by Research to Prevent Blindness, Inc.

Disclosure: The authors declare no conflict of interest.

Reprints: Joseph Caprioli, MD, Glaucoma Division, Jules Stein Eye Institute, David Geffen School of Medicine, University of California Los Angeles (UCLA), 100 Stein Plaza, Los Angeles, CA 90095 (e-mail: caprioli@jsei.ucla.edu).

Received April 10, 2018

Accepted July 21, 2018

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