Goodpasture’s syndrome with neurologic and renal vascular involvement and negative ANCA

Goodpasture’s syndrome (GPS) is a rare but severe vasculitis disease, characterized by rapidly progressive glomerulonephritis (RPGN), diffuse pulmonary hemorrhage in the presence of anti-glomerular basement membrane antibody (anti-GBM antibody). Central nervous system (CNS) was rarely involved, especially in the absence of anti-neutrophil cytoplasmic antibodies (ANCA). Typically, CNS vasculitis was one of important causes. Herein we present a case of GPS presenting RPGN, clonic-tonic seizures and bloody sputum. She underwent renal puncture and bleeding complication occurred afterwards. Due to the insufficiency of conservative therapy including transfusion, fluid infusion, selective renal arteriography embolization was then employed. Angiogram results showed beading changes of renal artery. Brain CT scan was found negative, but MRA imaging demonstrated the obvious involvement of cranial vessels. The changes of renal and cranial vascular involvement were highly suspicious of nervous vasculitis based on the primary disease. The patient was treated with corticoids and plasma exchange. Patient’s condition improved quickly and anti-GMB antibodies proved to be negative, but renal failure persisted, dependent on long-term hemodialysis. We described for the first-time association of Goodpasture’s syndrome with both renal and nervous vascular involvements.