Does IgG4 level at the time of diagnosis correlate with disease outcome in IgG4-Related disease?

BACKGROUND/OBJECTIVES: Serum IgG4 level is used as a diagnostic criterion for immunoglobulin G4-related disease (IgG4-RD) but whether it predicts disease progression is unclear. Aim of the study was to investigate if serum IgG4 level at the time of diagnosis correlates with disease outcome.

METHODS

Patients with a definitive diagnosis of IgG4-RD were included in this study. They were divided into two groups - Group 1: Elevated serum IgG4 at diagnosis and Group 2: Normal serum IgG4 at diagnosis. Outcome parameters including multiple organ involvement, exocrine and endocrine dysfunction, relapse and mortality were compared. Data was subanalysed for outcomes on 2 levels of serum IgG4 cut-off - A: The upper limit of normal (ULN) and B: Twice the ULN.

RESULTS

Of 47 patients, 31 (66%) patients had elevated serum IgG4 at diagnosis. There was no statistically significant difference between the two groups in any of the outcome parameters. Data analysed with the serum IgG4 levels > ULN showed no difference between the 2 groups for any of the outcome parameters. However, when the serum IgG4 cut-off was set to twice the ULN, there was a significantly higher rate of disease relapse (42.9% vs 11.5%, p = 0.02) and pancreatic exocrine insufficiency (PEI) (76.2% vs 42.3%, p = 0.041).

CONCLUSION

Raised serum IgG4 greater than two times the ULN was significantly associated with disease relapse and PEI in patients with IgG4-RD. Larger multicentre studies with longer follow-up are required to corroborate these findings and define the role and cut-off value of serum IgG4 in outcomes of IgG4-RD.