AOXD and IgG4-ROD share overlapping clinical, imaging, and histopathologic features. Comprehensive assessment - including serum IgG4, tissue IgG4+ plasma cell counts, and systemic organ involvement - is recommended to avoid misclassification. The role of IgG4 in AOXD remains unresolved; evolving criteria and mechanistic studies are needed to distinguish a true pathologic overlap from an epiphenomenon. Corticosteroids remain first-line, with selected cases benefitting from steroid-sparing agents.
Silva et al. (Wed,) studied this question.