Immunosuppressive options are available in treatment resistant cases in Granulomatosis with polyangiitis

What other immunosuppressive options are available in treatment resistant cases in Granulomatosis with polyangiitis?

• Plasmapheresis or plasma exchange (PLEX) may be considered in life-threatening disease (rapidly progressive glomerulonephritis [RPGN] and DAH), although this is a controversial area. Data from the MEPEX trial has documented short-term improvement in the number of patients progressing to end-stage renal disease following PLEX, but long-term data from this same trial failed to show improvement in long-term renal outcomes and/or mortality. Preliminary data from the PEXIVAS trial has also questioned the benefit of PLEX in this setting, with further analysis needed for a definitive answer on the utility of this treatment. Note that PLEX removes 25% of the rituximab dose if PLEX is done within 24 hours of receiving rituximab, and removes 50% of the drug if PLEX is done daily for 3 days after receiving rituximab.

• IV gammaglobulin (2 g/kg divided over 5 days) has been described in some patients with GPA.

• A maximal B-cell depletion strategy (rituximab + cyclophosphamide) has been described in patients with GPA and advanced renal failure (RITUXIVAS study; see Question 18).

• Oral cyclophosphamide dosing regimens (up to 2 mg/kg daily) can be considered, albeit at higher risk of complications.

• Autologous stem cell transplant (bone marrow transplantation) has been used as salvage therapy in some patients.

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