Clinical features and outcomes of anti-neutrophil cytoplasmic autoantibody-associated vasculitis in Chinese childhood-onset patients

Data on anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) are limited in children. This study is to determine the clinical features and outcomes of childhood-onset AAV. A retrospective study was performed on patients who were diagnosed with AAV before 18 years old in Xiangya Hospital....

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Published in:Clinical and experimental medicine 2022-08, Vol.22 (3), p.447-453
Main Authors: Meng, Ting, Shen, Chanjuan, Tang, Rong, Lin, Wei, Ooi, Joshua D., Eggenhuizen, Peter J., Zhou, Ya-Ou, Chen, Jinbiao, He, Fang, Xiao, Zhou, Ao, Xiang, Peng, Weisheng, Nie, Wannian, Zhou, Qiaoling, Xiao, Ping, Zhong, Yong, Xiao, Xiangcheng
Format: Article
Language:English
Subjects:
Autoantibodies
Biopsy
Children
Diagnosis
Dialysis
End-stage renal disease
Granulomatosis
Hematology
Immunomodulation
Immunosuppressive agents
Induction therapy
Internal Medicine
Intravenous administration
Kidney diseases
Leukocytes (neutrophilic)
Medical records
Medicine
Medicine & Public Health
Methylprednisolone
Neutrophils
Oncology
Original Article
Patients
Remission
Vasculitis
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Summary:Data on anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) are limited in children. This study is to determine the clinical features and outcomes of childhood-onset AAV. A retrospective study was performed on patients who were diagnosed with AAV before 18 years old in Xiangya Hospital. Their medical records were analyzed by retrospective review. Sixteen patients were diagnosed with AAV before 18 years old in the past 9 years, with an average age of 13.3 ± 3.3 years and 13 of them were female. There were 15 patients with microscopic polyangiitis (MPA) and 1 with Wegener’s granulomatosis. The interval between onset of disease and diagnosis of AAV was 2 (1.5–3) months. Most patients (15/16, 93.8%) had multi-organ involvement, and all patients had renal involvement with 7 (43.8%) patients requiring dialysis at presentation. Eleven patients underwent a renal biopsy, of which mixed class and sclerotic class were the most two common histological types. All patients received immunosuppressive therapy for induction therapy including intravenous administrations of methylprednisolone (MP) pulse therapy for 8 patients. 8 patients (50%) achieved remission after induction therapy. After a median follow-up of 46.3 ± 36.1 months, nine (56.3%) patients progressed to end-stage renal disease (ESRD) and 5 (31.3%) patients died. Childhood-onset AAV showed similar clinical and pathological features compared to those of adults, except that it usually occurs in girls. The most commonly involved organ was the kidney, and it had a high risk of progression to ESRD. Early diagnosis and initiation of appropriate immunomodulatory therapy would be important to improve outcomes.
ISSN:1591-9528
1591-8890
1591-9528
DOI:10.1007/s10238-021-00762-4