Lung biopsy in the diagnosis of pediatric ANCA‐associated vasculitis. Issue 1 (9th November 2020)

• Record Type: Journal Article
• Authors: Sayad, Edouard; Vogel, Tiphanie P.; Cortes‐Santiago, Nahir; Patel, Kalyani R.; McNeill, David M.; Spielberg, David; Silva‐Carmona, Manuel
• Is Part Of: Pediatric pulmonology. Volume 56:Issue 1(2021)
• Page Start: 145
• Page End: 152
• Publication Date: 2020-11-09
• Subjects: anti‐neutrophil cytoplasmic antibody‐associated vasculitis (AAV) -- granulomatosis with polyangiitis (GPA) -- lung biopsy -- lung histopathology -- microscopic polyangiitis (MPA) -- pediatric AAV -- pediatric vasculitis -- pulmonary hemorrhage; Pediatric respiratory diseases -- Periodicals; Pediatrics -- Periodicals; 618.922
• DOI: 10.1002/ppul.25151
• Languages: English
• ISSNs: 8755-6863

Abstract

Abstract: 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.