title: Increased IgG4-Positive Plasma Cells in Granulomatosis with Polyangiitis: A Diagnostic Pitfall of IgG4-Related Disease.
abstract: Granulomatosis with polyangiitis (Wegener's) (GPA) may mimic IgG4-related disease (IgG4-RD) on histologic examination of some biopsies, especially those from head and neck sites. IgG4 immunostain is often performed in this context for differential diagnosis with IgG4-RD. However, the prevalence of IgG4+ cells in GPA has not been explored. We examined the IgG4+ cells in 26 cases confirmed as GPA by a thorough clinical and pathologic assessment. Twenty-six biopsies consisted of 14 sinonasal/oral cavity/nasopharynx, 7 orbit/periorbital, 3 lung/pleura, 1 iliac fossa/kidney, and 1 dura specimens. Eight of 26 (31%) biopsies revealed increased IgG4+ cells (>30/HPF and >40% in IgG4+/IgG+ ratio). The IgG4+ cells and IgG4+/IgG+ ratio ranged 37-137/hpf and 44-83%, respectively. Eight biopsies with increased IgG4+ cells were from sinonasal (n = 4) or orbital/periorbital (n = 4) sites. In conclusion, increased IgG4+ cells are not uncommonly seen in sinonasal or orbital/periorbital biopsies of GPA, which could pose as a diagnostic pitfall.
authors.affiliation: Division of Anatomic Pathology, Mayo Clinic, Rochester, MN 55905, USA.
authors.forename: Sing Yun
authors.initials: SY
authors.lastname: Chang
authors.forename: Karina
authors.initials: K
authors.lastname: Keogh
authors.forename: Jean E
authors.initials: JE
authors.lastname: Lewis
authors.forename: Jay H
authors.initials: JH
authors.lastname: Ryu
authors.forename: Eunhee S
authors.initials: ES
authors.lastname: Yi