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The authors discuss a report on the description of clinical features and disease course of orbital masses in ANCA-associated vasculitis (AAV). Topics discussed include factors predicting the onset of orbital masses in the context of AAV, a diagnostic pathway to minimize diagnostic delay and improve the management of subset of patients, and comparison of first-line treatment of a cyclophosphamide- to a rituximab-based regimen.

The article discusses the case of a 54 year old female patient of Caucasian descent presented with a past medical history including Hashimoto thyroiditis diagnosed in 2006. Further investigations were initiated including ENT, ophthamological and neurological assessments due to a suspected diagnosis of granulomatosis with polyangiitis. The case is the first description of an IgG4-related tubulointerstitiial nephritis with concomitant positivity of cytoplasmic ANCA and PR3.