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Abbreviations CFA common femoral artery CFV common femoral vein CIA common iliac artery eGFR estimated GFR EIA external iliac artery EIV external iliac vein ICU intensive care unit IIV internal iliac vein LMWH low-molecular-weight heparin PN partial nephrectomy US ultrasonography Case Presentation A 62-year-old man was referred to our team with a 2.1-cm mass in the upper pole of his transplanted kidney, located in the right iliac fossa. The right common iliac artery (CIA), proximal right external iliac artery (EIA), proximal right external iliac vein (EIV) and internal iliac vein (IIV) were all dissected and looped. The patient's 2.1-cm tumour was deemed amenable to partial nephrectomy (PN), which was preferred to radical nephrectomy given the expressed desire of the patient to avoid the need for dialysis if practicable. [Extracted from the article]

Objective: The treatment of antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV) aims to suppress disease activity and prevent subsequent disease flare. This study sought to explore the association of early disease control with long‐term outcomes to validate early disease control as an end point for future clinical trials in AAV. Methods: Data from 4 European Vasculitis Society inception clinical trials in AAV (1995–2002) and subsequent data on long‐term outcomes from the trial data registry were studied. Clinical parameters in patients with AAV at baseline and at 3 and 6 months after diagnosis were assessed to study the long‐term risk of death and end‐stage renal failure (ESRF). At 6 months, outcomes were defined based on a disease status of either sustained remission (remission by 3 months, sustained to 6 months), late remission (remission after 3 months and by 6 months), relapsing disease (remission by 3 months but relapse by 6 months), or refractory disease (no remission by 6 months). Results: Of the 354 patients with AAV who were followed up for a median of 5.7 years, 46 (13%) developed ESRF, 66 (18.6%) died, and 89 (25.1%) had either died or developed ESRF. At 6 months, predictors of the composite end point of death or ESRF were as follows: age (hazard ratio [HR] 1.02, 95% confidence interval [95% CI] 1–1.05; P = 0.012), estimated glomerular filtration rate (HR 0.94, 95% CI 0.92–0.95; P < 0.001), and disease status at 6 months (late remission, HR 2.94, 95% CI 1.1–7.85 [P = 0.031]; relapsing disease, HR 8.21, 95% CI 2.73–24.65 [P < 0.001]; refractory disease, HR 4.89, 95% CI 1.96–12.18 [P = 0.001]). Similar results were observed when these analyses were performed separately for death and for ESRF. Conclusion: The results of this study suggest that disease status at 3 and 6 months following the diagnosis of AAV may be predictive of the long‐term risk of mortality and ESRF, and therefore these may be valid end points for induction trials in AAV. The current findings need to be validated in a larger data set. [ABSTRACT FROM AUTHOR]

Objective The NORAM (Nonrenal Wegener's Granulomatosis Treated Alternatively with Methotrexate [MTX]) trial demonstrated that MTX can replace cyclophosphamide (CYC) as remission-inducing treatment for patients with newly diagnosed early systemic antineutrophil cytoplasmic antibody-associated vasculitis. Duration of relapse-free survival was longer among CYC-treated patients than among MTX-treated patients during short-term followup. The aim of the present study was to describe the long-term outcome in patients enrolled in the randomized clinical trial. Methods Outcome questionnaires were sent to investigators who had recruited patients for the NORAM trial. Patients treated with MTX for induction of remission (n = 49) were compared to CYC-treated patients (n = 46) with respect to immunosuppressive therapy during followup, relapse-free survival, mortality, and occurrence of other clinical events. Results The median duration of followup was 6 years (range 0.1-10.8 years). One patient developed end-stage renal disease, and 11 died. The number of patients affected by serious infection, malignancy, or severe organ failure did not differ between treatment groups, and no difference in survival rate was observed. The duration of corticosteroid therapy was longer in the MTX group during the 18 months of the trial ( P = 0.005). During subsequent followup, patients who were in the MTX group in the NORAM trial received corticosteroids, CYC, and other immunosuppressive agents (azathioprine, MTX, and/or mycophenolate mofetil) for longer periods than those who were in the CYC group ( P = 0.004, P = 0.037, and P = 0.031, respectively). The cumulative relapse-free survival tended to be lower in the MTX group ( P = 0.056). Conclusion In the NORAM cohort, no difference in occurrence of major adverse events was observed between treatment groups during long-term followup. However, first-line treatment with MTX was associated with less effective disease control than CYC-based induction therapy. [ABSTRACT FROM AUTHOR]

Objective To determine the association between characteristics at diagnosis and the time to first relapse in a large cohort of patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV). Methods We studied long-term followup data from 4 clinical trials that included newly diagnosed patients with a broad spectrum of AAV severity and manifestations. Patient and disease characteristics at baseline were used in competing risk regression models with relapse as the event of interest and death as the competing event. Results We assessed 535 patients with 1,804 patient-years at risk of relapse. At diagnosis, the median age was 60.7 years (interquartile range [IQR] 48.8-69.1 years), 284 patients (53%) had granulomatosis with polyangiitis (Wegener's), and the median creatinine level was 203 μmoles/liter (IQR 97-498). A total of 201 patients (38%) experienced a relapse and 133 patients (25%) died, 96 of whom had not had prior relapse. Anti-proteinase 3 antibodies (subhazard ratio [sHR] 1.62 [95% confidence interval 1.39-1.89]) and cardiovascular involvement (sHR 1.59 [95% confidence interval 1.07-2.37]) were independently associated with a higher risk of relapse. Compared with patients with a creatinine level ≤100 μmoles/liter, patients with higher creatinine levels had a lower risk of relapse (sHR 0.81 [95% confidence interval 0.77-0.85] for a creatinine level of 101-200 μmoles/liter; sHR 0.39 [95% confidence interval 0.22-0.69] for a creatinine level >200 μmoles/liter). Conclusion Relapse of disease is common for patients with AAV. A creatinine level >200 μmoles/liter at the time of diagnosis is strongly associated with a reduced risk of relapse and may help guide monitoring and treatment of patients with AAV. [ABSTRACT FROM AUTHOR]

Objective To create a prognostic tool to quantify the 5-year cardiovascular (CV) risk in patients with newly diagnosed Wegener's granulomatosis (WG) and microscopic polyangiitis (MPA) without premorbid CV disease. Methods We reviewed CV outcomes during the long-term followup of patients in the first 4 European Vasculitis Study Group (EUVAS) trials of WG and MPA. CV events were defined as CV death, stroke, myocardial infarction, coronary artery bypass graft, or percutaneous coronary intervention. Logistic regression was performed to create a model to predict the absolute risk of a CV event. The model was tested using the Wegener's Granulomatosis Etanercept Trial (WGET) cohort. Results Seventy-four (13.8%) of 535 patients with 5 years of followup from the EUVAS trials had at least 1 CV event: 33 (11.7%) of 281 WG versus 41 (16.1%) of 254 MPA. The independent determinants of CV outcomes were older age (odds ratio [OR] 1.45, 95% confidence interval [95% CI] 1.11-1.90), diastolic hypertension (OR 1.97, 95% CI 0.98-3.95), and positive proteinase 3 (PR3) antineutrophil cytoplasmic antibody (ANCA) status (OR 0.39, 95% CI 0.20-0.74). The model was validated using the WGET cohort (area under the receiver operating characteristic curve of 0.80). Conclusion Within 5 years of diagnosis of WG or MPA, 14% of patients will have a CV event. We have constructed and validated a tool to quantify the risk of a CV event based on age, diastolic hypertension, and PR3 ANCA status in patients without prior CV disease. In patients with vasculitis, PR3 ANCA is associated with a reduced CV risk compared to myeloperoxidase ANCA or negative ANCA status. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented which discusses consideration of steroid-sparing treatment strategies for patients with early systemic antibody-associated vasculitis (AAV) who experience a relapsing disease.