Prognostic Factors and Long-Term Outcome with ANCA-Associated Kidney Vasculitis in Childhood.

BACKGROUND AND OBJECTIVES: ANCA-associated vasculitis is extremely rare in children. We report the clinicopathologic features, long-term outcomes, and prognostic factors of a large pediatric cohort of patients with ANCA-associated kidney vasculitis. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: This retrospective study included 85 consecutive patients with kidney biopsy specimen-proven ANCA-associated vasculitis from tertiary referral centers in Italy and Canada. Kidney biopsy specimens were categorized as focal, crescentic, sclerotic, or mixed, according to the Berden classification. The prognostic significance of baseline clinical, laboratory, and histologic findings was analyzed with respect to kidney failure or CKD stage 3-5/kidney failure. RESULTS: A total of 53 patients had microscopic polyangiitis (62%), and 32 had granulomatosis with polyangiitis (38%). Rapidly progressive GN was the most frequent presentation (39%); a third of the patients also had nephrotic-range proteinuria. Kidney biopsy specimens were classified as focal in 21% of the patients, crescentic in 51%, sclerotic in 15%, and mixed in 13%. Remission-induction therapies included cyclophosphamide in 78% of patients. A total of 25 patients (29%) reached kidney failure. The median (interquartile range) time to kidney failure or last follow-up was 35 (6-89) months in the whole cohort, and 73 (24-109) months among the patients who did not reach this outcome. Patients whose biopsy specimens showed sclerotic histology had significantly shorter kidney survival (hazard ratio, 11.80; 95% confidence interval, 2.49 to 55.99) and survival free of CKD stage 3-5 (hazard ratio, 8.88; 95% confidence interval, 2.43 to 32.48), as compared with those with focal/mixed histology. Baseline eGFR, low serum albumin, hypertension, central nervous system complications, and sclerotic histology, which reflected severe kidney involvement, were associated with both kidney failure and CKD stage 3-5/kidney failure at unadjusted analysis; no independent prognostic factors emerged at multivariable analysis. CONCLUSIONS: Children with ANCA-associated kidney vasculitis often have aggressive presentation; a third of such children progress to kidney failure and this usually occurs early during follow-up. A severe clinical presentation is associated with the development of CKD or kidney failure.

Association between Satisfaction with Dialysis Treatment and Quality of Life: A Cross-Sectional Study.

INTRODUCTION: CKD is associated with a reduction of patients' health-related quality of life. Considering the time spent in dialysis, satisfaction with care is essential for patients QOL. OBJECTIVE: Since the possible association between satisfaction with the dialysis care and QOL has never been studied, in this study, we explore this plausible link. METHODS: One hundred three patients on hemodialysis (HD) and peritoneal dialysis (PD) filled-in patient-reported outcome measures (PROMs) and patient-reported experience measures (PREMs). QOL was assessed by Kidney Disease Quality of Life-36 and satisfaction by Choices for Healthy Outcomes in Caring for End-Stage Renal Disease (CHOICE) questionnaire. The analysis was conducted on patient-level, considering for single patient sociodemographic characteristics and presence of depression/anxiety. One-way ANOVA was used to compare QOL mean scores for patients who answered "excellent" and for those who answered "other ratings" in CHOICE questionnaire and the Pearson χ2 test to compare the patients' characteristics between these 2 groups of patients. RESULTS: The analysis showed a significant positive association between PREM and PROM scores for 8 out of 23 CHOICE items. Six of them were related to the figure of nephrologist, 1 to dialysis access site, and 1 to the social worker support. Significant association (p < 0.05) were between frequency of seeing nephrologist and physical component plus mental component, accuracy of information from nephrologist and burden of disease, accuracy of instructions from nephrologist and burden of disease, coordination between nephrologist and other physicians plus mental component, attention to cleanliness of access site and mental component, amount of dialysis information from staff and burden of disease, information from staff when choosing between HD or PD and physical component plus burden of disease, and ease of seeing social worker and burden of disease. CONCLUSIONS: The study provides support for the relationship between the care satisfaction and QOL, highlighting the central role of the nephrologist-patient communication in the QOL of dialysis patients.