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Granulomatosis with Polyangiitis with Pituitary Involvement

Imaging findings

Chest CT shows two large, mass-like areas of consolidation with irregular cavitation, surrounding ground-glass halos, and no satellite nodules. There is also marked thickening of the central airways, including the posterior membranous trachea, and head imaging shows pituitary involvement (necrotizing granulomatous hypophysitis) and sinus disease. Laboratory evaluation reveals highly elevated PR3-ANCA titers.

Key takeaways

Granulomatosis with polyangiitis (GPA) can rarely present with central nervous system manifestations such as granulomatous hypophysitis leading to panhypopituitarism. Airway thickening that involves the posterior membranous trachea, sinus disease, and cavitary pulmonary nodules with ground-glass halos are highly characteristic thoracic features of GPA.

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