| Theme |
Management of Systemic Diseases with Blood Purification : Aute and Chronic Stage of Diseases |
| Title |
Goodpasture syndrome (lung hemorrhage and RPGN) |
| Author |
Masahiro Hagiwara |
Department of Internal Medicine, Sakura National Hospital |
| Author |
Kunihiro Yamagata |
Institute of Clinical Medicine, University of Tsukuba |
| Author |
Akio Koyama |
Institute of Clinical Medicine, University of Tsukuba |
| [ Summary ] |
Goodpasture's syndrome is an autoimmune disorder characterized by the presence of autoantibodies directed against a target restricted to the glomerular and a few other specialized basement membranes. Its classical manifestations are rapidly progressive nephritis and lung hemorrhage. The prognosis for this disease is improved by using a combination of both immunosuppressive and plasmapheresis theray, however renal outcome is still poor. To improve renal outcome, very rapid diagnosis and initiation of therapy is required. Although a recurrence of this disease is rare, 20-40% of patients have complications of ANCA related vasculits. Patients who have both ANCA and anti-GBM antibody have a higher possibility of recurrence during the course of the disease, even if they are receiving dialysis therapy. |