Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2021 Jun;40(6):2293-2300.
doi: 10.1007/s10067-020-05484-8. Epub 2020 Nov 5.

Prognostic factors in IgG4-related disease: a long-term monocentric Chinese cohort study

Ji Zongfei  1   2 , Ma Lingying  1   2 , Zhang Lijuan  1   2 , Sun Ying  1   2 , Chen Rongyi  1   2 , Liu Dongmei  1   2 , Kong Xiufang  1   2 , Dai Xiaomin  1   2 , Ma Lili  1   2 , Chen Huiyong  1   2 , Jiang Lindi  3   4
Affiliations

Prognostic factors in IgG4-related disease: a long-term monocentric Chinese cohort study

Ji Zongfei et al. Clin Rheumatol. 2021 Jun.

Abstract

Objectives: Patients with IgG4-related disease (IgG4-RD) suffer high relapse rates during long-term treatment, but factors that predict relapse outcomes are not well established. In the present study, we aimed to identify predictive factors for treatment resistance and disease relapse in a Chinese IgG4-RD cohort.

Methods: This study enrolled 102 patients newly diagnosed with IgG4-RD. Disease prognosis was determined by evaluating disease activity and dosage of glucocorticoids. Predictive factors for refractory and relapsed disease were identified by univariate analysis and Cox regression.

Results: Among the 102 patients, 78 cases received medical treatment with regular follow-up (21 [6-111] months). During the follow-up period, 55 (70.5%) patients sustained clinical remission, and 23 (29.5%) patients suffered refractory or relapsed disease. The relapse rate of the patients with IgG4-RD was significantly higher among patients who stopped taking medicine than among those who continued treatment with glucocorticoids (GC) + immunosuppressor (IM). Serum TNF-α ≥ 13 pg/mL, sIL-2R ≥ 1010 U/mL, total cholesterol < 3.55 mmol/L, low-density lipoprotein < 2.0 mmol/L, IgG ≥ 20.2 g/L, and drug withdrawal were predictive factors for refractory and relapsed IgG4-RD. Multivariate Cox regression revealed that both sIL-2R and TNF-α were independent risk factors for refractory and relapsed disease. The combination of GC and IM treatment was an independent protective factor against refractory and relapsed IgG4-RD.

Conclusions: High serum levels of sIL-2R and TNF-α may be informative risk factors for refractory and relapsed IgG4-RD. Our data suggest that a combination treatment of GC along with IM may be protective against refractory and relapsed IgG4-RD. Key Points • High sIL-2R and TNF-α levels are informative risk factors for refractory and relapsed IgG4-related disease. • Combination treatment of GC with IM protects against refractory and relapsed IgG4-related disease.

Keywords: IgG4-related disease; Prognosis; Prognostic factors; Relapse; Treatment resistance.

PubMed Disclaimer

References

    1. Takahashi H, Yamamoto M, Suzuki C, Naishiro Y, Shinomura Y, Imai K (2010) The birthday of a new syndrome: IgG4-related diseases constitute a clinical entity. Autoimmun Rev 9:591–594 - DOI
    1. Lian L, Wang C, Tian JL (2016) IgG4-related retroperitoneal fibrosis: a newly characterized disease. Int J Rheum Dis 19:1049–1055 - DOI
    1. Li PH, Ko KL, Ho CT, Lau LL, Tsang RK, Cheung TT, Leung WK, Lau CS (2017) Immunoglobulin G4-related disease in Hong Kong: clinical features, treatment practices, and its association with multisystem disease. Hong Kong Med J 23:446–453 - PubMed
    1. Inoue D, Yoshida K, Yoneda N, Ozaki K, Matsubara T, Nagai K, Okumura K, Toshima F, Toyama J, Minami T, Matsui O, Gabata T, Zen Y (2015) IgG4-related disease: dataset of 235 consecutive patients. Medicine (Baltimore) 94:e680 - DOI
    1. Culver EL, Sadler R, Bateman AC, Makuch M, Cargill T, Ferry B et al (2017) Increases in IgE, eosinophils, and mast cells can be used in diagnosis and to predict relapse of IgG4-related disease. Clin Gastroenterol Hepatol 15:1444–1452.e6 - DOI

Substances

LinkOut - more resources