Abstract
Results Thirty-six patients were included in the final study. Cyclophosphamide was used in 24 patients (66.7%) and, comparatively, rituximab in 7 patients (19.4%) for induction. Seven patients (19.4%) had a documented relapse, and six patients (85.7%) had rituximab as induction therapy for relapse. The majority of patients were on azathioprine (61.1%, 57.1% relapse population) as maintenance therapy. Progression to ESRD occurred in 11 (30.6%), death in 4 (11.1%), established CKD in 15 (41.7%), and preservation of renal function in 6 (16.7%) patients by the end of the follow-up period. Conclusions While cyclophosphamide remains the choice of induction immunosuppression therapy, we favour rituximab as an induction agent in the relapse of AAV. Despite aggressive immunosuppression therapy, the incidence of ESRD and death remains high in these patients.
Highlights
Antineutrophil cytoplasmic antibody- (ANCA-) associated vasculitis (AAV) is a group of disorders including granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), and microscopic polyangiitis (MPA), which are characterised by systemic necrotising inflammation in small- and medium-size vessels leading to end-organ damage [1, 2]
In lifethreatening or organ-threatening AAV, cyclophosphamide or rituximab with glucocorticoid is recommended as induction agents. e addition of plasmapheresis (PLEX) can be considered for rapidly progressive renal failure or severe diffuse pulmonary haemorrhage [2,3,4]
E inclusion criteria were those with a clinical diagnosis of AAV consistent with the Chapel Hill consensus definition, positive serology, i.e., antiproteinase or antimyeloperoxidase antibody positivity, renal biopsy showing pauci-immune necrotising glomerulonephritis, or active urine sediment characterised by glomerular haematuria or red cell casts and proteinuria, or clinical features consistent with AAV. e exclusion criteria were patients with a diagnosis of vasculitis other than granulomatosis with polyangiitis or microscopic polyangiitis or renal histological features suggestive of other types of glomerulonephritis such as linear IgG deposition on immunofluorescence, age less than 16 years, and patients with incomplete records
Summary
E overall annual incidence of AAV is estimated to be around 0.4–24 per million population per year [3]. E prevalence of MPA approximately ranges from 9 to 94 per million population [3]. E prevalence of AAV is more common in people of European ancestry [5]. In Southern Europe and Asia, MPA is more prevalent, while in Northern Europe, GPA is more common [6]. In lifethreatening or organ-threatening AAV, cyclophosphamide or rituximab with glucocorticoid is recommended as induction agents. Azathioprine, rituximab, or methotrexate, with glucocorticoid tapering, is recommended maintenance therapies [8]. Cyclophosphamide is the conventional induction agent used by nephrologists in AAV.
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