| Theme | Management of Systemic Diseases with Blood Purification : Aute and Chronic Stage of Diseases | |
|---|---|---|
| Title | Goodpasture syndrome (lung hemorrhage and RPGN) | |
| Publish Date | 2001/03 | |
| 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. | |