Paraproteinemic maculopathy

Abstract

Purpose: Paraproteinemia relates to monoclonal gammopathy producing pathologic antibodies with increased blood viscosity. Serous macular detachment is an uncommon ocular manifestation of paraproteinemia. We ascertain the clinical course of maculopathy in paraproteinemia and propose a mechanism for development and resolution of subretinal fluid based on various therapeutic modalities.

Methods: The records of patients with paraproteinemia with ocular coherence tomography (OCT) documentation of serous macular detachment were reviewed. Data collection included coexisting morbidity, rheology data (immunoglobulin level, hematocrit, and blood viscosity), clinical examination, as well as findings on OCT.

Results: A total of 33 cases were collected with 11 new and 22 previously reported in the literature. Diabetes was present in 7, systemic hypertension in 9, and anemia in 18. Mean initial immunoglobulin level was 6,497mg/dL and mean serum viscosity was 5.5cP. Mean logMar initial visual acuity was 0.55 (20/71) in the right eye and 0.38 (20/48) in the left eye. Final visual acuity was right eye 0.45 (20/56) and left eye 0.50 (20/63). Plasmapheresis was given in 18, chemotherapy in 30, blood transfusion in 2 and transplant of progenitor hematopoietic cells in 2. Oral rituximab was used in 10 patients. Immunoglobulin levels normalized in 8 patients and were unchanged in 1 patient after plasmapheresis and/or chemotherapy. Ocular therapy in 8 patients included vitrectomy in 1, laser photocoagulation in 4, intravitreal bevacizumab in 5, intravitreal triamcilonone in 2, intravitreal dexamethasone implant in 1, intravitreal rituximab in 1, and subtenon corticosteroid in 1. The maculopathy resolved partially or completely in 17 patients and worsened or remained unchanged in 14 patients over a median follow-up of 7 months. The maculopathy was unilateral in 9 cases. Maculopathy occurred at a lower initial immunoglobulin level in diabetics.

Conclusions: Paraproteinemic maculopathy can be unilateral. Decreasing the blood immunoglobulin level is the primary goal in the therapy of paraproteinemic maculopathy and this can be achieved by systemic route. Coexisting diabetes facilitates leakage of immunoglobulins at lower levels than non-diabetics. Despite systemic treatment of paraproteinemia, the long term visual prognosis in maculopathy remains guarded.

Keywords: 688 retina • 585 macula/fovea