A Case Report of Eosinophilic Granulomatosis with Polyangiitisin a Pediatric Patient
DOI:
https://doi.org/10.47363/JPRRR/2022(4)142Keywords:
Churg Strauss Syndrome, Asthma, Vasculitis, Hyper Eosinophilia, Eosinophilic Granulomatosis PolyangiitisAbstract
Introduction: Eosinophilic Granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome (CSS), is a rare systemic vasculitis of small and medium-sized vessels that primarily develops in middle-aged individuals. It is characterized by asthma, blood eosinophilia, and extrapulmonary manifestations. In childhood, EGPA is extremely rare. Pulmonary and cardiac involvement is predominant in pediatric EGPA, and mortality is substantial. The key to the treatment of EGPA lies in the early diagnosis of the disease. When glucocorticoids and immunosuppressants are used early, they can improve
both the symptoms and the overall outlook of EGPA.
Case Presentation: We presented a case of an 8-year-old boy with a history of short-term asthma, marked eosinophilia, and multi-organ involvement. The extremely high eosinophil level in the blood (72.50%) prompted the examination of eosinophilic leukemia before the EGPA diagnosis was made. Subsequently, this disease was successfully treated.
Conclusion: EGPA in children has unique clinical, imaging, and histological characteristics different from those of adults. In pediatric patients, the development and diagnosis of systemic symptoms are often delayed, mainly occurring in the eosinophilic phase, which will lead to specific manifestations. At the same time, we cannot detect a genetic relationship related to EGPA.