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

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Abstract

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.

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Funding

This work was funded by the National Natural Science Foundation of China (81800641 to TM), the National Key R&D Program of China (2020YFC2005000 to XX), the Key Research and Development Program of Hunan province (2018WK2060 to XX and 2020WK2008 to YZ), the Science and Technology Innovation Program of Hunan Province (2020RC5002 to JO), the Natural Science Foundation of Hunan Province (2021JJ31130 to YZ, 2020JJ6109 to CS and 2019JJ40515 to WN), and Chinese Society of Nephrology (18020010780 to YZ).

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Authors and Affiliations

  1. Department of Nephrology, Xiangya Hospital, Central South University, Changsha, Hunan Province, China

    Ting Meng, Rong Tang, Joshua D. Ooi, Zhou Xiao, Xiang Ao, Weisheng Peng, Wannian Nie, Qiaoling Zhou, Ping Xiao, Yong Zhong & Xiangcheng Xiao

  2. Department of Hematology, The Affiliated Zhuzhou Hospital Xiangya Medical College, Central South University, Zhuzhou, Hunan Province, China

    Chanjuan Shen

  3. Department of Pathology, Xiangya Hospital, Central South University, Changsha, Hunan Province, China

    Wei Lin

  4. Centre for Inflammatory Diseases, Department of Medicine, Monash University, Monash Medical Centre, Clayton, VIC, Australia

    Joshua D. Ooi & Peter J. Eggenhuizen

  5. Department of Rheumatology and Immunology, Xiangya Hospital Central South University, 87 Xiangya Road, Changsha, 410008, Hunan, People’s Republic of China

    Ya-Ou Zhou

  6. Department of Medical Records and Information, Xiangya Hospital, Central South University, Changsha, Hunan Province, China

    Jinbiao Chen

  7. Department of Pediatrics, Xiangya Hospital, Central South University, Changsha, Hunan Province, China

    Fang He

  8. Hunan Intellectual and Developmental Disabilities Research Center, Changsha, China

    Fang He

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  1. Ting Meng
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  2. Chanjuan Shen
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  3. Rong Tang
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  4. Wei Lin
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  5. Joshua D. Ooi
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  7. Ya-Ou Zhou
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  9. Fang He
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  10. Zhou Xiao
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  11. Xiang Ao
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  17. Xiangcheng Xiao
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Correspondence to Ya-Ou Zhou.

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Meng, T., Shen, C., Tang, R. et al. Clinical features and outcomes of anti-neutrophil cytoplasmic autoantibody-associated vasculitis in Chinese childhood-onset patients. Clin Exp Med 22, 447–453 (2022). https://doi.org/10.1007/s10238-021-00762-4

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  • DOI: https://doi.org/10.1007/s10238-021-00762-4

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