Scleritis: Immunopathogenesis and molecular basis for therapy

Scleritis is a heterogeneous group of diseases characterized by inflammation of the sclera, which may be due to local or systemic infections or immune mediated diseases. Numerous studies over the last decade have lead to significant progress in understanding the pathogenesis and treatment of this severe and potentially blinding disease. Immunological investigations of non-infectious scleritis and associated diseases have indicated that scleritis is an autoimmune disease and studies on the nature of the local inflammatory response have revealed the prominent role of T and B cells and cytokines, such as TNF-alpha, which in turn has resulted in clinical trials showing the effectiveness of local steroid treatment, anti-TNF and anti-B cell therapy. The widespread use of imaging has led to the realization that posterior scleritis is more common than previously recognized and testing for ANCA antibodies has revealed the prominent role of immune mechanisms in a subset of patients with scleritis and associated systemic vasculitis.