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Granulomatosis with Polyangiitis (GPA) with Necrotizing Pulmonary and Skin Lesions

Granulomatosis with Polyangiitis (GPA) with Necrotizing Pulmonary and Skin Lesions▶ Watch on YouTube — age-restricted, cannot embed here

Imaging findings

Chest radiographs showed large, rounded, non-segmental opacities in the lower lung zones. CT revealed extensive internal necrosis and cavitation within these large opacities, with a small right pleural effusion. The patient also had skin disease, with a lesion around the right elbow, and biopsy confirmed a granulomatous vasculitis. Elevated serum protease and ANCA positivity were present.

Key takeaways

Granulomatosis with Polyangiitis (GPA) is a systemic vasculitis characterized by necrotizing granulomatosis. Pulmonary manifestations include large, cavitating nodules with extensive internal necrosis. It often affects other organs like skin, kidneys, and sinuses. Elevated serum protease and ANCA positivity support the diagnosis.

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