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Assessment of Pathologically Diagnosed Patients with Castleman’s Disease associated with Diffuse Parenchymal Lung Involvement Using the Diagnostic Criteria for IgG4-Related Disease

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Abstract

Background

IgG4-related disease (IgG4RD) is a recently recognized disease entity. Differentiating IgG4RD from plasma cell type Castleman’s disease (PCD) is important but also difficult using only pathological findings. In addition, little is known about the association between these two diseases with diffuse parenchymal lung involvement.

Methods

We analyzed the serum IgG4 levels and the ratio of IgG4/IgG-positive plasmacytes in the lung and lymph node specimens of eight patients previously pathologically diagnosed of PCD with diffuse parenchymal lung involvement (DL-PCD). We also compared the clinical and laboratory findings observed in these patients.

Results

Six of the eight patients exhibited abundant IgG4-positive plasmacytes in the lung and lymph node tissues and elevated serum IgG4 levels, thereby fulfilling the diagnostic criteria of IgG4RD with DL (DL-IgG4RD) in addition to having obstructive phlebitis and massive lymphoplasmacytic infiltration with fibrosis. However, three of these six patients exhibited higher levels of serum interleukin-6 and were still diagnosed with DL-PCD. Accordingly, three of these eight patients were considered as IgG4RD with DL (DL-IgG4RD), and the other five patients were ultimately given a diagnosis of DL-PCD. These two diseases have different characteristics in terms of age, symptoms, serum levels of C-reactive protein, and IgA, complicating allergic disorders, response to corticosteroids, and prognosis.

Conclusions

This is the first report to show a high prevalence of DL-IgG4RD in DL-PCD patients, although additional large investigations are necessary. Clinical and laboratory findings are important for distinguishing between these two diseases in other organs, as previously described.

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Conflicts of interest

The authors declare no conflicts of interest.

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Authors and Affiliations

  1. Department of Respiratory Medicine, University of Occupational and Environmental Health, Japan, 1-1 Iseigaoka, Yahatanishiku, Kitakyushu, Fukuoka, 807-8555, Japan

    Takaaki Ogoshi, Takashi Kido, Kazuhiro Yatera, Keishi Oda, Toshinori Kawanami, Hiroshi Ishimoto & Hiroshi Mukae

  2. Respiratory Division, Saiseikai Shimonoseki General Hospital, Shimonoseki, Yamaguchi, Japan

    Takaaki Ogoshi

  3. Second Department of Internal Medicine, Nagasaki University School of Medicine, Nagasaki, Japan

    Noriho Sakamoto

  4. Third Department of Internal Medicine, Miyazaki University, School of Medicine, Kiyotake, Miyazaki, Japan

    Arisa Sano

  5. Department of Respiratory Medicine, Wakamatsu Hospital for the University of Occupational and Environmental Health, Japan, Kitakyushu, Fukuoka, Japan

    Chiharu Yoshii

  6. Department of Pathology and Cell Biology, University of Occupational and Environmental Health, Japan, Kitakyushu, Fukuoka, Japan

    Shohei Shimajiri

Authors
  1. Takaaki Ogoshi
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  2. Takashi Kido
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  3. Kazuhiro Yatera
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  4. Keishi Oda
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  5. Toshinori Kawanami
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  6. Hiroshi Ishimoto
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  7. Noriho Sakamoto
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  8. Arisa Sano
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  9. Chiharu Yoshii
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  10. Shohei Shimajiri
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  11. Hiroshi Mukae
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Correspondence to Kazuhiro Yatera.

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Ogoshi, T., Kido, T., Yatera, K. et al. Assessment of Pathologically Diagnosed Patients with Castleman’s Disease associated with Diffuse Parenchymal Lung Involvement Using the Diagnostic Criteria for IgG4-Related Disease. Lung 191, 575–583 (2013). https://doi.org/10.1007/s00408-013-9497-x

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  • DOI: https://doi.org/10.1007/s00408-013-9497-x

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