Abstract Introduction Granulomatosis with polyangiitis (GPA), a form of ANCA-associated vasculitis, primarily affects the upper respiratory tract, lungs, and kidneys. Although the classic presentation includes sinusitis, pulmonary infiltrates, and glomerulonephritis, patients may present with isolated or atypical manifestations. Case Presentation A 38-year-old male, with no significant past medical history, presented to the emergency department with persistent fever, night sweats, headaches and shortness of breath. The patient reported a 2-week history of symptoms, including yellowish/pinkish sputum and pulsatile headaches. Chest CT revealed bilateral cavitary pulmonary lesions with central necrosis in the right lower lobe, measuring 4.1 x 5 x 4.1 cm and a 1.5 cm solitary nodule in the right lower lobe and mediastinal lymphadenopathy. His initial work-up was unremarkable, including negative MTB PCR, AFB cultures, no proteinuria and a negative ANA-ENA panel. However, lab results were notable for positive c-ANCA and positive PR3 antibodies, suggesting the possibility of ANCA-associated vasculitis. The patient underwent robotic bronchoscopy and lung biopsy, but initial results were non-diagnostic. He was managed empirically with antibiotics (piperacillin-tazobactam and doxycycline) for a suspected lung abscess. The final diagnosis of GPA was established after further workup, including the biopsy and continued clinical monitoring. The patient received pulse-dose steroids, and rituximab and was considered for induction therapy. Throughout his course, the patient had persistent epistaxis, managed conservatively, and required follow-up with ENT. He was also evaluated by nephrology for worsening renal function and proteinuria, with renal biopsy confirming crescentic glomerulonephritis, consistent with GPA. Discussion GPA, formerly referred to as Wegener’s granulomatosis, is a systemic autoimmune vasculitis that typically manifests with pulmonary nodules, sinusitis, and glomerulonephritis. The diagnosis is primarily established through the detection of c-ANCA and PR3 antibodies, in conjunction with clinical findings and histopathological confirmation. In this case, bilateral cavitary lung lesions were the presenting feature, occurring in the absence of proteinuria and without the classic signs of sinus involvement. A multidisciplinary approach was pivotal in both the diagnosis and management of this patient. Epistaxis, indicative of upper airway involvement, prompted an ENT consultation, which revealed sinus opacification. Nephrological evaluation identified crescentic glomerulonephritis on renal biopsy. The patient was successfully treated with rituximab and corticosteroids, which played a critical role in controlling the vasculitis and preventing further organ damage. This case highlights the critical importance of early recognition and a thorough diagnostic work-up in patients presenting with pulmonary lesions, systemic symptoms, and positive ANCA markers. This abstract is funded by: None
Tayyab et al. (Fri,) studied this question.