Involvement of two or more sets of lacrimal glands and/or major salivary glands is related to greater systemic disease activity due to multi-organ involvement in IgG4-related dacryoadenitis/sialadenitis

Objectives In IgG4-related dacryoadenitis and/or sialadenitis (IgG4-DS), involvement of two or more sets of lacrimal glands (LGs) and/or major salivary glands (MSGs) is regarded as a specific finding with diagnostic significance. This study aimed to clarify the influence of this factor on the overall clinical picture of IgG4-DS. Methods We retrospectively reviewed the medical records of 130 patients with IgG4-related disease, 97 of whom were diagnosed with IgG4-DS. We determined their clinical features according to the presence/absence of involvement of >= 2 sets of LGs and/or MSGs and compared the results with those obtained in 33 DS-limited patients. Results The IgG4-DS patients comprised 60 men and 37 women (median age 65 years). The median serum IgG4 level at diagnosis was 548 mg/dL. The patients with involvement of >= 2 sets (n = 44) had significantly more affected organs, lower serum C3 and C4 levels, and a tendency to have higher serum IgG levels and IgG4-RD responder index than did those without it (n = 53). In the 33 DS-limited patients, these two groups had no significant differences in clinical features. Conclusions Involvement of >= 2 sets of LGs and/or MSGs suggests greater systemic disease activity mainly reflected by involvement of more organs.

Automated summary

In patients with IgG4-related dacryoadenitis and/or sialadenitis, involvement of two or more sets of lacrimal glands and/or major salivary glands suggests greater systemic disease activity, characterized by more affected organs, lower serum C3 and C4 levels, and a tendency towards higher serum IgG levels and IgG4-RD responder index.