Abstract Introduction Diffuse alveolar hemorrhage (DAH) is a life-threatening pulmonary condition, characterized by acute or subacute accumulation of red blood cells within the alveolar spaces. DAH typically presents with dyspnea, hemoptysis, a rapid drop in hemoglobin without any other source of bleeding, and new pulmonary infiltrates. Rheumatoid vasculitis (RV) is a severe extra-articular manifestation of rheumatoid arthritis (RA). DAH secondary to RV is exceptionally rare. Case Description A 79-year-old female with a history of hypertension, osteoarthritis, type 2 diabetes mellitus, chronic kidney disease, and a 10-year history of untreated rheumatoid arthritis presented with a 2-month history of progressive dyspnea on exertion, intermittent hemoptysis, generalized weakness, and a 25-pound weight loss. Initial laboratory evaluation revealed life-threatening anemia with a hemoglobin of 4.6 g/dL. Chest tomography revealed diffuse ground-glass and consolidative opacities, mediastinal/axillary lymphadenopathy, bilateral small pleural effusions, and cardiomegaly. The patient underwent bronchoscopy with bronchoalveolar lavage. Transbronchial biopsy was notable for interstitial fibrosis and hemosiderin-laden macrophages (Figure 1). All pulmonary segments were diffusely stained with blood, and three aliquots were taken with progressively bloody samples consistent with DAH. Her cyclic citrullinated peptide antibody immunoglobulin G (anti-CCP IgG) level was higher than 250 U/mL; antineutrophil cytoplasmic antibody (ANCA) testing was negative. Infectious workup did not reveal any underlying infection. The patient was started on high-dose IV methylprednisolone. Despite efforts, she developed acute hypoxic respiratory failure requiring high-flow nasal cannula and intermittent BiPAP. Goals of care conversations led to comfort care status. Discussion Rheumatoid vasculitis typically occurs in patients with long-standing, seropositive RA, and DAH represents a rare but life-threatening pulmonary manifestation. In this case, DAH developed in a patient with a prior diagnosis of RA who had not been under regular medical care or on immunosuppressive therapy, likely contributing to the severity and fulminant progression of the disease. Notably, the likelihood of confirming vasculitis in a transbronchial biopsy is low and less sensitive than a surgical lung biopsy. This presentation underscores the importance of adherence to rheumatologic follow-up and the early institution of immunomodulatory treatment in preventing severe extra-articular complications. Despite high-dose corticosteroid therapy, outcomes in RV-associated DAH remain poor. Given the rarity of this condition, current management relies largely on case reports and expert opinion. Further research and multicenter studies are needed to establish standardized, evidence-based guidelines for timely diagnosis and optimal immunosuppressive therapy. This abstract is funded by: None
Archila et al. (Fri,) studied this question.