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Immunologic Research

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14.07.2017 | Original Article

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

verfasst von: Xiang Tong, Min Bai, Weiya Wang, Qingbing Han, Panwen Tian, Hong Fan

Erschienen in: Immunologic Research | Ausgabe 4/2017

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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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Titel: IgG4-related disease involving polyserous effusions with elevated serum interleukin-6 levels: a case report and literature review
verfasst von: Xiang Tong
Min Bai
Weiya Wang
Qingbing Han
Panwen Tian
Hong Fan
Publikationsdatum: 14.07.2017
Verlag: Springer US
Erschienen in: Immunologic Research / Ausgabe 4/2017
Print ISSN: 0257-277X
Elektronische ISSN: 1559-0755
DOI: https://doi.org/10.1007/s12026-017-8934-y

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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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