Peripheral Ulcerative Keratitis Associated with Autoimmune Diseases

Abstract

Peripheral ulcerative keratitis (PUK) is a destructive inflammatory disease of the juxtalimbal cornea associated with crescent-shaped corneal stromal thinning, epithelial defect, and inflammatory corneal infiltrate. Inflammation of other adjacent tissues, particularly the sclera, is seen quite frequently. Predilection of the peripheral cornea for PUK is explained by its anatomical and physiological characteristics. Both cell-mediated and humoral immunity, in conjunction with the corneal tissue-destroying action of metalloproteinases (MMPs), are implicated in the pathogenesis of PUK. Nearly half of all cases of noninfectious PUK are associated with connective tissue diseases (rheumatoid arthritis (RA) is the most frequent underlying disease) and vasculitis (mostly granulomatous with polyangiitis (GPA)). It is important to determine the etiology and exclude conditions that could mimic PUK e.g., marginal keratitis or Terrien’s marginal degeneration (TMD). Therapy should comprise the attenuation of ophthalmic inflammation, but the underlying disease should be treated as a priority. For autoimmune diseases, it is crucial to work closely with internist/rheumatologist to determine an effective immunomodulatory therapeutic approach. PUK is also known to be a potentially devastating and vision-threatening condition that may lead to corneal melting and perforation, requiring surgical intervention. This chapter provides a comprehensive update of current knowledge and therapeutic methods.