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IgG4-related disease involving polyserous effusions with elevated serum interleukin-6 levels: a case report and literature review

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Abstract

Immunoglobulin G4-related disease (IgG4-RD) is a recently described immune-mediated fibroinflammatory disease with a characteristic histopathologic appearance that can affect various organs. We report a 43-year-old Chinese female patient with IgG4-RD involving polyserous effusions with reports of worsening exertional dyspnea for 3 months. Laboratory blood tests revealed that serum interleukin (IL)-6, carbohydrate antigens (CA-199 and CA-125), and alpha-fetoprotein levels were significantly increased, but serum IgG4 levels were normal. Repeat pleural effusion and ascite analysis showed lymphocyte-predominant exudates. In addition, computed tomography scan showed massive pleural effusion in the right pleural cavity, abdominal effusion, and some pericardial effusion with a partial compression atelectasis. Further, medical thoracoscopy was performed to examine the pleural cavity and found multiple nodules on the pleura and partly thickened pleura with a reddish color. Histopathologic and immunohistochemical examination revealed marked lymphocytes and IgG4-positive plasma cell infiltration. The patient was finally diagnosed with IgG4-RD according to the comprehensive diagnostic criteria, although the patient presented similar serological and pathological manifestations of Castleman disease (CD). Our case suggests that IgG4-RD may be one of the causes of polyserous effusions and shows the difficulties in differentiating between IgG4-RD and CD.

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Acknowledgments

We thank Elsevier Language Editing Services for its linguistic assistance during the preparation of this manuscript.

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Authors and Affiliations

  1. Department of Respiratory and Critical Care Medicine, West China Hospital/West China School of Medicine, Sichuan University, Guoxuexiang 37, Chengdu, Sichuan, 610041, China

    Xiang Tong, Min Bai, Qingbing Han, Panwen Tian & Hong Fan

  2. Department of Pathology, West China Hospital/West China School of Medicine, Sichuan University, Chengdu, 610041, China

    Weiya Wang

  3. Lung Cancer Center, West China Hospital/West China School of Medicine, Sichuan University, Chengdu, 610041, China

    Panwen Tian

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  1. Xiang Tong
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Correspondence to Panwen Tian or Hong Fan.

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Tong, X., Bai, M., Wang, W. et al. IgG4-related disease involving polyserous effusions with elevated serum interleukin-6 levels: a case report and literature review. Immunol Res 65, 944–950 (2017). https://doi.org/10.1007/s12026-017-8934-y

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