P22 | Granulomatosis with polyangiitis: plasmapheresis as a method to reduce permanent renal failure and incidence of side effects of rituxibab

V. Bosco¹, F. Mauceri¹, V. Nicotra², P. Gargano¹, M. Carbone¹, M. Romano¹, S. Urso³ | ¹Department of Clinical and Experimental Medicine, University of Catania, Garibaldi Hospital, Catania, ²Geriatric Department, Garibaldi-Nesima Hospital, Catania, ³UOSD Nephrology, Garibaldi-Nesima Hospital, Catania, Italy

Introduction: Granulomatosis with polyangiitis (GPA) is a rare vasculitis affecting small to medium-sized vessels with circulating anti-neutrophil cytoplasmic antibodies (ANCA).
Case report: A 57-year-old man was admitted to Nephrology Department due to new-onset acute renal failure. The blood chemistry tests showed positivity of ANCA while chest CT demonstrated a detection of two foci of a thickening nature in the right lung. Patient’s condition was critical due to kidney damage and so patient was started on emergency hemodialysis. Kidney biopsy demonstrated a paucimmune crescentic necrotizing glomerulonephritis ANCA-associated and so patient was treated with steroid therapy and rituximab for 4 weeks. He also underwent six plasmapheresis sessions with good clinical response. After such therapy, the patient’s renal function improved and hemodialysis was discontinued. With this protocol, he didn’t have disease recurrence in the following 24 months, neither as kidney disease nor as lung disease. After approximately 24 months, patient had a recurrence of lung disease and a single infusion of rituximab was administered with clinical improvement.
Conclusions: Our case demonstrates that plasma exchange makes additional benefit in cases of GPA with severe renal failure. Furthermore, a single dose of rituximab upon disease recurrence allows for a reduction in relapse rate. We propose that in remission phase a personalized rituximab infusion protocol should be preferred rather than a fixed protocol (at 6, 12 and 18 months) to reduce the incidence of side effects of drug.