Table 1.
Initial Treatment of Anti-GBM Disease
| Agent | Details and Duration | Cautions |
| Plasma exchange | Daily 4 L exchange for 5% human albumin solution. Add fresh human plasma (300–600 ml) within 3 d of invasive procedure (e.g., kidney biopsy) or in patients with alveolar hemorrhage. Continue for 14 d or until antibody levels are fully suppressed. Monitor antibody levels regularly after cessation of treatment because plasma exchange may require reinstatement if antibody levels rebound. | Monitor and correct as required: platelet count, aim >70 × 109/L; fibrinogen, aim >1 g/L (may require cryoprecipitate supplementation to support PEX); hemoglobin, aim for >90 g/L; corrected calcium, aim to keep in normal range |
| Cyclophosphamide | 2–3 mg/kg per d given orally for 2–3 mo. Reduce dose to 2 mg/kg in patients>55 yr. | Stop if leukocyte count falls to <4 × 109/L and restart at reduced dose when recovered. Insufficient evidence to recommend use of IV cyclophosphamide. |
| Corticosteroids | Prednisolone 1 mg/kg per d (maximum 60 mg) given orally. Reduce dose weekly to 20 mg by 6 wk, then gradually taper until complete discontinuation at 6–9 mo. | There is no evidence to support the use of methylprednisolone, and it may increase the risk of infection |
| Prophylactic treatments | Prophylaxis against oropharyngeal fungal infection (e.g., nystatin, amphotericin, or fluconazole) while on high-dose steroids. Peptic ulcer prophylaxis (e.g., with PPI) while on high-dose steroid treatment. Prophylaxis against PCP (e.g., cotrimoxazole) while receiving high-dose corticosteroids and cyclophosphamide. Consider acyclovir for CMV prophylaxis. Consider prophylaxis against HBV reactivation (e.g., lamivudine) in patients who have evidence of previous infection (HBV cAb positive). | H2 receptor antagonists in those who are intolerant of PPI. Cotrimoxazole may contribute to leukopenia; monitor leukocyte count. Alternatives include nebulized pentamidine. |
Modified from reference 13, with permission. GBM, glomerular basement membrane; PEX, plasma exchange; IV, intravenous; PPI, proton pump inhibitor; PCP, Pneumocystis jiroveci pneumonia; CMV, cytomegalovirus; HBV, hepatitis B virus; cAb, core antibody.