Background/Purpose:

Renal disease is the most common manifestation of pediatric anti-neutrophil cycloplasmic antibody (ANCA) associated vasculitis (AAV). Renal disease course and early trajectories have not been well studied. The aim of this project was to describe renal disease at time of diagnosis (TOD) and follow glomerular filtration rate (GFR) trajectories over 12-months.

Methods:

PedVas is a multi-center international study that is currently collecting clinical and biological data from children with chronic vasculitis. The clinical data is stored in ARChiVe (A Registry for Childhood Vasculitis). Patients entered into ARChiVE with granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA) and renal disease were included. Renal disease was defined as biopsy confirmed pauci-immune glomerulonephritis OR dialysis dependence at TOD. Patients had to be less than 18 years of age at TOD. GFR was estimated using the bedside Schwartz formula. GFR based outcomes were reported as proportion of children with normal GFR (>90 ml/min/1.73m²), mildly reduced (MildR) (60-89 ml/min/1.73m²), moderately reduced (ModR) (30-59 ml/min/1.73m²), severely reduced (SR) (15-29 ml/min/1.73m²), and renal failure (RF) (<15 ml/min/1.73m², or requirement of dialysis).

Results:

109 patients met inclusion criteria. 64% were female. 81% had GPA and 19% had MPA. No patients with EGPA had renal disease. Median age at diagnosis was 13.8 years (range 3.9 to 17.8 yrs). Ethnicity data was available for 83% of patients. Of those, 61% were Caucasian, 13% East Indian/South Asian, 9% Hispanic, and 17% other (Asian, Black, Aboriginal, Mixed, or Other). Renal manifestations at TOD included: hematuria (92%), proteinuria (84%), hypertension (29%), oliguria (20%), and nephrotic syndrome (14%). 29 patients (27%) required dialysis at TOD. Cyclophosphamide (CYC) and/or rituximab (RTX) were used as primary induction treatments in 93% of patients. 10% of patients received both CYC and RTX. 30% of patients also received plasmapheresis. All patients received corticosteroids.

At TOD, 36% of patients had a normal GFR. The remaining patients had reduced GFRs as follows: 6% MildR, 16% ModR, 12% SR, and 30% RF. By post-induction (4-6 months following diagnosis), 28% of patients had a normal GFR, 32% MildR, 17% ModR, 4% SR, and 19% RF. 71 patients had complete 12-month visit data. 21 patients (30%) had normal GFR, 34% MildR, 17% ModR, 3% SR, 14% RF, and 2 patients had been transplanted. Of the 21 patients with normal GFR, 17 (81%) had achieved a normal GFR by post-induction. Of patients requiring dialysis at TOD, 85% had ModR GFR or worse at 12-months, 50% remained on dialysis or were transplanted.

Conclusion:

More than half of children with AAV associated renal disease have moderately reduced renal function or worse at diagnosis. In this cohort, only modest improvements in GFR were seen by post-induction, and minimal improvements were seen beyond that despite aggressive treatment. At 12-months, only 30% of children had normal GFRs. The majority of patients who required dialysis at TOD continued to have significantly reduced renal function at 12-months.

« Back to 2017 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/renal-disease-course-in-pediatric-anti-neutrophil-cytoplasmic-antibody-associated-vasculitis-in-the-first-12-months-a-pediatric-vasculitis-initiative-pedvas-study/