Lung biopsy in the diagnosis of pediatric ANCA-associated vasculitis

Edouard Sayad*, Tiphanie P. Vogel, Nahir Cortes-Santiago, Kalyani R. Patel, David M. McNeill, David Spielberg, Manuel Silva-Carmona

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

2 Scopus citations


Objective: To investigate pulmonary histopathologic features in a cohort of pediatric patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) who underwent a lung biopsy as part of their evaluation. We report the safety and the findings of lung biopsies in this population. Methods: After IRB approval, we performed a retrospective chart review of all patients <18 years of age presenting to our institution with a diagnosis of pediatric AAV (pAAV) who underwent lung biopsy. We reviewed histopathologic features, serologies, the timing of biopsy, and complications. Results: Fourteen patients met inclusion criteria, nine patients with microscopic polyangiitis (MPA), and five patients with granulomatosis with polyangiitis (GPA). All patients had positive ANCA serology. 13/14 patients required admission on initial presentation for respiratory symptoms; 11/13 required respiratory support. The indication for biopsy was confirmation of diagnosis before initiating therapy in 11 patients (78%), part of the infectious evaluation in two (14%), and part of interstitial lung disease evaluation in one (7%). 11/14 (78%) biopsies had findings consistent with AAV diagnosis: 6/9 (67%) of the MPA patients compared with 5/5 (100%) of the GPA patients. The most common findings on histopathology were vascular inflammation and signs of alveolar hemorrhage. The only reported complication after lung biopsy was pneumothorax in four patients (28%). Conclusion: Lung biopsy had a higher diagnostic yield in GPA compared with MPA patients. In our cohort, a diagnosis of AAV could be made with clinical features and positive serology but was confirmed by lung histopathology in the majority of cases. Obtaining a lung biopsy for diagnostic purposes in pAAV should be reserved for uncertain cases where the diagnosis cannot be confirmed clinically and with serology.

Original languageEnglish (US)
Pages (from-to)145-152
Number of pages8
JournalPediatric Pulmonology
Issue number1
StatePublished - Jan 2021
Externally publishedYes


  • anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV)
  • granulomatosis with polyangiitis (GPA)
  • lung biopsy
  • lung histopathology
  • microscopic polyangiitis (MPA)
  • pediatric AAV
  • pediatric vasculitis
  • pulmonary hemorrhage

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Pulmonary and Respiratory Medicine


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