Limited Granulomatosis With Polyangiitis Presenting as Recurrent Lung Abscess Without Pneumonia

Granulomatosis with polyangiitis (GPA), previously called Wegener's granulomatosis, is a systemic necrotizing vasculitis affecting small and medium-sized vessels and is associated with antineutrophil cytoplasmic antibodies (ANCA). GPA is a systemic disease but can present in a limited form, where the respiratory system is the most commonly involved. Here, we report a case of a 54-year-old African American female who presented with chronic cough and got diagnosed with recurrent right-sided empyema without pneumonia. She underwent a right thoracotomy, and the biopsy showed necrotizing granulomatous inflammation with vasculitis and rare non-necrotizing granuloma, suggesting GPA. Diagnosis of GPA was confirmed by strongly positive anti-proteinase 3 ANCA antibodies. Interestingly, her GPA is a limited form, and she had an excellent recovery after initiation of immunosuppression. Early diagnosis and treatment are critical for better outcomes and survival in GPA.