Ocular Findings in Patients with Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes Syndrome

Objective: Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a rare disorder. This study investigated the types of ocular signs and symptoms in patients with POEMS and any systemic factors that may be associated with development of such ocular findings. Design: Case series from tertiary referral center. Participants: A total of 33 patients with POEMS syndrome underwent at least 1 ophthalmologic examination and were included in the study. Methods: A 10-year retrospective chart review of patients diagnosed with POEMS syndrome was performed. Main Outcome Measures: Visual symptoms, visual acuity, presence of optic disc edema (ODE), and levels of systemic factors (including plasma vascular endothelial growth factor [VEGF], plasma interlukin-6 [IL-6], and raised intracranial pressure) and their relationship to ODE. Results: Five of the patients (15%) reported diplopia, 15 patients (45%) had blurred vision, and 3 patients (9%) had ocular pain. The most common ocular finding was bilateral ODE in 17 patients (52%). Of the patients with ODE, 5 (29%) were asymptomatic at the first ocular examination. Among patients with ODE, there was a significant difference (P = 0.03) between the mean plasma VEGF level at the time of diagnosis of the ODE compared with when the ODE resolved. There was no difference in plasma IL-6 levels between people with and without ODE. Patients with ODE had a higher mean lumbar puncture opening pressure (276 +/- 14 mm H2O; normal range, 100-250 mm H2O) than patients without ODE, although the difference was not statistically significant (P = 0.08). Conclusions: Optic disc edema is a common finding in patients with POEMS. Because patients can be asymptomatic, eye examinations should be performed in all patients with POEMS. There may be an association between elevated VEGF and intracranial pressure and ODE; further studies are required.