Goodpasture syndrome
ICD-10 M31.0 · ICD-11 MF85

Goodpasture Syndrome When Standard Therapy Fails to Suppress Anti-GBM Antibodies

In Goodpasture syndrome, achieving full suppression of anti-glomerular basement membrane (anti-GBM) antibodies is the central treatment target. When first-line immunosuppressive therapy does not meet this goal within the expected window, a defined next-line protocol applies.

Previous line — insufficient response

Standard first-line therapy — plasma exchange, cyclophosphamide, and corticosteroids — is considered insufficient when anti-GBM antibody levels are not fully suppressed within 14 days of initiating plasma exchange. Non-achievement of this target is the criterion for escalating to the next therapeutic step.

The protocol defines a cyclophosphamide-replacement strategy. Cyclophosphamide is substituted with one of two specified alternative agents — rituximab or mycophenolate mofetil — each with distinct timing requirements relative to ongoing corticosteroids and plasma exchange. The full regimen, agent selection criteria, and sequencing details are available in the structured protocol.

Reduction of anti-GBM antibodies.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.55563/clinexprheumatol/tep3k5

Therefore, waiting for randomised trials, RTX should be reasonably used in patients that are refractory to standard treatment or with contraindications to cyclophosphamide.

Few reports on successful treatment with mycophenolate mofetil and cyclosporine have been reported in literature.

RTX demonstrated a rapid reduction of anti-GBM antibodies, but without benefits on renal outcomes.

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