Granulomatosis with polyangiitis, previously known as wegener’s granulomatosis, is a systemic granulomatous disease. It is histologically characterized by necrotizing vasculitis of medium and small vessels. We present the case of a seven-year-old girl patient who presented unilateral, non-painful dacryoadenitis as the first manifestation of this disease. An incisional biopsy of the lacrimal gland was performed for diagnosis. Evidence of active small-vessel vasculitis was found in the histologic study. Systemic treatment was initiated immediately after, with an excellent initial response. Subsequently, the patient developed pulmonary and renal involvement, and systemic treatment was started with a multidisciplinary team for her treatment.
A literature review was carried out regarding the most relevant aspects of pediatric characteristics of GPA, as it is a rare and often overlooked pathology when it presents itself as a lacrimal gland inflammation.
Published Date: 2022-11-07; Received Date: 2022-07-23