Rapidly Progressive Glomerulonephritis with Delayed Appearance of Anti-Glomerular Basement Membrane Antibody Successfully Treated with Multiple Courses of Steroid Pulse Therapy

摘要

Patients with anti-glomerular basement membrane (GBM) antibody glomerulonephritis typically exhibit rapidly progressive glomerulonephritis (RPGN). The renal outcome as well as the prognosis of this disease is worse than other forms of RPGN such as those from microscopic polyangiitis. Therefore, early therapeutic intervention is essential to improve its prognosis. One month before referral to our hospital, a 54-year-old female attended another hospital because of macrohematuria. At that time, she had proteinuria and macrohematuria with normal renal function, was negative for anti-GBM antibodies, and was diagnosed with chronic glomerulonephritis. A month later when she was admitted to our hospital, she showed renal insufficiency and was positive for anti-GBM antibodies. Immediately after recognizing the anti-GBM antibody status, plasma exchange and the first course of steroid pulse therapy was started. After 5 days of therapy, renal biopsy confirmed severe crescentic glomerulonephritis in which all the observed glomeruli were involved with cellular crescents. Despite this, she survived without end-stage renal disease after three courses of steroid pulse therapy and seven sessions of plasma exchange. This favorable outcome reflects the repeated analysis of anti-GBM antibodies within a very short period and the rapid therapeutic intervention in addition to the intensive immunosuppressive therapies.