Abstract
BackgroundThis 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).MethodsA 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.ResultsThe 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.ConclusionResults 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.
Highlights
IgG4-related disease (IgG4-RD) is a fibro-inflammatory disease with single or multi-organ involvement; the pathology of the involved tissue includes a large amount of lymphocyte and plasma cell infiltration, storiform fibrosis, and obliterative phlebitis, accompanied by eosinophilia of the related tissue [1,2,3]
The diagnosis of IgG4-RD was based on the following criteria: (1) a clinical examination showing characteristic diffuse/localized swelling or masses in single or multiple organs, (2) an elevated serum IgG4 concentration (> 135 mg/dL), and (3) a histopathologic examination showing (a) marked lymphocytic and plasma cell infiltration and fibrosis or (b) the infiltration of IgG4+ plasma cells
Clinical characteristics of the IgG4-RD patients A total of 121 newly diagnosed IgG4-RD patients were enrolled in our study
Summary
IgG4-related disease (IgG4-RD) is a fibro-inflammatory disease with single or multi-organ involvement; the pathology of the involved tissue includes a large amount of lymphocyte and plasma cell infiltration, storiform fibrosis, and obliterative phlebitis, accompanied by eosinophilia of the related tissue [1,2,3]. Dacryoadenitis and sialoadenitis (DS) is the bilateral, painless, and symmetrical swelling of the lacrimal and salivary glands, previously referred to as “Mikulicz’s disease” by some researchers [2, 6,7,8] Many of these patients were found to have elevated serum IgG4 levels and infiltration of IgG4-expressing plasma cells in the lacrimal and salivary glands and are recognized to have IgG4-RD [9,10,11], defined in this clinical presentation as IgG4-related dacryoadenitis and sialoadenitis (DS) [2, 12,13,14]. There has been no larger prospective cohort study to explore the onset symptoms, laboratory parameters, or treatment efficacy in IgG4-RD patients with and without DS To further understand these distinctions, we analysed the clinical presentation, laboratory examination results, and treatment efficacy in patients with and without DS. 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)
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