Relationship between serologic profile (ANCA type) and clinical features of renal involvement in ANCA-associated vasculitides

Abstract

Objective. To compare the frequency, clinical features and outcomes of renal involvement in ANCA-associated vasculitides (AAV) in patients with antibodies against proteinase-3 (pr3-ANCA) and myeloperoxidase (MPO-ANCA). Materials and methods. In our retrospective study we enrolled 264 patients, 94 males and 170 females, median age 53 [36; 62] years. Among them 157 were pr3-ANCA positive and 107 were MPO-ANCA positive. AAV was diagnosed according to ACR criteria and Chapel Hill consensus conference definition (2012). Median follow up was 44 [18; 93] months. We assessed baseline BVAS and VDI by the end of the follow up. Serum creatinine (sCr), estimated glomerular filtration rate (eGFR), hematuria and daily proteinuria were estimated. Diagnosis and stage of chronic kidney disease (CKD) and acute kidney injury (AKI) were established according to KDIGO guidelines (2012) and Scientific Society of Russian Nephrologists (2016). Results. Renal involvement was present in 181 (68.6%) patients, and its frequency was similar in pr3-ANCA and MPO-ANCA subgroups. Patients with MPO-ANCA developed rapidly progressive glomerulonephritis and hypertension significantly more often than patients with pr3-ANCA: 50.7% vs 35.6% (p=0.049) and 46.1% vs 29.8% (p=0.029) respectively. At disease onset, median sCr was significantly higher and eGFR was significantly lower in patients with MPO-ANCA (p

Healthcare and economic burden of ANCA-associated vasculitis in Italy: an integrated analysis from clinical and administrative databases

“…Thus, the unmet needs of early diagnosis, in particular for renal involvement, and novel treatments, especially carrying significant steroidsparing effect, clearly emerged from these results [24,25]. Second, the ANCA status was confirmed to be an important prognostic factor [14,26]. ANCA-positive patients, in particular ANCA/PR3-positive patients, more frequently show a relapsing-remitting disease [14].…”

Autoantibodies Against Lysosome Associated Membrane Protein-2 (LAMP-2) in Pediatric Chronic Primary Systemic Vasculitis

“…ANCA positivity and specificity for either PR3 or MPO aids phenotype classification in adult and pediatric AAV, and in adult-onset AAV is associated with general features of disease course (7)(8)(9)(10). Their utility as prognostic markers for renal disease, which has a high prevalence among patients with AAV, may have limitations given that MPO and PR3 are not expressed on the glomerular endothelium.…”

“…For example, patients positive for PR3-ANCA often have a more relapsing disease course, increased risk of severe inflammatory lung disease, and systemic disease involving multiple organs at diagnosis (7,8). In contrast, MPO-ANCA positive patients are more likely to have more severe renal-limited disease (9,10). Some data on adult patients also supports the value of serially measuring ANCA titers as a marker of disease activity (7,11), but whether ANCA are informative to organ-specific disease processes, which is a primary determinant in treatment decisions, remains to be shown.…”

Upper respiratory tract manifestations in patients with ANCA-associated vasculitides and their association with the presence and type of ANCA

“…Тем не менее, нами также было показано независимое влияние серотипа АНЦА на клинико-рентгенологический паттерн поражения ВДП в рамках ГПА. Ранее влияние типа АНЦА на характер течения и вовлечение тех или иных систем органов при ААВ было описано в многочисленных работах отечественных и зарубежных авторов, однако конкретные клинические особенности поражения ВДП в них затронуты не были [14][15][16][17][18]. В работе J.H.…”