Background/Purpose: To evaluate the relationship between cholinesterase, number of organ involvement and serum fibrotic markers 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 (IRDMOI) (n=10), IgG4-RD with limited organ’s involvement (IRDLOI) (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) and several fibrotic markers (ELF score and serum Dkk-1) in IgG4-RD group.

Results: Serum cholinesterase (ChE) levels were significantly lower in IRDMOI group than IRDLOI, AAV and SjS groups. All cases did not show hepatic dysfunction in laboratory examinations. Serum albumin and IgG levels were significantly lower and CRP levels were significantly higher in AAV group, compared with IgG4-RD and SjS group. There were no significant differences in these levels between IRDMOI and SjS. 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 IRDMOI were significantly lower than IRDLOI and healthy subjects (p<0.05). 

Conclusion: The ELF score and serum Dkk-1 level might be a clinically useful indicators of active fibrosis and the extent of disease in Japanese patients with IgG4-RD. Notably, serum ChE levels could predict these phenomena.

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