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Clinical and pathological features of microscopic polyangiitis in 20 children
Liangzhong Sun1, Haiyan Wang2, Xiaoyun Jiang1
1From the Children's Kidney Disease Center, Department of Pediatrics, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, P.R. China.L. Sun, MD, PhD, Associate Chief Physician; H. Wang, MD, Resident; X. Jiang, MD, PhD, Chief Physician; Y. Mo, MD, Associate Chief Physician; Z. Yue, MD, Associate Chief Physician; L. Huang, MD, Associate Chief Physician; T. Liu, Graduate Student. Drs. Sun and Wang contributed equally to this study. xyjiang-3208@163.com sunlzh@mail.sysu.edu.cn.
Objective:
To explore the clinical and pathological features of microscopic polyangiitis (MPA) in children.
Methods:
A retrospective analysis was performed of patients with pediatric MPA in our hospital over 10 years.
Results:
Data for 20 patients were collected; 16 patients had primary MPA (4 boys, 12 girls), with a median age of 8.9 years at the time of disease onset; 4 patients, all female, had antithyroid drug (ATD)-associated MPA, with an age range of 12.5 to 16.2 years at the time of disease onset. All patients exhibited renal involvement. Renal biopsies were performed in 14 patients. Fibrinoid exudation and necrosis of the glomerular capillaries were observed in all biopsy specimens. Crescents and scleroses were noted in 92.9% and 85.7% of these cases, respectively. The most frequent extrarenal organs involved were lungs, followed by the central nervous system (CNS), skin, and digestive system. Ninety percent of patients were positive for perinuclear antineutrophil cytoplasmic antibody, 94.1% were positive for myeloperoxidase, and 88.2% were positive for both. Forty-five percent of the patients had received steroid plus cyclophosphamide (CTX) pulse therapy for more than 3 months, and varying degrees of remission had been achieved in 88.9% of the patients.
Conclusion:
Both primary and ATD-associated MPA showed a female predisposition. Renal involvement was the most frequently observed condition, followed by involvement of lungs. CNS involvement was not rare in these pediatric patients. The efficacy of steroid plus CTX as induction therapy was evident in these patients.
Insights
Microscopic polyangiitis (MPA) in children predominantly affects females and frequently involves the kidneys and lungs. Steroid plus cyclophosphamide (CTX) therapy shows efficacy in achieving remission in pediatric MPA patients.
Area of Science:
- Pediatric Rheumatology
- Nephrology
- Immunology
Background:
- Microscopic polyangiitis (MPA) is a rare systemic vasculitis affecting small blood vessels.
- Understanding the clinical and pathological spectrum of pediatric MPA is crucial for timely diagnosis and management.
Purpose of the Study:
- To investigate the clinical and pathological characteristics of microscopic polyangiitis (MPA) in pediatric patients.
- To analyze the treatment outcomes and efficacy of immunosuppressive therapy in this cohort.
Main Methods:
- Retrospective analysis of 20 pediatric patients diagnosed with MPA over a 10-year period.
- Review of clinical data, renal biopsy findings, serological markers, and treatment responses.
Main Results:
- The study included 20 patients (16 primary MPA, 4 antithyroid drug-associated MPA), with a female predominance in both groups.
- All patients presented with renal involvement; lung and central nervous system (CNS) involvement were also common.
- High positivity rates for perinuclear antineutrophil cytoplasmic antibody (p-ANCA) and myeloperoxidase (MPO) were observed.
- Steroid plus cyclophosphamide (CTX) pulse therapy achieved remission in 88.9% of patients.
Conclusions:
- Pediatric MPA, both primary and ATD-associated, exhibits a female predilection.
- Renal and pulmonary involvement are hallmarks of pediatric MPA, with CNS involvement being notable.
- Combined steroid and CTX therapy demonstrates significant efficacy as induction therapy for pediatric MPA.
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