Differences and similarities between IgG4-related disease with and without dacryoadenitis and sialoadenitis: clinical manifestations and treatment efficacy

• Published in: Arthritis research & therapy Vol. 21; no. 1; p. 44
• Main Authors: Wang, Mu, Zhang, Panpan, Lin, Wei, Fei, Yunyun, Chen, Hua, Li, Jing, Zhang, Li, Zheng, Wenjie, Li, Yongze, Zeng, Xiaofeng, Zhou, Jiaxin, Lai, Yamin, Liu, Xiaowei, Xue, Huadan, Cui, Yueying, Zhou, Lian, Zhao, Jizhi, Zhang, Wen
• Format: Journal Article
• Language: English
• Published: England BioMed Central Ltd 01-02-2019; BioMed Central; BMC
• Subjects: Adult; Aged; Allergy; Analysis; Arthritis; Blood serum; Care and treatment; Corticosteroid drugs; Dacryoadenitis; Dacryocystitis; Dacryocystitis - blood; Dacryocystitis - complications; Dacryocystitis - drug therapy; Diagnosis; Drug Therapy, Combination; Eosinophils - metabolism; Female; Glucocorticoids; Glucocorticoids - therapeutic use; Humans; IgG4-RD; IgG4-RD RI; Immunoglobulin E; Immunoglobulin E - blood; Immunoglobulin G; Immunoglobulin G - blood; Immunoglobulin G4-Related Disease - blood; Immunoglobulin G4-Related Disease - complications; Immunoglobulin G4-Related Disease - drug therapy; Immunoglobulins; Immunologic diseases; Immunosuppressive agents; Immunosuppressive Agents - therapeutic use; Laboratories; Male; Medical imaging; Medical research; Medical schools; Middle Aged; NMR; Nuclear magnetic resonance; Pancreatitis; Recurrence (Disease); Rheumatology; Sialadenitis - blood; Sialadenitis - complications; Sialadenitis - drug therapy; Sialoadenitis; Steroids (Organic compounds); Treatment; Treatment Outcome; Treatment; Dacryoadenitis; IgG4-RD RI; IgG4-RD; Sialoadenitis
• ISSN: 1478-6362; 1478-6354; 1478-6362
• DOI: 10.1186/s13075-019-1828-8

This study aimed to compare the differences and similarities in the clinical manifestations and treatment efficacy of IgG4-related disease (IgG4-RD) in patients with and without dacryoadenitis and sialoadenitis (DS).
A total of 121 untreated IgG4-RD patients in Peking Union Medical College Hospital were enrolled in this study. The patients were divided into three groups: DS-predominant (group A), non-DS (group B), and DS with other internal organs affected (group C). The patients were followed up for at least 15 months. Baseline and follow-up data were collected. The disease activity was evaluated according to the IgG4-RD responder index.
The mean ± SD age at disease onset was 53.2 ± 14.1 years, and 71.9% of the patients were male. The prevalence of allergies was higher in groups A (21, 61.8%) and C (32, 69.6%) than group B (14, 34.1%). More patients with DS (17, 50.0%, and 17, 37.0%) had sinonasal lesions than those without DS (5, 12.2%). Moreover, an increased number of eosinophils were more common in patients with DS than in those without, as were increased serum IgG, IgG4, and IgE levels. More patients in group B and group C (28, 68.3%, and 31, 67.4%) received a combination therapy of corticosteroid and immunosuppressant. During the 15-month follow-up, 28 (23.1%) patients had disease relapse.
Results demonstrated that IgG4-RD patients with DS had distinctive clinical features compared with non-DS. Allergy and sinonasal involvement were more common in patients with DS. Patients with DS showed higher serum IgG4 levels than those without DS.