Plasmapheresis rescue therapy in progressive systemic ANCA-associated vasculitis: single-center results of stepwise escalation of immunosuppression.
OBJECTIVE
We evaluated 26 patients with antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) with progressive disease despite treatment with cyclophosphamide and steroids treated with additional plasmapheresis and compared outcome with 50 matched-disease controls.


METHODS
Patients diagnosed with AAV and treated with cyclophosphamide from January 1990 to December 2009 (n = 272) were included when plasmapheresis was not started at diagnosis but added for progressive disease during initial standard therapy (n = 26). We selected controls equal for age, Birmingham vasculitis activity score, and creatinine at diagnosis. Primary endpoint was estimated glomerular filtration rate (eGFR) or death.


RESULTS
Plasmapheresis was added 18 days (range 5-41) after start of therapy. In 11 patients, a rise in serum creatinine >30% led to plasmapheresis; insufficient response to induction (n = 11), progressive pulmonary disease (n = 3), or progressive necrotic lesions (n = 1) were other indications.In the plasmapheresis group, six patients were in need of renal replacement therapy (RRT), and three controls. Five years after diagnosis, four patients had died in the plasmapheresis against eight controls (P = 0.94). At baseline, mean eGFR was 44 ml/min/1.73 m(2) in plasmapheresis group versus 43 ml/min/1.73 m(2) in controls. At start of plasmapheresis, eGFR was 26 ml/min/1.73 m(2) (P = 0.003), at 6 months mean eGFR had significantly improved to 44 ml/min/1.73 m(2) (P = 0.0003), comparable to eGFR in controls, 48 ml/min/1.73 m(2). During long-term follow-up, there was no difference in renal function between the groups.


CONCLUSIONS
AAV patients with progressive disease despite standard induction therapy in whom plasmapheresis was added had significant improvement in renal function and similar long-term outcome in both renal and patient survival as matched disease controls.
Division of Nephrology, Department of Internal Medicine, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
Anoek A E
AA
de Joode
Jan S F
JS
Sanders
W Martin
WM
Smid
Coen A
CA
Stegeman
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000401:mortality / Q000503:physiopathology / Q000628:therapy*; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000032:analysis; D002097:C-Reactive Protein / Q000032:analysis; D005260:Female; D005919:Glomerular Filtration Rate; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D010956:Plasmapheresis / Q000009:adverse effects
D019268:Antibodies, Antineutrophil Cytoplasmic; D007166:Immunosuppressive Agents; D002097:C-Reactive Protein
apheresis; auto-immunity; granulomatosis with polyangiitis; microscopic polyangiitis