Abstract | November 8, 2021
A Very Rare Antibody Negative Goodpasture’s Disease Requiring Hemodialysis
Learning Objectives
- The absence of circulating anti-GBM antibodies in Goodpasture's syndrome can lead to late diagnosis which can be detrimental as studies have shown that early aggressive therapy leads to an improved prognosis
- Anti-GBM antibodies generally are of immunoglobulin G subclass 1 (IgG1) and can in most cases readily be detected in the circulation using enzyme-linked immunosorbent assays (ELISAs).
- On biopsy typical disease displays bright polytypic linear GBM staining for IgG by immunofluorescence and diffuse crescentic/necrotizing GN on light microscopy.
Introduction: Goodpasture’s disease is rare and occur in fewer than two cases per million. Classic anti-glomerular basement membrane (GBM) disease presents with rapidly progressive glomerulonephritis with or without pulmonary hemorrhage, in association with deposition of antibodies in a linear patternon the GBM. It is a rare cause of end-stage kidney disease, accounting for approximately 0.8 percent of all ESKD patients. Anti-GBM antibodies can in most cases readily be detected in the circulation using enzyme-linked immunosorbent assays. Although very rare, a few cases with absence of circulating anti-glomerular membrane antibodies have been described.
Case: Although very rare, we describe a case of Goodpasture’s disease in which no circulating anti-GBM antibodies were detectable in serum by well-established enzyme-linked immunosorbent assay. A 69-year-old Caucasian female with multiple co-morbidities presented with complaints of dyspnea with one episode of hemoptysis. She was found to have WBC 7.5, hemoglobin 8.3, hematocrit 25.5, platelets 184, BUN 63, creatinine 5.4. Rapid COVID unremarkable. CXR patchy bilateral infiltrates. ANA, C-ANCA, P-ANCA and anti-GBM antibodies were negative. Renal US negative for hydronephrosis, renal masses, stones. In light of her acute kidney injury of unclear etiology, findings of microscopic hematuria, renal biopsy was pursued which showed necrotizing and crescentic glomerulonephritis with anti-GBM antibody. Immunofluorescence revealed a linear immunoglobulin G deposition compatible with Goodpasture’s syndrome.
Management: The patient was started on pulse steroids, daily cyclophosphamide. Over the succeeding days, her kidney function continued to worsen, and although she remained nonoliguric, she was started on hemodialysis and received plasmapheresis with good response.
Conclusions: The absence of circulating anti-GBM antibodies in Goodpasture’s syndrome can lead to late diagnosis which can be detrimental as studies have shown that early aggressive therapy leads to an improved prognosis. Physicians should be aware of this rare presentation of Goodpasture’s disease and consider tissue diagnoses such with kidney biopsy.
