Treatment of Pulmonary Vasculitis

Benjamin Terrier, MD, PhD; Loïc Guillevin, MD

Disclosures

Semin Respir Crit Care Med. 2018;39(4):504-510. 

In This Article

Antiglomerular Basement Membrane Disease (Goodpasture's Disease)

Treatment should be started as the diagnosis is highly suspected or confirmed. Initial therapy includes TPE in combination with prednisone or prednisolone and CYC, and supportive care as needed with dialysis and mechanical ventilation.

TPE are performed daily, with 5% human albumin solution or in combination with fresh-frozen plasma in patients with pulmonary hemorrhage. TPE are continued for 14 days or until antiglomerular basement membrane (anti-GBM) antibody levels are fully suppressed. Prednisone or prednisolone are prescribed orally at 1 mg/kg/d (maximum 60 mg/d), then the dose is reduced weekly to 20 mg/d by week 6 and then more slowly to stop completely by 6 months. Even if pulses of methylprednisolone are frequently prescribed, there is no evidence of their benefit. CYC is classically administered orally, at 2 to 3 mg/kg/d for a duration from 2 to 3 months. Importantly, no maintenance therapy is recommended.[26]

Novel treatment options were described during the past decade. RTX was suggested to represent an additional and/or alternative therapy for the treatment of anti-GBM disease in case reports and small series.[27]

Recently, an IgG-degrading enzyme derived from Streptococcus pyogenes (IdeS), an endopeptidase, was shown to cleave human IgG into F(ab')2 and Fc fragments inhibiting complement-dependent cytotoxicity and antibody-dependent cellular cytotoxicity. IdeS was evaluated in human leukocyte antigen (HLA)–sensitized patients before the transplantation of a kidney from HLA-incompatible donor, and reduced or eliminated donor-specific antibodies and permitted HLA-incompatible transplantation.[28] An open-label phase II study in anti-GBM disease is currently ongoing (NCT03157037).

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