Noninfectious Autoimmune Scleritis: Recognition, Systemic Associations, and Therapy

The aim of this review is to provide the information necessary for recognizing scleritis in rheumatology patients, recognize associated systemic diseases in scleritis patients referred to rheumatology, and choose the best conventional synthetic disease-modifying antirheumatic drugs (csDMARDs) or biologic therapy, based on the most recent data.

Scleritis is most often associated with systemic conditions, particularly autoimmune conditions. Rheumatologists should be most vigilant about rheumatoid arthritis and ANCA-associated vasculitis. A recent systemic association has been found with IgG4-related disease as well. Most of the therapeutic decisions are based on observational data. Most recently, there is increasing evidence of the role of rituximab for refractory disease. Scleritis is a severe inflammatory ocular condition that leads to complications including vision loss. It can precede a systemic autoimmune disease diagnosis, especially in the case of vasculitis. Prompt recognition and adequate therapy are needed in order to avoid serious ocular complications. There is an unmet need for (1) head-to-heat trials on csDMARDs and biologic drugs for the treatment of idiopathic scleritis and (2) randomized controlled trials on the treatment of refractory cases.