Background/Purpose: To evaluate the relationship between serum cholinesterase (ChE) level, number of organ involvement, serum fibrotic markers and imaginational outcomes in Japanese patients with IgG4-related disease (IgG4-RD).

Methods: The clinical symptoms, laboratory, pathological and FDG-PET/CT findings of Japanese patients with IgG4-RD (n=20) were assessed. Several laboratory data of IgG4-RD with multiple organs’ involvements (IM) (n=10), IgG4-RD with limited organ’s involvement (IL) (n=10), ANCA-associated vasculitis (AAV) (n=10) and Sjogren syndrome (SjS) (n=10) were comparatively examined. Furthermore, we studied the relationship between the numbers of organ involvement (NOI), several fibrotic markers (ELF score and serum Dickkopf-1 (Dkk-1)) and imaginational outcome in IgG4-RD group.

Results: Serum ChE levels were significantly lower in IM group than IL, AAV and SjS groups. In total IgG4-RD cases, ChE levels inversely correlated with NOI and fibrotic score, and fibrotic score positively correlated with NOI. Finally, Dkk-1, one of Wnt inhibitors, levels in IM were significantly lower than IL and healthy subjects (p<0.05). Moreover, low level of Dkk-1 before and after treatment could predict the progression of organ atrophy.

Conclusion: The ELF score and serum Dkk-1 level might be clinically useful indicators of active fibrosis and the extent of IgG4-RD. Notably, continuous lower levels of Dkk-1 were related to organ atrophy and serum ChE levels could predict these phenomena.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/the-relationship-between-serum-cholinesterase-dickkopf-1-and-number-of-organ-involvement-in-japanese-patients-with-igg4-related-disease/