Introduction:
ANCA vasculitis is an uncommon disorder in children with a propensity for dialysis-dependent kidney failure and progression to end-stage kidney disease. Renal failure at onset is associated with poor prognosis. Because of the rarity of the disease, there is limited clinical data regarding ANCA vasculitis in children from India. We conducted this retrospective study to analyze the clinical presentation and outcome of ANCA vasculitis at a tertiary care teaching hospital in South India over past 4 years from June 2020 to June 2024.
Methods:
Objectives: To analyze the clinical profile and outcome of ANCA vasculitis in children, renal histopathology in biopsy specimens, and correlate their implications on the clinical parameters of disease activity with long-term outcomes.
Methodology: This is a 4 year single-centre retrospective study conducted in the Paediatric Nephrology division of Christian Medical College, Vellore from 2020 to 2024. Clinical data, renal histopathology and renal outcome were analysed.
Results:
ANCA vasculitis was diagnosed in 14 out of 1356 children (1%) during the study period. The majority were boys 57% (8/14) with mean age of 13.8 ± 1.25 years at presentation. The median duration of follow-up was 8 (2, 21) months. All children had fever, malaise, pallor, loss of appetite and weight, vomiting, edema, oliguria, and elevated ESR. Half of the children presented with renal failure of which one-half had rapidly progressive renal failure and the remaining presented with end-stage kidney disease. Figure 1 depicts the demography of children with ANCA vasculitis.
The mean eGFR at onset was 13.4 ± 2.3 ml/min. All patients with renal failure at presentation required hemodialysis and one-third of them had ESKD at onset. Anti-MPO positivity was predominant in 71% of patients (10/14), followed by anti-PR3 (43%). Concomitant anti-GBM positivity was observed in one-fifth (21.4%) of the patients. The median anti-GBM titer was 199 RU/ml with a range (64, 590) in the dual-positive children.
Diffuse global glomerulosclerosis was seen in a median of 30% (range 12, 86) and fibro cellular crescents were present in 25% (range 10, 70). 4 (28.6%) of the children had more than 50% crescents. Apart from corticosteroids, the most common second-line immunosuppressive agent was mycophenolate mofetil (50%) followed by Rituximab (28.6%) and Cyclophosphamide (21.4%). Plasma exchange (TPE) was instituted for 3 out of 14 patients (21.4%).
At 2 years follow up 57 % (8/14) were in complete remission and 29% (4/14) in partial remission. Risk factors for GFR <60 ml/min at one year were long latent period between onset of symptoms and initiation of treatment, renal failure at onset requiring RRT, and 50% crescents in renal biopsy. Two children progressed to ESKD. Children with concomitant anti-GBM positivity tend to have worse outcomes at 3 months with higher creatinine and persistence on dialysis at 3 months.
Conclusions:
ANCA vasculitis is a rare (1% of all renal biopsies in 4 years) glomerular disease in children with very high percentage of them presenting with renal failure requiring RRT (50%), and one third having ESKD at onset. ANCA vasculitis should be suspected in children with rapidly progressive renal failure. If not detected and treated early, ANCA vasculitis can cause significant morbidity and ESKD.
I have no potential conflict of interest to disclose.
I did not use generative AI and AI-assisted technologies in the writing process.