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Updated: Apr 19, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
[Clinical characteristics of IgG4-related retroperitoneal fibrosis]
Hua Chen1, Wei Lin1, Linyi Yan1
1Department of Immunology & Rheumatology, Peking Union College Hospital & Chinese Academy of Medical Sciences, Key Laboratory of Immunological & Rheumatologic Diseases under Ministry of Education, Beijing 100032, China.
Objective:
To explore the clinical characteristics of IgG4-related retroperitoneal fibrosis (RPF) in China.
Methods:
From December 2010 to September 2013, a total of 107 IgG4-related disease (IgG4-RD) patients were reviewed prospectively. Their clinical, laboratory and histopathological features were summarized. And the treatment responses were analyzed.
Results:
Among them, 22 RPF cases were identified. The male-to-female ratio and age were 4.5: 1 and 63 ± 11 years respectively. The follow-up period was 3-28 months. The most common onset symptom was low back pain (55%). Multiple organ involvements were documented in 20 patients (91%), including lymphadenopathy (n = 9), aortitis (n = 8), sialadenitis (n = 7), dacryoadenitis (n = 5) and autoimmune pancreatitis (n = 4). Allergic histories were reported by10 subjects (46%). Eosinophilia was present in 6 subjects (27%). Serum IgG4 levels were significantly elevated in all subjects. Serum total IgE level was elevated in 12/13 tested subjects. Tissue biopsies of 12 subjects revealed massive lymphocytic infiltration and obvious tissue fibrosis. Immunohistochemical staining showed a massive infiltration of IgG4-positive plasma cell. Glucocorticoid was administrated in all subjects. Most subjects received combined therapy with immunosuppressive agents or tamoxifen.
Conclusion:
IgG4-related RPF is a systemic autoimmune disease with favorable responses to the treatments of glucocorticoid and immunosuppressive agents.
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