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Background: Immunoglobulin A vasculitis (IgAV) is a rare condition that most commonly presents during childhood. Patients with adult-onset IgAV are more likely to exhibit severe symptoms at presentation with worse renal outcomes. Pulmonary manifestations of adult-onset IgAV have been described rarely in the literature and often indicate higher morbidity and mortality. Given the rarity of alveolar hemorrhage in IgAV, the literature describing the clinical entity and offering management recommendations is insufficient., Case Description: We describe a patient with known adult-onset IgAV who presented with one month of abdominal pain, bloody stools, new skin lesions, and progressive shortness of breath. She developed rapidly worsening hypoxic respiratory failure associated with a hemoglobin drop and diffuse pulmonary infiltrates on imaging. Bronchoscopy demonstrated progressively hemorrhagic effluent on bronchoalveolar lavage (BAL) consistent with a diagnosis of diffuse alveolar hemorrhage (DAH). She developed acute renal failure requiring the initiation of emergent renal replacement therapy. Given concomitant DAH and acute renal failure, methylprednisolone and rituximab (RTX) therapy were initiated. With this treatment regimen, she exhibited marked improvement in respiratory function and complete renal recovery., Conclusions: This case highlights the importance of considering DAH as a rare and life-threatening pulmonary manifestation of adult-onset IgAV. Our case demonstrates the novel and successful use of RTX in combination with steroids to treat a patient with adult-onset IgAV presenting with concomitant DAH and renal failure., Competing Interests: Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://acr.amegroups.com/article/view/10.21037/acr-23-43/coif). S.K. serves as the chair of the American Board of Internal Medicine Rheumatology Board which provides an honorarium as well as reimbursement for travel and lodging, and reports grant support from The Arthritis Foundation which ended in December 2021. The other authors have no conflicts of interest to declare., (2024 AME Case Reports. All rights reserved.)

The Rheumatology Informatics System for Effectiveness (RISE) Registry was developed by the American College of Rheumatology (ACR) to serve US rheumatologists for the significant challenges of a rapidly changing healthcare environment. More than 400 rheumatologists have sent data from more than 3 million encounters of more than 650,000 patients as of August 11, 2016, through their electronic medical records (EMRs), with no additional work or interference with workflow on the part of the rheumatologists. RISE includes patients with all diagnoses seen by participating rheumatologists, at no cost to the rheumatologist.