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A case of IgG4-related kidney disease complicated by eosinophilic lung disease

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Abstract

IgG4-related disease is a systemic chronic inflammatory disorder characterized by a high blood level of IgG4 and the organ injuries by marked infiltration of IgG4-positive plasma cells and fibrosis. A 71-year-old male was hospitalized for a cough, malaise and anorexia. IgG4-related disease was suspected due to marked elevation of the serum IgG4 level. However, on lung biopsy, only eosinophil infiltration was demonstrated with no plasma cell infiltration. Otherwise abdominal contrast-enhanced CT showed mild enlargement of the bilateral kidneys and many differed contrasted areas and FDG PET-CT. Moreover, renal biopsy specimens showed typical tubulointerstitial nephritis with a large number of IgG4-positive plasma cells infiltration (the IgG4/IgG-positive cell rate, 89 %) and fibrosis. We diagnosed this patient as typical IgG4-related kidney disease. He was treated by the moderate dose of prednisolone (0.8 mg/kg/day) alone, and showed prompt response in the clinical condition, and both the lung and kidney lesions. In this case, it was useful for diagnosis of IgG4-related diseases to evaluate an image such as abdominal contrast-enhanced CT and FDG PET-CT. Our case might be one of the possible patterns of IgG4-related lung diseases. In addition, we thought that there might be an association between hypereosinophilia and IgG4-related kidney disease.

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References

  1. Hamano H, Kawa S, Horiuchi A, Unno H, Furuya N, Akamatsu T, et al. High serum IgG4 concentration in patients with sclerosing pancreatitis. N Engl J Med. 2001;344:732–8.

    Article  CAS  PubMed  Google Scholar 

  2. Takeda S, Haratake J, Kasai T, Takaeda C, Takazakura E. IgG4-associated idiopathic tubulointerstitial nephritis complicating autoimmune pancreatitis. Nephrol Dial Transpl. 2004;19:474–6.

    Article  Google Scholar 

  3. Masaki Y, Dong L, Kurose N, Kitagawa K, Morikawa Y, Yamamoto M, et al. Proposal for new clinical entity, IgG4-positive multiorgan lymphoproliferative syndrome:analysis of 64 cases of IgG4-related disorders. Ann Rheum Dis. 2009;68:1310–5.

    Article  CAS  PubMed  Google Scholar 

  4. Umehara U, Okazaki K, Masaki Y, Kawano M, Yamamoto M, Saeki T, et al. Comprehensive diagnostic criteria for IgG4-related disease(igG4-RD), 2011. Mod Rheumatol. 2012;22:21–30.

    Article  CAS  PubMed  Google Scholar 

  5. Kawano M, Saeki T, Nakashima H, Nishi S, Yamaguchi Y, Hisano S, et al. Proposal for diagnostic criteria for IgG4-related kidney disease. Clin Exp Nephrol. 2011;15:615–26.

    Article  PubMed  Google Scholar 

  6. Nishimoto N, Terao K, Mima T, Nakahara H, Takagi N, Kakehi T. Mechanisms and pathologic significances in increase in serum interleukin-6(IL-6) and soluble IL-6 receptor after administration of an anti-IL-6 receptor antibody, tocilizumab, in patients with rheumatoid arthritis and Castleman disease. Blood. 2008;112:3959–64.

    Article  CAS  PubMed  Google Scholar 

  7. Kojima M, Nakamura N, Tsukamoto N, Itoh H, Matsuda H, Kobayashi S, et al. Atypical lymphoplasmacytic and immunoblastic proliferation of autoimmune disease: clinicopathologic and immunohistochemical study of 9 cases. J Clin Exp Hematopathol. 2010;50:113–9.

    Article  Google Scholar 

  8. Strehl JD, Hartmann A, Agaimy A. Numerous IgG4-positive plasma cells are ubiquitous in diverse localized non-specific chronic inflammatory conditions and need to be distinguished from IgG4-related systemic disorders. J Clin Pathol. 2011;64:237–43.

    Article  PubMed  Google Scholar 

  9. Takahashi N, Kawashima A, Fletcher JG, Chari ST. Renal involvement in patients with autoimmune pancreatitis. Radiology. 2007;242:791–801.

    Article  PubMed  Google Scholar 

  10. Saeki T, Nishi S, Imai N, Ito T, Yamazaki H, Kawano M, et al. Clinicopathological characteristics of patients with IgG4-related tubulointerstitial nephritis. Kidney Int. 2013;84:826–33.

    Article  CAS  PubMed  Google Scholar 

  11. Nguyen VX, Petris GD, Nguyen BD. Usefulness of PET/CT imaging in systemic IgG4-related sclerosing disease. A report of three cases. JOP. 2011;12:297–305.

    PubMed  Google Scholar 

  12. Saeki T, Kawano M, Mizushima I, Yamamoto M, Wada Y, Nakashima H, et al. The clinical course of patients with IgG4-related kidney disease. Kidney Int. 2013;84:824–33.

    Article  Google Scholar 

  13. Inoue D, Zen Y, Abo H, Gabata T, Demachi H, Kobayashi T, et al. Immunoglobulin G4-related lung disease: CT findings with pathologic correlations. Radiology. 2009;251:260–70.

    Article  PubMed  Google Scholar 

  14. Zen Y, Inoue D, Kitao A, Onodera M, Abo H, Miyayama S, et al. IgG4-related lung and pleural disease: A clinicopathologic study of 21 cases. Am J Surg Pathol. 2009;33:1886–93.

    Article  PubMed  Google Scholar 

  15. Deshpande V, Khosroshahi A, Nielsen GP, Hamilos DL, Stone JH. Eosinophilic angiocentric fibrosis is a form of IgG4-related systemic disease. Am J Surg Pathol. 2011;35:701–6.

    Article  PubMed  Google Scholar 

  16. Khosroshahi A, Stone JH. A clinical overview of IgG4-related systemic disease. Curr Opin Rheumatol. 2011;23:57–66.

    Article  CAS  PubMed  Google Scholar 

  17. Takekawa M, Imai K, Adachi M, Aoki S, Maeda K, Hinoda Y, et al. Hypereosinophilic syndrome accompanied with necrosis of finger tips. Intern Med. 1992;31:1262–6.

    Article  CAS  PubMed  Google Scholar 

  18. Campbell HD, Tucker WQ, Hort Y, Martinson ME, Mayo G, Clutterbuck EJ, et al. Molecular cloning, nucleotide sequence, and expression of the gene encoding human eosinophil differentiation factor (interleukin 5). Proc Natl Acad Sci USA. 1987;84:6629–33.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  19. Tanaka A, Moriyama M, Nakashima H, Miyake K, Hayashida JN, Maehara T, et al. Th2 and regulatory immune reactions contributes to IgG4 production and the initiation of Mikulicz’s disease. Arthritis Rheum. 2012;64:254–63.

    Article  CAS  PubMed  Google Scholar 

  20. Nagao Y, Yamanaka H, Harada H. A patient with hypereosinophilic syndrome that manifested with acquired hemophilia and elevated IgG4: a case report. J Med Case Rep. 2012;6:1–7.

    Article  Google Scholar 

  21. Nakashima H, Miyake K, Moriyama M, Tanaka A, Watanabe M, Abe Y, Sato H, et al. An amplification of IL-10 and TGF-β in patients with IgG4-related tubulointerstitial nephritis. Clin Nephrol. 2010;73:385–91.

    Article  CAS  PubMed  Google Scholar 

  22. James N, Allen W, Bruce D. Eosinophilic lung diseases. Am J Respir Crit Care Med. 1994;150:1423–38.

    Article  Google Scholar 

  23. Stone JH, Zen Y, Deshpande V. IgG4-Related Disease. N Engl J Med. 2012;366:539–51.

    Article  CAS  PubMed  Google Scholar 

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Conflict of interest

The authors have declared that no conflict of interest exists.

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Authors and Affiliations

  1. Department of Nephrology, Kanazawa Medical University School of Medicine, 1-1 Daigaku, Uchinada, Ishikawa, 920-0293, Japan

    Hiroki Adachi, Hiroshi Okuyama, Hideki Yamaya & Hitoshi Yokoyama

  2. Department of Pathology and Laboratory Medicine, Kanazawa Medical University School of Medicine, Uchinada, Ishikawa, Japan

    Nozomu Kurose

  3. Department of Respiratory Medicine, Kanazawa Medical University School of Medicine, Uchinada, Ishikawa, Japan

    Koji Kojima & Hirohisa Toga

Authors
  1. Hiroki Adachi
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  2. Hiroshi Okuyama
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  3. Hideki Yamaya
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  4. Nozomu Kurose
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  5. Koji Kojima
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  6. Hirohisa Toga
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  7. Hitoshi Yokoyama
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Correspondence to Hiroki Adachi.

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Adachi, H., Okuyama, H., Yamaya, H. et al. A case of IgG4-related kidney disease complicated by eosinophilic lung disease. CEN Case Rep 4, 162–168 (2015). https://doi.org/10.1007/s13730-014-0160-z

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  • DOI: https://doi.org/10.1007/s13730-014-0160-z

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