The aim of the present review was to summarize the available literature on AAVs in KT, particularly focusing on de novo pauci-immune glomerulonephritis. Keywords:chronic kidney disease, kidney transplant, ANCA-associated vasculitis, pauci-immune glomerulonephritis, patient survival, graft survival, outcome, review == 1. been obtained using high-dose steroids, mycophenolate mofetil, or cyclophosphamide. The aim of the present review was to summarize the available literature on AAVs in KT, particularly focusing on de novo pauci-immune glomerulonephritis. Keywords:chronic kidney disease, kidney transplant, ANCA-associated vasculitis, pauci-immune glomerulonephritis, patient survival, graft survival, outcome, review == 1. Survival of Patients with Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis == Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAVs) represents a heterogeneous group of small-vessel vasculitis including systemic forms such as granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA or ChurgStrauss syndrome) as well as forms limited to the kidney, also Rabbit polyclonal to FN1 known as renal-limited RIP2 kinase inhibitor 2 vasculitis (RLVs) [1,2]. Therefore, the spectrum of AAVs may range from isolated and indolent single-organ involvement to life-threatening fulminant disease. Until recently, the prognosis of untreated patients with AAVs has been extremely poor, with RIP2 kinase inhibitor 2 mortality rates as high as 80% within the first year of diagnosis and most of the deaths occurring due to renal or respiratory failure [3]. Over the years, tremendous advances in the understanding of the pathophysiology of AAVs and the RIP2 kinase inhibitor 2 development of more effective therapeutic strategies have led to a significant amelioration of results [4]. Nonetheless, long-term outcomes are still suboptimal. GPA and MPA have been associated with 5-year survival rates of 7479% and 4680%, respectively [5,6,7,8,9]. A large meta-analysis of observational studies including 3338 affected subjects concluded that there was a 2.7-fold increase in mortality among patients with AAVs compared with the general population, regardless of sex [10]. The risk of death is highest during the first year of follow-up, but excess mortality remains persistently elevated [11]. The leading causes RIP2 kinase inhibitor 2 of death in the short term are infections and active vasculitis, whereas long-term mortality is predominantly determined by cardiovascular disease (CVD), malignancy, or infections [5]. Remarkably, long-term survival may be further compromised by drug-induced side effects and associated comorbidities [8]. Recognized risk factors for adverse outcomes are severity of disease at onset, age at diagnosis, number of relapses, and duration of steroid administration [12]. The aim of the present review was to summarize current knowledge on AAVs in the KT setting, particularly focusing on the differences between recurrent and de novo forms of the disease. Most relevant follow-up and management strategies are also highlighted. == 2. End-Stage Renal Disease in ANCA-Associated Vasculitis == Renal vasculitis is a frequent manifestation of GPA and MPA, occurring in 50% of patients at onset and in up to 7085% during the course of the disease [13,14]. On the contrary, severe involvement of the kidney is seldom observed in EGPA. It has been estimated that a quarter of individuals with AAVs eventually develop end-stage renal disease (ESRD) within five years of diagnosis [13,15,16,17]. In particular, early renal replacement therapy (RRT) is often required in GPA and MPA patients RIP2 kinase inhibitor 2 presenting with rapid and progressive kidney failure. Among these subjects, one third actually fails to respond to treatment, thus requiring life-long dialysis or kidney transplantation (KT) [18]. Progressive deterioration of renal function can also be observed in patients with mild-to-moderate involvement of the kidney as a result of glomerular, tubular, or vascular scarring. The event of further episodes of renal vasculitis in association with systemic arterial hypertension, atherosclerosis, or diabetes may act as an important contributing element. There is a lack of information concerning AAVs individuals with chronic kidney disease (CKD), especially those on RRT. Analyzing data from 302 subjects with AAVs (MPA, 136 and GPA, 167), enrolled in six Western Vasculitis Society (EUVAS) tests and followed-up for 7.1 years, Robson and colleagues estimated a 14% incidence of ESRD [19]. Inside a Swedish observational cohort study having a 5-12 months follow-up and including.