Introduction Although the majority of cases of episcleritis are idiopathic, an estimated 27% to 36% have an underlying systemic cause. The most common etiologies are inflammatory, including rheumatoid arthritis, inflammatory bowel disease, and antineutrophil cytoplasmic antibody vasculitis. Less commonly, episcleritis is caused by infectious, neoplastic, and toxic sources. This case report details the newly described autoimmune disease VEXAS syndrome that is caused by a gene mutation in hematopoietic stem cells and results in blood dyscrasia and widespread aberrant inflammation. Eye findings in VEXAS syndrome are common and include both ocular and periocular diagnoses. Case Report An 81-year-old White man with VEXAS syndrome was referred for evaluation of bilateral red eyes. Examination findings revealed bilateral episcleritis that improved with reinitiation of a therapeutic oral prednisone dose. Conclusion The current standard of care for episcleritis is to not work up typical initial presentations. Recurrent or atypical presentations should raise suspicion of an underlying systemic cause. In those instances, a thorough case history often directs ancillary testing. When VEXAS syndrome is the underlying cause of episcleritis, a combination of constitutional symptoms in addition to hematologic and inflammatory findings may be present.
Chang et al. (Tue,) studied this question.