SHM Converge Scientific Abstract ePoster Gallery-When the Serologies Are Silent: Pauci-Immune Pulmonary Capillaritis Presenting as Recurrent Diffuse Alveolar Hemorrhage

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This case describes a 67-year-old man with recurrent diffuse alveolar hemorrhage (DAH), presenting with hemoptysis, dyspnea, hypoxemia, bilateral crackles, anemia, and ground-glass opacities on CT. Bronchoalveolar lavage confirmed DAH. Infectious studies were negative, and autoimmune serologies including ANA, ANCA, anti-GBM, RF, CCP, and dsDNA were also negative.<br /><br />He initially improved with pulse-dose corticosteroids but returned twice with recurrent bleeding. Because of persistent clinical concern despite negative serologies, a multidisciplinary team favored isolated pauci-immune pulmonary capillaritis (IPIPC), a rare form of immune-mediated capillaritis that can present without positive serologic markers early in disease. Cyclophosphamide was recommended, but the patient declined it. He was instead treated with rituximab during two admissions, along with corticosteroids, and achieved sustained remission for six months.<br /><br />The report emphasizes that DAH is a life-threatening emergency with mortality rates as high as 50% when diagnosis or treatment is delayed. It highlights that up to 20% of ANCA-associated vasculitis cases may initially be seronegative, making diagnosis challenging. The case supports maintaining suspicion for immune-mediated pulmonary disease even when serologies are negative.<br /><br />The authors conclude that rituximab may be an effective alternative induction therapy for seronegative IPIPC when cyclophosphamide is not acceptable or is contraindicated. They also stress the importance of early recognition, ongoing surveillance, medication adherence, and close coordination among hospitalists, pulmonologists, rheumatologists, and outpatient teams to prevent relapse and improve long-term outcomes.

Asset Subtitle

Harleen K. Sidhu

Meta Tag

Author List Adithan Ganesh, Harleen K. Sidhu, Jayakumaran Jeyanthan, Nitin Pendyala, Oikigbeme Oikeh

Category Clinical Vignettes

Concept Diffuse Alveolar Hemorrhage

Concept Pulmonary Capillaritis

Concept Hemoptysis

Concept Bronchoalveolar Lavage

Concept Pauci-Immune Pulmonary Capillaritis

Distinguished Non-Finalist

Presenter Organization Department of Medicine, NYC Health + Hospitals/Elmhurst, Icahn School of Medicine at Mount Sinai

Presenting Author Harleen K. Sidhu

Track Adult

Keywords

diffuse alveolar hemorrhage

hemoptysis

ground-glass opacities

bronchoalveolar lavage

pauci-immune pulmonary capillaritis

seronegative vasculitis

rituximab

corticosteroids

cyclophosphamide

ANCA-negative

Diffuse Alveolar Hemorrhage

Pulmonary Capillaritis

Hemoptysis

Bronchoalveolar Lavage

Pauci-Immune Pulmonary Capillaritis