Progressive Pituitary Involvement in a Patient with Localized Granulomatosis with Polyangiitis

Granulomatosis with polyangiitis (GPA) is a life-threatening disease characterized by granulomatous inflammation and antineutrophil cytoplasmic antibody (ANCA) associated systemic vasculitis, classically involving upper and lower respiratory tracts and kidney. Milder forms of disease restricted to respiratory tract is referred to as localized GPA (loc-GPA) [1,2]. The European Vasculitis Study Group (EUVAS) defined loc-GPA as disease restricted to ear, nose, and throat (ENT) tract and lung without clinical signs of systemic vasculitis or disruption on vital organ function [3]. Progression from loc-GPA to systemic form is rare, occurring in only 10% of patients with loc-GPA [4]. Involvement of pituitary gland as a manifestation of progression into systemic disease in loc-GPA is also unusual, not previously reported. Here we report an unusual case of ANCA-negative loc-GPA initially presented with multiple cranial nerve palsies which progressed into ANCA-positive systemic disease involving pituitary gland. Case