Serum IgG4 levels at diagnosis can predict unfavorable outcomes of untreated patients with IgG4-related disease

Abstract

The outcomes of patients with immunoglobulin G4 (IgG4)-related disease (IgG4-RD) who are not treated are unclear. This study aimed to clarify these outcomes and identify the factors related to them. We retrospectively evaluated various clinical features including laboratory data and involved organs at diagnosis in 107 patients with IgG4-RD, who were followed up for more than 6 months, at a single center in Japan. We compared the clinical features of the 27 untreated patients with those of the 80 patients treated with glucocorticoid. The patient outcomes were investigated, and logistic regression analysis was performed to identify factors related to them. The patients comprised 73 men and 34 women (median age 67 years). The untreated patients had significantly lower IgG4-RD responder index (9 vs. 12) and fewer affected organs (1 vs. 3) than did those treated with glucocorticoid. Of these 27 patients, 8 experienced deterioration of IgG4-RD after the diagnosis. In the age- and sex-adjusted logistic regression analysis, serum IgG4 elevation (per 100 mg/dL, odds ratio 1.194, 95% confidence interval 1.017–1.402) was the only significant factor related to disease deterioration in untreated patients with IgG4-RD, whereas not serum IgG4 levels (per 100 mg/dL, odds ratio 0.995, 95% confidence interval 0.921–1.075) but history of allergy (OR 3.134, 95% confidence interval 1.094–8.977, P = 0.033) related to deterioration in patients who underwent treatment. Serum IgG4 levels may be a useful predictor of unfavorable outcomes in untreated patients with IgG4-RD, who tend to have fewer affected organs and lower IgG4-RD responder index.

Laboratory characteristics of IgG4-related disease: A retrospective study from a single tertiary medical center

“…Previous meta-analysis study reported a similar rate of efficacy of first-line therapies (96%) and relapse (33%). [9,24] In the present study, the occurrence of relapse was not related to laboratory findings at baseline including IgG4, IgG, FLC, FLC ratio, HLC, and HLC ratio. The role of IgG4 as a marker of disease relapse has shown inconsistent results.…”

“…[24] However, Mizushima et al reported that baseline serum IgG4 concentration can be predictive of unfavorable outcomes in untreated patients. [24] Also, Culver et al, in a prospective cohort, have reported that baseline serum IgG4 concentration was higher in patients who relapsed after therapy than patients who did not relapse (median 505 mg/dL vs 195 mg/dL). [19] This inconsistency among reports may be due to the different size of the patient population, the design of the study, and different treatment outcomes, and the role of the initial IgG4 level as a marker of disease relapse should be resolved in future studies.…”

“…In line with our result, Mizushima et al have also reported that there was no correlation between baseline serum IgG4 concentration and outcomes in treated patients. [24] However, Mizushima et al reported that baseline serum IgG4 concentration can be predictive of unfavorable outcomes in untreated patients. [24] Also, Culver et al, in a prospective cohort, have reported that baseline serum IgG4 concentration was higher in patients who relapsed after therapy than patients who did not relapse (median 505 mg/dL vs 195 mg/dL).…”

Spontaneous regression of immunoglobulin G4-related dacryoadenitis and multiple organ involvement: A case report

“…[7] Another report presented that 1 of 27 untreated patients with IgG4-RD obtained spontaneous improvement of the lacrimal gland lesion; however, the detailed clinical findings were not presented. [8] The mechanisms of spontaneous regression remain unclear. A previous report postulated that decreased absolute amount of IgG4-positive plasmacytes after biopsy induces an abnormal immune response.…”

“…[5,6] In contrast, although rare, IgG4-related dacryoadenitis is spontaneously resolved without steroids. [7,8] There have been only a few reported cases of spontaneous regression of IgG4related dacryoadenitis. [7,8] Here, we report an additional case of IgG4-related dacryoadenitis and multiple organ involvement with spontaneous resolution.…”