Pediatric antineutrophil cytoplasmic antibody-associated vasculitis: A review on pulmonary manifestations, management, and outcomes

Abstract

Antineutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) is a rare systemic autoimmune disorder characterized by necrotizing inflammation of blood vessels infiltrated by neutrophils. It includes three distinct entities: granulomatosis with polyangiitis (GPA, formerly known as Wegener’s granulomatosis), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA, previously termed Churg–Strauss syndrome). The primary target antigens of AAV are perinuclear-ANCA/myeloperoxidase (MPO) and cytoplasmic-ANCA/proteinase 3 (PR3). MPO-ANCA is associated with MPA and EGPA, while PR3-ANCA is the marker antibody in GPA. AAV is a chronic disorder that frequently recurs or relapses and is potentially life-threatening, primarily affecting the renal and respiratory systems. Immunosuppressive therapy can improve the survival rate in pediatric AAV. However, severe cases with multiorgan involvement often have poor prognosis. Moreover, literature on pulmonary manifestations and outcomes in pediatric cases remains limited. This article aims to provide a comprehensive review of pediatric AAV, particularly focusing on pulmonary manifestations, and highlights recent advancements in therapeutic management.

Keywords

• anca-associated vasculitis; children; pulmonary hemorrhage