Antiglomerular basement membrane disease(Goodpasture syndrome) associated with ANCA-negative central nervous system vasculitis

Anti-glomerular basement membrane disease is a rare pediatric autoimmune disease characterized by anti-glomerular basement membrane antibodies that cause rapidly progressive glomerulonephritis and alveolar hemorrhage. Central nervous system (CNS) involvement is sporadic. We present the case of a 13-year-old female patient with cerebral hemorrhage, septic shock, and multiorgan involvement, in whom the diagnosis of anti-glomerular basement membrane disease and CNS vasculitis in the absence of antineutrophil cytoplasmic antibodies (ANCA-negative) was confirmed. Combined treatment with glucocorticoids, cyclophosphamide, and plasmapheresis was performed, resulting in complete neurological and renal recovery. The rare coexistence of these two entities is analyzed. The case highlights the importance of maintaining a high index of diagnostic suspicion in the face of atypical clinical presentations.

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Publication Details

Journal
Archivos Argentinos de Pediatria
Published
2026-01-08
DOI
https://doi.org/10.5546/aap.2025-10816.eng
Primary Topic
Vasculitis and related conditions
Type
article
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article

Antiglomerular basement membrane disease(Goodpasture syndrome) associated with ANCA-negative central nervous system vasculitis

Anabella Spierer, Bárbara Cosentino, Florencia Gorjón
Archivos Argentinos de Pediatria
Vasculitis and related conditions
article

Antiglomerular basement membrane disease(Goodpasture syndrome) associated with ANCA-negative central nervous system vasculitis

Anabella Spierer, Bárbara Cosentino, Florencia Gorjón
article en

Abstract

Anti-glomerular basement membrane disease is a rare pediatric autoimmune disease characterized by anti-glomerular basement membrane antibodies that cause rapidly progressive glomerulonephritis and alveolar hemorrhage. Central nervous system (CNS) involvement is sporadic. We present the case of a 13-year-old female patient with cerebral hemorrhage, septic shock, and multiorgan involvement, in whom the diagnosis of anti-glomerular basement membrane disease and CNS vasculitis in the absence of antineutrophil cytoplasmic antibodies (ANCA-negative) was confirmed. Combined treatment with glucocorticoids, cyclophosphamide, and plasmapheresis was performed, resulting in complete neurological and renal recovery. The rare coexistence of these two entities is analyzed. The case highlights the importance of maintaining a high index of diagnostic suspicion in the face of atypical clinical presentations.

Archivos Argentinos de PediatriaVol. 124(4)
Hospital General de Niños Ricardo Gutierrez (AR)
Good health and well-being
Openalex Percentile: Top 94%
Vasculitis and related conditions
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