• Lakshmi Saladi, Danial Shaikh, Muhammad Saad, Enny Cancio-Rodriguez, Vivette D D'Agati, Boris Medvedovsky, Kalpana A Uday, and Muhammad Adrish.
• Division of Pulmonary and Critical Care Medicine Department of Medicine, Bronx Care Health System, Bronx, NY Department of Pathology, College of Physicians and Surgeons, Columbia University Affiliated with Icahn School of Medicine at Mount Sinai, New York, NY.
• Medicine (Baltimore). 2018 Jun 1; 97 (23): e10954e10954.

RationalePulmonary renal syndrome (PRS) is a term most commonly used to describe a combination of glomerulonephritis and pulmonary hemorrhage as a manifestation of a multisystem autoimmune disease. It is usually associated with ANCA vasculitis and anti-GBM disease. Diffuse alveolar hemorrhage in a patient with ANCA and anti-GBM negative pauci-immune glomerulonephritis is rare and optimal management is unknown.Patient ConcernsAn 85-year-old man with hypertension, diabetes mellitus, prostate cancer and recently diagnosed pauci-immune necrotizing glomerulonephritis presented to our emergency department with worsening dyspnea and pedal edema for several days. Clinical presentation and radiological studies were suggestive of fluid overload but he developed worsening respiratory failure despite hemodialysis.DiagnosesBronchoscopy confirmed diffuse alveolar hemorrhage. ANCA and anti-GBM antibodies were negative. The patient was diagnosed with pulmonary renal syndrome - diffuse alveolar hemorrhage in the setting of ANCA and anti-GBM negative pauci-immune glomerulonephritis.InterventionsPatient was started on intravenous pulse steroids, cyclophosphamide and received seven sessions of plasmapheresis.OutcomesThere was an improvement in patient's respiratory status and repeat bronchoscopy at the end of treatment did not show diffuse alveolar hemorrhage.LessonsPauci-immune crescentic necrotizing glomerulonephritis is usually associated with the presence of ANCA, however, ANCA may be absent in 10% of these cases. Immunosuppression is the mainstay of treatment for ANCA and anti-GBM associated PRS. This case highlights the importance of immunosuppression and plasmapheresis in patients with ANCA negative vasculitis due to presence of unidentified serum antibodies. If left untreated, these patients can have a fulminant course with high mortality ranging from 25 to 50%.

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