C41-07 A Rare Case of Diffuse Alveolar Hemorrhage From Dual Positive Goodpasture Syndrome

Abstract Diffuse Alveolar Hemorrhage (DAH) is a life-threatening pulmonary emergency with the most common cause being a small-vessel vasculitis, such as ANCA-associated vasculitis (AAV) or anti-GBM vasculitis (Goodpasture syndrome). Concurrent positivity for both AAV and anti-GBM antibodies is rare but often signifies a more severe presentation and greater therapeutic challenge. A 53-year-old gentleman with no significant past medical history or oxygen requirement at baseline presented to the ED with one day of acute massive hemoptysis, dyspnea, and hypoxic respiratory failure requiring oxygen supplementation. Physical exam was notable for diffuse crackles bilaterally. Recent history was significant for hospitalization a month ago for new onset hematuria and an AKI, as well as an episode of hemoptysis. Kidney biopsy done at that time showed crescentic glomerulonephritis with positive anti-GBM, and serum studies showed positive PR3 ANCA antibodies at high titers. The patient was managed with pulse dose steroids, plasma exchange (PLEX), and rituximab, but missed a dose of scheduled Rituximab outpatient. CT scan showed bilateral patchy infiltrates (Image 1), concerning for DAH. A bronchoscopy with bronchoalveolar lavage showed washings with progressive bloody appearance, consistent with DAH. With rheumatology on board, the patient was diagnosed with AAV and Goodpasture syndrome overlap and treated with 5 sessions of plasma exchange, pulse-dose glucocorticoids, and one dose of cyclophosphamide. After his first session of plasma exchange and initiation of steroids, the patient’s hypoxemia resolved. After a two-week stay, the patient was discharged on a prednisone taper with a plan to continue cyclophosphamide outpatient. This case highlights a rare but clinically significant etiology of DAH—dual positive Goodpasture syndrome. This diagnosis is not only rare, but also associated with high mortality. A potential pathophysiology is AAV-associated damage to the glomerular basement membrane, leading to uncovering “hidden antigens” from the membrane and inducing the formation of antibodies. Classic treatment for AAV is glucocorticoids with rituximab or cyclophosphamide, whereas for Goodpasture is PLEX and immunosuppressive therapy. Dual-positive disease lies on a continuum between AAV and Goodpasture syndrome but behaves distinctly, often presenting with recurrent or refractory manifestations. Unlike isolated AAV or Goodpasture disease, management requires combining therapeutic principles from both. For this patient with dual disease, the key to successful management involved both PLEX and steroid therapy. This case emphasizes the importance of initial high suspicion for DAH in patients with hemoptysis, respiratory failure, and history of vasculitis. Also, dual-positive anti-GBM vasculitis exists and demands a tailored, hybrid treatment approach. This abstract is funded by: None