علاج التهاب الكبيبات التدريجي السريع في مرض الأجسام المضادة لغشاء الكبيبات القاعدي الإيجابي

References

• In patients with anti-GBM antibody glomerulonephritis presenting with RPGN, the combined use of corticosteroids and immunosuppressive agents, in addition to plasmapheresis, is suggested as the standard treatment.
• Patient survival and kidney survival in anti-GBM antibody-positive RPGN are poor.
• The clinical guideline in Japan recommends immunosuppressive therapy (corticosteroids and cyclophosphamide) plus plasmapheresis. We recommend cyclophosphamide (1–2 mg/kg/day) for patients with refractory GN. However, it is necessary to reduce the dose of cyclophosphamide in patients with advanced renal dysfunction.
• In patients with anti-GBM antibody disease presenting with RPGN, adding intravenous pulse corticosteroid therapy to oral corticosteroids is recommended to improve survival when pulmonary hemorrhage is present (i.e., Goodpasture syndrome).
• Therefore, initial therapy with corticosteroids alone is recommended, usually combined with plasmapheresis, in cases in which the use of immunosuppressive agents is not desirable because of adverse effects.