Antibody-Negative Relapse of Goodpasture Syndrome with Pulmonary Hemorrhage

Abstract

Goodpasture syndrome is a rare autoimmune disease comprising antiglomerular basement membrane (anti-GBM) crescentic glomerulonephritis and pulmonary capillaritis with circulating anti-GBM antibodies. Rarely, antibody-negative cases have been described. We report a young, African American adult woman admitted with flank pain and hematuria with laboratory testing and kidney biopsy demonstrating anti-GBM crescentic glomerulonephritis with elevated anti-GBM antibody levels. She received treatment but remained dialysis-dependent. She was seronegative and clinically stable until she presented 8 months later with dyspnea and hemoptysis requiring mechanical ventilation. Bronchoscopy revealed diffuse alveolar hemorrhage. She was treated for relapse of Goodpasture syndrome. However, anti-GBM antibodies were undetectable. This case emphasizes prompt diagnosis and treatment of Goodpasture syndrome to preserve renal function. Additionally, clinical manifestations of Goodpasture syndrome and its degree of activity do not necessarily correlate with the actual antibody titer on relapse. Clinicians should have enhanced awareness of this atypical presentation of a rare disease.

Atypical Anti-Glomerular Basement Membrane Disease

“… 4 , 5 However, reports describing severe relapse of the disease without the appearance of circulating antibodies in patients with previous positive serology are also known. 6 In the last decade, isolated case reports and series 7 , 8 describing similar IF findings as classic anti-GBM disease on kidneys but without other typical features such as aggressive clinical course and/or diffuse crescentic glomerulonephritis and/or circulating anti-GBM antibodies (tested by conventional assays) have increasingly emerged and are termed “atypical anti-GBM disease” 9 ( Table 1 ). A subset of patients with atypical anti-GBM disease have linear monotypic Ig deposits on IF without other features of monoclonal gammopathy of renal significance.…”