Abstract
Monoclonal gammopathy of ocular significance (MGOS) is a rare subset of monoclonal gammopathy of clinical significance occurring secondary to plasma cell disorders and causing ocular manifestations. We identified 23 patients with paraproteinemic keratopathy (PPK) in the setting of monoclonal gammopathy of unknown significance (MGUS, 10), smoldering multiple myeloma (SMM, 3) or multiple myeloma (MM, 10). Many of these patients with PPK (11/23) presented decreased vision. All patients with MM and 40% of those with other diagnoses such as SMM and MGUS received systemic therapy with or without autologous stem cell transplantation. Four eyes of four patients were treated by penetrating keratoplasty. In most cases, neither ocular nor hematologic treatment afforded a durable improvement in the visual acuity (recurrence after a median of 11 months), despite initial responses. Further studies will be required to determine the optimal strategy to treat and prevent the relapse of ocular symptoms in patients with PPK.
Acknowledgments
Patients consented to the publication of photographs and associated medical records. The study received no funding or grant support. Marcin Balcerzak of Medink provided medical writing advice.
Author contributions
Conceptualization, LG, NS, AJ; data curation, LG, M AH, J WP, MM, KK, CP, AG, ZX, A WG, LR, GM, MK, WL, DV, NS, AJ; writing of original draft, LG,NS, AJ; reviewing and editing, LG, M AH, J WP, MM, KK, CP, AG, ZX, A WG, LR, GM, MK, WL, DV, NS, AJ.
Disclosure statement
No potential conflict of interest was reported by the author(s).