Background
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a small- and medium-sized vasculitis classically seen in adult patients, with peak onset near the fifth to seventh ...decade of life. There is little data on ANCA-associated vasculitis in pediatric patients, and most studies have limited follow-up.
Methods
This is a retrospective chart review of 22 patients with ANCA-positive glomerulonephritis in a single institution from 1991 to 2013.
Results
Of the 22 patients, eight (36 %) required renal replacement therapy (RRT) at diagnosis; four of these patients recovered sufficient renal function to initially discontinue dialysis. Five patients (23 %) were treated with plasmapheresis at presentation. The median time from presentation until first clinical or serologic relapse was 1.7 ± 1.2 years. After a median follow-up of 5.8 years, just over half of our patients had chronic kidney disease (CKD) stages 1–3 (55 %). Seven (32 %) patients progressed to end-stage renal disease (ESRD) and eventually required kidney transplant.
Conclusion
ANCA-associated glomerulonephritis is a rare disorder in children. Presentation and outcomes vary significantly among patients. More research is required to follow these patients who are diagnosed in childhood to further characterize the long-term outcome of the disease.
Cardiovascular disease (CVD) is a significant cause of morbidity and mortality in children with chronic kidney disease (CKD). The cause of CVD in children with CKD is multifactorial and there are new ...and emerging data regarding prevalence and risk factors for CVD in this population.
A number of recent publications from longitudinal cohort studies of children with CKD have greatly increased our knowledge about the prevalence and risk factors for CVD including hypertension, obesity and dyslipidaemia. Masked hypertension and isolated nocturnal hypertension both correlate with surrogate markers of CVD in children. Obesity and adiposity are associated with an increased risk of CVD. Markers other than BMI such as waist to height ratio and fat-free tissue to fat tissue ratio better correlate with the presence of CVD in children. Dyslipidaemia is extremely prevalent in the paediatric CKD population, but there is a lack of consensus on treatment. More data on the relationship between bone mineral disease and CVD continue to emerge including an association between hyperparathyroidism and isolated nocturnal hypertension.
Children with CKD have multiple potentially modifiable risk factors for CVD. Research focused on CVD outcomes in children is needed.
The purpose of this review is to highlight recent studies that have emerged on the topic of ANCA-associated vasculitis with some historical context. The review also discusses how the adult data is ...relevant to pediatric patients.
Pediatric studies on AAV are lacking. Therapies targeted to the inflammatory cascade specifically implicated in AAV, such as MPO inhibitors and complement mediators, are emerging. The PEXIVAS study recently called into question the routine use of plasma exchange (PLEX) in severe AAV, with no difference in ESKD or mortality found between patients who did or did not receive PLEX. Longer maintenance duration of nearly 48 months is preferred as compared with shorter duration in patients who are not on dialysis because of higher relapse rates in children with AAV.
Current treatment in AAV includes corticosteroids, rituximab, and cyclophosphamide for induction. Maintenance therapy commonly consists of azathioprine or rituximab. Plasma exchange (PLEX) is no longer recommended for induction therapy for AAV but some experts still consider this as an option for patients who are not responding to therapy or have severe disease at presentation. However, emerging novel therapies may be on the horizon.
Advances in immunosuppression have improved graft survival in pediatric kidney transplant recipients; however, treatment‐related toxicities need to be balanced against the possibility of graft ...rejection. Several immunosuppressive agents are available for use in transplant recipients; however, the optimal combinations of agents remain unclear, resulting in variations in institutional protocols. Lymphocyte‐depleting antibodies, specifically ATG, are the most common induction agent used for pediatric kidney transplantation in the US. Basiliximab may be used for induction in immunologically low‐risk children; however, pediatric data are scarce. CNIs and antiproliferative agents (mostly Tac and mycophenolate in recent years) constitute the backbone of maintenance immunosuppression. Steroid‐avoidance maintenance regimens remain controversial. Belatacept and mTOR inhibitors are used in children under specific circumstances such as non‐adherence or CNI toxicity. This article reviews the indications, mechanism of action, efficacy, dosing, and side effect profiles of various immunosuppressive agents available for pediatric kidney transplantation.
Background
Corticosteroids have been an integral part of maintenance immunosuppression for pediatric kidney transplantation. However, prolonged steroid therapy is associated with significant ...toxicities resulting in several SW/avoidance strategies in recent years.
Method/Objective
This comprehensive review aims to discuss steroid‐related toxicities and the safety, efficacy, and benefit of steroid avoidance/withdrawal immunosuppression in pediatric kidney transplant recipients.
Results
Initial studies of SW/avoidance conducted in the setting of CSA and AZA showed an increased incidence of AR but no increase in graft loss or mortality with SW/avoidance maintenance immunosuppression. Studies performed under modern immunosuppression (induction therapy, Tac, and MMF) show no significant increase in AR or graft loss with SW/avoidance immunosuppression. Furthermore, SW/avoidance immunosuppression is associated with significant improvement in growth, BMI, BP control, and lipid profile in pediatric kidney transplant recipients. Despite these data, SW/avoidance remains controversial, and only 40% of pediatric kidney transplant recipients in the United States are currently on SW/avoidance maintenance immunosuppression.
Conclusion
SW/avoidance maintenance immunosuppression is safe and associated with fewer side effects compared with steroid‐inclusive maintenance immunosuppression in pediatric kidney transplant recipients.
Background: Renal biopsy plays an important role in the establishment of the diagnosis and the management of patients with lupus nephritis. Immunoglobulin A (IgA) nephropathy rarely has been reported ...in kidney biopsy of lupus patients. Lupus nephritis and IgA nephropathy can be readily diagnosed on renal biopsy when the classic patterns are present. However, atypical patterns can become a diagnostic challenge. Galactose-deficient IgA1 (Gd-IgA1) is a key element in the pathogenesis of primary IgA nephropathy. Glomerular Gd-IgA1 deposits, detected by immunofluorescent staining of KM-55 (a Gd-IgA1-specific monoclonal antibody), are consistently identified in the mesangium of IgA nephropathy but are significantly less or absent in lupus nephritis accompanied by significant IgA deposition. Case Presentation: Here we report the case of an 11-year-old girl who was recently diagnosed with systemic lupus erythematosus (SLE) and was found to have hematuria and proteinuria. Renal biopsy showed focal mesangial hypercellularity with IgA dominant, “full house” like pattern of mesangial deposition. The biopsy findings present a diagnostic dilemma with the differential diagnosis of IgA nephropathy versus lupus nephritis with atypical immunofluorescence, and IgA nephropathy is favored, in the absence of strong straining of C1q or C3, extraglomerular deposits, tissue antinuclear antibodies, and endothelial tubuloreticular inclusions. However, no detectable glomerular KM-55 staining was seen in the kidney biopsy. Conclusions: We demonstrate the unique diagnostic utility of immunostaining for KM-55 in a challenging kidney biopsy of an SLE patient with features suggestive of IgA nephropathy. The absence of KM-55 staining excludes IgA nephropathy, supporting a diagnosis of lupus nephritis with atypical immunofluorescence in this patient with SLE.
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