Limited Granulomatosis With Polyangiitis Presenting as Recurrent Lung Abscess Without Pneumonia

This is the authors' abstract. We don't add key points for this paper.

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.