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Chinese Journal of Clinicians(Electronic Edition) ›› 2020, Vol. 14 ›› Issue (01): 25-29. doi: 10.3877/cma.j.issn.1674-0785.2020.01.006

Special Issue:

• Clinical Research • Previous Articles     Next Articles

Clinical analysis of IgG4-related disease

Dehua Zhang1, Jie Zhao1,(), Yang Sun1, Huihui Wang1, Xiaoxiao Wang1, Zhiyong Chen1, Liuting Guo1   

  1. 1. Department of Gastroenterology and Hepatology, the Second Hospital of Shanxi Medical University, Taiyuan 030001, China
  • Received:2019-12-19 Online:2020-01-15 Published:2020-01-15
  • Contact: Jie Zhao
  • About author:
    Corresponding author: Zhao Jie, Email:

Abstract:

Objective

To explore the clinical characteristics, serology, imaging, histopathology, treatment, and prognosis of IgG4-related diseases (IgG4-RD).

Methods

Clinical data of 76 patients diagnosed with IgG4-RD at the Second Hospital of Shanxi Medical University from 2013 to 2019 were retrospectively analyzed.

Results

Among the 76 patients, there were 39 males (51.3%) and 37 females (48.7%), with an average age of (56.1±11.8) years old. The most common involved organ was the submandibular gland (32.9%, 25/76), followed by the labial gland (15.8%, 12/76), pancreas (13.2%, 10/76), parotid gland (11.8%, 9/76), retroperitoneum (7.9%, 6/76), lymph nodes (5.3%, 4/76), sinus (2.6%, 2/76), lung (2.6%, 2/76), kidney (2.6%, 2/76), bile duct (2.6%, 2/76), skin (1.3%, 1/76), and colon (1.3%, 1/76). IL-6 was positively correlated with IgG4 (r=0.483, P<0.01), Interferon-γ was positively correlated with IgG4 (r=0.228, P<0.05), IL-2, IL-4, IL-10 and TNF-α had no correlation with IgG4 (P>0.05). Histopathological examination was performed in 61 cases, which were mainly characterized by infiltration of lymphocytes and plasmacytes and fibrosis,>10 IgG4(+) plasma cells per high-power field, or ratio of IgG4(+)/IgG(+) cells > 40%. Of all patients included, 23.7%(18/76) were treated with glucocorticoid alone, 28.9% (22/76) were treated with hormone combined with immunosuppressive therapy, 44.7% (34/76) were treated with hormone combined with immunoregulatory therapy, 2.6% (2/76) were untreated, and 90.8% (69/76) responded well to initial treatment.

Conclusion

IgG4-RD is an autoimmune disease with multiple organ involvement. The most frequently involved organs are the submandibular gland, labrum gland, pancreas, and parotid gland. Combined detection of IL-6 and interferon-γ may play a predictive role in the pathogenesis of IgG4-RD, and they may become new targets for clinical treatment of IgG4-RD.

Key words: Immunoglobulin G, IgG4-related disease, Interleukin-6, Interferon-γ, Clinical characteristics

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