Rituximab, Intravitreal Bevacizumab and Laser Photocoagulation for Treatment of Macrophage Activation Syndrome and Retinal Vasculitis in Lupus: A Case Report

Abstract

Systemic lupus erythematosus (SLE) most commonly manifests as mild to moderate disease with severe manifestations such as diffuse alveolar hemorrhage, central nervous system vasculitis, macrophage activation syndrome (MAS) or retinal vasculitis (RV) with visual disturbances occurring in a significantly smaller proportion of patients, most of whom have a poor outcome. Macrophage activation syndrome and RV are insufficiently early and rarely recognized presentations of lupus—consequently there are still no treatment recommendations. Here we present the course of diagnosis and treatment of a patient with an SLE flare that resulted in both life-threatening disease (MAS) and vision-threatening disease (RV). The patient was successfully treated with systemic immunosuppressives, a high dose of glucocorticoids and rituximab (RTX), in parallel with intraocular therapy, intravitreal bevacizumab (BEV) and laser photocoagulation.

Special Issue “The Molecular and Cellular Pathophysiologic Mechanisms Underlying Ocular Diseases and Emerging Therapies”

“…In addition to reviewing the current state of knowledge, the authors have also attempted to identify gaps in the current understanding of the pathogenic mechanisms of C2orf71/PCARE and have proposed directions for future research. In a case report by Ikić Matijašević et al [15], an interesting and educational case of systemic lupus erythematosus (SLE) with two severe complications, namely life-threatening macrophage activation syndrome (MAS) and vision-threatening retinal vasculitis (RV), have been described. Both MAS and RV are manifestations of lupus that are often inadequately recognized in their early stages, and currently, no established treatment recommendations are available.…”