Antiglomerular basement membrane disease (Goodpasture syndrome) associated with ANCAnegative 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.