Abstract: TH-PO0813
Rare Case of Seronegative Anti-GBM Disease with Pulmonary Involvement
Session Information
- Glomerular Case Reports: Membranous, PGN, GBM, and More
November 06, 2025 | Location: Exhibit Hall, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Doak, William, Brown University, Providence, Rhode Island, United States
- Baptiste, Trevaughn Rawle, Brown University, Providence, Rhode Island, United States
- Kerns, Eric Scott, Brown University, Providence, Rhode Island, United States
- Lynch, Matthew Robert, Brown University, Providence, Rhode Island, United States
Introduction
Seronegative, or atypical anti-glomerular basement membrane (anti-GBM) disease comprises only an estimated 2-3% of patients with anti-GBM disease and portends a more favorable outcome than classic Goodpasture disease. The presence of pulmonary involvement in these atypical cases is exceedingly rare, with smoking history a risk factor for this finding.
Case Description
A 62-year-old non-smoking man with history of renal cell cancer and right nephrectomy, chronic kidney disease with baseline serum creatinine 1.3-1.7 mg/dl, hypertension, and asthma was admitted with several weeks of progressive dyspnea, weakness, and hemoptysis. The admission labs were notable for a hemoglobin of 3.6 g/dl and serum creatinine 5.07 mg/dl. Urinalysis showed 3+ blood and 2+ protein; urine protein-creatinine ratio was 1.24 g/g. Non-contrast CT revealed ground-glass opacities throughout both lungs; the left kidney was normal in appearance. Upper endoscopy showed gastritis. Bronchoscopy with bronchoalveolar lavage confirmed alveolar hemorrhage. Serologic evaluation showed negative ANA, anti-ds DNA, ANCA, and anti-GBM. kidney biopsy was subsequently preformed and was diagnostic of crescentic anti-GBM glomerulonephritis (Figure 1). He was treated with pulse steroids, cyclophosphamide, and six sessions of plasmapheresis. The serum creatinine improved to 3.71 mg/dl on day of discharge, and most recently to 2.98 mg/dl, seven months after the biopsy. Chest CT five months after discharge showed near complete resolution of multifocal airspace disease.
Discussion
Seronegative anti-GBM disease with pulmonary involvement is exceedingly rare and has a more favorable prognosis than classic Goodpasture disease. The seronegativity is thought to be due to either “hidden” or heterogenous GBM epitopes undetectable by current assays or relatively low concentrations of circulating anti-GBM antibodies. The most common finding on kidney biopsy is membranoproliferative glomerulonephritis with and without crescent formation. Clinical suspicion leading to tissue diagnosis despite negative serologic testing is key in ensuring appropriate treatment.