W e wish to thank Prof. Akiyama for his comments. This is a reported case of immunoglobulin G4-related disease (IgG4-RD) involving the nasal septum and trachea, which was, here, successfully managed with glucocorticoids. 1 Yes, we agree with Akiyama's position that our case is IgG4-related midline destructive disease, which is also called midline destructive lesion (MDL) in most works. [2][3][4] Midline destructive lesion is a clinical entity of unknown origin with nonspecific histopathological features. It is characterized by ulceration and progressive destruction of the nose, paranasal sinuses, and palate. 4 As Akiyama stated in the letter, although the etiologies of MDL are related to specific known diseases, some conditions of MDL still remain unexplained and are therefore labeled "idiopathic." Recently, a series of cases presenting as MDL and attributed to IgG4-RD have greatly expanded the spectrum of IgG4-RD manifestations. [4][5][6][7] It is essential to distinguish this entity from other conditions known to cause MDL because they require different approaches to treatment and management.We appreciate Akiyama's description of the correlation between antineutrophil cytoplasmic antibody-associated vasculitis (ANCA-associated vasculitis) and IgG4-RD. Antineutrophil cytoplasmic antibody-associated vasculitis includes granulomatosis with polyangiitis (GPA),