Increased IgG4-Positive Plasma Cells in Granulomatosis with Polyangiitis : A Diagnostic Pitfall of IgG4-Related Disease

Abstract EN

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.