Central diabetes insipidus as the presenting symptom of granulomatosis with polyangiitis

Granulomatosis with polyangiitis (Wegener's; GPA) is a necrotizing granulomatous vasculitis that can affect any organ system; however, central nervous system involvement is uncommon. We present 2 cases where central diabetes insipidus was the presenting manifestation of GPA. Pituitary involvement was documented by magnetic resonance imaging. Both patients were female and responded to combination therapy of prednisone and cyclophosphamide. We also review the literature for all reported cases of pituitary involvement in GPA. We conclude that pituitary involvement in GPA seems more common in females and may be the initial presenting clinical feature and may occur in the absence of systemic disease. Early recognition and treatment may prevent pituitary necrosis and irreversible damage.